Clinical Paper

Congenital Craniofacial Anomalies

Int. J. Oral Maxillofac. Surg. 2011; 40: 577–583doi:10.1016/j.ijom.2011.02.005, available online at http://www.sciencedirect.com

Surgical management ofvascular lesions of the head andneck: a review of 115 casesS. C. Nair, N. J. Spencer, K. P. Nayak, K. Balasubramaniam: Surgical managementof vascular lesions of the head and neck: a review of 115 cases. Int. J. OralMaxillofac. Surg. 2011; 40: 577–583. # 2011 International Association of Oral andMaxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Abstract. Vascular anomalies are amongst the most common congenitalabnormalities observed in infants and children. Their occurrence in the head andneck region is a source of functional and aesthetic compromise. This article reviewsthe surgical management of 115 cases of vascular anomalies involving the head andneck area treated by the authors between 1998 and 2009. It discusses the diagnosticaids, treatment protocol and the results obtained. A new classification based on theanatomical location and depth of the lesion has been proposed. This allowsguidelines for surgical ablation of the vascular lesions. The complicationsencountered are discussed. The use of external carotid artery control as opposed topre-surgical embolization has proved effective and the technique is described. Thelocation and extent of a vascular malformation should dictate the preoperativeinvestigations, surgical procedure and subsequent outcome.

0901-5027/060577 + 07 $36.00/0 # 2011 International Association of Oral and Maxillofacial Surge

S. C. Nair1, N. J. Spencer2,K. P. Nayak3, K. Balasubramaniam3

1Maxillofacial Surgery Department,Bangalore Institute of Dental Sciences,Bangalore, India; 2University of Cincinnatti,USA; 3B.M. Jain Hospital, India

Keywords: surgery; head; neck; lesions.

Accepted for publication 3 February 2011Available online 22 March 2011

Vascular anomalies are a group of lesionsderived from blood vessels and lymphaticswith widely varying histology and clinicalbehaviour. They constitute the most com-mon congenital abnormalities in infantsand children. James Wardrop, a Londonsurgeon, first recognized the differencesbetween true hemangiomas and the lesscommon vascular malformations in181814. Despite Dr. Wardrop’s work,descriptive identifiers such as Strawberryhemangioma and salmon patch continuedto be used until the 1980s. This terminol-ogy did not correlate with the biologicalbehaviour or histology of these lesions. In1982, Mulliken and Glowacki greatlyadvanced the field by introducing a

biological classification which differen-tiated vascular lesions into two distinctentities: hemangiomas and vascular mal-formations13,14. The term hemangiomanow describes a lesion that is neoplastic,demonstrating endothelial hyperplasia.Vascular malformations, conversely, donot demonstrate cellular hyperplasia butdisplay progressive ectasia of abnormalvessels lined by flat endothelial on a thinbasal lamina. A more practical classifica-tion integrating their biological behaviourwith dynamics of flow was later advanced(Table 1)7.

The diagnosis of this group of lesionsprimarily depends on the history of thelesion and the clinical presentation. Radio-

graphic evaluation may be helpful indetermining the exact extent, locationand flow dynamics of some lesions.

Patients and methods

One hundred and fifteen patients treatedby the authors between 1999 and 2009were reviewed retrospectively. Relevantdata including gender, age, age at presen-tation of symptoms, anatomical site oflesion, relevant radiographic investiga-tions and period of follow up were tabu-lated. Exclusion criteria includedsegmental lesions and those associatedwith syndromes such as Sturge-Weber.

ons. Published by Elsevier Ltd. All rights reserved.

578 Nair et al.

Table 1. Existing classification of hemangiomas and vascular malformations.

A. HemangiomasSuperficial (capillary hemangioma)Deep (cavernous hemangioma)Compound (capillary cavernous hemangioma)

B. Vascular malformationsSimple lesions

Low-flow lesionsCapillary malformations (capillary hemangioma, port-wine stain)Venous malformation (cavernous hemangioma)Lymphatic malformation (lymphangioma, cystic hygroma)

High-flow lesionsArterial malformation

Combined lesionsArteriovenous malformationsLymphovenous malformationsOther combinations

Table 2. Categorization of vascular malfor-mation based on anatomical presentation.

Type I – Mucosal/cutaneous (Fig. 2)Type II – Submucosal/subcutaneous (Fig. 3)Type III – Glandular (Fig. 4)Type IV – Intraosseous (Fig. 5)Type V – Deep visceral (Fig. 6)

All patients underwent surgery as theprincipal modality of treatment. Com-puted tomography (CT) with contrast,magnetic resonance imaging (MRI) andangiography were used based on the ana-tomical location and flow dynamics of thelesion. Selective control of the externalcarotid artery to reduce blood flow into thelesion was used effectively by the authorin lieu of routine preoperative emboliza-tion.

