Phẫu Thuật Quản Lý Và Điều Trị Dị Thường Mạch Máu

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Part III: Vascular Malformations
COMPLICATIONS
T e most common complications a er resection o an LM are in ection, bleeding, and wound
dehiscence. Postoperatively the surgical site should have a pressure dressing. When per orming
a subtotal resection that requires the approximation o ischemic tissues containing an LM, inter-
rupted sutures should be used and le in place until the wound has gained strength. Interrupted
sutures are less ischemic than a continuous suture and reduce the risk o dehiscence.
Intraoperative antibiotics are given when a subtotal excision is per ormed, because LMs have a
higher risk o in ection. Postoperative antibiotics are typically not administered. I possible, op-
erating around sensitive structures ( or example, a acial nerve) should be avoided, because the
risk o iatrogenic injury is high. Wounds should be closed with local tissues; a skin gra is rarely
required.
CONCLUSION
Lymphatic mal ormations do not mandate intervention and can be observed. T e primary mor-
bidity o LMs is lowered sel -esteem ollowed by bleeding and in ection. When possible, rst-line
intervention is sclerotherapy. Resection is reserved or small lesions that can be removed or pos-
sible cure, those that remain symptomatic a er sclerotherapy, or or microcystic LMs. Surgical
treatment is individualized. Di use LMs should undergo subtotal resections to improve the most
symptomatic areas rather than radical extirpation and complex reconstruction. T e de ormity
caused by operative treatment should not be worse than the appearance o the LM. Patients with
LMs are best managed in an interdisciplinary vascular anomalies center ocused on these lesions.
SUGGESTED READINGS
Chaudry G, Guevara CJ, Rialon KL, et al. Sa ety and e cacy o bleomycin sclerotherapy or microcystic lymphatic
mal ormation. Cardiovasc Intervent Radiol : , .
Choi DJ, Alomari AI, Chaudry G, et al. Neurointerventional management o low-f ow vascular mal ormations o the
head and neck. Neuroimaging Clin N Am : , .
Greene AK. Lymphatic mal ormation. In Greene AK, ed. Vascular Anomalies: Classi cation, Diagnosis, and Man-
agement. St Louis: Quality Medical Publishing, .
Greene AK, Maclellan RA. Operative treatment o lymphedema using suction-assisted lipectomy. Ann Plast Surg
Sept . [Epub ahead o print]
Greene AK, Perlyn CA, Alomari AI. Management o lymphatic mal ormations. Clin Plast Surg : , .
Luks VL, Kamitaki N, Vivero MP, et al. Lymphatic and other vascular mal ormative/overgrowth disorders are caused
by somatic mutations in PIK CA. J Pediatr : , .
Maclellan RA, Couto RA, Sullivan JE, et al. Management o primary and secondary lymphedema: analysis o
re errals to a center. Ann Plast Surg : , .
Schook CC, Kulungowski AM, Greene AK, et al. Male genital lymphedema: clinical eatures and management in
pediatric patients. J Pediatr Surg : , .
Schook CC, Mulliken JB, Fishman SJ, et al. Primary lymphedema: clinical eatures and management in pediatric
patients. Plast Reconstr Surg : , .

Arin K. Greene
135
A venous mal ormation (VM) results rom abnormal smooth muscle coverage o veins. Lesions
behave similarly to a congenital “varicose vein”; they slowly dilate over time. Blood stagnation
can cause thrombosis and pain.
T ere are several phenotypes o VMs, and many have a known causative mutation. Although
VMs are present at birth, they may not be noted until childhood or adolescence; lesions worsen
particularly during puberty. T e major morbidity o VMs is that they cause psychosocial prob-
lems because o their appearance. Other complications occur as they progress: pain, bleeding,
ulceration, and obstruction/destruction o tissues.
Ven o u s Mal f o r mat io n s
KEY POINTS
• Most venous malformations are treated with sclerotherapy.
• Venous malformations are not malignant and do not require radical resection for “cure.”
• Resection of venous malformations is reserved for small lesions, venous malformations not
amenable to sclerotherapy, or symptomatic venous malformations after sclerotherapy.
• Surgical management of venous malformations should not cause a worse deformity than the
appearance of the lesion.
• Before surgical intervention of diffuse lesions, sclerotherapy is performed to facilitate the
procedure.
Ch a Pt er 9

136
Part III: Vascular Malformations
T e management o a VM with sclerotherapy is shown. An adolescent emale presented with
recent onset o an enlarging lesion o the le side o the ace (A). An MRI showed a VM along
the le mandible (B). Her appearance improved a er sclerotherapy (C). T e patient was managed
with sclerotherapy rather than resection to avoid a scar on her cheek and possible injury to the
marginal mandibular branch o the acial nerve.
Management o VMs is based on the type o lesion; some are treated primarily by resection,
whereas others are managed with sclerotherapy or laser. Pharmacotherapy does not exist. Sclero-
therapy involves the cannulation o a vein, ollowed by the injection o a sclerosant ( or example,
sodium tetradecyl sul ate or ethanol). T e sclerosant causes endothelial injury, brosis, obliter-
ation o the vessel lumen, and shrinkage o the VM. An endovascular laser may be used to treat
phlebectatic veins. A carbon dioxide laser can be used to treat cutaneous bleeding areas ( or ex-
ample, verrucous venous mal ormation). Generally sclerotherapy is rst-line treatment o VMs,
and surgical intervention is reserved or small lesions or symptomatic patients a er sclerotherapy.
Because most VMs are di use and involve multiple tissue planes, complete extirpation is rarely
possible. Instead the goal is usually to alleviate symptoms and control the lesion. Despite subtotal
and presumed “complete” extirpation, most VMs reenlarge. Patients and amilies are counseled
that a VM is likely to recur a er resection, and urther treatment may be needed in the uture.
Table 9-1 Phenotypes o Venous Mal ormations
Lesion
Mutation
Blue rubber bleb nevus syndrome

TIE2
Cerebral cavernous malformation

KRIT1, malcavernin, PDCD10
Cutaneoumucosal venous malformation TIE2
Diffuse phlebectasia of Bockenheimer
Fibroadipose vascular anomaly

PIK3CA
Glomuvenous malformation
Glomulin
Phlebectasia
Sinus pericranii
Verrucous venous malformation

MAP3K3
KRIT1, Krev interaction trapped protein 1; MAP3K3, mitogen-activated protein kinase kinase kinase 3; PDCD10, programmed cell death protein 10;
PIK3CA, phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha; TIE2, receptor tyrosine kinase.
Fig. 9-1
A

B

C

137
Chapter 9: Venous Malformations
SURGICAL INDICATIONS
Intervention or a VM is not mandatory. reatment is reserved or lesions that lower sel -esteem,
cause pain, bleed, or obstruct/destroy tissues. Resection and reconstruction should not leave a
de ormity worse than the VM. T e primary variable that determines whether a lesion should be
resected is the type o VM; some can only be managed with excision, whereas others should be
treated with sclerotherapy. Small, localized VMs are more amenable to resection, because they
can potentially be removed or cure (see Video 9-1, Venous Mal ormation).
v e n o u s Ma l f o r Ma t io n
Approximately 95% o VMs are non amilial, solitary, and result rom a somatic mutation. Le-
sions can be small and localized or large and di use. Patients who have psychosocial morbidity,
pain, or reduced unction are candidates or treatment. Generally individuals are managed with
sclerotherapy, because it has better e cacy and is sa er than excision. Surgical treatment o VMs
is reserved or:
• Small lesions that can be completely removed or cure
• Mal ormations that remain symptomatic but can no longer be treated with sclerotherapy
because all o the channels have been obliterated
I pre er to resect large VMs only a er they have been treated with sclerotherapy because:
• T e lesion becomes smaller.
• Veins are converted to brotic tissue, which acilitates excision.
• T e sclerosant permeates small vessels at the periphery o the lesion and theoretically re-
duces the recurrence rate.
v e n o u s Ma l f o r Ma t io n s u b t y Pe s
Blu e Ru b b e r Ble b Ne vu s Syn d ro m e
Patients with this condition can have multi ocal lesions involving the gastrointestinal tract. T e
major indication or intervention is chronic gastrointestinal bleeding requiring trans usions. Al-
though VMs can be treated with a sclerosant, resection o individual lesions is the pre erred
treatment and gives avorable outcomes.
Ce re b ra l Ca ve rn o u s Ma lfo rm a tio n
Patients have small, localized cerebral VMs and may have associated hyperkeratotic skin lesions.
Cerebral VMs are at risk o bleeding and seizures and may require resection i symptomatic.
Bleeding skin lesions can be treated with a carbon dioxide laser. However, resection is pre erred,
because it gives more de nitive improvement and a better aesthetic outcome.
Cu ta n e o m u c o s a l Ve n o u s Ma lfo rm a tio n
VMs associated with this multi ocal, amilial condition are typically small and well localized.
Consequently, resection is usually the pre erred rst-line treatment instead o sclerotherapy.

