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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
March 2008, Vol. 37 No. 3
167Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang
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
1. C
linic
al D
etai
ls o
f the
10
Patie
nts
with
Fac
e or
Nec
k H
aem
angi
omas
Cas
eA
geSe
xL
ocat
ion
Size
(mm
2 )Pr
inci
pal i
ndic
atio
nsD
iagn
ostic
Prio
rT
ype
ofO
ther
Rec
urre
nce
Follo
w-u
pR
esul
ts(y
)of
res
ecte
dfo
r ex
cisio
nm
odal
itytr
eatm
ent
surg
ical
trea
tmen
tor
(mo)
tum
our
and
trea
tmen
tin
volv
edco
mpl
icat
ion
inte
rpre
tatio
n
13.
5F
Nec
k5
x 5
Hae
mor
rhag
eN
ilN
ilEx
cisi
onN
ilN
il12
Ver
y go
od
212
MN
eck
25 x
20
Dia
gnos
is, t
ende
rD
oppl
er –
bra
nchi
alN
ilEx
cisi
onN
ilN
il10
Goo
dcy
st
317
FU
pper
lip
20 x
5Pa
rtial
regr
essi
on, a
esth
etic
Nil
Nil
Exci
sion
Nil
Nil
5V
ery
good
417
FN
asal
tip
9 x
8Pa
rtial
regr
essi
on, a
esth
etic
MR
I – h
aem
angi
oma
Nil
Ope
nN
ilN
il15
Goo
drh
inop
last
yan
d ex
cisi
on
518
MLe
ft ch
eek
20 x
20
Dia
gnos
is, d
isco
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rtC
T –
lym
phan
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aN
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cisi
onN
ilPr
eaur
icul
ar56
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dor
hae
man
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aea
rlobe
num
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s
620
FU
pper
lip
12 x
12
Parti
al re
gres
sion
, aes
thet
icN
ilN
ilEx
cisi
onN
ilN
il12
Fair
724
MN
asal
dor
sum
30 x
30
Dia
gnos
is, a
esth
etic
MR
I – h
aem
angi
oma
Nil
Ope
nN
ilN
il5
Ver
y go
odor
oth
er s
oft t
issu
erh
inop
last
ytu
mou
ran
d ex
cisi
on
842
FM
assi
veM
assi
veM
inim
al re
gres
sion
, C
T –
haem
angi
oma
Rad
ioth
erap
y,Ex
cisi
onPr
eope
rativ
eM
ild b
leed
ing
96Fa
ir (n
asal
exte
nsiv
eps
ycho
logi
cal p
robl
ems,
Ang
iogr
aphy
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ultip
lean
dem
bolis
atio
nre
cons
truct
ion
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al in
volv
ing
impa
ired
eye
clos
ure
Hae
man
giom
aex
cisi
ons,
debu
lkin
gpr
opos
ed)
pala
tela
ser
948
FR
ight
che
ek15
x 1
5D
iagn
osis
, pai
nM
RI –
hae
man
giom
aN
ilEx
cisi
onN
ilR
ight
12G
ood
or s
chw
anno
ma
faci
al p
ain
168
Annals Academy of Medicine
Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang
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
March 2008, Vol. 37 No. 3
169Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang
Tabl
e 2.
Clin
ical
Det
ails
of P
atie
nts w
ith F
ace
or N
eck
Low
-flo
w V
ascu
lar M
alfo
rmat
ions
Cas
eA
geSe
xL
ocat
ion
Pres
entin
gSi
ze (m
m2 )
His
tolo
gyIn
itial
dia
gnos
isD
iagn
ostic
Prio
rN
umbe
r of
Oth
erR
ecur
renc
eFo
llow
-up
Res
ults
(y)
sym
ptom
sm
odal
itytr
eatm
ent
oper
atio
ns:
trea
tmen
tor
(mon
ths)
and
signs
and
type
of
invo
lved
com
plic
atio
nin
terp
reta
tion
surg
ery
106
MU
pper
lip
Con
geni
tal
15 x
11
Ven
ous
Hae
man
giom
aN
ilN
il2:
Exc
isio
nN
ilR
ecur
renc
e60
Goo
dsw
ellin
gR
ecur
renc
e - 3
5 x
18m
alfo
rmat
ion
4 fir
st y
ears
afte
r ope
ratio
n
117.
