Phẫu Thuật Quản Lý Và Điều Trị Dị Thường Mạch Máu

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Part II: Vascular Tumors
Fig. 6-6
A

B

C
n o n in vo l u t in g c o n g e n it a l h e Ma n g io Ma
Pa tie n t A
T is 10-year-old boy presented with a NICH since birth that was causing psychosocial morbidity.
T e preoperative (A) and postoperative appearance a er excision (B) is shown.
Pa tie n t B
T is adolescent boy had a NICH o the posterior neck. T e patient requested removal to improve
his appearance. T is patient is seen be ore surgery (A), with intraoperative markings (B), and
a er resection (C).
Fig. 6-5
A

B

85
Chapter 6: Rare Vascular Tumors
Pa tie n t C
T is 3-year-old had a NICH o the hand. T e lesion was removed to improve the de ormity be ore
the development o sel -esteem and long-term memory. T e tumor is shown during in ancy (A)
and at 3 years o age be ore resection (B). T e intraoperative markings (C), as well as a view o
the lesion through the incision (D), can be seen. T e wound is shown a er excision (E) and a er
wound closure (F).
Fig. 6-7
A

B
C

D
E

F

86
Part II: Vascular Tumors
k a Po s if o r M h e Ma n g io e n Do t h e l io Ma
Pa tie n t A
T is in ant girl had a biopsy-con rmed KHE o the right lower extremity. Because the lesion was
localized and not involving important structures, it was resected to obviate the need or chemo-
therapy. T e preoperative appearance is shown (A). An MRI illustrated the extent o the tumor (B).
T e markings or excision are seen (C). T e intraoperative view shows brous tissue and resected
specimen (D). Normal underlying musculature is visible a er extirpation (E). Wound closure is
shown (F). One year postoperatively, there has been no recurrence (G).
Fig. 6-8
A

B

C
F
D

E

G

87
Chapter 6: Rare Vascular Tumors
Pa tie n t B
T is 5-year-old boy had a KHE o the scalp. Because the lesion was resectable and at risk or de-
struction o the cranium and intracranial extension, it was excised. T e intraoperative outline o
the tumor (inner marking) and surgical margins (outer line) are shown (A). An MRI delineated
the lesion (B). T e patient is seen a er resection and a split-thickness skin gra o pericranium
(C). T e healed skin gra and tissue-expanded scalp are shown (D). T e patient is seen a er
excision o the skin gra , removal o tissue expanders, and reconstruction o the hair-bearing
scalp (E).
Fig. 6-9
A

B

C
D

E

88
Part II: Vascular Tumors
Pa tie n t C
T is boy developed an axillary contracture rom a KHE. During in ancy the patient was treated
with vincristine (A). Fibrosis caused a so tissue contracture, which contributed to the child’s
inability to ully raise his arm (B). T e axillary band was released with Z-plasties (C). A er surgery
the patient was better able to raise his upper extremity (D).
Fig. 6-10
A

B
C

D

89
Chapter 6: Rare Vascular Tumors
in f a n t il e Myo f ib r o Ma
T is newborn in ant had an in antile myo broma o the right neck. History, physical examina-
tion, and ultrasound ndings were inconsistent with an in antile hemangioma. Consequently,
an MRI was obtained, which ailed to clari y the diagnosis. A biopsy was then per ormed, which
con rmed the diagnosis. Because this tumor can spontaneously regress, the patient was observed.
T e initial appearance is shown (A). An MRI illustrated a heterogeneous-enhancing lesion (B).
T e tumor involuted at 1 year o age (C).
a r t e r io v e n o u s a c r a l t u Mo r
Pa tie n t A
T is child developed an enlarging lesion o his lower lip. T e di erential diagnosis was a pyogenic
granuloma or an arteriovenous acral tumor. Because o its small size, the tumor underwent com-
plete resection; histopathologic examination showed an arteriovenous acral tumor. T e lesion is
shown be ore (A) and a er (B) resection.
Fig. 6-11
A

B

C
A

B
Fig. 6-12

90
Part II: Vascular Tumors
Pa tie n t B
T is woman presented with a 2-year history o a slowly enlarging lesion o the le upper lip. T e
di erential diagnosis, which was based on history, physical examination, and imaging, was an
arteriovenous mal ormation, arteriovenous acral tumor, or Enzinger intramuscular hemangi-
oma. She underwent a subtotal resection to improve her de ormity; histopathologic examina-
tion showed an arteriovenous acral tumor. T e preoperative appearance is shown (A). An MRI
illustrated overgrowth and enhancement o the upper lip (B). Markings or subtotal resection
with a transverse mucosal resection are shown (C). T e patient is seen a er excision (D). She had
improved lip contour a er surgery (E).
Fig. 6-13
A

B

C
D

E

91
Chapter 6: Rare Vascular Tumors
e n z in g e r in t r a Mu s c u l a r h e Ma n g io Ma
T is 30-year-old man developed a lesion o his right lip and cheek. T e diagnosis was unclear
rom the history, physical examination, and imaging. Consequently, an incisional biopsy was
per ormed through an intraoral incision; histopathologic ndings were consistent with an En-
zinger intramuscular hemangioma. Because the lesion was large and di use, complete resection
and reconstruction would cause a worse de ormity than the tumor. T e patient was able to ade-
quately camouf age the skin discoloration with topical products and was not interested in surgical
intervention. T e patient is seen be ore incisional biopsy (A). T e lesion is shown on MRI (B).
COMPLICATIONS
Although vascular tumors, resection o these rare lesions is not associated with increased intra-
operative bleeding. I inject a local anesthetic containing epinephrine throughout the surgical
site to acilitate the procedure. In ection and wound dehiscence are uncommon. Recurrence
a er resection does not occur with KHEs, congenital hemangiomas, or in antile myo bromas.
Enzinger intramuscular hemangiomas and arteriovenous acral tumors can regrow a er excision.
CONCLUSION
Be ore a treatment plan is made or a rare vascular tumor, the lesion must be accurately diag-
nosed. A RICH regresses over the rst year o li e and may cause adipose hypoplasia that can be
improved with at gra ing. A NICH does not change and can be excised to improve the patient’s
appearance. A KHE is primarily treated with systemic pharmacotherapy; surgical intervention is
reserved or localized lesions or to improve a chronic de ormity. An in antile myo broma, En-
zinger intramuscular hemangioma, and arteriovenous acral tumor are usually excised to obtain
a histopathologic diagnosis and improve the patient’s appearance. Preoperative imaging is not
mandatory be ore resection o a vascular tumor, because most involve only the skin and subcu-
tis. However, a KHE should be evaluated by an MRI to determine whether deep structures are
involved, which may obviate resection.
A