138
Part III: Vascular Malformations
Diffu s e P h le b e c ta s ia o f Bo c ke n h e im e r
T is eponym is used or a di use VM o an extremity a ecting all tissues (skin, subcutis, mus-
cle, and bone). Patients have signi cant morbidity, including reduced unction o the extremity.
First-line treatment is targeted sclerotherapy to improve localized problematic areas. Resection
is reserved or patients with signi cant symptoms who have ailed or cannot be treated with
sclerotherapy.
Fib ro a d ip o s e Va s c u la r An o m a ly
T is lesion shares eatures with an intramuscular VM but is associated with more pain, contrac-
tures, and at. A broadipose vascular anomaly does not respond to sclerotherapy, because it
is solid. However, adjacent phlebectatic veins may be injected. Initial management can include
sclerotherapy o peripheral phlebectasia, corticosteroid injection, and/or cryotherapy. However,
most individuals ultimately undergo resection o the lesion, which is de nitive therapy.
Glo m u ve n o u s Ma lfo rm a tio n
T is type o VM is typically multi ocal, small, and isolated to the skin and subcutaneous tissue.
Because lesions are usually localized and do not respond well to sclerotherapy, rst-line treatment
is resection. Sclerotherapy is reserved or large, di use lesions that could be di cult to remove
surgically.
P h le b e c ta s ia
T e abnormal dilation o a vein can cause a de ormity and lowered sel -esteem. Less commonly
the vein may be susceptible to clotting and pain. Phlebectatic veins are rarely resected. Instead
symptomatic areas are treated with sclerotherapy or an endovascular laser.
Sin u s Pe ric ra n ii
Sinus pericranii is a venous anomaly that extends rom super cial tissues, through the cranium, to
the dura. Veins are obliterated surgically or endovascularly to prevent intracranial thrombosis. Re-
section o an overlying so tissue VM is per ormed a er the transcranial veins have been closed.
Ve rru c o u s Ve n o u s Ma lfo rm a tio n
T is type o VM is not amenable to sclerotherapy. A carbon dioxide laser can reduce cutaneous
bleeding. T e de nitive treatment is resection.
SURGICAL MANAGEMENT
t iMin g o f in t e r v e n t io n
Milestones or the resection o VMs are: (1) 6 months, (2) 3 years, and (3) late childhood/early
adolescence. I possible, a VM should not be removed be ore 6 months o age. Be ore this time
the patient’s risk rom anesthesia is greater than in an adult. In addition, an in ant is less able to
tolerate a surgical procedure. I a large lesion is located on the scalp, the surgeon should consider
removing it be ore 6 months o age to take advantage o scalp laxity in in ancy.

139
Chapter 9: Venous Malformations
I a patient will likely require surgery, I will commonly intervene between 3 and 4 years o age.
Because long-term memory and sel -esteem begin to orm at approximately 4 years o age, re-
moving a VM be ore this time will improve a de ormity be ore it causes psychosocial distress to
the child; the patient also will probably not remember the procedure. Another period or surgery
is during late childhood/early adolescence, when the child is able to communicate whether he or
she wants to have the procedure. I the patient has a minor de ormity or a large lesion requiring
signi cant reconstruction, it is o en pre erable to wait until the patient verbalizes that he or she
wants the surgery. I the lesion is minor, it may be able to be removed with a local anesthetic by
waiting until the patient is older. I the VM is signi cant, the child’s willingness to be a participant
acilitates the process or the amily and surgeon.
A VM causing signi cant symptoms requires intervention, regardless o the age o the patient.
Generally, i the lesion is amenable to sclerotherapy, this intervention should be done rst. Re-
section is per ormed or:
• Symptomatic patients who have ailed sclerotherapy
• Small/localized lesions
• VMs that are not amenable to sclerotherapy ( or example, verrucous venous mal ormations)
v e n o u s Ma l f o r Ma t io n
Localized lesions that are small can be removed without preoperative sclerotherapy. Large, di use
VMs should be treated with sclerotherapy be ore resection. Sclerotherapy acilitates resection by
decreasing the size o the lesion, making the VM brous, and reducing the recurrence rate. Sclero-
therapy is continued until all veins have been obliterated, which may require several treatments at
6-week intervals. When venous targets are no longer present on ultrasound imaging, excision is
per ormed at least 3 months a er the last treatment to allow adequate brosis to occur. Removal
o a scarred VM a er sclerotherapy acilitates the procedure and reduces bleeding. An exception
to the use o preoperative sclerotherapy is i a di use VM is located adjacent to important nerves
( or example, a acial nerve or digital nerves). I a resection is planned or an area in which nerve
dissection is necessary, preoperative sclerotherapy should be avoided, because scarring rom the
procedure will complicate the separation o neurovascular structures rom the VM.
Excision o a VM can be associated with signi cant bleeding. Blood loss is reduced by:
• Giving preoperative sclerotherapy
• In ltrating the operative area with epinephrine
• Using epinephrine-soaked sponges during the procedure
• Using a tourniquet or extremity lesions
• Applying iced saline solution intraoperatively to promote vasoconstriction
• Keeping the surgical area elevated and the patient’s blood pressure low
• Incorporating clips to prevent skin edge bleeding (particularly scalp lesions)
I the volume o local anesthetic with epinephrine is limited by the amount o anesthetic that can
be given based on the weight o the child, I will in ltrate an epinephrine-only solution to ensure
maximum vasoconstriction o the surgical site (1 ml o 1:1000 epinephrine in 200 ml o normal
saline = 1:200,000 solution). T e extirpation proceeds care ully by incising a localized area and
controlling bleeding be ore continuing. Long, deep incisions should not be per ormed because
o the risk o signi cant blood loss. None o the patients with a VM on whom I have operated
has required a blood trans usion.

140
Part III: Vascular Malformations
Lesions located in anatomically sensitive areas ( or example, the ace) should have minimal mar-
gins included in the resection. A VM is not a malignancy, and evidence does not show that a
signi cant margin lowers the recurrence rate. T e VM o en involves a larger area than is appre-
ciated clinically and radiographically. Intraoperatively, subcutaneous tissues can be cauterized at
the periphery o the VM without removing skin. Cautery during the procedure and brosis may
destroy a residual VM. I a lesion is located in a nonsensitive area ( or example, the abdomen),
larger margins can be taken as long as they do not complicate the extirpation and reconstruction.
Di use VMs are unable to be entirely removed without causing signi cant morbidity. Patients are
managed with: (1) sclerotherapy or palliation or (2) subtotal excisions o symptomatic areas. Sur-
gical intervention or a di use acial VM should be ocused on improving the patient’s appearance
or pain with localized, staged procedures without causing a signi cant de ormity. Diseased tissue
should not be removed i there is a risk o iatrogenic morbidity. It is critical to avoid acial nerve
injury. Facial nerve dissections or parotidectomy should not be per ormed. When the lesion is
throughout all structures o the ace, strategies to improve the area include: (1) excision o excess
skin and subcutaneous tissue, (2) removal o the buccal at pad, and/or (3) contour burring o
the zygoma. I overgrowth is located on the lateral cheek, a preauricular incision is used. Medial
ullness is resected through a mesolabial incision. Alternatively, a circular excision can be used
to access the overgrown tissue, and the purse-string closure can be revised at a second stage. Lip
overgrowth is improved with a transverse mucosal resection along the keratinized and nonkera-
tinized border o the vermilion. Vertical resections o the lip should be avoided.
Subtotal resections o di use VMs are closed with adjacent tissues. I have never required a skin
gra or ree tissue trans er to close a de ect a er extirpation o a VM. Large, cutaneous VMs can
be removed with serial excision. I the skin has minor telangiectasias/dilated venules causing blu-
ish discoloration and located in an un avorable location ( or example, the nasal tip or philtrum)
where skin resection would cause a signi cant de ormity, the area can be treated with sclerother-
apy or a laser. I inject 1% sodium tetradecyl sul ate (1 ml o 3% solution mixed with 2 ml o saline)
with a 30-gauge needle and 1 ml syringe. Repeat injections can be per ormed at 6-week intervals.
Skin gra s should be avoided, because they can cause a worse de ormity than the appearance o
the mal ormation and are more likely to ail i placed on an ischemic recipient site containing a
VM. issue expansion should also be used with caution i adjacent to a VM, because the risk o
in ection, extrusion, stimulation o the lesion, and skin necrosis is much higher compared with
reconstruction o an area that does not contain a vascular anomaly.
v e n o u s Ma l f o r Ma t io n s u b t y Pe s
Cu ta n e o m u c o s a l Ve n o u s Ma lfo rm a tio n
Because these lesions are small and localized, they do not require sclerotherapy and can be excised
and closed linearly.
Fib ro a d ip o s e Va s c u la r An o m a ly
Lesions that have ailed sclerotherapy, corticosteroid injection, and/or cryotherapy require resec-
tion. T e muscle containing the broadipose vascular anomaly is usually non unctional, causes
signi cant pain, and results in contractures. Consequently, subtotal or complete extirpation o
the muscle is typically required. Because this type o VM can cause signi cant pain, as much o
the lesion as possible should be removed while maintaining important structures.