5F
Upp
er li
pSw
ellin
g at
birt
h,20
x 2
0V
enou
sH
aem
angi
oma
Nil
Nil
1: E
xcis
ion
Nil
Nil
13G
ood
parti
al in
volu
tion
mal
form
atio
nby
6 y
ears
1215
FR
ight
che
ekPr
omin
ent l
ower
lip,
Low
er li
p –
30 x
10
Ven
ous
Hae
man
giom
aC
T –
lipN
il2:
Exc
isio
nN
ilN
il30
Goo
dan
d lo
wer
regr
essi
ng c
heek
Che
ek c
apill
ary
mal
form
atio
nle
sion
liple
sion
mal
form
atio
nha
eman
giom
aor ly
mph
angi
oma
1318
FN
eck
Swel
ling
25 x
25
Ven
ous
Saliv
ary
tum
our
Nil
Nil
1: E
xcis
ion
Nil
Nil
11V
ery
good
mal
form
atio
nor
lym
ph n
ode
or li
pom
a
1419
FR
ight
che
ekC
onge
nita
l les
ions
40 x
35
Ven
ous
Hae
man
giom
aM
RI –
Syst
emic
3: E
xcis
ion,
Nil
Nil
72Fa
iran
d lo
wer
lip
mal
form
atio
nva
scul
arst
eroi
dch
eek
and
mal
form
atio
nlip re
cons
truct
ion
1519
FLe
ft ch
eek
Pain
ful s
wel
ling
20 x
10
Ven
ous
Paro
tid tu
mou
rC
T –
slow
flo
wN
il1:
Sup
erfic
ial
Nil
Nil
16G
ood
mal
form
atio
nm
alfo
rmat
ion
vasc
ular
mal
form
atio
npa
rotid
ecto
my
1622
MEx
tens
ive
Pers
iste
nt re
curr
ent
Exte
nsiv
eV
enou
sV
ascu
lar
Ang
iogr
am –
Mul
tiple
10: E
xcis
ion,
Nil
Nil
96Fa
irrig
htle
sion
s sin
ce y
oung
mal
form
atio
nm
alfo
rmat
ion
vasc
ular
exci
sion
s,fla
pce
rvic
ofac
ial
mal
form
atio
nla
ser
reco
nstru
ctio
nin
volv
ing
right
ches
t and
uppe
r lim
b
1728
FN
eck
Con
geni
tal s
wel
ling,
35 x
20
Ven
ous
Ven
ous
Nil
Nil
1: E
xcis
ion
Nil
Nil
10G
ood
grow
ing
and
itchy
mal
form
atio
nm
alfo
rmat
ion
1830
FR
ight
Con
geni
tal s
wel
ling,
30 x
30
Ven
ous
Ven
ous
CT
– va
scul
arEx
cisi
on1:
Exc
isio
n of
Nil
Nil
60G
ood
tem
pora
lpr
evio
usly
exc
ised
,m
alfo
rmat
ion
mal
form
atio
nm
alfo
rmat
ion
recu
rren
cere
curr
ed a
nd p
ainf
ulor
hae
man
giom
aM
RI –
haem
angi
oma
1931
MR
ight
upp
erR
ecur
renc
e of
lesi
ons
Upp
er e
yelid
–Ey
elid
ven
ous
Hae
man
giom
asA
ngio
gram
– u
pper
Mul
tiple
7: E
xcis
ion,
Preo
pera
tive
Eyel
id72
Fair
eyel
idde
spite
pre
viou
s10
x 2
0m
alfo
rmat
ion
lip c
apill
oven
ous
exci
sion
s, s
car r
evis
ion
embo
lisat
ion
recu
rren
ceU
pper
lip
inte
rven
tion
Upp
er li
p –
60 x
40
Lip
capi
llove
nous
mal
form
atio
nem
bolis
atio
nLi
p5
year
san
dm
alfo
rmat
ion
scle
roth
erap
yaf
ter f
irst
colu
mne
llaop
erat
ion
170
Annals Academy of Medicine
Surgery for Cervicofacial Vascular Anomalies—Gavin CW KangTa
ble
2. C
ontd
.
