cleft lip and palate
DESCRIPTION
Presentation was given by me to the house of Oral and Maxillofacial Surgery at Pakistan Institute of Medical Sciences, Islamabad in August 2011.TRANSCRIPT
By: Dr. Waqar Jeelani
Layout Introduction Incidence Classification Embryological Background Etiology Diagnosis Problems of individuals with clefts Treatment of Cleft Lip and Palate Specialties involved in the treatment of Cleft Lip and
Palate Timings of surgical repair Cheilorraphy Palatorrhaphy Alveolar Cleft Grafts Secondary Surgical Procedures Valopharyngeal Insufficiency
IntroductionWhat is Cleft Lip and Palate?Congenital abnormal space or gap in the
upper lip, alveolus and palate
IncidenceMore common in south far Asians:
1 in 500Less frequent in Africans:
1 in 2000Prevalence in Europeans and Americans:
1 in 750*Prevalence in Pakistan:
1 in 523
*Elahi MM, Jackson IT, Elahi O, Khan AH, Mubarak F, Tariq GB, Mitra A. Epidemiology of cleft lip and cleft palate in Pakistan. Otolaryngol Clin North Am. 2007 Feb; 40(1):27-60
IncidenceBoys are more affected than girls by 3:2Cleft Lip and Palate occur twice as often in
boys as in girlsIsolated Clefts of Palate are more often in
girls75% of Clefts are Unilateral, rest are
BilateralLeft side is more involved than right side
Head and Neck of 4-Week Old Embryo
Trigeminal nerve
Facial nerve
Glossopharyngealnerve
Vagus nerve
Embryological Background
Embryological BackgroundDevelopment of the
Lip:Unpaired Frontonasal
ProminenceMedial and Lateral
Nasal prominences 2 maxillary
prominences2 mandibular
prominences
Embryological BackgroundFusion defects can occur anywhere
between these prominencesThe defect in the fusion between the
frontonasal and maxillary will lead to cleft lip
Embryological BackgroundDevelopment of Palate: We have two parts of two different embryonic origins: 1 ) primary palate : the triangular part of hard palate anterior to
incisor foramen which originate from the premaxilla ( frontonasal prominences).
develop between 4develop between 4thth and 8 and 8thth week of gestation week of gestation 2 ) secondary palate : remaining part of the hard palate and all soft
palate posterior to incisor foramen which comes from palatine shelves of the maxillary prominences
develop between 8develop between 8thth and 12 and 12thth week of gestation week of gestation
Embryological Background Various theories have been given for its
development. 1. Alteration in intrinsic palatal shelf
force 2. Failure of tongue to drop down 3. Non fusion of shelves4. Rupture of cyst formed at the site of
fusion
ClassificationWe classify as the follows: its combined (cl+cp) or isolated cleft(cl
or cp)?
is it unilateral or bilateral?
is it complete (if it cross the nasal philtrum) or incomplete ( if it doesn’t cross the nasal philtrum.
Classification Systems
Karnahan’s Classification Millard’s Modification of Karnahan’s Classifcation
Lip
Alveolus
Primary PalateHard Palate (Maxillary)Hard Palate (Palatine)Soft Palate
Kriens “LAHSHAL”Kriens “LAHSHAL”L = Lip (right)A = Alveolus (right)H = Hard Palate (right)S = Soft Palate (median)H = Hard Palate (left)A = Alveolus (left)L = Lip (left)
Capital letter = complete cleftLowercase letter = incomplete cleft“.” or “-” = normal
ExamplesLA….l = complete right cleft lip and alveolus, incomplete left cleft lipLAHS = complete right unilateral cleft lip, alveolus, hard, and soft palate
Other Types of CleftsMicroform Cleft:
May look like a little dent in the red part of the lip a scar from the lip up to the nostril.
Muscle tissue underneath the cleft can be affected and may require surgery
Submucous Cleft Palate:Midline deficiency or lack of
muscular tissueOften a submucous cleft palate is
associated with a bifid or cleft uvula
Posterior nasal spine is almost always missing
Speech Problems are common
Prenatal DiagnosisCleft lip can be easily
diagnosed by performing ultrasonography in the second trimester
Diagnosing a cleft palate with ultrasonography is very difficult
Three-dimensional imaging has been introduced to prenatal ultrasonography diagnostics of cleft anomalies
Diagnosis Advantages of Prenatal
Diagnosis:1. Time for parental education2. Time for parental
psychological preparation3. Opportunity to investigate
other associated anomalies4. Gives parents the choice of
continuing the pregnancy5. Opportunity for fetal
surgery
Etiology“Actually no one knows exactly what causes
clefts”Multiple factors may be involved, like:
Genetics (inherited characteristic) from one or both parents .