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Chapter 9: Venous Malformations
Glo m u ve n o u s Ma lfo rm a tio n
First-line intervention or this type o VM is typically resection, because the lesions are localized
and do not respond well to sclerotherapy. A er excision wounds are closed linearly.
Ve rru c o u s Ve n o u s Ma lfo rm a tio n
A verrucous venous mal ormation most commonly a ects the lower extremity and is not amena-
ble to sclerotherapy, because it is primarily composed o broadipose tissue. Large lesions can be
removed with serial excisions at 6-week intervals. Patients are o en placed into a knee immobi-
lizer to reduce the risk o wound dehiscence.
PATIENT EXAMPLES
l o c a l iz e D v e n o u s Ma l f o r Ma t io n : c o MPl e t e r e s e c t io n
Pa tie n t A
T is young child had an enlarging lesion o the scalp that was diagnosed radiographically as a VM.
A er sclerotherapy the patient’s parents requested excision o the residual brotic protuberance.
T e preoperative appearance is shown (A). An MRI illustrated the lesion (B). T e markings or
resection (C) and an intraoperative view a er excision (D) are shown. T e healed incision is seen
(E).
Fig. 9-2
A

B
C

D

E

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Part III: Vascular Malformations
Pa tie n t B
T is woman presented with the recent onset o a bluish lesion o her le cheek. She was unhappy
with the appearance o the VM and was unable to conceal it with makeup. Although sclerotherapy
was an option, resection was per ormed, because the lesion was small and could be excised or
cure. In addition, although sclerotherapy would have f attened the lesion, the skin in the area was
likely to remain bluish. T e patient is shown be ore (A) and a er resection (B).
Pa tie n t C
T is adolescent boy presented with an enlarging lesion o the posterior scalp. Although the VM
could have been treated with sclerotherapy, it was resected or cure, because it was well localized.
T e intraoperative view is seen (A). T e scalp is shown a er resection (B).
A

B
Fig. 9-3
A

B
Fig. 9-4

143
Chapter 9: Venous Malformations
Pa tie n t D
T is young child presented with an enlarging lesion o the hand. An MRI showed a VM o the so
tissue not involving neurovascular structures. Because the lesion was localized and sclerotherapy
could complicate later extirpation as a result o brosis in this location o the hand, resection was
chosen. T e preoperative appearance o the hand is shown (A and B). T e MRI illustrated the
VM (C). T e excised lesion is seen (D).
Pa tie n t E
T is in ant presented with a large lesion o the upper extremity that was present at birth. Ultraso-
nography illustrated a VM with calci cations. Because the lesion was pedunculated and located
in a avorable area or resection, it was managed surgically. T e preoperative appearance is seen
(A). Ultrasonography showed a heterogeneous, well-circumscribed vascular mass with ocal cal-
ci cations (B). T e intraoperative view is seen (C). T e patient is shown a er resection (D).
Fig. 9-5
A

B

C

D
Fig. 9-6
A

B
C

D

144
Part III: Vascular Malformations
A

B

C
Fig. 9-8
l o c a l iz e D v e n o u s Ma l f o r Ma t io n : s u b t o t a l r e s e c t io n
Pa tie n t A
T is child had a VM o the upper lip, causing a de ormity. Radical resection would cause a de or-
mity worse than the appearance o the VM. T e patient was initially managed with sclerotherapy
to acilitate the surgical procedure. T e bluish discoloration o the vermilion was treated later with
1% sodium tetradecyl sul ate. T e patient’s appearance during childhood is seen (A). T e lesion
increased in size during adolescence (B). A er sclerotherapy, the mal ormation was converted
rom venous channels to brotic tissue, which acilitated resection (C). T e patient is shown a er
subtotal excision o the overgrown lip with a transverse mucosal incision (D). Note the residual
bluish discoloration o the vermilion. T e patient has reduced discoloration o the vermilion a er
sclerotherapy o the super cial venules (E).
Pa tie n t B
T is child with an enlarging VM o the lower lip was managed with sclerotherapy, ollowed by a
subtotal excision with a transverse mucosal resection. T e appearance is shown be ore sclerother-
apy (A). A er several treatments the venous channels were no longer present (B). T e reduced
size o the lesion and brosis acilitated resection. T e patient’s appearance is shown a er subtotal
excision (C).
Fig. 9-7
A

B
C

D

E

145
Chapter 9: Venous Malformations
Pa tie n t C
T is adolescent girl presented with an enlarging lesion o the le knee. An MRI showed a VM
primarily involving the subcutaneous tissues. Resection was chosen, because: (1) the lesion had
a signi cant broadipose component and was predicted to have a marginal response to sclero-
therapy, and (2) the patient was unhappy with the appearance o her knee. Although the skin
was involved, a radical resection was not per ormed or a benign lesion. Instead as much o the
lesion as possible was removed, and the adjacent tissue was approximated to close the wound. T e
preoperative appearance is seen (A and B). T e MRI showed the extent o the lesion (C). Mark-
ings or the incision and resection are shown (D). T e outer line delineates the boundaries o the
VM. T e center marking shows the incision and skin that was excised. Removing an area o skin
in the center o the lesion allowed exposure o the subcutaneous VM with the shortest incision.
Because the mal ormation expanded the skin o the knee, the wound was approximated without
tension despite removing an area o integument. Intraoperatively the subcutaneous lesion was
exposed through a minimal incision (E and F). T e resected specimen is seen (G). Linear wound
closure is shown (H).
Fig. 9-9
A

C
B

D
E

F

G

H

146
Part III: Vascular Malformations
Dif f u s e v e n o u s Ma l f o r Ma t io n : s u b t o t a l r e s e c t io n
Pa tie n t A
T is young boy had a di use VM o the orbit, lower eyelid, and cheek. Radical resection and f ap
coverage o the area would cause a worse de ormity than the appearance o the lesion. He was
managed with sclerotherapy that e ectively reduced the size o the VM. He developed a ocal
cutaneous area that obstructed his vision and caused a severe de ormity that could only be im-
proved with resection. T e preoperative appearance is seen (A). An MRI showed a di use VM
(B). An intraoperative view (C) and postoperative appearance are shown (D).
Pa tie n t B
T is woman had an extensive VM o the le side o her ace. She underwent radiation treatment
in childhood and thus was not a candidate or sclerotherapy to the deep tissues. She was managed
with a subtotal resection o the lesion through an intraoral approach and cutaneous sclerotherapy
or dilated venules in the skin. Her preoperative appearance is seen (A). An MRI illustrated the
VM (B). She had improved acial contour a er surgery (C).
A