Cas
eA
geSe
xL
ocat
ion
Pres
entin
gSi
ze (m
m2 )
His
tolo
gyIn
itial
dia
gnos
isD
iagn
ostic
Prio
rN
umbe
r of
Oth
erR
ecur
renc
eFo
llow
-up
Res
ults
(y)
sym
ptom
sm
odal
itytr
eatm
ent
oper
atio
ns:
trea
tmen
tor
(mon
ths)
and
signs
and
type
of
invo
lved
com
plic
atio
nin
terp
reta
tion
surg
ery
2031
FN
eck
Swel
ling
40 x
30
Ven
ous
Lym
ph n
ode
orC
T –
ster
nom
asto
idN
il1:
Exc
isio
nN
ilN
il4
Goo
dm
alfo
rmat
ion
ster
nom
asto
idtu
mou
r, or
tum
our
haem
angi
oma
2135
FM
edia
lSw
ellin
g5
x 5
Ven
ous
Vas
cula
rN
ilN
il1:
Exc
isio
nN
ilN
il5
Goo
dca
nthu
sm
alfo
rmat
ion
mal
form
atio
n
2243
MFr
onta
lSw
ellin
g fr
om y
oung
130
x 30
Ven
ous
Hae
man
giom
aN
ilSc
lero
ther
apy
2: T
issu
eN
ilIn
fect
ed18
Goo
dm
alfo
rmat
ion
expa
nsio
n,tis
sue
exci
sion
,ex
pand
erfla
pre
cons
truct
ion
2343
MFr
onta
lSw
ellin
g40
x 4
0V
enou
sLi
pom
aIn
cisi
on b
iops
y –
Nil
1: E
xcis
ion
Nil
Nil
53G
ood
mal
form
atio
nve
nous
mal
form
atio
n
2452
MFr
onta
lC
onge
nita
l les
ion
37 x
18
Ven
ous
Vas
cula
rN
ilN
il1:
Exc
isio
nN
ilN
il12
Goo
dgr
owin
gm
alfo
rmat
ion
mal
form
atio
npr
opor
tiona
tely
with
age
2553
FN
asal
tip
Pedu
ncul
ated
nas
al15
x 1
3C
apill
o-V
ascu
lar
MR
I –N
il1:
Exc
isio
nLa
ser t
oN
il12
Goo
dan
d rig
htsw
ellin
g af
ter
veno
usm
alfo
rmat
ion
haem
angi
oma
capi
llary
chee
kch
ildbi
rthm
alfo
rmat
ion
or lo
w fl
owm
alfo
rmat
ion
Con
geni
tal r
ight
vasc
ular
chee
k ca
pilla
rym
alfo
rmat
ion
mal
form
atio
n
2628
FR
ight
che
ekSw
ellin
g20
x 2
0Ly
mph
angi
oma
Lym
phan
giom
aN
ilLi
posu
ctio
n3:
Exc
isio
n,La
ser
Hae
mat
oma
21Fa
irsc
ar re
visi
on
2735
MLe
ft ch
eek
Prev
ious
exc
isio
n40
x 4
0Ly
mph
angi
oma
Lym
phan
giom
aM
RI –
Exci
sion
2: E
xcis
ion
Nil
Faci
al12
Goo
dof
che
ekly
mph
angi
oma
(buc
cal)
lym
phan
giom
aTr
ucut
–pa
lsy
Now
recu
rren
tly
mph
oid
cells
swel
ling
Tabl
e 3.