B
Fig. 6-14

92
Part II: Vascular Tumors
SUGGESTED READINGS
Allen PW, Enzinger FM. Hemangioma o skeletal muscle: an analysis o cases. Cancer : , .
Croteau SE, Liang MG, Kozakewich HP, et al. Kaposi orm hemangioendothelioma: atypical eatures and risks o
Kasabach-Merritt phenomenon in re errals. J Pediatr : , .
Gupta A, Kozakewich H. Histopathology o vascular anomalies. Clin Plast Surg : , .
Jones EW, Orkin M. u ed angioma (angioblastoma): a benign progressive angioma, not to be con used with Kapo-
si’s sarcoma or low-grade angiosarcoma. J Am Acad Dermatol ( Pt ): , .
Merrell SC, Rahbar R, Alomari AI, et al. In antile myo broma or lymphatic mal ormation: di erential diagnosis o
neonatal cystic cervico acial lesions. J Cranio ac Surg : , .
Weiss SW, Goldblum JR. Benign tumors and tumor-like lesions o blood vessels. In Schmitt W, ed. Enzinger and
Weiss’s So issue umors, ed . St Louis: Mosby Elsevier, .

Par t III
Vas c u l ar Mal f o r mat io n s

(Trang không có chữ)

Arin K. Greene
95
A capillary mal ormation (CM) is the most common type o vascular mal ormation, a ecting
0.3% o the population. Recently the causative somatic mutation (GNAQ) has been identi ed.
Capillary mal ormations can cause overgrowth o tissues beneath the cutaneous stain. A child
with a CM involving the lower lip and chin is shown (A). Labial hypertrophy during adolescence
is seen (B). Continued enlargement in adulthood is shown (C).
Capil l ar y Mal f o r mat io n s
Ch a Pt er 7
KEY POINTS
• Pulsed-dye laser can reduce the color of capillary malformations.
• Capillary malformations may cause overgrowth of tissues beneath the stain.
• Pyogenic granulomas can develop in capillary malformations and are removed.
• Symptomatic overgrown tissues are managed by excision.
• Resection of a capillary malformation should not leave a worse deformity than the appear-
ance of the lesion.
Fig. 7-1
A

B

C

96
Part III: Vascular Malformations
A CM begins as a pink, cutaneous discoloration and then darkens to a purple color over time.
A child with a CM o the chest is shown (A). She underwent an unnecessary biopsy to diagnose
the lesion at an outside institution that resulted in an un avorable scar. T e diagnosis o a CM
is made by history and physical examination; imaging and histopathologic examination are not
required. An adolescent male with a darkened CM o the trunk is shown (B). Note the pyogenic
granuloma that has developed.
Clinically and histologically, CMs become more similar to venous mal ormations over time. CMs
can be a component o a syndrome and associated with underlying structural anomalies. T e pri-
mary morbidity o CM is psychosocial distress, because the lesion causes a visible de ormity. CMs
do not cause pain and are not at increased risk o in ection or bleeding. First-line management
o CMs is pulsed-dye laser, which e ectively lightens the lesion. Laser treatment in in ancy or
early childhood may yield better e cacy compared with later intervention. Despite pulsed-dye
laser, CMs typically redarken over time. Resection o a CM is much less common compared with
intervention with pulsed-dye laser.
SURGICAL INDICATIONS
Surgical management o CMs is not mandatory and is reserved or symptomatic lesions (see Video
7-1, Capillary Mal ormations). Resection and reconstruction should not leave a worse de ormity
than the appearance o the mal ormation. T e indications or surgical treatment o a CM are pyo-
genic granulomas, thickening and cobblestoning o the skin, and overgrowth o tissues beneath
the lesion. Bleeding pyogenic granulomas require resection. Asymptomatic pyogenic granulomas
are also removed, because they have a high risk o hemorrhage. T ickening o the integument and
hypertrophy o the subcutis, muscle, and bone beneath the CM typically do not begin until late
childhood. T e primary indication to debulk overgrown tissues is lowered sel -esteem. Patients
will request surgical intervention to improve their appearance. Rarely, osseous overgrowth can
cause malocclusion or a leg-length discrepancy. CMs do not have an increased risk o blood loss
during resection unless varicosities have developed. Because CMs are typically di use, complete
extirpation is rarely possible. Instead the goal o the procedure is usually to per orm a subtotal
resection to improve the patient’s symptoms.
A

