10-16-02 cleft lip and palate part 2 teknik

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Page 1: 10-16-02 Cleft Lip and Palate Part 2 TEKNIK
Page 2: 10-16-02 Cleft Lip and Palate Part 2 TEKNIK

PRIMARY CLEFT LIP REPAIR

The goals of cleft lip repair are normal appear-ance and function of the lip. Although techniquesvary widely among surgeons, most agree that pri-mary surgical repair is the most important deter-minant of an aesthetic and functional outcome.The current trend for primary reconstruction ofthe lip and nose during the first surgery yieldsexcellent results. Also advocated are musclereconstruction and adequate release of the lip andnasal elements from the abnormal maxillary plat-form during primary repair. The extent of muscledissection and whether to dissect in a subperi-osteal or preperiosteal plane are still debated. Sur-gical advances include the use of presurgical naso-alveolar molding and lip taping, a critical evalua-tion of the cleft lip anatomy, and tailoring therepair to address the specific deformity. Surgeonsare constantly refining and modifying their tech-niques in their quest for symmetry and balancewith minimal scarring.1

Outpatient cleft lip repair is gaining in popularityas a safe option for healthy patients. Proponentscaution that the decision to perform an outpatientrepair should be based on careful consideration ofcomorbidities, the extent of the operation, and thehome care situation.2 The patient’s early postopera-tive medical status guides the decision about same-day hospital discharge.3 Rosen et al3 retrospectivelycompared the complication rates of inpatient andoutpatient cleft lip repair at two major tertiary refer-ral centers and found no significant difference. Kimand Rothkopf4 performed a 10-year retrospectivestudy comparing outpatient and inpatient cleft liprepair and reported no complications in the outpa-tient group. They also reported that time to firstfeeding was shorter in the outpatient group.

Some surgeons5 advocate the use of octyl-2-cyanoacrylate tissue adhesive as an alternative tosutures for skin closure. Its advantages are no sutureremoval, minimal wound care, and rapid postopera-tive feeding.

CLEFT LIP AND PALATEPART 2: SURGICAL MANAGEMENT

Amanda A Gosman MD

MICROFORM CLEFT LIP

According to Mulliken,6 characteristics of themicroform unilateral cleft lip include 1) a notchedmucosa; 2) thin medial vermilion; 3) elevatedCupid’s bow peak; 4) furrowed philtral column;5) hypoplastic orbicularis oris; and 6) minor nasaldeformity. Although correction of the microformdeformity requires orbicularis oris reconstruction,the results (and scar) of lip repair in children withthis minor cleft are frequently worse that the origi-nal deformity. Mulliken6 describes a techniquefor microform repair that limits the scar to thelower half of the lip and uses a subcutaneous tun-nel to the nostril sill for muscle repair. It consistsof a double-limb Z-plasty at the white roll (vermil-ion–skin junction) and the red line (vermilion–mucosa junction), eversion of the orbicularis oris,dermal graft augmentation of the philtral column,medial positioning of the alar base, and elevationof the lower lateral cartilage (Fig 1). Mullikenreports excellent results using this minimal scartechnique.

Chae7 described an innovative technique formicroform repair that involves an intraoral mucosalincision for muscle and philtral reconstruction. Notch-ing of the vermillion border is corrected with a smallZ-plasty and the nasal deformity is corrected througha reverse-U incision and V-Y plasty.

Onizuka and colleagues8 review the preferredoperative methods for repair of microform clefts.Cleft severity is rated according to the degree of down-ward depression of the nostril rim, skin striae of theupper lip, notching of Cupid’s bow, and deformityof the vermilion border. The authors suggest thatfirst-degree microform clefts should be repaired byrhinoplasty, whereas second-degree deformitiesshould be repaired by rhinoplasty and either a Z-plasty or other small operation on the lip withoutwhole lip skin incision. Third-degree deformities areviewed as incomplete clefts and should be managedby full-thickness lip repair.

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UNILATERAL CLEFT LIP

Clifford and Pool9 describe the anatomy of uni-lateral cleft lip and its correction by Z-plasty.Besides establishing the normal vertical height oflips according to age, the authors categorize thevarious surgical repairs in terms of the Z-plastymethod (Fig 2).9 Burt and Byrd10 review the uni-lateral cleft lip deformity and its current manage-ment.

Straight-line Repair

Modifications of the straight-line repair introducedby Thompson11 continue to enjoy limited use inpresent-day plastic surgery for the correction of theminimal cleft lip deformity. The technique has wid-est application in correcting notch deformities at thevermilion. Nakajima and associates12 showed niceresults in the repair of incomplete unilateral cleft lipusing a modification of the straight-line technique.

Fig 1. Markings. (Left) Anatomic points at cutaneous-vermilion-mucosa junction on the side of microform cleft lip. (Right) Design oftwo lateral isosceles triangles (cutaneous and vermilion). Note alternatives to tighten alar base: lenticular excision in sill or Y-Vadvancement (insert). (Reprinted with permission from Mulliken JB: Double unilimb z-plastic repair of microform cleft lip. PlastReconstr Surg 116:1623, 2005.)

Fig 2. The Z-plasty principle in unilateral cleft lip repair.

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Triangular-flap Repair

The Tennison triangular flap repair, with Randall’s13

suggested revisions, is still popular today14 (Fig 3). Intheir long and distinguished experience, Brauer andCronin15–17 stressed the value of geometric planningas proposed by Randall and obtained excellent resultswith the Tennison lip repair. They recommenddesigning the repair so that the cleft side will be 1mmshorter than the non-cleft side to prevent excessivevertical height of the lip.

Fig 3. Tennison triangular flap repair. (Reprinted with permissionfrom Senders CW, Sykes JM: Unilateral cleft lip. In: Cotton RT,Myer CM III (eds), Practical Pediatric Otolaryngology. Phila-delphia, Lippincott-Raven, 1999. Ch 48, p 789.)

Saunders and colleagues18 reviewed the variousviewpoints on lip growth after repair by the triangu-lar flap technique and evaluated the results in 50children who had triangular flap repair of unilateralcleft lip, with follow-up of 5–14 years. Unlike Brauerand Cronin,17 the authors conclude that a repairedunilateral cleft lip retains the configuration and lengthgiven at the initial repair.

Indications for cleft lip repair by the triangular flapmethod are impossible to define. Some believe it isnever appropriate because of the tendency forunpredictable excessive lengthening of the lateral lipsegment and a resultant scar that crosses the pro-jected line for the philtrum.19 Some surgeons reservethe technique for use in extremely wide clefts, whilestill others use it routinely.

Rotation-Advancement Repair

The rotation-advancement repair consists of anupper triangular flap inset into the rotation defectof the medial segment. The technique allows thescar to follow the projected line of the philtral col-umn except in its uppermost portion, where it arcs

beneath the columella. Details of the procedurecan be found in Millard’s20 “Extensions of theRotation–Advancement Principle for Wide Unilat-eral Cleft Lips.” Critics of the rotation-advancementmethod mention its technical difficulty in wide clefts,the necessity for wide soft-tissue undermining, ten-sion across the nostril sill, and a tendency toward aconstricted nostril on the side of repair.

Later refinements of the rotation-advancement prin-ciple can be found in Millard’s19 Cleft Craft I andconsist of even greater use of the mucosal parings inrepairing the associated nasal deformity. This repair,with its more recent modifications, is one of the mostpopular approaches to the correction of unilateralcleft lip deformity among American surgeons today.The markings for Byrd’s10 modification of Millard’srotation-advancement are shown in Fig 4.

Salyer and colleagues1 report on their 34-yearexperience with unilateral cleft lip and nose repair.The technique described is significantly different fromthe initial rotation-advancement. The authorsemphasize that adequate release and mobilizationof the nasal and lip elements prior to reconstructionis critical to avoid secondary deformities. A straight-line incision is used on the medial segment along thebase of the columella with no back-cut or extensioninto the columella. A straight-line lateral incision ismade from the point marking the new lateral Cupid’sbow peak along the white-roll and is extended intra-nasally above the inferior turbinate. No transverseskin incision is used along the alar base. After releaseof the muscle (preperiosteal) and nasal elements,additional incisions in the skin, muscle, and mucosamay be necessary to achieve adequate lip length andsymmetric rotation the medial lip. The final skindesign is decided on after ensuring muscle and nasalsymmetry. Salyer estimates that approximately 35%of patients require early minor secondary proceduresbefore age 5.

Mulliken21 reported his technical modification ofthe rotation-advancement repair. He first performsa labionasal adhesion and then a definitive lip andnasal repair 2 months later. The elements of histechnique include the use of a “high” rotation inci-sion with a backcut towards the columella, release ofthe labial frenulum, and lateral triangular flaps toincrease the medial vertical length at the vermilion–cutaneous junction and to augment the deficientmedial dry vermillion. The need for revision wasdetermined by photos shown to a panel; 42% of

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patients were judged to need revision, most com-monly for correction of the mucosal free margin.

Functional Matrix Cleft Repair

Carstens22 described a new technique for primarylip, nose, and alveolar repair that is based on restor-ing the anatomic content and growth potential ofdevelopmental fields. The principles of this repairare to 1) correct the underlying pathologic processesof the cleft including tissue deficiency, failure of uni-fication, displacement of structures by unequal forcevectors, and distortion of structures over time; 2)respect embryonic separation planes; 3) preserveblood supply of osteosynthetic cambium layer bydissecting subperiosteally (not supraperiosteally); 4)restore force vectors and reunify anatomic structures;5) reassign developmental fields to their correct posi-tion; and 6) reconstruct functional matrix to achievenormal growth. The markings for this “cut as yougo” technique are shown in Fig 5.

The incisions are similar to a straight-line repairand are determined by embryology and vascular sup-ply. Subperiosteal dissection is used through bilat-eral sulcus incisions to create sliding sulcus flaps forclosure of the alveolus and centralization of the func-tional matrix. This technique has been used in combi-

nation with recombinant human bone morphogeneticprotein (rhBMP-2) as an alternative to autogenousbone graft for primary closure of the alveolar cleft.23

Fig 5. Unilateral cleft: functional matrix incisions. (Reprintedwith permission from Carstens MH: Functional matrix cleftrepair: principles and techniques. Clin Plast Surg 31:159, 2004.)

The Vermilion

Noordhoff24 discusses the anatomy of the vermil-ion and the proper orientation of the white skin rolland red line. The vermilion is widest at the base ofthe philtral column; there is an ever-present defi-ciency of vermilion on the cleft side. Noordhoff uses

Fig 4. (Above, left) Current markings for unilateral complete cleft lip. (Center) Nasal release along the piriform margin. (Above, right)Inset of the L-flap to augment the nasal lining along the access of the piriform margin. The M-flap traditionally augments the labial sulcus.(Below, left) Wide undermining of the nasal skin overlying the lateral cartilage, involving the use of an infracartilaginous incision. (Below,right) The completed repair. (Reprinted with permission from Burt JD, Byrd HS: Cleft lip: unilateral primary deformities. Plast ReconstrSurg 105:1043, 2000.)

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a lateral vermilion flap to augment the deficient ver-milion beneath the white skin roll on the cleft side ofthe Cupid’s bow. While the necessity for the lateralvermilion flap may be argued,25 there is little doubtthat proper attention to the vermilion component oflip repair adds significantly to the result.

Muscle Reconstruction

Delaire26 popularized the physiologic reconstruc-tion of the paranasal and labial muscles during pri-mary lip repair. Kernahan and Bauer27 emphasizethe need for a functional orbicularis reconstructionand realign the muscle fibers based on the results ofelectric stimulation of the orbicularis at the time ofrepair.

Muller28 advocates differential reconstruction of theorbicularis oris muscle in unilateral cleft lip, believingthat the most important step in correction is the dif-ferential rearrangement of the muscle componentsto reset their insertion and reorient the muscle fibers.The three different components of the orbicularisoris muscle should be repaired independently to per-mit each to function separately. The repair shouldconsist of insertion of the nasal bundle into the ante-rior nasal spine, correction of the misdirectednasolabial bundle, and end-to-end union of the deepfibers of the vermilion.

Park and Ha29 emphasize the importance ofaccurate repair of two different components of theorbicularis oris muscle. The superficial componentof the orbicularis muscle serves as lip retractor whilethe deep component serves as a constrictor. Theantagonistic actions of these two orbicularis musclegroups during lip movement affect the balance anddynamics of the repaired lip if not properly and ana-tomically aligned at the time of surgery.

Salyer and others1 believe that wide (>5mm) dis-section between the muscle and skin is unnecessaryand leads to unpredictable scarring. In contrast,Seagle and Furlow30 incorporate a basket-weavemethod of muscle reconstruction into their rotation–advancement repair. The lateral skin and mucosaare dissected from the orbicularis oris muscle forabout 10mm. The medial dissection stops at thenon-cleft philtral column. The medial and lateralmuscle flaps are split transversely into three muscleslips which are then interdigitated and sutured to thedermis of the overlying lip skin. This techniquereportedly results in a more functional reconstruc-

tion and eliminates the lateral muscle bulge com-monly seen after lip repair. The authors alsoemphasize the importance of paranasal musclereconstruction.

Outcome Comparisons

Holtmann and Wray31 compared the triangularflap and rotation–advancement techniques in therepair of unilateral cleft lip. Unfortunately, the ran-dom selection yielded dissimilar study groups, so thatan apparent meaningful difference between thegroups may have been due to the presence ofhypertrophic scars in the rotation–advancement repairgroup. No significant difference in vertical lip lengthwas noted, although both groups contained lips thatwere either too short or too long postoperatively.The results were scored and favored slightly the rota-tion–advancement repair, but not to a statistical sig-nificance.

Yamada et al32 compared the 3-dimensional facialmorphology of children with UCLP after triangularflap repair versus rotation–advancement plus smalltriangular flap (at white roll), and primary nasal repair.A 3D optical scanner was used to collect facial sur-face data that was then analyzed using a computer-aided anthropometric method. Nasal protrusion andnostril symmetry were better in the rotation–advancement group. The columellar base deviatedtoward the normal side in both groups, and the widthof the nostril sill on the cleft side increased graduallyover time. In the triangular repair group, the lengthof Cupid’s bow increased on the non-cleft side overtime and the peak on the cleft side was lower thanthe non-cleft side at 1.5 years. In the rotation group,the Cupid’s bow peak was higher at 2 weeks aftersurgery but gradually dropped and became symmet-ric at 1.5 years. The authors’ conclusion was thatthe rotation–advancement repair produced betterresults overall.

BILATERAL CLEFT LIP

Surgical correction of the bilateral cleft lip hasbeen much less satisfying than that of the unilateraldeformity. Early procedures excised the prolabiumor mistakenly assumed it to be a displaced columella,33

producing a lip that was grossly deficient horizon-tally. Later the prolabium was used to form theupper central lip, its vertical height supplemented

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with excess tissue from the lateral lip,34 resulting in alip that was too long and too narrow. These earlyrepairs reflect the failure to recognize the potentialof the prolabium to grow in width and height whenattached to the dynamic lateral lip elements.

Adams and Adams35 reviewed the results ofthese early techniques. Their findings, along withthe developmental studies of Stark and Ehrmann,36

proved the prolabium was part of the upper cen-tral lip and led to the evolution of more recentmethods of repair. The prolabium of the bilateralcomplete cleft lip is functionally sterile37 and doesnot contain mature muscle, only immature fibro-blastic tissue and fine collagen.38 The incompletebilateral cleft lip, on the other hand, may havenear-normal muscle penetration into the pro-labium. Muscle bundles are cylindrically bunchedin the remaining lip bridge, and fan out into theprolabium.38 Schultz39 initially proposed restora-tion of orbicularis oris muscle continuity andreconstruction of the oral sphincter by direct su-ture of the lateral muscle elements behind theprolabium. This concept has become a criticalcomponent of modern cleft lip repair. Morerecent advances that have been incorporated intomodern repairs include primary nasal repair, non-surgical columellar lengthening, and presurgicalnasoalveolar molding.

Children with a repaired bilateral cleft lip have avery characteristic appearance. The stigmata include

• a wide, long, bowed, undimpled, asymmetric phil-trum, with widened scars

• a wide and asymmetric Cupid’s bow without awhite roll

• a deficient central tubercle with a red patch ofchapped wet vermillion and frequent whistledeformity

• a tight upper lip, concave on lateral view, and aprotruding lower lip

• a broad underprojected nasal tip, large flaredand kinked nostrils, splayed alar bases, vestibularwebbing, and a short retrusive columella

After careful analysis of the bilateral cleft lip stig-mata, Mulliken40–42 offers principles for primarybilateral cleft lip repair, as follows:1. Maintain symmetry because small defects become

more obvious with growth.

2. Reconstruct the muscular ring, eliminate lateralbulges, and minimize philtral distortion.

3. Design proper philtral size and shape because itwill bow and elongate with growth.

4. Construct the tubercle from the lateral elementsbecause the prolabial segment is deficient.

5. Reposition the alar cartilages and columella dur-ing the first surgery.

In his review of primary bilateral cleft lip and nasalrepair, Mulliken40–42 emphasizes that optimal resultsfrom 3-dimensional reconstruction must include care-ful consideration of the “fourth dimension” alter-ations and distortions that occur with facial growth.He believes that surgeons have an obligation to peri-odically assess their results and whether the predictedchanges materialized as the child grew. He discussesthe use of photography, panels, direct anthropom-etry, computer-aided photogrammetry (indirectanthropometry), and laser scanning.

Straight-line Repair

Modern techniques concentrate on using the pro-labium for the entire central portion of the lip andcan be subdivided into straight-line closures and clo-sures involving the Z-plasty principle. Among propo-nents of the straight-line closure are Veau,37

Axhausen,43 Brown,44 Schultz,39 Vaughan,45 Cronin,46

Manchester,47 and Broadbent and Woolf.48

Z-plasty

The Z-plasties may be in the lower lip, the upperlip, or in both the upper and lower lip. Advocates ofthe lower lip Z-plasty include Berkeley49 and Baueret al.50 Proponents of the upper lip Z-plasty principleare Millard51 and Wynn.52 Skoog53,54 prefers thecombined upper and lower lip Z-plasty. Figure 6illustrates several preoperative designs and finalresulting scars.

Millard Repair

Millard’s technique is detailed in his 1971 paper55

and in Cleft Craft II.33 The Millard repair brings in anew white roll from the lateral lip elements, theprolabial vermilion is advanced toward the sulcus toform a tubercle, and the prolabial parings are banked

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for future columellar lengthening. Mulliken56 andNoordhoff57 recount their separate experiences withMillard’s bilateral lip repair, bringing lateral vermil-ion flaps beneath the prolabium and reconstructingthe orbicularis muscle beneath the elevated prolabium(Fig 7).

Mulliken56 advocates a very narrow, concaveprolabial paring because of the changes that occur inthe prolabial segment in response to forces from theattached lateral segments and with growth. He docu-ments inevitable prolabial widening after a Millardrepair to as much as 2.5 times greater than normalover a 3-year period. Consideration of these 4-dimensional changes40–42 is critical to optimizing resultsof the primary repair.

Farkas58 reviews the normal facial growth pat-terns and states that all but two nasolabial featuresare fast-growing and achieve two-thirds adult sizeby age 5. The exceptions are columellar length(sn-c) and nasal tip protrusion (sn-prn), which areslow-growing. Mulliken40,42 correlates the growthrates of specific features with the stigmata of therepaired bilateral cleft lip and recommends intra-operative adjustments for these anticipated 4-dimensional changes42,59 (Table 1).

Fast-growing features—nasal length, alar width,prolabial length and width—become too long or toowide and should be constructed smaller. The slow-growing features remain relatively short and shouldbe constructed larger than normal for the infant dur-ing primary repair. The median tubercle (ls-sto) is anoted exception to this pattern. Although normallya fast-growing feature, it remains deficient in chil-

Fig 6. The Z-plasty principle in bilateral cleft lip repair.

Fig 7. Details of the Millard repair of complete bilateral cleft lip.A turndown flap of vermilion from the prolabium is placedposteriorly to prevent a whistling deformity. Note the bankedflaps of alar base that will be used later to lengthen the columella.(Reprinted with permission from Millard DR Jr: Closure ofbilateral cleft lip and elongation of columella by two operationsin infancy. Plast Reconstr Surg (4):324, 1971.)

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dren after bilateral cleft lip repair, therefore Mullikenrecommends that the median tubercle be designedas full as possible during primary lip repair.

Modified Manchester Repair

Broadbent and Woolf48 described a modificationof the Manchester one-stage primary repair. Themodified incisions on the prolabium and lateral lipelements bring the repair out of the nostril floor,narrow the Cupid’s bow, and place the repair in agently curved pattern on the philtral edge. Unlikethe Millard repair, the prolabial white roll and ver-milion are preserved. Closure of the lateral flapsbeneath the unfurled prolabial mucosa provides liplining and a normal buccolabial sulcus. The unfurledprolabium is brought down to complete the centralmucosal tubercle (Fig 8).

Two-Stage Repair

Sumiya et al60 concluded that a one-stage repairof the bilateral cleft lip is impractical because of themultiple secondary deformities that result. Theseinclude a short columella, lack of a philtrum, hori-zontal and vertical insufficiency of the lip, and insuf-ficiency of the vermilion and alveolar vestibulum. In

TABLE 1Bilateral complete cleft lip: nasolabial dimensions

(Adapted from Mulliken JB: Bilateral complete cleft lip and nasaldeformity: an anthropometric analysis of staged to synchronousrepair. Plast Reconstr Surg 96:9, 1995 and Mulliken JB: Bilateralcleft lip. Clin Plast Surg 31:209, 2004.)

Fig 8. Modified Manchester one-stage repair of bilateralcomplete cleft lip. (Reprinted with permission from BroadbentTR, Woolf RM: Bilateral cleft lip: one-stage primary repair.(Reprinted with permission from Still JM Jr, Georgiade NG:Historical Review of Management of Cleft Lip and Palate. In:Georgiade NG (ed), Symposium on Management of CleftLip and Palate and Associated Deformities. St Louis,Mosby, 1974. Vol 8.)

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response to their dissatisfaction with one-stage repairs,the transformation method was developed: a 2-stage rotation–advancement repair for bilateral cleftlip. During the first repair a lip adhesion is per-formed on the contralateral side. The second side isthen repaired several months later. Excellent resultsare shown.

Inspired by Sumiya’s results, Wolfe61 adopted a 2-stage rotation-advancement approach to incompletebilateral cleft lips. Aside from the disadvantages ofhaving two surgeries instead of one and a scar acrossthe base of the columella, the author was pleasedwith this technique and the results obtained.

Outcome

The Millard technique33,55 and the modifiedManchester technique48 are the most popular andappear to give the most acceptable final lip appear-ance. While the modified Manchester repair keepsthe prolabial white roll and vermilion and discardsthe lateral prolabial paring, the Millard repair bringsin a new white roll from the lateral lip elements andthe prolabial parings are banked for future columel-lar lengthening. Both techniques benefit from com-plete muscle reconstruction across the prolabial seg-ment.

The Millard technique results in better tuberclefullness but is criticized for creating a tight lip and anunfavorable scar from the inset of the lateral whiteroll along the cutaneous margin of the prolabium.The modified Manchester technique results in a lipthat is less tight, but the tubercle (based on the pro-labium) is deficient and a leads to a whistle defor-mity. When the white roll and vermilion of theprolabium are of good quality, a modified Manches-ter repair as performed by Broadbent and Woolf48

appears to be a good choice. If the white roll andvermilion of the prolabium are of poor quality, aMillard repair55 that borrows these parts from thelateral lip element is probably the better method.

Mulliken59 describes a personal experience with amodified Millard repair that has evolved from stagedto synchronous surgical correction of bilateral com-plete cleft lip and nasal deformity. In a follow-upstudy, Mulliken41 reviewed 50 consecutive patientstreated with his one-stage primary repair of bilaterallip, nose, and alveolar defects. At a median age of5.4 years the revision rate was 33% for children withBCCLP and 12.5% for those with BCCL. The most

common secondary procedure was resuspension ofthe mucosa in the anterior gingivolabial sulcus.

Lengthening the Columella

The modern trend in cleft lip repair has been tocombine primary lip and nasal correction in oneprocedure. Traditionally the appearance of a shortcolumella after bilateral cleft lip repair has beenaddressed with a second columellar lengthening pro-cedure, either at the time of hard palate repair orduring the preschool years. According to Pigott,62

nasal projection is mainly a function of the alar domesfor the children, but at maturity the columella con-tributes more than half of nasal tip support and pro-jection. If the columella is short, the nose will inevi-tably appear flat and the tip will be too low, so thatprovisions for lengthening the columella ideally shouldbe made at the time of primary lip repair. Croninand Upton63 offer a comprehensive review of tech-niques for lengthening the short columella (Fig 9).

The most common methods used to lengthen thecolumella are those described by Millard55 andCronin.63 Millard55 banked the prolabial parings or“forked flaps” during the primary lip repair (Fig 9D)for future columellar lengthening. Cronin63

inferomedially rotated and advanced prolabial tissuepreviously banked in the nostril floor (Fig 9F) to elon-gate the columella. Tip projection is increased withsuturing of the nasal cartilages and use of autog-enous cartilage graft if needed.

In a reverse of the Cronin technique, Matsuo andHirose64 lengthen the columella by approaching itfrom the nasal tip rather than from the nostril sill orthe lip. The procedure involves an extended “flyingbird” incision with superomedial and ventral rota-tion of the dislocated alar domes.

Chen and Chen65 described a technique consistingof three V-flaps for extended open-tip rhinoplasty insecondary nasal deformity from bilateral cleft lip (Fig10). The columella was lengthened from 2.5mm toan average of 10mm without the use of columellargrafts. No hypertrophic scars were noted in 12 patientsafter a mean follow-up of 13.5 months.

Unhappy with traditional methods of columellarlengthening because of the visible scars on the noseor lip, Van der Meulen66 described a 3-dimensionalZ-plasty on the alar rim to lengthen the columellaand improve tip projection without superficial scars.

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McComb67 reviews his 15-year experience withcolumellar reconstruction by forked flaps in the pri-mary repair of bilateral cleft lip. The author notesthree unfavorable features of this procedure, namely(1) the columella may grow too long and the nos-trils too large; (2) often the nasal tip remains toobroad; and (3) the columellar base tends to driftand a scar traverses the lip–columella angle. Onthe basis of these long-term unfavorable conse-quences, McComb67 advocates a new treatmentplan involving a 2-stage repair. The first stage is lipadhesion and primary nasal repair at 6-8 weeks,followed by definitive lip repair one month later.68

Mulliken41,59 and others61,69 challenged the oldconcept that the columella was inadequate andthat tissue needed to be recruited from the pro-labium or nostril sills. They believe that the col-umella is not deficient but is concealed in the nose,and the reconstructive strategy should be lip repairand synchronous anatomic positioning of the alarcartilages with sculpturing/draping of the nasal softtissues. These authors protest that most of thecolumellar lengthening procedures create tertiarydeformities that are frequently worse. Nasoalveolarmolding has also been shown to be effective innonsurgical columellar lengthening.69

Fig 9. Columellar lengthening by A, V-Y advancement of part or the entire prolabium; B, wings from the long upper lip; C, incisions inthe vestibular floor and midline approximation of the medial crura; D, forked flaps from the lip; E, flaps of prolabium; F, flaps of prolabiumbanked in the nostril floor at the time of primary repair; or G, Bilateral Z-plasties in the soft triangles. (Reprinted with permission fromCronin TD, Upton J: Lengthening of the short columella associated with bilateral cleft lip. Ann Plast Surg 1:75, 1978.)

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PRIMARY REPAIR OF CLEFT LIP NASALDEFORMITY

Traditional methods of lip repair did little to addressthe associated cleft nasal deformity. While alar baserepositioning was common, release, repositioning,and dissection of the nasal complex was avoidedbecause of the general feeling that growth distur-bances would ensue. In addition to alar base reposi-tioning, other procedures needed to correct the nasaldeformity include release and repositiong of the alarcartilages, redraping and modification of the skinenvelope over the corrected cartilaginous framework,correction of the caudal septal deformity, and fre-quently septoplasty and nasal bone osteotomies fordeviation. Although the latter osteoplastic proce-dures should be deferred until maturity to avoidgrowth disturbance, the literature now strongly sup-ports primary correction of the nasal deformity at thetime of lip repair.

Whether the tissue is deficient or all the nasalcomponents are present but merely distorted by thecleft is still debated.70 Some surgeons advocate direct

exposure of the alar cartilages via external or intra-nasal alar incisions,42,71–76 while others (or the sameauthors now) believe this is unnecessary and poten-tially harmful.77–79 Presurgical orthopedics andnasoalveolar molding have greatly facilitated the pri-mary correction of the cleft nasal deformity. Somesurgeons advocate repair of the alveolar cleft to sta-bilize the platform before or during primary nasalrepair42,73–78 whereas others believe it is not neces-sary to address the skeletal deformity primarily.79

Presurgical orthopedics and nasal molding in thecare of the child with cleft lip are advocated by Cut-ting, Grayson, and Matsuo.73–75,80 In addition to thebenefits of presurgical molding, postoperative nasalstents are used by many.1,69,70,81–83 The benefits of adynamic nostril splint in maintaining surgical resultsby opposing healing contraction of the nasal tip areemphasized by Cenzi and Guarda.81

Nakajima et al82 also subscribe to the policy ofpostsurgical splinting for 3–4 months to retain thecorrected contour of the cleft lip nose and recom-mend moldable silicone rubber retainers to add vol-

Fig 10. Technique of columellar lengthening in secondary repair of bilateral cleft lip nasal deformity. (Reprinted with permission fromChen T-H, Chen Y-R: Extended open-tip rhinoplasty with three V-flaps for secondary correction of bilateral cleft lip nasal deformity. AnnPlast Surg 37:482, 1996.)

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ume to ready-made nostril splints. This methodachieves and maintains precise alignment of tissuesand slight overcorrection of the deformity.

Yeow and coworkers83 noted a tendency of thelower lateral cartilage to return to its preoperativemalformed state despite primary surgical correctionof the cleft lip nasal deformity. Subsequently they fitpatients with a nostril retainer for at least 6 monthsto maintain the correction. The children who hadpostoperative nasal splinting showed a significantlybetter aesthetic result than those who were notsplinted, and the authors recommend the use ofnostril retainers for at least 6 months postoperativelyin all patients undergoing primary correction of com-plete unilateral cleft deformity.

LaRossa and Donath84 favor primary cleft nasalcorrection without external incisions. Their approachconsists of the following:

• Wide undermining from the nasal tip through thecolumella and through a lateral mucosal incisioninferior to the lower turbinates.

• Repositioning of the nasal domes with sutures.

• Restoration of the overlap between the upperand lower lateral cartilages.

• Nasal floor reconstruction with V-Y flap.

• Alar base advancement with alar base flap.

• Muscle reconstruction to supplement the alar basepositioning.

