Phẫu Thuật Tạo Hình (Tập 3): Sọ Mặt, Đầu Cổ Và Nhi Khoa – Neligan (Ấn bản 4)

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SECTION III
CHAPTER 39 • Vascular anomalies
targeting specific symptomatic areas often the entire lesion is
too extensive to treat at one time. Sclerotherapy is repeated
until symptoms are alleviated or when injectable vascular
spaces are no longer present. Although sclerotherapy effec­
tively reduces the size of the lesion and improves symptoms,
the malformation remains. Consequently, patients may have
a mass or visible deformity after treatment that may be
improved by resection. In addition, VM usually re-expands
after sclerotherapy, and thus patients often require additional
treatments.
The preferred sclerosants for VM are sodium tetradecyl
sulfate (STS) and ethanol; STS is the most commonly used.
significant LIC who are at risk for DIC.83 Patients who develop
a serious thrombotic event require long-term anticoagulation
or a vena caval filter.
Sclerotherapy
Intervention for VM is reserved for symptomatic lesions that
cause pain, deformity, obstruction (i.e., vision, airway), or
gastrointestinal bleeding. First-line treatment is sclerotherapy,
which is safer and more effective than resection (Fig. 39.10).71
Good to excellent results are obtained in 75–90% of patients,
including reducing the size of the malformation and alleviat­
ing symptoms.71 Diffuse malformations are managed by
A
B
C
Fig. 39.10  Management of venous malformation with sclerotherapy. (A) A 15-year-old female with an enlarging lesion of the left cheek. (B) Axial T2 MR with fat
suppression illustrates a localized lesion involving the cheek. (C) Axial T1 MR exhibits heterogeneous enhancement of the lesion with contrast. (D) Ultrasound shows
compressible hypoechoic venous spaces with echogenic walls. (E) Venogram of the spongiform venous malformation with a minor draining vein. (F) Resolution of facial
asymmetry 2 months following sclerotherapy with sodium tetradecyl sulfate. (Reprinted from Greene AK, Alomari AI. Management of venous malformations. Clin Plast Surg.
2011:38(1):83–93, with permission from Elsevier.)
D
E
F

881
Vascular malformations
Arteriovenous malformation
Pathogenesis
An absent capillary bed causes shunting of blood directly
from the arterial-to-venous circulation, through a fistula
(direct connection of an artery to a vein) or nidus (abnormal
channels bridging the feeding artery to the draining veins).
AVM may enlarge because of increased blood flow causing
collateralization, dilatation of vessels (especially venous
ectasia), and thickening of adjacent arteries and veins.6 Latent
arteriovenous shunts may open, stimulating hypertrophy of
surrounding vessels from increased pressure.6 Alternatively,
aneurysms may increase the size of these lesions. Angiogenesis
(growth of new blood vessels from pre-existing vasculature)
and vasculogenesis (de novo formation of new vasculature)
also may be involved in AVM expansion.83 Although neo­
vascularization may be a primary stimulus for AVM growth,
it also could be a secondary event. For example, ischemia,
a potent stimulator of angiogenesis, causes enlargement of
AVM after proximal arterial ligation or trauma. Alternatively,
increased blood flow due to arteriovenous shunting may
promote angiogenesis; vascular endothelial growth factor
production and endothelial proliferation are stimulated
by increased blood flow.83 Both males and females have a
two-fold risk of progression in adolescence; increased cir­
culating hormones during this period may promote AVM
expansion.84
Clinical features
The most common site of extracranial AVM is the head and
neck, followed by the limbs, trunk, and viscera. Although
present at birth, AVM may not become evident until child­
hood. Early lesions present as a pink-red cutaneous stain
without a palpable thrill or bruit. Often they are initially
mistaken as a capillary malformation or infantile hemangioma.
Arteriovenous shunting reduces delivery of capillary oxygen
causing ischemia. In time, the patient is at risk for pain,
ulceration, and bleeding. AVM also may cause disfigurement,
destruction of tissues, and obstruction of vital structures.
High-pressure shunting of blood may lead to venous hemor­
rhage; ruptured arteries can form in weakened areas, such as
aneurysms. Arterial bleeding most commonly occurs at skin
or mucosal surfaces from erosion into a superficial component
of the lesion. AVMs can cause cardiac enlargement and result
in high-output cardiac failure by the direct communication
Although ethanol is more effective than STS, it has a higher
complication rate. Most patients, especially children, are
managed under general anesthesia using US or fluoroscopic
imaging. The most common local complication of sclerother­
apy for VM is cutaneous ulceration.71 Extravasation of the
sclerosant into muscle can cause atrophy and contracture.
Post-treatment swelling may necessitate close monitoring.
Compartment compression is a serious consequence of sclero­
therapy for extremity VM. Systemic adverse events following
sclerotherapy, including hemolysis, hemoglobinuria, and
DIC, are more common with large lesions. Patients with low
fibrinogen levels are given LMWH 14 days before and after
the procedure.82 Anticoagulation is held for 24 h periopera­
tively (12 h before and after the intervention) to prevent
bleeding complications.
Resection
In contrast to sclerotherapy, resection is rarely primary treat­
ment because (1) the entire lesion is difficult to remove; (2)
the risk of recurrence is high because hidden channels adjacent
to the visible lesion are not excised; (3) the risk of blood loss
and iatrogenic injury is greater. Resection should be consid­
ered for: (1) small, well-localized lesions that can be completely
removed, or (2) persistent mass or deformity after completion
of sclerotherapy (patent channels are no longer accessible for
further injection) (Fig. 39.11). When considering resection, the
postoperative scar/deformity following removal of the VM
should be weighed against the preoperative appearance of the
lesion. Subtotal resection of a problematic area, such as labial
hypertrophy, is indicated, rather than attempting “complete”
excision of a benign lesion that might result in a worse defor­
mity than the malformation itself. Patients and families are
counseled that VM can expand following excision, and addi­
tional operative procedures may be required in the future.
Almost all VMs should have sclerotherapy prior to opera­
tive intervention. After adequate sclerotherapy, the VM is
replaced by scar and thus the risk of blood loss, iatrogenic
injury, and recurrence is reduced. In addition, fibrosis facili­
tates resection and reconstruction. Because GVM is usually
small and less amenable to sclerotherapy, first-line therapy for
painful lesions may be resection. Gastrointestinal VM with
chronic bleeding, anemia, and transfusion requirements is
typically managed by resection. Solitary lesions can be treated
by endoscopic banding or sclerotherapy. Multifocal lesions of
BRBNS require removal of as many lesions as possible through
multiple enterotomies, instead of bowel resection, to preserve
intestinal length.
Fig. 39.11  Management of venous malformation with sclerotherapy followed by resection. (A) A 2.5-year-old female with an enlarging venous malformation of the upper
lip. (B) Reduction of the venous malformation after two sessions of sclerotherapy. Further sclerotherapy was not possible because venous spaces had been replaced by
fibrosis. (C) Improved contour 3 months after resection of residual scarred venous malformation using a transverse mucosal incision.
A
B
C

882
SECTION III
CHAPTER 39 • Vascular anomalies
long-term control or “cure”, intervention must be individual­
ized based on the deformity that would be caused by resection
and reconstruction. For example, a large Stage I AVM in a
non-anatomically important location (i.e., trunk, proximal
extremity) can be resected without consequence before it pro­
gresses to a higher stage where resection is more difficult and
the recurrence rate is greater. Similarly, a small, well-localized
AVM in a more difficult location (i.e., face, hand) may be
excised for possible “cure” before it expands and complete
extirpation is no longer possible.
In contrast, a large, asymptomatic AVM located in an
anatomically sensitive area is best observed; especially in a
young child who is not psychologically ready for major resec­
tion and reconstruction. Although the recurrence rate is lower
when Stage I AVM is resected, it is still high, and thus, even
after major resection and reconstruction the malformation can
recur. Some patients (17%) do not have significant long-term
morbidity.84
Intervention for Stage II AVMs is similar to Stage I lesions.
The threshold for treatment is lower if an enlarging lesion is
causing a worsening deformity or if functional problems are
occurring. Stage III and IV AVMs require intervention to
control pain, bleeding, ulceration, or congestive heart failure.
Embolization
Embolization is the delivery of an inert substance, through a
catheter into the AVM nidus to occlude blood flow and/or fill
a vascular space. Scarring reduces arteriovenous shunting,
shrinks the lesion, and diminishes symptoms. Embolization
is used either as a preoperative adjunct to resection or as
monotherapy for lesions not amenable to extirpation. Because
the AVM is not removed, almost all lesions eventually
re-expand after treatment. Stage I AVM has a lower recurrence
rate than higher-staged lesions. Most recurrences occur within
the first year after embolization, and 98% re-expand within 5
years.84 Despite the high likelihood of re-expansion, emboliza­
tion can effectively palliate an AVM by reducing its size,
slowing expansion, and alleviating pain and bleeding. Preop­
erative embolization also reduces blood loss during extirpa­
tion, but not the extent of resection.
Substances used for embolization are either liquid (n-butyl
cyanoacrylate (n-BCA), Onyx) or solid (polyvinyl alcohol
particles (PVA), coils). The goal of embolization is occlusion
of the nidus and proximal venous outflow. The embolic mate­
rial is delivered to the nidus, not to the proximal arterial
feeding vessels. Occlusion of inflow will cause collateraliza­
tion and expansion of the AVM; access to the nidus also will
be blocked preventing future embolization.86 For preoperative
embolization, temporary occlusive substances (Gelfoam
powder, PVA, embospheres) that undergo phagocytosis are
used. Permanent liquid agents capable of permeating the
nidus (n-BCA, Onyx) are employed when embolization is
the primary treatment.86 The most frequent complication of
embolization is ulceration.
Resection
Resection of AVM has a lower recurrence rate than embo­
lization alone; it is considered for a well-localized lesion or
to correct deformity (i.e., bleeding or ulcerated areas, labial
hypertrophy) (Fig. 39.12). Wide extirpation and reconstruc­
tion of large, diffuse AVM should be exercised with caution
because (1) cure is rare and the recurrence rate is high; (2)
between the high-resistance, high-pressure arterial system
and the low-pressure, low-resistance venous system. While
the presence of an AVM may be troublesome, it is the expan­
sion of the lesion that is the primary cause of morbidity. AVM
can be classified according to the Schobinger staging system
(Table 39.4).85
Most AVMs are diagnosed by history and physical exami­
nation. Hand held Doppler reveals fast-flow and excludes a
slow-flow vascular anomaly. If AVM is suspected, the diagno­
sis should be confirmed by US with color Doppler examina­
tion. MRI also is necessary to (1) confirm the diagnosis; (2)
determine the extent of the lesion; (3) plan treatment. To
adequately assess the anomaly, MRI with contrast and fat
suppression, as well as a T2-weighted sequences, is neces­
sary.20 MRI shows dilated feeding arteries and draining veins,
enhancement, and flow-voids.20 If the diagnosis remains
unclear after US and MRI, angiography is sometimes needed.
Usually angiography only is indicated prior to embolization
or when resection is planned. Characteristic features are tortu­
ous, dilated arteries with arteriovenous shunting and dilated
draining veins.20 The nidus is angiographically seen as tortu­
ous, small vessels with occasionally ill-defined larger contigu­
ous vascular spaces. CT may be indicated if the AVM involves
bone. Histopathologic diagnosis of AVM is rarely necessary,
but may be indicated to rule out malignancy or if imaging is
equivocal.
Management
Because AVM is often diffuse, involving multiple tissue planes
and important structures, cure is rare. The goal of treatment
usually is to control the malformation. Intervention is focused
on alleviating symptoms (i.e., bleeding, pain, ulceration),
preserving vital functions (i.e., vision, mastication), and
improving deformity. Management options include emboliza­
tion, resection, or a combination. Resection offers the best
chance for long-term control, but the re-expansion rate is high
and extirpation may cause a worse deformity.84 Almost all
AVMs will re-expand after embolization.84 Consequently,
embolization is used to reduce blood loss during resection or
sometimes for palliation of unresectable lesions.
Asymptomatic AVM should be observed unless it can
be completely removed with minimal morbidity; emboliza­
tion or incomplete excision of an asymptomatic lesion may
stimulate it to enlarge and become problematic. Intervention
is determined by (1) the size and location of the AVM; (2) the
age of the patient; (3) Schobinger stage. Although resection
of an asymptomatic Stage I AVM offers the best chance for
Table 39.4  Schobinger staging of arteriovenous malformation
(AVM)
Stage
Clinical findings
I (Quiescence)
Warm, pink-blue, shunting on Doppler
II (Expansion)
Enlargement, pulsation, thrill, bruit,
tortuous veins
III (Destruction)
Dystrophic skin changes, ulceration,
bleeding, pain
IV (Decompensation)
Cardiac failure

883
Vascular malformations
that AVM is likely to re-expand following resection, and thus
additional treatment may be required.
Capillary malformation-arteriovenous
malformation
Capillary malformation-arteriovenous malformation (CM-
AVM) is an autosomal dominant condition caused by a
loss-of-function mutation in the RASA1 gene; the prevalence
is 1 in 100 000 Caucasians.87 Patients have atypical capillary
malformations (CMs) that are small, multifocal, round,
pinkish-red, and often surrounded by a pale halo (50%) (Fig.
39.13).84 A total of 30% also have an AVM: Parkes Weber
syndrome (PWS) (12%), extracerebral AVM (11%), or intrace­
rebral AVM (7%).88 A patient presenting with multiple CMs,
especially with a family history of similar lesions, should be
evaluated for a possible AVM. Because 7% of patients with
CM-AVM will have an intracranial fast-flow lesion, brain
MRI should be considered.88 Exploratory imaging of other
anatomic areas is not necessary because extracranial AVM
have not been found to involve the viscera.88 Although the
CM is rarely problematic, associated AVMs can cause major
morbidity.
the resulting deformity is often worse than the appearance
of the malformation; (3) resection is associated with sig­
nificant blood loss, iatrogenic injury, and morbidity. When
excision is planned, preoperative embolization will facilitate
the procedure by reducing the size of the AVM, minimizing
blood loss, and creating scar tissue to aid the dissection. Mul­
tiple embolizations, spaced 6 weeks apart, may be required
prior to resection. Excision should be done 24–72 h after
embolization, before recanalization restores blood flow to
the lesion.
Resection margins are best determined by assessing the
type of bleeding from the wound edges. Some defects can be
reconstructed by advancing local skin flaps. Skin grafting
ulcerated areas has a high failure rate because the underlying
tissue is ischemic; excision with regional flap transfer may be
required. Free-flap reconstruction permits wide resection and
primary closure of complicated defects, but does not appear
to improve long-term AVM control.84 Despite sub-total and
presumed “complete” extirpation, most AVMs treated by
resection recur. The majority of recurrences occur within the
first year after intervention and 86.6% re-expand within 5
years of resection.84 Nevertheless, many of these patients
remain asymptomatic. Patients and families are counseled
C
B
A
Fig. 39.12  Management of arteriovenous malformation. (A) A 39-year-old woman with an enlarging, bleeding, ulcerated stage 3 lesion of the forehead and scalp.
(B) Intraoperative view following preoperative embolization. (C) Wound after extirpation. (D) Split-thickness skin graft coverage of periosteum. (E,F) Healed graft 4 months
postoperatively.
D
E
F

884
SECTION III
CHAPTER 39 • Vascular anomalies
(V1), extend into the maxillary (V2), or involve all three tri­
geminal dermatomes. Patients with maxillary or mandibular
involvement alone are at low risk for SWS. The leptomenin­
geal anomalies can be capillary, venous, or arteriovenous
malformations. Small foci may be silent, but extensive pial
vascular lesions can cause refractory seizures, contralateral
hemiplegia, and delayed motor and cognitive development.
The anomalous choroidal vascularity can lead to retinal
detachment, glaucoma, and blindness. Ophthalmic examina­
tion should be performed every 6 months until the age of 2
years and yearly thereafter. MRI best demonstrates pial vas­
cular enhancement in an infant or child thought to have
Sturge–Weber syndrome.
Klippel–Trénaunay syndrome
Klippel–Trénaunay syndrome (KTS) denotes a slow-flow,
capillary-lymphatic-venous malformation (CLVM) of an
extremity in association with soft tissue and/or skeletal
Eponymous vascular anomalies
with overgrowth
Sturge–Weber syndrome
Sturge–Weber syndrome (SWS) is a sporadic neurocutaneous
disorder estimated to occur in 1 in 50 000 live births (Fig.
39.14A).89 The three cardinal features are capillary malforma­
tion (CM) in the upper trigeminal neural distribution, ocular
abnormalities (glaucoma, choroidal vascular anomalies), and
leptomeningeal vascular malformation.89 Patients also com­
monly have soft tissue and/or bony overgrowth (60–83%); the
frequency is similar to that for glaucoma (65–77%) and for
neurologic sequelae (87–93%).57 In addition to facial capillary
staining; extra-craniofacial CMs (29%) and extremity hyper­
trophy (14%) are frequently present.57
In all patients with an upper facial CM, a diagnosis of
Sturge–Weber syndrome should be considered on initial
presentation. The capillary stain can be in the ophthalmic
Fig. 39.13  Capillary malformation-arteriovenous malformation (CM-AVM). (A) A 1-year-old female with multifocal capillary malformations of the chest and shoulder.
(B,C) Arteriovenous malformation of left lower extremity with overgrowth.
A
B
C
Fig. 39.14  Eponymous vascular anomalies with overgrowth. (A) A 36-year-old man with Sturge–Weber syndrome. (B) Infant female with Klippel–Trenaunay syndrome.
(C) A 7-year-old male with Parkes Weber syndrome. (D) An 11-year-old male with CLOVES syndrome.
A
B
C
D