B
Fig. 7-2

97
Chapter 7: Capillary Malformations
SURGICAL MANAGEMENT
t iMin g o f in t e r v e n t io n
A pyogenic granuloma requires intervention regardless o the age o the child. I possible, treat-
ment should be delayed until at least 6 months o age, because be ore this time the patient’s risk
o anesthesia is greater than or an adult. Overgrowth usually does not occur until adolescence,
and thus surgical procedures are rarely per ormed at younger ages. Patients typically present re-
questing improvement in their appearance or intervention to acilitate tting into clothes. Epiph-
ysiodesis o the distal emoral growth plate may be required around 11 years o age to correct a
leg-length discrepancy. Overgrowth o the maxilla/mandible can cause an occlusal cant and/or
malocclusion that may necessitate an orthognathic procedure a er the completion o skeletal
growth (16 years o age in girls and 18 years o age in boys).
A rare surgical indication in early childhood is to remove a problematic lesion on the scalp. Be-
cause the scalp has signi cant laxity during in ancy, resection o a CM may be considered be ore
6 months o age to acilitate the procedure. Although uncommon, i a young child already has
signi cant overgrowth o tissues o the ace that will require surgery to improve a de ormity, con-
sideration should be given to early resection. Because long-term memory and sel -esteem begin
to orm at approximately 4 years o age, debulking overgrown acial tissues be ore this time will
treat the de ormity be ore it causes psychosocial distress; the patient will also not remember the
procedure.
Pyo g e n ic g r a n u l o Ma
Because pyogenic granulomas involve the reticular dermis, interventions that do not treat the
entire depth o the skin have an approximately 50% recurrence rate ( or example, shave, cryo-
therapy, and super cial cautery). De nitive management is ull-thickness excision o the lesion.
I a pyogenic granuloma develops in a localized CM, the surgeon should consider removing the
entire CM to obviate the risk o uture pyogenic granulomas in the remaining CM. Small pyo-
genic granulomas can be managed with needlepoint cautery to coagulate the base o the lesion
through the dermis.
l iP o v e r g r o w t h
One hal to two thirds o patients with a CM o the ace will have so tissue hypertrophy; the
lip is the most commonly overgrown structure. Preoperative imaging is unnecessary. Vertical
lip resections should not be per ormed or CM, because the lesion is benign and the excision is
subtotal (margins are not required). Vertical scars on the cutaneous lip are avoided. T e contour
o the lip can be improved by partial resection with a transverse mucosal incision. T e procedure
may be per ormed in adults with a local anesthetic. T e area o the overgrown lip is outlined, and
the junction o the keratinized and nonkeratinized lip is tattooed. Next, an elliptical excision is
centered on either side o the keratinized/nonkeratinized junction. I inject the area with 0.25%
bupivacaine with epinephrine. As minimal a volume as possible is used to limit distortion o

98
Part III: Vascular Malformations
the lip and to reduce the amount o postoperative swelling that can cause increased brosis and
an enlarged lip. A small Beaver blade is used to resect the vermilion, mucosa, submucosa, and
a small amount o muscle. ooth show is used as a guide to determine how much tissue should
be resected. I overcorrect slightly to account or postoperative swelling and subsequent brosis,
which contribute to lip ullness. A er undermining the vermilion and mucosa, the wound is
closed with 5-0 Vicryl suture, ollowed by interrupted 6-0 chromic suture. Patients are maintained
on a ull-liquid diet or 2 weeks to prevent suture line dehiscence.
Occasionally an individual will have pre erential overgrowth o either the vermilion or mucosal
area o the lip. T e transverse elliptical resection can be adjusted to remove more o the vermilion
or mucosa as needed. Patients with severe hypertrophy may require a second-stage resection to
achieve the best outcome. Individuals who have overgrowth o the cutaneous aspect o the upper
or lower lip can have improvement with a mucocutaneous incision along the white roll. A skin
f ap is raised along the cutaneous lip, underlying subcutaneous tissue is removed, and excess skin
is trimmed.
c h e e k o v e r g r o w t h
Resection o a cheek CM is much less common than lip procedures. Surgical intervention or
a di use acial CM should ocus on improving the patient’s appearance with localized, staged
excisions without causing a signi cant de ormity. Diseased tissue should not be removed i there
is a risk o iatrogenic morbidity. It is critical to avoid acial nerve injury. Facial nerve dissections
or parotidectomy should not be per ormed. T e primary tissue that is overgrown with a CM is
the subcutis. When the lesion extensively involves the ace, strategies to improve the area include:
• Excision o excess skin and subcutaneous tissue
• Removal o the buccal at pad
• Contour burring o the zygoma
I the overgrowth is located on the lateral cheek, a preauricular incision is used. Medial ullness
is resected through a mesolabial incision. Patients o en lack a mesolabial crease, and placing a
scar in this location improves their appearance. Alternatively, a circular excision can be used to
access the overgrown tissue, and the purse-string closure can be revised at a second stage. An
end-stage CM with signi cant discoloration, skin thickening, and brovascular overgrowth may
bene t rom excision and reconstruction with a skin gra .
I generally do not use intraoral approaches to remove subcutaneous lesions. I pre er to make a
cutaneous incision and remove abnormal subcutaneous tissues sharply under direct vision. I
believe in the principle o trading scar or contour. Patients are most satis ed with achieving the
best symmetry, which is noticed beyond conversational distance at the expense o a scar that can
only be appreciated on close examination (similar to having a longer scar instead o a shorter
scar and dog-ears at each end). Intraoral excision is more di cult and less predictable than direct
cutaneous extirpation; there is also an increased risk o in ection and acial nerve injury.
I do not per orm preoperative imaging when resecting a acial CM. Blood loss is reduced by
in ltrating the surgical site with epinephrine. I the volume o local anesthetic with epinephrine
is limited by the amount o anesthetic that can be given based on the weight o the patient, I will
in ltrate an epinephrine-only solution to ensure maximum vasoconstriction o the surgical site
(1 ml o 1:1000 epinephrine in 200 ml normal saline = 1:200,000 solution).