In two separate articles, Trott and Mohan71,72

report the results of open-tip rhinoplasty at thetime of lip repair in unilateral and bilateral cleft lipand palate. The method consists of simultaneouslip closure and open-tip rhinoplasty involving nos-tril and columellar rim incisions. The surgical em-phasis is on alar cartilage manipulation, with theskin being adjusted secondarily. The tip of thenose is reconstructed from alar dome reposition-ing and fixation under direct vision, constructionof a subcutaneous soft-tissue pad, and caudaladvancement of dorsal nasal skin. Wide dissec-tion of nasal mucosa from the medial wall of themaxilla and piriform margin allows correct posi-tioning of the alar base and prevents intranasalstenosis. Wide dissection and advancement ofcheek soft tissues supports the alar base and allowsfor a tension-free closure of the lip.

UNILATERAL CLEFT LIP NASAL DEFORMITY

The normal course of the paranasal muscle spansaround the ala to the anterior nasal spine. Thismuscle insertion is interrupted in cleft patients, con-tributing to nasal deviation. Delaire85 and Joos86

emphasize the importance of paranasal musclereconstruction and recommend advancing theparanasal muscles as far as the midline during thecleft lip repair.

In 1975 McComb87,88 described his technique forprimary repair of the unilateral cleft lip nasal defor-mity. Treatment begins with presurgical orthope-dics. The nasal skin is then widely dissected from thealar rim to the nasion via the upper buccal sulcusand medial lip incisions. The alar cartilage andattached mucosal lining are then “lifted” to the nor-mal symmetric position using mattress sutures and astraight needle through the nasal vestibule. Thesutures pass through the intercrural angle and thelateral crus and emerge medially over the nasionwhere they are tied over small bolsters.

McComb emphasizes the importance of lifting thealar cartilage and lining before closure of the nasalfloor and before lip repair. Final adjustments of thesutures can be made after lip repair. The authoravoids nasal lining incisions. He believes that inter-cartilaginous incisions create a significant risk of steno-sis and are unnecessary to free the alar cartilage ifadequate dissection is performed. McComb andCoghlan77 reported the long-term results ofMcComb’s technique for primary repair of the uni-lateral cleft lip nose. A computer-based method ofphotographic analysis to measure nasal asymmetrywas used to evaluate McCombs first 10 consecutivepatients up to age 18. The authors demonstratedthat the primary repair achieved long-term correc-tion of the deformity with good symmetry and with-out growth disturbance.

Millard78 advocates development of a symmetricnasal platform by using active presurgical orthope-dics with a Latham appliance, gingivoperiosteoplasty,and lip adhesion before correcting the lip and nasaldeformity at 6 months of age. His technique forprimary nasal repair includes unilateral columellarlengthening with the C-flap, L-flap augmentation ofthe vestibular lining, alar cartilage repositioning, andalar base cinching. The medial two-thirds of the cleftside alar cartilage is freed from the overlying skin andthe vestibular mucosa via a unilateral membranousseptal incision that extends to the alar margin. The

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author previously extended this incision to the inter-cartilaginous line, but like McComb77 he now cau-tions that scarring, stenosis, and airway compromisecan result. The cartilage is then shaped and suturedto the contralateral alar cartilage and the septum toreconstruct the medial crus. After 10 years of follow-up, Millard reports symmetric results with this tech-nique.

Salyer et al1,79 report long-term follow-up of theirseries of primary cleft lip nasal repairs. LikeMcComb’s,88 the key to Salyer’s technique is the trans-location of the alar cartilage and its lining to a normalposition, which he believes will establish a normalvault and shape of the cartilage. Unlike McComb,88

Salyer uses an intranasal incision running from thealar base to above the inferior turbinate to free thesoft tissue from the abnormal skeletal base. Musclereconstruction is used to establish a soft-tissue plat-form and silicone nasal stents are kept in place for 3months after surgery to reduce scarring and stenosis.The authors believe that aside from aligning the bonysegments with passive presurgical orthopedics, it is notnecessary to address the abnormal skeletal base at thistime. On the basis of their extensive experience, theycontend that early correction of the nasal deformity isdurable and contributes to normal growth and devel-opment of the nose.

Byrd and Salomon89 present an 18-year experi-ence with primary correction of the unilateral cleftnasal deformity. The key technical details of Byrd’sprocedure include 1) wide undermining of the skinenvelope over the cartilaginous framework throughthe lip incisions of the rotation–advancement repair;2) release of the nasal lining and accessory cartilagechain from the piriform aperture; 3) rotation of the“muscular roll” underneath the nasal ala to recreatethe cleft-side maxillary platform; and 4) alar webeffacement with a mattress suture from the web car-tilage to the lateral cheek musculature (Fig 11).

Nostril and dome symmetry was achieved in 60%of cases after completing these steps. The other 40%had more severe cartilage deformity and required aninfracartilaginous incision and an alar dome support-ing suture to the contralateral upper lateral cartilage(similar to that described by Tajima90). This approacheffectively corrected the dorsal hooding of the lowerlateral cartilage (Fig 12). Preoperative orthopedicswith a nasoalveolar molding appliance and a post-operative silicone nasal stent for the first week arepart of the protocol. No interference with normal

nasal growth was noted after using this technique in1200 patients with a mean follow-up of 9 years.

Mulliken21 analyzed the long-term results of hisprimary unilateral cleft lip and nasal repair using photoassessment by a panel. The patients were dividedinto two groups, an early group (1981–1990) whodid not receive active presurgical orthopedics and alater group (1990–1995) who had presurgical ortho-pedics and gingivoperiosteoplasty. The treatmentsequence was otherwise similar, starting with a la-

Fig 11. (Above, left) The muscular roll (shaded area) preservedduring the dissection is rotated underneath the cleft alar base tocreate a new nasal platform. This muscular roll is sutured to thesoft tissue close to the nasal spine and to the contralateral medialcrura footplate. (Above, right) Recreation of nasal platform afterthe muscular roll is inset. Also, the cleft medial crura footplate maystill be splayed laterally after the C flap is inset; it can be broughtmedially by a suture to the non-cleft footplate. (Center, left)Depiction of alar web correction by a mattress suture betweenthe caudal aspect of the cleft lower lateral cartilage and theexposed facial muscles laterally. (Center, right) Tightening of thealar web mattress suture effacing the web because of a leteral pull.(Below, left) New vectors of force being applied to the cleftnostril: a medial pull of the alar base by means of the muscular rollsuture and a lateral vector of pull of the cleft lower lateral cartilageby means of the “web mattress suture.” (Below, right) Thiscombination of vectors creates a natual contour for the cleft ala.(Reprinted with permission from Byrd HS, Salomon J: Primarycorrection of the unilateral cleft nasal deformity. Plast ReconstrSurg 106:1276, 2000.)

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bionasal adhesion—including alar suspension su-tures—followed by rotation–advancement lip repairand nasal repair 2 months later. The author statesthe foremost advantage of the 2-stage repair is thesecond opportunity to accurately secure the alar car-tilage.

The elements of nasal repair are as follows:21

• caudal septal repositioning

• direct exposure of the alar cartilage through acrescent-shaped skin excision of the soft triangleafter lip repair

• suture placement from the alar cartilage to theipsilateral upper lateral cartilage

• interdomal sutures in the tip if needed

• vestibular web effacement by lenticular mucosalexcision at the intercartilaginous line

Although the need for revision decreased in thelater group, presumably due to improvement of theskeletal base with presurgical orthopedics andgingivoperiosteoplasty, the overall need for nasalrevision was 80%. The most common reason forrevision was to correct a vestibular web (52%).Mulliken21 believes that L-flap augmentation of nasallining78 is unnecessary and that repositioning of theala may actually reveal an excess of mucosa thatshould be excised.

Anderl91 studied the long-term results of simulta-neous repair of the cleft lip and nose in more than200 patients. Surgery entailed 1) wide underminingof the medial and lateral parts of the cleft, preservingperiosteum in the maxilla; 2) straightening the sep-tum and elevating the mucoperichondrium on eitherside of the caudal septum; and 3) mobilizing thecleft ala to separate the alar cartilage from its under-lying soft-tissue envelope, upper lateral cartilage, col-umella, and dome area. After 14 years of follow-upthe overall success rate was 80%. Surgery was con-sidered successful when there was no need for sec-ondary correction of either lip or nose.

Wolfe70 describes his “pastiche” for cleft nasalrepair. For the primary unilateral cleft lip nasal cor-rection he advocates nasoalveolar molding, lipadhesion and gingivoperiosteoplasty, followed by lipand nasal repair. To elevate the vestibular web,Wolfe passes sutures from the vestibular mucosa outthrough the skin of the alar crease and back throughthe same hole and ties them intranasally. Siliconestents are used for the first postoperative week.

Liou et al92 recommend overcorrection of the nasaldeformity on the basis of their evaluation of relapseand differential growth after nasoalveolar molding,cheiloplasty and limited primary nasal correction. Thenasal repair used in this series included alar basemobilization and repositioning, nasal floor recon-struction with mucosal flaps, and columellar length-ening with C flap. Though no cartilage dissectionwas performed, the lower lateral cartilages were sup-ported by alar transfixion sutures. A nasal conformerwas used for 3–6 months postoperatively. Theauthors compared multiple linear photographic mea-surements for up to 3 years after surgery and reporta widening of the nasal base and decrease in heightof the cleft-side nostril and nasal dome during thefirst year. They recommend surgical overcorrectionof the nasal vertical dimension, maximal narrowingof the alveolar cleft with presurgical nasoalveolarmolding, and use of a postoperative nasal conformerto prevent relapse.

Millard18 also notes lateral creep of the alar baseand suggests securing it firmly to the septum. Incontrast, Salyer93 cautions against medial overcor-rection, which may result in a nostril that is too small—a problem much more difficult to repair than a nos-tril that is too big. To prevent this problem, Salyer93

leaves the cleft-side nostril 1–2mm larger than thenostril on the noncleft side. Mulliken21 believes that

Fig 12. (Left) After completion of the repair, residual hooding ofthe cleft alar rim was present in 50% of cases. (Right) Placementof a Tajima suture from the cleft lower lateral cartilage to thecontralateral upper lateral/septal junction creates a cephalic pullon the rim and eliminates hooding. (Reprinted with permissionfrom Byrd HS, Salomon J: Primary correction of the unilateralcleft nasal deformity. Plast Reconstr Surg 106:1276, 2000.)

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the correct alar base position can be accurately main-tained if the muscle repositionings of the alar baseand the lateral lip are performed independently.

Cussons and associates94 assessed the overall aes-thetic results in unilateral cleft lip patients with andwithout primary nasal reconstruction. Pre- and post-operative photographs were reviewed by a blindedpanel of observers when patients were aged 10 years.Significantly better nasal symmetry was recorded inthe group who had had primary nasal reconstruc-tion.

BILATERAL CLEFT LIP NASAL DEFORMITY

Broadbent and Woolf95 believe the missing col-umellar tissue in clefts is simply shifted laterally intothe ala. They use no external incisions but ratherrotate the cleft lateral crus medially as a chondro-cutaneous composite flap and suspend it to the dor-sal septum and upper lateral cartilage for fixation.The columellar tissue is “rerotated” into proper posi-tion at the same time, from lateral to medial, obviat-ing the necessity for a C-flap in the lip repair.

After becoming disenchanted with forked flaps,McComb68 advocated repair of the bilateral cleft noseat age 6–8 weeks in combination with lip adhesion.The final lip repair is performed 1 month later. A V-shaped external nasal tip incision is used to exposeand suture the lower lateral cartilages. A V-Y plasty isthen used to narrow the nasal tip. At 4 years of age,the first 10 consecutive children operated by thisprotocol showed well-developed columella and mini-mal external scars. The early repair did not seem tointerfere with growth. The author now performs asimultaneous lip and nasal repair and does not usepresurgical orthopedics.96

Trott and Mohan72 reported the results of theirone-stage bilateral lip and nasal repair performed ona Malaysian population whose socioeconomic fac-tors precluded multiple stage procedures. Theiropen-tip method involves exposure and reposition-ing of the lower lateral cartilages by elevating aprolabial-columellar flap anterior to the medial crurabased on the paired columellar arteries (Fig 13).

Cutting and Grayson97 initially described a prolabialunwinding flap for one-stage repair of the bilateralcleft lip, nose, and alveolus: “The columella of thenose and the central lip are produced by ‘unwind-ing’ the columellar and labial sections of the pro-labium around a small central tab, which is used to

center the junction between the lip and columella.”In an update of their approach, Cutting andGrayson69,74,75 recommend presurgical orthopedicmolding of the lip, alveolus, and nose combinedwith a one-stage lip, alveolus, and nose repair. Theiropen-tip approach is similar to that described byTrott and Mohan72 except that the prolabial–columellar flap is incised in a deeper plane along themembraneous septum, elevating the medial andmiddle crura in the flap. The unexposed genua arethen apposed by a transvestibular mattress suture(Fig 14). The protruding premaxilla is aligned withthe maxillary arch by means of pesurgical orthope-dics, while acrylic stents lengthen the nasal columellaand nasal lining.74

Mulliken’s59 technique in three phases:

• Two-stage lip and nose repair with banked forkedflap and subsequent intranasal transposition ofthe tines.

• Two-stage lip and nose repair with later transec-tion of the banked tines.

• One-stage lip and nose repair without a forkedflap and with closure of alveolar clefts.

Anthropometric analysis showed that the anatomicresults of the single-stage repair were equivalent tothose of the two-stage repair, superior in terms ofalar and columellar width, and more reproducible.59

More recently Mulliken42 discontinued his use of anexternal vertical nasal incision for cartilage reposi-tioning and now uses only extended rim incisions.

Fig 13. The Trott method of open approach to nasal-tip cartilages.The philtral-columellar flap (with rim extensions) is turned up-ward to expose the anterior surface of the dislocated middle cruraand genua. (Modified from Trott and Mohan; reprinted withpermission from Mulliken JB: Primary repair of bilateral cleft lipand nasal deformity. Plast Reconstr Surg 108:181, 2001.)

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Figure 15 illustrates his current approach to the nasaltip and soft triangle deformity.

Kohout and colleagues98 compared Mulliken’stechnique and Trott’s technique for primary bilateralcleft lip and nasal repair using photogrammetricanalysis. The columellar length (as a proportion oftip projection) and philtral width were normal in theMulliken group but not in the Trott group. Bothgroups had above-normal tip projection, but theMulliken group’s was even greater than the Trottgroup’s. Both groups had an abnormally wideinteralar dimension.

CLEFT PALATE REPAIR

TECHNIQUES

Cleft palate repairs are of two basic types: one-stage procedures involving primary closure of theentire palate, usually with mucoperiosteal flaps, and

two-stage procedures that repair either the velum oranterior hard palate first followed by complete pal-ate closure. Still and Georgiade99 illustrate variousmethods of cleft palate repair in Figure 16. Many ofthese techniques are still in use today.

Intravelar Veloplasty

In 1969 Kriens100 described a method of soft pal-ate repair that detaches the malpositioned velar mus-culature from the posterior hard palate and recon-structs the levator sling end-to-end in the midline.This technique was a significant improvement overprevious soft palate repairs101 and has been usedalone and in combination with techniques for repairof the hard palate.

Ruding102 emphasized that normal speech dependsupon the reconstruction of the cleft velar muscula-ture into its normal anatomic “levator loop” with

Fig 14. The Cutting method of open approach to nasal-tip cartilages. The philtral-medial crural-columellar complex (incised through themembranous septum with extensions into the intercartilaginous ridges) is reflected by retrograde dissection to display the underside ofthe splayed middle crura and genua. (Reprinted with permission from Mulliken JB: Primary repair of bilateral cleft lip and nasal deformity.Plast Reconstr Surg 108:181, 2001.)

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complete detachment of all abnormal muscularinsertions into the palatine bone.

Marsh et al103 prospectively compared the effectsof intravelar veloplasty and traditional side-to-sideveloplasty on velopharyngeal competence. In a studyof 51 patients they found that anatomic reposition-ing of the levator with intravelar veloplasty was nobetter at improving VPI than side-to-side veloplasty.

Brown, Cohen, and Randall104 studied the impor-tance of levator muscle reconstruction in cleft palaterepair. They concluded that coaptation of the muscledoes give better resonance and decreased nasal emis-sion without additional morbidity from levator slingreconstruction.105

Von Langenbeck Procedure

“The von Langenbeck palatoplasty is the oldestcleft palate operation widely in use today. It is asimple closure technique and involves no attempt tolengthen the whole palate. It involves less dissectionand detachment of anterior mucoperiosteum thanthe pushback procedures do.”106 The original con-cept in the von Langenbeck palatoplasty was to fash-ion bilateral bipedicled mucoperiosteal flaps through

Fig 15. The displaced lower lateral cartilages are visualized through the rim incisions and three polydioxanone sutures are placed: oneto appose the genua and middle crura and one on each side to suspend the lower over the upper lateral cartilage. The sills are constructedby medial advancement of the alar bases; these are trimmed and slightly rotated endonasally to join the end of the tabs at the columellarbase. The interalar dimension is narrowed to 22 to 24 mm by tightening a nonresorbable cinch suture set between the alar bases. Eachalar base is secured to the underlying maxillary periosteum and orbicular muscle. This mattress suture serves three purposes: (1) it lowersposition of the base, (2) it forms a normal depression in the lateral sill, and (3) it stimulates the action of the depressor alae nasi to minimizenostril elevation with smiling. Following anatomic placement of the lower lateral cartilages, it becomes obvious there is redundant skinin the soft triangles and central nasal tip. This excess skin is sculpted by a crescentic excision that extends beyond the usual initial rimincision to include the upper and mid columella. This procedure serves to narrow the nasal tip, define the columellar-lobular junction,elongate the nostrils (and columella), and taper the columellar waist. The constricted columella (sn-c) should measure 5 to 6 mm (ie, slightlylonger than normal for a 5- to 6-mo-old infant; 3–4 mm). If the lower lateral cartilages are thin, place an internal resorbable splint to protectthem from scar contraction during healing. (Reprinted with permission from Mulliken JB: Bilateral complete cleft lip and nasal deformity:an anthropometric analysis of staged to synchronous repair. Plast Reconstr Surg 96:9, 1995.)

Fig 16. Techniques for repair of the cleft palate. (Reprinted withpermission from Still MJ Jr, Georgiade NG: Historical Review ofManagement of Cleft Lip and Palate. In: Georgiade NG (ed),Symposium on Management of Cleft Lip and Palate andAssociated Deformities. Vol 8. St Louis, Mosby, 1974.)

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two parallel incisions, one along the cleft margin andthe other along the lingual side of the alveolus. Theseflaps are mobilized and approximated in the mid-line.

The original design has been modified subse-quently. Reid and Watson107 attempted palatal clo-sure with only one incision along either side of thecleft margin. Their procedure involved wide subpe-riosteal undermining, freeing of the greater palatinevascular pedicle, and scoring incisions on theundersurface of the palatal flap, which allowedapproximation of the mucoperiosteal flaps without asecond incision along the alveolar margin.

Murison and Pigott108 described the ‘medialLangenbeck’ modification of the von Langenbeckrepair. The lateral palatal incision is moved from thelateral to the medial side of the greater palatine artery.The incision is limited in length to only what is requiredfor tension-free closure. This technique results in asmaller area of denuded palate and better preserva-tion of the primary blood supply to the alveolus com-pared to the von Langenbeck or pushback palato-plasty.109

The chief criticism of the von Langenbeck palato-plasty centers on the inferior speech results. In aseries of 40 patients operated on by the vonLangenbeck repair, Veau110 found 60% had VPI andan additional 15% were improved but not completelycompetent. He concluded that “the method ofoperation produces short and immobile palates.”Spurred by this shortcoming, Veau later went on todevelop a precursor to the V-Y pushback techniqueof palatoplasty.

Subsequent reports of speech results with thevon Langenbeck repair have been mixed. Trierand Dreyer111 combined primary von Langenbeckpalatoplasty with levator sling reconstruction onchildren 14–16 months of age and found 89%had velopharyngeal competence. In contrast, theirseries of primary von Langenbeck without levatorreconstruction resulted in 62% velopharyngeal com-petence.

Myklebust and Abyholm112 report 203 patientswhose cleft palates were repaired by modified VonLangenbeck palatoplasty at an average age of 24months. Speech evaluation at 6 years of agerevealed good articulation in 86% and somenasality in 81%. Pharyngoplasty was required in28 patients, and the final speech results were con-sidered good in 97%.

Palatal Lengthening—V-Y Pushback

Veau’s110 protocol for closure of congenital cleftlip and palate stressed

• closure of the nasal layer separately

• fracture of the hamular process

• suture of the muscles of the soft palate

• staged palatal repair following primary lip andvomer flap closure

• creation of palatal flaps based on a vascularpedicle

Veau’s speech results proved to be considerablybetter than those obtained with the von Langenbeckprocedure. In Europe the Veau hard palate repair isstill the most popular technique (22.7% of palaterepairs).113

Kilner114 and Wardill,115 working independently,devised a technique of palatal repair in 1937 thatwas more radical than Veau’s and which ultimatelybecame the V-Y pushback. It includes lateral relax-ing incisions; bilateral flaps based on posterior palatinearteries (Wardill originally divided this artery); clo-sure of nasal mucosa as a separate layer; fracture ofthe hamulus; separate muscle closure; and V-Y pala-tal lengthening116 (Fig 17). This protocol was usedfor all types of clefts to complete a one-stage repairwithout additional treatment or special apparatus.

Whether the benefits of the V-Y palatoplasty arepermanent is still in question. Steffensen117 believesthat little if any permanent lengthening is obtainedby the classic V-Y operation. Calnan118 reported<8mm of acquired length in the soft palate follow-ing V-Y repair. Any gain in palatal length due to thepushback is at the cost of denuding the anteriorportion of the palatal bone, which is thought to havean adverse effect on midfacial growth.

A recent retrospective study of one surgeon’sexperience disclosed satisfactory long-term midfacialgrowth based on dental relationship analysis afterthe Veau-Wardill-Kilner (V-W-K) repair.119 TheGOSLON yardstick was used to rank dental rela-tionships in 12-year dental models. Analysis showed52% were considered good, 20% fair, and 28%poor. These results were comparable to thosereported for the Eurocleft project113 and better thanfor most of the other centers in the United King-dom.120 Maxillary osteotomies were required in20% of the patients after V-W-K repair.

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V-Y Pushback with Nasal Mucosal Flaps(Cronin Modification)

Cronin121 felt that a major problem with palato-plasty techniques was the raw area on the nasalsurface of the repair, which he believed led to scarcontracture and was a source of poor speech results.He developed a technique involving transnasal dis-section of nasal mucosa off the bony floor of thenose. This tissue was shifted to the nasal side of the

soft palate. Aaronson, Fox, and Cronin122 analyzedthe speech results obtained with this technique in 92patients and report normal resonance and intelli-gible speech in 78% and normal articulation in 66%.

Haapanen and Rintala123 compared the speechof 124 patients with isolated cleft palate who hadrepair in one stage by either traditional V-Y pushbacktechnique or the Cronin modification. More patientswith the Cronin modified repair achieved normalresonance, while patients with V-Y pushback hadsignificantly more hypernasality. The number of sec-ondary operations was similar in both groups, yet theauthors believe that patients who had V-Y pushbackultimately would require secondary surgery.

The chief criticism of the Cronin modification hasbeen the technical difficulty of the procedure; indeed,the operation may not be possible to do in everycase. Stark124 as well as Samarrai and Reavie125 reporttheir modifications of nasomucoperiosteal flaps, whileother methods such as skin grafts and buccal mucosalgrafts have been proposed to line the raw area andprevent fibrosis and contracture.126–129

Two-Flap Palatoplasty

Bardach130 and Salyer131 independently modifiedthe two-flap palatoplasty to combine elements ofother operations with some innovative details. Themain goals are complete closure of the entire cleftwithout tension at an early age (<12 months) withminimal exposure of raw bony surfaces and the cre-ation of a functioning soft palate. The authors believethat a muscle sling within the soft palate, not velarlengthening, is essential to adequate speech.

Morris and colleagues132 note that 80% of patientstreated with this method developed velopharyngealfunction within normal limits, but 51% required speechtherapy before normal speech production could beexpected.

Double-Opposing Z-plasties

In 1986 Furlow133 described a single-stage palatalclosure technique consisting of double-opposing Z-plasties from the oral and nasal surfaces (Fig 18).

Two reverse Z-plasties are based on the cleft mid-line; the anteriorly based flaps contain mucosa andthe posteriorly based flaps contain the levator musclecomplex and mucosa. Once the flaps are inset, thepalate is lengthened and the levator sling is reposi-

Fig 17. Highlights of the pushback palatal lengthening techniqueof palate repair. (A) The cleft margins are incised on the oralmucosa. (B) The greater palatine vessels are mobilized for poste-rior displacement of the mucoperiosteal flaps. (C) The nasalmucosa may be divided behind the free margin of the bonypalate, but this frequently produces a short or inadequate lining.This shortage may be prevented by cutting it off the bone fartheranteriorly. (Reprinted with permission from Masters FW, LevinJM: Surgical Management of the Palatal Cleft by V-Y Technique(Wardill-Kilner Repair). In: Georgiade NG (ed), Symposium onManagement of Cleft Lip and Palate and Associated Defor-mities, Vol 8. St Louis, Mosby, 1974.)

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tioned transversely and posteriorly. Furlow empha-sizes avoidance of relaxing incisions in repair of thehard palate to minimize any potential gowth distur-bance.

A quantitative evaluation of palatal elongationreported a mean intraoperative elongation of16.11mm (69.1%) and a postoperative elongation at4.5 years of 12.47mm (55.5%).134 The transversedimension is shortened and closure of wide cleftscan be technically difficult.135 The incidence of fis-tula has been reported to be higher after Furlowrepair, especially in children with wide clefts.135

Recent retrospective studies have shown thatpatients with Furlow repairs have adequate soft pal-ate mobility, reduced hypernasality, and improvedspeech and articulation.136–139 The group from the

Children’s Hospital of Philadelphia (CHOP) describedtheir modification of the Furlow palatoplasty andreviewed their results in over 600 patients.140 The“CHOP modification” includes relaxing incisions,mucoperiosteal undermining of the hard palate, dis-section into the space of Ernst, infracture of thehamulus, and stretching of the greater palatine neu-rovascular bundle to close without tension. Theauthors report excellent speech results and a fistularate of 6.8%.

Vomer Flaps in Palatoplasty

Flaps of vomerine mucosa can be useful in theclosure of particularly wide clefts and bilateral clefts(Fig 19). In 1932 Veau and Plessier141 first proposedthe technique of vomer flap repair of the palate.This was introduced in the United States by Ivy andCurtis142 2 years later.

Fig 19. Bilateral inferiorly based vomer flaps for palatal closure.The technique avoids a dead space and minimizes the raw areaof bony palate. (Reprinted with permission from Nguyen PN,Sullivan PK: Issues and controversies in the management of cleftpalate. Clin Plast Surg 20(4):671, 1993.)

The primary concern regarding vomer flaps forpalatal closure has been their effect on facialgrowth.143 As Delaire and Precious144 point out, max-illary growth depends on the sliding action of thevomer along its maxillary surface (Fig 20). The scarthat results from the use of vomer flaps in the repair

Fig 18. Furlow’s palatoplasty technique of double opposing Z-plasties for palatal closure. The anteriorly based flap contains onlymucosa, whereas the levator-palatopharyngeus muscle is el-evated with the posteriorly based flap. Transposing the two setsof flaps overlaps the palatal muscles and lengthens the soft palate.(Reprinted with permission from Furlow LT Jr: Cleft palate repairby double opposing Z-plasty. Plast Reconstr Surg 78:724, 1986.)

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of palatal defects could be expected to inhibit thismotion and subsequent maxillary growth.

Fig 20. Three maxillary movements with normal vomeropalatinesuture (top) and with scarred vomeropalatine suture (bottom).(Reprinted with permission from Delaire J, Precious D: Avoid-ance of the use of vomerine mucosa in primary surgical manage-ment of velopalatine clefts. Oral Surg 60:589, 1985.)

Several studies seem to bear out this concern.Johanson and Friede145 examined 13 children withcomplete bilateral clefts and 50 children with com-plete unilateral clefts who were treated with vomerflaps. The authors found maxillary retrognathia andstraighter-than-normal skeletal facial profiles, andsubsequently abandoned their use of vomer flaps.

Enemark and colleagues146 followed 57 consecu-tive patients with complete unilateral cleft lip/palatefrom birth to age 21 years. Vomer flaps were used forrepair at 10 weeks of age and a pushback palato-plasty was performed at an average age of 22 months.At the time of evaluation none of the patients demon-strated a normal growth pattern, but 50 of 57 wereconsidered to have a normal or acceptable profile.

Other authors have noted that covering thedenuded surface of the vomer with either full-thickness skin grafts or mucosal flaps could reducethe growth disturbance induced. Jonsson, Stenstrom,and Thilander147 used full-thickness skin grafts andfound improved results in 10 cases over 10 years offollow-up.

Buccal Flaps

Buccal mucosal flaps based on the buccinator ves-sels have been used for over 30 years as an adjunctto palate repair.148–150 This versatile flap has beenused by different surgeons to lengthen or augmentthe nasal/oral palatal mucosa during primary or sec-ondary palate repair.

Jackson et al150 reported the long-term results ofhis cleft palate repair with a unilateral buccal mucosalflap to lengthen the nasal mucosal. His repair achievesclosure without raw areas and includes Veau flaps,levator muscle dissection and retropositioning, a trans-verse releasing incision in the nasal mucosa posteriorto the hard palate without any subperiosteal dissec-tion over the posterior tuberosity, and insertion of aunilateral buccal myomucosal flap into the nasal lin-ing defect posterior to the hard palate (Fig 21). Thebuccal flap can add 2cm of length to the nasal liningand theoretically prevent reattachment of thereconstructed levator muscle to its preoperative posi-tion. Patients operated on in 1989–2002 were evalu-ated for speech outcomes and demonstrated excel-lent results: 91.1% had good velopharyngeal clo-sure; 91.1% had normal resonance; 97.9% had nor-mal articulation; 89% had normal speech quality;and 8.8% had VPI. The fistula rate was 3.6%. In anearlier study Freedlander and Jackson150.5 used post-operative endoscopy to show that the buccal flapremains viable and maintains its dimensions overtime.

Isago et al151 evaluated the speech outcomes andcraniofacial morphology after cleft palate repair usingtheir modified Kaplan method with bilateral buccalmucosal flaps. Their repair technique combines Veau-Wardill incisions, transverse release of the nasalmucosa posterior to the hard palate, and insert ofbilateral buccal mucosal flaps to augment the nasallining. Good velopharyngeal function was reportedin 72.7% but articulation disorders were found in59.1%. No major changes in facial morphology wereobserved up to 6 years of age.

