forty-one cervicofacial vascular anomalies and their ...annals.edu.sg › pdf › 37volno3mar2008...

March 2008, Vol. 37 No. 3

165Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang

Forty-one Cervicofacial Vascular Anomalies and Their Surgical Treatment –Retrospection and ReviewGavin CW Kang,1MBBS, Colin Song,1FRCS (Edin), FAMS

IntroductionVascular anomalies of the head and neck can be

categorised as either haemangiomas or vascularmalformations.

Haemangiomas are common in young children (hencethe term “common infantile haemangioma”) and aconservative wait-and-see treatment is usually favoured

because most lesions regress spontaneously.1

Haemangiomas characteristically present shortly after or,in rare cases, at birth with a rapid proliferative phase overthe first 12 months. They then stabilise and slowly involute,with regression complete in 50% of cases by age 5 yearsand in 70% by age 7 years.1 Histologically, there isendothelial hyperplasia and increased number of mast cells

1 Department of Plastic, Reconstructive, and Aesthetic Surgery, Singapore General Hospital, Outram Road, Singapore 169608Address for Reprints: Dr Colin Song, Department of Plastic, Reconstructive, and Aesthetic Surgery, Singapore General Hospital, Outram Road,Singapore 169608.

AbstractIntroduction: Haemangiomas in children usually involute spontaneously and surgical treatment

is exceptional. Vascular malformations do not regress spontaneously and resection may becomenecessary. We present a series of surgically treated face and neck vascular anomalies during a9-year period, assessing the epidemiology, presenting signs and symptoms, diagnostic modalities,indications for surgery, treatment methods and clinical outcome post-treatment. Materials andMethods: The medical and pathological records of all patients with cervicofacial vascularanomalies treated surgically at our department from 1997 to 2005 were retrospectively reviewedin relation to current evidence. Results: Forty-one patients were identified. Of these, 9 patientshad haemangiomas and the remaining 32 had a variety of vascular malformations. Cervicofacialvascular anomalies were most commonly located at the lip. Atypical looking vascular anomalieslike masseteric intramuscular haemangiomas and parotid malformations were diagnostic problems.All 41 had surgical excision of their vascular anomalies for troubling symptoms, cosmesis ordiagnostic purpose. For cervicofacial arteriovenous malformations, 28% were classified asSchobinger stage I, 50% stage II, and the remainder stage III. Combined embolisation-resectionwas used to treat 6 arteriovenous malformations (stage II to III) and of these, 3 required flapreconstruction. Conclusions: Accurate diagnosis distinguishing between cervicofacialhaemangiomas and vascular malformations is key to best treatment. The diagnosis can usuallybe made by history and physical examination aided by early magnetic resonance imaging (MRI).Although cervicofacial haemangiomas can be managed conservatively or with medical therapy,surgery is indicated for preventing psychological distress and in cases of chronic aestheticalteration resulting from partial regression. Aesthetic concerns and prevention of psychosocialdistress point to early excision of venous malformation as the treatment of choice. Lymphaticmalformations are best treated by excision. Outcome after excision of localised cervicofacialhaemangiomas and low-flow vascular malformations is excellent. Large extensive low-flowmalformations as well as those located at the lips may require multiple procedures includingreconstruction; patients should be informed that the outcome is generally not as good. Combinedembolisation-resection is definitive treatment for arteriovenous malformations and flap recon-struction may prevent their recurrence. Tissue expansion is a useful reconstructive tool after theexcision of large vascular anomalies.

Ann Acad Med Singapore 2008;37:165-79

Key words: Face and neck, Haemangioma, Single institution outcome, Vascular malformation

Original Article

166

Annals Academy of Medicine

Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang

during the proliferative phase;2 the involutive phase ismarked by fibrofatty replacement, diminished cellularityand a normal mast cell count.

Surgery is indicated in situations of chronic unaestheticalteration, psychosocial trauma, bleeding, ulceration, painand functional compromise. Large periorbitalhaemangiomas occluding vision and predisposing to ocularcomplications, head and neck haemangiomas compressingairway or vital structures and labial tumours causing feedingdifficulty – all are documented instances of functionalcompromise with cervicofacial haemangiomas.3-5

Vascular malformations, while always present at birthand growing commensurately with body growth, mostcommonly present in adulthood and do not resolvespontaneously.4 They are not tumours but collections ofabnormal vessels displaying normal flat non-proliferativeendothelium and normal mast cell count. Vascularmalformations are further separated into low-flow (capillary,venous, lymphatic or combination) and high-flow (arterialcomponent, typically arteriovenous) lesions. Resection isusually necessary, especially for arteriovenousmalformations. Preoperative superselective embolisationis increasingly utilised for arteriovenous malformations.

Materials and MethodsAll patients with face and neck vascular anomalies

treated with surgical resection at our department between1997 and 2005 were retrospectively reviewed from themedical records of the Singapore General Hospital, thelargest tertiary hospital in Singapore.

Data collected included patient age at surgery, sex, race,presenting signs and symptoms, anatomical location of theanomaly, size, diagnostic modality, indication for surgery,surgery performed and other treatment modality,histopathological finding, and outcome after treatment.The vascular anomalies were categorised based onhistopathology into haemangiomas and vascularmalformations.

ResultsForty-one patients were identified. Of these, 9 had

haemangiomas and the remaining 32 had a variety ofvascular malformations. All 41 had surgical resection oftheir face or neck vascular anomalies, with or without theuse of other treatment modalities. For the sake of clarity, thepatients are discussed separately in the 2 categories.

HaemangiomaThe haemangioma series (Table 1) consisted of 3 males

and 6 females (male-to-female ratio, 1:2), ranging in agefrom 3.5 to 48 years (mean, 22.4).