99
Chapter 7: Capillary Malformations
t r u n k o v e r g r o w t h
CMs causing subcutaneous overgrowth o the trunk are generally well tolerated by patients. T e
trunk is concealed by clothing, and thus de ormities cause less psychosocial problems. In addition,
tting clothes is rarely a problem, because loose shirts can be worn. Options to improve trunk
overgrowth include resection with cutaneous incisions or liposuction. T e trunk is a avorable
area or serial excision o large skin lesions. I a patient is bothered by the color o a CM, it may
be able to be resected. Liposuction can remove overgrown subcutaneous tissue beneath the stain
i the overlying CM is too di use to remove or is causing a minimal de ormity.
e x t r e Mit y o v e r g r o w t h
Patients with a di use CM o an extremity can develop axial bone lengthening and circum er-
ential overgrowth o so tissues. T e lower extremity is a ected more requently than the upper
extremity. No intervention is necessary or vertical excess o the arms. In contrast, a leg-length
discrepancy may require orthopedic intervention. So tissue overgrowth o an extremity can cause
lowered sel -esteem because o asymmetry, as well as di culty tting clothing. Be ore considering
surgical intervention, I obtain an MRI to determine the relative overgrowth o the subcutis and
musculature. I the patient has signi cant subcutaneous adipose hypertrophy, he or she would
be predicted to have a avorable outcome a er liposuction. I the primary tissue that is enlarged
is the musculature, the individual would be expected to have less improvement a er removing
subcutaneous tissue. Most patients with an extremity CM have a di use lesion with overgrowth
o the entire limb. Less commonly, an individual will have a CM causing localized subcutaneous
hypertrophy.
Patients are counseled about the risks associated with liposuction, including bleeding, in ection,
skin breakdown, and pulmonary emboli. Liposuction does not cure the CM, and patients are
educated that they can have a recurrence o subcutaneous adipose. umescent solution (50 ml o
1% lidocaine, 1 ml o 1:1000 epinephrine, and 1000 ml o normal saline solution) is injected not to
exceed 35 mg/kg o lidocaine. T e tumescent or superwet technique is used so that the volume o
aspirate is equal to or less than the amount in used. Multiple 1 cm incisions are made along the
extremity in relaxed skin-tension lines and natural creases (5 to 10 or the arm and 10 to 15 or the
leg). Standard 3 to 5 mm cannulas are used or the upper extremity. Power-assisted liposuction
is typically incorporated or the lower extremity with 4 to 6 mm cannulas. Circum erential suc-
tioning is per ormed, and the incisions are closed loosely to allow drainage. T e limb is wrapped
with gauze and elastic bandages and is changed within 1 week. Immediately a er the procedure,
the patient is encouraged to use the limb as much as possible. A er the surgical bandages are
removed, patients wash the limb once a day and wrap the extremity with Ace wraps or 6 weeks
until the limb has reached a steady-state volume.
Patients undergoing upper extremity liposuction are discharged on the day o the procedure. De-
pending on the severity o lower extremity disease, procedures may be per ormed on an outpatient
basis, or patients may require inpatient recovery. I an individual has bilateral lower extremity
disease, only one leg is treated at a time to acilitate ambulation a er surgery. T e contralateral
extremity is reduced 3 to 6 months later.

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Part III: Vascular Malformations
PATIENT EXAMPLES
Pyo g e n ic g r a n u l o Ma
Pa tie n t A
T is in ant boy presented with a bleeding pyogenic granuloma in a localized CM. Instead o
removing only the pyogenic granuloma, the entire CM was excised to obviate the risk o uture
pyogenic granulomas developing in the lesion. T e age o the patient acilitated resection because
o scalp laxity. T e preoperative appearance is seen (A). T e patient is shown a er excision o the
CM and pyogenic granuloma (B).
Pa tie n t B
T is adolescent boy developed a pyogenic granuloma in a large CM o his trunk. Because excis-
ing the entire CM would take serial resections, only the pyogenic granuloma was removed. T is
patient is shown be ore (A) and a er (B) extirpation o the lesion.
A

B
Fig. 7-3
Fig. 7-4
A

B

101
Chapter 7: Capillary Malformations
l o w e r l iP o v e r g r o w t h
Pa tie n t A
T is man had a CM o the lower lip and chin that caused labial hypertrophy. T e right lower lip
underwent a subtotal resection with a transverse mucosal incision with local anesthesia. T e
preoperative appearance is seen (A). Markings or resection are shown (B and C). T e excised
specimen is seen (D). T e patient had improved lip contour a er surgery (E).
Pa tie n t B
T is woman requested improvement in the appearance o her lower lip. T e preoperative view is
seen (A). She had improved contour a er transverse mucosal resection o the le lower lip (B).
Fig. 7-5
A

B

C
D

E
A

B
Fig. 7-6

102
Part III: Vascular Malformations
u PPe r l iP o v e r g r o w t h
T is adolescent girl with a acial CM developed upper lip hypertrophy. T e lip was contoured with
a transverse mucosal resection. She is shown be ore (A) and a er (B) subtotal resection.
f a c ia l o v e r g r o w t h
Pa tie n t A
T is man with a di use acial CM complained o bleeding pyogenic granulomas o the cheek and
right brow/upper eyelid ptosis. He underwent resection o the pyogenic granulomas and a direct
orehead resection and browli . He is shown be ore surgery (A). Markings or excisions are seen
(B).T e patient is shown a er surgery (C).
A