885
Vascular malformations
affected extremity is subcutaneous, muscular, and bony with
diffuse microfistulas. The enlarged limb muscles and bones
exhibit an abnormal signal and enhancement. Angiography
demonstrates discrete arteriovenous shunts.
Treatment is predicated on symptoms. In rare instances, an
infant presents with high-output congestive heart failure
secondary to shunting through arteriovenous fistulas. This
situation mandates emergent embolization with permanent
occlusive agents, often followed by repeated procedures.
Children are observed annually with careful monitoring for
axial overgrowth and development of cutaneous problems.
Embolization may be useful for pain or cutaneous ischemic
changes. Occasionally, amputation is necessary.
PTEN hamartoma-tumor syndrome (Bannayan–
Riley–Ruvalcaba syndrome)
Patients with PTEN mutations, a tumor suppressor gene, have
PTEN hamartoma-tumor syndrome (PHTS) (Fig. 39.15). This
autosomal dominant condition is also referred to as Cowden
syndrome or Bannayan–Riley–Ruvalcaba syndrome.93 Males
and females are equally affected, and approximately one-half
(54%) of patients have a unique fast-flow vascular anomaly
with arteriovenous shunting, referred to as a PTEN-associated
vascular anomaly.93 Patients may have multiple lesions (57%),
and 85% are intramuscular.93
Suspicion of a PTEN-associated vascular anomaly usually
is initiated after reviewing the MRI or angiographic study of
a patient suspected of having an AVM. Unlike typical AVM,
these lesions may be multifocal, are associated with ectopic
adipose tissue, and have disproportionate, segmental dilation
of the draining veins.93 If a patient is suspected of having a
PTEN-associated vascular anomaly on imaging, a physical
examination is performed. All patients with PHTS have
macrocephaly (>97th percentile), and all males have penile
freckling.93 In addition, PHTS is associated with mental
retardation/autism (19%), thyroid lesions (31%), or gastro­
intestinal polyps (30%).93 Biopsy may aid the diagnosis of a
PTEN fast-flow lesion. Histopathology shows skeletal muscle
infiltration with adipose tissue, fibrous bands, and lymphoid
aggregates. In addition, tortuous arteries with transmural
muscular hyperplasia and clusters of abnormal veins with
variable smooth muscle are present.93 Genetic testing is con­
firmative, although a germline mutation is not found in 9% of
families clinically diagnosed with PHTS.92
If physical examination is consistent with PHTS, molecular
testing is necessary because this mutation is associated with
multiple benign and malignant tumors which require surveil­
lance. Patients are followed closely for the presence of tumors,
particularly endocrine and gastrointestinal malignancies. In
addition, the patient and family are counseled about the risk
of transmitting the gene to their offspring. Symptomatic
lesions are managed similarly to non-syndromic AVM, with
embolization or resection. It is our experience that the recur­
rence rate after these interventions is even higher than for
non-syndromic AVM, possibly because the loss of the tumor
suppressor protein favors a more proliferative environment.
CLOVES syndrome
Congenital Lipomatosis Overgrowth, Vascular malforma­
tions, Epidermal nevi, and Scoliosis (CLOVES) represents a
overgrowth (Fig. 39.14B).90 There is tremendous variability
in the presentation of this disorder, from a slightly enlarged
extremity with a capillary stain to a grotesquely enlarged limb
with malformed digits. KTS affects the lower extremity in 95%
of patients, the upper extremity in 5% of patients, and least
commonly, the trunk.90 Sometimes the contralateral foot or
hand is enlarged, often with a macrodactylous component and
frequently in the absence of a capillary stain. In 10% of patients
with KTS, the involved limb is hypoplastic. Pelvic involvement
is common with CLVM of the lower extremity. It is usually
asymptomatic, although hematuria, bladder outlet obstruc­
tion, cystitis, and hematochezia can occur. Upper extremity or
truncal CLVM can involve the posterior mediastinum and ret­
ropleural space, although this is rarely symptomatic. The capil­
lary malformation is distributed in a geographic pattern over
the lateral side of the extremity, buttock, or thorax. Whereas
the capillary malformation is typically macular in a neonate,
later it becomes studded with hemolymphatic vesicles. The
venous component of CLVM manifests as abnormal drainage
of the affected area. The lymphatic abnormalities are typically
macrocystic in the pelvis and thighs and microcystic in the
abdominal wall, buttock, and distal limb.
MRI is obtained to confirm the diagnosis and determine the
extent of the anomalies. A large, embryonal vein in the sub­
cutaneous tissue (the marginal vein of Servelle) is often
located in the lateral calf and thigh and communicates with
the deep venous system. Complications include thrombophle­
bitis (20–45%) and pulmonary embolism (4–24%).90 Unlike
some other hemihypertrophy syndromes, patients with KTS
are not at increased risk for Wilms tumor and screening US is
unnecessary.91,92 By 2 years of age, radiologic surveillance of
leg length by plain radiography is indicated. If the discrepancy
is >1.5 cm, a shoe-lift for the shorter limb can prevent limping
and scoliosis. Epiphysiodesis of the distal femoral growth
plate is typically done around 11 years of age. Enlargement of
the foot may require a ray, midfoot, or Syme amputation to
allow the use of footwear. Management of the VM component
is conservative with compressive stockings for insufficiency
and aspirin to minimize phlebothrombosis. Symptomatic
varicose veins may be removed or sclerosed. Sclerotherapy
may be necessary for focal macrocystic lymphatic malforma­
tion or to treat cutaneous vesicles. Excision and grafting is
occasionally necessary for diffuse bleeding and/or oozing
cutaneous vesicles. Circumferential overgrowth may be
managed by staged contour resection. Venous insufficiency
does not occur following staged subcutaneous excision or
removal of the marginal vein of Servelle. A functioning deep
venous system is present, although it is often difficult to
visualize because of predominant flow in the superficial veins.
Parkes Weber syndrome
Parkes Weber syndrome (PWS) is a diffuse AVM in an over­
grown extremity with an overlying CM (Fig. 39.14C).88 PWS
involves the lower extremity approximately twice as often as
the upper extremity; patients have microshunting in muscle.
The malformation is evident at birth with symmetric enlarge­
ment and pink staining of the involved limb. The cutaneous
stain tends to be confluent rather than patchy and is typically
warmer than a banal capillary malformation. The diagnosis is
confirmed by the detection of a bruit or thrill. MRI is obtained
to evaluate the extent of the malformation. Overgrowth in an

886
SECTION III
CHAPTER 39 • Vascular anomalies
specialist had sufficient knowledge to treat the wide variety
of disorders.
Terminologic confusion has been replaced with a common
language. Interested specialists can now communicate with
one another. Vascular anomaly teams, composed of various
disciplines on the basis of local interest, enthusiasm, and
capabilities, continue to form in many major referral centers.
These teams are in a unique position because the collective
knowledge of such a group provides a forum for problems
that otherwise appear “too complicated” or “insoluble”. In
addition, they serve as a focus for clinical and basic research
in this field.
The future of the field of vascular anomalies is exciting
because a significant opportunity exists to improve the lives
of these patients. Plastic surgeons are well-positioned to make
progress because of their training; management of these
lesions requires creativity, surgical problem-solving skills, and
a mastery of operative principles. Plastic surgeons, in collabo­
ration with other specialists, will continue to be primary
caretakers of patients with vascular anomalies.
newly described overgrowth syndrome.94 Many of these
patients previously were thought to have “Proteus syndrome”.
All patients have a truncal lipomatous mass, a slow-flow
vascular malformation (most commonly a CM overlying the
lipomatous mass), and hand/foot anomalies (increased width,
macrodactyly, first web-space sandal gap) (Fig. 39.14D).94
Patients also may have AVM (28%), neurologic impairment
(50%), or scoliosis (33%).94 Treatment for the lipomatous
lesions is resection, but the recurrence rate is high.
Conclusion
Patients with vascular anomalies all too often have been
medical “nomads”. During infancy and childhood, their
parents took them from one physician to another, because
no one seemed to understand the condition. The problem
usually was that these anomalies lay in the interface
between several medical and surgical disciplines. No single
A
B
C
Fig. 39.15  PTEN hamartoma-tumor syndrome (Bannayan–Riley–Ruvalcaba syndrome). (A) A 16-year-old male with enlarging, painful scalp lesion and (A) penile freckling
associated with the syndrome. (B) Axial T1 MR image shows enhancing soft tissue lesion consistent with PTEN-associated vascular anomaly. (C) Angiogram illustrates
arteriovenous shunting without a nidus. (D) Onyx cast of lesion following preoperative embolization. (E) Intraoperative view. (F) A 9 × 4.5 cm wound following resection.
(G) Linear closure after wide scalp undermining and subgaleal scoring.
D
E
F
G

887
Conclusion
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propranolol for infantile hemangioma: report of a consensus
conference. Pediatrics. 2013;131:128–140.
76. Vikkula M, Boon LM, Carraway KL, et al. Vascular
dysmorphogenesis caused by an activating mutation in the receptor
tyrosine kinase TIE2. Cell. 1996;87:1181–1190. Two families with
inherited multiple venous malformations were found to have an activating
mutation in the endothelial receptor tyrosine kinase TIE2, suggesting that
the TIE2 signaling pathway is critical for endothelial-smooth muscle cell
interaction. This was the first mutation implicated in the pathogenesis of
vascular anomalies; the disorder is called cutaneous-mucosal venous
malformation.
Access the complete reference list online at

887.e1
References
of 143 hemangiomas, 66 vascular malformations, 20 pyogenic granulomas,
and five hemangioendotheliomas. GLUT1 (erythrocyte-type glucose
transporter) only was expressed in infantile hemangioma (during
proliferation and involution). GLUT1 is a sensitive marker to differentiate
infantile hemangioma from congenital hemangiomas, other vascular
tumors, and vascular malformations.
22. Couto JA, Maclellan RA, Greene AK. Infantile hemangioma:
treatment rate during the proliferating phase. J Craniofac Surg.
2014;25:1933–1934.
23. Chamlin SL, Haggstrom AN, Drolet BA, et al. Multicenter
prospective study of ulcerated hemangiomas. J Pediatr.
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24. Garzon MC, Lucky AW, Hawrot A, et al. Ultrapotent topical
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25. McMahon P, Oza V, Frieden IJ. Topical timolol for infantile
hemangiomas: putting a note of caution in “cautiously optimistic”.
Pediatr Dermatol. 2012;29:127–130.
26. Couto JA, Greene AK. Management of problematic infantile
hemangioma using intralesional triamcinolone: efficacy and safety
in 100 infants. J Plast Reconstr Aesthet Surg. 2014;67:1469–1474.
27. Zarem HA, Edgerton MT. Induced resolution of cavernous
hemangiomas following prednisolone therapy. Plast Reconstr Surg.
1967;39:76–83.
28. Boon LM, MacDonald DM, Mulliken JB. Complications of systemic
corticosteroid therapy for problematic hemangiomas. Plast Reconstr
Surg. 1999;104:1616–1623.
29. Bennett ML, Fleischer AB, Chamlin SL, et al. Oral corticosteroid use
is effective for cutaneous hemangiomas. Arch Dermatol. 2001;137:
1208–1213.
30. Greene AK, Couto RA. Oral prednisolone for infantile hemangioma:
efficacy and safety using a standardized treatment protocol. Plast
Reconstr Surg. 2011;128:743–752.
31. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al.
Propranolol for severe hemangiomas of infancy. N Engl J Med.
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32. Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of
propranolol for infantile hemangioma: report of a consensus
conference. Pediatrics. 2013;131:128–140.
33. Lawley LP, Siegfried E, Todd JL. Propranolol treatment for
hemangioma of infancy: risks and recommendations. Pediatr
Dermatol. 2009;26:610–614.
34. Frieden IL, Drolet BA. Propranolol for infantile hemangiomas:
promise, peril, pathogenesis. Pediatr Dermatol. 2009;26:642–644.
35. Holland KE, Frieden IJ, Frommelt PC, et al. Hypoglycemia in
children taking propranolol for the treatment of infantile
hemangioma. Arch Dermatol. 2010;146:775–778.
36. Pavlakovic H, Kietz S, Lauerer P, et al. Hyperkalemia complicating
propranolol treatment of an infantile hemangioma. Pediatrics.
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37. Langley A, Pope E. Propranolol and central nervous system
function: potential implications for paediatric patients with infantile
haemangiomas. Br J Dermatol. 2015;172:13–23.
38. Scheepers JH, Quaba AA. Does the pulsed tunable dye laser have a
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based on 3 years’ experience. Plast Reconstr Surg. 1995;95:
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39. Batta K, Goodyear HM, Moss C, et al. Randomized controlled study
of early pulsed dye laser treatment of uncomplicated childhood
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40. Witman PM, Wagner AM, Scherer K, et al. Complications following
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41. Mulliken JB, Rogers GF, Marler JJ. Circular excision of hemangioma
and purse-string closure: the smallest possible scar. Plast Reconstr
Surg. 2002;109:1544–1554.
42. Boon LM, Enjolras O, Mulliken JB. Congenital hemangioma:
evidence of accelerated involution. J Pediatr. 1996;128:329–
335.
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2. Enjolras O, Mulliken JB. Vascular tumors and vascular
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11. Kilcline C, Frieden IJ. Infantile hemangiomas: how common are
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12. Drolet BA, Swanson EA, Frieden IJ. Infantile hemangiomas: an
emerging health issue linked to an increased rate of low birth
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14. Couto RA, Maclellan RA, Zurakowski D, et al. Infantile
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15. Horii KA, Drolet BA, Frieden IJ, et al. Prospective study of the
frequency of hepatic hemangiomas in infants with multiple
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16. Kulungowski AM, Alomari AI, Chawla A, et al. Lessons from a
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17. Huang SA, Tu HM, Harney JW, et al. Severe hypothyroidism
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18. Metry DW, Garzon MC, Drolet BA, et al. PHACE syndrome:
current knowledge, future directions. Pediatr Dermatol. 2009;26:
381–398.
19. Iacobas I, Burrows PE, Frieden IJ, et al. LUMBAR: association
between cutaneous infantile hemangiomas of the lower body and
regional congenital anomalies. J Pediatr. 2010;157:795–801.
20. Arnold R, Chaudry G. Diagnostic imaging of vascular anomalies.
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21. North PE, Waner M, Mizeracki A, et al. GLUT1: a newly discovered
immunohistochemical marker for juvenile hemangiomas. Hum
Pathol. 2000;31:11–22. This is a retrospective immunohistochemical study

887.e2
SECTION III
CHAPTER 39 • Vascular anomalies
66. Toriello HV, Mulliken JB. Accurately renaming macrocephaly-cutis
marmorata telangiectatica congenital (M-CMTC) as macrocephaly-
capillary malformation (M-CM). Am J Med Genet A. 2007;143A:3009.
67. Luks VL, Kamitaki N, Vivero MP, et al. Lymphatic and other
vascular malformative/overgrowth disorders are caused by somatic
mutations in PIK3CA. J Pediatr. 2015;166:1048–1054.e1–e5.
68. Limaye N, Boon LM, Vikkula M. From germline towards somatic
mutations in the pathophysiology of vascular anomalies. Hum Mol
Genet. 2009;18:65–75.
69. Greene AK, Burrows PE, Smith L, et al. Periorbital lymphatic
malformation: clinical course and management in 42 patients. Plast
Reconstr Surg. 2005;115:22–30.
70. Lala S, Mulliken JB, Alomari AI, et al. Gorham-Stout disease and
generalized lymphatic anomaly–clinical, radiologic, and histologic
differentiation. Skeletal Radiol. 2013;42:917–924.
71. Choi DJ, Alomari AI, Chaudry G, et al. Neurointerventional
management of low-flow vascular malformations of the head and
neck. Neuroimaging Clin N Am. 2009;19:199–218.
72. Chaudry G, Guevara CJ, Rialon KL, et al. Safety and efficacy of
bleomycin sclerotherapy for microcystic lymphatic malformation.
Cardiovasc Intervent Radiol. 2014;37:1476–1481.
73. Kim SW, Kavanagh K, Orbach DB, et al. Long-term outcome of
radiofrequency ablation for intraoral microcystic lymphatic
malformation. Arch Otolaryngol Head Neck Surg. 2011;137:
1247–1250.
74. Limaye N, Wouters V, Uebelhoer M, et al. Somatic mutations in
angiopoietin receptor gene TEK cause solitary and multiple
sporadic venous malformations. Nat Genet. 2009;41:118–124.
75. Boon LM, Mulliken JB, Enjolras O, et al. Glomuvenous
malformation (glomangioma) and venous malformation: distinct
clinicopathologic and genetic entities. Arch Dermatol. 2004;140:
971–976.
76. Vikkula M, Boon LM, Carraway KL, et al. Vascular
dysmorphogenesis caused by an activating mutation in the receptor
tyrosine kinase TIE2. Cell. 1996;87:1181–1190. Two families with
inherited multiple venous malformations were found to have an activating
mutation in the endothelial receptor tyrosine kinase TIE2, suggesting that
the TIE2 signaling pathway is critical for endothelial-smooth muscle cell
interaction. This was the first mutation implicated in the pathogenesis of
vascular anomalies; the disorder is called cutaneous-mucosal venous
malformation.
77. Fishman SJ, Smithers CJ, Folkman J, et al. Blue rubber bleb nevus
syndrome: surgical eradication of gastrointestinal bleeding. Ann
Surg. 2005;241:523–528.
78. Kubiena HF, Liang MG, Mulliken JB. Genuine diffuse phlebectasia
of Bockenheimer: dissection of an eponym. Pediatr Dermatol.
2006;23:294–297.
79. Tennant LB, Mulliken JB, Perez-Atayde AR, et al. Verrucous
hemangioma revisited. Pediatr Dermatol. 2006;23:208–215.
80. Couto JA, Vivero MP, Kozakewich HP, et al. A somatic MAP3K3
mutation is associated with verrucous venous malformation. Am J
Hum Genet. 2015;96:480–486.
81. Alomari AI, Spencer SA, Arnold RW, et al. Fibro-adipose vascular
anomaly: clinical-radiologic-pathologic features of a newly
delineated disorder of the extremity. J Pediatr Orthop. 2014;
34:109–117.
82. Kaplan RP, Wang JT, Amron DM, et al. Maffucci’s syndrome: two
case reports with a literature review. J Am Acad Dermatol.
1993;29:894–899.
83. Adams DM. Special considerations in vascular anomalies:
hematologic management. Clin Plast Surg. 2011;38:153–160.
84. Lu L, Bischoff J, Mulliken JB, et al. Increased endothelial progenitor
cells and vasculogenic factors in higher-staged arteriovenous
malformations. Plast Reconstr Surg. 2011;128:260e–269e.
85. Liu AS, Mulliken JB, Zurakowski D, et al. Extracranial
arteriovenous malformations: natural progression and recurrence
after treatment. Plast Reconstr Surg. 2010;125:1185–1194.
86. Kohout MP, Hansen M, Pribaz JJ, et al. Arteriovenous
malformations of the head and neck: natural history and
management. Plast Reconstr Surg. 1998;102:643–654.
43. Enjolras O, Mulliken JB, Boon LM, et al. Noninvoluting congenital
hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr
Surg. 2001;107:1647–1654.
44. Berenguer B, Mulliken JB, Enjolras O, et al. Rapidly involuting
congenital hemangioma: clinical and histopathologic features.
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45. Kasabach HH, Merritt KK. Capillary hemangioma with extensive
purpura: report of a case. Am J Dis Child. 1940;59:1063–1070.
46. Zukerberg LR, Nikoloff BJ, Weiss SW. Kaposiform
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neoplasm associated with Kasabach–Merritt syndrome and
lymphangiomatosis. Am J Surg Pathol. 1993;17:321–328.
47. Sarkar M, Mulliken JB, Kozakewich HP, et al. Thrombocytopenic
coagulopathy (Kasabach–Merritt phenomenon) is associated with
kaposiform hemangioendothelioma and not with common infantile
hemangioma. Plast Reconstr Surg. 1997;100:1377–1386.
48. Lyons LL, North PE, Mac-Moune Lai F, et al. Kaposiform
hemangioendothelioma: a study of 33 cases emphasizing its
pathologic, immunophenotypic, and biologic uniqueness from
juvenile hemangioma. Am J Surg Pathol. 2004;28:559–568.
49. Karnes JC, Lee BT, Phung T, et al. Adult-onset kaposiform
hemangioendothelioma in a post-traumatic site. Ann Plast Surg.
2009;62:456–458.
50. Mulliken JB, Anupindi S, Ezekowitz RA, et al. Case 13–2004: A
newborn girl with a large cutaneous lesion, thrombocytopenia, and
anemia. N Engl J Med. 2004;350:1764–1775.
51. Croteau SE, Liang MG, Kozakewich HP, et al. Kaposiform
hemangioendothelioma: atypical features and risks of Kasabach-
Merritt phenomenon in 107 referrals. J Pediatr. 2013;162:142–147.
52. Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-Merritt
phenomenon: a retrospective study of treatment with vincristine. J
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53. Drolet BA, Trenor CC 3rd, Brandão LR, et al. Consensus-derived
practice standards plan for complicated Kaposiform
hemangioendothelioma. J Pediatr. 2013;163:285–291.
54. Patrice SJ, Wiss K, Mulliken JB. Pyogenic granuloma (lobular
capillary hemangioma): a clinicopathologic study of 178 cases.
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55. Lee J, Sinno H, Tahiri Y, et al. Treatment options for cutaneous
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56. Shirley MD, Tang H, Gallione CJ, et al. Sturge-Weber syndrome and
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57. Greene AK, Taber SF, Ball KL, et al. Sturge-Weber syndrome:
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58. Tan OT, Sherwood K, Gilchrest BA. Treatment of children with
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59. van der Horst CM, Koster PH, de Borgie CA, et al. Effect of the
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60. Stier MF, Glick SA, Hirsch RJ. Laser treatment of pediatric vascular
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61. Chapas AM, Eickhorst K, Geronemus RG. Efficacy of early
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64. Lee MS, Liang MG, Mulliken JB. Diffuse capillary malformation
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65. Amitai DB, Fichman S, Merlob P, et al. Cutis marmorata
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92. Greene AK, Kieran M, Burrows PE, et al. Wilms tumor screening
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93. Tan WH, Baris HN, Burrows PE, et al. The spectrum of vascular
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87. Wu IC, Orbach DB. Neurointerventional management of high-flow
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88. Eerola I, Boon LM, Mulliken JB, et al. Capillary malformation-
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89. Revencu N, Boon LM, Mulliken JB, et al. Parkes Weber syndrome,
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90. Comi AM. Pathophysiology of Sturge–Weber syndrome. J Child
Neurol. 2003;18:509–516.