Excluding Case 8 who had an extensive confluent

haemangioma affecting most of the face, haemangiomasfor the other cases in this series ranged in size from 5 x 5 mmto 30 x 30 mm and were found with equal frequency in eachlocation – lip, cheek, neck and nose.

The presenting signs and symptoms varied according tothe tumour site. Every patient had a lump or swelling, with5 patients reporting multiple symptoms. All tumours wereconspicuous where they were located. In all cases, tumourresection was indicated either for diagnostic purpose or forrelief of symptoms and signs including incompleteinvolution, chronic unaesthetic alteration, discomfort, pain,and bleeding. One patient (Case 8) presented with thepersistence of a previously treated haemangioma. Thepatient had undergone surgical excision, laser resection,and even radiotherapy.

Five (55.6%) of the haemangioma patients underwentpreoperative computed tomography (CT) or magneticresonance imaging (MRI) to facilitate diagnosis,determine extent and delineate related anatomy. One (Case2) had only Doppler ultrasound of the tumour donepreoperatively. It was considered necessary to performdiagnostic angiography with preoperative embolisationin Case 8.

With the exception of Case 8, the tumours in the othercases were completely resected surgically with no recurrenceat last follow-up (Table 1). For all vascular anomalies, thesurgical approach varied depending on the preoperativepresumptive diagnosis, location and size of the tumour, aswell as surgeon preference. Haemangiomas of the neckwere resected via a transcervical approach. The 2 cheekmasseteric haemangiomas (Cases 5 and 9) were approachedas parotid masses and each was excised through a lazy Sparotidectomy incision preserving facial nerve brancheswhere necessary. The nasal haemangiomas were bothexcised via an external rhinoplasty approach. The liplesions were elliptically excised. In Case 8, the patient hadhad multiple previous subtotal facial haemangiomaresections and a recently skin-grafted palate for a post-resection palatal defect; surgical excision was aimed atpalliation in view of her bulky epatulous upper lip andbulky hemipalate with maxillary alveolar protrusionimpinging on the dentures.

Apart from minor transient complications encounteredwith the masseteric haemangiomas and Case 8, there wereno significant postoperative problems. The follow-up periodfor this group varied from 5 months to 8 years, with a meanof 2.1 years from the very latest operation.

The results were evaluated for each patient based on thegeneral aspect of the operated area, reduction of tumourvolume, correction of functional impairment, improvementof skin texture and cosmetic appearance. The results wereclassified as very good, good or fair. This method of result



assessment was applied uniformly to all vascularanomalies in our entire series. At the latest follow-up for the haemangiomas, the results were verygood in 33%, good in 45% and fair in 22% of cases.

Vascular MalformationThe vascular malformation series comprised 18

low-flow lesions (2 lymphangiomas and 16 largelyvenous malformations) and 14 high-flow lesions(arteriovenous malformations).

Low-Flow Vascular MalformationsEighteen patients (male-to-female ratio, 1:1.6)

aged between 6 and 53 years (mean age, 28.6) weretreated for low-flow vascular malformations. Allhad a lump or swelling of some sort, with 7 (38.9%)having multiple symptoms like recent progressiveenlargement (6), pain (3) and itch (1). Eleven(64.7%) noted the lesion at birth or shortly after ata young age. Three had multiple venousmalformations distributed over varying areas ofthe face (Cases 12, 14 and 19). Excluding thepatient (Case 16) with extensive venousmalformation, the 2 lymphangiomas were primarilysited at the cheek, while the remainder were foundat the lip (5) predominantly at the upper lip, frontal(3), cheek (4), neck (3), temporal (1), medialcanthus (1) and nose (1). Excluding Case 16, thesize of the lesions ranged from 15 x 11 mm to 130x 30 mm.

Four patients presented with lesion recurrence(Cases 10, 18, 19 and 27) or persistence (Case 16)in spite of prior treatment. Lesion imaging usingMRI and/or CT was done in 7 (38.9%) patients,and angiography was performed in cases 16 and19. Diagnostic biopsy was done in Cases 23 and27.

Except Case 16, all (Table 2) low-flow vascularmalformations were surgically excised completelywith no recurrence detected at the latest follow-up.Neck lesions were excised transcervically. Thepatient in Case 19 presented with recalcitrantrecurrent facial lesions that required preoperativeembolisation and even adjunctive lip sclerotherapy.In case 16 with such a widespread venousmalformation, the patient agreed to staged excisionand flap reconstruction that was ongoing at thetime of writing. The lesion was excised in Case 22and reconstruction was done utilising tissueexpanders – this was complicated by prostheticinfection. One patient (Case 15) required asuperficial parotidectomy in order to excise a parotidmalformation. Three patients (Cases 12, 14, 19)with lip involvement and 1 patient with a cheekTa

ble

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168



lymphangioma (Case 26) underwent further minor revisionprocedures to improve their appearance after the primarysurgery. All other low-flow malformations were excisedelliptically with direct closure.

There were 2 recurrences (Cases 10 and 19) alongfollow-up post-excision; the recurrent lesions were againexcised with no further recurrence at last follow-up.Postoperative complications were few and minor, involvingmainly the cheek lymphangiomas. Case 27 was excised viaa parotidectomy approach and suffered transient buccalbranch palsy. All complications had resolved by the latestfollow-up. The follow-up period for this group ranged from4 to 72 months, with a mean of about 2.7 years. The resultswere very good in 6%, good in 72% and fair in 22% ofcases.