Environmental factorsDrugs: corticosteroids (anti-inflammatory),
phenytoin (anticonvulsant), retinoid.Infections: like rubella during pregnancy. Alcohol consumption, smoking, hypoxia during
pregnancy, some of dietary and vitamins deficiencies (like folic acid and vitamin A deficiency)
Maternal Age
GENETICSCL/P Normal parents, one child with CL/P 4% risk CL/P in next child Normal parents, two kids with CL/P 9% risk CL/P in next child One parent CL/P, no affected kids 4% risk CL/P in next child One parent CL/P, one child CL/P 17% risk CL/P in next
child Risk of CL/P in siblings increases with severity of deformity - child with unilateral CL risk CL/P next child 2.5% - child with bilateral CL/P risk CL/P next child 5.7%CP Normal parents, one child with CP 2% risk of CP in next child Normal parents, 2 children with CP 7% risk of CP in next child Parent with CP, no affected children 6% risk for next child Parent with CP, one child with CP 15% risk for next child
• All infants with clefs must be evaluated for presence of other anomalies• Associated nomalies are more common in infants with isolated CP
Nonsyndromic CleftNonsyndromic Cleft
Pierre Robin Sequence is the most common associated nonsyndromic anomaly
is a relative term describing the small size of the lower jaw ) and Glossoptosis (is a medical condition and abnormality which refers to the downward displacement or retraction of the tongue)
Syndromic CleftSyndromic Cleft
Common Syndromes Stickler (25% of syndromic CP) – autosomal dominant type 2 collagen gene Pierre Robin, ocular/hearing/joint
malformations
Velocardiofacial (Shprintzen’s) – 15% of syndromic CP
Autosomal dominant, variable expressivity, CATCH 22 – Deletions at 22q11,
Facial, cardiovascular, immunologic, developmental anomalies
Van der Woude’s (19% of syndromic CL/P and CP)
Autosomal dominant CL/P or CP with bilateral lower lip pits
Problems Associated With Cleft Lip and Palate
FeedingDental problemsNasal Deformity and Esthetic ProblemsEar ProblemsSpeech DifficultiesAssociated Anomalies
Feeding DifficultiesCleft lip= makes it more difficult for an
infant to suck on a nippleCleft Palate= may cause formula or breast
milk to be accidently taken up into the nasal cavity
Inability to create negative pressure inside oral cavity
Frequent regurgitationsUpper respiratory tract infections
Dental ProblemsLocal Dental Problems:
Congenitally Missing teeth, Hypodontia, Hyperdontia, Oligodontia
Presence of natal and neonatal teethAnamalies of tooth morphology like microdontia,
macrodontia etcFused teethEnamel HypoplasiaPoor periodontal support, early loss of teethGemination, Dilacerations
Orthodontics Problems:Class III tendencyAnterior and Posterior Cross biteSpacing and crowding
Nasal Deformity and Esthetic ProblemsFacial DisfigurementsPoor nasal shapeScar marks of surgeriesPoor lip function during
speechPoor dental alignment and
smile
Ear ProblemsMiddle ear disease - 22% to 88% Conductive hearing loss and chronic
suppurative otitis media may result Repeated tympanostomy tube placement
Why do cleft kids have eustachian tube dysfunction? Abnormal curvature of the eustachian tube
lumenCephalometric data - width and angulation of
the skull base with respect to the eustachian tube are different
Abnormal insertions of the tensor and levator veli palatini muscles into the cartilages and skull base
Palatal muscle dysfunction
Speech Problems:Hearing loss hampers proper development of
speechVelopharyngeal Insufficiency (VPI)Abnormal airPoor pronunciation of Bilabial, Labiodental,
Linguoalveolar sounds
Schedule of TreatmentBirth:
Initial Assessment Pre-surgical assessment
3 Month: Primary Lip repair
9-18 month: Palate Repair
2 Year: Speech assessment
3-5 Year: Lip Revision Surgery
8-9 Year: Initial interventional
Orthodontics Preparation for alveolar
bone grafting
10 Year: Alveolar Bone Grafts
12-14 Year: Definite Orthodontics
16 Year: Nasal Revision Surgery
17-20 Year: Orthognathic Surgery
Multidisciplinary Cleft Lip And Palate TeamGenetic ScientistPediatricianPedodontistOrthodontistOral and Maxillofacial SurgeonProsthodontistENT SurgeonPlastic SurgeonPsychiatristSpeech TherapistSocial Worker
FeedingCleft lip= makes it more difficult for an infant to suck on
a nippleuse special nipples to allow the baby to latch
properly (either pump or use formula)
Cleft Palate= may cause formula or breast milk to be accidentally taken up into the nasal cavity don’t feed baby without palatal obturator
(prosthetic palate) feed in an upright position to keep milk from
coming out of the nose
Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder / Haberman Feeder
Pigeon Feeder Dr. Brown’s Natural Flow to relieve gas
Presurgical Orthopeadics:1. Reduces the size of cleft; Aids in Surgery2. Partial obturation aids in feeding3. Parental Reassurance at a crucial time
Maxillary StrappingNasoalveolar Moulding Appliances (NAM)
Require orthopedic repositioning of the nasal cartilages, columella, nasal tip, and lateral wall of the vestibule
Presurgical infant nasal remodelling nasal molding
Nasal molding by post surgical nasal stenting
Facial taping 2 to 3 months
BILATERAL CLEFT LIPThe most challenging