SECTION III • Pediatrics
40 
Pediatric chest and trunk defects
Lawrence J. Gottlieb, Russell R. Reid, and Mark B. Slidell
SYNOPSIS
■ Pediatric trunk defects require multidisciplinary comprehensive care in
order to maximize patient safety and successful outcome.
■ Reconstructive surgery for the pediatric patient requires consideration
for the paucity of tissue available in a small body habitus, the
necessity for growth, and the tenuous physiology that may accompany
children afflicted with congenital defects.
■ Closure of ventral body wall defects may pose significant challenges to
the neonatal circulation, whereas closure of dorsal body wall defects
must address exposed neural elements.
Introduction
Patients with severe congenital trunk defects are frequently
diagnosed during prenatal ultrasonography or at birth and
require the coordination of multiple disciplines, including
reconstructive plastic surgery. Reconstructive surgery for the
pediatric patient requires consideration for the paucity of
tissue available in a small body habitus, the impact of surgery
on future growth of the child, and the tenuous physiology that
may accompany children afflicted with congenital defects.
Body wall defects have the potential risk of exposing vital
structures and subsequent infection. Closure of ventral body
wall defects may pose significant challenges to the neonatal
circulation, whereas closure of dorsal body wall defects must
address exposed neural elements.
Embryology
Development of the body wall begins at 4 weeks’ gestation
when the mesoderm organizes into the paraxial, intermediate,
and lateral plate layers. The paraxial mesoderm, adjacent to
the neural tube, differentiates into the skeletal support and
surrounding soft tissue that characterize the dorsal body wall
and encases the central nervous system. Intermediate meso­
derm forms the urogenital structures. Lateral plate mesoderm
differentiates into both the soft tissue and skeletal components
of the ventral body wall. The lateral plate mesoderm, with
ectoderm covering, folds and fuses at the end of the 4th week
of gestation.1,2 Congenital ventral body wall defects result
from the lack of lateral plate fusion.
Thoracic wall defects
Pectus excavatum
Pectus excavatum, also known as “sunken chest” or “funnel
chest”, is the most common congenital anterior thoracic wall
deformity. It occurs in 1 in 300–400 live births and makes up
~90% of the thoracic wall defects seen. Pectus excavatum is
characterized by a depression of the sternum, as well as costal
cartilage displacement. Males outnumber females 3 : 1 and
patients are predominantly Caucasian. Pectus excavatum is
often identified within the first years of life although more
subtle defects are frequently not noticed until teenage years.
Most typically, a mild pectus deformity will become more
pronounced in the rapid growth period during puberty.
Pectus deformities often carry a familial component, with
37–47% of patients reporting a family history. Inheritance
patterns are not fully understood, but studies of different kin­
dreds have shown autosomal dominant, autosomal recessive,
X-linked, and multifactorial inheritance. No definitive genetic
mutations have been found, although the frequency of other
musculoskeletal deformities such as scoliosis and Marfan
syndrome suggest that abnormal connective tissue plays a
role. Poland syndrome, scoliosis, clubfoot, and syndactyly are
known to be associated with pectus deformities.3,4
Clinical presentation and evaluation
Patients with pectus excavatum exhibit a depressed sternum
that is frequently greater on the right with sternal retraction
on inspiration.5,6 Both the body of the sternum and the costal

889
Thoracic wall defects
function tests, and echocardiogram or EKG (Fig. 40.2). Haller
and others stratified anatomic severity of pectus excavatum
by defining a pectus severity index (PSI) using measurements
obtained from CT scans.7,8 The “Haller Index” is calculated by
dividing the internal transverse thoracic width by the smallest
anterior-posterior distance between the vertebrae and the
most depressed portion of the chest wall. Normal values
range between 2.5 and 3.25 and an index >3.25 is considered
one of the criteria for operative intervention.9
Surgical indications
Most patients are asymptomatic and seek elective interven­
tion for the correction of contour deformities, especially when
the features become more prominent during the pubertal
growth spurt. Although cardiopulmonary compromise is
controversial, anatomically severe versions of pectus excava­
tum are thought to benefit from surgical intervention.10,11
Kelly suggests that surgery is indicated when two or more
of the following occur: a severe, symptomatic deformity;
progression of deformity; paradoxic respiratory chest wall
motion; CT scan with a PSI >3.25; cardiac or pulmonary
compression or pathology; significant body image distur­
bance; or failed repairs.12 Historically, many pectus excavatum
repairs were performed in younger children, and the optimal
timing has been a subject of controversy. The largest single
institution series of patients had median age for operative
intervention of 14, and most surgeons prefer to operate on
children 12–18 years of age, during their pubertal growth
spurt.13
Treatment
Some patients with very mild deformities can be satisfactorily
treated with physical therapy to improve their posture and
increase the bulk of their pectoralis muscles. This approach
does not correct the deformity, but rather it diminishes its
cartilages at the sternocostal junction have posterior angula­
tion (Fig. 40.1). Rounded sloping shoulders, mild dorsal
kyphosis, and a protuberant abdomen are associated findings
but may be related to postural changes that patients exhibit
secondary to a conscious or subconscious attempt to hide
their deformity.
Patients will often complain of subjective dyspnea or chest
pain, and may have significant body image concerns. Proper
identification of the extent of structural irregularity, physio­
logic limitations, and psychological responses to the deformity
are crucial in treatment decision-making. Basic work-up
should include chest CT and/or radiographs, pulmonary
A
Fig. 40.1  Pectus excavatum is the most common congenital anterior thoracic wall
defect and is characterized by a depression deformity in the sternum and costal
cartilage displacement. A male predominance exists. (A) Anterior view of patient
with pectus excavatum. (B) Oblique view of patient. Note the decrease in anterior
posterior distance between the sternum and the vertebral column.
B
Fig. 40.2  CT scan of pectus excavatum deformities illustrate both the severity of
sternal depression as well as the morphology of the depression and its effects on
the involved organs. The pectus severity index (PSI) utilized CT scans to stratify
deformities by dividing the internal thoracic width by the smallest anterior-posterior
distance. Operative intervention is usually performed if PSI >3.25.

890
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
Fig. 40.3  Customized silicone implants are a common
method of camouflaging contour deformities in pectus
excavatum. (A) Preoperative (upper panels) and
postoperative (lower panels) photographs following
implant insertion. A transverse epigastric incision was
used for insertion. (B) Custom silicone implant.
A
B
prominence by improving posture and emphasizing natural
muscle development in other areas.
Surgical correction of pectus excavatum can be categorized
into procedures that either camouflage or repair the defect.
Camouflage of contour deformities has been successfully
accomplished with autologous tissue or custom prosthetics.
The disadvantage of autologous tissue is the donor site scar
and potential morbidity. Flaps containing muscle should not
be relied on for volume correction only unless innervated and
able to contract, as the muscle will invariably atrophy if not
innervated and contracting. The most common contour
camouflage technique is placement of customized silicone
implants (Fig. 40.3).14–17 Satisfactory results are obtained with
silicone implants with few short-term complications other
than seroma formation. Additional problems with prosthetics
are displacement, visualization of edges and potential extru­
sion, especially in thin patients. Autologous reconstruction
has been used as an alternative to prosthetics in selected
patients.17–21 Recently, Sinna et al. described a combination
of approaches covering a custom implant with bilateral
de-epithelialized thoraco-dorsal artery perforator flaps.22
Injection of autologous fat or prosthetic soft tissue fillers has
also been described.23,24
Whereas contour camouflage techniques are most appro­
priate for patients with mild deformities, patients with
moderate to severe deformities are better suited for contour
repair techniques. Ravitch described an open repair technique
of elevating bilateral pectoralis muscle flaps with resection of
abnormal costal cartilages followed by transverse osteotomy
of the sternum and fixation in a corrected position (Fig. 40.4).4
The Ravitch procedure has since been modified in many
ways: from preserving the perichondrium to minimal
cartilage resection and varying technique to support the
sternum.25,26 Haller and colleagues describe supporting the
elevated sternum by overlapping the sternal ends of the costal
cartilages over the costal ends in a tripod fixation.27,28 Almost
all open techniques utilize a transverse inframammary crease
or a short Chevron incision except for older patients or Marfan
patients where a vertical midline incision is usually used.
Bilateral pectoralis major and upper rectus abdominus
muscles are disinserted from the sternum and ribs bilaterally
and raised to expose the costal cartilages. With or without
sacrifice of the perichondrium, the costal cartilages are resected
to a greater or lesser degree. Since Ravitch’s initial experience,
it has become clear that the most medial and most lateral
portions of the costal cartilages should be preserved in young
children to minimize secondary growth disturbances. The
sternal angulation is corrected with a transverse, wedge
osteotomy that is done at the level of the third or fourth
intercostal space corresponding to the upper edge of the

891
Thoracic wall defects
Recently, bioabsorbable mesh placed for retrosternal support
in the Robicsek technique has been reported and found to be
associated with decreased inflammatory reaction, decreased
postoperative pain, and elimination of the risk of retrosternal
metal support device dislodgment.35
A radical method to repair contour deformities of the
sternum uses a sternal turnover bone graft.36 This technique
has been met with problems of sternal avascular necrosis due
to the interruption of blood supply. Vascularized sternal
turnover bone flaps with microvascular anastomoses of the
internal mammary vessels were later developed to prevent
such complications and have been successful in several
reports.37–39 Despite improvements in technique, this approach
has never been widely embraced.
With the development and advances in thoracoscopy,
minimally invasive repair of pectus excavatum has emerged
as a viable option.40–42 This approach is referred to as the
“Nuss procedure” after the surgeon Dr. Donald Nuss, who
sternal depression. The osteotomy can then be secured with
sutures, wire, or rigid fixation plates.29,30 The xiphoid process
is detached from the sternum and allowed to retract down.
Horizontal sternal fixation techniques are not universally
used but many surgeons now consider them essential for a
durable repair. Most surgeons support the sternum with a
metallic strut developed by Adkins and Blades.31 Fonkalsrud
and colleagues report on a modified open technique with
minimal cartilage resection and use of the Adkins strut in 450
patients. Most of these patients had retrosternal placement
but more recently they have converted to a suprasternal strut
to facilitate removal (at 6 months) and minimize the need to
enter the pleural cavity.32 Hayashi and Maruyama describe the
use of a vascularized rib strut based on the anterior intercostal
branch of the internal mammary artery instead of a metallic
strut.33 Robicsek et al. have extensive experience reporting on
over 600 patients treated with a retrosternal Marlex mesh
“hammock” technique with excellent long-term results.34
Fig. 40.4  In 1949, Ravitch described his classic approach to pectus excavatum repair. (A) Starting with a midline incision, the pectoralis muscles are incised and elevated
to expose the sternum and the sternocostal junction. (B) The lowest two costal cartilages are resected with perichondrium and the xiphoid is osteotomized to begin
mobilization of the sternum. (C) Five costal cartilages are divided bilaterally and a single cortex osteotomy is made superiorly. (D) The inferior sternum is reapproximated at
a corrected position and secured with braided silk mattress sutures. (E) The pectoralis muscles are reapproximated midline and the skin incision is closed.
A
B
C
D
E

892
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
donut-shaped attachment, which the patient wears for several
hours daily, and uses negative pressure to raise the sternum.
Initial results have been encouraging, but there is limited data
so far.45 Harrison et al. have developed the magnetic mini-
mover procedure (3MP), in which implantable magnets are
surgically attached to the sternum and the patient wears an
external brace which attracts the magnet and pulls the chest
wall into a more normal position. The initial FDA sponsored
clinical trial enrolled 10 patients and had moderate success.
Half of the patients required a second procedure to reposition
the magnet on the sternum and 30% of the patients dropped
out of the study and opted to undergo the Nuss procedure.
In patients who did complete the full treatment with the
3MP device, the costs were nearly half the cost of the Nuss
procedure.46
Pectus carinatum
Pectus carinatum, also known as “pigeon chest”, is less
common than pectus excavatum, but is considered to be in
the same spectrum of deformities. It is characterized as a
protrusion deformity of the anterior thoracic wall (Fig. 40.6).
Similar to excavatum deformities, pectus carinatum does not
have a defined etiology. The incidence is thought to be about
1 in 10 000, makes up ~5–7% of chest wall deformities, and is
six times more common in males.
Clinical presentation and surgical indications
Three types of anterior thoracic protrusion deformities have
been described in the pectus carinatum spectrum.47 The chon­
drogladiolar type is the most common version and is charac­
terized by anterior displacement of the body of the sternum
first developed it. This approach involves thoracoscopic
exposure of the sternum and the placement of a custom bent
steel or titanium bar (Fig. 40.5). A second procedure to remove
the bar is required. Unlike the Ravitch and modified Ravitch
procedures, skeletal resection is unnecessary with the Nuss
procedure, and this less invasive approach results in less scar­
ring and shorter operative times. However, the operation has
less satisfactory results in patients with asymmetric deformi­
ties and in these patients, the Ravitch procedure is often a
better choice.
Early complications of pectus excavatum repair are infre­
quent beyond pneumothorax (67%) or suture site infection
(1%), and pneumothorax is usually self-limiting, with only 4%
requiring chest tube drainage.13 Longer-term complications of
the Nuss procedure, such as bar dislocation (5.7%), over-
correction (3.7%), bar allergy (3%), and bar infection (0.5%)
have been reported.13 Late complications such as recurrent
pectus deformity (1%) are very few and have diminished now
that most surgeons leave the bar in place for 2–3 years. Sec­
ondary thoracic deformity is a serious late complication that
has been observed in patients who undergo correction at an
early age. Thought to be due to the interruption of growth
centers and intrathoracic scar formation, the patients develop
narrowed thoraces and severe pulmonary impairment. Haller
coined this phenomenon as acquired Jeune syndrome.43 In the
past two decades, most surgeons reserve repair for children
who are entering or are already in the pubertal growth phase.
Absorbable transsternal bars have been used in the Nuss
procedure but they are associated with higher breakage rates
and have been mostly abandoned.44
There are some novel corrective devices in development,
including the Vacuum Bell treatment and implanted magnets
with an external magnetic “brace”. The Vacuum Bell is a
A
Fig. 40.5  The Nuss repair, also known as the minimally
invasive repair of pectus excavatum (MIRPE), is a
thoracoscopic method of contour repair using a bent bar. (A) A
Kelly clamp is advanced across the mediastinum underneath
the sternum (left panel). The clamp guides the placement of a
bent bar into the substernal space (middle and right panels).
(B) For the insertion, the bar follows the concave curvature of
the deformed thorax (left panel). Once in place, the bar is
rotated 180 degrees on its axis such that it forces the sternum
anteriorly into a convex formation (right panel).
B
Metal bar