Clin
ical
Det
ails
of t
he 1
4 Pa
tient
s W
ith F
ace
or N
eck
Hig
h-flo
w V
ascu
lar (
Arte
riove
nous
) Mal
form
atio
ns
2810
FR
ight
che
ekSw
ellin
g w
ith th
rill
25 x
15
IIA
VM
MR
ang
iogr
am –
Preo
pera
tive
1: E
xcis
ion
Nil
Nil
7G
ood
AV
Mem
bolis
atio
n
2914
MN
asal
dor
sum
Swel
ling
that
enl
arge
s10
x 1
0II
AV
MM
RI –
smal
lN
il1:
Exc
isio
nN
ilN
il5
Goo
dup
on d
epen
denc
yha
eman
giom
a
3016
MLe
ft m
andi
ble
Con
geni
tal s
wel
ling
40 x
30
IIIA
VM
CT
- AV
MPr
eope
rativ
e1:
Hem
i-N
ilN
il48
Fair
with
left
eye
prop
tosi
sM
RI -
AV
Mem
bolis
atio
nm
andi
bule
ctom
yan
d bl
indn
ess
and
Low
er ja
w g
umtra
cheo
stom
ybl
eedi
ng
March 2008, Vol. 37 No. 3
171Surgery for Cervicofacial Vascular Anomalies—Gavin CW KangTa
ble
3. C
ontd
.
Cas
eA
geSe
xL
ocat
ion
Pres
entin
gSi
ze (m
m2 )
His
tolo
gyIn
itial
dia
gnos
isD
iagn
ostic
Prio
rN
umbe
r of
Oth
erR
ecur
renc
eFo
llow
-up
Res
ults
(y)
sym
ptom
sm
odal
itytr
eatm
ent
oper
atio
ns:
trea
tmen
tor
(mon
ths)
and
signs
and
type
of
invo
lved
com
plic
atio
nin
terp
reta
tion
surg
ery
3120
MFo
rehe
adPu
lsat
ile sw
ellin
g25
x 1
6II
AV
MN
ilN
il1:
Exc
isio
nN
ilN
il5
Goo
d
3221
MFo
rehe
adPo
sttra
umat
ic30
x 2
0II
Trau
mat
icA
ngio
gram
Preo
pera
tive
7: E
xcis
ion,
Nil
Rec
urre
nce
48Fa
irfo
rehe
ad sw
ellin
gan
eury
sm–
AV
Mem
bolis
atio
ntis
sue
expa
nsio
n,of
AV
Msc
alp
2 ye
ars
reco
nstru
ctio
naf
ter f
irst
oper
atio
nTi
ssue
exp
ande
rin
fect
ion
3321
FFo
rehe
adC
onge
nita
l10
x 1
0I
Cav
erno
usN
ilN
il1:
Exc
isio
nN
ilN
il3
Ver
y go
oddi
scol
oure
d sw
ellin
gm
alfo
rmat
ion
with
pos
itive
Val
salv
a si
gn
3422
MLe
ft ch
eek
Com
pres
sibl
e32
x 2
5III
Hae
man
giom
aM
RI –
Preo
pera
tive
1: E
xcis
ion
Nil
Faci
al56
Goo
dsw
ellin
g w
ithha
eman
giom
aem
bolis
atio
n(m
andi
bula
r)di
scom
fort
Ang
iogr
am -
AV
Mpa
lsy
3526
FLo
wer
lip
Con
geni
tal
30 x
32
IIV
ascu
lar
Nil
Exci
sion
,1:
Exc
isio
nLa
ser,
scle
ro-
Nil
12G
ood
swel
ling
with
mal
form
atio
nem
bolis
atio
nof
recu
rren
ceth
erap
ylip
ect
ropi
on
3632
MN
eck
Swel
ling
20 x
20
ILy
mph
nod
eC
T –
cold
abs
cess
Nil
1: E
xcis
ion
Nil
Nil
13V
ery
good
or T
B a
deni
tis
3735
MU
pper
lip
Con
geni
tal s
wel
ling
70 x
30
IIA
VM
MR
ang
iogr
am –
Parti
al2:
Exc
isio
nN
ilN
il96
Ver
y go
odw
ith e
leva
ted
loca
lha
eman
giom
are
sect
ion,
of re
curr
ence
,te
mpe
ratu
reA
ngio
gram
–la
ser,
free
flap
AV
Msc
lero
ther
apy
reco
nstru
ctio
nPr
eope
rativ
eem
bolis
atio
n
3839
MU
pper
lip
Puls
atile
swel
ling
20 x
10
IIIA
VM
Nil
Nil
1: E
xcis
ion
Nil
Nil
4G
ood
with
thril
l and
blee
ding
3944
FU
pper
lip
Swel
ling
30 x
32
IA
VM
MR
I – A
VM
Exci
sion
1: E
xcis
ion
Nil
Nil
4G
ood
of re
curr
ence
4045
FU
pper
lip
