Phẫu Thuật Quản Lý Và Điều Trị Dị Thường Mạch Máu

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Part IV: Anatomic Considerations
T e signi cant morbidity and mortality associated with intracranial hemorrhage typically avor
treatment o AVMs or CMs when possible, but the calculus o management can be complex. Risk
o iatrogenic injury, variable natural history, and a variety o treatment options ( or example, ra-
diation, surgery, and in AVMs, embolization) contribute to an active, constantly evolving debate
centered on providing the best evidence-based plan o care or patients with these lesions. T is
chapter ocuses solely on the surgical management o AVMs and CMs (more detailed papers on
multimodality care are ound in the Suggested Readings section at the end o this chapter).
ARTERIOVENOUS MALFORMATIONS
AVMs consist o direct arterial-to-venous connections without intervening capillaries; they occur
in the cerebral hemispheres, brainstem, and spinal cord. Functional neural tissue does not reside
within the lesion. Anomalies range rom simple arteriovenous stulas to complex tangled channels
connecting enlarged eeding arteries to draining veins. AVMs are arguably the most important
vascular anomaly in the nervous system in children. T ey are relatively common and usually
require treatment, given the risk o hemorrhage (associated with a 25% risk o death with each
bleeding event; they can also cause other symptoms, including seizure, ocal neurologic de cits
(ischemic “steal” phenomenon), and headache.
SURGICAL INDICATIONS
T e treatment goal is complete removal/obliteration o the lesion. T e decision to operate on an
AVM is based on several actors:
• Eloquence o cortical location (speech, motor unction, and sensation)
• Pattern o venous drainage
• Size
• Associated aneurysms
• Recent hemorrhage
• Clinical deterioration
• Risk o complication rom other modalities o therapy ( or example, radiation injury to the
developing brain)
Several o these actors are combined in the Spetzler-Martin AVM grading scale that incorpo-
rates eloquence o location, pattern o venous drainage, and size and is considered predictive o
outcome rom surgical management. T e Spetzler-Martin grade helps to predict surgical risk. I a
low-grade lesion (grades 1 to 3) is present, surgery should be considered. T is includes incidental,
asymptomatic lesions in select cases; recent data support this more proactive approach in younger
patients. Higher-grade lesions (grades 4 and 5) o en bene t rom a multidisciplinary approach
and may be considered or radiotherapy.

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Chapter 11: Central Nervous System
SURGICAL MANAGEMENT
Initial therapeutic maneuvers are dependent on the presentation o the child. For the healthy child
or the child who presents with chronic symptoms ( or example, seizure or developmental delay),
no immediate interventions are o en necessary (with the exception o antiepileptic medication
i seizures are present). T e ollowing steps are warranted or the child who presents with an
intracranial hemorrhage. T e severity o presentation can vary greatly, and thus treatment must
be individually tailored.
s t a b il iz at io n
• Vascular access: A large-bore (at least 2) intravenous arterial line, bladder catheter (airway
intubation i unable to protect airway), and nasogastric tube i intubated can be used.
• Blood pressure control: Antihypertensives such as labetalol or nitroprusside (Nipride) can be
used to control blood pressure, with a goal o normotension or the child’s age.
• Intracranial pressure control: An external ventricular drain can be placed i hydrocephalus
is present (NB: T e surgeon should avoid overdrainage o CSF to prevent rerupture, o en no
more than 5 ml at a time) and the head o the bed is elevated.
• Avoidance o seizures: Antiepileptic medication can be given i concern exists regarding seizure.
Pr e o Pe r at iv e Pr e Pa r at io n
• Prepare the operating room: In addition to the steps noted in the “Stabilization” section, the
operating room should be noti ed to prepare or surgery. Equipment should include the oper-
ating microscope, multiple suctions, bipolar electrocautery, an array o AVM/aneurysm clips,
a craniotome (drill), and a retraction system.
• Consider preoperative embolization: T e surgeon should consider this in select cases to reduce
the risk o bleeding.
• Consult with the anesthesiologist: Anesthesia should be consulted, and the appropriate mea-
sures should be taken to ensure that multiple, large-bore intravenous access is present and
adequate blood products are in the operating room.
• Prepare the equipment or microsurgery: I possible, the microscope should be draped and
the clips prepared be ore starting the case, so that quick access can be obtained i unexpected
bleeding occurs during opening.
Table 11-1 Spetzler-Martin Arteriovenous Mal ormation Grading Scale
Size
0-3 cm
> 3-6 cm
> 6 cm
1
2
3
Location
Noneloquent
Eloquent
0
1
Deep venous drainage
Not present
Present
0
1

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Part IV: Anatomic Considerations
Pa t ie n t Po s it io n in g
• Position the patient according to the location o the AVM: Patient positioning is dependent on
the location o the AVM and will vary widely. General principles include elevation o the head
to maximize venous drainage, avoiding kinking o vessels in the neck, and maximizing access
to the lesion by both the primary surgeon and the assistant.
• Head xation: Rigid head xation is pre erable, although in the very young child (under 2 years
o age), it may be necessary to use a padded headrest.
s u r g ic a l a PPr o a c h
• Varies with location o the AVM: Given the wide variety o AVM sizes and locations, it is impos-
sible to identi y any single approach or AVM surgery. Speci c approaches should be selected to
maximize access to the lesion, avoid the eloquent neurologic cortex (i possible), and a ord the
surgeon visualization o eeding and draining vessels to permit proximal control o blood f ow.
• Opening: T e approach or any AVM will vary greatly rom case to case, but general principles
should include a generous opening to allow access and visualization o relevant anatomy and a
care ul dural opening to avoid injuring AVM vessels (especially draining veins).
r e s e c t io n t e c h n iq u e
• Cauterize eeding arteries rst: A primary surgical principle or AVM resection is the oblitera-
tion o eeding arteries be ore occlusion o draining veins, because premature closure o outf ow
can lead to unexpected AVM rupture with uncontrolled bleeding.
• Work in a circum erential pattern, maintaining even depth: AVMs are o en wedge or cone
shaped, and resection can be per ormed in a circum erential pattern, staying close to, but not
entering, the nidus. T e surgeon should try to maintain an even depth o resection around the
lesion to avoid getting in a “hole,” and the surgeon must be care ul to minimize retraction on
draining vessels during dissection.
• Watch the surrounding brain: Repeated inspection o the surrounding brain or swelling or
bleeding can help the surgeon to prevent complications by early identi cation o poorly placed
retractors or clips.
• Clip versus coagulation o eeding vessels: AVM vessels may coagulate poorly, and the surgeon
should consider clip application or gentle tamponade (i the bleeding is o small volume) i
bipolar electrocautery is not working. Every attempt should be made to avoid operating within
the nidus itsel .
c l o s u r e
• Inspect the resection bed: Inspection o the operative cavity or residual nidus is important, and
perioperative angiography can be a use ul adjunct to ensure complete resection.
• Check or brain swelling: Evidence o brain swelling at closure may indicate occult bleeding,
untreated hydrocephalus, or poorly compensated redistribution o blood f ow, which can result
in per usion breakthrough hemorrhage. Causes or swelling should be thoroughly investigated
and treated i possible be ore the patient leaves the operating room.

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Chapter 11: Central Nervous System
PATIENT EXAMPLE
T is 13-year-old girl presented with acute onset o a headache, vomiting, and subsequent obtun-
dation, with right hemiparesis. Emergent imaging revealed a large le rontal intraparenchymal
hemorrhage and an AVM (A) (asterisk). Subsequent AP projection on an arteriogram with injec-
tion o the internal carotid artery showed a large, high-f ow rontal AVM (B) (arrow). A dilated
and arterialized draining vein is visible (C). T e method o circum erential dissection around
the AVM is seen, with preservation o the draining vein until the nal portion o the case (D).
A reduction in caliber o the draining vein and a change to the stagnant, deoxygenated venous
blood a er interruption o the arterial supply are seen. A er this initial stabilization, the patient
was managed with emergent clot evacuation (taking care to avoid the AVM), ollowed by emboli-
zation and de nitive resection at a later date. T e patient ultimately made a good recovery, with a
moderate residual right hemiparesis and some limited expressive aphasia at 1 year postoperatively.
Fig. 11-1
C

D
A

B

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Part IV: Anatomic Considerations
COMPLICATIONS
• Bleeding is the most immediate complication o surgery, and the risks are magni ed in smaller
children, who have little reserve. T e loss o one quarter o blood volume can induce shock, and
rapid decompensation may occur in children, who require care ul monitoring and replacement
o blood products by the surgical team.
• Normal per usion pressure breakthrough is a phenomenon that is thought to occur a er re-
section o high-f ow AVMs. T e blood previously transmitted through the AVM is redirected
to smaller, normal vasculature a er the AVM has been removed, with subsequent inability o
the vessels to handle the increased f ow. T is can result in brain swelling, increased intracranial
pressure, seizure, neurologic dys unction, or hemorrhage. T e problem may be minimized by
staged preoperative embolization and rigorous postoperative blood pressure control.
• Inadvertent occlusion o a normal cerebral artery can occur a er embolization or surgery. In
this situation the blood pressure should be increased temporarily to improve collateral supply
to the ischemic region. T is maneuver does not appear to be associated with increased risk o
rupture o the AVM.
• Neurologic de cit can occur a er AVM resection, although speci c rates are hard to derive,
given the wide variability in AVM size and location. Overall there is low postoperative morbid-
ity in low-grade (grades 1 to 3) Spetzler-Martin lesions (ranging rom 0% to 12%), along with
a high rate o complete obliteration (up to 100%). T is suggests that surgical resection o these
lesions is warranted, especially when per ormed in experienced centers.
CAVERNOUS MALFORMATIONS
Cerebral CMs consist o compact clusters o spongelike vascular spaces without intervening neu-
ral parenchyma. T e nomenclature or these mal ormations can be con using, because they have
been called cavernomas, cavernous angiomas, and cavernous hemangiomas. VMs are a conf uent
collection o enlarged veins, whereas a developmental venous anomaly (DVA) is a collection o
small veins converging into a dilated venous trunk. Neither VMs nor DVAs will be discussed
urther here, because they are not generally pathologic or causative o disease and as a rule should
not be treated.
CMs can cause symptoms by hemorrhage, growth with mass e ect, or irritation to surrounding
neural tissue with seizure or ocal neurologic de cits. Symptoms o en depend on the location
o the lesion within the CNS. As the presenting symptom, seizure is ound in 25% to 30% o pa-
tients. However, many CMs are asymptomatic and are requently ound incidentally. reatment
is predicated almost wholly on surgical resection (or observation), with extremely rare cases
considered or radiosurgery.
SURGICAL INDICATIONS
As with AVMs, the treatment goal or CMs is complete removal o the lesion. Symptomatic lesions
are resected when easible, whereas asymptomatic lesions remain more controversial. T e patient
who presents with symptoms and has multiple lesions is equally di cult. I the symptoms can be