893
Thoracic wall defects
axial skeletal maturation ceases. Patients rarely experience
discomfort during bracing, and most are very satisfied with
their chest wall appearance following bracing and would use
the brace again.51 Significant improvement in self-esteem was
also seen after bracing.52 If bracing fails, operative correction
remains an option.
The Ravitch procedure is the operation of choice for pectus
carinatum defects. Typically, the approach is through a trans­
verse chevron incision that extends across from one inframa­
mmary fold to the other. Classically, the pectoralis and rectus
muscles are elevated to expose the costal cartilages and the
sternum. For the chondrogladiolar deformity, subperichon­
drial resection of the costal cartilages is combined with a
single or double osteotomy to return the sternum to the correct
alignment (Fig. 40.7).47 For the asymmetric deformities, sternal
positioning is corrected after costal cartilage resection with
a wedge osteotomy. Recent refinements in this procedure
include a method in which costal cartilages are resected
through splitting the muscle along its fibers rather than elevat­
ing the entire muscle.49 There are also attempts to develop a
minimally thoracoscopic approach to pectus carinatum. In
this operation, chondrotomies are made thoracoscopically
and a suprasternal bar is placed to compress the carinatum
defect and correct the deformity.53 Only a handful of patients
have undergone this procedure, but it proves to be a useful
complement to the Ravitch in patients with very symmetric
carinatum defects.
Jeune syndrome
Asphyxiating thoracic dystrophy, Jeune syndrome, is the most
common thoracic deformity in the diffuse skeletal disorders.
The others are spondylothoracic dysplasia (Jarcho–Levin
syndrome) and cerebrocostomandibular syndrome, and since
there are no surgical interventions developed for these defects,
they will not be discussed further in this chapter.
Jeune syndrome is a rare familial autosomal recessive
osteochondrodystrophy characterized by a narrow, immobile
thorax and a protuberant abdomen (Fig. 40.8).54 Though the
early descriptions were in neonates who succumbed to respi­
ratory insufficiency, subsequent reports have demonstrated
that Jeune syndrome has a variable expression and can result
in viability.55 That said, 60–70% will die as infants due to
respiratory failure.56 In patients who have less severe ver­
sions of Jeune syndrome, renal and liver disease are common
(17% and 22%, respectively) and can lead to organ failure in
adulthood.
Clinical presentation and surgical indications
Jeune syndrome can be variably expressed.57 The neonate
with severe Jeune syndrome is generally described to have a
narrow, bell-shaped thorax that is narrowed in both the
transverse and sagittal dimensions with mild brachydactyly.
The ribs are short, wide, and barely reach the anterior axillary
line. Histologic examination of the costochondral junction
reveals disordered endochondral ossification. These patients
have severe restrictive lung disease and frequently require
mechanical ventilation and a tracheostomy. In contrast,
patients who have a moderate expression of Jeune syndrome
tend to have a narrow thorax without respiratory compromise,
severe brachydactyly, and renal failure at a later age. These
with costal cartilage concavity. Asymmetric mixed deformi­
ties can also occur with displacement of the costal cartilages
on one side with normal positioning of the sternum. Promi­
nence of the chondromanubrial junction with a depressed
sternum represents the third and least common type. Unlike
pectus excavatum, younger patients do not have cardiopul­
monary compromise and tend to display subtle findings on
clinical exam. Patients tend to be diagnosed at a later age and
seek surgical correction for their contour irregularities. Pectus
carinatum can also be an acquired defect secondary to pectus
excavatum repair.48 In contrast with pectus excavatum
patients, children with carinatum deformities are less likely
to have associated symptoms and the most common symptom
is chest pain at the costochondral junction. External bracing
is usually effective in these patients, and the main indication
for surgery is an extreme deformity or the failure to improve
cosmetic appearance via bracing.
Treatment
Non-surgical means of correction using orthotics have been
described in small case series with improvement of carinatum
deformities.49,50 Successful treatment with external bracing is
highly dependent upon the patient remaining compliant with
the bracing protocol. Success rates as high as 80% have
been achieved using the “Calgary Protocol” for bracing. This
requires that the patient be custom-fitted with a lightweight,
patient-controlled chest brace that they must initially wear for
23 h per day. Once the convex deformity was corrected,
bracing is reduced to 8 h per day and can be accomplished
nocturnally. This “maintenance phase” is continued until
A
Fig. 40.6  Pectus carinatum is an anterior protrusion thoracic deformity that is the
reverse of pectus excavatum but is generally considered to be in the same
spectrum of deformities. Shown here are (A) anterior and (B) oblique photographs
of a 28-year-old woman with pectus carinatum.
B

894
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
usually requires addition of bone graft, stainless steel struts,
or prosthetic spacers.58–61 Alternatively, Davis and Shah
described a lateral thoracic expansion technique (Fig. 40.9).
In this procedure, ribs 4–9 are differentially transected,
separated from periosteum, and different ribs are secured
together in an expanded fashion with titanium plates.62–64
This is generally performed as a staged procedure with
each operation separated by several months. The authors
noted good results in patients over 1 year of age and new
bone formation with this method. Waldhausen and col­
leagues report success with a technique in two children
using a vertical expandable prosthetic titanium rib (VEPTR)
thoracoplasty for the treatment children with thoracic insuf­
ficiency syndrome. Long-term follow-up is unavailable for
those patients.65
patients are usually identified upon presentation for renal
transplantation or renal replacement therapy. Finally, the
mildest form of Jeune syndrome may be manifested with only
polydactyly and severe brachydactyly. Surgical enlargement
of the thoracic cavity may be attempted in those patients with
ongoing respiratory compromise, but it usually is unsuccess­
ful, resulting in prolonged hospitalization and death from
respiratory compromise.
Treatment
Operative intervention in Jeune syndrome is focused on
expansion of the thoracic cavity. Two methods have emerged
as successful options: median sternotomy and lateral thoracic
expansion thoracoplasty. The median sternotomy technique
Fig. 40.7  Pectus carinatum repair. For the modified Ravitch
approach for pectus carinatum repair, a chevron incision is made
inferior to the nipples and the pectoralis and rectus muscles are
reflected to expose the sternum and costal cartilages. (A) A
section of costal cartilage is resected with perichondrium.
(B) Following costal cartilage resection, the anterior table of the
sternum is osteotomized to reposition the sternum. Shown here
are lateral diagrams of the osteotomy. (C) An Adkins strut with a
normal anterior thoracic contour is then wired to ribs laterally.
The repositioned sternum and medial costal cartilages are
sutured to the strut.
A
Costal cartilage
resected
B
Osteotomy
2
3
4
5
6
C
Adkins strut
Sutures holding rib or
sternum and rib to strut

895
Thoracic wall defects
classification system that is widely accepted today is based
upon tissue coverage of the heart and includes: cervical
ectopia cordis, thoracic ectopia cordis, thoraco-abdominal
ectopia cordis, and cleft or bifid sternum.
Clinical presentation and surgical indications
Cervical ectopia cordis is the most severe variant with supe­
rior displacement of the heart and craniofacial deformities. It
is distinguished from thoracic ectopia cordis by the extent of
superior displacement of the heart. To date, there are no
published cases of successful repair of cervical ectopia cordis
defects.
Thoracic ectopia cordis describes the classic extrathoracic
heart without soft tissue or bony covering. There are also
frequently associated congenital heart defects. There are only
a handful of survivors among children born with this defect.
Thoraco-abdominal ectopia cordis is a combination of both
thoracic wall and abdominal wall defects (Fig. 40.11). These
defects are associated with Cantrell’s pentalogy, which con­
sists of a midline abdominal wall defect, low sternal defect,
anterior diaphragmatic defect, pericardial defect, and intra­
cardiac anomalies.66
Finally, bifid sternum is a relatively benign finding with a
cleft upper sternum that rarely causes significant physiologic
disturbance (Fig. 40.12). This is the least severe of the sternal
defects, and associated congenital heart defects are rare.
Surgical correction is necessary in thoracic, and thoraco-
abdominal defects, with the initial goal of coverage of the
heart followed by ultimately returning the heart to the thoracic
cavity when patients have developed sufficient reserve. Suc­
cessful repair is much more likely in thoraco-abdominal
ectopia cordis compared with true thoracic ectopia cordis. The
absence of concomitant congenital heart disease is another
predictor of successful repair. In patients with cleft/bifid
sternum, the goal of surgery is to provide a protective bony
coverage for the heart and to possibly improve respiratory
mechanics. Coordinated multidisciplinary care involving
obstetricians, neonatologists, critical care anesthesiologists,
cardiothoracic surgeons, and plastic surgeons is critical to
reduce mortality in these high-risk neonates.
Ectopia cordis
Ectopia cordis represents a spectrum of four rare congenital
sternal deformities uniformly characterized by a midline
sternal defect. The incidence of ectopia cordis is 0.8 per 100 000
live births and can vary from asymptomatic benign clefts
to severe conditions with high mortality rates. Many cases
can be diagnosed by prenatal ultrasound (Fig. 40.10). The
Fig. 40.8  Radiograph of a 3-month-old infant with Jeune syndrome, asphyxiating
thoracic dystrophy. Note the horizontal orientation of the ribs, the characteristic
“bell-shaped” thorax, shorted lower limbs, and pelvic abnormalities.
Fig. 40.9  Jeune syndrome repair. The lateral thoracic
expansion technique as described by Davis involves
transection of ribs 4–9 and rigid fixation of alternate ribs. The
thoracic cavity is expanded by increasing the amount of
space between ribs.
4
5
6
7
8
9
4
5
6
7
8
9

896
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
years of age, the patient receives chest wall reconstruction and
repositioning of the heart. Chest wall reconstruction can take
the form of musculocutaneous flaps over autologous rib grafts
as well as alloplastic custom made struts.68,69 Hochberg and
colleagues68 described a method of elevating the pectoral and
rectus muscles as a unit and transposing bipedicled musculo­
cutaneous flaps medially with relaxing incisions laterally. The
resulting donor sites were skin grafted on the lateral aspects
of the patient.
Sternal clefts without an exposed heart are significantly less
complicated. Surgical repair can often be performed within
the first month of life. Bilateral pectoralis major muscle
advancement flaps over rib grafts have yielded good cosmetic
results.70 Rigid fixation with titanium plates has also been
reported.71
Poland syndrome
Poland syndrome is a rare disorder characterized by the
unilateral absence of the sternal head of the pectoralis major
muscle, breast hypoplasia or aplasia, absence or deformed
ribs, axillary alopecia, and ipsilateral upper extremity short­
ening and brachysyndactyly.72,73 Poland syndrome has been
reported to have an incidence of around 1–30 000 live births,
with males outnumbering females.74 The etiology is unknown.
One suggested mechanism for Poland syndrome is subclavian
artery insufficiency occurring around the 6th week of gesta­
tion, with the right side affected twice as often as the left.
Alternatively, unilateral developmental failure of the lateral
plate mesoderm has also been proposed.
Clinical presentation and surgical indications
Pediatric patients with Poland syndrome are heterogeneous.
The pathognomonic feature is the absence of the sternal
head of the pectoralis major muscle with anterior axillary
fold deficiency (Fig. 40.13). Varying degrees of chest wall
and hand involvement may occur (Fig. 40.14). In rare severe
Treatment
Thoracic and thoraco-abdominal ectopia cordis repair is most
successful when performed in a staged manner. The key
principles for successful operative management are the con­
struction of tissue coverage surrounding the heart as a partial
anterior chest cavity, and also to defer attempts to move the
heart to an orthotopic location. The first step is performed
within the first several hours of life and involves coverage of
the exposed heart with bilateral pectoral skin flaps, split thick­
ness skin grafts (STSG), or synthetic or biologic mesh. There
should be no attempt in returning the heart to the thoracic
cavity at this point, due to the cardiopulmonary compromise
that will occur with compression.67 At several months to 2
Fig. 40.10  Prenatal ultrasound diagnosis of ectopia cordis. The prenatal ultrasound
of a 32-week-old fetus demonstrating an extrathoracic heart (arrow).
Fig. 40.11  Thoraco-abdominal ectopia cordis. Same patient as in Fig. 40.10 with
ectopia cordis, midline supraumbilical defect and low sternal defect.
Fig. 40.12  Bifid sternum is a benign condition in the spectrum of ectopia cordis
defects. Similar to more severe forms, a failure of sternum fusion is evident.
However, the thoracic contents are positioned anatomically and there are no
associated physiologic abnormalities.

897
Thoracic wall defects
The latissimus dorsi muscle may be attenuated. Diagnosis of
Poland syndrome is possible by prenatal ultrasound but is
usually best evaluated with postnatal physical exam.
Basic work-up for Poland syndrome begins with the post­
natal physical exam. The trunk and upper extremities need to
be examined in its entirety with comparison to the contra­
lateral side. The presence of the pectoralis major, serratus
circumstances, depression deformities can occur with attenu­
ation of ribs 2–5 with absence of anterior cartilage leading to
paradoxical motion of the chest wall or lung herniation. Some
patients have a pectus carinatum deformity on the contra­
lateral side. Poland syndrome does not preclude correction
of the pectus defect. In one-third of patients, the breast is
affected and can range from hypoplasia to complete absence.
Fig. 40.13  Poland syndrome is characterized by a
unilateral absence of the sternal head of the pectoralis
major muscle, breast hypoplasia or aplasia, absence or
deformed ribs, axillary alopecia, and ipsilateral upper
extremity shortening and brachysyndactyly.
Pectoralis major muscle,
clavicular part
Pectoralis major muscle,
sternocostal part
Pectoralis minor muscle
Latissimus dorsi
muscle (hypoplastic)
Aplasia of ribs III–V
Missing phalanx in
fingers 1, 3, 4
Ipsilateral “mitten hand”
Fig. 40.14  Poland syndrome is a rare disease with a male predominance. A young boy with (A) right-sided Poland syndrome. (B,C) Note the presence of a hypoplastic
nipple and the absence of the sternal head of the pectoralis major.
A
B
C

898
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
evidence that pedicled latissimus dorsi flaps with or without
silicone implants can be accomplished with excellent results
improving chest wall contour in a staged fashion. Some
groups have modified this method with the use of minimally
invasive endoscopy to elevate the latissimus.77,78 Free tissue
transfer such as the transverse rectus abdominis musculocu­
taneous, deep inferior epigastric perforator, superior gluteal
artery perforator (SGAP), and anterolateral thigh perforator
flaps have been used for breast reconstruction and soft tissue
camouflage/fill of contour defects.79,80 Contralateral latissimus
dorsi microneurovascular transfer has been described for
functional replacement of the missing pectoralis muscle, as
well as reconstructing a more normal appearance to the
anterior axillary fold when the ipsilateral latissimus dorsi
muscle is attenuated or otherwise unusable.81
In more severe presentations of Poland syndrome, bony
support for chest wall reconstruction have traditionally been
accomplished with autologous split rib grafts (Fig. 40.15) or
Marlex mesh (Fig. 40.16).82,83 Both techniques have met success;
however, Marlex mesh placement tends to result in a flattened
appearance and, therefore, tends to be performed in conjunc­
tion with latissimus dorsi flaps. Alternatively, mesh prostheses
with rib graft, molded silicone prostheses, as well as cryopre­
served costal cartilage have been described with varying
success.16,84,85 Symmetry of the carinate deformity on the
contralateral side may subsequently be corrected with a
sternal osteotomy and rotation.
Abdominal wall defects
Omphalocele and gastroschisis
Congenital defects of the abdominal wall comprise a large
spectrum of deformities, ranging from umbilical hernias to
giant omphaloceles and the intestinal eventration associated
with gastroschisis. These conditions can vary widely in their
etiology, associated morbidity, and management.
Omphalocele defects exist along a spectrum ranging from
giant omphaloceles to umbilical hernias of the cord. It is
important to differentiate between a giant umbilical hernia
and an omphalocele. The umbilical hernia is a central abdomi­
nal wall defect involving the linea alba, with herniation of
intra-abdominal contents into the umbilical stalk. The skin
overlying the hernia is intact. In contrast, an omphalocele is
characterized by a midline defect of the ventral abdominal
wall, resulting in extrusion of intra-abdominal contents,
which are covered by a membrane comprised of peritoneum,
Wharton’s jelly, and amnion. The umbilical cord arises from
the membranous sac, and the defect may be located in the
central, upper, or lower abdomen. The defect in omphaloceles
is due to a failure of the body folds to complete their journey.
Purely lateral fold defects will be isolated at the umbilicus,
but cephalic fold defects result in associated defects above the
omphalocele such as ectopia cordis or the pentalogy of
Cantrell. In contrast, caudal fold defects cause defects below
the omphalocele such as bladder and cloacal exstrophy.
Depending on the size and location of the defect, the hernia
may contain bowel, liver, and other organs. Omphaloceles
may be termed “minor” or “giant” with the definition of a
giant omphalocele typical defined as a 5 cm defect or the
presence of the liver within the defect.86
anterior, and latissimus dorsi should be confirmed by palpa­
tion. A standard chest radiograph is necessary to visualize the
ribs. Computerized tomography or MRI with contrast may be
helpful for surgical planning. The primary surgical indication
for repair at a young age is rib cage aplasia75 resulting in
cardiopulmonary compromise. At later ages, surgery is
usually performed for concerns of contour abnormalities in
both males and females.
Treatment
A heterogeneous spectrum of reconstructive options exists for
correction of Poland syndrome. Mild forms of Poland syn­
drome generally have a strictly soft-tissue deficiency and may
not require any intervention. Similar to adult breast recon­
struction, both silicone prostheses as well as autologous tissue
reconstruction have been described. Custom prosthetic
implants for chest wall deformities have a high incidence of
displacement, erosion, discomfort, and edge visibility, espe­
cially in thin individuals.76 Multiple groups have provided
A
Division of cartilage
of 3rd rib
Division of cartilage
of 6th rib
Wedges excised
Fig. 40.15  (A,B) Split rib graft repair for Poland syndrome. Skeletal support in
Poland syndrome can be achieved by excising the existing superior and inferior
costal cartilages, splitting the cartilages, and re-fixation to the abnormal ribs.
B
Upper rib segment
sutured to aplastic 4th rib
Lower rib segment
sutured to aplastic 5th rib