Con
geni
tal s
wel
ling
30 x
10
IH
aem
angi
oma
Nil
Intra
lesi
onal
2: E
xcis
ion,
Nil
Nil
12G
ood
ster
oid,
lase
rsk
in g
rafti
ng
4145
MR
ight
che
ekC
onge
nita
l sw
ellin
g30
x 1
0II
AV
MN
ilPr
eope
rativ
e1:
Exc
isio
n,N
ilN
il24
Ver
y go
odem
bolis
atio
nfr
ee fl
apre
cons
truct
ion
AV
M:A
rterio
veno
us m
alfo
rmat
ion
172
Annals Academy of Medicine
Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang
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
Feed
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
al v
ein
into
the
inte
rnal
jugu
lar v
eins
Inte
rnal
car
otid
arte
ry s
tudi
es s
how
ed a
n A
VM
in th
esu
pras
ella
r cis
tern
/hyp
otha
lam
us re
gion
and
ano
ther
AV
Mal
ong
the
left
optic
trac
t
32Fo
rehe
adFe
eder
– o
phth
alm
ic a
rterie
s, br
anch
es o
f sup
erfic
ial
PVA
of 3
55 to
500
mic
rons
was
adm
inis
tere
dR
esid
ual A
VM
sup
ply
from
oph
thal
mic
tem
pora
l and
faci
al a
rterie
sfo
llow
ed b
y ge
lfoam
pel
lets
ar
terie
s an
d m
inim
al s
uppl
y fr
om ri
ght f
acia
lV
enou
s dr
aina
ge –
faci
al v
eins
and
sup
erfic
ial t
empo
ral v
eins
arte
ry
34Le
ft ch
eek
Feed
er –
bra
nche
s of l
eft i
nter
nal m
axill
ary
arte
ry a
nd le
ftB
ucca
l arte
ry c
ompo
nent
of A
VM
em
bolis
ed90
% re
duct
ion
in th
e fil
ling
of th
e
faci
al a
rtery
with
25%
glu
e to
lipi
odol
mix
ture
with
AV
M w
ith o
nly
resi
dual
filli
ng n
oted
at
Ven
ous
drai
nage
– d
eep
and
supe
rfic
ial f
acia
l vei
nssa
tisfa
ctor
y ni
dal p
enet
ratio
nsu
perio
r asp
ect
and
inte
rnal
max
illar
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
255
mic
rons
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
rterie
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
y fr
om le
ft tra
nsve
rse
Ven
ous
drai
nage
– fa
cial
vei
nfa
cial
arte
ry a
nd sm
all v
esse
ls
41R
ight
che
ekFe
eder
– fa
cial
arte
ryPV
A a
nd g
elfo
amSi
gnifi
cant
redu
ctio
n in
flow
thro
ugh
AV
MV
enou
s dr
aina
ge –
faci
al v
eins
AV
M: a
rterio
veno
us m
alfo
rmat
ion;
PV
A: p
olyv
inyl
alc
ohol
March 2008, Vol. 37 No. 3
173Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang
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
Annals Academy of Medicine
Surgery for Cervicofacial Vascular Anomalies—Gavin CW Kang
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
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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
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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.
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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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