High-flow Arteriovenous MalformationsThe 9 men and 5 women (Table 3) with arteriovenous

malformations had an age range of between 10 and 45 years(mean, 29.2). The most common primary zone was the lip(5), predominantly the upper lip, followed by the forehead(3), cheek (3), nose (1), mandible (1) and neck (1).Dimensions of the lesions ranged from 10 x 10 mm to 70x 30 mm. Four patients presented with recurrence (Cases35, 37 and 39) or persistence (Case 40) of a previouslytreated high-flow arteriovenous malformation. Every patientin this group noted a lump or swelling; this was associatedwith progressive enlargement (6), pulsatility and thrill (3),bleeding (2), pain and discomfort (1), positive Valsalvasign (1) and elevated local temperature (1).

Six patients (Cases 30, 33, 35, 37, 40 and 41) noted thecongenital history of the lump; in 2 patients (Cases 28 and29) the arteriovenous malformation was first noted duringchildhood/puberty. The malformation appeared soon aftertrauma in 1 patient (Case 32); in the remaining 5 patients,the lesion became apparent in adulthood. MRI and/or CTof the vascular malformation were done in 7 cases andangiogram with embolisation in 6 cases.

Excision (Table 3) alone was used in 8 patients withdiscrete arteriovenous malformations. Six arteriovenousmalformations (43%) – 4 Schobinger stage II and 2 stageIII malformations – were treated with superselectiveembolisation followed by en bloc resection after 24 hours.One patient (Case 37) required a radial forearm free flap forlip reconstruction and later flap debulking. The radialforearm flap was utilised again, this time for right cheekreconstruction in Case 41.

There was recurrence upon follow-up in 1 patient whoinitially had been treated with resection alone (Case 32); hesubsequently had preoperative embolisation and excisionof the recurrent (and larger than before) lesion, eventuallyrequiring scalp tissue expansion as part of reconstruction.

All other lesions were excised with direct closure. Therewere no recurrences for all other cases treated. The follow-up period ranged from 3 to 48 months (mean, 2 years). Atlatest follow-up, the results were very good in 29%, goodin 57% and fair in 14% of cases.

DiscussionThe appropriate management of vascular anomalies is

first dependent on an accurate diagnosis. It is important todistinguish between haemangiomas and vascularmalformations, as well as between fast-flow and low-flowvascular malformations. This leads to selection of the mostsuitable treatment modality for the lesion and appropriatefollow-up and assessment of the functional and aestheticoutcome. This approach for the series of anomalies will bediscussed separately. We will provide an algorithmicframework for the management of cervicofacial vascularanomalies based on our experience and current evidence,emphasising distinctly the place of surgical excision inoverall management.

HaemangiomaHaemangiomas are the most common tumours during

infancy, affecting up to 12%, 1.4%, and 0.8% of Caucasian,African American and Asian paediatric populationsrespectively.6 There is a female preponderance as wasevident from our series. Among affected children, 80%have a solitary lesion while the rest have multiple lesions.Some 60% of haemangiomas are cervicofacial and the bulkof these, appearing shortly after birth and expected toinvolute with time notwithstanding initial rapid growth, arecommonly managed conservatively at paediatric ordermatologic centres.5 The majority of cases do not presentto our centre for plastic surgical excision and treatment –herein lies the significance of this review.

Up to 93% of haemangiomas are easily diagnosed withoutadditional diagnostic tests. Superficial lesions turn raised,bosselated and vivid crimson (strawberry-like). Deeperlesions arise in the lower dermal or subcutaneous planes,appearing with healthy overlying skin or as pale blue orpurple masses easily confused with venous malformations.4

Lesions generally feel firm, rubbery and incompressible,becoming less turgid and fading with involution.

All cases in our series had histology compatible with adiagnosis of haemangioma. Histologically, haemangiomasshow plump endothelial cells with multilaminated base-ment membranes and numerous mast cells; immunohisto-chemistry demonstrates increased vitronectin, perlecan.6

The routine hospital use of the immunohistochemical markerGLUT-1 to accurately distinguish haemangiomas (GLUT-1 positive) from vascular malformations has been de-scribed and may be practical in our context.7

Cases 1, 3, 4, 6, 7, 8, and 9 (Table 1) presented with a



Tabl

e 2.

Clin

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Det

ails

of P

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nts w

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ace

or N

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Low

-flo

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alfo

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(mon

ths)

and

signs

and

type

of

invo

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106

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35

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170


Surgery for Cervicofacial Vascular Anomalies—Gavin CW KangTa

ble

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ths)

and

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of

invo

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nin

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2031

FN

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ling

40 x

30

Ven

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ph n

ode

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T –

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nom

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nN

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nom

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ter

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angi

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w fl

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mal

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sion

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ical

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4 Pa

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171Surgery for Cervicofacial Vascular Anomalies—Gavin CW KangTa

ble

3. C

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of re

curr

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geni

tal s

wel

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lesi

onal

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xcis

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Nil

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in g

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veno

us m

alfo

rmat

ion

172



lesion shortly after or at birth. The lesions inCases 1, 4, 6 and 8 were superficial and brighter.In Case 3 the lesion was more dermal andexhibited a purplish blue hue. The remaining 4patients had haemangiomas that did not re-semble any vascular anomaly and becameapparent only later in life, manifesting as aes-thetic worry or symptoms for concern (swell-ing, pain, tenderness, discomfort). Thehaemangiomas in these 4 patients were thelargest in our series and this seems to suggestthat large cervicofacial haemangiomas maylook atypical and are also more likely to causepain and discomfort. One (Case 2) complainedof a tender right neck lump that turned out to bea partially regressed subcutaneous haemangi-oma. Two patients (Cases 5 and 9), diagnosedinitially with a lipoma and a parotid tumourrespectively, were distressed by the presenceof a cheek lump in adulthood – in each amasseteric intramuscular haemangioma wasexcised. Intramuscular haemangiomas, whichare uncommon to begin with, are even rarer inthe head and neck, constituting 0.8% of allhaemangiomas in the region.8 When presentthe masseter is most frequently involved. Theymay be painful and are often misdiagnosedpreoperatively. The provisionally diagnosednasal dermoid in Case 7 turned out to be acapillary haemangioma. These behave differ-ently from common infantile haemangiomasin that they tend not to involute.