189
Chapter 11: Central Nervous System
localized to a single lesion, which is amenable to surgical resection, that lesion should generally
be removed. Recent literature suggests that a reasonable practice is to surgically resect single CMs
when they are located in noneloquent cortex or the spinal cord i the lesion presents with:
• Symptoms
• Documented radiographic enlargement
• Hemorrhage
For lesions in eloquent cortex or the brainstem, it may be prudent to observe the lesion initially
to determine i it mani ests a pattern o recurrent hemorrhage that justi es the risk o surgical
intervention. I hemorrhage recurs, resection is considered.
SURGICAL MANAGEMENT
Initial therapeutic maneuvers are dependent on the presentation o the child, but typically the
course o CM presentation is less severe than that o AVM because o the absence o arterial bleed-
ing in CM. Consequently, surgical resection can o en be delayed several weeks to allow swelling
to diminish and blood to resorb, a ording the surgeon a more relaxed operative eld and better
de nition o surgical planes.
Pr e o Pe r at iv e Pr e Pa r at io n
• Equipment should include the operating microscope, multiple suctions, bipolar electrocautery, a
craniotome (drill), and a retraction system. For CMs, the use o intraoperative ultrasonography
and rameless stereotaxy may be especially use ul.
• Patient positioning and surgical approach are identical to that or AVMs.
r e s e c t io n t e c h n iq u e
• Localization o CMs may be di cult i small or deep seated. Frameless stereotaxy and intra-
operative ultrasonography may be help ul to success ully navigate to the lesion. In addition,
placement o a catheter along the planned trajectory o approach a er opening the dura can
be help ul as a guide to the lesion during dissection.
• Preservation o normal surrounding vasculature, especially associated DVAs, is important to
minimize iatrogenic injury.
• Removal o the surrounding hemosiderin ring, i the lesion is cortical and the patient has
associated seizures, may improve long-term seizure control. However, i the CM is located in
eloquent cortex, brainstem, or spinal cord, surgery should consist solely o lesional resection
without removal o sensitive surrounding tissue (even i stained with hemosiderin).
• T e use o gentle coagulation on the sur ace can o en help to reduce the bulk o the lesion
while preserving integrity. T e use o gentle dissection around the margins o the lesion with
microdissectors will help to tease the mal ormation out o surrounding brain tissue and de ne
dissection planes.
• Care ul inspection o the surgical cavity, ideally with an operating microscope at the conclusion
o the case, is important. Small ragments o lesional tissue can easily be overlooked, especially
i embedded in hemosiderin-stained tissue or when obscured by blood. Removal o the entire
lesion will minimize the chance o postoperative recurrence.

190
Part IV: Anatomic Considerations
PATIENT EXAMPLE
T is teenage boy presented with a headache, and a preoperative axial 2-weighted MRI revealed
a CM o the right rontal lobe near the oramina o Monro (A) (asterisk). Given the hemorrhage
seen on the MRI, along with the symptomatic presentation and risk o acute obstructive hy-
drocephalus with uture rebleeding, the decision was made to resect the lesion. Intraoperative
rameless stereotaxy and ultrasonography were used to nd a deep-seated CM (B). A catheter
was passed to the CM to provide intraoperative guidance directly to the deep-seated lesion (C).
T e CM was revealed, which was readily exposed with a minimal opening because o accuracy
o catheter localization (D) (asterisk). A postoperative MRI demonstrated complete resection o
the lesion, with a small hemosiderin-stained residuum at the oramina o Monro (E). T us the
CM was success ully resected, and the child made an unevent ul recovery.
Fig. 11-2
C

D
A

B
E

191
Chapter 11: Central Nervous System
COMPLICATIONS
Perioperative risks generally correlate with location o the CM. Lesions in eloquent tissue, brain-
stem, or spinal cord typically have higher rates o neurologic de cits a er surgery. Recurrence can
occur, particularly with larger or deep-seated lesions, likely because o limited visualization o the
margins at surgery. Seizure may persist or recur, particularly i cortical hemosiderin-stained tissue
remains. T e surgeon should be particularly aware o the risk o causing venous hemorrhage,
resulting rom inadvertent obliteration o a DVA in proximity to a CM.
CONCLUSION
AVMs and CMs are the most common symptomatic vascular lesions in the CNS o children.
Surgery should be o ered as rst-line therapy or symptomatic or growing lesions in accessible
regions o the CNS, and strong consideration should be given to resection o asymptomatic lesions
i easible. Care ul operative planning and meticulous surgical technique are necessary to ensure
the success ul treatment o these mal ormations.
SUGGESTED READINGS
Gross BA, Scott RM, Smith ER. Management o brain arteriovenous mal ormations. Lancet : , .
Gross BA, Smith ER, Goumnerova L, et al. Resection o supratentorial lobar cavernous mal ormations in children:
clinical article. J Neurosurg Pediatr : , .
Gross BA, Storey A, Orbach DB, et al. Microsurgical treatment o arteriovenous mal ormations in pediatric patients:
the Boston Children’s Hospital experience. J Neurosurg Pediatr : , .
Korja M, Bervini D, Assaad N, et al. Role o surgery in the management o brain arteriovenous mal ormations: pro-
spective cohort study. Stroke : , .
Roach ES, Golomb MR, Adams R, et al; American Heart Association Stroke Council; Council on Cardiovascular
Disease in the Young. Management o stroke in in ants and children: a scienti c statement rom a Special Writ-
ing Group o the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the
Young. Stroke : , .
Spetzler RF, Martin NA. A proposed grading system or arteriovenous mal ormations. J Neurosurg : , .

(Trang không có chữ)

193
Arin K. Greene, Cory M. Resnick, Bonnie L. Padwa
Vascular anomalies involve bone much less commonly than so tissues. In the cranio acial region
capillary, venous, and lymphatic mal ormations most requently a ect osseous structures. Other
vascular anomalies that can involve bone include arteriovenous mal ormations (AVMs) and rare
vascular tumors ( or example, epithelioid hemangioendotheliomas and kaposi orm hemangio-
endotheliomas). Major vascular anomalies that do not a ect bone are in antile hemangiomas,
congenital hemangiomas, and pyogenic granulomas.
SURGICAL INDICATIONS
Vascular anomalies o the cranium and acial bones are rarely malignant, and thus treatment is
usually not mandatory. T e primary morbidity is psychosocial, because lesions can cause a visible
de ormity. Other problems include occlusal cant, malocclusion, exophthalmos, enophthalmos, and
Cr an io f ac ial Bo n es
Ch a Pt er 12
KEY POINTS
• Vascular anomalies involving the craniofacial bones are relatively uncommon.
• Lesions do not always require treatment; intervention is based on the type of vascular anom-
aly and symptoms.
• Bone overgrowth is managed differently than bone loss.
• Treatments available for osseous vascular anomalies include sclerotherapy, embolization,
vincristine, sirolimus, interferon, bisphosphonates, resection, and bone grafting.
• Patients with an osseous vascular anomaly are best managed in a vascular anomalies center
by physicians who are focused on these lesions.

194
Part IV: Anatomic Considerations
cranial de ects. Be ore considering intervention, the diagnosis must be con rmed. MRI and/or C
are per ormed to identi y the lesion, determine the extent o disease, and develop a management
plan. Vascular anomalies can cause either osseous hypertrophy or loss. Physical examination
and imaging will determine whether the patient has increased or decreased bone. reatment o
overgrowth is reduction by contour burring or osteotomies. Management o hypoplasia includes
intralesional injection o sclerosants, embolization, systemic pharmacotherapy, bone replacement,
and/or osseous augmentation.
I an individual has an obvious de ormity, improvement o the area can be per ormed be ore
4 years o age when long-term memory and sel -esteem begin to orm. Early skeletal intervention,
however, may be associated with a higher rate o recurrent de ormity compared with delayed
treatment. Another period to consider intervention or psychosocial morbidity is when the patient
is older and requests surgery. Correction o a malocclusion or an occlusal cant is typically not
initiated until completion o acial growth (16 years old in emales and 18 years old in males). I a
vascular anomaly o the ace or cranium is causing signi cant symptoms ( or example, bleeding,
in ection, pain, and threatening vital structures), intervention is necessary independent o the
age o the patient (see Video 12-1, Cranio acial Bones).
SURGICAL MANAGEMENT
c a Pil l a r y Ma l f o r Mat io n
A capillary mal ormation is the most common type o vascular mal ormation and requently
a ects the head and neck. So tissue and bone underneath the cutaneous stain can hypertrophy
over time; the mechanism is unclear. Facial lesions can cause overgrowth o the maxilla and/or
mandible that may result in asymmetry, occlusal cant, or malocclusion. A LeFort I osteotomy can
improve vertical maxillary overgrowth; other orthognathic procedures may be necessary to cor-
rect occlusion or mandibular enlargement. In some cases orthodontic intervention can improve
the occlusion to maximize masticatory unction until children have completed acial growth and
are candidates or orthognathic procedures. Overgrowth o the acial bones causing a de ormity
can be corrected by burring the cortex to reduce the projection o the area at any age. Although
capillary mal ormation o the scalp may cause hypertrophy o the underlying cranium, treatment
is rarely necessary, because the area is camouf aged by hair.
v e n o u s Ma l f o r Ma t io n
A venous mal ormation causes bone destruction and ultimately hypoplasia. T e most common
location o an intraosseous venous mal ormation is the cranio acial region. Bony involvement
can occur in combination with adjacent so tissue disease, or the bone can be a ected primarily.
In the literature intraosseous venous mal ormations have been erroneously called hemangiomas.
(in antile hemangiomas do not involve bone). Venous mal ormations o the cranio acial region
typically cause osteolysis, with a spongy, sunburst-like expansion o the bone.

195
Chapter 12: Craniofacial Bones
An osseous venous mal ormation does not require intervention unless the lesion is symptomatic.
Management includes sclerotherapy, resection, or orthognathic surgery. Generally sclerotherapy
is our pre erred treatment, because it is less morbid than resection. Many lesions will respond
avorably and not require urther intervention. I sclerotherapy is not possible or ails, the area
is excised. Cranial lesions undergo ull-thickness calvarial resection with minimal margins; the
de ect is usually lled with autologous bone gra (especially in the pediatric population). Recon-
struction o the cranium with alloplastic materials ( or example, porous polyethylene) is generally
reserved or adults who have completed cranial growth. Venous mal ormations o the acial bones
( or example, the zygoma, maxilla, and mandible) are most commonly reconstructed with bone
gra s rom the cranium. I the venous mal ormation is primarily intraosseous without adjacent
so tissue involvement, recurrence is uncommon a er resection. In contrast, i a signi cant so
tissue component is present, urther procedures to treat the underlying bone gra s may be neces-
sary. Orthognathic intervention can be required i a venous mal ormation causes a malocclusion.
l y MPh at ic Ma l f o r Ma t io n
A lymphatic mal ormation can cause either bone overgrowth or loss. T e most common osseous
site o a lymphatic mal ormation is the cranio acial region. Isolated macrocystic or microcystic
lymphatic mal ormations involving so tissues and bone can cause hypertrophy. Osteolysis and
marginal sclerosis are illustrated on radiographic examination; solitary spaces less than 2 cm
may be present. Lesions o the scalp may result in a prominence o the cranium that may not be
camouf aged by hair. T e area can be f attened by burring the ectocortical sur ace. A lymphatic
mal ormation o the maxilla or mandible can lead to acial asymmetry, occlusal cant, and/or
malocclusion. Ostectomy or orthognathic procedures may be necessary.
Gorham-Stout disease and generalized lymphatic anomaly are anomalies that primarily a ect
bone. Gorham-Stout (“disappearing bone disease”) causes osteolysis o contiguous sites. T e bone
resorbs, resulting in pain, ractures, and signi cant morbidity. Imaging shows progressive osteoly-
sis and cortical loss. T e ribs are most commonly involved, ollowed by the cranium, clavicle, and
cervical spine. Almost all lesions have an adjacent so tissue lymphatic mal ormation. Patients are
treated with weekly subcutaneous inter eron and monthly intravenous bisphosphonates. Recently
oral sirolimus has shown e cacy. T ese drugs may prevent continued bone loss but rarely cause
remineralization. Severe morbidity can require surgical intervention with bone gra s that have a
high resorption rate, because they are placed into a diseased recipient site.
Generalized lymphatic anomaly (“lymphangiomatosis”) is a multisystem disorder a ecting non-
contiguous sites. Osseous lesions have discrete lytic areas and radiolucencies con ned to the
medullary cavity. T e number and size may increase, but progressive osteolysis and cortical loss
do not occur. Approximately one hal o individuals have a so tissue lymphatic anomaly at the
site o bone involvement. Patients can also have lesions o the spleen or liver and pleural e usions.
Symptomatic individuals are treated similarly to patients with Gorham-Stout disease.