B

C
Fig. 9-11
Fig. 9-10
A

B
C

D

147
Chapter 9: Venous Malformations
Pa tie n t C
T is young adult presented with a large VM o his posterior trunk that was previously treated
with sclerotherapy at an outside hospital. Ultrasonography at our institution showed that minimal
targets or sclerotherapy were available, and thus the only management option was resection. A
subtotal muscle-sparing excision was per ormed. T e preoperative appearance is seen (A). An
MRI showed the VM (B). T e outer marking identi es the borders o the lesion (C). T e inner
outline illustrates the skin excision used to access the rest o the mal ormation. Intraoperative
views o the lesion are shown (D and E). T e resected specimen is seen (F). T e wound was closed
linearly over a drain (G).
Fig. 9-12
A

C
B
D

E

F

G

148
Part III: Vascular Malformations
Pa tie n t D
T is boy was born with a VM a ecting his superior orbital area, orehead, scalp, and rontalis
muscle. Imaging ruled out sinus pericranii. He underwent several sclerotherapy treatments to
acilitate the resection, which was complicated by ulceration and a wide orehead scar. T e scar
was incorporated into the incision or the resection, so that the area would have an improved
appearance. T e pretreatment appearance is seen (A). An MRI showed a VM (B). His appear-
ance a er sclerotherapy treatments is shown (C). Note the orehead scar. T e underlying VM
was accessed with an incision to revise the previous orehead scar (D). T e resected specimen is
shown (E). T e rontalis muscle that contained the VM was not removed. T e orehead is seen
a er excision (F). T is child had improved contour a er surgery (G).
Fig. 9-13
A

B
C

D

E
F

G

149
Chapter 9: Venous Malformations
Dif f u s e v e n o u s Ma l f o r Ma t io n : c o MPl e t e r e s e c t io n
T is in ant girl presented with a large VM o the parietal scalp that did not extend intracranially.
Be ore resection the lesion was treated with sclerotherapy to acilitate the surgery. Because o the
large size o the lesion, serial excision was initiated in early childhood to take advantage o the
scalp laxity that is present during this time. T is patient is shown be ore (A) and a er sclerother-
apy (B). T e outline o the rst resection is seen (C). T e patient is seen a er the initial excision
(D). Markings or the second-stage resection are shown (E). T e scalp is shown a er the nal
procedure (F). Wide subgaleal undermining and scoring were per ormed to approximate the scalp
a er each procedure. Large, interrupted sutures were placed or 4 weeks to minimize widening
o the scar.
c u t a n e o Mu c o s a l v e n o u s Ma l f o r Mat io n
T is woman was unhappy with the appearance o a localized VM o the lip. Although it could have
been treated with sclerotherapy, it was small enough to be removed or more de nitive treatment.
T e patient is shown be ore (A) and a er transverse mucosal excision (B).
Fig. 9-14
D

E

F
A

B

C
A

B
Fig. 9-15

150
Part III: Vascular Malformations
f ib r o a DiPo s e v a s c u l a r a n o Ma l y
T is adolescent girl had a progressive lesion primarily a ecting her gluteus maximus muscle. T e
cutaneous component was initially excised. She became nonambulatory because o pain and was
con ned to a wheelchair. Subsequently, she underwent complete resection, including the entire
gluteus maximus muscle. Shortly a er the procedure, her pain resolved, and she returned to nor-
mal activity. T e preoperative appearance is seen (A). An MRI showed a broadipose vascular
anomaly o the gluteus maximus muscle (B). An intraoperative view is seen be ore resection o
the muscle (C). An exposed gluteus maximus muscle contained the lesion (D). T e muscle was
non unctional and did not contract. Dissection o the muscle is shown (E). T e excised specimen
(F) and wound be ore closure (G) are seen. T e postoperative appearance is shown (H).
Fig. 9-16
A

B
C

D

E
F

G

H

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Chapter 9: Venous Malformations
g l o Mu v e n o u s Ma l f o r Ma t io n
T is adolescent boy developed multi ocal glomuvenous mal ormations. His primary complaint
was medial le thigh pain. Because glomuvenous mal ormations do not respond as well to sclero-
therapy compared with sporadic VMs and the lesions were small, they were removed. T e patient
had multi ocal lesions o his neck (A). Symptomatic glomuvenous mal ormations o the lower
extremity are shown (B). T e patient is seen a er resection o the leg lesions (C).
v e r r u c o u s v e n o u s Ma l f o r Ma t io n
Pa tie n t A
T is girl presented with a bleeding lesion o the right elbow that also was causing a de ormity.
Because this type o venous mal ormation is not amenable to sclerotherapy, it was resected. T e
preoperative appearance is shown (A and B). T e intraoperative view o the lesion is seen (C).
T e elbow is shown a er linear wound closure (D).
A

B

C
Fig. 9-17
Fig. 9-18
A

B

C

D

152
Part III: Vascular Malformations
Pa tie n t B
T is adolescent girl had a bleeding lesion o her lower extremity that also was causing a de ormity.
It was managed by serial excision. A skin gra was not used to reconstruct the area, because the
gra would have an in erior aesthetic outcome compared with a linear scar. T e preoperative ap-
pearance is seen (A). T e patient is shown a er rst-stage resection (B) and second-stage excision
(C). T e scar is shown (D).
COMPLICATIONS
Because VMs are vascular and ischemic, the two most common complications a er resection are
bleeding and wound dehiscence. Postoperatively the surgical site should have a pressure dressing.
Pain is aggressively managed, so that the patient’s blood pressure is not elevated, which would
increase the risk o hematoma. When a surgeon per orms a subtotal resection that requires the
approximation o ischemic tissues containing a VM, interrupted sutures should be used and le in
place until the wound has gained strength. Interrupted sutures are less ischemic than a continuous
suture and reduce the risk o dehiscence.
Intraoperative antibiotics are given when a subtotal excision is per ormed, because the ischemic
surgical site is at higher risk o in ection. Postoperative antibiotics are not administered. I possi-
ble, operating around sensitive structures ( or example, a acial nerve) should be avoided, because
the risk o iatrogenic injury is high. Unlike capillary or lymphatic mal ormations, VMs can be
associated with signi cant intraoperative bleeding. I possible, wounds should be closed with
local tissues. A skin gra placed over an area containing a VM has a high ailure rate, because the
recipient site is ischemic and unable to revascularize the gra .
Fig. 9-19
A

B

C

D

153
Chapter 9: Venous Malformations
CONCLUSION
VMs do not mandate intervention and can be observed. T e primary morbidity o VM is lowered
sel -esteem, ollowed by pain rom thrombosis. When possible, rst-line intervention is sclero-
therapy. Resection is reserved or small lesions that can be removed or possible cure, VMs that
remain symptomatic a er sclerotherapy, or phenotypes that are not amenable to sclerotherapy.
Surgical treatment is individualized. Localized lesions are excised or cure. Di use VMs should
undergo subtotal resections to improve the most symptomatic areas. Radical extirpation and
complex reconstruction should be avoided. T e de ormity caused by excision and reconstruction
should not be worse than the appearance o the VM. Patients with a VM are best managed in an
interdisciplinary vascular anomalies center with physicians ocused on these lesions.
SUGGESTED READINGS
Alomari AI, Spencer SA, Arnold RW, et al. Fibro-adipose vascular anomaly: clinical-radiologic-pathologic eatures
o a newly delineated disorder o the extremity. J Pediatr Orthop : , .
Boon LM, Mulliken JB, Enjolras O, et al. Glomuvenous mal ormation (glomangioma) and venous mal ormation:
distinct clinicopathologic and genetic entities. Arch Dermatol : , .
Choi DJ, Alomari AI, Chaudry G, et al. Neurointerventional management o low-f ow vascular mal ormations o the
head and neck. Neuroimaging Clin N Am : , .
Couto JA, Vivero MP, Kozakewich HP, et al. A somatic MAP K mutation is associated with verrucous venous
mal ormation. Am J Hum Genet : , .
Greene AK. Venous mal ormation. In Greene AK, ed. Vascular Anomalies: Classi cation, Diagnosis, and Manage-
ment. St Louis: Quality Medical Publishing, .
Greene AK, Alomari AI. Management o venous mal ormations. Clin Plast Surg : , .
Limaye N, Wouters V, Uebelhoer M, et al. Somatic mutations in angiopoietin receptor gene EK cause solitary and
multiple sporadic venous mal ormations. Nat Genet : , .