Deeper lesions mimicking other lesions,questionable superficial lesions and vascular-like lesions presenting in later life (to excludemalignancy and the occasional non-involutingor partially involuted haemangioma) requireimaging. CT and MRI are useful in confirmingdiagnosis and for evaluating the extent of thehaemangioma, intracranial involvement or anyassociated abnormalities (such as in thePHACE syndrome in which extensive facialhaemangiomas are linked to the central nerv-ous system, vascular, cardiac and ocular anoma-lies).9,10 MRI of a haemangioma demonstratesa lobulated soft tissue mass with flow voids;T1 images are isointense or hypointense tomuscle and T2 images are hyperintense; gado-linium provides intense homogenous enhance-ment. CT provides similar findings. Tumoursbearing little resemblance to haemangiomas(Cases 5, 7, 9) were diagnostic problems solvedwith the aid of MRI and CT. The diagnosis ofhaemangioma was suggested or made in 100%of the CT or MRI scans done. The Dopplerultrasound utilised in Case 2 provided a mis-Ta

ble

4. D

etai

ls o

f Em

bolis

ed F

ace

or N

eck

Hig

h-flo

w V

ascu

lar (

Arte

riove

nous

) Mal

form

atio

ns

Cas

eL

ocat

ion

Ves

sels

Embo

lisat

ion

tech

niqu

eEm

bolis

atio

n re

sult

28R

ight

che

ekFe

eder

- rig

ht fa

cial

arte

ry (s

uper

ior l

abia

l bra

nch)

PVA

of 3

00 to

600

mic

rons

with

occ

lusi

on o

fLe

sion

filli

ng a

t dec

reas

ed ra

te th

roug

hV

enou

s dr

aina

ge –

faci

al v

ein

the

faci

al a

rtery

supp

lysu

perio

r lab

ial b

ranc

h re

trogr

ade

flow

via

nas

alre

gion

col

late

rals

from

inte

rnal

mam

mar

ybr

anch

es

30Le

ft m

andi

ble

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er –

left

exte

rnal

car

otid

arte

ry (l

eft f

acia

l bra

nche

s,30

% g

lue

to li

piod

ol m

ixtu

res

with

goo

dR

educ

tion

in fl

ow th

roug

h A

VM

with

som

e st

asis

left

inte

rnal

max

illar

y br

anch

es, d

ista

l lef

t ext

erna

lpe

netra

tion

of th

e ni

dus

seen

in v

enou

s po

uche

sca

rotid

bra

nche

s)V

enou

s dr

aina

ge –

inte

rnal

max

illar

y ve

in,

and

faci

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into

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jugu

lar v

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car

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ry s

tudi

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how

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n A

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in th

esu

pras

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r cis

tern

/hyp

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lam

us re

gion

and

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ther

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ong

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left

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anch

es o

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of 3

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was

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inis

tere

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sup

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from

oph

thal

mic

tem

pora

l and

faci

al a

rterie

sfo

llow

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lfoam

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lets

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terie

s an

d m

inim

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uppl

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om ri

ght f

acia

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34Le

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of A

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em

bolis

ed90

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duct

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in th

e fil

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of th

e

faci

al a

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with

25%

glu

e to

lipi

odol

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ture

with

AV

M w

ith o

nly

resi

dual

filli

ng n

oted

at

Ven

ous

drai

nage

– d

eep

and

supe

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ial f

acia

l vei

nssa

tisfa

ctor

y ni

dal p

enet

ratio

nsu

perio

r asp

ect

and

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rnal

max

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y ve

in a

nd th

en in

to th

e ju

gula

r sys

tem

Faci

al a

rtery

com

pone

nt e

mbo

lised

with

PV

A 1

50 to

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mic

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and

gel

foam

ple

dget

s

37U

pper

lip

Feed

er –

faci

al a

rterie

s and

bra

nche

s of l

eft i

nter

nal m

axill

ary

Faci

al a

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s an

d in

tern

al m

axill

ary

arte

ry80

% to

90%

redu

ctio

n of

flow

ar

tery

bra

nch

embo

lised

with

PV

A a

nd g

elfo

amR

esid

ual s

uppl

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om le

ft tra

nsve

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ous

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vei

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all v

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ekFe

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– fa

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gnifi

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redu

ctio

n in

flow

thro

ugh

AV

MV

enou

s dr

aina

ge –

faci

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AV

M: a

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us m

alfo

rmat

ion;

PV

A: p

olyv

inyl

alc

ohol



The presence of a partially regressed haemangioma onthe nose (Cases 4 and 7) causes great psychological distressto a young adult. In extreme cases, the haemangioma cancreate a Cyrano de Bergerac nasal deformity,14 and a caseof a 4-year-old girl who tried to remove her nasal tiphaemangioma with scissors has been reported.15 The tumourmass effect can cause nasal cartilage aplasia in youngchildren; also, they are slow to regress and contourdeformities may result from the remnant fibrofatty tissueafter any involution. Surgical excision with preservation ofanatomy seems to be the treatment of choice. Variousexternal rhinoplasty approaches allowing for open access,correction of cartilaginous disruption and cosmesis havebeen suggested.15,16 Both our cases did well after excisionby external rhinoplasty. For larger lesions, excision andreconstruction by local tissue transfer or rearrangementmay be necessary.