196
Part IV: Anatomic Considerations
a r t e r io v e n o u s Ma l f o r Ma t io n
An arteriovenous mal ormation can cause bone overgrowth and destruction. Imaging typically
illustrates osteolysis, thinning o the cortex, expansion, and trabeculation. An osseous AVM is
almost always associated with adjacent so tissue disease. Lesions are seldom localized and thus
are rarely amenable to complete excision. An osseous AVM is associated with signi cant bleed-
ing, unlike other vascular anomalies that a ect bone. T e most common site o hemorrhage is
the tooth-bearing alveolar area. Embolization can ameliorate bleeding and may require serial
treatments. Recalcitrant hemorrhage requires resection and reconstruction with bone gra s or
alloplastic materials. Because the reconstructed area is typically surrounded by a so tissue AVM,
recurrence and complications are common. I the lesion involves the oral cavity, periodontal lig-
ament space, and erupted teeth, in ections and gingival hypertrophy can occur. Removal o teeth
in the a ected areas may be necessary to reduce the risk o in ection and bleeding.
I the AVM is causing bone hypertrophy that is contributing to overgrowth o the ace, patients
may request intervention to improve their appearance. T e rontal bone, zygoma, maxilla, or
mandible can be reduced with contour burring. Involved portions o the mandible can be re-
sected and reconstructed with bone gra s that have a more avorable contour. I an occlusal cant
or malocclusion is present, orthognathic procedures can be considered in late adolescence. An
AVM o the jaws requently results in destruction o teeth that require removal. Extractions may
be associated with signi cant bleeding and should be per ormed under general anesthesia. Re-
placement o missing teeth is challenging, because dentures can cause recurrent gingival bleeding,
and diseased bone o en cannot accept implants. Restorative plans with dental implants usually
require extensive bony resection and gra ing.
v a s c u l a r t u Mo r
T e most common vascular tumors that a ect the cranio acial skeleton are hemangioendotheli-
omas (epithelioid or kaposi orm). An epithelioid hemangioendothelioma has an unpredictable
clinical course and a chromosomal translocation, resulting in WWTR1-CAMTA1 usion. Lesions
may remain stable, slowly grow, or metastasize (30%). Seven percent a ect the pediatric popu-
lation and can involve skin, bone, liver, lung, or other sites. When bone is involved, the cranium
is the most common site (about 50%). On radiographic examination lesions appear as areas o
osteolysis with cortical erosion and expansion. reatment is based on the clinical course o the
disease. I serial imaging shows minimal progression, patients can be observed. Severe symptoms,
progression, or metastasis requires systemic chemotherapy, radiation, and/or resection. An epi-
thelioid hemangioma is a benign lesion on the spectrum o epithelioid hemangioendotheliomas
that can a ect the mandible or cranial base; problematic osseous lesions are treated with curettage.
An osseous kaposi orm hemangioendothelioma usually a ects the extremity but can involve the
cranio acial skeleton. Lesions are rarely isolated to bone but also involve the skin and so tissue.
Imaging shows irregular osteolysis with so tissue disease. reatment is either intravenous vin-
cristine or oral sirolimus. Resection o the involved bone and reconstruction are rarely indicated.

197
Chapter 12: Craniofacial Bones
PATIENT EXAMPLES
v e n o u s Ma l f o r Ma t io n
Pa tie n t A
T is 2-year-old boy had swelling o his scalp. T e preoperative appearance illustrated the cuta-
neous mani estation o the lesion (A). An MRI showed a venous mal ormation that was subcu-
taneous, intraosseous, and intracranial (B). An intraoperative view o the cranial de ect and area
o a ected bone (circular marking) is shown (C). T e excised intraosseous venous mal ormation
o the cranium is seen (D). A view o the brain is seen a er neurosurgical removal o the intra-
cranial lesion (E). Reapproximated dura is exhibited (F). T e osseous de ect was reconstructed
with cranial particulate bone gra (G). T e postoperative imaging showed ossi ed bone gra (H).
Fig. 12-1
C

D
A

B
E
F

G

H

198
Part IV: Anatomic Considerations
Pa tie n t B
T is 15-year-old boy had progressive enlargement o his lef cheek. T e patient is shown be ore
surgery (A). C showed an intraosseous venous mal ormation o the zygoma (B and C). T e
resected zygoma is shown (D). A postoperative C illustrated the reconstructed zygoma with
ull-cranial and split-cranial bone graf s (E). T e patient is shown 3 months af er surgery (F).
(Courtesy o John B. Mulliken, MD. From Mulliken JB, Burrows PB, Fishman SJ. Mulliken and
Young’s Vascular Anomalies: Hemangiomas and Mal ormations, ed 2. Ox ord University Press,
New York, 2013. By permission o Ox ord University Press.)
Fig. 12-2
C

D
A

B
E

F

199
Chapter 12: Craniofacial Bones
Pa tie n t C
T is 17-year-old girl had a venous mal ormation o her lower ace, f oor o the mouth, and tongue.
Be ore surgery she had a large anterior open bite (A through C). A lateral cephalogram showed
an open bite and proclined maxillary incisors (D). T is patient is seen 2 years a er sclerotherapy
and a LeFort I osteotomy (E and F). T e open bite has been closed (G). A lateral cephalogram
illustrated the maxillary xation hardware and orthodontic appliances in place (H).
Fig. 12-3
C
D
A

B
E

F
G

H

200
Part IV: Anatomic Considerations
l y MPh at ic Ma l f o r Ma t io n
Pa tie n t A
T is 17-year-old girl was born with a microcystic lymphatic mal ormation o the le side o the
ace. She developed maxillary hypoplasia and an anterior crossbite managed with a LeFort I
osteotomy. T e patient is shown be ore surgery (A). An MRI illustrated a microcystic lymphatic
mal ormation (B). T e preoperative C is shown (C and D). T e occlusion is seen be ore the or-
thognathic procedure (E). T e postoperative C is shown (F and G). T e patient is seen 2 months
a er 5 mm o maxillary advancement (H).
Fig. 12-4
C

D
A

B
E
F

G

H

201
Chapter 12: Craniofacial Bones
Pa tie n t B
T is 16-year-old girl was born with a large cervico acial lymphatic mal ormation. She is shown
be ore her orthognathic procedure (she had previous so tissue resections) (A). Her pro le shows
lower acial hypertrophy with a class III skeletal pattern (B). She had malocclusion with a large
anterior open bite (C). Note the lymphatic mal ormation involving the tongue. A preoperative C
demonstrated the overgrown mandibular body, anterior open bite, and steep mandibular plane
(D and E). She is shown a er a LeFort I osteotomy, mandibular subapical osteotomy, reduction
genioplasty, and cervical debulking (F and G). Occlusion with a corrected open bite is seen (H).
A postoperative C showed skeletal movements and hardware (I and J).
Fig. 12-5
C
A

B
D

F

G
E
H

I

J

202
Part IV: Anatomic Considerations
a r t e r io v e n o u s Ma l f o r Ma t io n
T is 17-year-old girl developed a worsening AVM o the le side o the ace. She is shown be ore
surgery (A). A panoramic radiograph demonstrated a large separation between the erupted le
mandibular second molar and impacted third molar (B). An angiogram be ore embolization il-
lustrated shunting and nidus (C). A panoramic radiograph a er embolization with Onyx is shown
(D). T e intraoral view o the le mandibular dental quadrant is seen be ore removal o the second
molar (E). Note the blue discoloration o the buccal gingiva rom the AVM. T e intraoral view is
shown a er removal o the tooth and hemostasis o excessive bleeding (F).
Fig. 12-6
C

D
A

B
E

F

203
Chapter 12: Craniofacial Bones
k a Po s if o r M h e Ma n g io e n Do t h e l io Ma
T is 5-year-old boy presented with an enlarging lesion o the posterior scalp. Imaging con rmed
a vascular tumor that was diagnosed as a kaposi orm hemangioendothelioma on histopathologic
ndings. T e lesion was resected to prevent destruction o the cranium and potential intracranial
extension. T e preoperative view is seen (A). T e inner marking delineates the outline o the tu-
mor, and the outer line illustrates the extent o resection with 1 cm margins. Preoperative imaging
showed the lesion (B). A er resection the wound was closed with a split-thickness skin gra over
the periosteum (C). T e scalp underwent tissue expansion to reconstruct the hair-bearing scalp
(D). T e scalp is seen a er excision o the skin gra , removal o the tissue expanders, and rotation-
advancement o the expanded scalp f aps (E).
COMPLICATIONS
Resection o an osseous vascular anomaly is associated with postoperative bleeding and recur-
rence. Bleeding is most likely to occur rom an arteriovenous mal ormation but can also result
rom excision o other types o bony lesions. A er an overgrown area is reduced with contour
burring, the bone can become hypertrophied again, necessitating repeat intervention. Osseous
lesions may recur or progress a er nonsurgical treatments such as systemic pharmacotherapy,
sclerotherapy, and embolization. I a vascular anomaly is resected and reconstructed with autol-
ogous bone, the gra has a high risk o ailure, because it can be a ected by residual disease at
the recipient site.
Fig. 12-7
A