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Chen and Zhong152 described a different tech-nique of cleft palate repair using bilateral buccalmyomucosal flaps to lengthen the nasal lining andto avoid mucoperiosteal dissection. Their repairincludes incisions only along the cleft margin,vomer flap repair of the hard palate nasal lining,a transverse incision through both the oral andnasal mucosa posterior to the hard palate, leva-tor muscle reconstruction, closure of the nasalmucosa of the soft palate, then one buccalmyomucosal flap is inset into the nasal ling defectposterior to the hard palate and a contralateralbilobed buccal flap is rotated into the remainingoral mucosal defect. Good velopharyngeal clo-sure was reported in 83% of patients and 95%demonstrated normal maxillary growth andocclusion in a follow-up of 2–8 years.

Two-Stage Palate Repair

Although some surgeons advocate closure of theanterior hard palate at the time of lip repair to facili-tate closure of the nasal floor, the more commontwo-stage sequence of palate repair starts with thevelum. Proponents of early veloplasty believe thatthe intact velum will optimize speech developmentduring the pre-linguistic period and minimize growthdisturbance by delaying the mucoperiostal dissectionof the palate and vomer.

Rohrich and Byrd153 give their rationale for repairof the lip and velum at age 3 months with secondaryclosure of the hard palate at 15 to 18 months of age.They note that there is no dissection of nasal mucosaor mucoperiosteum at this early age, and as such noraw surfaces. In their words, “the major benefit ofsimultaneous closure of the lip and soft palate at an

Fig 21. Repair of cleft palate. A: Design of repair outlined, without retrotuberosity dissection. B: Veau flaps elevated together withnasal lining. Soft palate muscles being dissected out. Division of the palatopharyngeus tendon is pointed laterally. C: Closure of nasallayer and approximation of the palatal musculature. Dotted line indicated division of the nasal layer transversely. D: Division of the nasallayer and elongation of the soft palate. E: Design for elevation of buccal flap. F: Buccal flap used to repair the nasal layer defect. Thisis introduced behind the great palatine vessels. Donor site closed directly. G: Oral layer closed in the midline. H: Closure of the lateraldefects. (Reprinted with permission from Jackson IT, Moreira-Gonzalez AA, Rogers A, Beal BJ: The buccal flap—a useful technique incleft palate repair? Cleft Palate Craniofac J 41(2):144, 2004.)

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early age is that it narrows the hard palate gap sothat less extensive hard palate surgery is requiredlater.”103,154 Using this approach, Atkins, Byrd, andTebbetts155 found approximately 75% of their patientshad normal speech, while mobile but short palatesdeveloped in another 10%. The remaining 15%were felt to have neurogenic palates.

Delaire and Precious144 also recommend early (7months) closure of the soft palate so that it mayassume the normal functions of phonation, degluti-tion, tongue position, and skeletal morphogenesis.This is usually followed by narrowing of the hardpalatal cleft, which can then be closed with moreconservative surgery at 18 months of age. They alsofound vertical and anterior growth restriction afteruse of a vomer flap, which caused scar adhesions atthe vomero-premaxillary suture.

Other authors advocate early closure of the velumbut postpone hard palate repair until much later.Schweckendiek156,157 closed the soft palate at 6–8months but left the hard palate open (occluded with aprosthetic plate) until age 12–15y, when palatoplastywas performed. Schweckendiek reported normal jawdevelopment in patients treated by this protocol.Measurements of the width of the palatine arch, thelength of the maxilla, and the base of the skullapproximated those of unaffected adults; the speechresults, however, have been called into question.153,158

Another long-term assessment of 45 randomlyselected patients operated on by the Schweckendiekapproach, the Marburg project,159 found an unusu-ally high incidence of short palates and poor mobilityof the soft palate with a correspondingly high degreeof VPI. Facial growth, on the other hand, was judgedto be quite acceptable in the majority of patients.

Perko160 also advocated initial veloplasty at age 18months with closure of the hard palate at age 5–8y.Perko found that “the remaining cleft in the hardpalate does not disturb speech development to arelevant degree.” Van Demark and coworkers161

evaluated the speech results obtained when usingPerko’s protocol and noted adequate to marginalvelopharyngeal competence in 94% of patients.Speech analysis showed fewer compensatory articu-lation errors than with the Schweckendiek approach.

Palatoplasty With Primary Pharyngeal Flap

Stark and DeHaan162 used a pharyngeal flap basedeither inferiorly or superiorly in conjunction with

palatoplasty. The pharyngeal donor defect was closedprimarily and the flap sutured to the palate, whichwas repaired simultaneously in the manner of vonLangenbeck. Surgery was performed at age 1 yearin the belief that the pharyngeal flap permanentlyand effectively tethers the velum to the posteriorpharynx without reducing its dynamic properties.Velopharyngeal competence was noted in 95% oftheir cases compared with 75–80% of those whoreceived palatal pushback procedures. Curtin163

reserves primary pharyngeal flap for cases of tissuedeficiency.

Trigos and Ysunza164 compared palatoplasty withand without primary pharyngoplasty. In their seriespushback palatoplasty + pharyngoplasty was associ-ated with a residual VPI rate of 26% while thepushback-only group had a 40% incidence of VPI.

Riski and others165 combined palatoplasty, aWardill-Kilner or von Langenbeck, with a modifiedOrticochea pharyngoplasty in 15 patients. All devel-oped velopharyngeal competence although 54%achieved closure above the level of the pharyngoplastyby apposition of the velum to the adenoid pad. Also27% exhibited speech disorders such as glottal stopsassociated with VPI, “thus the primary pharyngoplastywas not always successful in preventing aberrant cleftspeech problems.”

Bröndsted and colleagues166 analyzed the speechresults in 140 cases of insufficient velopharyngealclosure managed by Fogh-Andersen of Copenhagenover a 19-year period. These patients were treatedwith palatopharyngoplasties—PPP, or a combinationof palatoplasty and primary pharyngeal flap, eitherinferiorly based or, when the VPI was severe, superi-orly based. The authors found normal nasal reso-nance in 74% of patients and improvement of mildsymptoms in 24%. Postoperative speech therapywas given to 38% of patients, mainly those with com-pensatory voice and articulation problems.

Alternative Techniques

Although the vast majority of cleft surgery is per-formed with the above techniques, novel approachesto palatal repair have been reported.

Sommerlad167 developed a technique for palaterepair that involves minimal hard palate dissectionwith radical retropositioning of the velar musculatureand tensor tenotomy. The author performs this repairunder an operating microscope to optimize the

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extensive muscle dissection and mobilization. Repairof 80% of the clefts was possible using only incisionsalong the cleft margin without elevation of muco-periosteal flaps or lateral incisions. Results are reportedover a 15-year period during which the rates of sec-ondary velopharyngeal surgery decreased from 10.2%to 4.6%. The overall fistula rate was 15%, which theauthor states is a small price to pay to avoid lateralreleasing incisions and the resultant scarring.

Abramo and colleagues168 described the use ofintraoperative rapid expansion in palatoplasty. Apair of 5cc Foley catheters are inserted between thepalatal bone and mucoperiosteum through incisionson either side of the palatal cleft. The catheters areintermittently inflated with saline for 20–30min tothe point of mucosal blanching and then deflated toallow for tissue perfusion. After 30min or so thecatheters are removed and the expanded flaps areapproximated. The authors claim a reduction in sizeof the cleft by up to 2cm with this technique. All 12patients treated by this technique had successfulrepairs without dehiscence. No long-term results asfar as speech or any other outcome variables werereported.

Brusati and Mannucci169 also attempted palatalclosure without lateral releasing incisions. They didso by means of an incision along the cleft margin andextensive subperiosteal dissection of the palatal andnasal mucoperiosteum that extended all the way tothe pterygoid process. Isolated cleft palates wereclosed at age 10–12mo, while clefts of the lip andpalate were staged with hard palate closure at 18–24mo. Of 88 patients treated in this fashion, 83%had primary approximation of the palate and 17%required lateral releasing incisions (7 from this lattergroup needed only retrotuberosity incisions). Heal-ing was by first intention in 98.4% of patients. Therewas one complete dehiscence of the palatal repair ina large bilateral cleft and a small oronasal fistula thathealed spontaneously. No long-term data arereported regarding any possible adverse consequencesof this extensive dissection on facial morphology.

Marks and Wynn170 report the results of bilateralosteotomies for palatal closure. The authors avoidmucoperiosteal dissection by performing osteotomiesof the palatal bone from behind the tuberosity to apoint approximately three-fourths the length of thehard palate. Relaxing incisions along the alveolarmargin facilitate movement. Of 363 recordsreviewed, 81.5% of patients were judged to have

normal or near-normal resonance in their speech.No data are available regarding long-term facialgrowth in these patients, but the authors believe thatthe risk of postpubertal growth disturbances isdecreased by eliminating the mucoperiosteal dissec-tion.

Decellularized dermal grafts (Alloderm) havebeen described in primary cleft palate repair. Clarket al171 reported using alloderm in 7 patients withclefts >15mm to reduce the risk of postoperativefistula. The graft was placed between the nasaland oral closure in the region of the hard palate–soft palate junction and the palate was repairedusing a two-flap technique with intravelarveloplasty. In 2 patients the oral mucosa dehiscedand exposed the graft, but in all cases the graftmucosalized and no fistulas developed up to oneyear after surgery.

Helling et al172 reported a series of 31 consecutivecleft palate repairs using the Furlow technique and adecellularized dermal graft. The graft was placed inthe region of the hard palate–soft palate junctionbetween the nasal and oral mucosa. The postopera-tive fistula rate was 3.2% (1) which compares favor-ably to the historic fistula rates (4.1–10 %) after Furlowpalatoplasty.

A group from Turkey173 reported the use of asynthetic tissue adhesive for fixation of mucoperi-osteal flaps to the hard palate during cleft palaterepair. N-butyl-2-cyanoacrylate (NB2C) was appliedto the underside of the mucoperiosteal flaps intraop-eratively in place of sutures to achieve a tension-freeclosure of the oral mucosa. This technique was usedin 15 patients with a follow-up time of 14–24 months,without noted complications. According to theauthors the advantages of using NB2C were shorteroperative times, better homeostasis, more pushbacklength, and better patient comfort.

CHOICE OF REPAIR TECHNIQUE

Sadove174 and LaRossa140 provide excellent reviewsof current cleft palate management. The choice ofrepair technique is surgeon-dependent; randomizedprospective studies are needed to evaluate the out-comes of different techniques and be able to makeobjective comparisons. There is debate whether it isethical to standardize repair for the purpose of clini-cal trials.174,175 The natural variety of the cleft defectmay require the use of different techniques that are

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individually suited to the specific deformity in orderto optimize outcome.

Clefts of the Secondary Palate

Pigott176 lists the following essential features forsatisfactory soft palate function: adequate length,adequate mobility with appropriate resting muscletension, and a midline dorsal convexity that con-forms to the dorsal surface of the pharyngeal wall.

According to Sadove,174 intravelar veloplasty andthe Furlow repair are both effective techniques ofsoft palate repair. Frequently the hard palate muco-periosteum needs to be mobilized to achieve atension-free closure of the soft palate. The use oflateral relaxing incisions or the pushback techniqueare effective methods of tissue mobilization. Somesurgeons prefer alternative methods such as hamulusfracture, uvular transposition, or tensor veli palatinitendon release.

Sadove174 recommends the use of the vonLangenbeck or Veau-Wardill-Kilner repair in combi-nation with an effective veloplasty technique such asintravelar veloplasty or Furlow repair for incompleteclefts of the hard and soft palate.

A study from Chang Gung Memorial Hospital inTaipei compared speech results after Furlow palato-plasty and von Langenbeck palatoplasty in patientswith incomplete clefts of the secondary palate.177

Velopharyngeal function was adequate in 98% ofpatients in the Furlow group and 70% in the vonLangenbeck group. One fistula was reported in eachgroup. The authors prefer the Furlow palatoplastyfor clefts that are not wide to prevent excessive ten-sion on the repair. This preference may have con-tributed to the low complication rate in the Furlowgroup.

CLEFTS OF THE PRIMARY AND SECONDARY PALATE

Sadove174 recommends the use of a the two-flapor four-flap palatoplasty (two-flap plus bilateral vomerflaps) for complete unilateral and bilateral cleft pal-ate. These techniques involve greater mucoperi-osteal flap mobilization which is useful in wide com-plete clefts.

Bishara and colleagues178 published the results oftheir cephalometric comparisons of the Wardill-Kilnerand von Langenbeck palatoplasty. They reported

no significant difference in malocclusion or maxillarygrowth rates between the two groups.

Dreyer and Trier179 also compared the results ofvarious palatoplasty techniques, including vonLangenbeck, V-Y island or pushback palatoplasty,and von Langenbeck with intravelar veloplasty. Sig-nificantly improved speech results and superiorvelopharyngeal function were noted when intravelarveloplasty was added. The authors recommend care-ful reconstruction of the levator sling at the time ofpalate repair.

Holtmann, Wray, and Weeks180 analyzed the earlyspeech results in 62 patients with cleft lip and palateor cleft palate after V-Y pushback, von Langenbeckpalatoplasty, and von Langenbeck + pharyngeal flaptechnique. No differences were noted among thetreatment groups in terms of VPI, but morbidity washigher with V-Y pushback than with other methods.Patients who had von Langenbeck palatoplasty com-bined with pharyngeal flaps experienced more upperairway obstruction. In at least 75% of patients theaddition of pharyngeal flap to primary palatoplastyseemed to be unnecessary. The authors interpretthese findings as supporting evidence for vonLangenbeck palatoplasty in primary cleft palate repair.

Pigott et al109 published a retrospective study com-paring the outcomes of three different methods ofhard palate repair: a pushback, a von Langenbeck,and a medial von Langenbeck. Maxillary growthwas assessed by means of dental model analysis. Inthe pushback group, 13.9% had favorable growthoutcomes and 55% had unfavorable outcomes. Inthe von Langenbeck group, the results were favor-able in 31.1% and unfavorable in 42.4%. In themedial von Langenbeck patients, they were 55%favorable and 11% unfavorable. Articulation wasbetter in the medial von Langenbeck group than inthe von Langenbeck group, which was better thanin the pushback group. No differences were foundin nasal emission, nasal resonance, or pharyngealflap rates among the groups. The fistula rate wasgreatest in the pushback group (19%) comparedwith the von Langenbeck (10.5%) and medial vonLangenbeck (0%). The authors attribute theimprovement in facial growth with the medial vonLangenbeck technique to less denuded palate andless periosteal undermining.

Spauwen et al135 used the Furlow technique forcleft palate repair and compared their results withthose obtained with the von Langenbeck technique.

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Speech production was superior with the Furlow tech-nique. Nasal escape was present in 50% of childrenoperated on by the von Langenbeck procedure com-pared with 10% in the Furlow group. The incidenceof nasality was 50% in the von Langenbeck groupand 0% in the Furlow group. Two oronasal fistulasoccurred in the Furlow group and none in the vonLangenbeck group. Both children had wide cleftsand the fistulas developed at the junction of the hardand soft palates. This complication underscores thedifficulty associated with use of the Furlow techniquein wide clefts. Occasionally a vomer flap is necessaryto complete the closure.

McWilliams et al137 also reported superior resultswith the Furlow palatoplasty compared with thepushback and von Langenbeck repairs in terms ofhypernasality, articulation, total speech scores, andpharyngeal flap requirements.

SUBMUCOUS CLEFT PALATE

From an anatomic standpoint, submucous cleftpalate is the direct result of malposition of the palatalmuscle complex.181 The levator muscle is displacedanteriorly and inserts onto the hard palate; in addi-tion, the musculus uvulae is often abnormal,182 andconsequently some patients with submucous cleftpalate will develop velopharyngeal insufficiency. Thisis felt to be due to the absence of the levator sling181

as well as the abnormal musculus uvulae. Velascoand colleagues182 point out that patients withvelopharyngeal insufficiency are most likely to dem-onstrate a coronal velopharyngeal closure pattern.Since the musculus uvulae occludes the major por-tion of the velopharyngeal sphincter in this pattern, itis not surprising that the muscle is underdeveloped insubmucous cleft palate.

VPI is the only indication for surgical interventionin patients with submucous cleft palate, and patientsshould be carefully watched for the developmentof this problem. Weatherly-White183 showed that 1in 9 patients with submucous cleft palate has VPI.A number of procedures have been proposed toaddress velopharyngeal insufficiency in submucouscleft palate.

Gosain and colleagues181 offer a comprehensivereview of the various techniques and their outcomes.Pharyngeal flaps have been successfully used alonefor the repair of submucous cleft palate. Brondstedand associates166 reviewed a series of pharyngeal

flaps in 104 patients with submucous clefts. Theauthors report normalization of speech in 81% ofpatients within 5 years of the flap procedure. Faraand Weatherley-White184 recommend treatmentwith superiorly based pharyngeal flaps but add pala-tal retrodisplacement when the palate is felt to beshort.

Furlow repair has been recommended in patientsyounger than 2 with a small velopharyngeal gap(<5mm).174 Chen and colleagues185 treated 30patients with submucous cleft palate and velo-pharyngeal incompetence with a Furlow palatoplasty.The selection criteria favored younger patients withcircular or sagittal patterns of velopharyngeal closureand those with small gaps and a positive response tobiofeedback speech therapy. Patients who did notfulfill these criteria were treated with pharyngeal flapoperations. Over 96% of those who underwentFurlow palatoplasty achieved competent velo-pharyngeal sphincters. The one poor result in thestudy was in a child who had very prominent actionof the musculus uvulae preoperatively. In this casethe Z-plasty transected the muscle and changed itsdirection, removing the effect it had on thevelopharyngeal closure pattern. Nevertheless, promi-nence of the musculus uvulae is not a commonoccurrence in submucous cleft palate, and thereforewould likely not affect the decision to perform Furlowpalatoplasty in most patients.

Other authors advocate palatal pushback proce-dures186 and levator repositioning with palatal length-ening.187 Pensler and Bauer187 recommend treat-ment with intravelar veloplasty and palatal lengthen-ing. Of patients operated on before age 2, 75% hadnormal velopharyngeal function and 25% had slightVPI that did not require secondary correction. Ofpatients operated on after age 2, only 14% had nor-mal velopharyngeal function while the remainderhad slight to severe VPI. Pensler and Bauer187 alsoremark that many patients with submucous cleftsoften have a history of recurrent otitis media, and90% of them improve after levator repositioning.Abyholm188 also reported better postoperative speechoutcomes in patients with submucous cleft palaterepaired at an early age.181

Sommerlad et al189 evaluated postoperativespeech outcomes in 40 consecutive submucous cleftpalate repairs using his veloplasty technique ofextensive muscle dissection and retropositioning per-formed under an operating microscope. A grading

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system is described to classify the severity of thepalatal deformity and was correlated with speechoutcomes. Overall, normal speech was achieved in50% of patients. Improvement was also noted inhypernasality (85%), nasal emission (63%), and velarclosure rates. Secondary pharygoplasty was requiredin 27.5% over a 6-year follow-up period. Severityof the submucous cleft was not associated with pre-operative speech parameters but was an importantpredictor of postoperative outcome. Patients withmore severe clefts had better postoperative speechoutcomes. Age was not found to be a predictor ofpostoperative success.

COMPLICATIONS OF CLEFT PALATE REPAIR

Operative mortality from cleft palate surgery is inthe range of 0.5%.190 Two potentially life-threaten-ing complications after CP repair are postoperativebleeding and respiratory compromise, yet the trueincidence of these complications is difficult to deter-mine from a review of the literature. Reported post-operative complications include oropharyngealinfection, upper respiratory tract infection, aspirationpneumonia, airway obstruction, otitis media, feed-ing difficulties, hyperthermia, flap dehiscence, andfistula formation.191 The fistulas will be discussed atlength below.

Eriksson and Henriksson192 prospectively evalu-ated anesthetic risk factors for children having palato-plasty. All children who had a recent or ongoingrespiratory infection or a parent who smoked hadsome degree of difficulty, frequently severe, duringintubation, extubation, or both. These are avoid-able risk factors that can potentially place the child atrisk for having a major or even deadly anesthesiacomplication. All patients with Pierre Robin also hadincreased airway difficulties.

A recent review of adverse anesthesia outcomes(laryngospasm and bronchospasm) after cleft repairin children with recent or ongoing upper respira-tory tract infections (URI) recommends that sur-gery should be postponed 4 to 6 weeks until symp-toms completely resolve.193 Bronchospasm andlaryngospasm occur more frequently in children,especially those <1yo, even in the absence of aURI. The incidence of laryngospasm in children is1.7% and increases to 9.6% in the presence of aURI.194 The decision to postpone surgery is com-plicated by the difficulty in diagnosing a URI clini-

cally and the high frequency of URIs in youngchildren, especially those with cleft palate. Theaverage <5yo child without a cleft palate has 5-6URIs per year with a duration of 7–10 days ofactive symptoms and residual pulmonary sequelaefor 2–6 weeks.195 Due to the prolonged respira-tory effects of URI, including airway hyperreactivityand respiratory muscle weakness, surgery shouldbe postponed for 6 weeks after URI to avoidpotential adverse anesthesia outcomes.

Takemura and associates196 studied the associa-tion between perioperative respiratory complicationsand the severity of common cold symptoms in 339infants undergoing surgery for cleft repair. Patientswere classified as healthy (96%), borderline (4%), orhigh-risk (0%) on the basis of the Common ColdScore. Respiratory complications occurred in 4% ofthe healthy patients and 23% of the borderlinepatients. Patients with bilateral cleft lip and palatehad a greater incidence of respiratory complications(8.9%) than patients with unilateral cleft lip and pal-ate (3.3%) or cleft lip alone (1.7%). The authorsrecommend postponing surgery in all patients withcommon cold symptoms.

Agrawal and Panda197 described a “hanging pal-ate” complication due to detachment of themucoperiosteal flap from the anterior hard palatethat occurred in 5 patients (0.5%) in their series. Thiscomplication was caused by either hematoma orinfection and was effectively treated by placement ofa methyl methacrylate obturator for 3 weeks.

Wray and associates198 reported greater morbid-ity with the Wardill-Kilner repair than with othermethods, typically involving increased postopera-tive bleeding following division of the anterior branchof the greater palatine artery. Airway obstructionwas considerably more common after a vonLangenbeck procedure with pharyngeal flap.198 Incontrast, Stark and DeHaan162 do not mention earlypostoperative airway obstruction in their patientswho had von Langenbeck palatal repair and pha-ryngeal flap.

Moore and others191 studied the complications ofprimary palatoplasty in 200 patients over a 20-yearperiod. Von Langenbeck and Wardill-Kilner tech-niques were used in 95% of patients; 34% hadintravelar veloplasty. Fistulas developed in 6%, upperrespiratory infections in 2%, airway difficulties in 3%,oropharyngeal infections in 4%, otitis media in 10%,and 22% of patients required reoperation. Bleedingwas not a problem (<1%).

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Lees and Pigott199 found 26.2% complications in aretrospective review of 164 primary procedures forcleft lip and palate performed at their center. Of the133 operated patients, there were 3 cases of earlyhemorrhage and 2 patients required blood transfu-sion after bleeding ceased spontaneously. There wasno need to take any patient back to surgery to controlthe bleeding, and there was no case of secondaryhemorrhage. They noted 7 major respiratory prob-lems that prompted admission to the intensive careunit for 4 patients. All of these respiratory complica-tions occurred within the first 48 hours after surgeryand 4 had a known history of respiratory problems.Lees and Pigott199 cite Shprintzen’s200 suggestion thatan early discharge from the hospital may reduce theincidence nosocomial infection, and advocate a post-operative stay of 5 days for cleft palate repairs.

Eaton and colleagues201 looked at admission sta-tus, length of stay, length of operation, and short-term morbidity of infants with cleft lip/palate treatedat their institution. In group 1 (first stage of repair),93% of patients were admitted the day before sur-gery. In group 2 (second stage of repair), 79% ofpatients were admitted the day of surgery. Therewas no difference between the groups in terms ofany of the variables studied. Of note, earlier dis-charge from the hospital was not reflected in increasedcomplications from surgery. The authors concludethat “reductions of hospital admissions and length ofstay do not affect outcome of cleft lip and palatesurgery in infants. The reduction in hospital days isequivalent to a savings of $138,000” [1991 dollars].

Canady et al202 examined the records of all cleftlip and palate patients who underwent primarypalatoplasty or cleft lip/palate revision at their cen-ter over a 14-year period. The admission policy attheir institution resulted in more frequent admis-sions the day of surgery, with a trend towardsreducing the hospital stay postoperatively. Theauthors report no statistically significant differencein postoperative complications between patientsadmitted to the hospital the day before surgery andthose admitted the day of surgery. The total lengthof hospital stay was 4–7 days for patients admittedthe day before surgery and 2–3 days for patientsadmitted the day of surgery.

Palatal Fistula

Incidence

The reported incidence of palatal fistula after cleftsurgery is 0–63%. This wide range results in part

from different definitions of what constitutes a fis-tula, which has made previous comparisons very dif-ficult. This discussion will focus on fistulas that repre-sent a failure of healing or breakdown of primarycleft palate repair, not intentionally unrepaireddefects. Factors that may lead to fistula formationafter primary cleft palate repair include poor selec-tion of technique, incomplete dissection, inadequatemuscle release, poor tissue handling, closure undertension, failure of multilayer closure, postoperativetrauma, bleeding, and infection.203

Fistulas may occur in the immediate postoperativeperiod or several years later during orthodontic treat-ment and arch expansion.203 Although some smallfistulas may heal spontaneously or be asymptomatic,most are clinically significant. Even small fistulas cannegatively affect speech. Common symptoms of fis-tulas include nasal regurgitation of liquids and solids,halitosis, hypernasality, increased nasal emission, andarticulation problems. Fistulas that have a negativeaffect on speech should be repaired early; if speechis adequate, fistula repair may be more successfulafter completion of arch expansion.

Cohen and others204 note a direct relation betweenseverity of the cleft lip/palate and occurrence of post-operative fistula along the site of original closure.Schultz205,206 also found that the severity of cleft is adeterminant of postoperative fistula: 61% in bilat-eral clefts, 26% in unilateral cleft lip/palate, and 15%in isolated cleft palate. Muzaffar et al207 reported asignificantly greater incidence of fistula in patientswith a Veau 3 and 4 cleft (9) compared to patientswith a Veau 1 and 2 cleft (0).

In contrast, Emory et al208 did not find an associa-tion between cleft severity and the incidence of fis-tula. In a review of their institutional experience,they found that fistula occurrence was related mostto the experience level of the operating surgeon.Emory et al208 and Rohrich209 found a decreasedincidence of fistula when primary palate repair wasperformed at an earlier age.

Cohen and others204 reported that fistula incidencewas associated with the surgeon performing the repairand the technique of repair. In their review, fistulaswere most common after V-Y pushback repairs (43%),compared to intravelar veloplasty (30%), vonLangenbeck repair (22%), and Furlow repair (10%).Amaratunga210 noted a higher fistula rate in patientsrepaired with the von Langenbeck palatoplasty ver-sus the V-Y pushback technique. Schultz205 found

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no difference in the incidence of palatal fistula aftercleft palate repairs of the Wardill-Kilner or vonLangenbeck type. In the series by Muzaffar et al,207

no association was found between fistula incidenceand surgeon, technique of repair, age, sex, presurgicalorthopedics, or arch expansion.

Patients with Treacher Collins syndrome are knownto have abnormal vascular supply to the palate.211

Bresnick et al211 investigated the correlation betweenTreacher Collins syndrome and fistula formation afterFurlow palatoplasty. They found that children withTreacher Collins syndrome had significantly greaterfistula rates (50%) compared with children who haveother syndromes (8.7%) and nonsyndromic children(4.1%).

Wilhelmi et al212 reported a 3.4% fistula rate intheir review of 119 consecutive patients after a two-flap palatoplasty. This low fistula rate was attributedto the greater flap mobilization and tension-free clo-sure that is possible with the two-flap technique. Areview of the University of Texas Southwestern Medi-cal Center experience with two-stage palate repairfound an overall fistula incidence of 8.7%.213 Thenarrowing of the hard palate gap with the two-stagerepair facilitates a tension-free closure with minimalhard palate dissection.

Most studies report that the most common site offistula formation is in the postalveolar segment of thehard palate207,214,215 (Fig 22).

Fistula Repair

The outcome of fistula repair has been disappoint-ing, with recurrence rates of 9–65%.205,207,208,215 Thechoice of fistula repair technique depends on thelocation of the defect, the quality and availability oflocal tissue, the need for bone grafting, and the qual-ity of speech. Multiple methods of fistula treatmenthave been described in the literature, including pala-tal obturators; excision and primary closure; exten-sive mobilization and closure of local mucoperiostealflaps; a variety of nasal lining flaps; buccal or vestibu-lar mucosa flaps; local pedicled flaps of skin, muscleand/or mucosa; tongue flaps; bone grafts; cartilagegrafts; tissue expansion; and free tissue transfer.Adequate closure of both the nasal and oral layers isessential to a successful repair.

Schultz205 noted a 22% overall incidence of pala-tal fistula in cleft palate repairs of the Wardill-Kilneror von Langenbeck type. Approximately 50% ofpatients with fistula required treatment, and conven-tional methods of surgical closure were successful inonly 35% of these. Schultz206 subsequently modifiedhis protocol to include free periosteal or cancellousbone grafts, and reports a much improved outcome.Grafts are harvested from either the tibial perios-teum or iliac crest. The author also recommends atwo-layer closure, excision of all scar tissue betweenthe maxillary segments, repair before age 10, andsimultaneous bone grafting of the anterior maxillaryand alveolar clefts.

On the basis of this series, Schultz206 emphasizesthe importance of orthodontic arch alignment beforefistula repair, noting that it is not uncommon forfistula to develop in completely closed clefts follow-ing arch expansion by orthodontic means. Randall216

stresses the need for bone grafts to the anterior max-illary and alveolar fistula for permanent closure.

Jackson and associates217 recommend repairingeven small anterior buccal fistulas, which often havelarge underlying bony defects, and advocate bonegrafting to improve resonance and reduce nasal tones.They note that prealveolar fistulas cannot beadequately sealed by an obturator, for air is expelledinto the nose whenever the lip loses contact with theprosthesis. In their opinion it is not enough to restorevelopharyngeal competence and fit an obturator insecondary repairs; the fistula must be solidly closed.

Isberg and Henningsson218 studied the influenceof palatal fistulas on velopharyngeal movements. The

Fig 22. Locations of postoperative cleft palate fistulas. (Reprintedwith permission from Cohen SR, Kalinowski J, LaRossa D, RandallP: Cleft palate fistulas: a multivariate statistical analysis ofprevalence, etiology, and surgical management. Plast ReconstrSurg 87:1041, 1991.)

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authors remarked that, with an open fistula, lateralwall activity was significantly reduced, while with afistula covered by an obturator, velopharyngeal move-ments improved or even normalized in all patientsregardless of fistula size.

In the review by Cohen and others,204 patientswith anteriorly located fistulas and fistulas associatedwith residual alveolar clefts were usually treated bybone grafting and local mucoperiosteal flaps, with orwithout turnover nasal lining flaps.