Lip haemangiomas were encountered twice as partiallyinvoluted lesions in our series. Zide et al12 feel that liphaemangiomas may not spontaneously involute as often asor to the degree observed in haemangiomas involving otherfacial areas. Moreover, this limited involution may result inan abnormal distorted lip contour as the endpoint. Earlysurgical treatment is advocated while observing key tenetsthat include avoidance of aggressive muscle excision, thefact that surgery and secondary intention healing mayexpedite involution, and avoidance of denervation bymaking reductions in a central or paracentral fashion.

Morbidity was associated with cheek tumours andextensive haemangiomas. Postoperative dysesthesia withthe masseteric haemangiomas was probably caused byiatrogenic neuropraxia of the surrounding cutaneous nerves;the right facial pain in Case 9 settled with transcutaneouselectrical nerve stimulation. Our results show that outcomeand prognosis after surgical excision of localisedcervicofacial haemangiomas is excellent.

Low-flow Vascular MalformationsVascular malformations are often present at birth and

grow commensurately with the patient, usually onlybecoming significant later in childhood. There is generallyno spontaneous involution. Venous and lymphaticmalformations are low-flow lesions, while malformationswith an arterial component (most commonly arteriovenous)are considered high-flow lesions. All 18 cases in our serieshad histology compatible with a diagnosis of vascularmalformation,2 displaying normal non-proliferativeendothelium, normal basement membranes and a normalnumber of mast cells.

Venous MalformationsLike haemangiomas, the diagnosis of a venous

malformation is usually straightforward at clinical

taken diagnosis. Nevertheless, an ultrasound in experi-enced hands is a portable and available tool that can easilyconfirm a suspected haemangioma without additional test-ing.11 Doppler colour flow imaging is notable for its abilityto distinguish between high-flow and low-flow lesions.

In children, uncomplicated haemangiomas can bemanaged conservatively (“masterful neglect”), since 70%of haemangiomas involute spontaneously by age 7 andthere is continued improvement in the remaining until age10 to 12. However, periorbital, intranasal and laryngeal,and parotid haemangiomas may threaten vision, airwayand hearing respectively; bleeding, ulceration, infection,or even congestive cardiac failure can complicatehaemangiomas. These complicated haemangiomas deserveactive treatment and a wide array of both medical andsurgical therapeutic options is available – pharmacotherapy(steroids, interferon, chemotherapy), surgical excision,and less commonly, laser, embolisation, radiation,cryotherapy and compression.5,6 Pharmacotherapy isfrontline treatment for complicated haemangiomas, withsurgical excision indicated in those unresponsive topharmacologic therapy, those with psychological problems,and those with partially involuted or non-involutingunaesthetic lesions.

Surgical excision was carried out in all our cases.Unsurprisingly, in our series of mainly adult patients,chronic unaesthetic alteration (56%) and partially regressedhaemangioma (44%) were the most common principalindications, followed by need for diagnosis (44%), andsymptoms of pain, tenderness and discomfort (33%). Bychronic aesthetic alteration we refer to disruption of theinvolved aesthetic unit with excessive tissue or skinirregularities that are psychologically debilitating. For thepatient in Case 8, her mimimally regressed facialhaemangioma engulfing her central face and extendinginto the palate was grotesque and a source of greatpsychological distress in spite of multiple prior debulkinginterventions. With such extensive cervicofacialhaemangiomas, surgery serves only a palliative role.Children with disfiguring cervicofacial haemangiomas aresusceptible to psychological body image problems,especially at the school-going age of 4 to 5, yet for thesechildren judging the optimal timing for surgical interventionis difficult since it is not possible to predict the timing ofinvolution. Earlier haemangiomas that present afterchildhood would not be surgically treated until age 8 to 12.However authors are now making a paradigm shift inthinking towards surgical intervention in children from 2 or3 years of age.1,12,13 One child (Case 1) was troubled withfrequent bleeding from a neck haemangioma that mandatedearly surgical excision. She incidentally has the Klippel-Trenaunay syndrome characterised in her case by rightupper limb capillary malformation and hypertrophy.

174



examination. Venous malformations are the most commonsymptomatic vascular malformations, becomingsymptomatic in older children or young adults with bluishskin discolouration, local swelling and pain. They arecharacteristically soft and compressible bluish masses thatengorge with dependency; deep lesions may not be visibleat birth.4,14 Cervicofacial venous malformations can rangefrom tiny lesions to huge vascular malformations that causesignificant facial asymmetry and progressive distortion.No sexual predilection is recognised.

As is usually the case with venous malformations,4,14 themajority in our series had swellings noted at birth or soonafter that grew with the patient and, upon obtaining fullgrowth remained stable until presentation to medicalattention much later either for aesthetic or other concerns.Those with onset of clinical symptoms at a later age and/orrecent progressive enlargement in all likelihood had deeperinconspicuous malformations whose acute enlargementwas triggered by some unknown or unmemorable traumaticor hormonal event, much like for high-flow lesions. Thenasal malformation in Case 25 in particular appeared afterthe birth of her child, becoming cosmetically undesirablelater in life. In our series, more than a quarter of low-flowvascular malformations were located at the lip (notably theupper lip) making it the most common site of occurrence.One left cheek lesion (Case 15) was preoperativelydiagnosed as a parotid tumour but found intraoperatively tobe an infrequently encountered parotid venousmalformation. This patient presented with 3 months of aparotid swelling that fluctuated in size through the day andcaused pain whenever it enlarged; her features were typicalof previously reported parotid vascular malformations.17

The initial preoperative diagnosis was correct in 6 of 16patients (37.5%) while an equal number were mistakenlydiagnosed with haemangiomas. This was more likely tooccur in younger patients (Cases 10, 11, 12 and 14) inwhom the venous malformation would have been easilydiagnosed as a partially regressed haemangioma. Theremaining were diagnosed as salivary tumours, lymphnodes, lipomas, and other soft tissue tumours.