Technique for external carotid control

The external carotid artery (ECA) of theinvolved side is exposed through a cervi-cal incision, which often forms part of theaccess for removal of the malformation(Fig. 1). The sternocleidomastoid muscleis retracted posteriorly at the level of thegreater cornu of the hyoid bone, exposingthe carotid sheath. The external carotid[()TD$FIG]

Fig. 1. ECA snared with vascular sling.

distal to the carotid bifurcation is identi-fied. The vessel is snared with a vascularsling passed through a red rubber catheter.Gentle strangulation of the vessel can beaccomplished by advancing the catheter.This additional compression of the vesselserves to reduce blood flow to the lesion.The lesion is exposed with great care takennot to disturb the vascular network. Feed-ing arteries and draining vessels are iden-tified and ligated, permitting total excisionof the lesion. The wound is closed primar-ily with vacuum drains in situ. The mal-formations were categorized into fivetypes depending on their anatomy anddepth of location in the head and neckregion (Table 2). In type I superficiallesions requiring excision of skin ormucosa, local or regional flaps have beenused in defect reconstruction (Fig. 2).Type II submucosal lesions require com-plete excision after elevation of skin flaps

(Fig. 3). Type III lymphovenous malfor-mations or venous malformations invol-ving salivary glands are excised alongwith the affected gland (Fig. 4). TypeIV intraosseous lesions require excisionwith involved bone and reconstructionwhen required (Fig. 5). Type V lesionsinvolving deep visceral spaces, such as theparapharyngeal or infra-temporal fossa,require mandibular access osteotomy forcomplete exposure and total excision(Fig. 6). The above classification helpedin determining the surgical approach andreconstruction necessary for the type ofvascular lesion.

Results

Of the 115 patients evaluated, 63 weremale and 52 female. The youngest patientwas a 2-year-old girl with a lymphaticmalformation in the parotid region (typeIII) and the oldest was a 58-year-old malewith a venous malformation involving theentire tongue and submandibular region(type II). Table 3 shows the patients cate-gorized into types with gender distribu-tion. 38 patients with type I, 44 patientswith type II, 12 patients with type III, 11patients with type IV and 10 patients withtype V anomalies were treated success-fully by surgical ablation of their vascularlesions. Four patients with type I lesionsrequired reconstruction with local orregional flaps and 2 patients with typeIV lesions required reconstruction ofresected mandible. Only 88 patients couldprovide an approximate time of appear-ance of the lesion. In 27 patients the lesionhad been noticed at birth or soon after. Theremaining 61 patients were clinicallyaware of it shortly before their first surgi-cal visit. Table 4 highlights the differentimaging techniques used according to the

Table 3. Patients and age according to thetypes of the various vascular lesions.

Type Age (years) Female Male

I 7–44 (24.705)a 15 23II 3–52 (23.27)a 25 19III 2–43 (26.2)a 7 5IV 8–49 (22.8)a 3 8V 18–56 (32.8)a 4 6

a Average age.

Surgery for vascular head and neck lesions 579[()TD$FIG]

Fig. 2. Type I low flow cutaneous venous malformation.

[()TD$FIG]

Fig. 3. Type II low flow vascular malformation in the buccal region.

Table 4. Imaging techniques used in the different types of vascular lesion.

Type In = 38

Type IIn = 44

Type IIIn = 12

Type IVn = 11

Type Vn = 10

CTC 32 34 7 2 6MRI 0 1 1 0 0Angiogram 0 5 4 8 4No investigation 6 4 0 1 0

type of malformation. At the authors’centre CT scanning with contrast is themost frequently used imaging modality.Table 5 demonstrates the method of hae-morrhage control used for the malforma-tion. Pre-surgical embolization wasrestricted to two patients and externalcarotid artery control was required in 52patients. Complications encountered arelisted in Table 6. One hundred and elevenpatients gained an acceptable aestheticoutcome with a single procedure. Table7 summarizes the surgical plan employedfor each type of lesion and the reconstruc-tion used when required.

Discussion

The first public demonstration of etheranaesthesia by William Green Morton in1846 was for surgical removal of a venousvascular malformation14. Numerousattempts to understand, classify and treatthese lesions have met with unpredictableoutcomes. The classification proposed byMulliken and Glowacki differentiated thisgroup of lesions into the biologically activehemangiomas and inactive vascular mal-formations. Classification led to improvedunderstanding of the behaviour of theselesions. Timing of treatment could be basedon a scientific understanding of the lesion’sbiological behaviour rather than clinicalappearance or the surgeon’s sense ofgestalt14. Subsequently, Mulliken andKaban introduced the flow dynamics ofvascular lesions, describing hi-flow andlow-flow vascular malformations10. Morerecently, a practical classification (Table 1)has helped to consolidate all previous clas-sification7. The authors have categorizedvascular lesions requiring surgery into fivetypes. This simplified categorization pro-vides input into the investigation and effec-tive surgical management of variouslesions based on anatomical presentation.