Denny and Amm219 described a technique forfistula repair that involves elevation of the palatalginigivoperiosteum starting at the dental arch, exci-sion of the fistula, and separate closure of the nasaland palatal mucosa. The success rate of fistula repairin their series of 60 consecutive cases was 90%. Allcases of recurrence were patients with bilateral cleftlip and palate. No cases of tooth loss or gingivalrecession were reported, but the minimum follow-up period was only one month.

Erçöçen and colleagues220 described the use ofbilateral superiorly based full-thickness nasolabialisland flaps for repair of a large anterior fistula in anadult. The skin, muscle and mucosa of the flap wereinset into the defect and were supplied by the retro-grade flow through the flap pedicle (Fig 23). Theauthors recommend this innovative technique whenlocal tissue is inadequate.

Tongue flaps have been a common method ofrepair when mobilization of surrounding tissue isinadequate. Closure of palatal fistulas with tongueflaps was first described by Guerrerosantos andAltamirano221 in 1966. Kummer and Neale222 notethat articulation and lingual mobility are unaffectedby the excision of tongue tissue necessary for theprocedure. Coghlan, O’Regan, and Carter223

describe their technique for elevation and inset of ananteriorly based dorsal tongue flap, which they usedsuccessfully in 17 of 20 cases of oronasal fistula fol-lowing cleft palate repair. Although evidence pointsto the posteriorly based midline flap of the tongue ashaving the best blood supply,224 the rich vascularplexus at the tip of the tongue (the ranine arch) willsupport a flap based anteriorly, which is better suitedfor closure of the more common anterior fistulas.The authors make the base of the flap 2.5–3cmwide and 6cm long. The flap includes the mucosaand approximately 5mm of underlying muscle formaximum vascularity. Patients are placed inmaxilllary-mandibular fixation for 3 weeks, at whichtime the fixation is released and the tongue flapdivided under local anesthesia.

Argamaso225 and Barone226 report their separateexperiences with posteriorly based tongue flaps. Theflap is designed slightly wider than the fistula and iselevated to one side of the midline based on the

Fig 23. A. Bilateral full-thickness nasolabial island flaps are elevated. The flaps include skin (s), muscle (ms), and buccal mucosa (m) , andare nourished by the distal portion of the facial artery (fa) and accompanying vein by means of a retrograde blood flow. B. The flaps aretransferred to the defect area through a tunnel dissected along the cleft line to the base of the flap bilaterally. Oa = ophthalmic artery;tfa = transverse facial artery; Ia = infraorbital artery. (Reprinted with permission from Erçöçen AR, Yilmaz S, Saydam M: Bilateralsuperiorly based full-thickness nasolabial island flaps for closure of residual anterior palatal fistulas in an unoperated elderly patient.Cleft Palate Craniofac J 40(1):91, 2003.)

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proximal pedicle. The flap consists of mucosa, longi-tudinal muscle, and a layer of transverse muscle, yetis thin enough for reconstruction in the nasopharynxand, once in position, to be covered with bilateralmucoperiosteal palatal flaps (Fig 24).

Fig 24. Posteriorly based tongue flap technique for closure ofpalatal fistulas. (Reprinted with permission from Barone CM,Argamaso RV: Refinements of the tongue flap for closure ofdifficult palatal fistulas. J Craniofac Surg 4:109, 1993.)

Abubaker and Abouzgia227 published a retrospec-tive review of the temporalis muscle flap for closureof palatal defects in 8 patients. The anterior portionof the temporalis muscle was advanced into the oraldefect by temporarily removing the zygomatic archand creating a bony tunnel in the posterior lateralmaxillary wall. The donor defect was filled with theposterior segment of the muscle and a Medporeimplant to reduce temporal hollowing. The muscleflap epithelialized in 4–6 weeks. There were nocomplications and the authors concluded that thetemporal muscle is a versatile and reliable option forthe repair of large palatal defects.

The buccal myomucosal flap and the facial arterymyomucosal (FAMM) flap have been used success-fully for the repair of palatal fistulas.228,229 A recentseries reported use of the FAMM flap for closure of

recurrent palatal fistulas in 22 patients.230 The FAMMaxial flap, based over the facial artery, can be usedas either a superiorly based (retrograde) flap foranterior defects or as an inferiorly based (orthograde)flap for midcentral and soft palate defects. Thesuperiorly based flap requires a gap in the dentalarch for passage. The inferiorly based flap can bepassed behind the maxillary arch when there is in-tact dentition. Secondary division and insetting maybe required for inferiorly based flaps. In this series ofFAMM flap repairs, one total flap loss and 2 partiallosses were reported.

Free-tissue transfer has been used successfullyfor large or difficult fistulas. Chen and associ-ates231 describe their experience with 4 radial fore-arm flaps in the closure of difficult palatal fistulas.Their procedure includes deepithelializing a 0.5cmmargin of the flap for insetting between the nasaland palatal mucosa. The cutaneous portion ofthe flap is oriented toward the oral side while asplit thickness skin graft is placed on the nasal sur-face of the flap (Fig 25). After the flap is inset, thepedicle is passed through the maxillary cleft alonga subcutaneous tunnel and is sutured to the ante-rior facial vessels (Fig 26). Three of 4 fistulas weresuccessfully closed with this technique; one had asmall area of dehiscence that was later closed byadvancement of adjacent tissue.

Fig 25. Free forearm flap for closure of palatal fistula. Thedeepithelialized flap margin is “sandwiched” between oral-sidepalatal mucosa and periosteum of the hard palate or nasal mucosaof the soft palate. (Reprinted with permission from Chen H-C etal: Free forearm flap for closure of difficult oronasal fistulas incleft palate patients. Plast Reconstr Surg 90:757, 1992.)

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Eufinger and Machtens232 reported a series of 3patients who underwent free-tissue transfer for clo-sure of complex recurrent palatal fistulas. One myo-osseous scapular flap and 2 radial forearm flaps wereused. A small fistula recurred in the patient with thescapular flap and was successfully closed second-arily. The patients with the radial forearm flapsunderwent secondary bone grafting with iliac crest.Both required flap debulking and one of the bonegrafts died.

Schwabegger et al233 used free-tissue transfer torepair recurrent palatal fistulas in 6 patients, 3 withosseous scapular flaps and 3 with dorsalis pedis flaps.The flap pedicle was passed through the retromolartrigone and tunneled below the mandibular angle tobe anastomosed to the facial vessels. One dorsalispedis flap was partially lost due to insufficient pediclelength; 5 patients had complete long-term closure oftheir palatal defects without major complications.The use of free flaps to repair palatal fistulas is rec-ommended in patients who meet the following crite-ria: 1) more than 3 failed repairs; 2) scarred, poorlyvascularized local tissue; 3) unsuitable for labialmucosal flaps due to closed dentition (or narrowgap); 4) hard palate defect; 5) fistula size >1cm2; 6)obturator declined or not feasible; 7) osseous flap

when alveolar bone graft required; 8) consent andcompliance of patient.

ALVEOLAR CLEFT REPAIR

The morphology of alveolar clefts is widely vari-able. In unilateral clefts, the cleft or lesser segment isprone to medial collapse due to lack of transversestability, which frequently results in a crossbite. Thepremaxilla is usually rotated anteriorly toward thenon-cleft side. In bilateral clefts, the premaxilla oftenprotrudes anteriorly with medial collapse of the lat-eral alveolar segments. The goals of alveolar cleftrepair are as follows:

• stabilization of the maxillary arch

• elimination of the oronasal fistula

• odontogenic bony support for tooth developmentand favorable migration of dental units

• enhancement of dental hygiene, function, andaesthetics

• bony support of the nasal base

• improvement of articulation

Bone grafts to the alveolar cleft are the standardmethod of bridging the bony gap. Boyne234 estab-lished the suitability of particulate, autogenous,osteogenic marrow and cancellous bone grafts inreconstructing the dental arch. Preoperative orth-odontics are used for alignment, rotation, andadvancement of the lateral segments of the maxillaryarch. Postoperative orthodontic treatment is usedfor the movement and alignment of teeth into thegrafted cleft. Although the timing and technique ofalveolar cleft repair is widely variable and controver-sial, the goals are the same. It is important to under-stand the different techniques and chronologicnomenclature of alveolar cleft repair.

Gingivoperiosteoplasty involves primary closure ofalveolar cleft by advancing bilateral mucoperiostealflaps, usually at time of primary cleft lip repair.Presurgical orthopedics are usually needed to nar-row the cleft and align segments so that primaryrepair is technically feasible. This approach takesadvantage of the osteogenic potential of the muco-periosteum to generate bone in the alveolar cleft.

The conventional nomenclature of alveolar bonegrafting follows a chronological order:

Fig 26. Free forearm flap for closure of palatal fistula. The vascularpedicle is passed through the maxillary cleft and a subcutaneoustunnel to the anterior facial vessels. (Reprinted with permissionfrom Chen H-C et al: Free forearm flap for closure of difficultoronasal fistulas in cleft palate patients. Plast Reconstr Surg90:757, 1992.)

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• Primary – younger than 2 years of age, beforepalate repair

• Early Secondary – between 2 and 5 years of age,after complete eruption of primary dentition butbefore eruption of permanent dentition

• Secondary or Intermediate – between 5 and 12years of age, during the stage of mixed dentition

• Late Secondary – older than 12 years, after erup-tion of permanent dentition

The terminology and timing of alveolar bone graft-ing based on patient age represent the interaction ofsurgery on maxillary growth and dental develop-ment.235 Secondary alveolar bone grafting is by farthe most common approach, but successfulgingivoperiosteoplasty and primary bone grafting havebeen reported from a growing number of centers.The critical issues of interference with maxillary growth,establishment of adequate bony support, andocclussal outcomes dominate the debate over theoptimal technique and timing of alveolar cleft repair.Alveolar cleft repair performed at the same stage ofdevelopment may have different outcomes relatedto the the morphology of the initial cleft, the tech-nique of repair, the extent of dissection, the sequenceof interventions, the use of presurgical orthopedics,and the type of bone graft.

In a retrospective analysis, Walle and Forbes236

studied the effect of size of alveolar clefts on bonegraft success. The authors note that the area of thedefect and the surgeon influenced the degree ofbony support and percent bone fill that was attained.Bilateral clefts had a lower percentage of bone for-mation than unilateral clefts, and more surgeries wereneeded to achieve a satisfactory result. Eppley237–239

reported significant correlation between cleft width,the potential for orthopedic arch alignment, and thesuccess of primary bone grafting. In contrast, Aurouzeand others240 did not find a correlation between thesize of the cleft defect and the success of secondaryalveolar bone grafting.

Orthodontics

Evans241 reviews the orthodontic treatment ofpatients with clefts from the neonatal period to adult-hood. The current goals of orthodontic cleft treat-ment are to promote normal growth, advance themaxilla rather than setback the mandible, retain natu-

ral teeth if possible, and use implants for toothreplacement. Early treatment of infants begins withorthopedic positioning of the alveolar segments ornasoalveolar molding prior to surgical repair. Earlyprimary bone grafting may take place during theneonatal period. Preschool children benefit frompediatric dental care starting at age 2 to detect car-ies, assess dental hygiene, educate parents, and moni-tor tooth development.

Orthodontic treatment of school age childreninvolves preparation for secondary bone grafting.Orthodontists align the dental arches and teeth inpreparation for bone grafting before eruption of eitherthe cleft lateral incisor or the canine. Alignment maybe achieved using a variety of expanders. A quadhelixexpander is a fixed maxillary appliance recommendedfor unilateral clefts; a traditional expansion screw isrecommended for bilateral clefts.241 Orthodonticguidance and occasionally surgical exposure is usedto facilitate tooth eruption through the bone graftand close the gap. Successful orthodontic gap clo-sure has been associated with less bone graft resorp-tion.242,243 Children with mild midface retrusion maybenefit from orthopedic maxillary protraction;unfortunately, palatal scarring and lip tightness fre-quently restrict the amount of skeletal movement.The use of osteotomies and distraction osteogenesisis discussed for adolescent and adult patients. Evans241

notes that tooth anomalies are present in cleft patientsat all stages of development and may be iatrogenicor due to natural causes.

In their review of the management of alveolarclefts, Daw and Patel244 emphasize that 4–6 monthsor orthodontic preparation should precede second-ary alveolar bone grafting. The objectives ofpresurgical treatment include expansion of the ante-rior and posterior maxilla to achieve a favorable archform and eliminate crossbites, alignment andderotation of malpositioned incisors, and improve-ment of dental function and aesthetics. Inadequateorthodontic preparation will lead to poor results thatusually necessitate secondary surgical procedures forcorrection.

Rygh and Tindlund245 detail the appropriate orth-odontic treatment plan beginning in the deciduousdentition phase. Conventional correction of themaxilla was mainly by rotation and expansion of thelateral segments, with some lateral movement of themaxillary bones and labial tipping of the lingually

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positioned upper incisor teeth. These methods failedto yield any appreciable forward movement of themaxillary dentoalveolar arch and none of the basalparts. The authors, following the method of Delaire,suggest application of external forces through a pro-traction face-mask technique to promote an increasein the vertical height as well as the sagittal length ofthe maxilla. They emphasize that sutural growth inthe upper jaw is most active at age 6–7 years andthen declines until the pubertal spurt. They con-clude that maxillary protraction therapy must beinstituted before age 8 if significant effect is to beobtained. The basic principle of therapy is shortperiods of active, controlled, efficient treatment, andlong periods of effective retention. Age 6 seems theideal time to begin therapy, since eruption of thepermanent maxillary incisors takes place during thetreatment period.

The orthodontic plan for the adolescent cleft childwith a relative class III relationship secondary to aretruded maxilla must be carefully scrutinized. Whilethe orthodontist may be able to narrow the upperand lower arches through extraction of first molar orbicuspid teeth and improve occlusal relationships,this scheme may well limit the appropriateorthognathic correction based on the soft-tissue rela-tionships in children. These children generally ben-efit from an orthodontic plan that allows the maxi-mum orthognathic advancement of their Le Fort Isegment.

The problem of edentulousness in the cleft areaof a patient with cleft lip and palate has historicallybeen approached by construction of a fixed bridge.This solution is not optimal due to problems withhygiene, violation of adjacent tooth structure, andfrequent need for bridge replacement. A recentsurvey of ACPA team management of alveolar cleftsreported that the preferred treatment for missingteeth was gap closure by orthodontic movement ofthe canine into the lateral position (25%), fixed bridgeplacement (21%), dental implants (19%), andremovable bridge placement (11%).246 The successrate of implants was >75% in the majority of cen-ters, but 72% reported the need for regrafting afterimplant placement to maintain adequate bony sup-port. Lilja and associates247 in Sweden and Jansmaand others248 in the Netherlands describe their expe-rience with osseointegrated titanium implants for den-tal rehabilitation of patients with cleft lip and palate.

Härtel and associates249 reviewed their experiencewith titanium dental implants placed into bone graftedalveolar clefts. The reported success rate of implan-tation was 96%, but repeat bone grafting was requiredin some patients to achieve adequate bone stock.They recommend that implants be placed no laterthan 6–8 weeks after bone grafting. Delayedimplantation may result in early bone resorption suchthat the graft is insufficient to support a dental implant.

Kearns and colleagues250 also reviewed theirexperience with endosseous implants in graftedalveolar clefts and reported a success rate of 90%. Inthis retrospective series, patients who requiredregrafting had an increased interval between initialbone grafting and implant placement. They con-cluded that the greater the interval beyond 4 months,the greater the likelihood that there will not beadequate bone to accept an implant.

Hillerup and others251 described autologous toothtransplantation into bone grafted clefts as a viabletreatment modality for dentoalveolar reconstruction.Hamamoto and coworkers252 looked into the idealtiming for autotransplantation of teeth into bone-grafted alveolar clefts in humans. At 6 months thebone graft was still undergoing remodeling andreplacement with newly formed bone. The activeremodeling was completed by 12 months. Theauthors conclude that tooth transplantation shouldbe performed soon after the formation of a bonebridge is confirmed, when bone remodeling is stillunderway, to provide an occlusal load to the graftand prevent resorption. Orthodontic treatment ofthe transplanted teeth can be started 3 months aftertransplantation when regeneration of the periodon-tal space and lamina dura is confirmed on dentalradiographs.

Gingivoperiosteoplasty

Gingivoperiosteoplasty or periosteoplasty involvesraising local periosteal flaps to create a periostealtunnel across the alveolar cleft to support bonegrowth. Hauben and van der Meulen253 documentedbone regeneration following periosteal flaps in thecraniofacial region and Schultz254 proposed the useof free periosteal grafts to repair secondary maxillarycleft deformities in adolescents.

In 1965 Skoog255 described a “boneless bone graft”to repair clefts of the primary palate. It consisted of awide flap of periosteum from the anterior maxillary

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wall based medially along the lateral edge of thepiriform aperture (Fig 27). On follow-up, however,Skoog’s method showed no distinct advantage oflong-term stability, and may interfere with growth asmuch as primary bone grafting.

Fig 27. The Skoog-type gingivoperiosteoplasty involving wideundermining of periosteal flaps to cover widely separated cleftalveolar segments. (Reprinted with permission from Lee CTH,Grayson BH, Cutting CB, et al: Prepubertal midface growth inunilateral cleft lip and palate following alveolar molding andgingivoperiosteoplasty. Cleft Palate Craniofac J 41(4):375, 2004.)

Renkielska et al257 evaluated the impact of earlygingivoperiosteoplasty on occlusal relationships bycomparing dental models of 5-year-old patientstreated with the Skoog method versus patients with-out alveolar intervention. No presurgical orthope-dics was used in either group. Group I was treatedwith lip repair (Randall-Tennison) and gingivo-periosteoplasty at age 6 months and Wardill-Kilnerpalatoplasty at age 12–18 months. Group II wastreated with the same protocol but did not undergogingivoperiosteoplasty at the time of lip repair. TheGoslon occlusion grading scale was used to evaluatedental models at a mean age of 5.3 years, prior toorthodontic intervention. In Group I, 50% of patientsdemonstrated poor occlusal relationships (Goslonscores 4 and 5) compared with 19.6% in Group II.Only 40% in Group I had clinical and radiographicevidence of bone formation and all would requiresecondary bone grafting due to poor bone quality.The authors concluded that primary alveolar repairusing the Skoog method has an adverse effect onmidfacial growth and the development of occlusalrelationships.

Rintala and Ranta258 reviewed the outcome of 90patients segregated according to closure with maxil-lary periosteal flaps (67) and pretibial periosteal grafts(23). Definite bone bridge developed in 64% and

85% of patients respectively, but >70% requiredsecondary bone grafting regardless of the type ofprocedure used. Lateral crossbite was seen in allpatients and anterior crossbite occurred in 80% ofboth groups. The primary use of periosteum wasabandoned because it did not prevent maxillary col-lapse, nor did it lessen the need for secondary bonegrafting.

Recent modifications of the gingivoperiosteoplastydiffer from the method described by Skoog. Skoog255

described widely undermined periosteal flaps usedto cover a wide alveolar cleft. Modern proponentsof gingivoperiosteoplasty use presurgical orthopedicsor nasoalveolar molding devices to align and apposethe alveolar segments. Minimally invasive local flapdesign is then used to cover the narrow alveolardefect256 (Fig 28). The group from NYU259 revieweda series of 20 unilateral and bilateral alveolar cleftstreated with presurgical orthopedics and gingivoperi-osteoplasty and report that 60% of patients did notneed an alveolar bone graft. Hellquist260 noted 80%of children who underwent delayed periosteoplastyat age 4–7 later developed good bone formation.

Wood, Grayson, and Cutting261 from NYU reportthe effect of gingivoperiosteoplasty on growth of themidface skeleton 6 years after primary surgical repair.All patients received preoperative orthopedics withpassive molding appliances followed by repair of thelip, alveolus, and nose in a single operation at age 3months. The only difference between treatmentgroups was whether or not gingivoperiosteoplasty wasperformed. The reason for not performinggingivoperiosteoplasty was incomplete approximationof the alveolar segments, usually because of a latestart of therapy. After relatively short follow-up, nosignificant difference in mean position of ANS-PNSwas found between the groups. In a follow-up study,the group from NYU256 compared the cephalomet-ric outcomes of prepubertal patients treated withnasoalveolar molding prior to lip repair with or with-out simultaneous gingivoperiosteoplasty. No signifi-cant difference in midface growth was noted betweengroups up to the mean follow-up age of 11.5 years.Pfeifer et al262 compared the average cost of treat-ment for patients treated with NAM, GPP, and lip/nose repair versus patients treated with primary lip/nose repair followed by secondary alveolar bonegrafting at NYU. The average cost savings of NAMand GPP compared to secondary alveolar bone graftwas $2999.

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Smahel and Mullerova263 compared x-ray cepha-lometric findings in boys 10 years of age who hadcomplete unilateral CLP according to whether theywere treated by periosteal flap (35) or primary bonegraft (20). Both groups were judged against the con-trols, who had the same type of cleft but receivedneither bone grafting nor periosteoplasty. The authorsnote improved jaw development with a periostealflap, with less jaw retrusion, better lip posture, andmaintenance of dental overjet. Five years later theauthors again analyzed facial growth and develop-ment in the same groups.264 The series withperiosteoplasty showed a marked proclination of theupper dentoalveolar component with the restora-tion of a positive overjet, whereas boys who receivedbone grafts showed a persistent anterior crossbite.The periosteoplasty group showed larger protrusion

of the lower jaw, while the bone graft patients exhib-ited a larger posterior rotation of the mandible. Max-illary growth did not differ between the operativegroups, both of which showed marked reduction inhorizontal and vertical dimensions. All patientsexhibited flattening of the face and impaired occlusalrelations from the increased mandibular protrusionand maxillary retrusion. In a 1998 update of theirseries, Smahel and Mullerova265 note that “primaryperiosteoplasty reduced maxillary retrusion, and themarked proclination of the upper dentoalveolar com-ponent with fixed appliances resulted in a positiveoverjet.” A more recent review led the authors toconclude that “the original outcome differencesobserved in childhood and adolescence were maskedby skeletal and dental compensations in adult-hood.”266

Fig 28. The Millard type gingivoperiosteoplasty involving minimal undermining of periosteal flaps to cover closely approximated cleftalveolar segments. The approximation of cleft alveolar segments is achieved by presurgical orthopedic molding. (Reprinted withpermission from Fig 28. Lee CTH, Grayson BH, Cutting CB, et al: Prepubertal midface growth in unilateral cleft lip and palate followingalveolar molding and gingivoperiosteoplasty. Cleft Palate Craniofac J 41(4):375, 2004.)

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Lehman and coworkers267 report an experiencewith one-stage closure of the entire primary palate atage 3mo in 61 patients. A single procedure is per-formed that closes the lip, anterior hard palate, andalveolus. Mucosal turnover flaps from the vomerand lateral nasal mucosal flaps provide the nasallining, and a buccal sulcus flap completes the oralrepair. The secondary palate is repaired by age 12months. The authors report that 95% of the patientshad complete and stable closure. The incidence offistula was 3 of 61 (5%). It remains to be seen inlonger follow-up if alveolar bone grafting is avoidedby this protocol. To date, of 22 patients who havereached the age of 8 or above, none has requiredbone grafting. The authors note that proceduresinvolving mobilization of large regions of periosteumcan exaggerate a skeletal deformity, leaving the patientwith marked malocclusion and facial disharmony. Ifthe prevomerine suture is not disturbed during sur-gery, many authors feel that early surgery is no moredeleterious to maxillary growth than surgery later inlife.267

Graft Donor Sites

Many different kinds of bone have been sug-gested for alveolar grafting, including bone fromthe mandible, rib, tibia, cranial bone, and iliac crest.Although most authors contend that the source ofthe bone graft does not seem to be the primaryfactor in determining successful outcome of alveo-lar bone grafts, much debate has been generatedover the optimal donor site.268 Disagreement existsregarding the viability of bone from different sites,the advantages of the biologic behavior of mem-branous (craniofacial) bone versus endochondral(extremity) bone, the type of bone (cortical or can-cellous) needed, the amount of bone required, andthe amount of bone available at various donorsites.269

Iliac crest cancellous bone is the most popularsource for secondary alveolar bone grafts due to thelarge amount of graft available, the ease of a two-team approach for simultaneous graft harvest, and areported success rate >90%.242,270–272 In a recentACPA survey of cleft palate teams in North America,246

83% of teams reported that the iliac crest was theirpreferred donor site. Disadvantages of the iliac crestdonor site are donor site pain and prolonged hospi-talization. Limited incision approaches to the iliac

crest and the use of a percutaneous trephine tech-nique have reportedly reduced postoperative painand hospital stay after iliac crest bone graft har-vest.273,274

Rib grafts are commonly used in primary bonegrafting but have limited application in secondarybone grafting due to the paucity of cancellous bone.Reported difficulty with orthodontic tooth movementin a cortical graft, the risk of pneumothorax, chestwound infections, and an unfavorable chest wall scarare further disadvantages of rib grafts.275,276 Never-theless, a review of 211 patients who underwent ribgraft harvesting for primary bone grafting disclosedno major complications.277 Tibia can provide anabundance of cancellous graft with minimal postop-erative pain and can be harvested quickly using atwo-team approach.269 Disadvantages of tibial graftsinclude a visible scar and potential damage to theepiphyseal growth plate in patients under 18 years ofage.244,278

Cranial and mandibular bone grafts are advocatedby some surgeons because the donor site is in thesame operative field as the cleft, there is minimalpostoperative pain, and membranous bone graft sur-vival may be superior to that of endochondral bonegrafts. Cranial bone was the preferred donor site for8% of ACPA teams in a recent survey.246 Comparedwith endochondral bone (iliac crest and tibia), mem-branous or mesenchymal-derived craniofacial bonegrafts have a greater fraction of cortical bone anddifferent architecture.279 Proponents of membranousbone grafts report less graft resorption compared withendochondral bone.270,280–282

Disadvantages of cranial and mandibular graftsare penetration of the inner table of the cranium,potential injury to tooth roots or mental nerve, lim-ited availability of cancellous bone in children, andthe high proportion of cortical bone that may increaseresistance for tooth eruption through the graft.278,282,283

Cortical bone grafts heal by creeping substitution andincorporation is dependent upon vascular ingrowth.Cortical grafts take longer to revascularize than can-cellous grafts, which heal by osteoinduction andosteoconduction. Cancellous bone grafts are pre-ferred by most surgeons because they becomerevascularized (within 21 days), incorporate faster,and remodel to the alveolar defect.271,276

Wolfe281 champions the use of cranial cancellousbone grafts and describes the harvesting technique.He reports successful graft take in 89.1% of patients

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treated with diploic bone for secondary anterior pala-tal closure. LaRossa and colleagues284 performed aretrospective study of 115 patients and noted thatalthough the success of iliac and cranial bone graftsseemed clinically similar in terms of graft “take,” iliacbone was statistically superior to cranial bone in allcleft types when judged radiographically. Cohen268

reported equivalent results with grafts obtained froncranial bone and iliac crest; however, the operatingtime was longer for cranial bone grafts and thepotential for significant complications such as CSFleak, dural tear, hemorrhage, and epiduralhematoma was greater.

Mandibular bone grafts have the additionaladvantages of reduced operating time, absence ofcutaneous scar, and easily accessible donor site.283,285

Enemark and colleagues282 retrospectively compared101 patients grafted with either mandibular or iliaccrest bone. Although both groups achieved satisfac-tory bone levels of the alveolar graft, the mandibulargroup was significantly better. Both groups had asimilar number of patients with impacted caninesthat required surgical exposure for eruption. Theauthors concluded that the higher cortical content ofmandibular bone did not seem to impede tooth erup-tion. The presence of the cleft lateral incisor, how-ever, was a significant predictor of natural canineeruption. Complications due to mandibular bonegraft harvest included 4 infections, loss of 2 perma-nent mandibular canines, and radiographic injury intwo canine roots. No complications were reportedfrom iliac crest harvest.

Borstlap and associates285 compared mandibularand rib grafts in secondary grafting of the alveolarcleft. They noted that 16% of rib graft cases showed50% or greater resorption of the graft. In contrast,resorption was not noted in any patient grafted withmandibular bone.

A recent prospective study286 compared the opti-cal density of secondary alveolar bone grafts obtainedfrom the iliac crest and the tibia. A significant decreasein relative bone density was seen in both groupsduring the 3mo postoperative period. No differencein bone density was noted between grafts from theiliac crest or the tibial donor site. The average post-operative hospital stay was 5 days for the iliac crestgroup and 3 days for the tibia group.

Sadove and coauthors270 compared the use of cra-nial bone harvested by two different techniques toiliac donor bone in the closure of alveolar clefts. Both

iliac bone and calvarial bone dust harvested with theHudson brace resulted in high rates of osseous conti-nuity between the maxillary segments and in clinicalresolution of the alveolar defect, as evident radio-graphically. Calvarial bone obtained with a cran-iotome had a statistically lower success rate (53%)than calvarial bone harvest with a Hudson brace (80%)and iliac crest bone (93%). This study implicates themethod of bone harvest, not just location of donorsite, may influence success of outcome.

McCanny and coworkers273 compared the tre-phine technique with the open hip technique forharvesting cancellous bone grafts. The open tech-nique resulted in greater postoperative morbidity,such as a limp and an infected hematoma, and theauthors conclude that trephine is the preferred har-vesting technique for alveolar bone grafts.

Rudman287 prospectively evaluated the postopera-tive morbidity after iliac crest bone graft harvest in 22consecutive patients. He advocates the use ofbupivacaine and limits muscle dissection and peri-osteal elevation during outfracture of the medial cor-tex. The average incision length was 29mm, a suffi-cient graft volume was obtained in all but one patient,and postoperative ambulation occurred at an aver-age 3h 18min, with only mild discomfort. The hos-pital stay was significantly shorter than that reportedby other authors.286,288 One patient was dischargedthe day of surgery and the remainder were dischargedthe day after surgery. No hematoma, paresthesia, orinfection occurred. Patients favored the postopera-tive side during ambulation for the first postoperativeweek but no gait disturbance was seen at the 2weekfollow-up visit.

Boustred and associates274 described a minimallyinvasive harvesting technique for cancellous bonegrafts from the ilium. Through a small step incision5–8mm in length, the midportion of the iliac crest israised. With a curette, a core of fresh cartilage isremoved and cancellous bone is scraped frombetween the inner and outer cortices. The amountof bone obtained is sufficient for narrow as well aswide defects. The technique is quick and needs nospecial equipment. There is minimal donor site mor-bidity and the patient walks comfortably from thefirst postoperative day.

Nique and associates289 used particulate alloge-neic bone for grafting alveolar clefts in 20 patientsand report universal x-ray and clinical signs of bonebridging in the cleft. Although Allard and others290

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have found that frozen homologous bone is equal inclinical behavior to autogenous bone grafts in sec-ondary cleft repairs, osteotomies, and ridge augmen-tations, questions regarding bone density and peri-odontal bone support remain unanswered. As ElDeeb291 notes, it is generally agreed that an autograftis superior to allogeneic graft for repair of an alveolarcleft defect.