Imaging is required for indeterminate lesions and toassess the extent of the lesion and associated abnormalities.Some large vascular malformations are associated withsinus pericranii and developmental intracranial venousanomalies.18 Those of trigeminal localisation are associatedin 15% of patients with glaucoma, or choroidal andleptomeningeal haemangiomas. On radiographs and CTscans, they typically appear as soft tissue masses containingphleboliths. Venous malformations are high-signal intensitylesions on MRI T2 images and low-signal intensity lesionson T1 images; they have lobulated margins and multipleround signal voids representing phleboliths. Their

gadolinium contrast enhancement is more patchy andcentral, compared to lymphatic malformations, which haveno or minimal peripheral enhancement. MRI was done in2 cases, CT in 3 cases and angiography in 2 cases. All MRIscans, 2 CT scans and both angiograms were able todiagnose or suggest the diagnosis of a vascularmalformation. Low-flow vascular malformations onimaging were usually confused with haemangiomas in ourseries. Angiography was applicable to Case 16 with anextensive right cervicofacial and upper body vascularmalformation in helping to differentiate venousmalformations from high-flow vascular anomalies; itdemonstrated low-flow, low shunt vascular dynamics butdoes not have diagnostic value in assessing low-flowanomalies. Valuable information obtained included itsarterial supply, the absence of fast-flow lesion or aneurysm,and the absence of intracranial aneurysm or concurrentarteriovenous malformation. Biopsy performed in 2 casesprovided histopathological confirmation but is usuallyunnecessary unless history, physical examination or imagingis confounding.

While venous malformations in most areas can be treatedconservatively with the use of compressive therapy forlocal pain and swelling and aspirin for thrombosis,cervicofacial venous malformations are cosmeticallysignificant and best treated actively. This may entail the useof sclerotherapy, embolisation, surgical resection or acombination of these techniques. Sclerosing agents used inour series included sodium tetradecyl sulphate andthombovar.

Because venous malformations do not involute and growcommensurately with the patient even turning nodular andthickened with age, early surgical intervention is appropriateand psychosocially beneficial.14,19 Based on our experience,complete surgical excision is probably the best definitivetreatment for discrete small to moderate-sized venousmalformations considering the minimum morbidity andlow overall recurrence rate. It may be better to do MRIimaging at an early stage (possibly by age 5) for vascularanomalies that are diagnosed as haemangiomas but notregressing as quickly as expected. This allows earlieridentification of a misdiagnosed venous malformation thatcan then be expediently excised, preventing a futile wait forspontaneous involution and associated psychosocialdistress. This also avoids ineffective treatment such assteroid (Case 14). In the recurrent cases, we observe thatrecurrent lesions (Cases 16, 19) were more likely to recurafter excision; also, a recurrent lesion may grow to be muchlarger than the original lesion (Case 10).

Nearly a third of the venous malformations were foundon the lips. Of these 5 lip anomalies, 3 were on the upperlip. Lip lesions tended to require multiple procedures, be it



for excision of recurrence or revision, and the aestheticresult was generally not as good as for malformations inother locations. Surgeons should anticipate the need formultiple procedures and manage patient expectationsaccordingly. Zide et al19 recommended the following tenetsfor optimal surgical outcome: for upper lip lesions –minimal (about 10%) over-correction of deformities,adjustment of the affected side using the contralateral sideas an exact template, correction of vermillion discrepanciesas a final stage with expectation of future revisions in thisarea especially due to gravity; for lower lip lesions –overcorrect (about 20%) deformities expecting the needfor revisions, and step the vermillion in large central andvertical reductions to reduce notching.

Complex cervicofacial malformations (Cases 16, 19, 22)may be more suited to a combination of preoperativeembolisation, resection and sclerotherapy, and if neededfollowed by ingenious reconstruction using skin grafts,local tissue flaps or free flaps.14 Multiple procedures andoperations were often performed for large or multi-focalvascular malformations; they appeared to have a high riskof recurrence and the surgical result was not as good ingeneral. In Case 16, an extensive venous malformationrequired judicious staged debulking and reconstructionusing a pedicled contralateral deltopectoral flap. Thedeltopectoral flap was delayed and “waltzed” stepwisefrom the opposite chest wall and strategically inset into theserially created right-sided excision defect. At the time ofwriting this patient would require further surgery to clearthe vascular anomaly satisfactorily. Case 22 with a largefrontal malformation needed a tissue expanded local flapfor defect coverage and did well despite superficialprosthetic infection. Jackson et al20 compartmentalisedmassive head and neck vascular malformations using non-absorbable sutures followed by large dose sclerosantinjection into each compartment; this reduces the lesionvascularity for a safer subsequent resection and forhaemostasis in life-threatening haemorrhage. There wereno instances of haemorrhage as a complication in ourseries.

Lymphatic MalformationsThese lesions, also known as lymphangiomas or

lymphovenous malformations, are usually present at birthand their appearance varies ranging from blebs to large ormultiloculated cysts to the diffuse involvement of a bodypart or organ. They are classified as microcystic, macrocysticor mixed. About 75% of lymphatic malformations arecervicofacial. Again, most cases are clinically obvious andrequire no imaging studies for diagnosis.

However, MRI is especially useful for full evaluation:lymphatic malformations have low signal-intensity on T1images and marker hyperintensity on T2; most are non-

enhancing after intravenous contrast – this is the main MRIfeature distinguishing lymphatic and venous malformations.Only Case 27 had an MRI done that demonstrated arecurrent lymphangioma.

These lesions rarely resolve spontaneously and surgicalexcision is the most commonly accepted form of treatment.14

Other therapeutic modalities are less useful and althoughsuction-assisted lipectomy has been used, this has not beeneffective. The patient in Case 26 had been treated withliposuction twice without satisfactory effect and requiredsurgical resection of the lymphangioma.