B

C
D

E

204
Part IV: Anatomic Considerations
CONCLUSION
Vascular anomalies a ect bone much less commonly than so tissue. Intervention is not manda-
tory and is based on the type o lesion and symptoms. reatment is guided by whether the bone is
overgrown or resorbed. Excessive bone is treated with resection and/or orthognathic procedures.
Bone loss can be treated with sclerotherapy (venous mal ormations), embolization (AVMs), or
systemic drugs (lymphatic mal ormations or hemangioendotheliomas). Bone gra s used to re-
construct osseous de ects can be a ected by the disease and resorb.
SUGGESTED READINGS
Bruder E, Perez-Atayde AR, Jundt G, et al. Vascular lesions o bone in children, adolescents, and young adults. A
clinicopathologic reappraisal and application o the ISSVA classi cation. Virchows Arch : , .
Croteau SE, Liang MG, Kozakewich HP, et al. Kaposi orm hemangioendothelioma: atypical eatures and risks o
Kasabach-Merritt phenomenon in re errals. J Pediatr : , .
Errani C, Zhang L, Panicek DM, et al. Epithelioid hemangioma o bone and so tissue: a reappraisal o a controver-
sial entity. Clin Orthop Relat Res : , .
Greene AK, Alomari AI. Management o venous mal ormations. Clin Plast Surg : , .
Greene AK, Perlyn CA, Alomari AI. Management o lymphatic mal ormations. Clin Plast Surg : , .
Greene AK, aber SF, Ball KL, et al. Sturge-Weber syndrome: so -tissue and skeletal overgrowth. J Cranio ac Surg
(Suppl ): , .
Lala S, Mulliken JB, Alomari AI, et al. Gorham-Stout disease and generalized lymphatic anomaly—clinical, radio-
logic, and histologic di erentiation. Skeletal Radiol : , .
Mulliken JB, Burrows PB, Fishman SJ. Mulliken and Young’s Vascular Anomalies: Hemangiomas and Mal orma-
tions, ed . New York: Ox ord University Press, .
Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor o en mistaken or a carcinoma.
Cancer : , .
Table 12-1 Cranio acial Osseous Vascular Anomalies
Vascular Anomaly
E ect on Bone
Morbidity
Treatment
Capillary malformation
Overgrowth
Deformity
Contour burring
Orthognathic procedures
Venous malformation
Destruction
Deformity
Pain
Bone deficiency
Sclerotherapy
Resection
Bone grafting
Lymphatic malformation
Overgrowth
Destruction
Deformity
Pain
Infection
Occlusal cant
Malocclusion
Bone deficiency
Contour burring
Orthognathic procedures
Bone grafting
Interferon/bisphosphonates
Sirolimus
Arteriovenous malformation
Overgrowth
Destruction
Deformity
Bleeding
Occlusal cant
Malocclusion
Embolization
Contour burring
Orthognathic procedures
Hemangioendothelioma
Destruction
Deformity
Pain
Bone deficiency
Systemic pharmacotherapy
Resection

205
Ryan D. Walker, Reza Rahbar
Cervico acial vascular mal ormations can cause both short-term and long-term morbidity, in-
cluding airway obstruction, eeding di culty, bleeding, dysarthria, bony remodeling, and altered
appearance. Although macrocystic lymphatic mal ormations typically occur below the level o the
mylohyoid muscle and are amenable to excision or sclerotherapy, most lesions o the oral cavity
have a microcystic component. T e submucosal, in ltrative pattern o spread o these lesions o en
results in extension to adjacent subsites and deep neck spaces. T e unctional consequences o
complete surgical resection may be unacceptably morbid because o the proximity o important
neural, vascular, and muscular structures. Lesions a ecting the airway can cause airway obstruc-
tion and become li e-threatening. Subtotal excision can improve symptoms but is associated with
a high rate o recurrence. For these reasons, extensive lesions o the tongue, oral cavity, and airway
are particularly di cult to treat.
Or al Cavit y an d Air w ay
Ch a Pt er 13
KEY POINTS
• Airway management is of paramount concern in patients with vascular malformations of the
head and neck.
• Complete resection of head and neck malformations is rarely possible and recurrence is
common.
• Functional considerations and the potential for large-volume blood loss may dictate surgical
timing and planning.
• Endoscopic and/or laser techniques may be necessary for upper airway lesions.
• Radiofrequency ablation is a promising technique, which is most useful for superficial, mi-
crocystic lesions of the tongue and oral cavity.

206
Part IV: Anatomic Considerations
SURGICAL INDICATIONS
a ir w ay c o MPr o Mis e
T e airway is the primary concern in an in ant with a vascular mal ormation involving the head
and neck. Signs and symptoms usually correlate with the extent o the lesion. Extrinsic com-
pression on the airway by the vascular anomaly or intrinsic involvement o the hypopharynx or
larynx can occur. Narrowing o the airway demands prompt recognition and treatment, because
a marginal airway can rapidly obstruct rom small changes resulting rom viral in ection or in-
tralesional bleeding.
A prenatal diagnosis o vascular mal ormations by ultrasonography and/or MRI is becoming in-
creasingly common. I prenatal imaging identi es the potential or airway compromise, ex utero
intrapartum treatment (EXI ) with intubation or tracheostomy should be planned.
in it ia l e va l ua t io n
Patients with vascular mal ormations o the oral cavity and upper airway will o en present with
recurrent in ections, swelling, and tenderness. In addition, involvement o the tongue may cause
dysphagia, dysarthria, or airway obstruction. Mucosal lymphatic vesicles may cause bleeding,
tenderness, and oozing, which may o en worsen during upper respiratory tract in ections or
a er trauma.
All patients presenting with a vascular mal ormation o the head and neck with possible airway in-
volvement should undergo f exible beroptic nasopharyngolaryngoscopy to evaluate the involve-
ment o the hypopharynx, supraglottis, and glottis. Examination ndings should be integrated
with radiologic evaluation to determine the complete extent o the lesion and its proximity to vital
structures. In lymphatic mal ormations, ultrasonography will typically demonstrate multilocular
cystic tissue without f ow except in the septal areas; a C scan reveals a low-attenuated lesion
with enhancement o the channel walls a er injection o contrast. An MRI with gadolinium is the
single best radiologic technique or characterizing lymphatic mal ormations and typically shows
cystic spaces and rim enhancement on 1-weighted images.
SURGICAL MANAGEMENT
reatment goals or vascular mal ormations o the head and neck include providing a sa e airway,
reducing symptoms, correcting unctional problems, and improving appearance. Because com-
plete resection is rarely possible, treatment ocuses on reducing symptoms while causing as little
damage as possible to adjacent tissue.
Options or treatment include expectant management, surgical excision, sclerotherapy, and ra-
dio requency ablation (RFA).
c o n s e r va t iv e Ma n a g e Me n t
Spontaneous regression o vascular mal ormations is rare, but invasive treatment can be avoided
in a small subset o patients with no complaints, minor symptoms, or intermittent symptomatic

207
Chapter 13: Oral Cavity and Airway
periods e ectively controlled with medical management. Medical therapy may include systemic
antibiotics to treat in ection and oral care with requent rinses and dental treatment.
s u r g ic a l e xc is io n
Resection is the only way to potentially “cure” a vascular mal ormation. T e surgeon must con-
sider possible involvement o neural and vascular structures. Limiting blood loss and preservation
o vital structures are o paramount importance in these cases.
Op e n Re s e c tio n
A number o issues should be considered when planning surgical resection o a vascular mal-
ormation. First, the timing o surgery is important, as is the mode o dissection, whether by
sharp dissection, electrocautery, or laser. Second, it is important to consider whether it is best to
attempt complete resection in a single procedure or whether staged resection is indicated. Divid-
ing vascular mal ormations into subtypes is also o en use ul or surgical planning. T ose lesions
located below the level o the mylohyoid muscle in general may be sa ely resected within the rst
year o li e. In this case sharp dissection is requently the modality o choice, and they can o en
be resected during one procedure. Mal ormations above the level o the mylohyoid muscle are
requently poorly de ned, and the planes between the mal ormation itsel and the normal anat-
omy may be di cult to dissect. Complete surgical resection in these circumstances can be quite a
challenge. T e use o loupes or operating microscope is help ul, and a nerve stimulator is impera-
tive, because normal anatomy is requently disrupted. We recommend that surgery be per ormed
be ore 5 years o age i possible. Factors such as expected blood loss, duration o the procedure,
and extent o dissection may help to determine whether multiple procedures are needed.
Lingual excision should be avoided except in rare cases o solitary lesions o the anterior tongue.
ransoral resection o the tongue base or f oor o the mouth should be avoided because o the high
likelihood o postoperative dysphagia and/or aspiration. We have ound that RFA is a sa e and
e ective treatment modality or many patients with lymphatic mal ormations o the oral cavity.
En d o s c o p ic a n d La s e r Su rge ry
In sites in which disease is not readily resectable by open, sharp dissection, such as lesions o the
larynx, oropharynx, and oral cavity, endoscopic techniques can be used. Retraction sutures or
suspension laryngoscopy may be necessary or exposure. Binocular microscopic visualization is
essential to provide depth perception. Palpation o the lesion with a probe or spatula provides the
haptic eedback necessary to ully appreciate the extent o the lesion. Endoscopic management
will rarely allow a complete resection but can be particularly e ective or debulking obstructive
lesions in the upper airway.
T e carbon dioxide or neodymium-doped yttrium aluminum garnet (Nd:YAG) laser can be used
to per orm resection or debulking. Energy rom laser light can be used to coagulate, vaporize, or
cut mal ormed tissue. T e pulsed-dye laser has been used to shrink vascular lesions that contain
blood. T e intralesional bare ber laser technique has been used or deep vascular lesions with
a potassium titanyl phosphate (K P), argon, or Nd:YAG laser attached to a beroptic wand.
T is approach may be help ul in conjunction with conventional sur ace laser therapy or lingual
lesions with both deep and super cial components. Nd:YAG and carbon dioxide lasers seem most
appropriate or vesicular oozing or sur ace bleeding that accompanies in ection or minor trauma.

208
Part IV: Anatomic Considerations
Sclerotherapy
Numerous sclerosing agents have been used or the treatment o venous and macrocystic lym-
phatic mal ormations, including bleomycin sul ate, ethanol, tetracycline, dextrose, inter eron
al a-2a, brin sealant, and OK-432. OK-432 is a lyophilized mixture o group A streptococci and
Streptococcus pyogenes. It produces an inf ammatory reaction, ollowed by gradual regression o
the lesion. OK-432 is thought to leave minimal perilesional brosis, making subsequent resection
less hazardous. OK-432 is more e ective or macrocystic than or microcystic lesions; the e cacy
rates are 86% versus 66%, respectively. In patients with microcystic lesions or those in whom the
use o OK-432 has ailed to produce an impressive result, surgical resection should be considered.
Absolute ethanol is also used or macrocysts and mucosal vesicles but must be injected with care
near the hypoglossal and lingual nerves when used in the tongue. Doxycycline is another e ective
sclerosing agent; it does not cause neural toxicity or stain the teeth.
Embolization
Fluoroscopically guided embolization with coils or glue is used to control recurrent lingual bleed-
ing that can be traced to speci c vessels in the oral f oor, usually rom an arteriovenous mal orma-
tion. Bleeding rom a lymphatic mal ormation is rom vesicular eruptions on the lingual sur ace.
Lingual arterial embolization can shrink a tongue with a lymphatic mal ormation.
Radiofrequency Ablation
RFA, or coblation, is a relatively new technology that is used in various areas o otolaryngology.
Because o its mechanism o low-temperature tissue destruction, thermal injuries to surround-
ing tissues are limited, and there ore patients may have a better course o recovery. wo modes
o RFA have been described. T e high- requency mode is used or destruction o deep tissue,
resulting in a reduction in size rom secondary brosis without a ecting the mucosal sur ace. In
low- requency mode, energy is transmitted by way o a conduction medium in small volumes or
the removal o a thin, super cial layer, with minimal thermal injury to nearby tissue. For selective
patients with lesions that are localized and super cial, we have observed excellent results with
RFA in terms o postoperative recovery, complication rate, and clinical outcome.
Patient selection is critical when considering RFA treatment. Patients are typically treated with
systemic antibiotics or acute in ection and optimization o oral hygiene with mouth rinse and
requent dental care be ore and a er the procedure. reatment is per ormed with the patient un-
der general anesthesia with oral or nasal intubation. Intraoperative antibiotics and corticosteroids
(0.5 mg/kg o dexamethasone) are administered. issue is removed in layers rom super cial to
deep in broad strokes with continuous saline solution irrigation. A er surgery, patients do not
typically have signi cant pain issues and tolerate eeding almost immediately.
T e complication rate is very low and includes postoperative hemorrhage, leading to acute swell-
ing and airway obstruction. Given the possibility o airway swelling and obstruction, however,
we recommend that every patient who undergoes RFA treatment o the oral cavity be observed
in the hospital during the immediate postoperative period.
For patients with more extensive microcystic lymphatic mal ormations o the oral cavity, radio re-
quency ablation is not the ideal primary treatment modality o choice. T e characteristic eature o
RFA—low-temperature tissue destruction with limited injury to surrounding tissue—also serves
as a limitation or lesions that are deep and involve underlying muscle. For lesions involving
deeper structures, multimodal treatments, including surgical excision and/or sclerotherapy, may
be necessary.