Diagnosis of vascular malformationsdepends on precise identification, accuratehistory, physical examination and theproper use of imaging. Advances in ima-ging have led to the unnecessary exposureof many lesions. Grey scale ultrasoundand Doppler analysis are useful in definingwhether the lesion is solid or cystic and inestablishing the flow dynamics of alesion17. In evaluating vascular malforma-tions, MRI has a major advantage over CTor angiography in differentiating heman-giomas from the surrounding structures,but its cost and limited availability can beprohibitive to its use. In the authors’experience, imaging is restricted to CTwith contrast for most lesions for costreasons. MRI is restricted to 2 patients

580 Nair et al.[()TD$FIG]

Fig. 4. (a) Type III lymphovenous malformation in left parotid gland. (b) Exposure of lesionthrough preauricular incision with cervical extension. (c) Excised specimen showing cysticspaces. (d) 2-Month postoperative appearance after total excision of lesion with gland.

[()TD$FIG]

Fig. 5. Type IV intra bony hi-flow arterial malformation in maxilla.

and angiography to 18 patients (Table 4).Angiography, particularly digital subtrac-tion angiography (Fig. 7), has a specific butlimited role in the diagnosis of vascularlesions. It is restricted to lesions requiringtherapeutic endovascular intervention7.

Selective embolization as a single treat-ment modality is rarely successful withhigh flow anomalies because of rapid estab-lishment of new pathways of flow. Ligationof main feeder vessels is also forbidden dueto low success rates and its elimination ofaccess for future embolization3,5,12,16.

The use of temporary control (ligation)of the ECA instead of presurgical embo-lization has proven effective in reductionof blood flow to the lesion, allowing effec-tive excision with minimal blood loss.Where blood replacement is required,autologous transfusion is preferred. Whenembolization is chosen subsequent to digi-tal subtraction angiograph (DSA) it shouldproceed from distal to proximal thus ablat-ing both the nidus and its source18. Choiceof embolic agents is purely the clinician’spreference. Gelfoam, polyvinyl alcohol,silicone fluid and isobutyl-2 cyanoacrylateare commonly used agents7. When embo-lization is used, surgery is carried outwithin 24–48 h to prevent the develop-ment of collateral blood supply1,4,6,9,11.The use of presurgical embolization wasrestricted to two patients with type V(deep visceral) lesions, both of whichrequired ECA control intraoperativelydespite embolization. One of thesepatients presented for surgical manage-ment after undergoing an emergent embo-lization. The second presented with bothECAs feeding into the lesion; one wasembolized and the other controlled withtemporary intraoperative ligation.

Sclerotherapy has a promising but lim-ited role in the management of vascularlesions. Success has been realized in thetreatment of macrocystic lesions. The ther-apy has been less effective in treatingmicrocystic vascular malformations2,8,15.The different agents used include sodiumtetradecyl sulphate (3%), sodium tetradecylacetate and more recently OK 432 (lyophi-lized Streptococcus pyogenes treated withbenzyl penicillin)7.

Surgery has been used effectively toeradicate or minimize the lesion in thisreview of 115 cases. Surgery must beaimed at removal of the entire nidus alongwith any structure associated with thelesion because any remaining vasculaturewill probably lead to recurrence. The pro-posed classification (Table 2) was used tohelp plan the approach and extent ofresection. Superficial lesions requiredexcision of skin or mucosa with recon-

Surgery for vascular head and neck lesions 581[()TD$FIG]

Fig. 6. Type V MRI showing venous malformation in lateral and post-pharyngeal space.

Table 5. Number of patients who had ECA control as against pre-surgical embolization.

Type In = 38

Type IIn = 44

Type IIIn = 12

Type IVn = 11

Type Vn = 10

ECA control 1 32 11 11 10Non-ECA control 37 12 1 Nil NilEmbolization Nil Nil Nil 1a 1a

a Had ECA control along with presurgical embolization.

struction using local or regional flaps.Lesions involving the parotid or subman-dibular gland require excision of the glandwith preservation of nerves. Deeper

Table 6. Complications encountered in the diffe

SL Classification Age Sex

1 Type IV 8 M Lt maxill2 Type II 23 F Rt cheek3 Type IV 8 M Lt maxill

4 Type I 19 F Lt upper5 Type II 31 F Rt cheek6 Type I 25 F Tongue7 Type I 22 F Lower lip8 Type II 21 F Lt lower9 Type V 18 F Lt infra t

10 Type V 23 M Lt tempo

Table 7. Surgical treatment advocated for the t

Classification

Type I – Mucosal/cutaneous lesion arising frompapillary dermis involving skin or mucosa (n