Primary Bone Grafts

Primary bone grafting of the alveolar cleft is per-formed before palate repair qwhen the child isyounger than 2yo. Primary bone grafting has beenadvocated because early stabilization of the maxil-lary arch minimizes transverse maxillary collapse, re-duces occlusal distortions between the upper andlower arch, may decrease the need for subsequentorthodontic and orthognathic correction, and earlyclosure of the oronasal fistula improves oral hygieneand speech development.235,237,276,292,293 Disadvan-tages of primary bone grafting include potential max-illary growth disturbance, poor dental arch form,inadequate bone formation, and possible need forsecondary bone grafting (25% reported byEppley).237,276,294,295

Primary bone grafting of cleft palates was popularin Europe and Scandinavia since the early 1960suntil 1982, when Friede and Johanson294 publisheda long-term follow-up report of maxillary growthimpairment in adolescents who had had primarybone grafting in infancy. Rosenstein and others296

described a modified technique for primary bonegrafting which they have been using since 1965. Onlong-term follow-up, they report no significant max-illary growth disturbance.

In 1982 Friede and Johanson294 reviewed the ado-lescent facial morphology of children who hadundergone early bone grafting of their alveolar seg-ment. Subject age ranged from 15–20y and included19 bilateral and 42 unilateral cleft patients. Cepha-lometric data indicated maxillary retrognathia in bothcleft categories as well as deficient vertical descent ofthe maxilla, especially in the anterior part. In about40% of the bilateral and 50% of the unilateral cleftpatients, midfacial growth attenuation had reachedsuch magnitude that surgical advancement of themaxilla was necessary. Fusion of the suture betweenthe premaxilla and vomer was suggested as the rea-son for the typical midfacial morphology seen in the

patients with the most pronounced growth impair-ment. The bone grafting sequence consisted of ini-tial lip adhesion and closure of the cleft in the ante-rior palate by means of vomerine flap. The secondsurgery consisted of bone grafts to the alveolar pro-cess and hard palate clefts with concomitant final lipclosure. The grafts were autogenous cancellous bonetaken from the tibia. A final closure of the soft palatewith the pushback method completed the repair. Asa result of this analysis, the authors concluded thatearly bone grafting of the alveolus should be avoided.In direct contrast to their earlier impression, theauthors noted pronounced midfacial and alveolargrowth impairment in these patients, which promptedthem to withdraw the recommendation for primaryearly bone grafting in cleft cases.

In a study of maxillary asymmetry in children withclefts of the lip and palate treated at three differentcleft palate centers in Europe, Molsted et al297 deter-mined that children who receive presurgical ortho-pedics and primary bone grafting at the time of lipclosure—at age 4–6mo—achieve a more symmetri-cal dentoalveolar development than if bone graftingis not performed at an early age. However, maxil-lary development in these children is retarded notonly in the sagittal plane, but also in the transverseplane. Children who are treated by early lip closureand vomerplasty with no involvement of the alveolarprocess exhibit asymmetrical development, with atilting premaxilla and deviating inclination of the cen-tral incisors. This study once again confirms the nega-tive effect of vomerplasty on midfacial growth.

An 11y follow-up of the effects of early bone graft-ing in infants born with complete clefts of the lip andpalate is presented by Robertson and Jolleys.298

Matched pairs of similar cleft types were treatedaccording to the same treatment protocol, except thatonly one of each pair received an autogenous ribbone graft at age 15mo. The overall impression wasthat early bone grafting is not beneficial to the patient.

Nylen et al299 report successful early primarybone grafting of cleft palate defects since 1953,albeit with very careful orthodontic treatment andfollow-up. Nordin, Larson, and Ideberg,300–303

Nelson and associates,304 and Perofsky et al305

report successful outcomes after early bone graft-ing of alveolar clefts. These investigators advocateprimary bone grafting in the management of cleftpalate as long as grafting is accompanied byappropriate maxillofacial orthopedics.

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Rosenstein et al296 published the longest follow-upof patients treated with primary bone grafts. Thesurgical sequence that the authors advocate involvespremaxillary orthopedics followed by lip repair atabout 6 weeks of age.292,293,306–309 The appliance isretained following lip repair to mold the greater andlesser maxillary segments into place. At approximateage 4–5mo, when the segments abut and good archalignment has been achieved, an autogenous split-rib bone graft is inserted in the upper buccal sulcus,with margin flaps off the alveolus turned for orallining. The method avoids the use of any vomerineflaps for closure. The prosthesis is retained until pal-ate repair is completed at about 12 mo of age.

In over 35 years of experience using the sameprotocol, multiple studies comparing the craniofacialand dental outcomes of primarily grafted andungrafted (or secondarily grafted) groups have shownlittle difference in growth, similar maxillomandibularrelationships, and a tendency toward better toothsupport after primary grafting.292,293,306–310 Accord-ingly, the authors conclude that there is no real growthdisturbance with the procedure, and that an improvedfunctional and more stable dental relationship isachieved. The success of their technique is attrib-uted to the fact that bone grafting is not performeduntil the maxillary segments are aligned withpresurgical orthopedics, which facilitates placementof an onlay rib graft requiring only minimal peri-osteal dissection.

In their most recent review, Rosenstein et al292

evaluated the need for secondary surgery after pri-mary bone grafting and assessed the status of adja-cent teeth. The incidence of orthognathic surgerywas 18.3% (compared to 25% reported by Ross295);pharyngoplasty was required in 3.65%; and oronasalfistula closure was required in 29.3%. The need forsecondary bone grafting was not reported. One ofthe potential benefits of early bone grafting is bettersurvival and function of the cleft lateral incisors byproviding an adequate bony base for eruption.Rosenstein et al292 reported agenesis of the cleft lat-eral incisor in 41% of patients with unilateral cleftsand 50% of patients with bilateral clefts. Of thosepatients with present cleft lateral incisors, alignmentand use of this tooth was possible in 53.1% of unilat-eral clefts and 57.7% of bilateral clefts. Studies com-paring primary and secondary bone grafting haveshown less tooth loss, better tooth attachment, and

fewer missing or malformed teeth adjacent to cleftstreated with early bone grafts.311,312

Eppley313 advocates primary bone grafting byRosenstein’s method in properly selected patients(Fig 29).

Fig 29. Primary alveolar bone grafting technique. A, Incisionsand mucosal flap design around cleft site. B, C, After palatal andnasal closure, a split rib graft is onlaid superperiosteally across thelabial surface of the maxillary segments. D, Advancement ofmucosal flap over graft for oral closure. (Reprinted with permis-sion from Rosenstein SW, Long RE Jr, Dado DV, et al: Comparisonof 2-D calculations from periapical and occlusal radiographsversus 3-D calculations from CAT scans in determining bonesupport for cleft-adjacent teeth following early alveolar bonegrafts. Cleft Palate Craniofac J 34:199, 1997.)

Eppley reports a success rate of 95% in patientswith adequate preoperative arch alignment.313 Ini-tial cleft width correlated with ability to align thearches preoperatively. Initial cleft widths of 9mmhad a greater chance (90–95%) of achieving ad-equate alignment with presurgical orthopedics thanwider clefts, 75% for clefts 9–12mm, and 37% forclefts >12mm.237,239,313 The effect of residual cleftwidth (after presurgical orthopedics) on primary bonegraft success was also significant. Primary grafts per-formed on clefts with residual widths of 0.0–0.2mmhad a success rate of 92%, but graft incorporation

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decreased to 70% when the residual cleft width was>2.0mm. On the basis of these findings, Eppleyadvocates primary bone grafting for patients whohave obtained end-to-end alignment of the maxil-lary segments and recommends secondary bone graft-ing for patients who are unable to obtain arch align-ment.313 Secondary bone grafting was needed in25% of patients after primary bone grafting. Eppleyconsiders this part of a staged repair because patientsstill benefit from primary grafting due to fistula clo-sure, have better oral hygiene, and better arch stabil-ity. They also require a smaller volume of bone graftand less flap dissection in the second procedure.

SECONDARY BONE GRAFTING

In a recent survey of ACPA cleft palate teams, 90%of the respondents reported that they performed sec-ondary bone grafts, and 83% repaired the alveolarclefts when the patient was 6–9yo.246 This populartechnique for repairing the alveolar cleft during themixed dentition stage was initially reported by Boyneand Sands314 in 1972. They preferred repairing thecleft at age 9–11 before the eruption of the canineteeth. Since sagittal and transverse facial growth ofthe maxilla is essentially complete by age 8, no adversegrowth effect has been associated with repair at thisstage.315–317 Some authors report successful repairbetween ages 5 and 7 without adverse growtheffects.244,318 Although most surgeons use the stage ofcanine root development (¼ to ¾ root formation) fordetermining the timing of graft placement, someauthors attempt to preserve the cleft lateral incisorand advocate grafting prior to its eruption.246,282

Early secondary bone grafting is performed onpatients between ages 2 and 5 years. Advocates ofrepairing the cleft during this stage of primary denti-tion, prior to the eruption of secondary dentition,believe that the advantages are better bone forma-tion and orthodontic movement of the central inci-sors as well as the canine.275 Late secondary bonegrafting (>12yo) has a significantly lower success ratethan earlier grafting before eruption of thecanine.319,320–322 Adolescent and adult patients need-ing alveolar cleft repair may benefit from segmentalLe Fort I osteotomies for simultaneous correction ofmidface retrusion and alveolar gap closure withcoincident bone grafting.244

Despite the reported success rate of >90% forsecondary bone grafts,242,271,272 surveys of cleft care

have revealed inconsistent and disappointing results.The Clinical Standards Advisory Group (CSAG) studyevaluated dentofacial outcomes from cleft centers inthe United Kingdom and found that 15% of 12yohad not received an alveolar bone graft and only58% of bone grafts performed were successful.323

The ACPA survey of cleft repair by teams in the U.S.revealed wide variability in the dental criteria forgrafting, and 86% of teams did not routinely usepostoperative x-rays to evaluate bone grafts.246

Cohen268 states that the keys to secondary graftingof residual alveolar clefts are• adherence to meticulous surgical technique• simultaneous closure of coexisting oronasal or pala-

tal fistulas• use of cancellous bone particles only• coverage of the grafts with well-vascularized flaps

Eppley and Sadove235 emphasize that flap design isone of the most crucial determinants of secondarybone graft success. The use of keratinized gingivalmucoperiosteal flaps, not labial-based (non-keratinized)mucosal flaps, is very important for the success of theprocedure because keratinized mucoperiosteal flapsprovide the essential periodontal qualities to sustaintheir longevity and support tooth development. Thereis a synergistic interaction between tooth eruption,alveolar bone growth, and the keratinized gingivawhich attaches to the teeth via fibers of the periodon-tal membrane.271 Bergland271 reported that grafts cov-ered with the loose non-keratinized mucosa resultedin a deficient interdental bony septum and less opti-mal gingival margins. Physiological osteogenesis occurswhen a tooth erupts through the bone graft and theattached keratinized gingiva because it elicits accretionof bony tissue on the alveolar crest.271,319

Abyholm and colleagues319 offer an excellent dis-cussion of the appropriate timing for secondary bonegrafting of alveolar clefts. A review of their casessuggests that optimal results were obtained whenbone grafting was performed before full eruption ofthe cleft-side canine. In this situation the knownpotential of an erupting tooth to induce alveolarbone generation proved to be of great advantage.By deliberately guiding the erupting canine throughthe grafted area close to the incisor, a nearly normalintraalveolar septum was formed, and the gap in thedental arch was closed orthodontically in 23 of 26clefts. Complications in this series of 89 clefts con-sisted of 2 bone graft losses secondary to infection.

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Bergland and associates271,272 reported a combinedsurgical–orthodontic approach designed to eliminatealveolar clefts by means of secondary bone grafting at9–11yo, before the eruption of the cleft canine. Goodbone formation was noted in 98% of the clefts grafted,with no difference whether the deformity was unilat-eral or bilateral. The good outcome was ascribed tothe close multidisciplinary relationship that facilitatedpreoperative orthodontics; the timing of the alveolargraft (before canine eruption); careful bone graftingtechnique with properly designed gingival flaps; post-operative orthodontic treatment; and meticulousradiographic follow-up.

The long-term results after secondary bone graft-ing of alveolar clefts was evaluated by Enemark andassociates.320 Three groups of patients (224 clefts)were studied: One group had alveolar bone graftsbefore the eruption of the canines; a second grouphad alveolar bone grafts after the eruption of thecanines; and the third group had alveolar bone graft-ing at age >16y. Significantly better results areachieved with secondary bone grafting if the treat-ment is performed before eruption of the canines. Inthe younger groups, the marginal bone level wasfound to be significantly higher among unilateral CL/P patients than those with bilateral deformities. Noinfluence of the procedure on sagittal growth of themaxilla could be demonstrated, whereas the ante-rior facial height was reduced.

Many surgeons agree that the optimum time forbone grafting is chosen according to the stage ofcanine development. Following Abyholm’s319 lead,El Deeb et al324 have determined that the best timefor grafting in terms of normal canine eruption is at9–12y when the roots of the canines are ¼ – ½formed.

Kalaaji and associates321 compared the outcomeof secondary and late secondary bone grafting in 46patients with complete unilateral cleft lip and palate.Factors evaluated in this study were the surgeon’sexperience, morphology of the cleft prior to grafting,stage of eruption of permanent canines, and pres-ence or absence of permanent lateral incisors. Thedehiscence rate was 23% and total failure rate was4%. Alveolar bone height was >75% of normalalveolar bone height in 81% of patients; between50% and 75% of normal bone height in 13%; and<50% of normal bone height in 6%. The cleft spacewas closed by orthodontic means in 49% of patients.The best results were obtained when bone grafting

was performed before the canines erupted. Themore experienced surgeons obtained the best resultswith regard to alveolar bone height.

Paulin and colleagues322 compared the maxillarydimensions in two groups of patients undergoingalveolar bone grafts, one group before the eruptionof the canine tooth and the second group after theeruption of the canine tooth. Initial healing wasmore favorable in the group that was grafted prior tothe eruption of the canines. Among those havingbone grafts prior to the eruption of the canines, theinterdental bone height in the grafted area was morethan 75% of normal bone height, whereas it wasonly 66% of normal bone height in the group thatwas grafted after the canines erupted. The authorsconclude that bone grafting should be done prior tothe eruption of the canines when possible.

A recent retrospective study analyzed the outcomeof patients treated with Byrd’s limited-incision tech-nique for secondary alveolar bone grafting.242 Thetraditional approach for secondary bone graftinginvolves incisions that extend back to the molar witha posterior back cut and resultant raw area, theelevation of broad based mucoperiosteal flaps, andremoval of the gingival mucosal tissue inside the cleft319

(Fig 30). Flap design in the limited incision tech-nique utilizes the attached gingiva inside the cleftand the gingival incision extends only one toothbreadth on either side of the cleft to limit periostealand periodontal dissection (Fig 31). Cancellous bonegrafts from the iliac crest were placed prior to theeruption of the canine in most patients (some lategrafts were performed due to delayed patient pre-sentation). No postoperative splints were used andno diet restrictions were enforced. The incidence ofsuccessful bone grafts was 95% (3 failures) which iscomparable to reports from authors using the tradi-tional approach. Agenesis of the cleft lateral incisorwas associated with poor coronal height and graftfailure. Extraction of the cleft lateral incisor wasassociated with graft resorption. Orthodontic gapclosure was associated with less graft resorption butincreased root resorption in the adjacent teeth. Theadvantages of this technique include minimal poten-tial for periodontal injury due to limited dissection,no postoperative splinting which may improve post-operative oral hygiene and plaque control, and nodiet restrictions.

Honma and others325 used computed tomogra-phy to evaluate the fate of secondary cancellous

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Fig 30. (a) Location of incisions for repair of unilateral alveolar cleft. (b) Nasal layer is repaired. (c) Boly alveolar cleft is filled withautologous bone graft. The graft is placed over the inferolateral pyriform rim as an onlay graft to augment the alar base. (d) Closure ofthe oral layer. The site of the back cut in the lesser segment mucoperiosteal flap is allowed to heat secondarily. (Reprinted with permissionfrom Daw JL Jr, Patel PK: Management of alveolar clefts. Clin Plast Surg 31:303, 2004.)

Fig 31. Secondary alveolar bone grafting utilizing a limited incision technigue. (Reprinted with permission from Gosman A, Potter JK,Byrd HS: Secondary alveolar bone grafting utilizing a limited incision technique. Presented at the annual meeting of the American CleftPalate-Craniofacial Association, Myrtle Beach, South Carolina, April 2005.

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bone graft of alveolar clefts. They noted decreasingvolume of new bone in the cleft site from 3mo to 1yafter grafting. This trend was reversed in 2 patientswho had tooth eruption into the grafted site, suggest-ing that a functional load in this area, be it a func-tioning tooth or endosseous implant, might preventbone resorption.

Tai and others326 used computed tomography todetermine the percentage of bone loss between theimmediate postoperative period and one year aftersecondary bone grafting. The total average volumeloss was 43.1% after 1y. Eruption of the caninethrough the graft accounted for 53.4% of the totalaverage volume loss. The authors concluded thatthe volume loss with canine eruption did not repre-sent a detrimental effect on the graft but shows thatthere are transient volumetric changes involvingresorption during tooth eruption.

Arctander et al327 used computed tomography tocompare secondary bone graft volume at the cleft sitewith the corresponding non-cleft side in patients whowere grafted more than 20y previously. The bonemass was significantly less on the grafted side (97mm2)compared with the non-cleft side (157mm2), yet allpatients had satisfactory clinical and functional resultswith orthodontic gap closure at the grafted site.

RECENT INNOVATIONS IN ALVEOLAR CLEFT MANAGEMENT

Recent innovations in the treatment of alveolarclefts include the use of platelet-rich plasma (PRP),bone morphogenic protein (BMP), bone substitute,and distraction osteogenesis.

Platelet-rich plasma is an autologous source ofgrowth factors (TGF ß-1, TGF ß-2, and PDGF) thatmay increase bone formation after grafting.328

Whitman et al329 reported the use of autogenousplatelet gel in the repair of alveolar clefts. Segura-Castilla et al330 performed a prospective, single blind,randomized clinical trial to evaluate the possiblereduction in bone graft resorption by the applicationof fibrin glue. Commercially available fibrin glue(fibrinogen + thrombin) is a hemostatic and adhe-sive agent based on cryoprecipitates of human plasmaand is considered 99.5% biosecure.330 Patients whoreceived fibrin glue during secondary alveolar bonegrafting (13) were compared with controls who didnot (12). Overall, the results of the fibrin glue groupwere better than controls, although statistical signifi-cance was not obtained except for mean coronal

bone volume. Potential problems with the use offibrin glue include a 0.5% chance of infectious trans-mission, risk of anaphylaxis, and cost. Long termresults and a larger sample population are needed todetermine efficacy of fibrin glue.

Guided bone regeneration (GBR) with membranebarriers has been used to regenerate bone, oftenprior to the placement of dental implants. Mem-branes function to exclude the ingrowth of gingivalcells and fibroblasts into a bony defect and thereforefacilitate osteocyte repopulation with subsequentbone regeneration. Peled and colleagues331 appliedthis novel technique to the treatment of alveolarclefts. Patients were randomized to one of threesurgical groups: 1) autogenous alveolar bone graftplacement (ABG); 2) nonresorbable expandedpolytetrafluoroethylene Gore-Tex membrane (GTM)placement; or 3) ABG plus resorbable polylactic-polyglycolic acid (Resolut XT) membrane barrier(ABM) placement. The reduction in the overallmean defect size was significantly greater in theABM group (177mm2) compared with the GTM(20.51mm2) and ABG (41.69mm2) groups. Althoughgreater defect fill was achieved with the adjunctiveuse of membrane barriers, 40% of patients had mem-brane exposure. Further studies are required to con-firm the benefits of guided bone regeneration usingmembrane barriers in the treatment of alveolar clefts.

Chin and colleagues23 reported the successful useof recombinant human bone morphogenic protein(rhBMP-2) as an alternative to iliac crest bone grafts inthe repair of alveolar clefts. In a series of 43 patients,50 cleft sites were treated with rhBMP-2 embeddedin an absorbable collagen sponge (ACS). Successfulosseous union was achieved in 49 of 50 cleft sites.The authors reported histologically normal viable boneat the treatment sites as well as normal tooth eruptionand normal response to orthodontic tooth movement.The authors concluded that rhBMP-2 is a viable treat-ment option for alveolar clefts.

Another recent innovation in alveolar cleft man-agement is interdental distraction osteogenesis. Liouand associates332 used bifocal distraction osteogen-esis to close large alveolar clefts. A segmental poste-rior maxillary osteotomy was performed and aninterdental distraction device was used to close thealveolar cleft followed by rapid orthodontic toothmovement to close the dental gap. The resultingposterior dental gap filled with bony regenerate. Yenet al333 reported a similar technique using a continu-

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ous spring force distraction device for closure of largealveolar clefts. Mitsugi et al334 reported the successof a bone-borne distractor in combination with anorthodontic arch wire for guidance in alveolar cleftclosure using transport distraction osteogenesis. Theauthors advocate this method over the use of tooth-borne distractors, which in their previous experiencewere inadequate, unstable, and imposed a signifi-cant burden on the teeth. Distraction osteogenesisfacilitates the closure of large clefts, corrects occlusalrelationships, overcomes soft-tissue deficiencies, andeliminates or reduces the volume of bone graftrequired.

VELOPHARYNGEAL FUNCTION

The velopharynx is a rectangular structure thatacts as a sphincteric valve to separate the oral andnasal cavities during speech and swallowing. Theanterior border of the velopharynx is the velum, thelateral borders are the lateral pharyngeal walls, andthe posterior border is the posterior pharyngeal wall.During sphincteric closure the velum moves superi-orly and posteriorly primarily by contraction of thelevator veli palatini. Pharyngeal wall movement ismediated primarily by the superior constrictor andthe palatopharyngeus muscles. The lateral walls movemesially during valve closure. The posterior wallsmove anteriorly either in a diffuse manner or as adiscrete shelf known as Passavants ridge.

The velopharyngeal valve selectively channels air-flow and acoustic energy under different pressures tothe oral and nasal cavities. Rapid and competentvalve function is needed for normal speech produc-tion. The valve is closed to prevent the nasal escapeof air and sound during production of oral sounds(most consonants and all vowels) and during swal-lowing to prevent nasal regurgitation. The valve isopen during nasal consonant production (n, m, ng)and to permit nasal respiration.

Velopharyngeal dysfunction (VPD) results from fail-ure of the sphincteric mechanism due to structuralor neurologic impairment or mechanical interference.Structural impairment may result from tissue defi-ciency, unrepaired cleft palate, submucous cleft pal-ate, short immobile palate after cleft palate repair,large deep nasopharynx, or palatal fistula. Neuro-muscular dysfunction due to neurologic disorders ortrauma may impair the function of the muscularsphincter. Mechanical interference to valve closure

may result from hypertrophic tonsils or adenoids orfrom wide pharyngeal flaps.

There is confusion and a lack of consensusregarding the preferred terminology of the hypernasalvelopharyngeal dysfunction in patients with cleft pal-ate. Velopharyngeal inadequacy has been used torefer to inadequate velopharyngeal closure of anycause. Velopharyngeal insufficiency refers to struc-tural or anatomic abnormalities of the velopharyngealsphincter. Velopharyngeal incompetency has tradi-tionally been used to denote sphincter abnormalitiesdue to neuromuscular disorders, although someauthors use this term generically to refer to sphincterdysfunction of any cause.337 Because the abbrevia-tion VPI has been used to refer to any of these con-ditions, the distinctions are frequently overlooked.Marsh338 advocates use of the term velopharyngealdysfunction (VPD) because it represents the physi-ologic impairment without attempting to name theetiology. Patients with cleft lip and palate may showhyponasal velopharyngeal dysfunction secondary toeither nasal obstruction from the cleft nasal defor-mity or severe midface retrusion.

Velopharyngeal dysfunction occurs in about 20%of cleft patients after primary palatoplasty.165,339–341

The characteristic speech patterns of VPD are due toincreased transmission through the nasal cavity,hypernasality, nasal emission, and weak pressure con-sonants and frication. Hypernasality is secondary toexcessive nasal resonance. Nasal emission resultsfrom turbulent airflow through the nasal cavity. Theinability to generate sufficient oral pressure due tonasal leak results in weak consonant production,fricatives, and compensatory articulation errors.Patients with VPD compensate for the lack of ante-rior oral cavity pressure by using their glottis, phar-ynx, or other posterior structures as valves to gener-ate pressure. These compensatory patterns arelearned behaviors that persist after surgical treatmentof VPD and require speech therapy for correction.The likelihood that a child will be able to overcomecompensatory speech patterns is greater when surgi-cal treatment of VPD is performed at a younger age,ideally between ages 4 and 6.342

The comprehensive evaluation of VPD is basedon subjective and objective assessment by a cleftpalate team. The current recommendations for treat-ment of VPD are based on the specific anatomic andfunctional characteristics of valve closure. Many dif-ferent surgical techniques have been proposed for

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treatment of VPD and there is little convincing evi-dence that any one operation produces significantlybetter speech than any other.106,180,343–347 Mostauthors agree that certain procedures are more suit-able for addressing problems with the pharyngealcomponent of closure, while other techniques maybe better for velar deficiencies.

EVALUATION AND DIAGNOSIS

Smith and Guyette348 recommend complete evalu-ation by a speech pathologist to include 1) percep-tual speech, 2) anatomy, and 3) physiology of thevelopharygeal complex. Indirect speech evaluationcan begin in the first year of life by questioning theparents about speech (nasal quality?) and swallowing(frequent regurgitation?). At age 2–3, when childrenare more cooperative, a speech pathologist is usuallyable to perform a perceptual evaluation ofvelopharyngeal function and administer standard-ized articulation tests.

Johns and others337 emphasize that speech assess-ment should focus on resonance quality, airflow, airpressure, and compensatory articulation. Nasal reso-nance may be detected clinically by comparingoccluded and nonoccluded nares for the presenceof nasal vibration during vowel production. Nasalemission of airflow can be identified by nasal flaringor audible emission. Inaudible nasal emission canbe detected by placing a mirror under the nares andwatching for fog. Inadequate air pressure can beevaluated during the production of oral consonantsparticularly plosives (p) and fricatives (f). Compensa-tory articulations that result from the use of alterna-tive airflow valves include pharyngeal fricatives, glot-tal stops, posterior nasal fricatives, velar fricatives,pharyngeal stops, and middorsal palatal stops.337

Anatomic examination provides additional infor-mation about velopharyngeal function. Intranasalexam should be performed to evaluate for septaldeviation and other causes of obstruction that mayresult in hyponasality. Intraoral examination is nec-essary to evaluate structures that may contribute topoor speech, including reflexive behavior of the pal-ate (gag reflex), palatal movement with phonation,fistula, size and presence of tonsils and adenoidaltissue, contour of the hard palate, occlusion prob-lems, and tooth malposition.

Information about velopharyngeal structures, func-tion, and closure patterns is provided by instrumentevaluation. Instruments that permit the observation

of velopharyngeal function include nasoendoscopyor nasopharyngoscopy and videofluoroscopy. Physi-ologic assessment uses acoustic and aerodynamicinstruments to quantify velopharyngeal function.Instrument assessment of the velopharynx may beunreliable in patients with inadequate articulatoryeffort of oral consonants. Marsh338 recommendsspeech therapy to optimize the production of oralconsonants prior to instrument evaluation.

Nasopharyngoscopy is used to assess the patternof velopharyngeal closure by visualizing the excur-sion of the velum, movement of the lateral and pos-terior pharyngeal walls, and the associated contribu-tions of these structures during attempted valve clo-sure. Endoscopy provides important informationabout velar movement but is limited in that it onlyprovides one cephalad viewpoint of the velum; visu-alization of the more inferior structures is difficultduring speech production. The pattern ofvelopharyngeal closure is important and may beassociated with the degree of hypernasality and nasalemission (Fig 32). Coronal pattern closure is themost common, then circular, and finally sagittal.342

Igawa and colleagues349 predict the velopharyngealclosure mechanism in infants with repaired cleft pal-ate on the basis of their preoperative velopharyngealmovement. Their impression is that infants with themedial movement type are more prone to VPI post-operatively.

Videofluoroscopy is another useful method ofevaluating and diagnosising VPD. In this techniquethe patient swallows a barium-containing substancewhich coats the surface of the oropharynx. Immedi-ately afterwards the patient is asked to duplicate cer-tain sounds while fluoroscopic images are recorded.The lateral, frontal, and submentovertex video-fluoroscopic views are most reliable for assessingvelopharyngeal competence.350 When the adenoidsare enlarged, the Towne view demonstrates thevelopharyngeal orifice better than the basal projec-tion.351 Interpretation of videofluroscopic images issometimes difficult due to shadows produced byadjacent anatomic structures. Another disadvantageof this technique is that it exposes patients to ionizingradition although the doses are relatively small.342,352

Tracings of lateral cephalometric radiographs at restand during speech production provide quantitativeinformation about velar length, extent of velar con-tact, velopharyngeal gap, and depth of the posteriorwall348 (Fig 33).

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Many clinicians believe that endoscopy andvideofluoroscopy should be indispensable andcomplementary techniques, the former giving pri-marily qualitative information and the latter quanti-tative data.353,354 Both types of information aid inthe planning of surgical interventions so thatthe procedure can be tailored to the patient’s spe-cific anatomic or functional deficiency.

Computerized tomography and magnetic reso-nance imaging are less common methods for visual-izing the velopharyngeal mechanism.355,356 Real-timemotion studies are possible with these noninvasiveimaging techniques.352 The lack of exposure to ion-izing radiation makes MRI an attractive option. MRIhas also been used for imaging the orientation andattachments of the muscles in the velum, which mayhelp determine whether surgery or speech therapy isthe recommended treatment.348 High cost and lackof outcome studies currently limit their clinical appli-cation.

Mitnick and associates357 used magnetic reso-nance angiography in patients scheduled for pha-ryngeal flap surgery to rule out velocardiofacialsyndrome, also known as Shprintzen’s syndrome.Abnormal anatomy of the internal carotid arteries,sometimes with medial displacement, is known tobe a feature in many patients with this syn-drome.358–360 In this case, elevation of a pharyn-geal flap places the carotid artery at risk of acci-dental injury. In Mitnick’s study all 20 patientsimaged were found to have anomalies of eitherthe carotid arteries, the vertebral arteries, or both.In 2 patients the internal carotid arteries werealmost directly under the mucous membrane ofthe pharynx. The information obtained from themagnetic resonance angiography allowed for safeflap design.357

Physiologic evaluation of velopharyngeal functioncan be preformed using acoustic nasometery, pres-sure-flow aerodynamic studies, and rhinomano-metry.348 Nasometry is used to evaluate the ratio oforal acoustic energy to oral + nasal acoustic energyduring speech production. This ratio is referred to as‘nasalance.” Patients with VPD exhibit highernasalance scores than their normal-speaking coun-terparts (32% vs 15%).361,362 Low nasalance scoresare associated with nasal obstruction. Nasalancescores are useful in the assessment of nasality, theevaluation of treatment, and as biofeedback duringspeech therapy.348

Fig 32. Velopharyngeal closure patterns. The heavy arrowsrepresent the pharyngal wall (anterior, lateral, or posterior),which contributes the most to velopharyngeal closure. Forinstance, in the coronal closure pattern, the soft palate contributesmost to velopharyngeal closure. In the circular pattern withposterior pharyngeal wall contribution (PPW), the velopharyngealclosure has relatively equal contribution from the anterior andlateral pharyngeal walls, with some posterior wall contribution.The thin arrows represent the minor amount of pharyngeal wallmotion. (Reprinted with permission from Senders CW: Manage-ment of velopharyngeal competence. Fac Plast Surg Clin NorthAm 9(1):27, 2001.)