209
Chapter 13: Oral Cavity and Airway
PATIENT EXAMPLES
e x u t e r o in t r a Pa r t u M t r e at Me n t a ir w ay Ma n a g e Me n t
T is in ant boy with a large, cervico acial lymphatic mal ormation was diagnosed prenatally and
born via the EXI procedure. T e patient was success ully intubated during the EXI airway
procedure with direct laryngoscopy and intubation over a rigid telescope (A). Sclerotherapy per-
ormed in the neonatal period did not reduce the mass signif cantly, and thus signif cant airway
compression remained, which prevented extubation (B). T e patient required partial resection o
the anterior cervical portion o the mass or a tracheostomy to be per ormed (C).
Fig. 13-1
C
B
A

210
Part IV: Anatomic Considerations
l y MPh at ic Ma l f o r Ma t io n Pr e s e n t in g a f t e r t r a u Ma
T is 9-year-old boy with a previously undiagnosed lymphatic mal ormation presented a er
trauma during a ootball game. Be ore admission, no lesion was noted (A). On admission, a
large, so , compressible mass was visible in the lateral neck (B). A gadolinium-enhanced 1 MRI
demonstrated a large mixed macrocystic and microcystic lymphatic mal ormation throughout the
lateral neck (C). Open resection was per ormed, and the lesion was removed en bloc (D and E).
Fig. 13-2
C
A

B
D

E

211
Chapter 13: Oral Cavity and Airway
s c l e r o t h e r a Py f o r a c e r v ic o f a c ia l l y MPh at ic Ma l f o r Ma t io n
T is 10-month-old boy with a large cervico acial lymphatic mal ormation presented or sclero-
therapy o multiple macrocystic components. A gadolinium-enhanced 1- (A) and 2-weighted
(B) MRI showed an extensive mixed macrocystic and microcystic mass extending bilaterally and
occupying both the suprahyoid and in rahyoid spaces. Lateral (C) and anteroposterior (D) views
were obtained f uoroscopically during sclerotherapy.
Fig. 13-3
C

D
A

B

212
Part IV: Anatomic Considerations
r a Dio f r e q u e n c y a b l a t io n o f a n o r a l c a v it y l y MPh a t ic
Ma l f o r Mat io n
T is 6-year-old boy with a microcystic lymphatic mal ormation o the tongue presented with
recurrent mucosal bleeding. Intraoperatively a tongue suture was placed or retraction (A). RFA
was per ormed bilaterally (B). T e tongue was partially mucosalized 1 week a er surgery (C).
Healing was nearly complete 4 weeks a er surgery (D).
Fig. 13-4
C

D
A

B

213
Chapter 13: Oral Cavity and Airway
COMPLICATIONS
T e most serious complication rom vascular mal ormations o the head and neck and their
treatment is airway obstruction. T is may occur as a result o the lesion or so tissue inf amma-
tion a er treatment. reatment, whether by surgical excision, RFA, or sclerotherapy, can induce
swelling and cause airway obstruction. For this reason, we typically treat patients with systemic
steroids and observe overnight a er any o these procedures. Great care should be taken to avoid
neurovascular injury during any surgical excision in the head and neck because o the proximity
to cranial nerves or major vessels. Other complications are similar to treatment o vascular anom-
alies in any region o the body, including postoperative seromas, hematomas, or lesional bleeding.
CONCLUSION
Management o vascular mal ormations in the head and neck can be especially challenging,
because the surgeon must consider the e ects on the airway, voice, swallowing, articulation,
dentition, acial growth, and cosmesis while avoiding potentially li e-threatening consequences.
Fortunately, prenatal diagnosis o obstructive lesions is improving and allows advanced surgical
planning. Many options exist or treatment o these lesions beyond open excision, including en-
doscopic laser surgery, RFA, and sclerotherapy. Each technique, o course, has both advantages
and pit alls, making patient selection critical to success.
SUGGESTED READINGS
Edwards PD, Rahbar F, Ferraro NF, et al. Lymphatic mal ormation o the lingual base and oral f oor. Plast Reconstr
Surg : , .
Grimmer JF, Mulliken JB, Burrows PE, et al. Radio requency ablation o microcystic lymphatic mal ormation in the
oral cavity. Arch Otolaryngol Head Neck Surg : , .
Kim SW, Kavanagh K, Orbach DB, et al. Long-term outcome o radio requency ablation or intraoral microcystic
lymphatic mal ormation. Arch Otolaryngol Head Neck Surg : , .
Rowley H, Perez-Atayde AR, Burrows PE, et al. Management o a giant lymphatic mal ormation o the tongue. Arch
Otolaryngol Head Neck Surg : , .

(Trang không có chữ)

215
Aladdin H. Hassanein, Steven J. Fishman
Vascular anomalies can a ect the chest, abdomen, and genitalia. Although less common than
integumentary lesions, visceral vascular mal ormations can be problematic. Hepatic hemangi-
omas are the most common liver tumor o in ancy. Vascular mal ormations o the thorax and
abdominal viscera are more di cult to diagnose than skin lesions, which typically require only
history, physical examination, and ultrasonography. Visceral lesions must be visualized by im-
aging or endoscopy. Management o these mal ormations and tumors is dictated by the clinical
presentation; symptoms are inf uenced by the extent and type o lesion and the organ system
involved. T is chapter will ocus on surgical management o vascular anomalies o the chest,
abdomen, and genitalia.
Ch es t , Abd o men , an d Gen it al ia
Ch a Pt er 14
KEY POINTS
• Thoracic vascular malformations can result in symptoms from mass effect or lymph leakage.
• Resection is required for mass lesions, and pleurodesis may improve chylothorax.
• Aggressive removal of multifocal intestinal venous malformation lesions is effective in erad-
icating gastrointestinal bleeding caused by blue rubber bleb nevus syndrome.
• Diffuse venous malformations involving the pelvis can be treated with partial colectomy,
anorectal mucosectomy, and coloanal pull-through.
• Genitalia affected by vascular malformations and lymphedema can be improved with surgi-
cal debulking.

216
Part IV: Anatomic Considerations
SURGICAL INDICATIONS
c h e s t
Lymphatic mal ormations (LMs) are more common in the thorax than venous mal ormations
(VMs) or arteriovenous mal ormations (AVMs). Extracutaneous chest LMs are typically located
in the mediastinum or retropleural area and may be in continuity with the neck, thoracic inlet,
retroperitoneum, or so tissue lesions. Mediastinal LMs are usually asymptomatic; small-to-
moderate–sized lesions may be observed. Large LMs can cause airway compression or pulmonary
compromise and require treatment. Macrocystic LMs are amendable to image-guided sclerother-
apy. However, symptomatic microcystic lesions may need resection. Lymphatic anomalies o the
chest can result in pleural e usion. Medical management or a chylothorax can be attempted ( or
example, a at-restricted diet, octreotide, or sirolimus). Small e usions or those causing tolerable
symptoms can be treated nonoperatively. Interventions ( or example, pleurodesis, lymphovenous
anastomosis, or thoracic duct ligation) are per ormed i symptoms continue despite medical ther-
apy. LMs o the lung parenchyma are uncommon. Congenital pulmonary lymphangiectasia is a
rare anomaly that is characterized by severe respiratory distress o the newborn; the prognosis
is poor. Supportive treatment with ventilation and extracorporeal membrane oxygenation may
be indicated. VMs and AVMs rarely occur in the chest cavity. Symptomatic lesions typically are
managed with endovascular interventions rather than resection.
a b Do Me n
Although hemangiomas are the most common vascular anomaly, hemangiomas o the chest cavity
and abdomen are typically clinically silent because o their predictable pattern o involution. T e
liver is the most requent extracutaneous site o these tumors. T e presence o ve or more skin
hemangiomas (hemangiomatosis) may be indicative o a liver lesion. Hepatic hemangiomas are
categorized into (1) ocal, (2) multi ocal, or (3) di use. Focal hepatic hemangiomas are rapidly
involuting congenital hemangiomas (RICH), which undergo more than 90% volume reduction
by 13 months o age. A small subcapsular hepatic nodule remains a er involution, and therapy is
rarely required. Multi ocal hepatic hemangiomas are in antile hemangiomas that have a growth
pattern similar to skin lesions. Most patients are asymptomatic, but a subset develops: (1) con-
sumptive hypothyroidism because o the expression o a deiodinase by a hemangioma, which
inactivates the thyroid hormone, and/or (2) high-output cardiac ailure rom shunting. Di use
hepatic hemangiomas replace an extensive portion o liver parenchyma. Patients may be diag-
nosed within several months o birth because o massive hepatomegaly, respiratory compromise,
abdominal compartment syndrome, or heart ailure. Hypothyroidism is treated with aggressive
thyroid hormone supplementation. T yroid unction normalizes with tumor involution. Children
exhibiting heart ailure can be managed with pharmacotherapy (steroids or propranolol). Endo-
vascular embolization is rarely used or cardiac ailure. Patients with di use hepatic hemangiomas
who have abdominal compartment syndrome and show clinical deterioration may require liver
transplantation.