B
Fig. 7-7
Fig. 7-8
A

B

C

103
Chapter 7: Capillary Malformations
Pa tie n t B
T is woman previously underwent resection and skin gra ing to her cheek, as well as le orbital
exenteration, at an outside institution. She complained o swelling o the nose and upper eyelid
and underwent localized resections o these areas. T e patient is shown be ore surgery (A). Intra-
operative markings illustrate excision margins (B). T e patient is seen a er surgery (C).
c h e e k o v e r g r o w t h
T is woman developed progressive overgrowth o her cheek and upper lip ptosis. She underwent
resection o the subcutaneous adipose hypertrophy with a mesolabial incision to re-create the
crease and allow direct access to the overgrown tissue. A skin resection was also per ormed to
reduce the area and to elevate the lip. T is patient is shown be ore surgery (A). Note the absence
o the mesolabial crease. Intraoperative markings o the incision are shown (B and C). Skin f aps
were elevated to directly access hypertrophied subcutaneous adipose tissue (D). T e patient is
seen a er wound closure (E).
Fig. 7-9
A

B

C
Fig. 7-10
A

B

C
D

E

104
Part III: Vascular Malformations
t r u n k o v e r g r o w t h
T is woman developed progressive overgrowth o her posterior trunk and complained o di culty
tting clothing. She underwent suction-assisted lipectomy to reduce the amount o subcutaneous
adipose tissue. T is patient is seen be ore surgery (A). An MRI illustrated signi cant adipose
excess (B). Intraoperative view (C) and processed lipoaspirate (D) are shown. She had improved
trunk contour a er surgery (E).
Fig. 7-11
E
A
B

C

D

105
Chapter 7: Capillary Malformations
l o c a l iz e D e x t r e Mit y o v e r g r o w t h
Pa tie n t A
T is adolescent girl presented with a CM o her ankle and oot that was inhibiting her ability to
wear shoes. She was managed with suction-assisted lipectomy o the localized area o overgrowth.
T e patient is seen be ore surgery (A). An MRI showed abnormal subcutaneous adipose hyper-
trophy (B). An intraoperative view is shown (C). Markings indicate the borders o resection.
Lipoaspirate is seen (D). She had improved contour a er surgery (E).
Pa tie n t B
T is adolescent boy with a di use CM o his leg developed localized overgrowth o his lateral
thigh that inter ered with his ability to t clothing. T e abnormal adipose tissue was removed with
liposuction. T is patient is seen be ore surgery (A). An MRI illustrated the adipose hypertrophy
(B). He had improved contour a er surgery (C).
Fig. 7-12
A

B

C

D

E
Fig. 7-13
A

B

C

106
Part III: Vascular Malformations
Dif f u s e e x t r e Mit y o v e r g r o w t h
T is teenage girl had a CM o her lower extremity that caused di use overgrowth and problems
tting clothing. She was managed with suction-assisted lipectomy. T e patient is shown be ore
surgery (A). An MRI showed excess subcutaneous adipose tissue o the le leg (B). T e intraop-
erative view (C) and harvested lipoaspirate (D) are seen. T e patient is shown a er surgery (E).
Fig. 7-14
A
B

C
D

E

107
Chapter 7: Capillary Malformations
COMPLICATIONS
Complications a er resection o a CM are less common compared with extirpation o other types
o vascular mal ormations. In ection and bleeding are rare. Wound dehiscence can occur, and
the area is allowed to heal secondarily. Postoperatively the surgical site should have a pressure
dressing. When a subtotal resection is per ormed that requires the approximation o ischemic
tissues containing a CM, interrupted sutures are used and le in place until the wound has gained
strength. Interrupted sutures are less ischemic than a continuous suture and reduce the risk o
dehiscence. Perioperative antibiotics are not required unless the resection is in a contaminated
location. Wounds should be closed with local tissues; rarely a skin gra is required. Patients and
amilies are counseled that a CM progresses a er subtotal resection, and urther treatment may
be needed in the uture.
CONCLUSION
T e surgical management o a CM is much less common than lightening the lesion with pulsed-
dye laser. Resection o a CM is indicated to treat pyogenic granulomas, improve skin thickening
and brovascular overgrowth, or remove subcutaneous adipose hypertrophy. Most CMs are di -
use and cannot be completely excised. T e goal o surgical intervention is usually to per orm
localized procedures to improve the patient’s appearance and unction. Subcutaneous adipose
overgrowth can be managed with suction-assisted lipectomy. Radical extirpation and complex
reconstruction should be avoided. T e de ormity caused by excision and reconstruction should
not be worse than the appearance o the CM. Patients with a problematic CM are best managed
in an interdisciplinary vascular anomalies center ocused on this lesion.
SUGGESTED READINGS
Greene AK. Capillary mal ormation. In Greene AK, ed. Vascular Anomalies: Classi cation, Diagnosis, and Manage-
ment. St Louis: Quality Medical Publishing, .
Greene AK, Liu AS, Mulliken JB, et al. Vascular anomalies in patients: guidelines or re erral. J Pediatr Surg
: , .
Greene AK, aber SF, Ball KL, et al. Sturge-Weber syndrome: so -tissue and skeletal overgrowth. J Cranio ac Surg
: , .
Gupta A, Kozakewich H. Histopathology o vascular anomalies. Clin Plast Surg : , .
Huikeshoven M, Koster PH, de Borgie CA, et al. Redarkening o port-wine stains years a er pulsed-dye-laser
treatment. N Engl J Med : , .
Maguiness SM, Liang MG. Management o capillary mal ormations. Clin Plast Surg : , .
Shirley MD, ang H, Gallione CJ, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation
in GNAQ. N Engl J Med : , .
an O , Sherwood K, Gilchrest BA. reatment o children with port-wine stains using the f ashlamp-pulsed tunable
dye laser. N Engl J Med : , .