condition1. The premaxilla is
extremely protrusive
2. The premaxilla and prolabium can be of variable size
3. The columella is deficient/almost nonexistent
4. The palatal shelves are collapsed
Protrusive maxilla imperative to be
repositioned
Premaxillary orthopedics with inraoral aplliance
Denture adhesive
Elastic strap
Latham Appliance
Rule of Ten Primary repair- repaired at approximately 10
weeks The surgeon usually uses the “Rule of Ten”The child weighs 10 pounds The child has a hemoglobin of at least 10
grams The child has a white count of no higher than
10,000 The child is at least 10 weeks of age
Surgical TechniquesCleft Lip Repair
unilateral rotation-
advancement flap developed by Millard
complicationsdehiscence
infection excess tension
Surgical TechniquesCleft Lip Repair
bilateral bilateral rotation
advancement with attachment to premaxilla mucosa
Cleft Palate Repair - TimingDorf and Curtin
10% occurrence of articulation errors when palatoplasty was completed by 1 year
86% incidence of articulation errors when repair was complete after 1 year
Haapanen and Rantala – Significantly fewer children in the groups
repaired before 18 months had hypernasal speech, articulation errors, or required secondary surgery to correct speech
Cleft Palate RepairSchweckendick’s Primary VeloplastyV-Y PushbackVon Langenbeck Palatal RepairFurlow Palatoplasty
Cleft Palate RepairSchweckendick’s Primary Veloplasty
Incisions made in soft palateMuscle bundles released from the posterior
hard palate and rotatedReconstruction of levator sling Closure of mucosal layers separately
Cleft Palate RepairV-Y Pushback (WARDILL OPERATION)
Two uni-pedicled flaps (greater palatine artery) and one or two anteriorly based pedicled flaps
Posterior flaps rotated in a V-Y advancement technique - increasing the length of the palate
Nasal mucosa not closedImproved speech results compared with bipedicled
techniques Indicated for incomplete clefts
Von Langenbeck Operation:
Cleft Palate RepairFurlow Palatoplasty
Lengthens the soft palateReconstructs the muscle sling. Also commonly used to correct velopharyngeal
insufficiency in patients with submucous cleft palateSpeech outcomes are improved compared with other
palatoplasty techniques.
How do you manage ear disease? 96% of children with cleft
palate required tympanostomy tube placement
50% of these children required repeat tympanostomy tube placement.
Frequency of otitis media decreases as the child with CP ages
Audiology and tympanometry as well as exams / clinical history
Orthodontic Treatment of Transitional Dentition The purpose the dentition adjacent to
cleft has to be orthodontically repositioned to prepare the cleft side for the secondary alveolar bone graft
Preparing the maxillary arch for a bone graft (6-12 months) :
1. Bonded edgewise appliance2. Supported with a maxillary expander quad
helix expander
Alveolar Bone GraftingPrimary Bone Grafting
Bone graft done at the time of primary cheiloplasty
Bone graft done during the first 2 years of lifeBone graft done prior to the eruption of the
primary canine
Secondary Bone GraftingDone before eruption of the permanent
canineUsually when the root of the canine is 1/3 to
2/3 formedUsually between ages 8-10In CLP dental age is usually behind
chronological age
Quadhelix to expand prior to ABG
angle brackets to keep roots away from cleft
Alveolar Bone Grafting1. Provide bone for the eruption and/or
orthodontic repositioning of teeth2. Closure of oro-nasal fistulas3. Support and elevation of the alar base4. Stabilization of the pre-maxilla in bilateral
cases5. Provide continuity of the alveolar ridge
Types of bone grafts Autogenous
Cancellous- iliac crest Cortical- calvarium, mandible Cortico-cancellous- iliac, rib, tibia, mandible
*Allogeneic Graft resorbs, remodels, may contribute to osteoinduction and
osteoconduction **Alloplast
Bone grows into, around alloplast No active osteoinduction but some osteoconduction Teeth do not erupt through alloplast
*Nique T, Fonseca RJ, et al: Particulate allogeneic bone grafts into maxillary alveolar clefts in humans- A preliminary report. J Oral Maxillofac Surg 45: 386-392, 1987
**Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate model. J Oral Maxiilofac Surg 47:946-952, 1989
Alveolar Bone Grafting
Alveolar Bone Grafting
Preoperative Cleft Defect Postoperative Bone Graft
•Correction of anterior crossbite•Arch expansion
Quad HelixExpansion screws
Orthodontic Treatment For Permanent Dentition
Orthognathic SurgeryMidfacial
AdvancementLeForte osteotomies
leave vascular pedicle attached in back of maxilla - prevents necrosis
RhinoplastyRhinoplasty
standard techniques tip projection alar rotation columellar length
Age =17-20
Surgical Management of Velopharyngeal InsufficiencyMajor Goals of Surgery• Close the gap or hole between the roof of
the mouth and the nose. • Reconnect the muscles that normally make
the palate work. • Make the repaired palate long enough so
that when the muscles are working, the palate can perform its function properly.
Velopharyngeal Insufficiency VPI
Wardill Operation: WY push back
Dorrance and Brown’s – U shaped push back palatoplasty
Prosthetic Management of VPI
Thank You!