217
Chapter 14: Chest, Abdomen, and Genitalia
Intraabdominal LMs may involve the mesentery, omentum, or retroperitoneum. Large lesions
may be symptomatic ( or example, diarrhea, distention, nausea, or pain). Most LMs are macro-
cystic and are best managed with image-guided percutaneous sclerotherapy. Microcystic LMs
less commonly occur in the abdomen and are usually asymptomatic. Surgical extirpation may
be indicated or large, microcystic lesions causing mass e ect.
Abdominal LMs that leak into the peritoneum can orm chylous or nonchylous (clear) ascites.
Chylous leaks occur rom the lymphatics o bowels, mesentery, or retroperitoneum that contain
lipids. LMs causing persistent ascites may require exploratory laparotomy, localization o the
origin o the leak, and removal o the inciting area.
VMs o the abdomen can be ocal, multi ocal ( or example, blue rubber bleb nevus syndrome
[BRBNS]), or di use. T ese lesions may result in gastrointestinal or intraperitoneal hemorrhage.
Symptomatic ocal lesions can be treated with endoscopic sclerotherapy or segmental bowel re-
section. Patients with multi ocal VMs who require chronic blood trans usions may undergo sur-
gical eradication o all the luminal lesions with intraoperative endoscopic assistance. Nonsurgical
management (pharmacologic therapy or endoscopic treatment) or BRBNS has not been durably
e ective; however, early experience shows some optimism or sirolimus in this regard. In contrast,
di use VMs are treated with selective excision o the symptomatic area. T ese lesions typically
encompass the le colon to the anus and are managed with le colectomy, mucosal proctectomy,
and coloanal pull-through. In some patients repeated sclerotherapy o the submucosal anorectal
VM may avoid or delay the need or pull-through.
Hepatic VMs are common and requently mislabeled “hemangiomas” or “cavernous hemangi-
omas.” T ese lesions are typically asymptomatic until adulthood. T ey can cause pain during
pregnancy and rarely may rupture. Intralesional sclerotherapy is pre erred i treatment is required,
although surgical extirpation is also used. Rare abdominal “AVMs” are usually VMs that are in-
correctly classi ed. rue intestinal AVMs may be amendable to segmental intestinal resection.
g e n it a l ia
Vascular mal ormations o the genitalia can cause psychosocial morbidity and de ecatory, urinary,
and/or sexual dys unction. T e lesions are concealed by clothing and o en lower sel -esteem. LMs
o the scrotum can masquerade as inguinal hernias; an ultrasound examination may help with
diagnosis and prevent an inguinal exploration or suspected hernia. Compression garments are
recommended or slow-f ow vascular mal ormations. Interventions or lesions that are not causing
unctional symptoms are o en delayed until anatomic maturity, although extreme de ormity can
be addressed earlier. Genital LMs are rarely macrocystic. Resection is pre erable to sclerotherapy
or symptomatic slow-f ow mal ormations o the male genitals; surgical scrotal scars are well
hidden, and f uoroscopic testicular exposure is prevented. Bleeding endourethral VMs should
be treated with laser photocoagulation. AVMs are rare and usually managed with embolization
and debulking.

218
Part IV: Anatomic Considerations
Vascular mal ormations o the emale genitalia are usually slow-f ow lesions that can a ect the
clitoris, labia, or vagina. Symptomatic mal ormations are resected or sclerosed (with lead protec-
tion o the ovaries). Chyle leakage rom the vagina is treated with sclerotherapy, laser, or ligation
o intrapelvic ref uxing lymphatic channels.
Genital lymphedema a ects both genders. Conservative treatment involves prevention o in ection
and compression. Surgical intervention is reserved or patients with signi cant morbidity into
puberty despite nonsurgical management. Resections or emale genital lymphedema are generally
not per ormed. Excisional procedures are used or penile and scrotal lesions.
SURGICAL MANAGEMENT
c h e s t
Large vascular mal ormations o the thorax can cause airway compression or respiratory com-
promise rom mass e ect. In contrast to macrocysts, microcystic LMs are not amendable to
sclerotherapy and necessitate resection i they are symptomatic. T ese lesions typically involve
the mediastinum and retropleural and retrocrural spaces. Surgery is avoided be ore age 6 months
unless there is an immediate airway threat. At this age, the risk o anesthesia is diminished, and
the in ant is less susceptible to airway complications rom postoperative edema. Vascular mal-
ormations do not require total extirpation, because they are not malignant. However, extensive
removal o the lesion should be per ormed while preserving vital structures. Remnant LMs can
reexpand, and reoperation is more challenging. Large, anterior mediastinal microcystic LMs may
be debulked with median sternotomy. T ese lesions can involve the airway, esophagus, nerves,
thymus, and vascular structures. T e phrenic, recurrent laryngeal, and vagus nerves should be
preserved. Posterior mediastinal and apical lesions are better exposed with a thoracotomy.
A lymphatic anomaly may leak and cause a pleural e usion. T e chylothorax does not usually
resolve with excision o the lesion. Chemical ( or example, bleomycin, doxycycline, or talc) or
mechanical pleurodesis with stripping or abrasion o the parietal pleura can be used or treat-
ment. Pleurodesis creates inf ammation to acilitate adherence o the visceral and parietal pleura.
However, it is di cult to maintain pleural apposition with high-volume lymph leakage rom
mal ormations, and these techniques are less success ul than pleurodesis or other causes o e -
usion. Microvascular lymphovenous anastomosis o the thoracic duct has also been success ul.
T e thoracic duct can be ligated near the diaphragm to decrease chylothorax i other treatments
have ailed. T e duct can be localized during thoracoscopy or thoracotomy by enteral at loading
( or example, a bolus o heavy cooking cream through a nasoduodenal tube).
a b Do Me n
Abdominal LMs that cause ullness, pain, organ distortion, or gastrointenstinal/genitourinary tract
obstruction rom mass e ect require treatment. Macrocystic lesions are sclerosed. However, mal-
ormations with a large microcystic component require surgical intervention. T e lesion should

219
Chapter 14: Chest, Abdomen, and Genitalia
be aggressively debulked to excise as much as possible without injury to vital structures ( or
example, ureter or lumbosacral nerve plexus). Remaining lesions can reexpand postoperatively
and are more di cult to treat because o scarring.
LMs causing persistent ascites are di cult to manage, because localization o the leaking area
is challenging. Nonchylous ascites rom an LM o a solid organ ( or example, the kidney) may
have an atypical appearance on imaging and obvious swelling on laparotomy; nephrectomy may
be required. It can be more problematic to nd the cause o abdominal chylous ascites, because
the bowel, mesentery, or retroperitoneal sources have chyle- lled lymphatics that are di cult to
identi y.
VMs o gastrointestinal hollow viscera may cause bleeding. T e surgical approach is dependent
on whether the lesion is (1) localized, (2) multi ocal, or (3) di use. A segmental bowel resection
can be per ormed or a symptomatic, ocal VM localized to a limited region o bowel. Multi ocal
VMs occur in BRBNS. Patients with this condition have multiple VMs distributed throughout the
viscera and eventually have gastrointestinal hemorrhage and anemia. An aggressive resection is
indicated to eradicate all the VMs rom the bowel lumen to resolve the bleeding. Laparotomy is
per ormed with total intraoperative endoscopy rom mouth to anus. Segmental bowel resection
is limited to areas with many adjacent lesions. Enterotomies are created to access and remove
mucosal VMs that are sparsely distributed by intussuscepting and inverting adjacent bowel seg-
ments. T e mean operative time is 14 hours, and more than 130 lesions are typically removed.
Di use abdominal VMs may involve large segments o bowel, mesentery, and the retroperi-
toneum. In contrast to the multi ocal VMs ound in BRBNS, eradication o all lesions is not
possible. Only the areas o hemorrhage must be removed. Di use gastrointestinal VMs usually
cause chronic hematochezia. Di use colorectal VMs typically surround the rectum. Full-thickness
resection o the rectum may result in uncontrollable bleeding. T ese lesions can be treated with
anorectal mucosectomy, partial colectomy, and coloanal pull-through. T e le colon is resected
down to the peritoneal ref ection, and the mucosa o the remaining rectum is stripped rom
the muscular rectal wall similar to the management o ulcerative colitis or amilial polyposis.
Endoanal sensation is maintained by preservation o the rectal mucosa or about 1 cm above the
dentate line.
g e n it a l ia
Resection is indicated or symptomatic vascular mal ormations o the male genitalia. Sclerother-
apy or excision can be per ormed or a ocal VM o the glans penis. VMs o the penile sha are
typically super cial to the Buck ascia. T ese lesions can be excised, with preservation o the deep
dorsal nerves and vessels and closed linearly. LMs o the penis are also located super cially in the
subcutaneous tissues. Penile lesions are resected through a circum erential incision proximal to
the corona o the glans penis. T e sha skin is degloved circum erentially to the base. T e diseased
tissue is removed o the Buck ascia, and the sha integument is redraped. Excess circum erence
o the skin is trimmed to leave a longitudinal scar at the ventral midline. Penile lymphedema is
removed with a similar technique.

220
Part IV: Anatomic Considerations
Fig. 14-1
Fig. 14-2
Scrotal vascular mal ormations are amendable to resection; typically the testicles are spared. A
urinary catheter is placed to help protect the urethra. T e testicle and spermatic cord are mobi-
lized out o the scrotum through a transverse or vertical scrotal incision. A ull-thickness wedge
o scrotal tissue is removed, including skin, adipose tissue, and Dartos ascia. T e closure orms
a new midline raphe. Surgical management o scrotal lymphedema is similar.

221
Chapter 14: Chest, Abdomen, and Genitalia
Female genital vascular mal ormations may also be surgically debulked. VMs o the labia can be
sclerosed or resected. Sclerosants should not be injected into the clitoris. Lesions o the clitoral
hood are excised without di culty. LM o the corpora cavernosum causing clitoromegaly can be
resected with care ul preservation o the neurovascular bundle to the clitoral glans. Vaginal VMs
causing dyspareunia are removed or sclerosed.
PATIENT EXAMPLES
Mu l t if o c a l g a s t r o in t e s t in a l v e n o u s Ma l f o r Mat io n s
T is 36-year-old woman had a history o BRBNS. She had multiple so tissue VMs noted at birth
and chronic gastrointestinal blood loss requiring intravenous iron supplementation and more
than 100 blood trans usions (A). T e patient underwent exploratory laparotomy and endoscopy.
She had more than 500 VMs through the entire gastrointestinal track (B). T ree lesions in the
esophagus and 14 VMs in the stomach were treated with endoscopic band ligation. Enterotomies
were created to access and remove mucosal VMs throughout the small bowel by intussuscepting
and inverting adjacent bowel segments (C and D).
Fig. 14-3
C

D
A

B

222
Part IV: Anatomic Considerations
Pe n il e l y MPh e De Ma
T is patient had a history o genital lymphedema (A). Af er a urinary catheter was placed to
protect the urethra, the diseased penile tissue was resected through a circum erential incision
proximal to the corona o the glans penis (B). T e shaf skin was degloved circum erentially to
the base. T e lymphedematous tissue was removed rom the Buck ascia and the shaf skin was
redraped. Excess circum erence o the skin was excised, leaving a longitudinal midline scar.
v e n o u s Ma l f o r Ma t io n o f t h e f e Ma l e g e n it a l ia
T is 34-year-old woman presented with a symptomatic VM o the right labia (A). No sclerother-
apy was per ormed. T e lesion was resected and linearly closed (B). (Courtesy o John B. Mulliken,
MD. From Mulliken JB, Burrows PB, Fishman SJ. Mulliken and Young’s Vascular Anomalies:
Hemangiomas and Mal ormations, ed 2. Ox ord University Press, New York, 2013. By permission
o Ox ord University Press.)
Fig. 14-4
A