(Trang không có chữ)

Arin K. Greene
155
An arteriovenous mal ormation (AVM) is a problematic lesion. Arteries are connected to veins
through abnormal vessels called the nidus. Because a capillary bed is not present, blood is shunted
rom the arterial to venous circulation, resulting in venous hypertension and arterialized veins.
Reduced oxygen is delivered to tissues, causing them to be ischemic and prone to ulceration. Al-
though AVMs are present at birth, they may not be noted until childhood or adolescence. Lesions
slowly worsen over time, particularly during puberty.
Ar t er io ven o u s Mal f o r mat io n s
Ch a Pt er 10
KEY POINTS
• Surgical treatment of arteriovenous malformations must be individualized based on several
variables.
• Arteriovenous malformations are not malignant and do not require radical resection for
“cure.”
• Surgeries for arteriovenous malformations should not cause a worse deformity than the
appearance of the lesion.
• Diffuse arteriovenous malformations are best managed by focused, subtotal procedures to
improve symptoms and the appearance of the patient.
• Before surgical intervention, embolization is performed to reduce blood loss for regional and
diffuse arteriovenous malformations.

156
Part III: Vascular Malformations
AVMs can be localized, regional, or di use. T ey progress through our stages:
• Stage 1: Warm, skin discoloration, and ast f ow on Doppler examination
• Stage 2: Growth, palpable pulsations, and enlarged veins
• Stage 3: Ulceration, bleeding, and pain
• Stage 4: Congestive heart ailure
T e primary morbidity o AVMs is that their appearance causes psychosocial problems. Other
complications occur as they progress: pain, bleeding, and ulceration. reatments include embo-
lization and/or resection; pharmacotherapy does not exist. Embolization involves cannulation o
an artery remote rom the AVM (usually the emoral artery) and insertion o an embolic agent
into the nidus or draining veins. Generally, embolization is rst-line treatment o AVMs; surgical
intervention is reserved or small lesions or symptomatic patients a er embolization. Most AVMs
are di use and involve multiple anatomic structures, so complete extirpation is rarely possible;
the goal o intervention is usually to alleviate symptoms and control the AVM.
Despite subtotal and presumed “complete” extirpation, most AVMs reenlarge. Recurrence typi-
cally occurs during the rst year a er intervention, and 86% will reexpand within 5 years. Patients
who do not have a recurrence 5 years a er intervention are likely to have long-term control. How-
ever, 5% will experience regrowth more than 10 years a er surgery. Patients and amilies are told
that an AVM is likely to recur a er resection, and urther treatment may be needed in the uture.
SURGICAL INDICATIONS
Intervention or an AVM is not mandatory. Subtotal resection o a nonproblematic lesion may
cause ischemia and stimulate the AVM to enlarge and cause morbidity. Resection and reconstruc-
A

B
Fig. 10-1
T e progression o an AVM is shown.
T is 6-year-old patient had a stage 1
lesion (A). At 29 years o age he has a
stage 3 lesion (B).
Box 10-1 Surgical Principles o Arteriovenous Mal ormations
Intervention for an AVM is not mandatory; lesions can be observed
AVMs have a high recurrence rate after resection
Subtotal resection or embolization can stimulate an AVM to enlarge
Resection should not cause a worse deformity than the lesion
Lower-stage lesions are the least likely to recur
Surgical intervention is individualized based on stage, size, location, age, and symptoms

157
Chapter 10: Arteriovenous Malformations
tion should not leave a worse de ormity than the AVM. Variables that determine whether an AVM
should be resected include: (1) stage, (2) age, (3) location, and (4) size.
s t a g e 1
Stage 1 AVMs typically are not problematic unless they cause a de ormity and a ect the patient’s
sel -esteem. Localized lesions should be removed be ore they progress to a higher-stage AVM.
Resection o a stage 1 AVM gives the lowest recurrence rate and best chance or long-term con-
trol or cure. Localized lesions are most likely to be removed completely with the least morbidity.
Waiting until the lesion expands complicates the resection and reconstruction and increases the
likelihood o an un avorable long-term outcome. I a localized lesion involves an anatomically
un avorable area ( or example, the tip o the nose or upper eyelid) that would require complicated
reconstruction, leaving a worse de ormity than the lesion, I recommend waiting until the AVM
worsens and becomes symptomatic in the uture. Approximately 16% o stage 1 lesions do not
progress long term.
Resection o a regional or di use stage 1 AVM should be based on its size and location. Because
the entire lesion can rarely be removed, surgical intervention or large asymptomatic lesions is
less common. However, i it is possible to completely resect the AVM in an anatomically avorable
area ( or example, the trunk), prophylactic excision and reconstruction should be considered
be ore the lesion progresses. In contrast, i a large AVM is located on the ace and extirpation
and reconstruction would leave a signi cant de ormity, it is generally best to wait until the lesion
becomes problematic be ore intervention.
s t a g e 2
Stage 2 AVMs are managed similar to stage 1 lesions. However, there is a lower threshold to in-
tervene because the AVM is growing. Stage 2 lesions are more likely to be symptomatic, because
they are enlarging. Pulsations rom the lesion can also be bothersome to the patient.
s t a g e s 3 a n D 4
Stage 3 or 4 AVMs require treatment or de ormity, bleeding, pain, ulceration, and/or congestive
heart ailure.
SURGICAL MANAGEMENT
t iMin g o f in t e r v e n t io n
Stage 3 and 4 AVMs require immediate treatment, regardless o the age o the patient. Milestones
or resection o stage 1 and 2 AVMs are: (1) 6 months, (2) 3 years, and (3) late childhood/early
adolescence. Generally an AVM should not be removed be ore 6 months o age. At this time the
patient’s risk rom anesthesia is greater than or an adult. In addition, a young in ant is less able to
tolerate a surgical procedure. I a large lesion is located on the scalp, removing it be ore 6 months
o age should be considered to take advantage o scalp laxity that exists during in ancy.

158
Part III: Vascular Malformations
I it is likely that a patient will require surgery, a common time I will intervene is between 3 and
4 years o age. Because long-term memory and sel -esteem begin to orm at approximately 4 years
o age, removing an AVM at this time will improve a de ormity be ore the child’s sel -esteem be-
gins to orm, and the patient will likely not remember the procedure. Another period to intervene
is during late childhood/early adolescence when the child is able to communicate whether he/she
would like the procedure. I a patient has a minor de ormity or a large lesion requiring signi -
cant reconstruction, it is o en best to wait until the child verbalizes that he or she wants surgery.
I the lesion is minor, it may be able to be removed under local anesthesia by waiting until the
child is older. I the lesion is signi cant, then waiting until the child can be a willing participant
acilitates the process or the amily and surgeon. T e surgical strategy or AVMs is based on the
extent o the lesion.
l o c a l iz e D a v Ms
A localized AVM involves one to two tissue planes ( or example, the skin and subcutaneous tis-
sue), is well de ned, and theoretically is able to be entirely removed with linear closure. Un ortu-
nately, these are the least common types o AVMs. Generally, I remove localized AVMs without
preoperative embolization, because bleeding is not signi cant a er the area is in ltrated with a
local anesthetic containing epinephrine. Avoiding embolization reduces overall treatment morbid-
ity or the patient. I the volume o local anesthetic with epinephrine is limited by the amount o
anesthetic that can be given based on the weight o the child, I will in ltrate an epinephrine-only
solution to ensure maximum vasoconstriction o the surgical site (1 cc o 1:1000 epinephrine in
200 cc o normal saline = 1:200,000 solution).
Lesions located in anatomically sensitive areas ( or example, the ace) should have minimal or
no margins taken. An AVM is not a malignancy, and evidence does not show that a signi cant
resection margin lowers the recurrence rate. An AVM o en involves a larger area than is appre-
ciated clinically and radiographically. Findings o biopsies taken outside o the lesion have shown
abnormalities. In my experience i microscopic disease is le a er a localized AVM is removed,
the recurrence rate is very low. Cautery during the procedure and brosis may destroy residual
AVM tissue. I a lesion is located in a nonsensitive area ( or example, the abdomen), larger mar-
gins can be taken as long as they do not complicate the extirpation and reconstruction (see Video
10-1, Arteriovenous Mal ormation).
r e g io n a l a v Ms
Regional AVMs are di cult to remove and usually cannot be reconstructed with local tissue;
gra s or ree tissue trans er are typically required. Regional AVMs are managed by: (1) subtotal
Table 10-1 Types o Arteriovenous Mal ormations
AVM
Def nition
Surgical Treatment
Localized
1-2 tissue planes
Complete resection can be closed with local tissue
Subtotal excision if problematic location
Complete excision if nonproblematic location
Regional ≥2 tissue planes
Complete resection requires distant tissue
Subtotal excision if problematic location
Complete excision if nonproblematic location
Diffuse