High-flow Arteriovenous MalformationsLike most vascular malformations, arteriovenous

malformations are usually present at birth but may not beclinically evident. The arteriovenous malformation wasrecognised at initial presentation in most patients (50%),while in others it was suspected to be a venous vascularmalfomation, haemangioma, aneurysm or lymph node.Expansion involves increased blood flow, collateralformation and recruitment of normal adjacent vessels. Ofthese 14 patients, 78.6% of the arteriovenous malformationsbecame apparent either at birth or in adulthood, with equalincidence for both. The majority of our arteriovenousmalformations were found at the lip (36%), cheek (21%) orforehead (21%), and there were no auricular malformationsin our series, in contrast to the largest series to date ofarteriovenous malformations (n = 81) in which cheek(31%) and ear (16%) were the most common locations.21

Arteriovenous malformations may progress acutely dueto activating stimuli like trauma, pregnancy, puberty,infection, or even iatrogenic trauma (biopsy, proximalfeeder ligation, or subtotal excision).4,14,21 The patient inCase 32 had run into a tree trunk, traumatising his forehead.The arteriovenous malformation in Case 29 was noted inchildhood but suddenly expanded at puberty to becomemore obvious. Physically, as was seen with our patients, theoverlying skin can appear normal and there may be apulsatile mass, a thrill, increased warmth and redness;other possible features include pain, buzzing sound,overgrowth, haemorrhage and heart failure. Arteriovenousmalformations often remain undiagnosed until dramaticbleeding occurs as a result of dental manipulation.22 In Case30, the patient had congenital left facial swelling butbecame troubled by lower jaw gum bleeding shortly afterthe onset of puberty; he sought medical attention on theadvice of his dental surgeon. Categorised clinically by theSchobinger classification, 28% of patients were in stage I(cutaneous blush or warmth), 50% were stage II (bruit,audible pulsations, expanding lesion), 22% were stage III(pain, ulceration, bleeding, infection) and none were stageIV (cardiac failure).

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The high-flow nature of the lesion can be confirmedthrough Doppler examination. On MRI, the anomaly ischaracterised by enlarged vascular channels with dilatedfeeding and draining vessels; there is no discrete soft tissuemass. Abnormal arteriovenous connections are easilyrecognisable as linear or punctuate signal voids or ashyperintensities. Magnetic resonance angiography used inCases 28 and 37 confirmed the high-flow nature of thelesion and mapped out the feeding and draining vasculature.

Angiography, performed in 43% of our case series, isusually required to evaluate the abnormality in more detailbefore surgical excision or at embolisation; it shows a highdegree of shunting with high blood flow though the lesionand multiple feeders are usually seen. Angiography (Table4) revealed involvement of the facial artery (feeding vessel)and facial vein in all these cases. Angiography in Case 30demonstrated, in addition to the left mandibulararteriovenous malformation, hypothalamic and left opticpathway arteriovenous malformations consistent withfeatures of the Wymburn-Mason syndrome.

The arterial and venous phases are present in the sameangiographic image if there is significant shunting.Microshunts not demonstrated on angiography open upafter main feeding vessel ligation, incomplete embolisationor subtotal surgical resection leading clinically to recur-rence of the arteriovenous malformation often larger thanthe primary lesion. Therefore, selective embolisation on itsown, subtotal resection, or proximal ligation of vessels arerarely successful procedures with high-flow anomaliesbecause of the establishment of new flow pathways.14,21,23

The recurrences in Cases 32, 37, 39 were consequent onincomplete first resection, and the recurrence in Case 35resulted from both inadequate initial resection and thesubsequent suboptimal use of embolisation alone 6 yearslater. All recurrent lesions were notably larger than theprimary counterparts. Authors advise careful assessmentand combined treatment consisting of good highly selec-tive embolisation followed by total resection ideally within48 hours for most symptomatic arteriovenous malforma-tions.14,23

Compared to haemangiomas and venous malformations,arteriovenous malformations are rare in the head and neck,and the experience of most surgeons is limited. Kohout etal21 recommended combined embolisation and resectionfor early (stage I and II) lesions with the aim of preventingprogression to more complicated lesions, while painful orrapidly enlarging lesions warrant early intervention owingto a high likelihood of progression and risk of serioushaemorrhage. In our series, combined treatment waspractised in only 6 patients; the other 8 were subjected toexcision alone with no recurrence at a mean follow-up of2 years. Typically, a residual incompletely resected

arteriovenous malformation re-expands 1 to 2 yearspostoperatively but may remain quiescent for decades.24

Certainly resection alone has a place in the management ofsmall discrete non-expanding lesions if the surgeon isconfident of obtaining clear resection margins.

Of the 14 arteriovenous malformations, 6 (Cases 28, 30,32, 34, 37, 41) were subjected to preoperative superselectiveembolisation (Table 4). There are 2 forms of embolisation.24

Primary embolisation aims to deliver an ablative embolicagent to the nidus of the arteriovenous malformation tofacilitate endothelial destruction – this may have a role inpatients who are not ready for mutilating surgery. Particleembolisation, which was practised in this series, usesparticles such as polyvinyl alcohol foam (PVA) or acrylicmicrospheres to occlude flow into the nidus achievingpreoperative devacularisation, thus minimisingintraoperative haemorrhage and providing a dry operativefield. The particles come in different sizes and it is importantto choose the optimum size: oversized particles causephysiological proximal ligation and suboptimal preoperativedevascularisation whereas undersized particles do not servetheir occlusive purpose and may occlude the subdermalplexus leading to skin necrosis. Preoperative embolisationis not without its risks. It has the potential to cause stroke,cranial nerve ischaemia, skin necrosis, bleeding, blindness,adverse haemodynamic changes and even possiblepulmonary embolism.4

Some larger arteriovenous malformations have feedersarising from the internal carotid artery (Cases 30 and 32)and in these situations embolisation may not be possiblesuch that significant and even lethal bleeding may occur atresection.20 In such cases, the patient should be informed ofthe high risk of resection and if surgery proceedscardiopulmonary bypass may be considered, or thecompartmentalisation technique may be used in the eventof massive bleeding. Case 30 required emergency surgicalhaemostasis and excision via a hemimandibulectomy owingto extensive post-embolisation blowout bleeding from thearteriovenous malformation. This was not totally unexpectedlooking at its rich feeder vascularity originating from bothinternal and external carotid systems. The patient declinedmandibular reconstruction and was left with anacceptable jawline.