B
Fig. 14-5
A

B

223
Chapter 14: Chest, Abdomen, and Genitalia
COMPLICATIONS
c h e s t
Failure to remove signi cant areas o thoracic LMs can result in recurrence and symptoms and
require a more di cult secondary operation because o scar tissue. Injury to vital structures
during the procedure may result in morbidity. Management o thoracic chylothorax may also be
problematic. Stripping o the parietal pleura or mechanical abrasion can worsen the e usion by
opening additional LM channels and increasing f uid leaking.
a b Do Me n
Incomplete removal o a multi ocal abdominal VM in BRBNS may result in ongoing gastro-
intestinal bleed and blood trans usions. T ere is a risk o short gut syndrome i multiple large
areas o small bowel require segmental resection. During treatment o a di use colorectal VM,
intraoperative hemorrhage is common with anorectal mucosectomy. Full-thickness resection o
a di use VM involving the rectum can result in massive hemorrhage.
g e n it a l ia
Removal o vascular mal ormations in close proximity to the urethra may cause iatrogenic stric-
ture. Resection o lesions be ore adolescence can hinder pubescent genital growth. Clitoral scar
or injury to nerves can limit sexual unction.
CONCLUSION
reatment o vascular mal ormations o the chest, abdomen, and genitalia is challenging. T e
goal o surgical management o these lesions is to improve symptoms. Surgical principles or the
treatment o thoracic and abdominal vascular mal ormations can decrease the risk and morbidity
associated with these operations.
SUGGESTED READINGS
Dasgupta R, Fishman SJ. Management o visceral vascular anomalies. Semin Pediatr Surg : , .
Fishman SJ. runcal, visceral and genital vascular mal ormations. In Mulliken JB, Burrows PE, Fishman SJ, ed. Vas-
cular Anomalies: Hemangiomas and Mal ormations, ed . New York: Ox ord University Press, .
Kulungowski AM, Schook CC, Alomari AI, et al. Vascular anomalies o the male genitalia. J Pediatr Surg : ,
.
Schook CC, Kulungowski AM, Greene AK, et al. Male genital lymphedema: clinical eatures and management in
pediatric patients. J Pediatr Surg : , .
Vogel AM, Alesbury JM, Burrows PE, et al. Vascular anomalies o the emale external genitalia. J Pediatr Surg
: , .

(Trang không có chữ)

225
Amir H. Taghinia, Joseph Upton
T e past hal century has seen a steady, almost exponential increase in our knowledge o vascular
anomalies. In 1982 Mulliken and Glowacki introduced a use ul biologic system that has become
universally accepted and has improved treatment. History and physical examination remain the
most important tools to diagnose many o these anomalies. Re ned imaging modalities have
evolved to augment diagnostic accuracy. A multidisciplinary team approach o ers treatment op-
tions with some degree o predictability. T is chapter ocuses on surgical principles and technical
pearls in the treatment o these unique problems involving the upper extremity. I incorporated
into routine management, the techniques and principles covered in this chapter will maximize
outcomes and minimize complications.
H an d an d Upper Ext r emit y
Ch a Pt er 15
KEY POINTS
• The surgeon should plan carefully and counsel the patient thoroughly before surgery.
• Incisions must be placed strategically.
• The surgeon must operate in a bloodless field.
• Fine surgical instruments must be used.
• The surgeon must identify and tag important structures.
• The surgeon must dissect discreetly but thoroughly.
• The surgeon must avoid intraneural dissection and partial dissections within muscle groups.
• The wound must be closed meticulously.
• The extremity must be immobilized appropriately.
• The surgeon must follow the patient regularly.

226
Part IV: Anatomic Considerations
Surgical treatment o vascular anomalies requires care ul planning and meticulous execution.
Planning or blood loss and surgical exposure are o utmost importance. Although tourniquet
use in the upper extremity can minimize intraoperative blood loss, it does not substitute or good
exposure and judgment. For example, large venous mal ormations (VMs) can spill a lot o blood,
which causes staining and poor visualization. Furthermore, i not ully excised or ligated, the
“loose ends” can bleed signi cantly a er the tourniquet is def ated.
A well-balanced approach that incorporates calculated risk and cautious execution is necessary—
one that can only be cultivated with experience. T e surgeon whose practice is devoted to the
treatment o these lesions is obliged to document meticulously and analyze critically; these are
habits that will serve one well or li elong learning.
T ese lesions can never be absolutely eradicated. Persistence o the vascular anomaly is the rule
despite clean and thorough extirpative operations. T is is an important consideration in preop-
erative planning, amily/patient counseling, and uture interventions.
SURGICAL INDICATIONS
T e indications or surgical intervention in the upper extremity are similar to other areas o the
body. ypical reasons to intervene are pain, ulceration, persistent growth, unctional compromise,
and even appearance (on occasion). A trial o conservative treatment is always worthwhile be ore
surgical intervention. T is may include compression garments, blood thinners ( or example, aspi-
rin), injections, splinting, or hand therapy. Many children with signi cant vascular anomalies are
treated conservatively during childhood and present as teenagers requesting surgery. Lymphatic
mal ormations (LMs) and VMs tend to become more symptomatic in adolescent emales than in
males. In contrast, ast-f ow lesions are slow growing, indolent, and insidious. T e surgeon should
provide a clear explanation o all potential complications and expected short-term and long-term
outcomes be ore any surgical treatment.
T e risks and bene ts o surgical intervention must be weighed care ully against minimally in-
vasive intervention. During the past several decades, the treatment o most vascular anomalies
has improved as the result o minimally invasive techniques such that most lesions can now be
treated by interventional radiologists. As scientists urther elucidate the molecular origin and
structure o these conditions, medical therapy has continued to improve. More recently, sirolimus
has been used more requently with promising outcomes to treat larger, more di cult lesions.
Nevertheless, surgery is the best treatment or some o these lesions. For example, most LMs
are con ned to the subcutaneous tissue planes and are requently removed without the need to
explore the deeper tissues.
T e surgeon who has the courage to treat di cult ast-f ow lesions should also be prepared to
amputate a symptomatic non unctional or pain ul digit or limb a er unsuccess ul attempts at pal-
liation. Patients with severe pain will o en request amputation o the a ected part. Other patients
may wish to continue treatment despite a unctionless, parasitic limb or digit. T e most di cult
decisions are those in young children who do not verbalize their pain and some teenagers who
have minimal tolerance to pain and have not adapted well to their limb mal ormation. In di -
cult lesions, amputation should be considered and discussed be ore surgery. T e reconstructive
surgeon should not view amputation as a ailure.

227
Chapter 15: Hand and Upper Extremity
SURGICAL MANAGEMENT
Surgical principles gain acceptance with time and must be periodically reassessed and re ned,
particularly in this rapidly advancing eld. Principles should not be con used with techniques
that change requently and are modulated to a great extent by technology. Many o the ollowing
principles have evolved rom the early surgical experience with vascular mal ormations (especially
the ast-f ow types), which was punctuated with complications.
Pr e o Pe r at iv e Pl a n n in g
Preoperative planning is the cardinal principle o surgical treatment. Most poor outcomes can
be avoided by adhering to this principle alone. Planning should include correlation o the size,
extent, and involvement o structures with physical examination and imaging. Imaging modalities
include plain radiographs, ultrasonography, MRIs, and angiography. Be ore surgery is per ormed
on complex, ast-f ow lesions, a step-by-step plan o resection, including the approach and extent
o resection, must be determined and ollowed. Despite imaging, the extension o some anomalies
may not be completely delineated, and intraoperative decision-making may be required a er the
true extent becomes visible. Despite this, the surgeon must abide (as much as possible) by the
initial surgical plan; straying rom the plan may lead to a violation o uninvolved areas, an inad-
vertent injury to neurovascular structures, a waste o precious tourniquet time, and an incomplete
excision. For complex lesions, we have ound it help ul to print the angiogram and label the nidus,
shunts, and all large branches within that portion o the lesion to be excised. T ese scans should
be displayed in the operating room or easy re erence during the procedure. Other preparatory
steps or ast-f ow lesions include central venous and peripheral arterial monitoring, availability
o warmers and blood products or trans usion, and ICU monitoring a er surgery.
Pl a c e Me n t o f in c is io n s
Placement o incisions is important, particularly in children. When planning an incision, the sur-
geon should consider exposure, blood supply, unction, aesthetics, and uture surgery. Incisions
should be long enough to provide ull exposure o the entire lesion. Small incisions provide no
surgical advantage and risk injury to important structures, especially in the hand. In the digits,
a high midaxial incision is pre erred, because it provides excellent dorsal and palmar exposure,
can be used again, and is typically well hidden. Dorsal longitudinal hand and digital incisions
are avoided, i possible, because they provide less exposure and are more conspicuous. Palmar
incisions can lead to contracture i poorly planned; zigzag incisions and those that use normal
skin creases are recommended.
I multiple debulking procedures are contemplated in the upper arm or orearm, each incision
should be planned care ully to avoid unnecessary scarring near neurovascular structures. T e
medial sur ace o the arm, elbow, and orearm is the least conspicuous. Scars in the web spaces
or along eponychial and/or paronychial olds will contract and are likely to become problematic.
Ironically, vascular insu ciency can exist in the presence o a vascular anomaly, especially ast-
f ow types; thus blood supply should be care ully considered during planning o incisions. T e
dorsum o the hand has an axial blood supply above the overlying ascia, whereas the palm o the
hand receives per orators rom palmar arterial branches. Accordingly, large f aps can be developed
sa ely in the dorsum, but similar f aps are prone to ischemia in the palm. o avoid digital vascular
compromise, only one hal o a digit should be dissected at a time. When a critical arterial segment
to a digit or the hand is removed, this segment should be reconstructed with a vein gra so that

228
Part IV: Anatomic Considerations
at least one digital artery is preserved per digit, and one major artery supplies the hand with a
unctionally intact palmar arch.
Dis s e c t io n in a b l o o Dl e s s f ie l D
Dissection in a bloodless eld is critical or identi cation o important structures and delineation
o the vascular lesion. Accordingly, a pneumatic tourniquet is essential or all but the simplest
excisions. Limb exsanguination should be thorough be ore tourniquet inf ation. I bleeding occurs
in the wound within 5 to 10 minutes o tourniquet inf ation, the arm should be reelevated and the
process repeated. In small children we use the elastic Esmarch wrap as the tourniquet secured at
the midhumeral level. Dissection o VMs, lymphaticovenous mal ormations (LVMs), and arte-
riovenous mal ormations (AVMs) with arteriovenous stulas is most vulnerable to blood stain-
ing, whereas LMs are more orgiving. A blood-stained eld within any dissection will decrease
visibility, obliterate ascial planes, and set the stage or potential injury to important structures.
With a chest wall LM and capillary-LVM excisions or brachial plexus explorations, the tourniquet
cannot be used. During these long procedures, blood loss can be insidious, especially in small
children. T us a premium is placed on meticulous hemostasis, which is best accomplished by
care ul dissection and preemptive ligation or clipping o vessels be ore they are cut. o urther
minimize surgical blood loss and blood staining, our types o cautery devices are used selec-
tively: the monopolar cautery, the bipolar microsurgical cautery, the battery-operated handheld
ophthalmic cautery, and the Aquamantys system (radio requency energy with saline coolant).
Each device has a speci c use. T e monopolar cautery is used or subcutaneous dissection, the
bipolar is used or small vessels, and the ophthalmic cautery is used or developing surgical planes,
especially within muscles, which tend to asciculate with the monopolar cautery. T e Aquamantys
system coagulates larger vessels and high venous pressure/variceal types o bleeding commonly
encountered during excision o VMs. issue sealants have been a use ul adjunct at the end o a
procedure a er the tourniquet is def ated. Excision o ast-f ow lesions o the chest and axilla is
a dreaded (but not impossible) procedure and accomplished with the aid o intraarterial balloon
catheters, hypothermia, hypotensive anesthesia, cell savers, adequate blood and f uid resuscitation,
and experienced anesthesiologists and cardiovascular surgeons.
s u r g ic a l in s t r u Me n t s
T e use o ne surgical instruments is critical. We use all incendiary devices that are available,
including the traditional electrocautery and bipolar tool. In addition, the ophthalmic cautery is
excellent or use around muscles, because it does not cause asciculation o the muscle. Vessel
loops are critical or tagging important structures; they are excellent or retraction o these struc-
tures to aid in dissection.
Fig. 15-1