≥3 tissue planes
Complete excision is not advisable
Subtotal excision

159
Chapter 10: Arteriovenous Malformations
resection o a symptomatic area or (2) complete extirpation and reconstruction with distant tis-
sue. I a regional AVM involves an aesthetically sensitive area, a subtotal resection to improve a
de ormity or alleviate bleeding/ulceration is indicated. Complete removal o the lesion should be
considered i the reconstruction will not leave the patient with a major de ormity.
T e borders o an AVM can be di cult to determine, because the periphery o the lesion may be
pinkish-red rom inf ammation and/or reactive hyperemia. It is pre erable to have minimal, i any,
margins when a lesion is resected rom an aesthetically sensitive area. Intraoperatively subcuta-
neous tissues can be cauterized at the periphery o the AVM without removing skin. Recurrence
is low when completely resecting regional AVMs, even without margins.
Unlike localized AVMs, regional lesions should have preoperative embolization to reduce in-
traoperative blood loss and acilitate the procedure. Our center pre ers Onyx, and I schedule
the resection within a week o the embolization be ore neovascularization resupplies the AVM.
Blood loss is urther reduced by: (1) in ltrating the operative area with epinephrine, (2) using
epinephrine-soaked sponges during the procedure, (3) using a tourniquet or extremity lesions,
(4) using iced saline solution intraoperatively to promote vasoconstriction, (5) keeping the sur-
gical area elevated and the patient’s blood pressure low, and (6) incorporating clips to prevent
skin edge bleeding (particularly scalp lesions). T e extirpation proceeds care ully by incising a
localized area and controlling bleeding be ore continuing. Long, deep incisions should not be
per ormed because o the risk o signi cant blood loss. Generally regional AVMs bleed less than
di use lesions, because the edges o the de ect are disease ree. None o the patients with an AVM
on whom I have operated has required a blood trans usion with these techniques.
Complete removal o a regional AVM requires reconstruction with gra s or f aps. Skin gra s are
best placed on a recipient site that is ree o disease. I the underlying area contains an AVM, the
gra has a higher chance o ailure, because the wound bed is ischemic. An AVM causes shunting
o blood directly rom the arterial to the venous circulation (a capillary bed is not present), and
thus oxygenated blood is not delivered to the tissues. I the recipient site is considered signi -
cantly involved with an AVM, f ap reconstruction is pre erred because o the high likelihood o
gra ailure.
Dif f u s e a v Ms
Di use AVMs are unable to be entirely removed without causing signi cant morbidity. T ese
lesions had been previously treated in our center with wide resection and ree tissue trans er. Un-
ortunately, the AVM had a high recurrence rate, and the reconstruction caused patients to have
a worse de ormity than the appearance o the AVM. Consequently, my philosophy changed to
managing these patients with: (1) embolization only or palliation o bleeding, pain, or ulceration
or (2) subtotal excisions o symptomatic areas.
I the primary morbidity is bleeding or a chronic wound, the de nitive intervention should be
embolization. T is treatment e ectively reduces the risk o bleeding and shunting o oxygenated
blood. Symptoms are likely to recur, but patients may achieve long-term control o bleeding and
ulceration. Individuals o en desire an improvement in their appearance or have ocal problematic
areas ( or example, pyogenic granulomas or overgrowth o speci c structures). T e only way to
improve their appearance or eliminate areas o overgrowth is resection.
Surgical intervention or a di use acial AVM should be ocused on improving the patient’s ap-
pearance with localized staged procedures without causing a signi cant de ormity. It is critical
to avoid acial nerve injury. Facial nerve dissections or parotidectomy should not be per ormed.

160
Part III: Vascular Malformations
When the lesion is throughout all structures o the ace, strategies to improve the area include
(1) excision o excess skin and subcutaneous tissue, (2) removal o the buccal at pad, and/or
(3) contour burring o the zygoma. I overgrowth is located on the lateral cheek, a preauricular
incision is used. Medial ullness is removed through a mesolabial incision. Alternatively, a circular
excision can be used to access the overgrown tissue, and the purse-string closure can be revised
at a second stage. Lip overgrowth is improved with a transverse mucosal resection along the
keratinized and nonkeratinized border o the vermilion. Vertical resections o the lip should be
avoided. Because all surgical planes are a ected by AVMs, subtotal resections o a di use lesion
cause more bleeding than removal o a regional AVM. T e same strategies to reduce blood loss
described or regional AVMs should be carried out or di use lesions.
PATIENT EXAMPLES
l o c a l iz e D a v M
Pa tie n t A
T is 24-year-old man presented with a several year history o a pinkish-red cheek lesion. T e
stain was warm on palpation and exhibited ast f ow by hand-held Doppler examination. Imaging
showed a localized AVM. T e patient was unhappy with the appearance o the stage 1 lesion. T e
AVM was removed to improve his appearance and to prevent the lesion rom progressing to a
higher stage, at which point more complicated reconstruction would be required, resulting in a
less avorable aesthetic outcome. Because the AVM was located on the patient’s cheek, the resec-
tion was per ormed at the boundary o the discolored skin; additional margins were not taken.
T is patient is shown be ore surgery (A). Imaging showed a localized AVM (B and C). T e outline
o the resection without margins is seen (D). T e cheek is shown a er removal o the AVM (E).
Fig. 10-2
D

E
A

B

C

161
Chapter 10: Arteriovenous Malformations
Pa tie n t B
T is 65-year-old woman developed progressive enlargement o her nose, which caused a wors-
ening de ormity. T e area was contoured with local resections rather than radical extirpation,
necessitating reconstruction with regional f aps. T e patient was able to adequately camouf age
the skin discoloration with makeup. T e preoperative appearance o the nose is seen (A and B).
An MRI illustrated the extent o the lesion (C). T e markings or rst-stage resection to debulk
and elevate the ala are shown (D). T e nose is seen be ore second-stage excision o an area o
overgrowth (E and F). T e outline o the tissue or removal is seen (G). Wound closure is shown
(H). She had improved appearance a er surgery (I).
Fig. 10-3
D

E
A

B

C
F
G

H

I

162
Part III: Vascular Malformations
Pa tie n t C
T is 18-year-old girl had a 2-year history o an enlarging upper lip lesion. Physical examination
and hand-held Doppler examination showed ast f ow. Imaging revealed a localized AVM. T e
patient was unhappy with her appearance caused by the stage 2 AVM. A transverse mucosal exci-
sion was per ormed to prevent a scar on the cutaneous lip. Although the AVM involved all layers
o her lip ( or example, the skin, subcutaneous tissue, muscle, mucosa, and submucosa), a subtotal
excision was per ormed, removing redundant vermilion, a portion o the orbicularis muscle, and
submucosa. T e remaining tissues were cauterized. T e incision was closed with interrupted 5-0
Vicryl sutures ollowed by interrupted 6-0 chromic sutures. o prevent dehiscence o the incision,
the patient was prescribed a ull-liquid diet or 2 weeks. T e patient achieved improved contour
and has not had a recurrence 6 years a er surgery. T e preoperative appearance is seen (A). An
angiogram illustrated the AVM (B). An intraoperative view through the transverse mucosal in-
cision is seen (C). T e postoperative result is shown (D).
Fig. 10-4
C

D
A

B

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Chapter 10: Arteriovenous Malformations
Pa tie n t D
T is 32-year-old woman with a capillary mal ormation–AVM developed a gradual overgrowth o
her lower lip. A subtotal excision was per ormed with a transverse mucosal resection. T e preop-
erative view is shown (A). T e MRI delineated the extent o the lesion (B). Intraoperative markings
(C) and appearance a er excision (D) are shown. Her postoperative appearance is seen (E).
Fig. 10-5
C