Fig 33. Left, Tracing of lateral cephalometric roentgenogramduring rest showing resting palatal length (RPL), pharyngeal depth(PD), and cranial base angle (CBA). Right, Tracing of lateralcephalometric roentgenogram during sustained /s/showing mea-surement of effective palatal length (EPL), velopharyngeal gap(VPG), and velar height (VH). (Reprinted with permission fromSmith B, Guyette TW: Evaluation of cleft palate speech. ClinPlast Surg 31:251, 2004.)

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Pressure-flow (aerodynamic) assessment, originallydescribed by Warren and DuBois,363 provides a quan-titative measure of the magnitude of velopharyngealopening and obstruction. These measurements canbe used to determine the need for speech therapy orsurgical intervention and can also be used duringbiofeedback therapy. Kunkel and coworkers364 evalu-ated the acoustic pharyngometer as a new diagnosticmethod to obtain noninvasive quantitative measure-ment of velopharyngeal movement. They consideracoustic pharyngometry as a possibly helpful tool inmonitoring the progress of speech therapy.Rhinomanometry is a useful technique to assess theanatomic and functional characteristics of nasalobstruction and aid in the management ofhyponasality resulting from the cleft.

Shprintzen and Golding-Kushner365 contend thatindirect assessment techniques such as airflow stud-ies do not aid the decision-making process despiteseemingly objective data. Instead, the authors selectthe appropriate treatment for VPD on the basis of acombination of flexible fiberoptic nasopharyngoscopy,multiple-view videofluoroscopy, and behavioralassessment of the patient’s speech.

On the basis of a comprehensive speech evalua-tion using perceptual, anatomic, and physiologicassessement, patients that have evidence of VPD inall modalities are candidates for further surgicalintervention. Patients with borderline VPD may ben-efit from a trial of intensive speech therapy usingbiofeedback methods before proceeding with instru-ment evaluation.

As cleft palate patients mature, evaluation ofvelopharyngeal function is critical to track changesdue to adenoid involution or orthognathic surgery.Adenoidal involution at the time of puberty has beenassociated with the loss of velopharyngeal compe-tence.366 Morris and associates367 examined a groupof cleft palate patients whose lateral cephalometricfilms revealed enlarged adenoidal tissue invelopharyngeal contact. Longitudinal analysis of thesepatients showed that 3 of 39 lost velopharyngealcontact after adenoidal involution and another 4had to have surgery for velopharyngeal incompe-tence. These changes occurred during middle tolate adolescence.

Marsh338 emphasizes that the status of the tonsilsand adenoids needs to be assessed prior to treat-ment decisions because they can interfere withvelopharyngeal closure, velopharyngeal surgery, and

the results of surgical treatment. He recommendsthe removal of hypertrophic tonsils and adenoidsbefore surgery for velopharyngeal dysfunction.Instrument evaluation of velopharyngeal functionshould be performed 3 months after tonsillectomyor adenoidectomy to reassess the possible changes inclosure pattern and to determine the optimal surgi-cal intervention. Senders342 advocates tonsillectomy+ adenoidectomy 10–14 weeks before VPI surgery,so that the surgical bed has time to heal andrevascularize. Removal of adenoids also allows forinsertion of the pharyngoplasty flaps in a more supe-rior position closer to the anatomic velopharyngealsphincter.

Other authors prefer simultaneous pharyngoplastyand tonsillectomy for the surgical correction of VPI.368

Proponents postulate that the dual procedure mayhave better speech outcomes because the childrenadapt to only one change in pharyngeal conditions.Other stated advantages are reducing hospitalizationand anesthesia exposure. Outcome studies of thecombined procedure are equivocal, however, lacklong-term follow-up, and are associated with highrates of complications and surgical revisions. Themain disadvantages of the dual procedure arepotential for excessive scarring of the posterior pha-ryngeal wall, bleeding, and airway obstruction.

Orthognathic maxillary advancement has also beenassociated with the development of velopharyngealdysfunction. Okazaki and coworkers369 examined thespeech of 10 patients with cleft palate who under-went either Le Fort I or Le Fort II osteotomies: 8exhibited increased hypernasality and nasal emis-sions after surgery. Nasopharyngoscopy showed de-terioration in velopharyngeal closure.

Phillips and colleagues370 reviewed the predictivevalue of perceptual speech evaluation and naso-pharyngoscopy for determining the incidence of VPDafter Le Fort I maxillary advancement surgery. Allpatients with preoperative VPD predictably had VPDafterwards. By using both modalities, the authorswere able to accurately predict the risk of VPI in 25of 26 patients. Only one patient had a false-positivereading on nasopharyngoscopy. Among patients withnormal preoperative resonance, 12.5% developedhypernasality after surgery. Although naso-pharyngoscopy provided additional predictive infor-mation, its cost/benefit ratio could not be justified.

Janulewicz et al371 reviewed speech function in 54patients with cleft lip and palate after Le Fort I

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advancement. Competent velopharyngeal functionwas documented in 42% of patients preoperativelyand deteriorated to 18% after orthognathic surgery.Overall speech scores deteriorated significantly aftermaxillary advancement. Only articulation defectsrelated to anterior dentition were marginally improved(P=0.064).

McCarthy,372 on the other hand, found no effectof maxillary advancement on velopharyngeal inad-equacy in 14 patients with cleft palate, but did reportchanges in articulation and sibilant sound produc-tion after surgery.

NONOPERATIVE TREATMENT

A long-term follow-up study from Denmarkreported that 75% of patients with velopharyngealdysfunction achieved acceptable speech with the aidof speech therapy alone.166 Van Demark andHardin373 discuss the effectiveness of articulationtherapy in children with cleft palate and note lessand slower improvement than expected. Articula-tion disorders associated with hypernasality ideallyare corrected after palate closure but before surgeryfor VPI.374

Patients with phoneme-specific velopharyngealdysfunction produce nasal emission on some (s, z,ts, dz) but not all pressure consonants.337 It is impor-tant to identify patients with this specific pattern ofmisarticulation because they do not have a reso-nance disorder and frequently can be helped withspeech therapy alone.342 A few will not respondcompletely and may require surgical intervention toachieve normal speech.

Most speech pathologists advocate therapy thatfocuses on improving velopharyngeal function byaltering the manner in which the velopharyngealmuscles are used during speech.375 The goal is toelicit a different behavioral response. Kuehn376,377

applied the principle of progressive resistance train-ing for muscle strengthening to the muscles of thevelopharynx through continuous positive airway pres-sure (CPAP) to the nasal passages. In a recentmulticenter trial, Kuehn et al377 evaluated the effi-cacy of transnasal CPAP for treatment of hyper-nasality. Although the response was variable acrosspatients and clinical centers, those who received 8weeks of CPAP resistance training showed a signifi-cant overall decrease in speech hypernasality

Ruscello378 reviews nonsurgical palatal training pro-cedures such as articulation therapy, sucking andblowing exercises, electrical and tactile stimulation,speech appliances, and biofeedback techniques. Heagrees with others that deficits in closure are besttreated with surgical or prosthodontic measures.Conservative therapy simply will not overcome largegaps in the velopharyngeal closure mechanism.

Certain patients may not be candidates for surgi-cal treatment of VPI due to unacceptable anesthesiarisk, unstable airway, and neurologic disorders. Thesepatients can be managed with a velopharyngealspeech prosthesis. A velar or palatal lift prosthesis iseffective in patients with a long supple velum andnormal velar length–nasopharyngeal depth ratio whohave myoneural dysfunction (eg, as in neurologicdisorders).338 A velopharyngeal obturator can befitted to fill the velopharyngeal gap in patients withshort, scarred vela and large velar length–nasopharyngeal depth ratios (eg, after cleft palaterepair).338 A combined velar lift–palatal obturatormay be effective when a lift prosthesis is insufficientfor closure.338 Prosthetic management requires anintegrated multidisciplinary team approach andpatient cooperation.

OPERATIVE TREATMENT

Surgical techniques for the correction of VPIinclude pharyngeal flap, pharyngoplasty, posteriorpharyngeal wall augmentation, palate re-repair, andvelar muscle reconstruction (intravelar veloplasty orFurlow palatoplasty). Most authors agree that thesuccess of surgery is improved when the choice ofsurgical technique is tailored to the specific deformityand based on instrument identification of eachpatient’s functional and anatomic characteristics. Anysingle operation is unlikely to correct all cases of VPIbecause of the many factors that can contribute tothe dysfunction. There is less agreement about whichdiagnostic criteria should be used and which proce-dure is recommended for any given abnormality.Outcome comparisons of different protocols and pro-cedures are very difficult due to the myriad methodsof speech assessment, the lack of standardizedobjective criteria, and no clear definition of success.

Peat and colleagues379 choose the corrective tech-nique according to size and shape of the velo-pharyngeal defect as well as specific etiology of thevelopharyngeal disorder. Patients with poor velar

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elevation and good lateral pharyngeal wall motionare best suited for pharyngeal flaps, while those withabsent or minimal lateral wall movement are consid-ered candidates for pharyngoplasty.

Marsh338 presents multiple algorithms for the evalu-ation and management of velopharyngeal dysfunc-tion. Marsh recommends a surgical protocol that isbased on VP gap size and closure pattern (Fig 34).

Fig 34. Velopharyngeal management algorithm 11. (Reprintedwith permission from Marsh JL: The evaluation and managementof velopharyngeal dysfunction. Clin Plast Surg 31:261, 2004.)

Senders342 describes the protocol used at UC Davis,which depends on the shape of the VP defect andthe sphincter motion of the lateral and posterior pha-ryngeal walls (Table 2).

Seagle and others380 from the University of Floridareviewed speech outcomes in patients treated withtheir four procedures of choice: palatal pushback +pharyngeal flap lining; sphincter pharyngoplasty;

superiorly based obturating pharyngeal flap; andFurlow palatoplasty. The choice of procedure wasbased on the evaluation of velar mobility, lateralpharyngeal wall motion, and velopharyngeal gap size.Resolution of VPI was reported for 92% of patientsafter primary correction. Postoperative hyponasalitydeveloped in 7 of 75 patients overall (9%), including2 of 11 who received a pharyngeal flap. The authorsemphasize the importance of thorough preoperativeevaluation and the individualization of treatment.

Ysunza and colleagues381 compared customizedpharyngeal flaps and sphincter pharyngoplasties in aprospective study. The frequency of residual VPIafter individualized velopharyngeal surgery was notsignificantly different between the pharyngeal flapgroup (12%) and the pharyngoplasty group (16%).Regardless of procedure performed, careful preop-erative assessment of gap size and form, lateral wallmotion, and level of maximal sphincter movementwas used to individualize the flap width, location,level of flap insertion (pharyngeal flap or lateralpharyngoplasty flaps), and adjust flap design toaccommodate asymmetric closure patterns. Theauthors conclude that regardless of whether a pha-ryngeal flap or pharyngoplasty is selected, the mostimportant factor in successful surgical treatment ofVPI is to tailor the procedure so that the postopera-tive structure matches the preoperative sphinctermovement.

Albery and others382 studied 100 cleft palatepatients treated by pharyngeal flap, pharyngoplasty,or retropharyngeal implant. Although 97% werecured of unacceptable nasal escape and 93% ofunacceptable nasal resonance, there was still a highproportion of patients with hyponasality.

TABLE 2University of California – Davis Surgical Protocol

(Reprinted with permission from Senders CW: Management of velopharyngeal competence. Fac Plast Surg Clin North Am 9(1):27, 2001.)

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Pharyngeal Flaps

Schoenborn383 first described the inferiorly basedpharyngeal flap procedure in 1876. Rosenthal384

popularized it in Europe and Padgett385 introduced itto America in 1930. Inferiorly based flaps are bestsuited for repair of small gaps because only relativelyshort flaps can be designed without extending intothe region of the fragile and friable adenoids or low-ering the flap base below the level of maximal sphinc-teric closure. Superiorly based pharyngeal flaps arethe most common technique used to correctvelopharyngeal dysfunction, and because of their highsuccess rate are considered by many clinicians to bethe gold standard of treatment.342 San Venero-Roselli386 in 1935 is credited with the design of thesuperiorly based flap. Multiple variations have beenreported since.

The classic flap (Fig 35) is rectangular and verti-cally oriented along the posterior pharyngeal wall.The lateral incision placement is determined by therequired flap width. Flap length should be limited tothat which is required to inset into the soft palatewithout significant tension. Excessively long flaps havebeen associated with a greater risk of postoperativeairway complications. The mucosa and constrictormuscle are incised and the flap is elevated in thealar-facial plane up to the level of the palatal plane.The lateral ports can be secured with tube place-ment during flap inset into the soft palate. Lining ofthe raw surface of the flap using mucosa from thedorsum of the soft palate will reduce postoperative

contraction. The donor site can be closed primarilyor left open to re-mucosalize. Kapetansky387 prefersa transverse design in the belief that it helps reducenasality and postoperative nasal obstruction.Hogan388 advocates a lateral-port control pharyn-geal flap to try to recreate a velopharyngeal port thatis <20mm2.

The key to a successful pharyngeal flap is thoughtto be lateral pharyngeal wall motion.389 Zwitman390

noted that lateral wall motion may be reduced oreliminated by the surgical process. Shprintzen andcolleagues,391,392 however, showed that pharyngealflap surgery does not alter the movement of thelateral pharyngeal walls. The authors392 recommendtailoring the size of the flap to the dimensions of thegap in the velopharyngeal sphincter. Johns and oth-ers337 believe that flap width should be determinedon the basis of preoperative videofluoroscopy duringlateral wall motion. A relative scale determined bythe degree of lateral wall motion is proposed whichranges from 0 – no motion during quiet respiration –to 5 – maximal motion to the midline (Fig 36). Theproposed flap width is the difference between mid-line closure, or 5, and the patient’s lateral wallexcursion.

Wide pharyngeal flaps are associated with a greaterrisk of airway obstruction and hyponasality. Pharyn-geal flaps that are not lined with mucosa (usuallyfrom the dorsum of the soft palate) undergo signifi-cant contraction and tubing which results in unpre-dictable postoperative width. Flap contracure in the

Fig 35. Technique of nonobstructing pharyngeal flap. A, incision; B, flap insertion to the dorsum of the soft palate; C, flap is hidden behindsoft palate; D, healed flap represented as a narrow tube; note large lateral ports. (Modified from Argamaso RV: The pharyngeal flap. InKernahan DA, Rosenstein SW, eds: Cleft Lip and Palate. A System of Management. Baltimore, Williams & Wilkins, 1993. Ch 32,pp 263-269.)

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horizontal or vertical plane may result in residual VPIbecause of inadequate compensation by the lateralpharyngeal walls or migration below the level of maxi-mal sphincteric movement. Johns and colleagues393

and Fischer-Brandies and Nejedlo394 describeimportant technical considerations in pharyngeal flapconstruction, including measures to adequately lineraw surfaces to prevent tubing and shrinkage of theflap, and placing the base of the flap at a high levelwhere lateral pharyngeal wall motion may ensureclosure of the gap.

Flap contracture may still be significant despite thepresence of a lining. Vandervoort et al395 comparedpharyngeal flap width at the time of elevation and6mo after surgery in a series of 54 patients who hadsuperiorly-based pharyngeal flap procedures. Ini-tially flap width was 89% of the width of the poste-rior pharyngeal wall, but over the next 6 months itshrank to 45%. No relationship was found between

Fig 36. Diagrammatic frontal view of the oropharynx demon-strates gradations of mesial motion of the lateral pharyngeal walls.Zero represents no motion during quiet respiration, and 5 depictsmaximal motion of each lateral pharyngeal wall to the midline.(Reprinted with permission from Johns DF, Rohrich RJ, Awada M:Velopharyngeal incompetence: a guide for clinical evaluation.Plast Reconstr Surg 112:1890, 2003.)

residual flap width and speech results. The authorsconclude that preoperative tailoring of the pharyn-geal flap to the defect is “hit or miss.” The speechresults suggest that the degree of shrinkage is notcrucial as long as lateral wall movement is adequateto close against the flap.

Successful treatment of VPI with pharyngeal flapsis reported to range from 50% to 95%. The primarydisadvantages of pharyngeal flaps are the risk of post-operative airway compromise, obstructive sleepapnea, snoring, and hyponasality resulting from nasalairway obstruction. . Morris et al396 reported normalvelopharyngeal function in 83.1% of patients withVPI treated by pharyngeal flap. Although theyreported that pharyngeal flap surgery was highlyeffective in achieving normal velopharyngeal func-tion, they also reported postoperative snoring in89.2% of patients.

Seyfer, Prohazka, and Leahy397 found that thesuperiorly based pharyngeal flap was effective inreducing hypernasality in all age groups regardless ofpatient’s age at operation and the time interval ofVPI. Brondsted et al166 analyzed the speech resultsof 140 of 600 palatopharyngoplasty (PPP) opera-tions in Denmark over two decades, and found nor-mal nasal resonance in 74% of cases and improve-ment to mild symptoms in 24%. There was no dif-ference in velopharyngeal function between patientswho had superiorly based pharyngeal flaps and thosewho had inferiorly based flaps. Smith and others398

used pressure-flow aerodynamic studies to evaluate31 patients after pharyngeal flap surgery. By thesemeasures, 52% were judged successful while 35%still had significant nasopharyngeal obstruction orovercorrection for VPI.

Jarvis and Trier399 compared 91 patients whounderwent superiorly based pharyngeal flap andintravelar veloplasty with 39 patients who under-went superiorly based pharyngeal flap only. Morethan 92% from each group obtained velopharyngealadequacy. There was no statistical improvement inspeech when intravelar veloplasty was added.

Pushback palate re-repair with pharyngeal flap fornasal lining has been advocated by some surgeons asa successful technique for correction of VPI. Thepharyngeal flap is not lined and therefore undergoestubing which is considered beneficial because of thereduction in airway morbidity. Dixon-Wood et al400

reported velopharyngeal competence in all 23 patientsafter pushback with pharyngeal flap for nasal lining.

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Seagle et al380 recommend palatal pushback withpharyngeal flap lining for patients with good velar andlateral wall mobility and a velopharyngeal gap <10mm.In a series of 45 patients, the authors reported a successrate of 91% and hyponasality in 7%.

Pharyngoplasty

In 1950 Hynes401 described transposition of bilat-eral flaps from the lateral pharyngeal walls to join inthe palatal midline anterior to Passavant’s ridge (Fig37). The procedure was designed to tighten thecentral orifice and occlude the lateral ports in thetreatment of VPI. Each flap is 3–4cm long and con-sists of salpingopharyngeus muscle and its overlying

mucosa. The transposed flaps form the posteriorwall of the future palatopharyngeal sphincter. Theoperation is said to be associated with minimal bleed-ing and largely succeeds in producing a functionalsphincter. In Hynes’s series, 67% of flaps were notedto be contractile on postoperative examination and95% of patients achieved velopharyngeal compe-tence.

Orticochea402 described the dynamic musclesphincter modification of the pharyngoplasty proce-dure in 1968. This modification transplants the pos-terior tonsillar pillars with their enclosed palatopha-ryngeus muscles to below the palatal plane. Theflaps are sutured to each other and to a small, inferi-orly based musculomucosal flap elevated from the

Fig 37. Hynes’s pharyngoplasty technique in the management of velopharyngeal insufficiency. (Reprinted with permission fromHynes W: Pharyngoplasty by muscle transplantation. Br J Plast Surg 3:128, 1950.)

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posterior pharyngeal wall, creating a competent mus-cular sphincter. Clinical experience with the dynamicsphincter pharyngoplasty procedure has largely con-firmed its usefulness in correcting hypernasality andnasal escape associated with VPI.403–406

Riski and coworkers407 propose a higher insertionsite for the Orticochea pharyngoplasty based on datafrom 29 patients, all but 2 of whom had resolutionof hypernasal resonance when the lateral flaps weretransposed to the area in the nasopharynx ofattempted velopharyngeal contact. In contrast, only16 of 26 patients whose pharyngoplasties werelocated below the site of attempted velopharyngealclosure had correction of their VPI.

Jackson408,409 described the pharyngoplasty opera-tion that is commonly used today.410,411 In this pro-cedure the sphincter is constructed from the poste-rior tonsillar pillars, which are elevated to include thepalatopharyngeus muscles and combined with a smallsuperiorly based posterior flap to tighten thevelopharyngeal port. Jackson and Silverton412 foundthat the sphincter is frequently too low, particularlywhen the inferior flap is used, and contributes littleto the integrated closure of the velopharyngeal area.They have since modified their pharyngoplasty tech-nique413 to place the sphincter high on the posteriorpharyngeal wall, using a high transverse incision androtating the posterior faucial pillars into the resultingdefect. This leads to better velopharyngeal closurethrough concerted action of the sphincter, lateralpharyngeal walls, and soft palate. Ren and Wang’s414

modification of the Orticochea–Jackson operationinvolves lateral-to-lateral connection of the palatopha-ryngeus flaps and superior shifting of the posteriorattachment.

Moss and associates415 described their own ver-sions of the sphincter pharyngoplasty procedurewhich they recommend for patients with slight ormoderate nasal escape, a mobile velum, and a gapof ≤5mm. Seagle380 recommends sphincterpharyngoplasty for patients with good velar mobility,poor or fair lateral wall movement, and a gap of<10mm. They report a success rate of 92% in aseries of 24 patients, but 12% had postoperativehyponasality.

Losken et al416 retrospectively reviewed 250patients with VPI who were treated with sphincterpharyngoplasty. The primary success rate was 87% and surgical revision was required in 12.8%.The revision rate was highest for patients with

velocardiofacial syndrome (21.8%) who also hadmore severe preoperative hypernasal resonanceand larger velopharyngeal area. The lowest revi-sion rate (6.3%) was for patients with isolated VPI(not associated with cleft palate).

Graivier and associates417 report a case of post-tonsillectomy velopharyngeal insufficiency in whomthe palatopharyngeus muscles and mucosa of theposterior tonsillar pillars were resected at the time oftonsillectomy. The patient’s sphincter was recon-structed using the anterior tonsillar pillars and under-lying palatoglossus muscles.

Posterior Pharyngeal Wall Augmentation

A number of velopharyngeal augmentationtechniques have been suggested for the treatmentof VPI. These procedures bring autogenous,allogeneic, or synthetic materials into theretropharynx, displacing it anteriorly in an effortto achieve closure with the velum. Among thematerials that have been proposed for this pur-pose are injectable collagen,418 teflon,419,420

Silastic,421,422 Proplast,423 fat,419 and petroleumjelly.424 Trigos and colleagues425 suggested homolo-gous cartilage as a retropharyngeal implant. Theplace of insertion was determined by videonaso-pharyngoscopy.

Denny and colleagues426 used autologous carti-lage as a pharyngeal implant in 20 patients withVPI. The cartilage was harvested from the regionof synchondrosis of ribs 6 and 7. Hypernasalityand audible nasal emissions were completely elimi-nated in 5 patients (25%) while another 11 patients(55%) were improved but not cured. The authorsconclude that autologous cartilage implants aresuperior to pharyngeal flap techniques becausethe implants do not alter the nasal airway and aretherefore safer in children at risk of respiratoryproblems.

Senders342 recommends use of a rolled pharyn-geal flap for posterior wall augmentation in patientswith a coronal closure pattern, poor lateral wallmotion, good velar mobility, and minimal velo-pharyngeal gap. The flap must be placed high at thelevel of velopharyngeal sphincter closure and preop-erative instrument studies are critical for operativeplanning. The adenoids may limit the superior levelof flap placement and may need to be removed toallow for a more superior base.

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Palate Re-repair

Palate re-repair with levator muscle reconstruc-tion and lengthening has been a successful treatmentof VPI in specific subsets of patients.427–431 Theadvantage of this approach is that it is more physi-ologic. By repairing the anatomic defect withoutcreation of abnormal pharyngeal anatomy, palatere-repair does not rely on obstruction of thevelopharyngeal port and therefore avoids the poten-tial airway morbidity associated with pharyngeal flaps,pharyngoplasty, and augmentation.

Chen et al427 reported velopharyngeal competencein 16 of 18 patients (89%) with VPI after Furlowpalatoplasty. In this series the success rate was 100%if the preoperative velopharyngeal gap size, as mea-sured on videofluoroscopy, was ≤5mm. Only 1 in 3patients with gaps >10mm achieved velopharyngealcompetence after Furlow repair.

D’Antonio et al428 reported achieving completevelopharyngeal closure in 6 of 8 patients with previ-ous palate repairs (and in 1 of 4 patients with sub-mucous cleft palate) who were treated with a Furlowrepair for VPI. Criteria for patient selection werethe presence of a central V-shaped trough of thevelum on endoscopy, good velar movement, and asmall velopharyngeal gap. A significant increase inpostoperative velar length and thickness wasreported. The patients who achieved completevelopharyngeal closure had greater velar length andwidth than the patients who demonstrated incom-plete closure after surgery. The authors suggest thatpharyngeal depth/velar length ratio may be an indi-cator of patients for whom the Furlow repair wouldprovide appropriate treatment of VPI. Despite sig-nificant lengthening and increased thickness of thevelum after Furlow repair, if the depth/length ratiois highly unfavorable, it is unlikely that the patientwill achieve closure.

Sie et al429 retrospectively reviewed 48 patients withprevious palate repairs or submucous cleft palate whowere treated for VPI using the Furlow repair. Patientselection for Furlow repair was based on clinical evi-dence of sagittally oriented levator veli palatini muscu-lature. The results were not segregated based onwhether the patients had a previous palate repair or asubmucous cleft palate. Complete resolution of VPIwas reported in 40% of patients. Minimal residualVPI was reported in 16.7% of patients. In his com-mentary to this study, Furlow432 states that a success

rate of 40% does not warrant the use of this operationin all patients with sagittally oriented muscles. Furtherstudy is needed to evaluate the speech outcomes afterFurlow repair in all patients with VPI so that accuratepreoperative criteria can be identified.

Sommerlad and colleagues430 analyzed the speechoutcomes of 85 patients with VPI who were treatedby palate re-repair with radical dissection and repo-sitioning of the velar muscles using an operatingmicroscope. Using a blinded speech assessment andinstrument evaluation, significant improvement wasreported in hypernasality, nasal emission, nasal tur-bulence, and intelligibility. Normal nasality and nasalairflow or mild but inconsistent hypernasality, nasalemission, or turbulence was reported in 82.4% ofpatients on postoperative speech evaluation. Post-operative videofluoroscopy demonstrated significantimprovement in velar function with decrease invelopharyngeal gap during closure but not at rest,thereby minimizing the risk of postoperative airwayobstruction. Patients with better speech and velarfunction preoperatively were more likely to achievenormal closure, but patients with more severe pre-operative dysfunction demonstrated a greater degreeof improvement. Other preoperative factors thatwere correlated with normal postoperative nasalityand intelligibility were a gap <9mm and a closureratio of >0.45. Pharyngoplasty was required in11.8% of patients for persistent VPI.

Nakamura et al431 retrospectively reviewed thevelopharyngeal function of 15 patients with VPI whowere treated by re-pushback palatoplasty, intravelarveloplasty, and buccal mucosal grafts to the nasalsurface. Complete velopharyngeal closure wasreported in 8 of 15 patients. Velopharyngeal closureimproved sufficiently in 86.7% of patients so that nofurther surgical treatment was required. Velar lengthand length/depth ratio was significantly different fromthe non-cleft control group before surgery and statis-tically similar after re-pushback repair. The preop-erative length/depth ratio was significantly greater inpatients who achieved complete closure after sur-gery than in patients who had incomplete closureafter surgery.

COMPLICATIONS OF VPI SURGERY

One of the most common complications of pha-ryngeal flap surgery for VPI is obstructive sleep apnea(OSA), which has been reported postoperatively in

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as many as 9 of every 10 patients.433–436 Sirois andcolleagues437 found that while 35% of patients hadabnormal postoperative polysomnograms, most ofthe abnormal tests returned to normal in the monthsfollowing surgery. Morris et al396 report that 58 of 65patients (89%) with cleft palate snored often or some-times, and one had evidence of right ventricularhypertrophy on electrocardiogram. Shprintzen435 wasunable to correlate flap width with the degree ofpostoperative OSA, but notes contributing factorssuch as decreased airway size, enlarged tonsils, alter-ation of the respiratory pattern, and syndromic ele-ments after pharyngeal flap surgery.

Liao and others438 recently investigated the inci-dence and severity of OSA after pharyngeal flap sur-gery to determine the effect of patient age and flapwidth. Overnight polysomnographic studies wereperformed on 10 adults and 28 children 6 monthsafter pharyngeal flap. The overall incidence of OSAwas greater than 90%, but no significant differencewas noted between the adults (90%) and the chil-dren (93%). There was a significant difference in theseverity of OSA between age groups, however. Inthe adult group, mild OSA was diagnosed in 89%and 11% had moderate to severe OSA. In the pedi-atric group, mild OSA was found in 42% and 58%had moderate to severe OSA. No correlation wasfound between OSA and flap width.

Liao and colleagues439 also compared the relativeincidence and severity of obstructive sleep apneasyndrome (OSAS) in patients treated with eitherFurlow repair or pharyngeal flap for VPI. The pha-ryngeal flap patients had a significantly lower meanpercentage of stage 2 sleep, larger respiratory distur-bance index (RDI), and larger oxyhemoglobindesaturation index (DI). Overall, the pharyngeal flappatients had more frequent and more severe OSAScompared with patients treated with Furlow palato-plasty. On the basis of these results, the authorsconcluded that a Furlow palatoplasty should be per-formed instead of a pharyngeal flap for treatment ofVPI.

Ysunza and associates440 found enlarged tonsils in13 of 15 cases of postoperative obstructive sleepapnea. Shprintzen435 recommends primary tonsil-lectomy as a separate procedure if it appears that theenlarged tonsils will be a problem. Others441 con-clude that tonsillectomy can be performed safely atthe time of pharyngeal flap construction.

In a 7-year retrospective review of 219 childrenundergoing surgery for VPI, Valnicek and colleagues442

noted an overall 16.4% incidence of complications,including one death. Postoperative bleedingoccurred in 8%; airway obstruction, usually withinthe first 24 hours postoperatively, developed in 9%;and 1% required reintubation. The incidence ofobstructive sleep apnea was 4%. Four of these 9children required take-down of the pharyngeal flap,while 7 had revisions of the flap.