899
Abdominal wall defects
ectopia cordis, and OEIS (omphalocele, exstrophy of bladder,
imperforate anus, and spinal defect) sequence (Fig. 40.17).89
Cardiac anomalies are common in patients with omphalocele,
occurring in up to 45%. Anomalies range from ventricular
septal defects, atrial septal defects, ectopia cordis, tricuspid
atresia, coarctation of the aorta, and persistent pulmonary
hypertension of the newborn.90 In patients with giant ompha­
locele, survival is often predicated on the extant of pulmonary
hypoplasia and degree of pulmonary hypertension. In con­
trast to omphaloceles, patients with gastroschisis are less
likely to have other associated defects and approximately 14%
of infants with gastroschisis will have associated unrelated
genetic defects. The most common associated anomaly is of
an intestinal atresia.91
Gastroschisis is broadly classified as simple or complex
based on the presence of additional bowel anomalies such as
intestinal atresias, stenosis, volvulus, or perforation. Intestinal
atresias are the most frequent associated anomaly and the full
range of intestinal atresias can be found. The size of the defect
varies and one occasionally finds a very narrow defect and
only a small amount of midgut external to the abdomen.
Closer examination often reveals a paucity of bowel, which
most likely is due to antenatal volvulus. This is known as a
“vanishing gastroschisis” as the defect is so small that it is
essentially closed in utero.92
Treatment
Reconstructive methods for omphalocele and gastroschisis
are directly related to the size of the ventral abdominal wall
defect and the magnitude of the intra-abdominal “loss of
domain” (LOD). Small defects can be closed primarily or with
minimal undermining of surrounding soft tissues. Giant
omphaloceles usually cannot be closed primarily due to the
size of the defect and the severe physiologic consequences of
returning the liver and bowel to the abdomen in the setting
Gastroschisis is characterized by a full-thickness defect
of the abdominal wall, usually to the right of midline and
not involving the umbilicus. This results in evisceration
of abdominal contents without any sac or covering. Small
intestine is always present, while stomach, colon, and gonads
may also be involved. In contrast to omphalocele, it is rare
for the liver to herniate outside the abdominal cavity. The
exposed organs typically appear normal at birth, but they
quickly develop a rind consisting of a fibrinous exudate. The
rind is thought to be due to a combination of chronic exposure
to amniotic fluid and the air. This inflamed bowel frequently
demonstrates dysmotility and malabsorption. The presence of
the bowel external to the abdomen precludes normal intestinal
fixation in utero, and therefore, all children with gastroschisis
have an anomaly of intestinal rotation or “malrotation”.
They do not typically require a Ladd procedure to correct the
anomaly of intestinal rotation, but it is important to remember
that their intestines are not normally rotated.
Omphalocele and gastroschisis occur at 1–2.5 in 5000 live
births and 2–4.9 in 10 000 live births, respectively.87,88 Ompha­
loceles have a tendency to be larger in size than gastroschisis
and involve the herniation of bowel, liver, and other organs
into the umbilical cord with the amniotic sac covering. Males
and females are equally affected. The genetic causes of
omphalocele and gastroschisis are unknown but the two are
thought to be different entities.
Clinical presentation and surgical indications
The abdominal wall defect in omphalocele typically ranges
from 4 to 7 cm in diameter, whereas gastroschisis defects tend
to be slightly smaller. Infants with omphalocele frequently
have concurrent genetic disorders such as chromosomal
abnormalities (e.g. Trisomy 18), Beckwith–Wiedemann syn­
drome (congenital abdominal wall defect, macroglossia, and
hypoglycemia with increased abdominal tumors later in life),
Fig. 40.16  (A–C) Mesh usage in Poland syndrome repair. In addition to autologous split rib grafts, Marlex mesh placed above the rib grafts serve to correct the skeletal
contour deformity. However, the appearance of the thorax after either rib grafts alone or Marlex mesh over rib grafts is flattened. The contour deformity also requires addition
of soft tissue or prostheses over the bony reconstruction.
A
B
C
Harvested rib
split for grafts
Sternal notches
4th rib
harvested
for grafts
Rib
grafts
Transverse
sternotomy
Prosthetic
mesh cover
Figure-8 suture

900
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
Fig. 40.17  (A) A 3D reconstruction of a CT scan of a neonate with OEIS (omphalocele, exstrophy of bladder, imperforate anus, and spinal defect). Note the wide pelvic
separation of pubic symphysis and spinal defects. (B) Preoperative view of same neonate with OEIS demonstrating extensive abdominal wall defect. (C) Postoperative
photograph of patient a few weeks after multi-staged omphalocele repair, genitourinary reconstruction, abdominal closure, and orthopedic correction of pelvis.
A
B
C
of decreased abdominal domain. Exacerbation of pulmonary
disease and decreased venous return are typical problems
encountered.
Small omphaloceles may be closed primarily in the first
few days of life. An omphalocele of the cord may benefit
from suspension of the defect above the abdomen for
24–48 h to allow gravity to assist with reduction of the
omphalocele contents back into the abdomen (Fig. 40.18).
Whether early or delayed closure is attempted, it is important
to monitor the patient’s physiology as the contents of the
omphalocele are returned to the abdominal cavity. Methods
of assessing physiologic response to closure are further
discussed below.
Giant omphaloceles tend to present unique challenges. A
common approach is to apply a sclerosing agent such as 0.25%
Mercurochrome or 0.5% silver nitrate to the omphalocele sac
until complete epithelialization occurs, and to delay definitive
operative management until any problems with pulmonary
insufficiency have been stabilized. Silver sulfadiazine (Silvad­
ene) has emerged as an excellent alternative with less potential
toxicity (Fig. 40.19).93 Dressings are performed twice daily
until epithelialization is complete, and may potentially be
Fig. 40.18  (A) Omphalocele of the cord with very narrow defect at the umbilicus. (B) Small bowel reduced from omphalocele via an incision below the umbilical ring.
This child required a Ladd procedure to remove constricting Ladd bands and broaden the narrow mesentery. (C) After closure of the umbilical defect and the incision below
the umbilicus.
A
B
C

901
Abdominal wall defects
implantation included a Teflon sheet attached to the perito­
neum and a Marlex sheet to the anterior rectus sheath as a
secondary procedure.107 Bioprosthetic materials such as peri­
cardial patches108 and Alloderm have also been described in
case reports.109 The authors’ practice has used either Strattice
bioprosthetic mesh or a silicone mesh to allow a staged
closure. Secondary procedures after abdominal closure are
frequently required. In omphalocele patients, umbilicoplasty
is performed either in an immediate or delayed fashion.110,111
Bladder and cloacal exstrophy is often associated with
omphalocele. The reconstruction of the urogenital system is
addressed in Chapter 43.
In gastroschisis, the lack of amniotic sac covering results in
the exposure of a large surface area to the external environ­
ment immediately following birth. This can lead to rapid
insensible fluid losses and hypothermia, and adequate fluid
resuscitation along with early physiologic coverage is crucial
to survival of the neonate. Complications from the defect or
subsequent closure include pneumonia, bowel obstruction,
ileus, sepsis, and necrotizing enterocolitis.
The bowel should be examined for other pathology includ­
ing atresia, necrosis, or perforation, and the baby examined
for other associated anomalies. Tight defects of the abdominal
wall may require urgent enlargement, while volvulus may
necessitate urgent reduction. In the absence of these findings,
the decision must be made whether to attempt immediate
reduction and repair, or to perform staged (silo) closure.112
Primary repair is preferred whenever feasible; however, a
number of key variables may potentially prevent this, includ­
ing: the size of the defect; the degree of abdominovisceral
disproportion; the condition of the bowel; extreme prematu­
rity; severe pulmonary disease; and associated conditions or
anomalies.
The risk of primary reduction and closure is related to the
inevitable increase in intra-abdominal pressure resulting from
returning the viscera to the abdomen, which in turn may lead
to respiratory compromise, renal insufficiency, and abdominal
compartment syndrome.113 As the degree of abdominovisceral
disproportion increases, these risks also rise accordingly.
Similarly, in extremely premature infants or infants with
severe pulmonary disease, the pulmonary status may be quite
tenuous and pulmonary function may be compromised more
easily and/or more severely. In these cases, it is often more
prudent to undertake staged closure. Several studies have
compared the outcomes of primary closure versus silo closure
for gastroschisis, looking at overall complication rates, time
on the ventilator, time to full feeds, and length of hospital
stay. While the results of these studies can be difficult to
interpret due to inherent selection bias, it has been shown
that with judicious patient selection, the outcomes can be
equivalent.114,115
Whether closing a giant omphalocele or a large gastroschi­
sis defect, there are risks associated with the acute change in
intra-abdominal pressures during closure. There are a few of
ways to measure and monitor these changes. A urinary cath­
eter should be placed to periodically measure bladder pres­
sures. Bladder pressure and peak airway pressures are proxies
for determining intra-abdominal pressure, and significant
elevation of this value following reduction of the viscera may
portend the severe complications listed above.
Bladder pressures >10 mmHg are considered elevated in
children,116 and some authors advocate a strict cut-off value
completed as an outpatient in selected patients.94 Alternatively,
skin coverage may be expedited through the use of skin grafts
directly on the omphalocele sac, avoiding the need for a
prolonged period of dressing changes.
Once the new epithelial covering completely envelops the
omphalocele, gentle external pressure with an ACE wrap can
often gradually compress the abdominal contents back into
the abdomen, and definitive closure of the defect can often be
achieved in one stage. In complicated cases, there are many
creative approaches that have been employed: skin grafting;95
stretching of the abdominal wall via intentional pneumoperi­
toneum;96 tissue expanders to stretch the abdominal wall in
preparation for closure;97–99 partial hepatectomy;100 lateral
relaxing incisions in the fascia;101 and division of the rectus
abdominis muscles.102
Surgical closure techniques are frequently staged or
delayed. In 1948, the first description of an omphalocele
closure with staged skin flaps was reported.103 Although the
closure was successful, the disadvantage of using skin alone
was the resultant large ventral hernia. A more definitive
closure would necessitate the reapproximation of fascia or the
interposition of a synthetic or alloplastic material.
Intraperitoneal tissue expansion is an alternative method
of abdominal wall expansion to treat LOD. In two reports,
intraperitoneal tissue expansion was performed with an
intrapelvic expander over the course of 3–5 weeks.104,105 Both
groups reported successful closure with low complication
rates. Finally, the component separation technique has also
been described in giant omphalocele closure. In 10 children
with giant omphaloceles and a median age of 6.5 months,
component separation was performed by incising the external
oblique aponeurosis.106 Unlike the previous techniques,
closure was performed after a period of non-operative therapy
until complete re-epithelialization of the omphalocele. Com­
plications in three patients included central line sepsis, midline
skin necrosis, and hematoma.
Fascial substitution can be accomplished using prosthetic
or bioprosthetic materials. The early reports of prosthetic
Fig. 40.19  Giant omphalocele treated with topical silver sulfadiazine totally
re-epithelialized in approximately 6 months.

902
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
Evaluation of the neonate should begin with consultation
by the neurosurgeon and the reconstructive surgeon for cov­
erage of cord elements. Frequently, neural tube defects are
accompanied by other defects such as limb deformities and
neuropathic bladder requiring consultations to orthopedics
and urology, respectively.
Surgical indications
Prior to the 1960s, meningomyelocele patients bore a mortal­
ity rate of approximately 65–75% within the first 6 months of
life with rare survival to 6 years of age.122,123 Since that time,
early closure has been recognized to be essential to prevention
of infection and is now considered standard of care. Success­
ful closure within 24–48 h of life has resulted in survival to
approximately 85% at 3 months, 60–70% at 1 year, and 40–50%
at 3 years.124 Despite advances, patients continue to suffer
from significant morbidity due to coexisting conditions such
as hydrocephalus and or tethered cord. On the other hand,
meningoceles with adequate skin coverage can be deferred
until 3 months of age. Bony defects in spina bifida are gener­
ally not addressed.
Treatment
As mentioned above, early physiologic closure of exposed
spinal elements is essential. The primary goals are to protect
cord elements, avoid infection, and seal any cerebrospinal
fluid (CSF) leak. Soft tissue closure over the dural repair,
whether with muscle, skin grafts, or skin flaps, is important
to maintain the integrity of the dural repair.
Historically, primary skin closure of meningomyeloceles
accounted for approximately 75% of all repairs.125 The remain­
ing 25% of large meningomyelocele defects required other
means of reconstruction (Table 40.1). Sizable defects up to
20 cm2 have been accomplished with modest skin undermin­
ing. De Brito Henriques, et al. described obtaining primary
closure in 15 of 16 patients with defects up to 64 cm2 using
acute intraoperative tissue expansion with an intermittent
skin traction technique.150 The disadvantage in this approach
was the increase in operative time necessary to perform the
intraoperative slow traction technique.
Variations of random pattern skin flaps and fasciocutane­
ous flaps have been described by a number of investigators
to prevent the skin-edge necrosis common to primary closure.
Adjacent tissue rearrangements including rotation, transposi­
tion, advancement, Z-plasty, and Limberg flaps have all been
described using dermal vascular supply. Cruz and colleagues
described a creative double-Z rhomboid skin flap, in which
margins of skin were excised in the form of a rhombus with
adjacent angles of 60° and 120°. Two sets of 60° equilateral
Z-plasty flaps were drawn on opposite sides of defect by
extending incisions from the 120° angles and transposed into
the defect.127 Despite the success of this report, local tissue
rearrangements relying on random pattern blood supply are
generally not reliable for large defects. The amount of skin
and subcutaneous tissue required for coverage is usually not
well perfused without defined perforator or axial vascular
supply thus resulting in frequent wound breakdown.
Although random pattern skin flaps are usually not reli­
able for large defects, the development of such flaps con­
tributed to the design of geometric pattern fasciocutaneous,
of 20 mmHg in deciding whether to proceed with repair. In
contrast to bladder pressures indicating intra-abdominal
hypertension and acute abdominal compartment syndrome,
there are no absolute values for peak airway pressures, which
may vary significantly between patients, dependent on age
and pre-existing pulmonary comorbidity. As such, relative
changes in peak airway pressures are used as a proxy for
increased intra-abdominal pressure and the ability to extubate
the patient following surgery. Trending the changes in bladder
pressure and airway pressures should be continued into the
immediate postoperative period.117
Near-infrared spectrometry (NIRS) is an additional non-
invasive modality that reliably reflects visceral tissue oxygen­
ation and perfusion in neonates <10 kg. Its reliability and
usefulness in larger babies, however, is controversial.118 Our
preliminary experience with NIRS in the neonatal ICU setting
has been quite promising and we advocate its use in neonates
with abdominal fascial defects where bowel vascular supply
is at risk.
Posterior defects
Embryology
The neuroectoderm begins as a single sheet of cells. At 4
weeks’ gestation, the same time the body wall develops from
the lateral plate mesoderm, neural plates emerge and migrate
towards each other to form the neural tube.119 Fusion occurs
in an anterior to posterior direction and in a cephalad to
caudad manner. Failure of anterior closure results in diaste­
matomyelia and anterior meningocele. Failure of posterior
closure results in the spina bifida spectrum.120
Spina bifida
Spina bifida is classified into three major groups. Spina bifida
aperta is an open myelocele with no covering over the neural
elements. Neurologic loss is present at birth. Spina bifida
cystica encompasses meningoceles, meningomyeloceles, and
syringomyeloceles. Meningoceles account for 14% of neural
tube defects and usually occur in the lumbar spine. Meningo­
celes are defined by the herniation of meninges without cord
elements. Meningomyeloceles occur at the conus medullaris
and are characterized by a herniation of the meninges with
spinal cord through the vertebrae. Meningomyelocele is the
most common neural tube defect, accounting for 85% of all
neural tube defects with a worldwide prevalence of 0.17–6.39
per 1000 live births.121 It is frequently associated with motor
and sensory deficits. Syringomyeloceles refer to a meningocele
with a dilated central canal. Spina bifida occulta is the most
benign neural tube defect with minimal clinical significance.
Patients frequently present with dermal sinuses, a posterior
hair patch, or lipoma but do not usually have neurologic
symptoms.
Clinical presentation and evaluation
Patients with spina bifida cystica are frequently diagnosed
with prenatal ultrasound and upon birth. Exposed cord in
spina bifida cystica have a thin membranous meninges sac
and need to be prevented from desiccation with moist dress­
ings to preserve neurologic function.

903
Posterior defects
Table 40.1  Meningomyelocele repairs
Type
Method
No. patients
(n = 74)
Timing
Defect size
(avg. or range)
Complications
Ref.
Skin flap, muscle flap,
skin graft
Skin flaps, reverse and
advancement
latissimus, STSG
Skin flap (n = 37)
Lat flap (n = 5)
STSG (n = 32)
Acute
Primary:
22.7 cm2
STSG: 37.3 cm2
Primary: 41% flap
necrosis, 13.5%
CSF leak, 10.8%
sepsis, 2.7% death
STSG: 6.3% partial
graft loss, 6.3%
CSF leak, 3.1%
sepsis, no deaths
126
Skin flap
Double-Z rhomboid
10
Acute
4–23 cm2
Partial flap necrosis in
one patient, CSF
leak in one patient
127
Fasciocutaneous
Modified bipedicled V-Y
11
Acute
7–40 cm2
None
128
Fasciocutaneous
Bilateral rotation
advancement
5
Acute
30–80 cm2
None
129
Fasciocutaneous
Bilateral rotation
advancement
9
5 acute, 4
delayed
24–48 cm2
Skin necrosis in one
patient
130
Fasciocutaneous
Triangular unequal
Z-plasty
5
Acute and
delayed
54–102 cm2
Hematoma in one
patient
131
Fasciocutaneous
Bilobed
20
Acute
38.4 cm2
Partial flap loss with
CSF leak in one
patient
132
Fasciocutaneous
Bilobed
5
Acute
12.25–36 cm2
None
133
Fasciocutaneous
Z-advancement rotation,
bilateral
11
10 acute, 1
delayed
45–114 cm2
None
101
Fasciocutaneous
Rhomboid
1
Acute
42 cm2
None
134
Fasciocutaneous
Modified Limberg flap
4
Acute
16–68 cm2
Wound breakdown in
one patient
135
Fasciocutaneous
Bipedicled flap
12
Acute
6–7 cm (width)
None
136
Musculocutaneous
Reverse latissimus
island, unilateral
12
7 acute, 5
salvage
Unknown
Two patients with
minor wound
breakdown at
medial aspect of
donor site
120
Musculocutaneous
Latissimus transposition,
STSG to secondary
defect
2
Delayed
Unknown
None
137
Musculocutaneous
Proximally-based
latissimus island,
bilateral
20
Delayed
6 ± 1.2 cm2
None
138
Musculocutaneous
Unilateral and bilateral
latissimus (bipedicled,
proximal, and reverse
flow)
23
Acute
35–74.16 cm2
Wound dehiscence in
two patients, distal
flap necrosis in two
patients
139
Musculocutaneous
Rhomboid latissimus
transposition
30
Acute
Max: 60 cm2
Unknown
140
Muscle and
musculocutaneous
Latissimus, gluteus with
STSG
8
Acute
97.9 cm2
CSF leak until VP
shunting in all
cases
141
Musculocutaneous
Latissimus, V-Y
advancement
1
Acute
117 cm2
None
142
Continued