Type II – Submucosal or subcutaneous with noof overlying skin (n = 44)

Type III – Lesions involving glands ex-parotid/submandibular (n = 12)

Type IV – Skeletal – involving the facial skeletex-maxilla/mandible/zygoma (n = 11)

Type V – Deep visceral ex-parapharyngeal/infratemporal (n = 10)

lesions necessitate access osteotomiesfor excision. Lesions within bone, under-went bone resection followed with recon-struction using autologous grafts. In one

rent types of vascular anomalies with the site a

Site Investigation

a Angio ExcAngio Inco

a Angio with presurgicalembolization

Rec

lip CTC OveCTC TemCTC ResiCTC Inco

eyelid CTC Tememporal fossa Angio with presurgical

embolizationVII

ral fossa CTC Intra

ypes of vascular anomalies.

Male Female

= 38)23 15 Excision with ove

Primary closure odiscoloration 19 25 Surgical access to

closure5 7 Surgical access to

with the involvedon 8 3 Excision of invol

6 4 Mandibulotomy t

patient with arteriovenous malformation(AVM) in the mandible, successful repla-cement of the resected mandible afterenucleation of the pathology was per-formed. Skeletal deformities secondaryto lymphangiomas were common andrequired secondary correction of the ske-letal deformity. Table 7 demonstrates theauthors’ surgical approach to vascularmalformations based on anatomical pre-sentation. The complications wererestricted to morbidity with no mortality.The most common problem encounteredwas incomplete excision requiring anotheroperation at a later date. Temporary par-esis of branches of facial nerve and exces-sive intraoperative haemorrhage were alsoseen. Excessive haemorrhage was definedas blood loss requiring more than auto-logous transfusion. The overall satisfac-tion quotient was high.

In conclusion, the use of intraoperativecontrol of branches of the external carotidartery has proved a successful, safe andeffective method of intraoperative hae-morrhage control when removing thesepotentially bloody lesions. The approachis easy to incorporate into the accessnecessary to remove the lesion. Anincrease in morbidity by this approachwas not seen compared with lesions trea-ted with preoperative embolization. Thepresent accepted classification (Table 1)attempts to correlate the biological classi-fication by Mulliken and Glowacki withthe flow dynamics of the lesion. Whilst

nd prescribed imaging modality.

Complication

essive intraoperative haemorrhagemplete excisionurrence in mandible

rexcision with hypoplastic apearanceporary neuroparesis VII nervedual lesion cheekmplete excision and scarringporary ectropionnerve weakness

operative haemorrhage from cavernous sinus

Treatment

rlying skin or mucosar regional flaplesion with total excision and primary

glandular lesions with excision alonggland and primary closure (Fig. 4A–D)

ved skeletal structure with reconstruction

o access the lesion followed by total excision

582 Nair et al.[()TD$FIG]

Fig. 7. Digital subtraction angiography of submandibular hi-flow AVM.

this is helpful in understanding the lesion’sbehaviour, a further categorization oflesions that require operative interventionbased on the technique needed for surgicaltreatment would be helpful to the managingsurgeon. The authors describe a simplifiedalgorithm for effective management of vas-cular lesions requiring surgery (Table 7).For example, hemangiomas are treatedwith a wait and watch policy since theyfrequently undergo resolution, but vascularmalformations causing functional or aes-thetic deformity are dealt with at the earliestopportunity. Proper management dependsnot only on the biological behaviour, butalso on site of anatomical presentation.Presentation of a lesion not only as a venousmalformation, but as a type V venous mal-formation gives the surgeon the additionalinformation needed to plan treatment prop-erly. Adequate imaging techniques are thekey to the successful diagnosis and effec-tive treatment of all vascular anomalies.Angiography should be restricted to

anomalies requiring endovascular interven-tion and lesions that may have feeders fromthe internal carotid artery. MRI with fatsuppressed images is most effective. Theuse of alternative therapy, such as emboli-zation and sclerotherapy, has an effectivebut limited role in treating vascular lesions.The use of clinical data with non-invasiveimaging techniques, followed by precisesurgery has been successful in providingsatisfactory treatment in the majority ofpatients. Segmental and large compositelesions require multiple therapies. Eradica-tion is unlikely with either surgery alone orcombination therapies.

Funding

None.

Competing interests

None declared.

Ethical approval

Retrospective case review – ethical clear-ance not required.

Acknowledgements. Prof. Paul Stoelinga,Nijmegen, Netherlands and Dr. DeepakGopalakrishnan, University of Cincin-natti, USA are acknowledged for theirsupport in preparing this manuscript.

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Address:Sanjiv C. NairMaxillofacial SurgeryBangalore Institute of Dental SciencesWilson GardenBangalore 560 029IndiaTel.: +91 98454 33106E-mail: snmaxfax@gmail.com