Significant hypernasality following pharyngeal flapprocedures often prompts revision of the pharyngealflap. Cosman and Falke443 used inferiorly basedpharyngeal flaps to augment cases in which thesuperiorly based flap had narrowed.

Barone and associates444 report persistentvelopharyngeal insufficiency after superiorly basedpharyngeal flap surgery in 21 patients. Treatmentwas selected according to whether or not one orboth of the lateral nasal ports was too large. In casesin which the initial flap was too narrow, dehisced, orotherwise had to be taken down, a new superiorlybased pharyngeal flap was elevated using the “sand-wich” technique. The initial flap was left in situ andthe donor site on the pharyngeal wall was allowed togranulate. In cases of unilateral port insufficiency, apatch flap was elevated and inset. After treatmentwith this technique, 18 patients had normal reso-nance, one was hyponasal, and 2 had mild nasalturbulence. There was no flap loss or dehiscence inthe series.

Another potential complication of pharyngeal flapsurgery is inhibition of facial growth, possibly from atethering effect of the flap on the velum that mayrestrict maxillary advancement. Isberg and col-leagues445 prospectively evaluated 20 consecutivechildren with cleft lip and palate who underwentcorrection of VPI with pharyngeal flap. The facialgrowth of these patients was followed for 5 yearspostoperatively. The only change that could bedetected was reorientation of mandibular growthwith posterior and inferior rotation, which did notresult in any significant change in facial form. Max-illary growth inhibition was not significantly differ-ent from that seen in a control group of 20 cleftpatients who did not have pharyngeal flap surgery.Mandibular rotation begins to reverse at age 10,and after puberty the mandible resembles the man-dible of unoperated patients.446

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REPAIR OF SECONDARY CLEFT LIPDEFORMITIES

Despite improvements in primary cleft care fromadvances in surgical technique and a comprehensiveteam approach to management, secondary deformi-ties of cleft lip are still common and are frequentlymore challenging to repair than the primary cleftitself.

Secondary cleft lip and nasal deformities are com-plicated by scar, distorted anatomy, and high patientexpectations. Successful correction of a secondarycleft lip deformity depends on a systematic evalua-tion of the anatomic derangement and a carefullyconceived treatment plan that minimizes the num-ber of interventions necessary. The total number ofsurgeries necessary to achieve optimal treatment ofpatients with cleft lip and palate remains unclear.203

The entire clinical team should be involved in thedecision regarding the timing and extent of the pro-cedures to be performed.

A standard approach to the repair of a secondarycleft lip deformity should take into account the fol-lowing variables:

• Anatomic elements—which components are nor-mal (to be preserved) and which are abnormal(to be corrected), and whether the abnormal ele-ments should be rearranged or discarded. Allcomponents should be evaluated at rest and dur-ing animation.

• Residual deformities—whether they are uncor-rected components of the original deformity,recurrences following surgical repair, or iatrogenicevents. The individual deformities of the lip, nose,and hard tissues must be considered an integralunit even if each component is approached sepa-rately in the operating room.

• Realistic surgical goals—which procedure(s) willmost predictably achieve the goals in the feweststages and with the least disruption of normalstructures.

• Timing of surgery—at what age does the benefitfrom the procedure become important to thepatient, and what is the most opportune time,from a technical standpoint, to achieve these goalsand interfere least with normal growth.

Residual lip deformities vary in severity. Minordeformities may be amenable to correction with a

limited procedure, while major deformities frequentlyrequire complete revision of the primary repair. Mostsurgeons agree that significant deformities should berevised before the patient enters first grade to avoidany psychological trauma from peers. As patientsmature, it is best to consider the patient’s own per-ception of his/her deformity when recommendingthe timing of elective revisions.

RESIDUAL LIP DEFORMITIES – UNILATERAL CLEFTS

Residual deformities after primary cleft lip repaircan be the result unsatisfactory scars, malalignedtissue, tissue deficiency, tissue excess, or a combi-nation.447 The severity of the deformity dictatesthe extent of the revision required. Commondeformities include scars, discrepancies in tissuequantity, misplaced landmarks, vermilion defor-mities, buccal sulcus deformities, and orbicularisoris derangement.

Scars

Most authors agree that the first scar is often thebest, and emphasize conservatism in revising a well-healed cleft lip scar. Millard448 states that “from theage of about 8 to 18 years, surgery is followed byexaggerated reaction, with longer periods of scarerythema and hypertrophy,” and urges restraint inlip scar revision during these years. Hypertrophicscars may result from poor tissue handling or for noapparent reason in certain patients. Scar hypertro-phy usually fades slowly during the first postopera-tive year without the need for additional surgicalintervention.203 Adjunct therapies for scar manage-ment —such as silicone sheeting or cream, vitaminE, and massage—may help in resolving hypertrophicscars.

Revisional techniques include excision and clo-sure by either a straight-line, “wavy-line”, Z-plasty,W-plasty, diamond-shaped excision or stair-steptechnique. Z- and W-plasties reorient the scarcomponents across the central upper lip landmarksand can be manipulated to lengthen a tight upperlip,449 relax a notched vermilion449 (Fig 38), or lift adrooping lateral lip.450 The width of the resectionis adjusted and the limbs of the incision are modi-fied to give maximum correction where it is neededthe most.

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When the scar lies over the philtral column, onlythe scar epithelium is resected, keeping the bulk ofthe dermis and mucosa to stack beneath the incisionand add projection to the closure449 (Fig 39).

Fig 39. Scar revision with “vest-over-pants” dermal closure. Theepithelial component of the scar is excised. The epidermis onone side is separated from the dermis, and the dermal flap isburied under the contralateral dermis. (Reprinted with permis-sion from Stal S, Spira M: Secondary reconstructive proceduresfor patients with clefts. In: Serafin D, Georgiade NG (eds),Pediatric Plastic Surgery. St Louis, CV Mosby, 1984. Vol 1, Ch23, p 352.)

An advantage of the rotation-advancement tech-nique is that it places the scar within the naturalcontours of the philtral column. Any scar revisionthat places new scar outside this line, such as Z-plasties do, may result in a less-normal appearance.

While superficial scar revision techniques may besufficient for the correction of minor irregularities, themajority of secondary deformities after cleft lip repair,at least those performed by rotation-advancement,are due to inadequate rotation and misalignment ofthe muscular elements during the initial closure. Tocorrect the residual deformity it may be necessary toreopen the lip along the original scar lines and to re-rotate or advance the muscular elements.

The cleft lip scars can be camouflaged with der-mal micrografts as described by Chen and col-leagues.451 In men the dermal grafts may be obtainedfrom hair-bearing areas.

Discrepancies in Quantities of Tissues

Disproportionate amounts of lip tissue after cleftrepair may be the result of vertical maldistribution (along or short lip), horizontal deficiency (a flat or tightupper lip), or nostril sill/alar base deficiencies.

Vertical Excess – Long Lip

Excessively long lips after cleft surgery are com-monly associated with Tennison, LeMesurier, andtriangular flap repairs. Le Mesurier’s repair has falleninto disfavor because of this problem.450 The long lipdeformity is less common with the rotation-advancement repair but can result when the medialsegment is over-rotated.

Mild vertical excess can be corrected by full-thickness excision of tissue from the upper portion ofthe lip below the nostril sill . Distortion of landmarksis kept to a minimum by horizontal perialar exci-sions452 instead of resections lower in the lip, wherethey are more obvious.

Moderate or severe long lip deformities are moreeffectively corrected by a complete revision of theprimary repair. Stal and Hollier453 contend thatsuperficial excisions under the alar base are frequentlyinadequate to achieve long lasting correction of thelong lip deformity. The authors advocate revision ofthe entire repair with appropriate reduction in boththe vertical and transverse dimensions. Permanent

Fig 38. Excising the scar epithelium and increasing the length withZ-plasty. (Reprinted with permission from Stal S, Spira M:Secondary reconstructive procedures for patients with clefts. In:Serafin D, Georgiade NG (eds), Pediatric Plastic Surgery. StLouis, CV Mosby, 1984. Vol 1, Ch 23, p 352.)

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suspension sutures to the periosteum are an effectivemeans for maintaining lip elevation.453

Vertical Deficiency – Short Lip

A lip is considered short when the philtral columnon the cleft side is at least 3mm shorter than thenoncleft philtrum.453 In the first year after rotation-advancement lip repair, the lip segment often movesupward from scar fibrosis and contraction and thelip seems short. This apparent height deficiency willresolve spontaneously as the wound matures pro-vided the muscular elements were approximatedcorrectly during the primary repair. Treatment shouldbe expectant.454

Short lips are more frequent after straight-linerepair, the Rose-Thompson repair,449 and rotation–advancement maneuvers when used for the correc-tion of extremely wide clefts. If straight-line repairwas previously performed and the deficiency is severe,then the cleft should be reopened and rotation-advancement flap(s) used.

Stal and Hollier453 report that a diamond-shapedscar excision and closure can lengthen a lip by 2mmand a Z-plasty can lengthen the lip by up to 3mm.Contracture of mucosal flaps can also result in short-ening of the lip which can be corrected by mucosalrelease. Other lengthening techniques include trian-gular flap transpositions along previous vertical scarsand V-Y advancements emphasizing downwardrotation of the medial segment448,449 (Fig 40).

Severe short lip deformities after a rotation–advancement repair are usually due to improperdesign and inadequate rotation of the medial seg-ment. Failure to anatomically reconstruct theorbicularis muscle can also result in shortening of thelip and can be recognized by lateral muscle bulgingwhen the lips are pursed.453 A repeat rotation-advancement with anatomic muscle repair is neces-sary to correct these severe deformities.

Horizontal Deficiency – Tight Lip

Horizontal tissue shortage—a tight lip—is uncom-mon after correction of unilateral deformities unlessthere has been overresection of the cleft marginsduring the primary surgery. Millard448 usually finds atight lip associated with early straight-line or triangu-lar flap repairs.455 If the flat upper lip is visibly dispro-portionate to the protruberant lower lip and deservescorrection, an Abbe flap may be considered.

McGregor456 details pedicle design and placementof Abbe flaps and suggests applications of the tech-nique in the correction of unilateral and bilateralpostcleft deficiencies. The Abbe flap should be posi-tioned in an upper lip midline incision and the previ-ous scars from the lip repair should be revised at alater date (when used for unilateral clefts).457

Missing or Misplaced Landmarks

The most common residual deformities of cleft liprepair are loss of philtral definition and obliterationof the Cupid’s bow. Failure to preserve the philtralcolumn and central dimple on the cleft side at thetime of primary repair gives the lip a blurred, oper-ated look.

Philtral Column Effacement

As early as 1931, Veau458 emphasized preserva-tion of the philtrum during closure of a cleft lip. Theunilateral cheiloplasty techniques of Tennison,459

Randall,460 and particularly Millard’s rotation–advancement461 preserve philtral components in themedial segment of the cleft and best approximatethe normal configuration.

Surgical attempts at reconstituting the philtral col-umns are limited in their ability to simulate the deli-cate, soft structures of the normal philtrum. Varioustechniques have been suggested, including subcuta-

Fig 40. Lengthening the short upper lip. (Reprinted with permis-sion from Stal S, Spira M: Secondary reconstructive procedures forpatients with clefts. In: Serafin D, Georgiade NG (eds), PediatricPlastic Surgery. St Louis, CV Mosby, 1984. Vol 1, Ch 23, p 352.)

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neous rotation flap (O’Connor and McGregor462), arollover flap of muscle tissue out of the central dimple(Onizuka463), a chondrocutaneous composite flap(Schmid464), a subcutaneous auricular cartilage graft(Neuner465), or a muscle-splitting technique and vest-over-pants muscle closure.466

Cupid’s Bow–White Roll Deformities

Secondary Cupid’s bow deformities involve absentvermilion peaks and/or asymmetries of the white roll.Malalignment of the white roll by as little as 1 mm isnoticeable. Many techniques for the correction ofsecondary central vermilion/white roll deformitieshave been published, including diamond-shaped orelliptical excision,203,453 quadrilateral flap,467 mobili-zation of a secondary white roll flap, small localizedZ-plasty, and white roll free graft448 (Fig 41). Millard448

preserves the white roll-mucocutaneous junction byexcising above the junction during rotation–advancement repair of the cleft lip.

Fig 41. Procedures to correct vermilion malalignment. A, “Whiteroll” flap. B, Tiny Z-plasty. C, “White roll” free graft. (Reprintedwith permission from Millard DR Jr: Cleft Craft—The Evolu-tion of Its Surgery. I. The Unilateral Deformity. Boston, LittleBrown, 1976.)

Severe deformities are likely due to inadequaterotation of the medial lip segment and require repeatrotation-advancement. When Cupid’s bow peakshave been destroyed in the primary repair, as oftenseen after Mirault-Blair-Brown-McDowell triangularflap corrections,448 secondary reconstruction is ham-pered by the presence of scar either at or above thevermilion–skin line.

Takato and others468 described a modification of theAbbe flap for reconstruction of the vermilion tubercle

and Cupid’s bow in cleft lip patients. This flap, designedon the central portion of the lower lip vermilion, con-tains full-length lower lip vermilion and orbicularis orismuscle, but only a tiny portion of skin (Fig 42).

Fig 42. Modified Abbe flap containing vermilion and orbicularisoris muscle transferred to the upper lip for secondary repair ofcentral vermilion deficits. (Reprinted with permission from TakatoT, Yonehara Y, Susami T, Yoshima K: Modification of the Abbe flapfor reconstruction of the vermilion tubercle and Cupid’s bow incleft lip patients. J Oral Maxillofac Surg 54:256, 1996.)

Vermilion Deformities

Vermillion deformities can result from eithermalalignment, tissue excess or most commonly fromtissue deficiency. Lack of vermilion bulk may producenotching at the free vermilion border (whistling defor-mity), deficiency of the central vermilion tubercle, ordeficiency of the lateral vermilion. Vermillion defi-ciency can be corrected by rearranging existing ver-milion tissue with Z-plasties, V-Y advancement flaps,mucosal transposition flaps, mucosal grafts, lower lipflaps, and tissue augmentation procedures (Fig 43).

Whistling Deformity

A whistling deformity is characterized by the pres-ence of a notch in the vermilion at the lip repair site.In addition to the previously mentioned V-Y and Z-plasty flaps, whistling deformity can also be correctedwith free composite grafts.469 These techniquesdepend on sufficient amounts of vermilion remain-ing to furnish tissue for the correction.

Cohen and Kawamoto470 described a free tonguegraft for correction of vermilion deficiencies. Thegraft is harvested from the lateral aspect of the tonguebelow the papillary line. Indications for use are eithera V-shaped vermilion defect or a whistling deformityassociated with insufficient lateral vermilion bulk.Severe whistle deformities may require reconstruc-tion with an Abbe flap.

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Central Tubercle Deficiencies

Mid-lip tissue for transfer to the tubercle is usuallyavailable only in the less severe clefts. V-Y and Z-plasties are often used in the correction of centraltubercle deficiencies.

Fig 43a. Vermilion deficiency corrected by Z-plasty. (Reprintedwith permission from Millard DR Jr: Cleft Craft—The Evolutionof Its Surgery. I. The Unilateral Deformity. Boston, LittleBrown, 1976.)

Fig 43b. Vermilion deficiency corrected by V-Y plasty. (Re-printed with permission from Millard DR Jr: Cleft Craft—TheEvolution of Its Surgery. I. The Unilateral Deformity. Boston,Little Brown, 1976.)

Fig 43c. Vermilion deficiency corrected by mucosal transpositionflaps in two designs. (Reprinted with permission from Millard DRJr: Cleft Craft—The Evolution of Its Surgery. I. The UnilateralDeformity. Boston, Little Brown, 1976.)

Hirano and coworkers471 reconstruct the centraltubercle using a “tulip flap” technique that is basedon local vermilion transposition flaps.

Juri and colleagues472 accentuate the centraltubercle by rotating inferiorly the medial edges ofvermilion-mucosal flaps. Denuded lateral vermilionhas also been used to correct central tubercle defor-mities (Fig 44).

Fig. 44. Vermilion deficiency corrected by combination Z-plastyand denuded lateral vermilion flap tunneled medially. (Reprintedwith permission from Millard DR Jr: Cleft Craft—The Evolutionof Its Surgery. I. The Unilateral Deformity. Boston, LittleBrown, 1976.)

Fat grafts, dermal fat grafts, and fascia have beenused successfully for vermilion augmentation. Chenand colleagues473 described a temporoparietal fas-cial graft for augmentation of the free border of thelip. These grafts have been used in 20 patients in thecorrection of secondary cleft lip deformities. Theprocedure is useful in augmenting both central andlateral lip deficiencies. The added tissue is soft andresorbs minimally over time.

Lateral Vermilion Deficiencies

Major deficiencies of lateral vermilion can be cor-rected with a vermilion flap from the lower lip assuggested by Kawamoto.474 His modification of Gilliesand Millard’s475 technique offers improved contourand color match by using a centrally based cross-lipflap of exposed vermilion surface.

Wagner and Newman476 describe a bipedicledlower to upper cross-lip visor flap to augment theupper lip vermilion in the event of major tissue defi-cits. The flap is based on the coronary arteries nearthe commissures and transfers wet vermilion andmucosa from the lower lip to the site of a releasingincision at the wet/dry vermilion border of the upperlip. The procedure is done in two stages, flap eleva-

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tion and transfer followed by flap division and inset10 days later.

Muraoka et al477 described a similar 2-stage proce-dure for vermilion augmentation of the upper lip usinga unipedicled mucosal flap based on the inferior labialartery. The mucosal flap is elevated from the entirelength of the lower lip and can include muscle if addi-tional bulk is required. The advantage of theunipedicled design is less restriction of oral movementduring the postoperative period. (Fig 45).

Fig 45. Technique of mucosal flap transfer from lower lip tocorrect vermilion deficiencies secondary to cleft lip. (Reprintedwith permission from Muraoka M, Harada T, Wakami S, et al:Mucous-flap method for cleft-lip revision using transverse evertedfull-length lower-lip flap. Ann Plast Surg 54:538, 2005.)

Excessive Lateral Vermilion

Rotation-advancement repair of unilateral cleft lipsoccasionally produces excessive bulk of vermilionfrom advancement of lip mucosa in relation to max-illary mucosa, creating an apparent excess of vermil-ion. If this tissue is not redistributed properly duringclosure, the net result is essentially a V-to-Y augmen-tation of the upper lip. Correction is by transverseexcision of the wide vermilion.

Buccal Sulcus Abnormalities

Buccal sulcus adhesions or adherent scar may becorrected by Z-plasty or V-Y techniques. Deepeningof the upper labial sulcus may require split-thickness

skin grafts,478 oral mucosal free grafts,479 thin premax-illary mucosal flaps,480 or local mucosal advancementflaps. A prosthetic splint may be useful postopera-tively for flap or graft inset into the sulcus.

Orbicularis Oris Derangement

Reconstruction of the orbicularis oris muscle is acritical component of primary lip repair. Failure toanatomically reconstruct the orbicularis or dehiscenceof the muscle repair results in lateral bulging, centraldepression, and asymmetries that are accentuatedwith animation. Minimal deformities may be cor-rected by excising the scar, releasing abnormal mus-cular attachments, and suturing of the muscletogether.203 Severe deformities require repeat rota-tion-advancement.

Stal and Spira449 contend that “all short, previ-ously repaired lips should be completely incised, withexcision of all scar.” The orbicularis muscle fibersare located, mobilized, and repositioned transversely.The medial muscle is sutured in the midline of thelip, the oblique fibers are anchored to the perios-teum of the nasal spine, and the lower fibers aredirected into the pouting tubercle of the lip.

RESIDUAL LIP DEFORMITIES-BILATERAL CLEFTS

Many of the considerations and surgical measuresreviewed above for secondary correction of the uni-lateral cleft lip deformity also apply to bilateral clefts.

Tissue Deficiency or Excess

Horizontal Shortage — Tight Lip

Horizontal lip deficits after cleft repair are usu-ally associated with severe clefts and are the resultof either innate shortage of lip tissue or overresectionof tissue during the primary repair. Correction ofthe tight lip may require a lip-switch procedure tobalance the lips and minimize the disproportion.Abbe481 described the first successful transfer of alower lip flap to the upper lip in a bilateral cleftdeformity. The Abbe flap is placed in the midlineand every attempt is made to recreate the phil-trum.482,483 Flap size should approximate normalphiltral dimensions in reverse, because the flap isgoing to be transposed 180° (Fig 46).484 In otherwords, the flap should be made

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— 0.8–1.2cm wide at the vermilion border— 0.6–0.9cm wide at the base of the columella— 1.7cm high485

Fig 46. Raised Abbe flap in reconstruction of the philtrum.(Reprinted with permission from McGregor IA: The Abbe flap. Itsuse in single and double lip clefts. Br J Plast Surg 16:46, 1963.)

Millard486 states that “the common mistake madeby many is fitting the Abbe flap to the apparentdefect rather than reducing or rearranging the defectto philtrum dimensions.”

Jackson and Soutar487 report transfer of a “sand-wich” Abbe flap consisting only of skin and mucosathat avoids the excessive bulk and static appearancewhich often results from transferring the lower liporbicularis to the upper lip. Z-plasties along themucocutaneous junctions help prevent secondarynotching of the vermilion margin.455 The pediclecan be safely divided 7–12 days after transfer.483

Millard reports survival of an Abbe flap on a mucosalpedicle devoid of the marginal artery.488

The appearance of a tight upper lip is oftenaccentuated by mesodermal insufficiency and maxil-lary hypoplasia of the primary cleft anomaly. Mea-sures to correct maxillary retrusion in secondary cleftsurgery are reviewed below.

Horizontal Tissue Excess – Wide Lip

The overly wide lip after bilateral cleft repair iscommon and may result from designing the newphiltrum too widely during primary repair. Staland Hollier453 emphasize that no more than 4–5mm of the prolabial skin should be used forphiltral reconstruction during primary bilateral liprepair. There is a tendency of the philtrum towiden over time due to either muscle pull on the

lateral philtrum or because the muscle is not ad-equately reconstructed in the midline. Secondarycorrection involves muscle-realigning techniquesand removing excess skin of the philtrum to recre-ate acceptable philtral dimensions. Stal andSpira449 mention possible adjunctive reduction ofthe wide prolabium and lateral lip combined withcolumellar advancement.

Vertical Shortage – Short Lip

A short lip is more common with bilateral cleftdeformities, as there is an inherently greater tissuedeficiency. Z-plasty techniques may lengthen theshort lip but depend on sufficient tissue along thetransverse vector of the lip to effect the rearrange-ment. When the lip is not wide enough horizon-tally, an Abbe flap may be necessary.481

Vertical Excess – Long Lip

A long lip deformity may occur after bilateral liprepair due to errors in initial repair design, failure tosuspend the orbicularis from the periosteum, or amaxillary retrusion. The cutaneous portion of theupper lip has a tendency to lengthen over time eventhough there may be vermilion deficiency. Match-ing the height of the lateral segments to the height ofthe philtrum during primary repair lessens the verti-cal pull on the central lip that contributes to long lipdeformity. Correction can be achieved by the meth-ods described for secondary deformities of the uni-lateral cleft lip.

Missing or Misplaced Landmarks

The prolabium lacks philtral landmarks in bilat-eral clefts. During primary repair it is very difficult tosimultaneously approximate the central muscle andcreate a philtral dimple. Philtral and Cupid’s bowdeformities in bilateral clefts are amenable to proce-dures mentioned above for secondary correction ofunilateral cleft lip defects.

Vermilion Deficiencies

Central vermilion deficiencies are typically seenafter bilateral cleft repair. When the whistling defor-mity is only relative—that is, secondary to excessivevermilion laterally, not vermilion deficiency medi-

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ally—simple transverse wedge excisions should beconsidered.

Guerrero-Santos and associates489 interdigitate adenuded flap from the “excess” lateral vermilion tis-sue into the medial vermilion to minimize notching.Kai and Ohishi490 also prefer medially transposedlateral vermilion flaps. The double lateral V-Yadvancement491 (Fig 47A) or double-pendulum islandflap492 (Fig 47B) are other options in the correctionof central vermilion deficiencies.

Correction of large absolute tissue deficits in thecentral vermilion may require the transfer of vermil-ion flaps from the lower lip.474 Holmstrom493

described a deepithelialized Abbe island flap thatbrings ample bulk and suitable lining for tuberclereconstruction and to help balance the lips. Guerrero-Santos494 suggests pedicled tongue flaps that are either

Fig 47b. Central whistling deformity corrected by double lateralpendulum flaps. (Reprinted with permission from Millard DR Jr:Cleft Craft—The Evolution of Its Surgery. II. Bilateral andRare Deformities. Boston, Little Brown, 1977.)

Fig 47a. Central whistling deformity corrected by double lateralV-Y advancement. (Reprinted with permission from Millard DRJr: Cleft Craft—The Evolution of Its Surgery. II. Bilateral andRare Deformities. Boston, Little Brown, 1977.)

transferred directly from the tip of the tongue ordenuded and buried in the central upper vermilion.

Buccal Sulcus Abnormalities

Primary bilateral cheiloplasty techniques that donot elevate the prolabium off the premaxilla willalways require secondary reconstruction of the uppersulcus, preferably with local flaps. Free grafts usuallyneed to be splinted and are second choices in chil-dren.

Hogan and Converse495 described a large V-Yadvancement of the entire labial sulcus. After releaseof the adhesions, medial and inferior advancementof the labial mucosa and vermilion produces aninternal vertical scar. This method was later adoptedand modified by O’Connor,496 who undermined thelabial mucosa widely on either side and sutured it inthe midline as advancement flaps. Because of theirtendency to contract, mucosal transposition oradvancement flaps should be designed with size andbulk to spare. Horton and coworkers497 recreatedupper sulcus depth using combinations of Z-plastyand V-Y flaps supplemented with mucosal grafts onthe denuded alveolar bone (Fig 48).

Fig 48. Shallow buccal sulcus corrected by combination V-Zadvancement. (Reprinted with permission from Horton CE et al:The upper lip sulcus in cleft lips. Plast Reconstr Surg 45:31,1970.)

Orbicularis Oris Deformities

Veau498 first recognized the importance ofmuscle approximation in bilateral cleft lip in 1938.Proper reconstruction of the perioral and paranasalmuscles helps shape the bones of the midface andcan prevent or minimize secondary skeletal defor-mities to the point where they can be treatedorthodontically.499

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Schafer and Goldwasser500 address the secondaryorbicularis oris deformity by dissecting all compo-nents of the orbicularis muscle and adjacent paranasalgroups, as recommended by Schendel and Delaire.501

The lateral nasal muscles are mobilized, rotateddownward and medially as far as possible, andattached to the base of the septum and columella orinto the upper portion of the orbicularis oris. Theorbicularis fibers are anchored to the periosteum ofthe anterior nasal spine and directed downward tothe pouting tubercle of the lip.

Whistling deformities and other apparent centrallip tissue deficiencies are in many instances correctedsimply by approximating or plicating the orbicularis.Jackson502 notes that lips tend to lengthen second-arily following orbicularis reconstruction. Oneal andassociates499 documented a doubling of the verticalvector of the lip following orbicularis union in themidline, with resultant elongation of the central lip.These observations suggest that orbicularis approxi-mation is potentially helpful in dealing with the con-genitally short prolabium503 and should be done asearly as possible.

Lower Lip Changes

The degree of acquired deformity in the lower lipparallels the severity of the cleft and is particularlynoticeable in patients with bilateral complete clefts.

Pensler and Mulliken504 describe the typical ana-tomical alterations in the cleft lower lip. The soft-tissue components manifest as lower lip hypertrophyand ectropion with anterosuperior displacement ofthe soft-tissue points and inferolateral displacementof the oral commissure. The skeletal changes consistof “variable and inconsistent” vertical elongation ofthe mandible and posterior displacement of the chin.Patients who exhibit skeletal changes require skeletalsurgery initially, followed by soft tissue procedures.Lower lip reduction procedures may be necessary toachieve a balanced soft-tissue profile after skeletaldiscrepancies are addressed.

REPAIR OF SECONDARY CLEFT LIP NASALDEFORMITIES

The greatest advance in cleft lip nasal surgeryhas been a nonsurgical intervention, nasoalveolarmolding.505 Nasoalveolar molding in the first fewmonths of life can reshape the deformed alar

cartilages and stretch the nasal lining, which greatlyfacilitates primary nasal repair and diminishes sec-ondary nasal deformities.505 Although primary nasalrepair at the time of lip repair has become muchmore popular and may have decreased the needfor secondary nasal revision during childhood, mostpatients still require surgery for correction of thesecondary cleft nasal deformity when growth ends,if not before.

Randall506 and Rifley and Thaller507 reviewed theliterature of repair of cleft nasal deformity. The func-tional objectives of surgery for correction of the cleftnose deformity include:

• relieving the soft-tissue occlusion of the cleft air-way

• repositioning the nasal skeleton to provide for apatent airway

• skeletal rearranging to permit Class I occlusion

• manipulating the hard and soft tissues to allownormal speech

The aesthetic objectives are to achieve:

• bilateral symmetry of nasal components

• improvement of nasolabial and nasofacial rela-tionships

• a final result that is subtle in detail, with minimalsurgical tracks

Maxillary hypoplasia is also an important compo-nent of the cleft nasal deformity, as was recognizedby Gillies and Kilner508 and McIndoe.509 As Millard448

states, “even when there is no bony cleft, the dis-crepancies in maxillary contour are responsible forsome degree of nasal asymmetry.”

Occasionally alveolar bone grafting alone is suf-ficient to stabilize the nasal base, and at othertimes onlay grafts are all that is needed, but mostoften correction of the hypoplastic maxillarequires combined orthodontic and surgicaltherapy. Augmentation of the deficient maxillaryplatform on the cleft side using autogenous graftsof cancellous bone or cartilage has been describedby many.495,510–513 Because of problems withresorption of bone and distortion or displacementof cartilage, Byrd and Hobar514 prefer augmenta-tion with hydroxyapatite granules.

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TIMING OF SURGERY

The best time to attempt correction of a cleft nasaldeformity remains controversial. The repair can besimultaneous with primary lip repair, in the preschoolyears (age 5–6), during puberty (age 10–12), or inthe adult. The overriding clinical questions are:1) When can we predictably perform each proce-

dure without adversely affecting the integrity orgrowth of normal structures?

2) When does correction of the deformity becomeimportant to the patient?

3) Can the procedure correct the deformity com-pletely, and if not, would it render later correc-tions more difficult?

The cleft rhinoplasty performed on the growingpreschool age patient is distinctly different from therhinoplasty performed after growth is complete. Theprimary goal of the cleft nasal revision during thepreschool years is to reshape and reposition the tipand alar cartilages to achieve better projection andsymmetry. Extensive septal dissection and osteoto-mies are avoided during childhood to avert anygrowth disturbance. After a patient’s growth is com-plete, a definitive osseocartilaginous septorhinoplastycan be performed safely.