904
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
Several other fasciocutaneous flap designs reported
in smaller numbers of studies include the large bilobed
transposition, the rhomboid perforator, and the unequal
Z-plasty.128,132,133,155 Both the bilobed and the rhomboid flaps
are elevated in the classical manner with careful attention to
the incorporation of perforating vessels. Mutaf’s unequal
Z-plasty, however, has unique geometric criteria.131 The
advantage of this flap design is that after transposition, the
skin incisions are not overlying the dural repair but the exci­
sion of skin in the conversion to a triangular defect and the
rather complicated design limits its utility.
The unilateral island SGAP flap was described by Duffy for
smaller defects averaging 4.8 × 6.8 cm2.144 Despite preserva­
tion of the perforator pedicle with a cuff of muscle, flaps in
this were commonly plagued with venous congestion.
Though bony reconstruction is not thought to be required
for meningomyelocele repairs, Mustarde proposed an innova­
tive method of reconstructing a bony spinal canal under
turnover paraspinal muscle flaps (Fig. 40.22).148 Following
closure of the dura, the paraspinal muscles are incised and
transverse processes are osteotomized. The paraspinal muscles
are brought together in the midline and covered with a split
thickness skin graft.
Mustarde’s paraspinous muscle advancement and turnover
flaps have been found to be useful to others in providing a
well-vascularized layer above the closed neural placode.156
Skin closure can then be performed by skin graft, direct
closure, or a variety of flaps. The particular advantage of
paraspinous muscle flap closure is that it protects the dural
repair should there be any overlying skin breakdown.
musculocutaneous, and perforator based-flaps providing a
more reliable tension-free skin closure techniques (Figs. 40.20
& 40.21).
Doppler confirmation of perforators in the base of these
flaps has been helpful in intraoperative planning. Three vas­
cular perforator territories were identified by Iacobucci during
the description of bilateral superior and inferior fasciocutane­
ous transposition flaps.151 Superolaterally, parascapular and
scapular fascial branches of the circumflex scapular artery
represent the dominant blood supply to the superior transpo­
sition flap. The vascular pattern of the middle-third of the area
originates from muscular perforators and lateral cutaneous
branches of the costal groove segment of the lower intercostals
arteries. The inferior flap receives contributions from the
superficial circumflex iliac artery perforators. Permutations of
this flap include the fasciocutaneous Z advancement rotation
(ZAR) flap and a bilateral rotational transposition flap with
curvilinear incisions.130,134 Both of these flaps are robust flaps
based on the preservation of the thoraco-lumbar fascia and
the perforating vessels. The curvilinear advancement rotation
flap has the added advantage of moving skin incisions such
that they do not overlie the dural repair incision line.
One of the most common fasciocutaneous transposition
techniques utilizes V-Y advancement from the flanks. Several
modifications of this technique have been reported such as
placing the apical extensions of the V incisions such that they
are supplied by paraspinous perforators, preserving skin
bridges at the superior and inferior aspects of the bilateral
flaps129,152,153 and the design of crescentic-shaped flaps in our
practice.154
Type
Method
No. patients
(n = 74)
Timing
Defect size
(avg. or range)
Complications
Ref.
Muscle
Reverse latissimus,
unilateral with STSG
over muscle
1
Acute
primary
repair,
salvage
Unknown
None on salvage
143
Musculocutaneous
SGAP
6
Acute
32.64 cm2
Venous congestion
common,
epidermolysis in
one patient
144
Musculocutaneous
Bilateral latissimus with
gluteal fascia, relaxing
incisions
19
Most
acute,
some
delayed
20–50 cm2
None
145
Musculocutaneous
Latissimus and/or
trapezius
82
Acute
Unknown
None
146
Musculocutaneous
Bilateral interconnected
latissimus and gluteus
maximus
9
Acute
42–80 cm2
None
147
Osteomusculocutaneous
Paraspinous muscle/bifid
spine turnover ± STSG
Unknown
Acute
Unknown
Unknown
148
Osteomusculocutaneous
Latissimus, trapezius
6
Acute and
salvage
Unknown
None
149
SGAP, superior gluteal artery perforator; STSG, split thickness skin graft.
Table 40.1  Meningomyelocele repairs—cont’d

905
Posterior defects
A
Fasciocutaneous crescentic VY advancement
B
Fasciocutaneous VY advancement
C
Modified fasciocutaneous VY
E
B’
Y
A
A
B
C
X
b
C’
45˚
60˚
Fasciocutaneous unequal Z-plasty
D
Fasciocutaneous transposition one superior, one inferior
F
Fasciocutaneous Z advancement flap
Fig. 40.20  Fasciocutaneous flaps in meningomyelocele repair. (A) Bilateral crescentic V-Y advancement. (B) Classic bilateral V-Y advancement. (C) Modified bipedicle V-Y
advancement. (D) Superior and inferior rotational transposition. (E) Unequal Z-plasty. (F) Z advancement. (G) Bilobed transposition. (H) Rhomboid transposition.
G
Fasciocutaneous bilobed flap
H
Fasciocutaneous rhomboid

A
Distally based lat island musculocutaneous
B
Bilateral lat VY advancement
C
Paraspinal muscle flap
E
SGAP
D
Muscle only reverse lat transposition
F
Musculocutaneous lat without relaxing incisions
Fig. 40.21  Muscle and musculocutaneous flaps in meningomyelocele repair. (A) Distally-based latissimus (lat) musculocutaneous advancement. (B) Bilateral latissimus
V-Y musculocutaneous advancement. (C) Paraspinous muscle advancement or turnover. (D) Distally-based latissimus (lat) turnover muscle flap with split thickness skin
graft coverage over flap. (E) Superior gluteal artery perforator (SGAP) interposition. (F) Bilateral latissimus (lat) musculocutaneous advancement. (G) Bilateral latissimus
(lat) musculocutaneous advancement with lateral relaxing incisions covered with split thickness skin grafts (STSG). (H) Bilateral latissimus (lat) and gluteus maximus
interconnected musculocutaneous advancement.
G
Musculocutaneous lat with superior gluteal fascia in relaxing
incisions, STSG to the sides
H
Musculocutaneous lat and gluteus advancement

907
Posterior defects
the latissimus with perforator obliteration. The gluteus
maximus is then de-originated from the iliac crest and sacrum
and dissected free from the gluteus medius. The entire unit
comprised of latissimus dorsi connected to gluteus maximus
is then advanced bilaterally without relaxing incisions and
closed in the midline
Skin grafting over dural closures was popularized by Luce
and Walsh.126 The authors retrospectively reviewed their
experience of 74 neonates that included management by
primary closure (n = 37), latissimus muscle flaps (n = 5), and
split thickness skin grafts (n = 32). The population of patients
treated with wide skin flap undermining displayed significant
wound healing complications resulting in higher rates of CSF
leak, sepsis, and deaths. Despite success with latissimus flaps,
the concern for blood loss led these authors to prefer closure
with split-thickness skin grafts (either temporary xenograft or
immediate autologous skin grafts). They noted that conver­
sion to grafting decreased all immediate complications seen
with primary closure and did not cause large volume blood
loss. Skin grafting, therefore, offers a simple way to obtain
rapid coverage of dural repairs; however, it lacks adequate
soft tissue to protect the repair from trauma. In long-term
follow-up, lumbosacral skin grafts did not have an increase
in skin ulceration, but thoraco-lumbar and thoracic patients
covered with split thickness skin grafts not only had an
increased incidence of skin ulceration but also had an increased
incidence of developing a gibbus deformity when compared
with primary skin closure techniques.159 The etiology of the
gibbus deformity and the association with skin graft recon­
struction of thoraco-lumbar and thoracic meningomyeloceles
are not clear. Considering the results of this long-term
follow-up study, skin grafts should only be used in the
thoraco-lumbar and thoracic regions as a temporizing measure
for immediate neonatal closure. Mustoe et al. reported the use
Muscle and musculocutaneous flaps frequently involve
usage of the latissimus dorsi muscle. Unilateral proximally-
based latissimus flaps are most useful for higher lesions,
whereas reverse and island flaps can reach low lumbosacral
defects.120,138,143,156 However, many of these defects require
bilateral muscle flaps,157 as recognized first by Desprez and
colleagues.149 The authors documented six children, ranging
from neonates to 2 years of age, with large meningomyeloceles
that were closed with bilateral bipedicled advancement of the
latissimus dorsi and trapezius muscles through lateral skin
incisions. The muscles were transected laterally and advanced
medially. Bifid spinous processes were osteotomized similar
to Mustarde’s technique of reconstructing the spinal canal.
The lateral incisions were closed with V-Y advancement flaps.
Moore and colleagues modified this technique with relaxing
incisions along the lateral border of the latissimus in the
posterior axillary line.145 Dissection is carried out under the
latissimus muscle with elevation of the thoraco-lumbar fascia
in continuity with the superficial gluteal fascia. Intercostal
and lumbosacral perforators are divided medially. The para­
spinous fascia is incorporated to provide additional strength
to the deep layer of the closure. While the authors reported
no complications from this series, the maximum defect they
closed was 50 cm2. The need for skin grafts in the relaxing
incisions added scarring to the patient, making this technique
less desirable. In contrast, McCraw and colleagues reported
82 patients who received a similar bilateral latissimus dorsi
and trapezius musculocutaneous advancement flaps without
the need for relaxing incisions.146,158
A more extensive version of the bilateral latissimus muscu­
locutaneous advancement was proposed by Ramirez and
colleagues.147 In nine neonates with large thoraco-lumbar
meningomyeloceles, the authors elevated the thoraco-lumbar
fascia over the paraspinous muscles to the lateral border of
Fig. 40.22  (A–D) In 1968, Mustarde proposed the usage of bifid spinal processes in the reconstruction of the spinal canal. He argued that the skin cover alone was
insufficient as a means to protect the spinal cord. Following dural closure, the paraspinal muscles are incised laterally. The spinal processes are osteotomized and the
paraspinal muscle flaps with the associated spinal processes are combined into turnover flaps.
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908
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
Fig. 40.23  Thoracolumbar meningomyelocele involving most of the back. (A) Note the fine membrane covering most of the defect. (B,C) After neural tube repair and
reinforcement with paraspinous muscles, gluteus, latissimus, and trapezius muscles advanced to midline with minimal undermining. (D) V-Y relaxing incision through skin
and subcutaneous tissue to allow for tension-free midline closure.
A
B
C
D
of tissue expansion and delayed primary closure in older
children who had poor quality skin coverage over their spinal
defects.160
Preferred methods
Meningomyeloceles display heterogeneity, therefore necessi­
tating the need for a varied armamentarium of reconstructive
options individualized to each patient, with the goal of pro­
viding reliable closure using well-vascularized tissue without
tension (Fig. 40.23). While many of the studies mentioned
above refer to measurements, the size of defects is less impor­
tant than the relative amount of the uninvolved surrounding
back skin (Fig. 40.24).154
The primary goals are to protect cord elements, avoid infec­
tion, and seal any CSF leak. With that, surgery within the
first 24 h of life is routinely performed by a multidisciplinary
team of neurosurgeons and plastic surgeons. After closure
of the spinal placode by the neurosurgeon (Fig. 40.25), the
plastic surgery team provides a multilayer, tension free,
well-vascularized definitive closure. The first layer above
the neural closure is paraspinous turnover flaps to help seal
and protect the neural repair (Fig. 40.26). Perforator-based
crescent-shaped V-Y advancement/rotation flaps are then
performed to provide well vascularized tissue. This flap
is designed so that the tail of the Y is oriented to where
there is most skin laxity laterally. Its goal is to minimize skin
tension in the midline. With a paucity of skin lateral to the
defect, the crescent design of the V-Y allows for recruitment
of tissue from the lateral buttock and thigh area. Flaps used
to close lumbar-sacral defects are based on gluteal perforators
advancing and rotating the skin from inferior-lateral to
midline (Fig. 40.27). Flaps used to close thoraco-lumbar
defects are usually based on the paraspinal or latissimus
perforators rotating the skin (with or without muscle) from

909
Posterior defects
neonatal period, the patients tend to recover well with rare
incidence of malignancy and recurrence. Malignant tumors
require adjuvant chemotherapy.
Clinical presentation and surgical indications
Patients are usually identified at birth with a midline round,
cystic, or solid mass attached at the sacrum or coccyx. Terato­
mas grow in the posterior and inferior direction with stretch­
ing of the muscles surrounding the tumor. Large tumors may
cause difficulty in delivery. There is occasional intra-abdominal
extension. Dr. Altman and colleagues developed a classifica­
tion system based upon anatomic location of the teratoma.
Type I (46.7%) is predominantly external with minimal exten­
sion in the presacral region; Type II (34.7%) is also predomi­
nantly external, but has a significant intrapelvic component;
Type III (8.8%) is primarily a pelvic and abdominal mass, but
also has a smaller external component; finally, Type IV (9.8%)
consists of a presacral mass without any external portions
visible.162 Teratomas can usually be detected by elevated
maternal AFP levels and routine prenatal ultrasound. Types
I–III are readily classified on prenatal ultrasound. It is common
to also find polyhydramnios on the prenatal ultrasound.
Treatment
Resection is usually performed shortly after birth when the
neonate is deemed medically stable to undergo surgery. The
Fig. 40.25  Meningomyelocele. Neurosurgeons preparing to close the neural
elements prior to any soft-tissue closure.
A
Fig. 40.26  Paraspinal muscle flaps. (A) Lateral edge of paraspinous muscles are
incised and advanced to ward midline. (B) Paraspinous muscle flap closure
completed providing a well vascularized layer to protect the spinal closure beneath
it.
B
Fig. 40.24  Lumbosacral meningomyelocele. Not enough skin lateral to the defect
to allow for primary closure without tension. The location of the iliac crests are
marked, as is the midline (dotted line) and edge of the compromised skin.
superior-lateral to midline, similar to the case report described
by Sarifakioglu et al.157
Sacrococcygeal teratoma
Sacrococcygeal teratomas are the most common congenital
tumors in the posterior trunk with an incidence of 1 in 40 000
live births with a female predominance.161 Teratomas are
thought to originate from embryonic cell material derived
from totipotent cells, thus organ parts are frequently found
with elements or various different tissue types. Most sacrococ­
cygeal teratomas are benign and consist of well differentiated
mature tissues. They uniformly can undergo malignant trans­
formation with increasing age and therefore usually removed
shortly after birth. When the tumors are excised during the

910
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
Postanal pits occur over the coccyx and appear to tract
similar to pilonidal cysts. Asymptomatic pits pose no danger
and can be observed. When infected, postanal pit resection
usually only requires primary closure.
Neonates with tethered cord frequently present with a tuft
of hair or an angiomatous lesion at the site of tethering.
Neurosurgical repair is important for prevention of future
neurologic dysfunction. Skin is virtually always primarily
closed.
Diastematomyelia presents similarly to tethered cord.165
The spinal cord is divided with a median septum and patients
tend to have focal neurologic deficits. Prognosis is excellent
for such patients and primary closure is routine.
resulting defect usually can be closed primarily via a chevron
incision with local tissue rearrangement. One case report of a
giant sacrococcygeal teratoma resection with a resulting skin
defect has required advancement of gluteal musculocutane­
ous flaps for closure.163
Other posterior malformations
Dermal sinus and postanal pits are congenital back lesions
that rarely require extensive reconstruction.164 Dermal sinuses
are found in the midline back between the occiput and the
sacrum and are sometimes associated with an angioma. Infec­
tion of the sinus tract can result in recurrent meningitis.
Fig. 40.27  Crescent V-Y. (A) Following closure of the paraspinal muscle flaps, the skin defect is addressed.
With a paucity of skin lateral to the defect, the crescent design of the V-Y allows for recruitment of tissue
from the lateral buttock and thigh area. An intraoperative hand held Doppler helps identify perforating vessels
and facilitate flap planning and elevation. (B) The superior limbs of the fasciocutaneous crescent V-Y
advancement flaps with V-Y back-cuts are incised and the flap is isolated on its main perforators. The inferior
limbs are only completed (to islandize a true V-Y flap) if needed to release tension. (C) Skin closure
accomplished without tension in the midline. V-Y back-cut converted to a Z to facilitate insetting of the
lateral tip of the flap. (D,E) Incisions at 3 months postoperatively.
A
B
C
D
E

911
Posterior defects
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giant omphalocele. J Pediatr Surg. 2006;41:e37–e39.
110. Lee SL, DuBois JJ, Greenholz SK, et al. Advancement flap
umbilicoplasty after abdominal wall closure: postoperative results
compared with normal umbilical anatomy. J Pediatr Surg.
2001;36:1168–1170.
111. Park S, Hata Y, Ito O, et al. Umbilical reconstruction after repair of
omphalocele and gastroschisis. Plast Reconstr Surg. 1999;104:
204–207.
112. Schuster SR. A new method for the staged repair of large
omphaloceles. Surg Gynecol Obstet. 1967;125:837–850.
113. Kidd JN Jr, Jackson RJ, Smith SD, et al. Evolution of staged versus
primary closure of gastroschisis. Ann Surg. 2003;237:759–764.
114. Pastor AC, Phillips JD, Fenton SJ, et al. Routine use of a SILASTIC
spring-loaded silo for infants with gastroschisis: a multicenter
randomized controlled trial. J Pediatr Surg. 2008;43:1807–1812.
115. Schmidt AF, Gonçalves A, Bustorff-Silva JM, et al. Does staged
closure have a worse prognosis in gastroschisis? Clinics (Sao Paulo).
2011;66:563–566.
116. Malbrain ML, De Laet IE, De Waele JJ, et al. Intra-abdominal
hypertension: definitions, monitoring, interpretation and
management. Best Pract Res Clin Anaesthesiol. 2013;27:249–270.
117. Sanders RC, Blackmon LR, Hogge WA, et al. Omphalocele.
Structural Fetal Abnormalities: the Total Picture. 2nd ed. St. Louis:
Mosby; 2002:221–223.
118. Scott JP, Hoffman GM. Near-infrared spectroscopy: exposing the
dark (venous) side of the circulation. Pediatr Anesth. 2014;24:
74–88.
119. Wallingford JB. Neural tube closure and neural tube defects:
studies in animal models reveal known knowns and known
unknowns. Am J Med Genet C Semin Med Genet. 2005;135C:59–68.
120. Scheflan M, Mehrhof AI Jr, Ward JD. Meningomyelocele closure
with distally based latissimus dorsi flap. Plast Reconstr Surg.
1984;73:956–959.
121. Bowman RM, Boshnjaku V, McLone DG. The changing incidence
of myelomeningocele and its impact on pediatric neurosurgery: a
review from the Children’s Memorial Hospital. Childs Nerv Syst.
2009;25:801–806.
122. Katzen M. The total care of spina bifida cystica. Surg Annu.
1981;13:325–339.
123. Laurence KM. Effect of early surgery for spina bifida cystica on
survival and quality of life. Lancet. 1974;1:301–304.
124. Brocklehurst G. Spina Bifida for the Clinician. London: Heinemann
Medical for Spastics International Medical Publications; 1976.
125. Patterson TJ. The use of rotation flaps following excision of lumbar
myelo-meningoceles: an aid to the closure of large defects. Br J
Surg. 1959;46:606–608.
126. Luce EA, Walsh J. Wound closure of the myelomeningocoele
defect. Plast Reconstr Surg. 1985;75:389–393.
127. Cruz NI, Ariyan S, Duncan CC, et al. Repair of lumbosacral
myelomeningoceles with double Z-rhomboid flaps. Technical note.
J Neurosurg. 1983;59:714–717.
128. Komuro Y, Yanai A, Koga Y, et al. Bilateral modified V-Y
advancement flaps for closing meningomyelocele defects. Ann
Plast Surg. 2006;57:195–198.
129. Turhan Haktanir N, Eser O, Demir Y, et al. Repair of wide
myelomeningocele defects with the bilateral fasciocutaneous flap
method. Turk Neurosurg. 2008;18:311–315.
130. Ozcelik D, Yildiz KH, Is M, et al. Soft tissue closure and plastic
surgical aspects of large dorsal myelomeningocele defects (review
of techniques). Neurosurg Rev. 2005;28:218–225.
131. Mutaf M, Bekerecioglu M, Erkutlu I, et al. A new technique for
closure of large meningomyelocele defects. Ann Plast Surg.
2007;59:538–543.
132. Atik B, Tan O, Kiymaz N, et al. Bilobed fasciocutaneous flap
closure of large meningomyeloceles. Ann Plast Surg.
2006;56:562–564.
133. Lapid O, Rosenberg L, Cohen A. Meningomyelocele reconstruction
with bilobed flaps. Br J Plast Surg. 2001;54:570–572.
134. Gumus N. A new approach to closure of myelomeningocele defect:
z advancement-rotation flap. Ann Plast Surg. 2008;61:640–645.
135. Ohtsuka H, Shioya N, Yada K. Modified Limberg flap for
lumbosacral meningomyelocele defects. Ann Plast Surg.
1979;3:114–117.
136. Habal MB, Vries JK. Tension free closure of large
meningomyelocele defects. Surg Neurol. 1977;8:177–180.
137. Blaiklock CR, Demetriou EL, Rayner CR. The use of a latissimus
dorsi myocutaneous flap in the repair of spinal defects in spina
bifida. Br J Plast Surg. 1981;34:358–361.
138. Hosseinpour M, Forghani S. Primary closure of large
thoracolumbar myelomeningocele with bilateral latissimus dorsi
flaps. J Neurosurg Pediatr. 2009;3:331–333.
139. El-khatib HA. Large thoracolumbar meningomyelocele defects:
incidence and clinical experiences with different modalities of
latissimus dorsi musculocutaneus flap. Br J Plast Surg.
2004;57:411–417.
140. Jaworski S, Dudkiewicz Z, Lodzinski K, et al. Back closure with a
latissimus dorsi myocutaneous flap. J Pediatr Surg. 1992;27:
74–75.
141. Lehrman A, Owen MP. Surgical repair of large
meningomyeloceles. Ann Plast Surg. 1984;12:501–507.
142. Hayashi A, Maruyama Y. Bilateral latissimus dorsi V-Y
musculocutaneous flap for closure of a large meningomyelocele.
Plast Reconstr Surg. 1991;88:520–523.
143. VanderKolk CA, Adson MH, Stevenson TR. The reverse latissimus
dorsi muscle flap for closure of meningomyelocele. Plast Reconstr
Surg. 1988;81:454–456.
144. Duffy FJ Jr, Weprin BE, Swift DM. A new approach to closure of
large lumbosacral myelomeningoceles: the superior gluteal artery
perforator flap. Plast Reconstr Surg. 2004;114:1864–1870.
145. Moore TS, Dreyer TM, Bevin AG. Closure of large spina bifida
cystica defects with bilateral bipedicled musculocutaneous flaps.
Plast Reconstr Surg. 1984;73:288–292.
146. McCraw JB, Penix JO, Freeman BG, et al. Soft-tissue repair of
myelomeningocele defects using bilateral latissimus dorsi and
trapezius musculocutaneous flaps. Ann Plast Surg. 1987;18:
147–155.