D
A

B
E

164
Part III: Vascular Malformations
Pa tie n t E
T is 7-year-old boy presented with a stage 3 AVM that was ulcerated, pain ul, and bleeding. It
was removed without margins because o its large size on the scalp; including margins would have
signi cantly reduced the ability to close the wound linearly. Wide subgaleal undermining and
scoring were per ormed to approximate the wound. I I were unable to completely close the area,
I would have allowed a less than 3 cm wide opening to heal secondarily rather than placing a skin
gra or per orming a f ap. I the resulting scar was wide enough to cause alopecia, it could easily
be revised at a second stage. issue expansion should be avoided or AVMs, because it increases
neovascularization to the area and can worsen the AVM. Interrupted 2-0 Ethibond sutures were
placed and removed 4 weeks later to reduce the risk o wound dehiscence and scar widening
(suture marks are hidden in the scalp). T e patient has not had a recurrence 2 years a er the pro-
cedure. T e preoperative appearance is seen (A). An MRI and angiogram illustrated the AVM (B
and C). T e intraoperative view be ore resection is shown (D). T e wound is seen a er excision
(E). Linear wound closure is illustrated (F). T e postoperative appearance o the scalp is seen (G).
Fig. 10-6
D

E
A

B

C
F

G

165
Chapter 10: Arteriovenous Malformations
Pa tie n t F
T is 20-year-old man had a stage 1 AVM o the scalp that was removed prophylactically be ore it
progressed to cause morbidity. T e preoperative appearance is seen (A). An MRI and angiogram
showed the lesion (B and C). T e outline o resection without margins is seen (D). T e appearance
a er excision is shown (E). Linear wound closure is illustrated (F). T e center o the incision was
not completely approximated; this was allowed to contract and heal secondarily.
Fig. 10-7
A
B

C
D

E

F

166
Part III: Vascular Malformations
r e g io n a l a v M
Pa tie n t A
T is 13-year-old girl presented with a stage 2 AVM o the ear, neck, and scalp. T e lesion was
present at birth and began to enlarge during puberty, causing a prominent ear de ormity. She was
unhappy with the appearance o her ear. Complete removal o the AVM would require amputation
o the auricle, resulting in a much worse de ormity than the appearance o the lesion. Conse-
quently, she underwent a subtotal resection o the postauricular overgrown area and conchal bowl
resection to set back the ear. She has not had a recurrence 3 years a er surgery. T e preoperative
appearance is seen (A through C). T e MRI and angiogram exhibited the regional AVM (D and
E). T e postoperative result is shown (F and G).
Fig. 10-8
D

E
A

B

C
F

G

167
Chapter 10: Arteriovenous Malformations
Pa tie n t B
T is 21-year-old man presented with an enlarging and increasingly pain ul right ear. T e patient
is shown be ore surgery (A and B). An MRI illustrated the extent o the AVM (C). An angiogram
showed di use shunting and nidus (D). Subtotal excision o the AVM was per ormed with a
posterior approach (E). Overgrown conchal cartilage was resected to decrease the prominence
o the ear (F). T e ear is seen a er repair o the cartilage (G). T e postoperative appearance is
shown (H and I).
Fig. 10-9
D

E
A

B

C
F
G

H

I

168
Part III: Vascular Malformations
Pa tie n t C
T is 13-year-old girl was unhappy with the appearance o her right upper lip. At an outside hos-
pital she was managed with embolization, which caused signi cant worsening o the lesion. T e
patient is shown be ore (A) and 6 months a er embolization (B). T e markings or rst-stage
transverse mucosal resection are seen (C). T e lip is seen a er excision (D) and wound closure
(E). T e lip is shown a er second-stage transverse mucosal excision (F).
Fig. 10-10
C

D
A

B
E

F

169
Chapter 10: Arteriovenous Malformations
Pa tie n t D
T is 39-year-old woman with a stage 3 AVM o the orehead and scalp developed pain, bleeding,
and ulceration. T e lesion involved the skin, subcutaneous tissue, galea, and rontalis muscle.
Because the entire AVM was causing morbidity, she underwent complete extirpation with min-
imal (about 1 mm) margins. T e rontalis muscle and periosteum were not removed. T e area
was reconstructed with a split-thickness skin gra to ensure maximum contraction and a smaller
de ect to reconstruct i she desired later removal o the gra . issue expansion was not chosen
initially because: (1) it could stimulate the AVM, and (2) there is a signi cant risk o complications
i expanders are placed adjacent to an ulcerated, bleeding AVM. T e patient was able to camou-
f age the skin gra with her hair and was not interested in urther reconstruction to remove the
gra ( or example, tissue expansion or a ull-thickness skin gra ). She has not had a recurrence o
the lesion 5 years a er surgery. T e preoperative appearance is seen (A). An MRI and angiogram
showed the AVM (B and C). Intraoperative views be ore and a er resection are shown (D and
E). A split-thickness skin gra was harvested rom the buttock and applied to the wound (F). T e
bolster was a vacuum-assisted wound closure device. T e early appearance o the skin gra (G)
and healed gra (H) are shown. T e gra was camouf aged by the patient’s hair (I).
Fig. 10-11
D

E
A

B

C
F
G

H

I

170
Part III: Vascular Malformations
Pa tie n t E
T is 10-year-old girl developed a bleeding, ulcerated, pain ul stage 3 AVM o the lower extrem-
ity. Preoperative imaging showed that the lesion was primarily in the skin and subcutaneous
tissues but that the underlying muscle ascia and muscles might be involved. A wide resection
with minimal margins was per ormed, and the wound was reconstructed with a split-thickness
skin gra (harvested rom the contralateral buttock) to maximize take and contraction. Be ore
the gra was placed, as much o the wound as possible was closed linearly to reduce the area that
needed to be gra ed. T e gra did not take, likely because the recipient site contained an AVM
and was ischemic, unable to vascularize the gra (although the recipient site appeared grossly
normal intraoperatively). Instead o attempting to regra the wound, an external tissue expansion
device was placed, and the area healed by delayed primary closure. She has not had a recurrence
3 years a er the procedure. T e preoperative appearance is shown (A). An MRI and angiogram
illustrated the AVM (B and C). T e wound is seen a er extirpation (D). Approximately 50% o
the area was closed linearly, and the remaining wound was reconstructed with a split-thickness
gra (E). T e appearance o the wound a er ailure o the skin gra is seen (F). External wound
closure device is shown (G). T e postoperative result is shown (H).
Fig. 10-12
D

E
A

B

C
F
G

H

171
Chapter 10: Arteriovenous Malformations
Pa tie n t F
T is 13-year-old boy presented with a stage 3 AVM o the oot. Imaging showed that the lesion
involved the skin and subcutaneous tissues. T e area was resected with 1 to 2 mm margins. T e
wound could not be gra ed because o the exposed tendons and un avorable location or a thin
gra . T e area was reconstructed with an anterolateral thigh ree f ap. T e preoperative appearance
o the oot is seen (A). An MRI illustrated the lesion (B). T e removed specimen is shown (C). T e
de ect to be reconstructed is seen (D). Donor-site harvest is shown (E and F). T e transplanted
f ap is seen (G). T e appearance o the oot at the end o the procedure is shown (H).
Fig. 10-13
A

B

C

D
E

F

G

H

172
Part III: Vascular Malformations
Pa tie n t G
T is 15-year-old boy had a bleeding, ulcerated, pain ul stage 3 AVM o the ace. Grossly, the
pinkish-red anterior hal o the ear appeared involved. Because removing a signi cant part o his
ear ( or example, the root o the helix, tragus, anterior lobule, antitragus, and part o the concha)
would cause a major de ormity, it was not excised. A parotidectomy and deeper resection, which
would have risked acial nerve injury, were not per ormed. A er extirpation o the cheek lesion,
the ear returned to a normal color, and thus its initial red appearance was likely rom inf amma-
tion and/or hyperemia rom the adjacent AVM. He has not had a recurrence 3 years a er surgery.
T e appearance be ore treatment is shown (A). An angiogram be ore preoperative embolization
is illustrated (B). Reduced shunting and nidus a er embolization can be seen (C). Intraoperative
markings with small margins are seen (D). T e resected specimen (E) and de ect to reconstruct
(F) are shown. Anterolateral thigh ree f ap harvest is illustrated (G and H). Early and late post-
operative results are seen (I and J).
Fig. 10-14
D