Preschool Age

At age 5–6, social pressures heighten patients’ per-ceptions of any residual nasal deformity and intensifytheir (and their parents’) demands for correction.Millard515 considers the alar cartilage too delicate forsurgical manipulation before age 4–5, after whichtime it can be freed and repositioned in relative safety.He recommends complete release and mobilizationof the medial three-quarters of the cleft alar cartilagefrom both skin and lining; medial advancement ofthe cartilage; formation of a new crus angle; andsuture fixation to the dorsum and tip of the sep-tum454 (Fig 49).

The nasal septum grows most actively betweenages 6 and 10, with most of the growth in thesuprapremaxillary area and anterior border.516–518

Takato and coworkers519 described early correctionof the nose through an open method in unilateralcleft lip patients. The cleft nasal deformity was cor-rected during the preschool years, and follow-upevaluation was done when the patients were 15–

19yo. Although early results appeared satisfactory,as the patients approached their adolescent growthspurt at approximate age 15, undesirable featuresbecame obvious. Patients showed strikingly largenoses with large amounts of subcutaneous fat, thickskin, and a wide nasal tip. These features wereconsidered unique to patients undergoing early cleftnasal repair by the open method. Consequently,the authors discontinued open rhinoplasty inpreschoolers and now delay secondary correctionuntil age 9–12.

In contrast, Lamont520 states that “rhinoplasty maybe done when the patient is 7 or 8 years of agewithout deleterious effects. Care must be taken thatthere is no injury to the vomer during the process ofstraightening and readjusting the septum.” In sup-port stands Siegel’s521 study of the effects of septalresection in 2–4-month-old chimpanzees, from whichhe concluded that “resection of the septal cartilageat an early age has no detectable effect on subse-quent hard tissue growth.”522

Ortiz-Monasterio and Olmedo523 evaluated 44patients who had “complete” rhinoplasties at age 8–12, primarily because of residual cleft deformities.Septoplasty “limiting the dissection of mucoperi-chondrium as much as possible” and keeping septalresection to a minimum was performed in 23 patients.The authors found that “nasal and facial growth werenot altered when the rhinoplasty was performedbefore 12 years of age.”

Fig 49. Medial repositioning of the displaced alar cartilage.(Reprinted with permission from Millard DR Jr: Unilateral CleftLip Deformity. In: McCarthy JG (ed), Plastic Surgery. Philadel-phia, WB Saunders, 1990. Vol 4, Ch 52, pp 2627-2652.)

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Despite the facial growth data, these series indi-cate that conservative septoplasty with septal reposi-tioning in the preschool-age child, if indicated becauseof airway obstruction or significant cosmetic defor-mity, probably has little if any adverse effect on skel-etal development of the face.

Puberty or Adolescence

Nasal growth is complete between the ages of 11and 12 for women and 13 to 14 for men.505

Millard448,515 and Ortiz-Monasterio523 argue fordelaying correction of the cleft nasal deformity, ifpossible, until age 10–15 when definitive osteoplas-tic rhinoplasty can be performed safely and predict-ably. The patient’s level of maturity and ability toparticipate in decision-making are important factorsin determining the appropriate timing of definitiverhinoplasty. Patients who will require orthognathicsurgery should have correction of the bony platformbefore the final nasal revision.

RESIDUAL NASAL DEFORMITIES-UNILATERAL CLEFTS

The anatomy of the secondary unilateral cleft nasaldeformity is well described by the tilted tripod con-cept.524 The tripod is composed of the septum, whichprovides central support; the nasal sidewalls andlower lateral cartilages, which make up the sides;and the maxilla, which is the base. The hypoplastic,laterally displaced maxilla associated with the cleft lipdeformity creates an altered platform for the tripodthat manifests as a laterally splayed ala, loss of nasaltip projection and definition, an oblique alar-facialangle, and septal deviation.

Millard448 chronicles the evolution of proceduresfor correction of the unilateral cleft nasal deformityin Cleft Craft I. Secondary procedures escalate incomplexity according to severity of the residualdeformity, and may involve:525,526

a) repositioning of cleft side nasal components ascomposite chondrocutaneous units

b) release and manipulation of cartilaginous com-ponents separately from soft-tissue components

c) septoplasty with or without repositioning of septalcomponents

d) septal resection and bony manipulation involvingosteotomies and grafts

Correction of the Alar Deformity

External Incisions

External incision–excision techniques for second-ary correction of the cleft lip nasal deformity havehad many proponents.508,527–538 Advantages ofexternal incisions are wide exposure and access tothe subcutaneous nasal architecture for precisedelineation of anatomic derangement; easier mobi-lization of structures; greatest correction of dorsonasaldeformities; and accurate reconstruction of nasalshape under direct vision.

Open rhinoplasty for the correction of cleft lipnasal deformities is most commonly performedthrough bilateral marginal or rim incisions connectedby a stair-step transcolumellar incision or a V-shapedincision at either the base of the columella or itsmidpoint, depending on the amount of columellarlengthening desired at the time of closure.539 Whenthere is excessive lip tissue, Bardach451,536 carries theincision onto the lip scar and closes the incision in V-Y fashion. Kirschbaum and Kirschbaum540 describemedial-to-lateral rotation of a chondromucosal sleeve(Fig 50) for secondary correction of the cleft lowerlateral cartilage. They report satisfactory results in 52patients.

Chen and Noordhoff541 describe their experiencewith open tip rhinoplasty in the correction of sec-ondary nasal deformities in 122 patients. Their pro-cedure entails an open rhinoplasty, complete releaseof the cleft lower lateral cartilage, and advancementin a V-Y fashion. The cleft-side alar cartilage is suturedto the lower edge of the upper lateral cartilage andthe medial crura are fixed to each other (Fig 51).

Other techniques incise portions of the alarcartilages and relocate them.542–546 Hubli et al547 reportsuccessful removal of the alar cartilages from the nasalvestibule and reconstruction of the entire nasal tipunit on the side table. The new nasal tip is thenreplaced and secured with bolster dressings. This car-tilage splitting, excision and transposition maneuver isrisky, and most authorities would rather preserve theexisting anatomy and make corrections by reposition-ing or reshaping the alar cartilages.

Others520,548–561 prefer cartilage graft augmenta-tion of the deformed ala (Fig 52). The graftsapparently keep their intraoperative dimensionsand positions reasonably unchanged when the sur-gery is performed on adolescents or adults.448,523,562

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Tschopp’s510 modification of Potter’s533 open skyapproach involves exposure of the skeletal frame-work of the nose through an external incision andgraft augmentation of the lateral crus using strips ofcartilage from the septum and ipsilateral ala.

The alar unit (lobule complex) rotation techniqueconsists of a vertical incision on the columella as wellas dome and nostril sill incisions (Fig 53). The proce-dure elongates the cleft columella and dome whilenarrowing the nostril sill.

Blair527 first described superior and medial rota-tion of the cleft nostril through an incision extendingfrom the alar crease across the nostril sill and up the

Fig 50. Chondromucosal sleeve for secondary correction of thealar cartilage in cleft lip. A and B, the skin of the nose is incisedand elevated. C, the chondromucosal sleeve flap is rotated andsutured in place. D, after closure of the skin. (Reprinted withpermission from Kirschbaum JD, Kirschbaum CA: Thechondromucosal sleeve for the secondary correction of theunilateral cleft lip nasal deformity. Ann Plast Surg 29:402, 1992.)

Fig 51. Open tip rhinoplasty for the correction of severe cleft lipnasal deformities. The lower lateral cartilage is advanced in V-Yfashion and fixed to the lower edge of the upper lateral cartilage.The medial crura are also sutured to each other. (Reprinted withpermission from Chen K-T and Noordhoff MS: Open tip rhino-plasty. Ann Plast Surg 28:119, 1992.)

middle of the columella to the tip. His procedurewas widely adopted and has since been modified bymany.563–567 Unfortunately, the scars left by thesetechniques may be prohibitive.

Koh and colleagues568 advocate an asymmetric inci-sion to correct the nostril overhang through an openapproach. The ala on the cleft side is lifted with forcepsto achieve symmetry with the nasal tip, then an intrana-sal rim incision is marked from the normal side acrossthe columella and onto the raised nostril rim. Whenthe elevating forceps are removed, the incision line onthe cleft side extends outside the nostril (Fig 54). Noconchal cartilage grafts were used. Nasal retainers werekept in place for 6 months in all patients.

Intranasal Incisions

Some surgeons still prefer intranasal incisions forthe correction of secondary cleft nasal distortions,but intranasal incisions are less common todaybecause of the reliable record of open rhinoplastiesand the extensive exposure they afford in the repairof these complex deformities. Figure 55 illustratestechniques based on mobilization of the deformedalar cartilage from its surrounding soft tissue, medialand superior advancement, and suture suspensionto other nasal structures.532,537,569–577

Long-term correction depends on adequate ana-tomical mass of the cleft alar components, structural

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integrity of the elements following dissection, andmaintenance of position by suture tension or subse-quent adherence and scar formation. The likeli-hood of a successful outcome with this type of pro-cedure is limited by scarring in the operative field,marked deformity or attenuation of the cleft alarcartilage, inadequate soft-tissue mobilization, or injuryto delicate structures during dissection.

Coghlan and Boorman578 evaluated the early andlate results of the Tajima cleft nose correction.Although nasal symmetry within 3mo of the proce-dure was considered good, over time (1y+) therewas progressive worsening of nasal symmetry and“nasal shape could not be statistically separated fromthe preoperative appearance.”

Septal Deformity and Nasal Airway Obstruction

In a unilateral cleft lip septal deformity, the sep-tum follows a typical pattern of malformation. There

Fig 52. Graft augmentation techniques. (Reprinted with permission from Millard DR Jr: Cleft Craft—The Evolution of Its Surgery. I.The Unilateral Deformity. Boston, Little Brown, 1976; Dibbell DG: A cartilaginous columellar strut in cleft lip rhinoplasties. Br J PlastSurg 29:247, 1976; Chait LA: The ‘C’ costal cartilage graft in reconstruction of the unilateral cleft lip nose. Br J Plast Surg 34:169, 1981;and Thomson HG: The residual unilateral cleft lip nasal deformity: A three-phase correction technique. Plast Reconstr Surg 76:36, 1985.)

is subluxation of the anterior septal edge toward thenoncleft side. As a consequence of deformity of thepiriform aperture, alar bases, lower lateral cartilages,and nasal septum, there is deviation of the entirenose toward the healthy side.

Correction of the septal deformity is an importantstep to address the deviation of the external noseand to obtain a satisfactory aesthetic and functionalresult. The septal deformity is usually corrected withsubmucous cartilaginous resection, repositioning andfixation at the midline of the caudal septum, correc-tion of the intrinsic remaining L-strut deformities withcontrolled Mustardé sutures, conservative scoring, andsplinting with previously harvested strut of septal car-tilage in order to nullify the intrinsic deformationaltendency. The septal correction can be performedthrough a closed or an open approach. The openapproach appears to provide better and more stablecontrol of the final result.

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Fig 53. Lobule complex rotation techniques. (Reprinted with permission from Millard DR Jr: Cleft Craft—The Evolution of ItsSurgery. I. The Unilateral Deformity. Boston, Little Brown, 1976; and Dibbell DG: Cleft lip nasal reconstruction: Correcting theclassic unilateral defect. Plast Reconstr Surg 69:264, 1982.)

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Gubisch and others579 advocate the use ofextracorporeal septoplasty when the septal defor-mity is extremely severe. Through a closed oropen approach, the cartilaginous and bony sep-tum are removed as one plate. Remodeling of astraight septum is obtained with scoring technique,selective division of the septum in straighter com-ponents that are rearranged and inset together toobtain a straight shape, and selective splinting witha thin piece of the lamina perpendicularis of theethmoid. The authors performed 141 extracorpo-real septoplasties and seemed satisfied with theirresults. The main problem with this technique isthe residual postoperative irregularity of the nasaldorsum, though this can be camouflaged by theuse of a cartilage onlay graft or autogenous tem-poral fascia.

Nasal airway obstruction can be caused by septaldeviation, inferior turbinate hypertrophy, internalnasal valve collapse, external valve constriction, orvomerine bone spurs. An inferior turbinectomy,speader grafts, expansion of the external nasal valve,and removal of bone spurs may be required in addi-tion to septoplasty.

Nasal Tip Support

Nasal tip support is an important component ofsecondary cleft nasal reconstruction. The tip mayneed additional support for projection and symme-try. The deformed cleft alar cartilages may require avariable amount of support for symmetry. Septalcartilage columellar strut grafts or extended spreadergrafts are usually sufficient in mild deformities, butmore severe deformities may require rib cartilagecolumellar strut grafts.203,505,580

When nasal tip support is deficient, Gubisch andothers579 suggest rotating the septum 180° so that thebony septum comes forward to support the nasal tip.The nasal spine is relocated in the midline, where itis secured with a microplate. The anterior caudalseptum is repositioned and inset with sutures in agroove carved on the superior portion of the nasalspine.

RESIDUAL NASAL DEFORMITIES – BILATERAL CLEFTS

The characteristic components of the secondarynasal deformity in bilateral clefts are as follows483

(Fig 56):

• a short columella

• a depressed, flat-appearing nasal tip, sometimesnotched in the midline

• lateral displacement of both alar domes withbilateral dislocation of the lateral crura from theseptum

• hooding of the alar rims

• flaring alar bases

• scarred vestibular floors

• bilateral maxillary hypoplasia

In Millard’s483 words, “the only positive light in thedarkness of the double deformity is that . . . [it]usually has a quality of symmetry.”

Nostril Overhang

Ombredanne581 used a nostril rim excision tocorrect the cleft nostril overhang, while Gillies andKilner508 excised the free margin of the cleft alarcartilage together with its lining in elliptical fashion,rolling external skin into the resultant lining defectfor closure.

Fig 54. Raising the overhanging nostril with an asymmetric alarrim incision. (Reprinted with permission from Koh KS, Eom JS:Asymmetric incision for open rhinoplasty in cleft lip nasaldeformity. Plast Reconstr Surg 103:1835, 1999).

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Fig 55. Suture suspension techniques. (Reprinted with permission from Millard DR Jr: Cleft Craft—The Evolution of Its Surgery.I. The Unilateral Deformity. Boston, Little Brown, 1976; Millard DR Jr: Earlier correction of the unilateral cleft lip nose. PlastReconstr Surg 70:64, 1982; Tolhurst DE: Secondary correction of the unilateral cleft lip nose deformity. Br J Plast Surg 36:449, 1983;and Cronin TD, Denkler KA: Correction of the unilateral cleft lip nose. Plast Reconstr Surg 82:419, 1988.)

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Straith582 described a Z-plasty of the alar hood toelongate the columella and simultaneously correctthe nostril overhang. Using external and internal rimincisions respectively, Pitanguy583 and Meyer584

infolded the overhanging rim while advancing adeepithelialized flap of this tissue beneath an exter-nal skin flap.

Gubisch585 described a complex triple swing flaptechnique consisting of a flap from the columella, aflap from the cleft ala, and a flap based at the junc-tion of the columella and ala. The author has usedthis flap in 201 patients to secondarily correct asym-metrical nostrils in unilateral cleft lip. The illustratedresults are largely satisfactory.

Vestibular Web

Berkeley528,586 coined the term vestibular web todescribe a bowstring contracture of the nasal liningextending from the tip across the lateral vestibule tothe piriform aperture. This vestibular fold is formedby abnormal position of the lower border of thelower lateral cartilage.587 When the alar base isrepositioned following secondary correction of thecleft nasal deformity, the vestibular web may eitherdisappear or be exaggerated, protruding into the nos-tril lumen and interfering with airflow. From a basi-lar view it seems as if a curtain partially occludes thevestibule.

Correction of a vestibular web involves either amedially based chondromucosal flap advanced inV-Y fashion533,588 or Z-plasties inside the ala alongthe longitudinal axis of the web.574,589–591 Reesand associates573 modified Potter’s chondromucosalflap by advancing the cleft lateral crus and dome

medially and closing the resultant lateral defectwith a full-thickness retroauricular skin graft.

Deficits of Nasal Lining

When the nostril is stenotic or heavily scarred,Z-plasties and V-Y advancements such as describedabove will not be sufficient to restore nasal liningthat is deficient after primary lip repair. Matsuoand Hirose592 described a technique for replacingnasal lining in secondary cleft deformities with alarge, elliptical composite graft from the auricularconcha that is placed in the lateral wall of theala, from the lateral crus to the piriform aperture(Fig 57).

Fig 57. Deficiency of nasal lining corrected by a conchal compos-ite graft, which also helps raise the sunken ala. (Reprinted withpermission from Matsuo K, Hirose T: Secondary correction of theunilateral cleft lip nose using a conchal composite graft. PlastReconstr Surg 86:991, 1990.)

Vissarionov593 believes that the slumped nasal tiptypical of secondary cleft deformities is in large partdue to “inadequate restoration of the lining defecton the cleft side.” With healing and subsequentshrinkage of tissues back to their original position,the tip is pulled down and toward the cleft-sidenostril. His method uses skin from the lip scar thatis brought into the vestibule as a sliding flapVissarionov reports satisfactory correction and sym-metry of the nasal tip in 85% of more than 500adults (Fig 58).

Depressed Nostril Sill

When the cleft-side orbicularis oris is mobilizedand transposed downward from its abnormal posi-tion, additional deficiency beneath the alar base mayresult. Augmentation with dermis fat grafts594 or bymuscle interdigitation595 from the contralateral side

Fig 56. The bilateral cleft nasal deformity.

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may improve the result. Patients who have a widescar and depressed nostril sill after lip repair may alsobenefit from sill augmentation with a superiorly basedscar flap as described by Uhm and coworkers.596

The primary disadvantage of this procedure lies inits use of scar tissue from the site of primary cleftrepair, and is therefore contraindicated in patientswho have a symmetrical lip and a well-healed, finescar.

Agarwal and associates597 recommend the use ofa superiorly based turnover orbicularis oris muscleflap to augment the deficient nasal sill. In his discus-sion of this paper, Randall598 reminds us that defi-cient alveolar bone also contributes to the deformity,and aesthetic correction frequently requires eitherbone graft or hydroxyapatite augmentation.

RESIDUAL SKELETAL DEFORMITIES

Oral clefts affect normal skeletal growth of themaxilla. Maxillary hypoplasia, retrusion, and medialcollapse of the cleft segments are common. Rota-tion and positional changes of the mandible developin compensation to the abnormal maxillary arch.Cleft surgery also contributes to maxillary growthrestriction, residual secondary skeletal deformities,and malocclusion. It is estimated that 25-40% ofcleft patients require orthognathic correction of their

midface retrusion to achieve a functional relation-ship of the jaws and teeth.599,600

MAXILLARY HYPOPLASIA

Patients with clefts can have severe maxillaryhypoplasia in ventral, horizontal, vertical and trans-versal planes.601 Secondary deformities of the max-illa have a negative effect on speech, occlusion andfacial appearance. An integrated team approach tothe correction of secondary skeletal deformities isessential, and in particular close cooperation betweenthe surgeon and the orthodontist is needed to opti-mize final outcome.602 Significant discrepanciesbetween the maxillary and mandibular arch due tomaxillary hypoplasia can be treated using conven-tional orthognathic techniques, modified orthognathicprocedures that incorporate closure of the cleft anddental gap, and by distraction osteogenesis.

The timing of surgery for skeletal deformities inpatients with clefts is controversial. Correction ofskeletal deformities is traditionally performed duringthe stage of permanent dentition and after patientsreach skeletal maturity. Some surgeons advocateorthognathic procedures during the stage of mixeddentition, however. Reported benefits of early cor-rection of maxillary retrusion include fewer emo-tional problems and improved occlusion, mastica-tion, maxillary growth potential and respiratory func-tion.603 In contrast, other authors report that earlyjaw surgery in cleft patients often requires revisionalorthognathic procedures once skeletal maturity isreached.604 Additional risks of early surgery includeinjury to developing permanent teeth and fibrouscallous formation at the osteotomy site.605 The func-tional and psychological advantages of early surgerymust be carefully weighed against the risk of toothinjury, potential impairment of maxillary growth, andneed for future revisional surgery.

ORTHOGNATHIC SURGERY

The Le Fort I advancement osteotomy is the main-stay of orthognathic treatment for maxillary hypopla-sia in the cleft patient. Posnick and Ricalde605 reviewthe evolution of and their experience with cleftorthognathic surgery. Patients with significant maxil-lary hypoplasia who have undergone successful treat-ment of their alveolar cleft with alveolar bone graftand fistula repair are usually treated with orthodon-tic closure of their dental gap and conventional Le

Fig 58. Deficiency of nasal lining corrected by a sliding flap of lipscar (with excision of a crescent of skin from the overhanging alarrim). (Reprinted with permission from Vissarionov VA: Correc-tion of the nasal tip deformity following repair of unilateral cleftsof the upper lip. Plast Reconstr Surg 83:341, 1989.)

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Fort I advancement. The reported recurrence rateafter conventional Le Fort I advancement is approxi-mately 20%.606,607

Variations in the presenting pathology of theresidual cleft deformity on the midface may includepersistent alveolar defects, oronasal fistulas, maxil-lary deficiency, and concomitant nasal, mandibular,or chin abnormalities. Modifications of the maxillaryLe Fort I osteotomy in cleft orthognathic surgery havebeen described to deal with these sundry problems.These operations are designed to allow for simulta-neous and safe management of soft-tissue scarringand skeletal abnormalities.

For the subset of adolescent patients with residualalveolar clefts and oronasal fistulas, Posnick etal605,608,609 described a one-stage procedure involvingsegmental Le Fort I osteotomies, bone grafting, rigidfixation, and mucosal closure using incisions that pre-serve the vascularity of the dento-osseous-musculo-mucosal flaps. Posnick and Tompson609 prospec-tively assessed the results of their modified Le Fort Iprocedure in 126 consecutive adolescents with UCLP(66), BCLP (33), and isolated CP (17). Successfulmaxillary advancement and oronasal fistula closurewas achieved in 92% of patients with UCLP and79% of patients with BCLP. Keratinized mucosa wassuccessfully maintained along the labial surface ofthe cleft-adjacent teeth in all patients. At least oneyear after surgery, 97% of patients with UCLP and94% of patients maintained a positive overjet. Apositive overbite was maintained in 91% of patientswith UCLP and 82% of patients with BCLP. Nopatients required reoperation because of poor facialaesthetics or malocclusion. No serious complica-tions were reported.

Velopharyngeal function must be carefully evalu-ated before maxillary advancement. Previouslyplaced pharyngeal flaps may limit the amount ofadvancement that can obtained, and in rare circum-stances the pharyngeal flaps may need to betransected. Posnick and Ewing610 reported morerelapse in patients who had prior pharyngoplasty.Nasoendoscopy is used to predict changes invelopharyngeal function after advancement. Patientswith borderline velopharyngeal function may developVPI after Le Fort I advancement and need to be coun-seled preoperatively regarding treatment options andsequencing.

Schendel and Delaire501 and Kinnebrew and col-leagues611 combine maxillary osteotomies with

definitive soft-tissue (lip–nose) correction in the sec-ondary cleft deformity. Because optimal results inadults require precise adjustments of the maxillarysegments within 2–3mm, most surgeons agree thatthe final bony position should be established beforerevision of the soft-tissue deformities.

DISTRACTION OSTEOGENESIS

Patients with severe maxillary hypoplasia whorequire significant maxillary advancement may notachieve the desired results with traditional orthodon-tic techniques and conventional orthognathic sur-gery. Because of maxillary scarring, advancement>6mm is difficult to achieve by conventionalorthognathic surgery.612 To compensate for this lim-ited advancement, concomitant setback of the oth-erwise normal mandible is frequently performed inthese patients, camoflagued by advancment genio-plasty.

Because of the positive experience with distrac-tion osteogenesis of the hypoplastic mandible, sur-geons have applied these methods to distraction ofthe midfacial skeleton. Maxillary distraction hasbecome an effective alternative to conventional meth-ods for correcting significant maxillary hypoplasia inpatients with clefts, and is associated with a low rateof recurrence.

Molina, Ortiz Monasterio, and colleagues613

describe their experience in 38 patients aged 6–12y:18 had unilateral cleft lip and palate, 9 had bilateralcleft lip and palate, 7 had unilateral cleft palate, 2had prognathia, and 2 had nasomaxillary dysplasia.An incomplete horizontal osteotomy was done abovethe tooth buds with pterygomaxillary dysjunction anddissection along the medial nasal floor and the baseof the septum. The distraction started on the fifthpostoperative day and lasted about 3–4 weeks; itconsisted of a facial mask and an intraoral fixedappliance system. At the conclusion of distraction,the range of maxillary advancement was 4–12mmand all patients had satisfactory Class I or II occlusalrelationship. If needed, the hypoplastic maxilla wasalso elongated with a combination of forward anddownward distraction forces. The authors reportimproved nasal breathing and unchanged velo-pharyngeal function after the procedure. No relapseswere observed in follow-up of 6–36mo. In a subse-quent report, however, Molina603 noted relapse in 3patients aged 7–8yo. No change in velopharyngeal

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function was observed following advancement of upto 12mm.

Polley and Figueroa614 used maxillary distractionosteogenesis for the treatment of 18 consecutiveorofacial cleft patients with severe maxillary hypo-plasia. Patient age ranged from 5.2–25.2y. Allpatients underwent high Le Fort I maxillary osteotomywith pterygomaxillary and septal dysjunction. Four-teen patients had rigid external distraction (RED) witha halo external distraction device, and 4 patients hadface mask distraction with elastics. The halo externaldistraction device was very effective, for a mean hori-zontal maxillary advancement of 11.7mm. No relapsewas reported. In the patients who had face maskdistraction, the maxillary hypoplasia was under-corrected, with a mean advancement of only 5.2mm.The authors conclude that full correction of the skel-etal and soft-tissue deficiency of the midface requires,at minimum, rigid external distraction osteogenesis.

Kozák et al601 compared the results of maxillaryadvancement using an intraoral (internal) bone-anchored midface distractor (IMD, 5 patients) versusa tooth-anchored rigid external distractor (RED, 11patients) with halo. The average patient age in theIMD group was 19y; in the RED cohort, 11.5y. Theaverage advancements were 10.6mm and 10.1mmfor IMD and RED respectively. A relapse of 2mmwas observed in patients treated with the IMD aftera mean follow-up of 4+ years. There was no wors-ening of velopharyngeal function after distraction.The authors recommend that alveolar bone graftingbe completed prior to distraction due the the risk ofpathologic mobility of the premaxilla, which occurredin one patient with an ungrafted alveolar cleft. Otherauthors have used dental splints to unite ungraftedcleft, segments with reported success.614

The RED device has the advantage of controllingmultiple vectors of advancement and can be adjustedduring distraction to direct change in a vertical, ven-tral, and lateral direction. The internal distractiondevice transfers force from bone to bone and is bestapplied after skeletal maturity, unlike the RED devicewhich transfers force from teeth to bone and can beapplied to younger patients. The main disadvan-tages of the internal distraction device are that it onlyhas one or two vectors of distraction and cannot beadjusted after device placement. Unlike the RED,the internal device requires surgical removal afterdistraction is completed. On the other hand, inter-

nal devices are better tolerated by patients psycho-logically than the RED device with halo.

Kita et al615 published a case report of a 15yopatient with BCLP, ectodermal dysplasia, severe max-illary deficiency, and numerous congenitally missingteeth who was treated with predistraction alveolarbone grafting and Le Fort I distraction using an REDdevice with halo and miniplate skeletal (not dental)anchorage. The maxilla was moved 11mm anteri-orly and 13mm inferiorly at the completion of dis-traction. After 2y the sustained anterior and inferiormovement was 8.5mm and 8mm respectively. Theadvantages of a bone-anchored RED device are a1:1 ratio of dental-skeletal movement and control ofmultiple vectors. Disadvantages of this techniqueare miniplate fixation to thin maxillary bone, thepotential for tooth bud injury, and the need forminiplate removal. The authors recommend thistechnique for use in patients with permanent denti-tion.

Gateno et al616 reported their experience with anew Le Fort I internal distraction device that is pre-bent on a stereolithographic model of the patient tocalculate the distraction vector. The activation armis placed in the maxillary sinus to avoid trauma to thebuccal mucosa, a problem seen with other internaldevices. In this pilot study of 3 patients aged 12–16y, the relapse rate ranged from 0–5mm. Relapseoccurred during the consolidation phase, but noadditional relapse was noted on 3mo follow-up. Theactual vector of distraction was noted to be differentfrom the planned vector, and was attributed to theclockwise rotation of the maxilla with a tendencytoward a posterior open bite. There was no maxil-lary growth after distraction but continued mandibu-lar growth. Dental compensation was used to main-tain a positive overbite, but the authors recommenddelaying maxillary distraction until the completion offacial growth.

OVERVIEW OF SURGICAL MANAGEMENT

Cohen and coworkers335 looked at the cumula-tive operative procedures in patients aged 14 yearsand older who were born with CLP. Lip and palaterepair was carried out in all patients. The averagenumber of operations per patient was 6.12 for uni-lateral CLP and 8.04 for bilateral CLP. Lip adhe-sions were done in 29% and 62%, pharyngoplastiesin 39% and 38%, alveolar bone grafts in 82% and

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79%, Abbe flaps in 0 and 10%, and orthognathicsurgery in 10.5% and 13.8%, respectively.

Mackay and others336 reviewed the medical chartsof 374 cleft lip/palate patients treated at the LancasterCleft Palate Clinic between 1965 and 1977, withparticular attention to the number of operative pro-cedures needed to obtain the best possible result.This information was to be disseminated to insur-ance carriers and politicians in hopes of improvingclaim reimbursement. An average of 3.3 reconstruc-tive procedures and 1.2 otolaryngological procedureswere done per patient. The 51 patients with incom-plete unilateral cleft lip had a mean 1.5 reconstruc-tive procedures, 29% secondary lip revisions, and

10% rhinoplasties. The 19 patients with completeunilateral cleft lip had 2.5 reconstructive procedures,37% secondary lip revisions, and 47% rhinoplasties.Of the 110 patients with complete unilateral cleft lipand palate, each had a mean 3.9 reconstructive pro-cedures, 36% secondary lip repairs, and 45% rhino-plasties. Two-stage palate repair was done in 72%,alveolar bone grafts in 37%, pharyngeal flaps in 42%,16% had fistula closure, and 8% had orthognathicsurgery. The 51 children with bilateral cleft lip andpalate had a mean 6.8 reconstructive procedures,including 84% secondary lip revision, 73% rhino-plasties, 84% two-stage palate repair, 61% alveolarbone grafting, 57% pharyngeal flap transfers, 18%fistula closure, and 14% orthognathic surgery.

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119. Choudhary S, Cadier MAM, Shinn DL, et al: Effect of Veau-Wardill-Kilner type of cleft palate repair on long-termmidfacial growth. Plast Reconstr Surg 111:576, 2003.

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524. Hogan VM: The tilted tripod: A theory of unilateral cleftlip nasal deformity. In: Hueston JT (ed), Transactions ofthe Fifth International Congress of Plastic and Recon-structive Surgery, Melbourne, February 1971. Australia,Butterworths, 1971, p 382.

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