911.e4
SECTION III
CHAPTER 40 • Pediatric chest and trunk defects
147. Ramirez OM, Ramasastry SS, Granick MS, et al. A new surgical
approach to closure of large lumbosacral meningomyelocele
defects. Plast Reconstr Surg. 1987;80:799–809.
148. Mustarde JC. Reconstruction of the spinal canal in severe spina
bifida. Plast Reconstr Surg. 1968;42:109–114.
149. Desprez JD, Kiehn CL, Eckstein W. Closure of large
meningomyelocele defects by composite skin-muscle flaps. Plast
Reconstr Surg. 1971;47:234–238.
150. De Brito Henriques JG, Filho GP, Gusmao SN, et al. Intraoperative
acute tissue expansion for the closure of large myelomeningoceles.
J Neurosurg. 2007;107:98–102.
151. Iacobucci JJ, Marks MW, Argenta LC. Anatomic studies and
clinical experience with fasciocutaneous flap closure of large
myelomeningoceles. Plast Reconstr Surg. 1996;97:1400–1410.
152. Thomas CV. Closure of large spina bifida defects: a simple
technique based on anatomical details. Ann Plast Surg.
1993;31:522–527.
153. Ulusoy MG, Kocer U, Sungur N, et al. Closure of
meningomyelocele defects with bilateral modified V-Y
advancement flaps. Ann Plast Surg. 2005;54:640–644.
154. Butz DR, Seitz IA, Frim DM, et al. A ten-year review of
myelodysplastic defect management and use of novel closure
technique with V-Y crescentic rotation advancement flaps. J Plast
Reconstr Aesthet Surg. 2014;67:533–539.
155. Muneuchi G, Matsumoto Y, Tamai M, et al. Rhomboid perforator
flap for a large skin defect due to lumbosacral meningocele: a
simple and reliable modification. Ann Plast Surg. 2005;54:670–672.
156. Richards TA, Kortesis BG, Glazier S, et al. Double
myelomeningocele: case report and review. Br J Plast Surg.
2003;56:306–308.
157. Sarifakioglu N, Bingul F, Terzioglu A, et al. Bilateral split
latissimus dorsi V-Y flaps for closure of large thoracolumbar
meningomyelocele defects. Br J Plast Surg. 2003;56:303–306.
158. McCraw JB, Penix JO, Baker JW. Repair of major defects of the
chest wall and spine with the latissimus dorsi myocutaneous flap.
Plast Reconstr Surg. 1978;62:197–206.
159. Luce EA, Stigers SW, Vandenbrink KD, et al. Split-thickness skin
grafting of the myelomeningocele defect: a subset at risk for late
ulceration. Plast Reconstr Surg. 1991;87:116–121.
160. Mustoe TA, Gifford GH, Lach E. Rapid tissue expansion in the
treatment of myelomeningocele. Ann Plast Surg. 1988;21:70–73.
161. Barksdale EM Jr, Obokhare I. Teratomas in infants and children.
Curr Opin Pediatr. 2009;21:344–349.
162. Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma:
American Academy of Pediatrics Surgical Section Survey-1973. J
Ped Surg. 1974;9:389–398.
163. Baccarani A, Jacob V, Pedone A, et al. Composite lower back and
buttock reconstruction with gluteal myocutaneous flap in the just
born. Plast Reconstr Surg. 2009;124:269e–270e.
164. Radmanesh F, Nejat F, El Khashab M. Dermal sinus tract of the
spine. Childs Nerv Syst. 2010;26:349–357.
165. Weinzweig J, Holman PD, Rekate HL. Diastematomyelia: a
congenital anomaly not to be confused with a giant hairy nevus.
Plast Reconstr Surg. 1995;96:183–193.

SECTION III • Pediatrics
41 
Pediatric tumors
Sahil Kapur and Michael Bentz
SYNOPSIS
Neurofibromatosis
■ Neurofibromatoses are a group of inherited disorders with established
diagnostic criteria based on symptoms of presentation.
■ Craniofacial presentation of this disease is classified based on the
surgical treatment options available.
Juvenile aggressive fibromatosis
■ Fibroblastic or myofibroblastic neoplasms are locally aggressive
lesions.
■ Different presentations include:
• Fibromatosis colli
• Congenital solitary or generalized fibromatosis
• Infantile digital fibroma
• Gingival fibromatosis
• Juvenile nasopharyngeal angiofibroma.
Dermoids
■ They are present from birth and consist of both ectoderm and
endoderm.
■ They commonly arise in the head and neck. They can grow large to
compress adjacent structures.
■ Different types include: nasal dermoids, intradural dermoids, extra-
angular dermoids and dermoid cysts of the neck.
Branchial cleft anomalies
■ Each branchial arch, pouch, and groove complex forms a particular
area of the head and neck.
■ The 1st and 2nd branchial cleft anomalies comprise 98% of all such
anomalies. The 3rd and 4th brachial cleft anomalies form the other
2%. Definitive treatment involves surgical excision.
Thyroglossal duct cysts
■ A persistent embryological remnant of the descending thyroglossal
duct gives rise to a cyst.
■ This clinically presents in the first two decades of life.
■ Treatment involves excision using the Sistrunk technique.
Pilomatrixoma
■ These are ectodermal in origin and arise from the outer root sheath
cells of the hair follicle.
■ These tumors usually occur in the head and neck region of children.
■ Treatment is surgical excision, with radiation therapy for malignant
lesions.
Rhabdomyosarcoma
■ This is a malignant tumor of mesenchymal origin.
■ In the head and neck, the most common presentations are in the orbit,
nasopharynx, paranasal sinuses, and middle ear.
■ Four histologic types include embryonal, spindle-cell alveolar, and
pleomorphic rhabdomyosarcoma.
Synovial soft-tissue sarcoma
■ These sarcomas arise from synovioblastic differentiation of pluripotent
mesenchymal stem cells.
Alveolar soft part sarcoma
■ A rare tumor that occurs primarily in skeletal muscles or
musculofascial planes of extremities.
Lymphadenopathy
■ Acute bacterial lymphadenitis presents with mild fever, tender,
indurated, and erythematous lymph nodes, or systemic toxicity.
■ Chronic regional lymphadenopathy is observed in diseases such as cat
scratch disease.
■ Cervical tuberculous adenitis (Scrofula) begins with a pulmonary
infection that spreads to the lymph nodes.
Neurofibromatosis
Introduction
Neurofibromatoses are a set of inherited disorders comprising
neurofibromatosis-1 (NF-1), neurofibromatosis-2 (NF-2), and

913
Neurofibromatosis
Treatment/surgical technique
Treatment is local excision.
Craniofacial manifestation
Diagnosis/patient presentation
In the craniofacial region, the orbitotemporal area is most
commonly involved. Orbitotemporal neurofibromatosis asso­
ciated skeletal malformations and deformities include:11–17
■ Sphenoid wing hypoplasia, which leads to expansion of
the middle cranial fossa into the posterior orbit and
causes proptosis
■ Remodelling and/or decalcification of the posterior
orbit
■ Supraorbital tumors, which cause defects of the orbital
roof and lead to a downward and outward displacement
of the globe
■ Thinning of the lateral and inferior orbital rims
■ Depression of the orbital floor and elevation of the
orbital roof and supraorbital rim, which increases orbital
volume
■ Dysplasia and downward dislocation of the zygoma
■ Progressive plexiform neurofibromatosis of the temporal
area, which leads to continued enlargement of the
eyelids, excessive mechanical ptosis, pulsating proptosis,
eye pain, and epiphora.
Treatment/surgical technique
Treatment is based on Jackson’s Classification:11,14
Class 1: Significant soft tissue involvement with minimal
bone involvement and normal vision. Treatment involves
debulking of the soft-tissue component of the tumor
through an anterior, lateral, or anterolateral orbitotomy. If
blepharoptosis is present, levator resection is performed.16
In cases where only a partial resection is carried out, a
technique of netting the remaining tissue using Teflon
mesh has been proposed (Fig. 41.1).18
Class 2: Soft tissue and bone involvement with normal
vision. Treatment involves an intracranial approach for
tumor debulking and posterior orbital wall
reconstruction.16 After the tumor is debulked, the herniated
temporal lobe is reduced and bone grafts, obtained from
splitting the contralateral frontal bone, are used to
reconstruct the posterior and superior orbital walls. Orbital
volume is increased with osteotomies and the globe is
elevated by raising the canthal ligaments and building the
floor. A two-stage approach has been suggested in which
the intracranial portion of tumor debulking and orbital
reconstruction is completed in stage one. A second-stage
procedure is performed in which subcutaneous debulking,
eyelid, face, and orbital reconstruction is completed
(Fig. 41.2).19
Class 3: Soft-tissue and bone involvement with blindness or
an absent globe. Treatment involves debulking the tumor
with exenteration. An orbital approach is used to reduce
the herniated temporal lobe into the middle cranial fossa.
The bony defect is covered with a split-rib/bone graft. The
orbital volume is reduced and its position is adjusted with
Schwannomatosis. All of these lead to the formation of benign
nerve sheath tumors.1
Basic science/disease process
Neurofibromatosis-1 is an autosomal dominant disorder with
complete penetrance and variable expression. Chromosome
17 is involved. The incidence is 1/3000, and the average age
of children at the time of presentation is 7 years.1–3
Café-au-lait spots
Diagnosis/patient presentation
Café-au-lait spots are characterized as cutaneous, hyperpig­
mented lesions, typically 20–30 mm in diameter. They contain
keratinocytes with increased macromelanosomes. Greater
than six lesions are found in 90–99% of cases.4,5
Treatment/surgical technique
Surgery is rarely recommended. Laser treatment can be con­
sidered for cosmetic improvement.6,7
Lisch nodules
Diagnosis/patient presentation
Lisch nodules are dome-shaped, melanocytic hamartomas
found on the surface of the iris.4,5 They appear around age 10
years and are present in nearly all people with NF-1 by
age 20.
Treatment/surgical technique
No treatment is required.
Optic nerve gliomas
Diagnosis/patient presentation
Optic nerve gliomas are the most common central nervous
system tumors in NF-1 patients. They occur in 15% of cases
and are histologically identified as low-grade pilocytic astro­
cytomas.4,5,8 They are relatively indolent and sometimes
asymptomatic. If symptomatic, they can cause proptosis,
squint, abnormal color vision, visual field loss, pupillary
abnormalities, and hypothalamic dysfunction.7
Treatment/surgical technique
Treatment involves vincristine and cisplatinum.9 Surgery is
necessary if proptosis is present and if there is a need to
debulk extensive chiasmal gliomas.
Glomus tumors
Diagnosis/patient presentation
Glomus tumors are usually solitary lesions, but occur in
multiples in individuals with NF-1. They present under fin­
gernails with symptoms of cold sensitivity and increased
localized tenderness.10

914
SECTION III
CHAPTER 41 • Pediatric tumors
type of neurofibroma and represent fusiform enlargement of
peripheral nerves. These are the most common neurofibro­
mas of the upper extremity and account for 85% of cases.22
Spinal and cranial nerves may also be involved. Massive
soft-tissue neurofibromas (elephantiasis neurofibromatosa)
lead to distortion of the face and require complete excision.23
Plexiform neurofibromas are composed of nerve sheath cells
proliferating along the length of the nerve and are associated
with hypertrophy of the overlying soft tissue, hyperpigmen­
tation, and hypertrichosis of the overlying skin. Plexiform
neurofibromas occur in 16–40% of patients with NF. These
lesions involve the trunk (43–44%), extremities (15–38%), and
head and neck (18–42%).20 They are congenital in origin and
become evident by 2 years of age. Plexiform neurofibromas
are locally destructive lesions that grow during periods of
hormonal change, and may involve multiple nerve branches
and plexi.24
Treatment/surgical technique
Preoperative contrast-enhanced CT, MRI, angiography, and
embolization have been recommended.20–22,25 The highly
vascular nature of these lesions makes surgical removal com­
plicated.24 Multiple non-surgical management options such
as farnesyl transferase inhibitors, antiangiogenesis drugs, and
fibroblast inhibitors are being explored.26 Since the mechanistic
target of rapamycin (mTOR) pathways are a major mediator of
tumor growth in NF-1, drugs such as sirolimus are demonstrat­
ing beneficial effects such as the halting of tumor progression
and reduction of pain.27 Recent studies have demonstrated the
benefits of farnesyl transferase inhibitors such as Tipifarnib
in reducing rate of tumor progression.28 Tumors resected in
children below the age of 10 years re-occur in 60% of the cases,
while those resected above the age of 10 years re-occur in 30% of
the cases.29
Malignant degeneration of peripheral nerve
sheath tumors
Diagnosis/patient presentation
There is an 8–13% lifetime risk for these tumors to undergo
malignant degeneration. This occurs predominantly between
ages 20 and 35.4,7 Medium and large nerves of the thigh,
buttock, brachial plexus, and paraspinous areas are involved.
Signs of malignant degeneration include increased pain,
new neurologic deficits, sphincter disturbance, rapid increase
in the size of the neurofibroma, or change in texture.30
Fluorodeoxyglucose positron emission tomography helps
with the quantification of glucose metabolism in the cells
and can help distinguish between benign and malignant
lesions.31,32
Treatment/surgical technique
Prompt surgical intervention is necessary, which consists of
debulking and nerve grafting. Complete removal with tumor-
free margins is necessary. Adjuvant radiotherapy for tumors
>5 cm, high-grade lesions, or incompletely excised tumors is
recommended.7,30 Chemotherapeutic regimens consisting of
tamoxifen and trifluoperazine are proving to be efficacious in
halting progression of the tumor in rodent models.33
osteotomies and bone grafts. Finally, an orbital prosthesis
is fitted.
Neurofibromas
Diagnosis/patient presentation
Nerve sheath tumors arise between the dorsal root ganglion
and terminal nerve branches.4,5,20 They are composed of
schwann cells, fibroblasts, mast cells, and perineural cells.7,20,21
Localized cutaneous neurofibromas are the most common
nerve sheath tumors. They present as multiple slow growing,
pedunculated lesions that progressively increase in promi­
nence.7 These lesions can be surgically excised for symptom­
atic benefit. This, however, may lead to hypertrophic scarring.
The benefits of carbon dioxide laser treatment have not been
clearly established. Diffuse cutaneous neurofibromas present
as a plaque-like thickening of the dermis and subcutaneous
tissue and are most frequently found in the head and neck
region. These are non-destructive, soft, compressible lesions
that grow along the fibrous septae in children and young
adults. Removal of these subcutaneous lesions may lead
to neurologic deficits in the region of the concerned nerve.
Localized intraneural fibromas are the second most frequent
A
Fig. 41.1  (A,B) Anterior orbitotomy with debulking of left orbitotemporal
neurofibromatosis. (Courtesy of Dr. Delora L. Mount.)
B

915
Juvenile aggressive fibromatosis
Fig. 41.2  (A–C) Stealth-guided left frontal craniotomy, orbital osteotomy, orbital roof/anterior clinoid resection, extradural orbitofrontal tumor dissection, and periorbital
cranial reconstruction for craniofacial neurofibromatosis. (D) Specimen: neurofibroma lesion. (Courtesy of Dr. Delora L. Mount.)
A
B
C
D
Malignant schwannomas
(neurofibrosarcomas)
Diagnosis/patient presentation
Neurofibrosarcomas can involve either the cervical vagus
nerve or the sympathetic chain and present as parapharyngeal
masses, with paresthesias, pain, and muscle weakness.34 They
may also be of parotid origin. Children with NF-1 are at
increased risk for developing these lesions.35
Treatment/surgical technique
Surgery is the primary treatment modality. Adjuvant chemo­
therapy and radiation may be indicated. Local recurrence and
lung metastases are common.36,37
Juvenile aggressive fibromatosis
Basic science/disease process
Fibroblastic or myofibroblastic neoplasms are locally aggres­
sive lesions. The average age of onset is the third decade of
life. These tumors, however, may also occur during the 1st
month of life. About 5% of cases are found in the hand.38
Fibromatosis colli
Diagnosis/patient presentation
Fibromatosis colli are solitary tumors of the sternocleidomas­
toid muscles (SCM) and present as a cervical mass during
infancy. They are the most common cause of neonatal torticol­
lis. The tumors are first seen between 3 and 4 weeks of age.
They develop in the lower portion of the SCM and may
involve both sternal and clavicular heads of the muscle. The
diagnosis is made through fine-needle aspiration that yields
fibroblasts, degenerative atrophic skeletal muscle cells, and
numerous giant muscle cells. Ultrasound can also be used as
a non-invasive modality to facilitate diagnosis. Natural
regression of the tumor is seen during the first 6 months of
life. Torticollis is associated with fibromatosis colli in 23–33%
of patients and may continue after treatment in 17% of patients
because of progressive fibrous replacement of the muscle
tissue.39
Treatment/surgical technique
Surgery involves releasing both heads of the SCM through a
limited transverse incision. The clavicular head is readvanced
and attached to the sternal head to lengthen the muscle and
preserve the anatomic landmarks of the sternal column.