(Trang không có chữ)

109
Arin K. Greene
A lymphatic mal ormation (LM) results rom the abnormal development o the lymph system. T e
primary morbidity is psychosocial distress, because the lesion causes a de ormity. LMs may also
bleed, become in ected, leak lymph f uid (lymphorrhea), and obstruct/destroy important struc-
tures. T ere are several phenotypes o LMs; many have a known causative mutation. Although
LMs are present at birth, they may not be noted until childhood or adolescence. Lesions worsen
particularly during puberty.
Lymph at ic Mal f o r mat io n s
Ch a Pt er 8
KEY POINTS
• Macrocystic lymphatic malformations are treated with sclerotherapy.
• Lymphatic malformations are not malignant and do not require radical resection for a cure.
• Excision of lymphatic malformations is reserved for symptomatic macrocystic lesions after
sclerotherapy or for microcystic lymphatic malformations.
• Treatment options for microcystic lymphatic malformations include resection, radiofrequency
ablation, carbon dioxide laser, bleomycin injection, and oral sirolimus.
• Resection of a lymphatic malformation should not leave a worse deformity than the appear-
ance of the lesion.

110
Part III: Vascular Malformations
T e management o LMs is based on the type o lesion. Macrocystic LMs have cysts greater than
or equal to 5 mm that are large enough to be cannulated by a needle and treated with sclerother-
apy. Sclerotherapy is the injection o a sclerosant ( or example, doxycycline or ethanol) into a cyst,
which causes irritation, shrinkage, and brosis o the lesion. Microcystic LMs with cysts less than
5 mm are typically not amenable to sclerotherapy. Microcystic lesions are managed with resec-
tion, bleomycin injection, carbon dioxide laser, radio requency ablation, and/or oral sirolimus.
Combined LMs containing both macrocysts and microcysts can be treated with sclerotherapy.
T e cystic composition o LMs is determined by ultrasound examination. Primary lymphedema is
managed with compression (custom- tted garments, a pneumatic pump, and complex deconges-
tive therapy); surgical intervention is reserved or severe overgrowth. Gorham-Stout disease and
a generalized lymphatic anomaly a ecting bone are treated with inter eron and bisphosphonates;
sirolimus also may reduce bone destruction and promote remineralization.
SURGICAL INDICATIONS
T e treatment o LMs is not mandatory. Intervention is reserved or lesions that lower sel -esteem,
cause pain, bleed, or obstruct/destroy tissues. Resection and reconstruction should not leave a
de ormity worse than the LM. T e primary variable that determines whether a lesion is resected
is the type o LM; some can only be managed with excision, whereas others should be treated
with sclerotherapy, carbon dioxide laser, radio requency ablation, or pharmacotherapy. Small,
localized LMs are more amenable to resection, because they can potentially be removed or a cure.
Table 8-1 Phenotypes o Lymphatic Mal ormations
Lesion
Mutation
Treatment Options
Macrocystic

PIK3CA
Sclerotherapy (generally first-line)
Resection (if localized or after sclerotherapy)
Microcystic

PIK3CA
Resection
Bleomycin injection
Carbon dioxide laser
Radiofrequency ablation
Cutaneous cautery/sclerotherapy
Sirolimus
Combined (macrocystic and
microcystic)
PIK3CA
Sclerotherapy (generally first-line)
Resection
Bleomycin injection
Carbon dioxide laser
Radiofrequency ablation
Cutaneous cautery/sclerotherapy
Sirolimus
Primary lymphedema

VEGFR3, FOXC2, SOX18,
CCBE1, KIF11, PTPN14,
GJC2/connexin 47, GATA2
Compression (first-line)
Resection
Gorham-Stout disease
Generalized lymphatic anomaly
Interferon and bisphosphonate
Sirolimus
CCBE1, Collagen and calcium-binding EGF domain 1; FOXC2, forkhead box protein C2; GATA2, GATA binding protein 2; GJC2/connexin 47, gap
junction gamma-2/connexin 47; KIF11, kinesin family member 11; PIK3CA, phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha;
PTPN14, protein tyrosine phosphatase, nonreceptor type 14; SOX18, sex-determining region Y box 18; VEGFR3, vascular endothelial growth factor
receptor 3.

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Chapter 8: Lymphatic Malformations
Ma c r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n
Macrocystic LMs usually are managed best with sclerotherapy, because the treatment is more
e ective and sa er than excision. T is emale was born with a large macrocystic LM o her right
ace and neck. She underwent resection o the lesion at an outside institution and su ered a acial
nerve injury. Her appearance is shown at rest (A). An asymmetrical smile resulting rom injury
to her right marginal mandibular branch o the acial nerve is shown (B).
Macrocystic and combined LMs are managed with sclerotherapy, because this technique is sa er
and more e ective than resection. Asymptomatic macrocystic lesions should generally be treated,
because they are at risk o bleeding and in ection. I a macrocyst has intralesional bleeding, the
LM can be converted to microcysts, which are no longer amenable to sclerotherapy. Although
sclerotherapy does not remove an LM, it e ectively shrinks the lesion and improves symptoms.
Resection o a macrocystic LM is reserved or:
• Small lesions that may be completely removed or cure
• Symptomatic LMs that can no longer be managed with sclerotherapy (all o the macrocysts
have been treated)
Mic r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n
Asymptomatic microcystic LMs may be observed; intervention is reserved or problematic le-
sions. Generally rst-line therapy is resection. Excision usually is subtotal, because microcystic
LMs are o en di use and involve multiple tissue planes. I a microcystic LM is in an un avorable
location or resection, other interventions are considered. Di use acial lesions may be improved
by injecting bleomycin throughout the a ected tissues. Extensive lesions o the skin can be man-
aged with a carbon dioxide laser, cutaneous sclerotherapy, or cautery. Intraoral microcystic LMs
may be improved with radio requency ablation. Recently oral sirolimus has shown e cacy or
problematic LMs.
Pr iMa r y l y MPh e De Ma
Primary lymphedema is caused by hypoplastic lymphatic development. High-protein f uid is
unable to be returned to the venous circulation, and pitting edema develops. Over time the f uid
stimulates subcutaneous adipose deposition, which causes the area to enlarge. Ninety- ve percent
o patients managed in our lymphedema program can be treated conservatively with compression.
Surgical intervention is reserved or individuals with subcutaneous adipose deposition that is
causing lowered sel -esteem, in ections, and/or di culty with the extremity.
Fig. 8-1
B
A