229
Chapter 15: Hand and Upper Extremity
Magni cation, either loupe or microscope, makes a tremendous di erence in the identi cation
and preservation o normal neurovascular structures. Small vessels such as vincular pedicles
to the f exor tendons, nutrient vessels to the digital joints and carpal bones, and dorsal sensory
nerves should be preserved i possible. Vascular anomalies will usually displace but not invade
neighboring so tissues. As such, they are likened to digitopalmar Dupuytren cords that distort
normal structures but do not obliterate them. Indeed, despite the anatomic distortion, an areolar
connective tissue plane is usually preserved along the epineural sur ace o nerves and the adven-
titial plane o normal arteries and veins. T e trick to surgical isolation is upward traction o the
adjacent so tissue and blunt scissor dissection directly along the sur ace o the vessel or nerve.
T ese dissections cannot be per ormed without the use o delicate, undamaged top-quality scissors
and ne-toothed orceps. Our surgical kits contain at least six di erent pairs o scissors or this
purpose. Colored vessel loops are used to encircle veins (blue), arteries (red), nerves (yellow), and
tendons (white) or both retraction during resection and easier identi cation a er the tourniquet
has been released. Large vessels needed or subsequent reconstruction are always marked and
oriented—two clips or veins and one clip or arteries.
An experienced surgical assistant is also an invaluable asset. For the academic surgeon, a se-
nior resident and/or ellow is pre erred; or the private practitioner, these lesions are usually not
handled alone unless they are simple. Nothing eases a tedious dissection better than a pair o
experienced hands to provide retraction, countertraction, and control o unwanted bleeding. For
larger proximal lesions, one surgeon can dissect while another obtains hemostasis. T is type o
collaboration minimizes switching o instruments, thus decreasing blood loss and operative time.
Strength and endurance o both the surgeon and all assistants become important in the treatment
o large, di use LMs, VMs, and LVMs and any ast-f ow lesion involving the axilla, arm, and elbow
regions, in which the maintenance o traction and countertraction o surgical planes is essential.
t a g g in g n o r Ma l s t r u c t u r e s
T e surgeon should identi y and tag normal structures rst. Despite considerable distortion by the
vascular mal ormation, the normal limb anatomy remains constant. T us intimate knowledge o
anatomy is critical to avoid complications. Anatomic anomalies always exist but are the exception
rather than the rule, even in vascular anomalies.
Dorsal veins are marked on the skin be ore exsanguination. Nerves are recognized by the omni-
present perineural at, even in vascular mal ormations and densely scarred regions. T e palmer
dissection in the presence o a di use VM is a di cult undertaking. In the hand, compulsive iden-
ti cation o the sensory nerves and palmer arch should be per ormed in proximal, normal regions
o the hand and then dissected distally; the loops are used or retraction. T e rst 90 minutes
o surgery is the best time to complete this most di cult portion o the procedure, because the
tissues have the least blood staining and swelling. T is approach to identi ying the surrounding
normal structures, aptly termed circling the wagons, is critical in preventing complications. T e
surgeon must move rapidly and con dently through the dissection. T ese lesions can be compli-
cated and un orgiving; the surgeon who stops requently to teach, ponder, and procrastinate will
soon nd himsel or hersel operating within a heavily blood-stained eld. Sterling Bunnell, a
pioneer hand surgeon, aptly likened this experience to “. . . operating in the bottom o an inkwell.”
t h o r o u g h e xc is io n
Discrete but thorough excision is advocated. T e dissection should be limited to a speci ed area
(thus discrete) but should be per ormed thoroughly so that subsequent reentry into a densely
scarred bed will be unnecessary. O en the mal ormation extends well beyond its anticipated
limits. In certain regions limited staged excisions are better than one extensive and protracted dis-

230
Part IV: Anatomic Considerations
section that leaves abnormal tissue behind. For example, the digits should be debulked in stages:
rst, one side through a high midaxial incision and then the other side in another stage. A single,
separate procedure is usually best or the dorsum or palm o the hand and/or wrist in a patient
with a di use LM or VM. T e axilla, brachial plexus, and anterior sur aces o the elbow and wrist
are pre erably dissected in one operation when the neurovascular structures are undamaged. T e
palm o the hand can be approached more than once as long as a thorough dissection is per ormed
within each given region and the tissues to be subsequently removed remain untouched. T e
palmar arches, intrinsic muscles, and all nerves (including the deep motor branch o the ulnar
nerve) can be preserved.
a vo iDin g in t r a n e u r a l Dis s e c t io n
T e surgeon should avoid intraneural dissection whenever possible despite gross involvement. T e
initial identi cation o a nerve or an artery provides the optimal opportunity to place encircling
loops, which may be help ul with retraction and isolation o adjacent structures. Excessive dissec-
tion and/or excessive manipulation o nerves, particularly within VMs, o en leave incontinuity
neuromas, with partial or complete loss o distal sensory or motor unction. T ese secondary
symptoms o pain, dysesthesia, and hyperesthesia are usually much worse than those initially
associated with the vascular mal ormation.
Brachial plexus dissections are challenging in all patients and are per ormed in symptomatic
patients with LMs, VMs, or mixed lesions. Interconnecting macrocysts typically permeate the
plexus, which need not be skeletonized.
a vo iDin g Pa r t ia l Dis s e c t io n
T e surgeon should avoid partial dissections within large muscle groups. VMs, LVMs, and other
mixed lesions can involve the muscle; the decision to resect muscle and the amount to be removed
must be made by the surgeon and can o en be determined be ore surgery. I a signi cant portion
o the muscle is involved, removal o the entire involved muscle en bloc is pre erred to avoid
redissection in the scar. Localized lesions within a muscle can be simply excised. VMs, mixed
capillary-LVMs, LVMs, and capillary-AVMs are the most problematic lesions. Pure LMs do not
characteristically penetrate deep to the muscular ascia; however, they may extend along ascial
planes between muscle groups. It is easy to progress around and between muscle groups without
realizing the depth o the dissection within the axilla, arm, or elbow.
Fig. 15-2

231
Chapter 15: Hand and Upper Extremity
w o u n D c l o s u r e
Meticulous and thought ul closure is mandatory. Frequently, wound closure is considered the
least important portion o the procedure and thus relegated to the most junior member o the
team. However, in complex cases, a poorly planned or executed closure can be a signi cant source
o complications. It takes an experienced eye to identi y poorly per used skin and experienced
hands to eel undue tension on f aps.
It is usually pre erable to remove and replace skin that is heavily scarred rom a previous pro-
cedure, chronically in ected and ulcerated, severely atrophic, populated by coalesced lymphatic
vesicles, or deprived by a proximal steal phenomenon. At the end o the operation, tissues with
questionable viability can be excised and resur aced or observed and resected later. Regions noto-
rious or f ap loss are those in the palm with large VMs and large areas o coalesced skin vesicles
and capillary staining in large LMs or mixed lesions.
Subcuticular closures with absorbable sutures are pre erred in the arm, orearm, and dorsum o
the hand. Care ul eversion o glabrous skin wounds is per ormed with absorbable chromic sutures
in children and nonabsorbable sutures in adults. Despite meticulous technique, any incision o
skin containing an LM may become hypertrophic.
Drains should be used liberally, and delayed primary closure o the wound should be consid-
ered with excessive bleeding. Persistent postoperative bleeding is usually best treated with direct
pressure, elevation, and immobilization rather than by reexploration. Liberal use o tissue sealant
products is encouraged and has been used to control oozing rom unresected portions o the
mal ormation.
iMMo b il iz at io n
T e surgeon should immobilize the operated extremity. Postoperative management can be as
important as preoperative planning and intraoperative execution, especially in children. In the
treatment o children with vascular anomalies, the postoperative lack or loss o immobilization is
the single most important cause o wound dehiscence, maceration, and chronic in ection. T ose
who have had children tend to appreciate this variable more than those who are childless.
However, the avorable aspect o operating on children is that they do not develop sti ness or
joint contractures. T us implementing postoperative rehabilitation is not as important in children
as it is in adult patients.
Pa t ie n t f o l l o w -u P
Follow-up evaluation should be per ormed compulsively at yearly intervals. T ese evaluations
provide: (1) tailoring o the treatment plan based on growth o the lesion, (2) continued learning
or the parents and patients, and (3) continued learning or the surgeon, with increasing awareness
o both surgical and nonsurgical cause and e ect.
Early childhood, adolescence, and pregnancy are times when change may occur in vascular mal-
ormations because o hormonal inf uences. Both slow-f ow and ast-f ow lesions may expand
dramatically during pregnancy with the use o high estrogen antiovulant medication or a er
trauma. Large AVMs, LMs, or mixed lesions may be tolerated well by young children only to
become burdensome to teenagers because o their expansion, bulk, ulceration, and appearance
or steal symptoms. It is unnecessary to repeat scans and other studies unless they will inf uence
treatment decisions.

232
Part IV: Anatomic Considerations
T ese patients and parents o en have questions that are not easily answered by their amily
physician because o the paucity o in ormation available in the medical literature. For them, the
surgeon is not a treating physician but also a valuable resource o in ormation. Similarly, patients
are the surgeon’s most cherished teachers. A surgeon can only improve surgical outcomes in this
di cult eld o surgery with care ul documentation and critical long-term analysis o one’s results.
Pa t ie n t c o u n s e l in g
T e diagnosis and treatment o vascular anomalies is a rapidly changing eld. Both parents and
patients at the appropriate age must be clearly in ormed about the natural history o their partic-
ular mal ormation, options or treatment, and new types o therapies. T ese patients and parents
o en have questions that are not easily answered by their amily physician.
PATIENT EXAMPLES
Pa t ie n t a
T is 4-year-old girl presented with a pain ul and bulky palmar VM a er unsuccess ul sclerother-
apy and partial excision. She had started to neglect the hand. A generous incision was planned
in the thenar f exion crease (A). All neurovascular structures were tented palmarly, with several
extending through the lesion. All these were ully identi ed, dissected, and protected (B). A er
excision o the lesion, the adductor pollicis muscle was seen with the motor branch o the ulnar
nerve (under the background) (C). A er surgery, she experienced signi cant improvement in
unction and pain.
Fig. 15-3
A

B
C