916
SECTION III
CHAPTER 41 • Pediatric tumors
Gingival fibroma
Diagnosis/patient presentation
Gingival fibromas are common lesions of the oral cavity
caused by chronic irritation that leads to connective tissue
hyperplasia. They occur on any oral mucosal surface, includ­
ing the tongue, palate, cheek, and lip. The lesions are pale,
smooth and firm, with sessile or pedunculated bases, and are
usually <1 cm in diameter.
Treatment/surgical technique
Treatment is surgical excision. Recurrence is rare if the source
of irritation is removed.
Juvenile nasopharyngeal angiofibroma
Diagnosis/patient presentation
Juvenile nasopharyngeal angiofibromas are benign, locally
aggressive, vascular lesions, typically found in adolescent
males between the ages of 10 and 17. They originate in
the posterolateral nasopharynx, near the sphenopalatine
foramen, and present with nasal obstruction and epistaxis.
About 66% of patients present with local disease and 20%
have intracranial invasion. CT scans show widening of
the pterygopalatine fossa and the pterygomaxillary fissure.
MRI is used to delineate the soft tissue invasion, while
angiography helps determine the vascular supply. Given the
highly vascular nature of these tumors, biopsy should not be
attempted.
Treatment/surgical technique
Treatment involves embolization followed by surgery. A
transpalatal, transfacial, midface degloving or LeFort I oste­
otomy approach can be used. An anterior subcranial approach
is used for transcranial lesions.42–44
Endoscopic approaches are becoming more popular due to
progresses made in instrumentation, skull base anatomy,
and surgical strategy. A recently published systematic review
comparing open and endoscopic treatment approaches found
that endoscopic approaches are associated with lower blood
loss and recurrence rates.45,46
Outcomes, prognosis, and complications
The recurrence rate is 73%, but can be as high as 90% with
positive margins. Recurrence usually occurs within 3 months
of excision.38
Dermoids
Introduction
Dermoids are present from birth, and form due to embryonic
inclusion of germ cells between fusing tissue layers. They
commonly arise in the head and neck.
Surgery is performed on patients in whom torticollis persists
for 1 year.40
Congenital fibromatosis
Diagnosis/patient presentation
Congenital fibromatosis can present in a solitary or general­
ized form. Solitary lesions can be seen as well-demarcated,
firm, palpable masses (<3 mm) in skin, subcutaneous tissue,
muscle, or as hyperlucent masses in bone. These lesions are
usually present at birth, but additional lesions may develop
later.
Treatment/surgical technique
Treatment is surgical resection. The rate of recurrence is about
32%.41
Infantile digital fibroma
Diagnosis/patient presentation
These generally appear as single or multiple gelatinous or
firm nodules on fingers or toes. They are rare and are seen in
both males and females. They are generally harmless but are
removed because of the discomfort they cause by rubbing on
footwear. The diagnosis can be confirmed with a skin biopsy
that shows spindle-shaped cells and collagen fibers in the
dermis. A radiograph is usually taken to determine the extent
of the lesion (Fig. 41.3).
Treatment/surgical technique
Surgical procedure is relatively simple and calls for shaving
off the lump. Recurrence rate after surgery is high.
Outcomes, prognosis, and complications
A conservative approach can also be undertaken because
many fibromas can resorb and disappear by themselves over
2–3 years.
Fig. 41.3  Infantile digital fibroma. (Courtesy of Dr. Michael L. Bentz.)

917
Dermoids
open rhinoplasty approach. This approach has been reported
to have improved exposure of the osteotomies, the upper
lateral cartilages, and the septum (Fig. 41.5).50
A closed rhinoplasty technique has been proposed for the
excision of superficial distal nasal tip dermoids. Since the
majority of nasal dermoid cysts are confined to the superficial
nasal area, this technique can prove to be beneficial.51 For
lesions with suspected/confirmed intracranial extension,
failure to completely excise the tract can lead to abscess for­
mation, meningitis, or osteomyelitis.52,53 Multiple approaches
have been proposed.54–67 The traditional approach calls for
combining an intracranial procedure such as a bifrontal cra­
niotomy, with an extracranial procedure, such as a transverse,
vertical, inverted U, lateral rhinotomy, or rhinoplasty to
address the entire sinus tract.54,55
A second approach, described as the “keystone” technique,
involves a bifrontal craniotomy superior to the supraorbital
rims, two paramedian sagittal osteotomies extending down
the length of the nasal bones, followed by out-fracturing of
the keystone component. This technique allows complete
exposure of the sinus tract and enhanced exposure of the
Nasal dermoids
Basic science/disease process
Between the 3rd and 8th week of embryogenesis, when the
neural groove deepens to form the neural tube, incomplete
sequestration of the neuroectoderm from the somatic ecto­
derm leads to a persistent connection of the foramen cecum
with the fonticulus nasofrontalis, and the foramen cecum with
the prenasal space. These connections cause the formation of
nasal dermoids, dermal sinuses, gliomas, and encephalo­
celes.47 Nasal dermoids can be present at any point between
the glabella and the base of the columella. Intracranial exten­
sion can exist via the tract through the nasal septum and
foramen cecum, or through the widened frontonasal suture
(fonticulus nasofrontalis) and foramen cecum. In these cases,
the presence of the dermoid between the leaves of falx and a
bifid crista galli are noted (Fig. 41.4).
Diagnosis/patient presentation
Dermoids present as firm, cystic lesions, or infected persistent
abscesses. They expand slowly and cause destruction of the
nasal bones and widening of the nasal ridge. MRI can help
differentiate between normal anatomic variants of the anterior
cranial base versus the intracranial extension of dermal sinus
tracts. CT scans can help delineate the bony anatomy of the
nose and the cranial base, and help in operative planning with
the use of 3D reconstruction.48,49
Treatment/surgical technique
Dermoid cysts or sinuses that are present at the columella
usually extend to the nasal spine. Resection involves a circum­
scribed removal of the sinus tract. If a cyst is associated with
it, it is dissected through the labial sulcus. Sinuses and cysts
that are present from the radix to the nasal tip, but are without
intracranial extension, are excised in combination with an
Fig. 41.4  Dermoid embryology.
Frontal bone
Dura mater
Nasal bone
Cartilage
Brain
Prenasal space
Base of
skull
Foramen
cecum
Dura in
contact
with skin
A
Fig. 41.5  (A,B) Excision of nasal dermoid without intracranial extension. (Courtesy
of Dr. Diane G. Heatley.)
B

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SECTION III
CHAPTER 41 • Pediatric tumors
Secondary procedures
Immediate reconstruction is preferred and may involve the
use of conchal or costal cartilage grafts, as well as bone grafts
for reconstruction of the cartilaginous skeleton of the nose.
Reconstruction of “keystone” skull base defects can be carried
out with bone grafts taken from the parietal bone of the skull.
The origin of the frontal sinus may be disturbed, and the result­
ing defect, if not in continuity with the sinus, can occasionally
be corrected, some reporting the use of hydroxyapatite.
Intradural dermoids and dermal sinus tracts
Basic science/disease process
The incomplete sequestration of neuroectoderm and somatic
ectoderm during embryogenesis, can lead to persistent dermal
sinus tracts from the occiput to the sacrum.68,69 About 1% of
these tracts are found in the cervical spine, 10% in the thoracic
spine, 41% in the lumbar spine, and 35% in the lumbosacral
spine.70 These sinus tracts are cephalically oriented, lined with
stratified epithelium, and may lead to the vertebral column,
ending as intradural dermoid cysts.68
Diagnosis/patient presentation
Dermal sinus tracts can present as hypertrichosis, skin tags,
abnormal pigmentation, subcutaneous lipomas or angio­
mata.71,72 The presence of these tracts can also lead to recurrent
bacterial meningitis. Additionally, traction on the spinal cord
can occur and can lead to symptoms of motor weakness,
autonomic irritation, or sphincter dysfunction. MRI is the
imaging tool of choice, and helps with the evaluation of other
associated pathologies such as inclusion tumors, dermoids,
epidermoids, teratomas,73–80 split cord malformations, and
tethered cords (Fig. 41.7).81–83
Treatment/surgical technique
Surgical excision involves tracing the tract through the sub­
cutaneous tissue, lumbosacral fascia, and bony defect. If the
dura is involved, laminectomy is necessary to open the dura
and explore the intradural space. Laminectomy may be
required to inspect the intradural extension within the sub­
arachnoid space. Intramedullary dermoids located at the
conus are usually associated with cephalic extension. Occa­
sionally, there is an associated thickened tethered cord, which
requires sectioning of the filum.83 The presence of adhesions
at the site of previous resection and associated progressive
neurologic deficits, require re-exploration and lysis of
adhesions.84,85
External angular dermoids
Basic science/disease process
External angular dermoids are usually fixed to the orbital rim
periosteum and the frontozygomatic suture, with very rare
intraosseous extension. Intraosseous extension presents as
intraosseous cysts that can very rarely erode intracranially.86
External angular dermoids usually lie along the trajectory of
the Tessier No.10 cleft. They may also be present more medi­
ally at the brow and supraorbital region. If they approach the
anterior cranial base.59 A similar subcranial-transglabellar
approach involves horizontal osteotomies above the supraor­
bital rims and at the level of the nasal bones, and vertical
osteotomies at the supraorbital rims to expose the intracranial
portion of the dermoid. This technique allows one to approach
the lesion from one direction, thereby maintaining a single
field. It is also attributed to require decreased frontal lobe
retraction and therefore has a lower risk of contusions, cere­
bral edema, and long-term neurologic defects.61 The osteotomy
size is smaller than the traditional frontal craniotomy
approach, which reduces the risk of dural tears or CSF leaks
(Fig. 41.6).64
Outcomes, prognosis, and complications
The recurrence rate of nasal dermoids after surgical excision
is 12%.67 A meta-analysis showed that the rate of complica­
tions with the traditional craniofacial approach was 30%,
while the subcranial approach reduced the complication rate
to 16%.63 Complications included tension pneumocephalus,
CSF leakage, subdural hematoma, longer operative times, and
longer ICU stays.64,65
Fig. 41.6  Subcranial approach to intracranial dermoids: (A) frontonasal bone
fragment; (B) subcranial approach with simultaneous access to the frontal lobe, the
anterior skull base, and the nasal cavity; (C) reconstruction with replacement of the
frontonasal bone fragment, cantilever rib grafts to the nasal dorsum, and orbital
floor reconstruction with split calvarial bone graft.
A
B
C
Frontonasal bone flap
Cantilever rib graft

919
Dermoids
to gain access. Endoscopic assisted excision is discouraged
laterally because it has been associated with injury to the
facial nerve.
Dermoid cysts of the neck
Basic science/disease process
In contrast to dermoids of the head, which are found in dif­
ferent fusion planes, these present as subcutaneous masses.
About 28% of them are dermal in origin. They are located in
the midline of the neck and are composed of ectodermal
tissue, sebaceous glands, and hair.87–89 The epidermoid cyst is
the most common dermoid cyst.89
frontonasal junction, they need to be evaluated as frontonasal
dermoids.
Diagnosis/patient presentation
External angular dermoids are slow growing and rarely
exceed 4 cm in size. Size varies based on sweat gland
activity.86
Treatment/surgical technique
Most lesions are excised through an incision in the lateral
portion of the upper lid. Dermoids are generally found
underneath the orbicularis muscle. If the dermoid is large
with extension temporally, a hairline incision can be made
Fig. 41.7  (A) External manifestation of intradural dermoid. (B,C) MRIs showing
intradural dermoid. (Courtesy of Dr. Dennis P. Lund.)
A
B
C

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SECTION III
CHAPTER 41 • Pediatric tumors
Treatment/surgical technique
Fistulous tracts can be identified by injecting methylene blue
or radio-opaque material into the tract followed by a CT
scan.95 This step can be avoided since the tract may easily be
followed during the excision. While dissecting out the tract,
care is taken to preserve the hypoglossal and glossopharyn­
geal nerves, and the internal and external carotid arteries. If
the tract extends into the base of the tonsillar fossa, the tonsil
may need to be resected (Fig. 41.10).
The 1st branchial cleft anomaly (8%)
Basic science/disease process
Normal fusion of the 1st branchial arch, presents as the exter­
nal auditory meatus. A complete non-fusion of the 1st arch
leaves a cleft extending from the external auditory canal
traveling along and under the border of the mandible up to
the midline.
Diagnosis/patient presentation
About 66% of the associated lesions are cysts. These usually
present as masses in the parotid region along with pits or
depressions near the external auditory meatus.96 Sinuses and
fistulas are less common. These tracts may pass through the
parotid gland and their excision may jeopardize facial nerve
branches. Fistulas can be traced from the junction of the ear
and cartilaginous canal, to the overlying skin (Fig. 41.11).
Fistulas present as two types:
The type 1 anomaly is a duplication of the membranous
external meatal canal and is composed of ectodermal
elements. The tract begins medial, antero-inferior or
posterior to the pinna and conchal cartilages. It then runs
parallel to the external canal and has a blind end in the
middle tympanic region.
The type 2 anomaly is a duplication of the membranous ear
canal and the auricle and therefore contains both
ectodermal and mesodermal structures. The tract begins
with an opening in the anterior neck, superior to the hyoid
Diagnosis/patient presentation
In newborns, these can present as masses in the floor of the
mouth, with extension to the midline of the neck.90
Branchial cleft anomalies
Introduction
Fusion of branchial arches takes place 3–6 weeks’ gestation.
Failure of fusion of these arches leads to branchial cleft
anomalies, which present as cysts, internal sinuses, external
sinuses, fistulas or combinations of the previous. A branchial
anomaly is present inferior to all the embryonic derivatives of
its associated arch, and superior to all the embryonic deriva­
tives of the next arch.91 Cysts are the most common and are
lined by squamous or columnar epithelium. Cysts and the
masses associated with them are usually found in the anterior
cervical triangle near lymph tissue.92 Internal sinuses present
with infection and halitosis. External sinuses present as open­
ings in the middle to lower neck in the anterior cervical triangle
or by the external ear.
The 2nd branchial cleft anomaly (90%)
Basic science/disease process
A complete 2nd branchial cleft begins near the sternal origin
of the SCM, passes along the anterior border of the SCM
lateral to the hypoglossal and glossopharyngeal nerves, and
ends at the tonsillar fossa, superior to the hypoglossal and
glossopharyngeal nerves (Fig. 41.8).
Diagnosis/patient presentation
Cystic masses are more common. Sinuses and fistulas may be
present along the superior two-thirds of the tract.93 The tract
may be a blind sinus, or may extend all the way up to the
tonsillar fossa, leading to chronic salivary drainage problems.
These lesions also present as recurrent deep neck infections,
and CT scans are helpful in recognizing them (Fig. 41.9).94
Fig. 41.8  Branchial cleft.
First branchial cyst type II
Second branchial cleft cyst
Hyoid bone
Parotid gland
Sternocleidomastoid
muscle
Duct opens anterior to
sternocleidomastoid
muscle
Fig. 41.9  Second branchial cleft cyst. (Courtesy of Dr. Diane G. Heatley.)

921
Thyroglossal duct cysts
deep to the carotid artery, toward or through the thyrohyoid
membrane. It originates at the base/cranial end of the pyri­
form sinus and then passes above the superior laryngeal
nerve.100
The 4th branchial cleft anomaly
The 4th branchial cleft anomaly begins at the apex of the
pyriform sinus and passes through the cricothyroid mem­
brane beneath the superior laryngeal nerves. It then travels
inferiorly in the tracheoesophageal groove, posterior to the
thyroid gland and into the thorax. The tract then loops around
the arch of the aorta on the left and the subclavian artery on
the right and courses superiorly, posterior to the common
carotid artery. It finally makes another loop around the hypo­
glossal nerve and terminates at the medial border of the SCM.
The descending portion of the tract has the highest likelihood
of being infected. Patients present with respiratory distress,
mediastinal abscesses, and suppurative thyroiditis.101,102
Outcomes, prognosis, and complications
Recurrence rates are increased when there is a history of
multiple preoperative infections associated with the anomaly,
and when there is no epithelial tissue found in the
specimen.91
Thyroglossal duct cysts
Introduction
Thyroglossal duct cysts are the most common tumors of the
anterior cervical region, which are usually located in the
midline at or below the level of the hyoid. They are persistent
embryologic remnants of the descending thyroglossal duct.
bone and anterior to the SCM. It extends superiorly
through subcutaneous tissue, pierces the substance of the
parotid gland, and then passes superficial, deep, or in
between two branches of the facial nerve.97,98
Treatment/surgical technique
Incision and drainage of infected cysts may be necessary
before definitive surgery. The sinus/fistulous tracts extend
into the parotid gland and their excision may jeopardize the
facial nerve.93,98,99 Complete excision consists of a superficial
parotidectomy with facial nerve dissection.
The 3rd branchial cleft anomaly
The 3rd branchial cleft anomaly presents with a tract that has
an opening along the anterior border of the SCM and extends
Fig. 41.11  First branchial cleft cyst. (Courtesy of Dr. Diane G. Heatley.)
Fig. 41.10  (A) Excision of second branchial cleft cyst. (B) Excision of second
branchial cleft fistula. BA, branchial arch.
A
B
Second BA cyst
with sinus tract
Second BA
fistula with tract
CNXII
Carotid bifurcation
CNXII
Carotid bifurcation