More sophisticated reconstructive solutions were utilisedin this category of anomalies. Of note, Case 32 (Fig. 1) hada traumatic forehead arteriovenous malformation that wasexcised only to recur 2 years later. Preoperative embolisationwas performed on the recurrent malformation followed byresection and split thickness skin grafting to cover thedefect. Scalp tissue expansion was used to advance hisdisrupted hairline but this was complicated by infection ofthe tissue expander that had to be removed. Tissue expansion



Fig.1. (Above, left) A 21-year-old man (Case 32) with a traumatic forehead enlarging arteriovenousmalformation that was excised. (Above, centre) Recurrence 2 years after the first operation. Note the oldscar. (Above, right) Angiogram at preoperative embolisation showing the embolised feeding vessels.(Below, left) Resected specimen of the recurrent lesion. (Below, centre) Result – 17 months afterpreoperative embolisation with aesthetic unit resection of the recurrence, showing a disrupted anteriorhairline post split skin grafting of the defect. (Below, right) Tissue expander inserted to facilitate hairlineadvancement.

Fig.2. (Above, left) A 37-year-old man (Case 37)with an upper lip gradually enlarging arteriovenousmalformation. (Above, centre) Coronal magneticresonance imaging (MRI) showing the vessels.(Above, right) Angiogram of the lesion doneshowing preoperative embolisation of the facialartery (Below, left) The left radial forearm free flapdonor site 4 days postoperatively. (Below, centreand right) Result – 3 months after resection withreconstruction.

Fig.3. (Above, left and centre) A 45-year-old man (Case 41) with a rightcheek enlarging arteriovenous malformation. (Above, right) Angiogramat preoperative embolisation showing the embolised feeding vessels.(Below, left) Resected specimen of the lesion. (Below, centre and right)Result – 3 months after resection and immediate radial forearm flapreconstruction.

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is particularly suited for scalp reconstruction here whereresection of the vascular anomaly disrupts the hairline. InCase 37 (Fig. 2) the patient suffered from a persistent upperlip arteriovenous malformation that had previously beensubjected to subtotal resection; he also had laser treatmentand sclerotherapy – modalities, along with steroid andirradiation, found to be ineffective for these anomalies.21

His malformation was finally subdued with radical en blocresection and reconstruction with a left radial forearm freeflap anastomosing the radial artery to the facial artery andthe radial venae comitantes to the facial veins, as well asanastomosing lateral cutaneous forearm nerve to the cervicalcutaneous branch. At 8-year follow-up there was norecurrence and the cosmetic and functional result was verysatisfactory to both the patient and the surgeon. The patientin Case 41 had a post-excision right cheek defectreconstructed using the versatile radial forearm flap onceagain fulfilling form and function (Fig. 3). As practised inCases 16, 37 and 41, the coverage of resulting defects withmyocutaneous flaps serves as a haemostypt and the transferof healthy tissue also helps create normal micro-revascularisation, hence preventing recurrence.4 Adequacyof malformation resection is best judged intraoperatively

from the bleeding quality and with Doppler assessment andfrozen section, as well as clinically at follow-up and againwith Doppler.

This single-institution study explored a dedicated seriesof surgically treated cervicofacial vascular anomalies interms of clinical characteristics, diagnosis, treatment andits outcome. Accurate diagnosis to distinguish betweencervicofacial haemangiomas and vascular malformationsis key to optimum management (Fig. 4). The diagnosis canbe made in the majority by history and physical examination.Cervicofacial vascular anomalies were most commonlyfound at the lip and atypical-looking vascular anomalieswere often misdiagnosed. In questionable cases diagnosisshould be aided at an early age with MRI, so that besttreatment may be chosen before the lesion progressesfurther. Although haemangiomas are expected to involutespontaneously and effective medical therapy is available incases threatening function or life, surgical treatment hasshed its traditional “last resort” image and is now clearlyindicated from a young age for preventing psychologicaldistress, in cases of chronic aesthetic alteration resultingfrom partial regression of haemangiomas, and where medicaltherapy has failed. While low-flow malformations usually

Fig.4. Algorithm highlighting the role of surgery in the management of cervicofacial vascular anomalies.



cause little morbidity, they do not involute and aestheticconcern and prevention of psychosocial distress points toearly excision as the treatment of choice. Lymphaticmalformations are best treated by excision. Outcome aftersurgical excision of localised cervicofacial haemangiomasand low-flow vascular malformations is generally excellent,with low recurrence rate and minimum morbidity. Largeextensive low-flow malformations as well as those locatedat the lips often require multiple procedures includingreconstruction; the outcome is generally not as good andthe patient must be made aware in these cases. Except forsmall localised lesions which can be directly excised,combined preoperative embolisation followed by completeexcision within 48 hours is the treatment of choice forarteriovenous malformations. Myocutaneous flapreconstruction may prevent the recurrence of arteriovenousmalformations. Tissue expansion is a useful technique forreconstruction after the excision of large vascular anomalies,particularly in the scalp and forehead.

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