E
A

B

C
F
G

H

I

J

173
Chapter 10: Arteriovenous Malformations
Dif f u s e a v M
Pa tie n t A
T is 11-year-old boy developed a stage 2 AVM involving most o the structures o the le side o
the ace. Wide resection with a ree tissue trans er would have le a worse de ormity than the
lesion. He underwent a subtotal resection to improve his worsening appearance. Within 12 months
he had a recurrence and worsening o the lesion. T e patient is shown be ore surgery (A). An
angiogram and MRI illustrated the AVM (B and C). T is patient had improved appearance a er
subtotal resection o the chin and cheek through a submental incision (D). Postoperative pro-
gression o the lesion is seen (E and F).
Fig. 10-15
D

E
A

B

C
F

174
Part III: Vascular Malformations
Pa tie n t B
T is 51-year-old woman presented with a stage 2 AVM o the le cheek and was unhappy with her
appearance. T e lesion was located throughout anatomically sensitive structures. She underwent a
subtotal resection with an intraoral and preauricular incision. An attempt was not made to do an
aggressive dissection with acial nerve monitoring. Instead subcutaneous and submucosal tissue
was removed under direct vision, and an AVM involving the acial muscles was not manipulated.
Postoperatively she had improved appearance and has not had a recurrence 3 years a er surgery.
T e patient is shown be ore surgery (A and B). An MRI showed the AVM (C and D). T e post-
operative result is shown (E and F). T e preauricular incision is visible (G).
Fig. 10-16
C

D
A

B
E

F

G

175
Chapter 10: Arteriovenous Malformations
Pa tie n t C
T is 12-year-old girl had a stage 2 AVM o the right side o the ace. Because o the signi cant skin
excess and medial location o the lesion, I per ormed a direct excision instead o using a preauric-
ular incision. With a circular incision I accessed the entire overgrown cheek and removed tissue
sa ely under direct vision. In addition, the resection o skin over the most prominent area also
helped to reduce the size o her cheek. T e wound was closed with a purse-string suture to limit
the length o the scar. She may bene t rom urther procedures to convert the circular scar into
a line, as well as possible buccal at pad removal and contour burring o her zygoma. An attempt
was not made to do a parotidectomy or aggressive resection that would injure her acial nerve.
She is shown be ore surgery (A and B). An MRI and an angiogram exhibited a di use AVM (C
and D). T e intraoperative markings are seen (E). T e outer line is the extent o the subcutaneous
excision, and the inner circle is the area o skin to be resected. T e intraoperative appearance o
the circular excision is seen (F). T e appearance is seen af er wound closure (G). T e early post-
operative result is shown (H and I).
Fig. 10-17
A

B
C
D
E

F
G

I
H

176
Part III: Vascular Malformations
Pa tie n t D
T is 21-year-old woman with a capillary mal ormation–AVM developed progressive acial over-
growth. Preoperative views are shown (A and B). An MRI showed the extent o the lesion (C). An
angiogram illustrated di use shunting (D). First-stage resection o skin and subcutaneous tissue
with a mesolabial incision is seen (E). During this procedure the patient also underwent removal
o the buccal at pad, contour burring o the zygoma, and transverse mucosal resection o the lips.
Second-stage resection o subcutaneous tissue and skin with a circular excision is shown (F). T e
outer marking illustrates the extent o the subcutaneous resection. T e inner circle delineates the
skin excision.
Fig. 10-18, A-F
C

D
A

B
E

F

177
Chapter 10: Arteriovenous Malformations
T e appearance a er removal o skin is seen (G). Wide undermining in a 360-degree direction
allowed access to most o the subcutaneous tissue o the right side o the ace (H). T e resected
specimen is seen (I), along with purse-string closure o the wound (J). T e patient is shown 3
months a er surgery (K and L).
Fig. 10-18, G-L
I

J
G

H
K

L

178
Part III: Vascular Malformations
Pa tie n t E
T is 28-year-old woman presented with a stage 3 AVM o the le side o the ace. Because she had
an indentation o the le cheek with telangiectasias, I per ormed a resection o this abnormal area
to improve her cheek contour, eliminate part o the acial discoloration, and remove subcutaneous
tissue rom her upper lip. In addition, I reduced and li ed her upper lip with a transverse mucosal
resection. She had an improvement in her appearance and has not had a recurrence o the AVM
4 years a er surgery. T e patient is shown be ore surgery (A through C). An MRI and angiogram
illustrated the AVM (D and E). T e patient is seen a er surgery (F through H).
Fig. 10-19
D

E
A

B
F

G

H
C

179
Chapter 10: Arteriovenous Malformations
Pa tie n t F
T is 29-year-old man had a stage 3 AVM o the right side o the ace. He had an ulcerated, bleed-
ing, and pain ul upper lip. Because o the signi cant lip overgrowth, I per ormed a vertical wedge
resection o his upper lip. He has not had a recurrence o the lip area 8 years a er surgery. T e
preoperative appearance is shown (A). An MRI and angiogram illustrated the extent o the AVM
(B and C). T e postoperative result is seen (D).
COMPLICATIONS
Because AVMs are highly vascular and ischemic, the two most common complications are bleed-
ing and wound-healing problems. Postoperatively the surgical site should have a pressure dressing
applied. Pain is aggressively managed so that the patient’s blood pressure is not elevated, which
would increase the risk o a hematoma. When a surgeon per orms a subtotal resection that re-
quires the approximation o ischemic tissue containing an AVM, interrupted sutures should be
used and le in place or a prolonged period until the wound has gained strength. An interrupted
suture is less ischemic than a continuous suture and reduces the risk o suture line dehiscence.
Fig. 10-20
C

D
A

B

180
Part III: Vascular Malformations
Intraoperative antibiotics are given when operating on regional and di use AVMs, because the
ischemic surgical site is at higher risk o in ection. Generally postoperative antibiotics are not
administered. AVMs treated with preoperative embolization have increased ischemia o the area.
I possible, operating around sensitive structures ( or example, a acial nerve) should be avoided,
because the risk o iatrogenic injury is high. Unlike other types o lesions, an AVM may have sig-
ni cant intraoperative bleeding and is an in ltrative lesion. I possible, wounds should be closed
with local tissue or vascularized f aps. A skin gra placed over an area containing an AVM has a
high ailure rate, because the recipient site is ischemic and unable to revascularize the gra .
CONCLUSION
A treatment algorithm or an AVM is shown.
AVMs are problematic and do not mandate intervention. Surgical treatment is individualized
based on stage, age, location, and size. Localized AVMs should be excised or cure, i possible.
Regional AVMs may be managed with subtotal resections; complete extirpation typically requires
distant tissue to reconstruct the surgical de ect. Di use AVMs should undergo subtotal resections
to improve the most symptomatic areas. T e de ormity caused by excision and reconstruction
should not be worse than the appearance o the AVM. Patients with an AVM are best managed
in an interdisciplinary vascular anomalies center ocused on these lesions.
SUGGESTED READINGS
Greene AK. Arteriovenous mal ormation. In Greene AK, ed. Vascular Anomalies: Classi cation, Diagnosis, and
Management. St Louis: Quality Medical Publishing, .
Greene AK, Orbach DB. Management o arteriovenous mal ormations. Clin Plast Surg : , .
Kohout MP, Hansen M, Pribaz JJ, et al. Arteriovenous mal ormations o the head and neck: natural history and
management. Plast Reconstr Surg : , .
Liu AS, Mulliken JB, Zurakowski D, et al. Extracranial arteriovenous mal ormations: natural progression and recur-
rence a er treatment. Plast Reconstr Surg : , .
T iex R, Wu I, Mulliken JB, et al. Sa ety and e cacy o onyx embolization or extracranial head and neck and vas-
cular anomalies. AJNR Am J o Neuroradiol : , .
Wu JK, Bisdor A, Gelbert F, et al. Auricular arteriovenous mal ormation: evaluation, management, and outcome.
Plast Reconstr Surg : , .
Wu IC, Orbach DB. Neurointerventional management o high-f ow vascular mal ormations o the head and neck.
Neuroimag Clin N Am : , .
STAGES 1 AND 2
STAGES 3 AND 4
Resection
Embolization
(palliation)
Localized
favorable area
Diffuse
unfavorable area
Resection
Observation
Fig. 10-21

Par t IV
An at o mic Co n sid er at io n s

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