112
Part III: Vascular Malformations
SURGICAL MANAGEMENT
t iMin g o f in t e r v e n t io n
LMs causing signi cant symptoms require intervention, regardless o the age o the child. I
possible, treatment should be delayed until at least 6 months o age, because be ore this time the
patient’s risk rom anesthesia is greater than in an adult. In addition, a young in ant is less able to
tolerate a surgical procedure. I a large lesion is located on the scalp, removing it be ore 6 months
o age should be considered to take advantage o scalp laxity that exists during in ancy. Large,
asymptomatic macrocystic LMs are treated prophylactically at 6 months o age, be ore the lesion
bleeds or becomes in ected, which would obviate uture sclerotherapy.
I it is likely that a patient will require surgery to improve a de ormity, I will commonly intervene
between 3 and 4 years o age. Because long-term memory and sel -esteem begin to orm at approx-
imately 4 years o age, removing an LM be ore this time will treat the de ormity be ore it causes
psychosocial distress; the patient will also likely not remember the procedure. Another period
to intervene is during late childhood or early adolescence when the child is able to communicate
whether he or she would like to have a procedure. I a patient has a minor de ormity or a large
lesion requiring signi cant reconstruction, the surgeon should wait until the child verbalizes the
desire to have surgery. I the lesion is minor, it may be able to be removed with a local anesthetic
by waiting until the patient is older. I the LM is signi cant, waiting until the child is a willing
participant acilitates the process or the amily and surgeon.
Ma c r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n
Generally macrocystic/combined LMs should undergo sclerotherapy be ore resection because:
• T e lesion will be smaller.
• Excision is acilitated, because cysts are converted to brotic tissue.
• Peripheral vessels are permeated by the sclerosant, which theoretically reduces the recur-
rence rate.
Small lesions that can potentially be removed or a cure do not require preoperative sclerother-
apy. Generally sclerotherapy is per ormed at 6-week intervals until the posttreatment ultrasound
examination does not show that the remaining cysts are amenable to urther injections.
Pure macrocystic LMs are most commonly located in the neck and axilla. T ese lesions can leave
behind redundant skin a er they have been def ated with sclerotherapy. In the axilla overresection
o skin should be avoided to prevent a contracture. Large LMs can also have residual symptomatic
microcystic disease a er sclerotherapy, which may necessitate excision.
Most LMs are combined lesions containing both macrocysts and microcysts. T ese lesions should
initially be managed with sclerotherapy to collapse the macrocysts. Usually the patient’s symp-
toms are adequately improved and resection is unnecessary. I the individual continues to have
morbidity a er sclerotherapy, extirpation o the area is acilitated.

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Chapter 8: Lymphatic Malformations
Mic r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n
Microcystic LMs are more di cult to manage compared with macrocystic lesions. Microcystic
LMs usually involve the ace and extremities. I the disease a ects the integument, a patient may
develop lymphatic vesicles that can bleed, become in ected, and cause lymphorrhea. T e best
approach is to resect localized areas o skin involvement, which can recur along the scar. Larger
regions o integument can be removed with serial excision. Di use lymphatic vesicles that are
problematic to excise can be treated with a carbon dioxide laser. Alternatively, cutaneous vesicles
can be managed with super cial sclerotherapy or cauterization. Intraoral lymphatic vesicles are
best treated by radio requency ablation. T is technique causes low-temperature tissue destruc-
tion, which reduces damage to adjacent structures. Compared with a carbon dioxide laser, there
is less edema, which is particularly avorable in the oral cavity, because it minimizes the risk o
airway obstruction.
Recent options to treat microcystic LMs are bleomycin sclerotherapy and oral sirolimus. Bleomy-
cin can be di usely injected throughout the tissues containing LM, and most patients will have a
reduction in the size o the area and improvement in symptoms. Bleomycin is use ul or patients
with mild acial asymmetry, because the treatment may obviate the need or resection and a cu-
taneous scar. Individuals with di use, problematic microcystic LMs that have not responded to
other interventions are candidates or sirolimus pharmacotherapy. Our center and others have had
avorable outcomes with this medication; lesions have reduced size, bleeding, and lymphorrhea.
A large area o integument that has ailed conservative therapies requires resection. Wounds are
reconstructed by allowing them to heal secondarily, mobilization o local tissues, or skin gra s.
issue expansion, regional muscle f aps, or ree f aps are usually unnecessary. A recipient site
containing a residual LM is more likely to accept a skin gra compared with a venous or an ar-
teriovenous mal ormation. An underlying LM usually does not recur through the skin gra but
may develop vesicles along the edges.
Localized LMs do not require preoperative imaging i only resection is planned. I pre er to obtain
an MRI be ore I per orm a resection o a large lesion to determine which surgical planes and
anatomic structures are involved. Excision o an LM is associated with less bleeding compared
with a venous or arteriovenous mal ormation. Blood loss is reduced by preoperative sclerotherapy
and in ltrating the surgical area with epinephrine. I the volume o local anesthetic with epineph-
rine is limited by the amount o anesthetic that can be given based on the weight o the child, I
will in ltrate an epinephrine-only solution to ensure maximum vasoconstriction o the surgical
site (1 ml o 1:1000 epinephrine in 200 ml normal saline = 1:200,000 solution). T e extirpation
proceeds care ully by incising a localized area and controlling bleeding be ore continuing. Long,
deep incisions should not be per ormed because o the risk o signi cant blood loss. None o the
patients with an LM on whom I have operated has required a blood trans usion. I administer
prophylactic intraoperative antibiotics to reduce the risk o postoperative in ection, because LMs
are predisposed to in ection. I wide skin undermining is per ormed, drains should be placed,
because tissues have a higher risk o lymph drainage.
Lesions located in anatomically sensitive areas ( or example, the ace) should have minimal mar-
gins included in the resection. An LM is not a malignancy, and evidence does not show that a
wide margin lowers the recurrence rate. An LM o en involves a larger area than is appreciated
clinically and radiographically. Intraoperatively, subcutaneous tissues can be cauterized at the

114
Part III: Vascular Malformations
periphery o the LM without removing skin. Cautery and brosis may destroy a residual LM and
reduce recurrence. I a lesion is located in a nonsensitive area ( or example, the abdomen), larger
margins can be taken as long as they do not complicate the extirpation and reconstruction.
Because most LMs are di use and involve multiple tissue planes, complete extirpation is rarely
possible. Instead the goal is usually to alleviate symptoms and control the lesion. Despite subtotal
and presumed “complete” extirpation, most LMs reenlarge. Patients and amilies are counseled
that an LM is likely to recur a er resection, and treatment may be needed in the uture.
Surgical intervention or a di use acial LM should ocus on improving the patient’s appearance
or symptoms with localized, staged procedures without causing a signi cant de ormity. Diseased
tissue should not be removed i there is a risk o iatrogenic morbidity. Facial nerve dissections or
parotidectomy generally should be avoided. When the lesion is throughout all structures o the
ace, strategies to improve the area include:
• Excision o excess skin and subcutaneous tissue
• Removal o the buccal at pad
• Contour burring o the zygoma
I overgrowth is located on the lateral cheek, a preauricular incision is used. Medial ullness is
resected through a mesolabial incision. Alternatively, a circular excision can be used to access the
overgrown tissue, and the purse-string closure can be revised at a second stage. Lip overgrowth is
improved with a transverse mucosal resection along the keratinized and nonkeratinized border
o the vermilion. Vertical resections o the lip should be avoided.
I typically do not use intraoral approaches to remove subcutaneous lesions. I pre er to make a
cutaneous incision and remove abnormal subcutaneous tissues sharply under direct vision. I
believe in the principle o trading scar or contour. Patients are most satis ed with achieving the
best symmetry, which is noticed beyond conversational distance at the expense o a scar that can
only be appreciated on close examination (similar to having a longer scar instead o a shorter
scar and dog-ears at each end). Intraoral excision is more di cult and less predictable than direct
cutaneous extirpation; there also is an increased risk o in ection and acial nerve injury.
l y MPh e De Ma
T ere are two categories o procedures to treat lymphedema. Excisional procedures remove
subcutaneous broadipose tissue; the two most common types are suction-assisted lipectomy
(liposuction) and staged skin/subcutaneous excision. Physiologic surgeries attempt to improve
lymphatic f ow most o en by lymphaticovenous anastomosis or vascularized lymph node trans er.
My pre erred surgical intervention or lymphedema is liposuction, because I think the technique
gives the most consistent improvement and has minimal morbidity (see Video 8-1, Lymphatic
Mal ormation).
Physiologic procedures do not predictably restore lymphatic f ow and ail to remove excess sub-
cutaneous adipose tissue. Lymphaticovenous anastomosis and vascularized lymph node trans er
may have bene ts or patients with early secondary lymphedema rom axillary or inguinal lymph-
adenectomy/radiation (be ore broadipose tissue has developed). However, scarring rom a phys-
iologic surgery may worsen lymphedema. Physiologic procedures are probably contraindicated
in patients with primary disease, because they have absent or hypoplastic lymphatics. Patients

115
Chapter 8: Lymphatic Malformations
undergoing vascularized lymph node trans er may also develop donor-site lymphedema at the site
where the lymph nodes are harvested. T is risk is higher in patients with primary lymphedema
who have an underlying anomaly o their lymphatic system.
Symptomatic patients who have ailed conservative therapy undergo lymphoscintigraphy and an
MRI be ore proceeding with suction-assisted lipectomy. Lymphoscintigraphy con rms the diag-
nosis o lymphedema and determines the severity o lymphatic dys unction. An MRI documents
subcutaneous broadipose overgrowth. Imaging o both extremities is necessary to compare the
diseased limb with the contralateral side. I an MRI shows that the arm or leg is primarily en-
larged because o f uid, the patient is not a candidate or the excisional procedure, and his or her
compression regimen is maximized. It typically takes at least 6 years a er the onset o edema or
su cient subcutaneous adipose tissue to be ormed. I an MRI illustrates signi cant subcutaneous
adipose tissue, the patient may have improvement with liposuction.
Individuals are counseled about risks o the procedure, including bleeding, in ection, skin break-
down, and pulmonary emboli. Liposuction does not cure lymphedema, and patients are educated
that they must continue their compression regimen a er surgery to slow recurrence. umescent
solution (50 ml o 1% lidocaine, 1 ml o 1:1000 epinephrine, and 1000 ml o normal saline solution)
is injected into the subcutaneous compartment not to exceed 35 mg/kg o lidocaine. umescent
or superwet technique is used, so that the volume o aspirate is equal to or less than the amount
in used. Multiple 1 cm incisions are made along the extremity in relaxed skin-tension lines and
natural creases (5 to 10 or the arm and 10 to 15 or the leg). Standard liposuction is used or the
upper extremity with 3 to 5 mm cannulas. Power-assisted liposuction is typically incorporated or
the lower extremity with 4 to 6 mm cannulas. Circum erential suctioning is per ormed, and the
incisions are closed loosely to allow drainage. T e limb is wrapped with gauze and elastic bandages
and is changed within 1 week. Patients undergoing upper extremity liposuction are discharged
on the day o the procedure. Depending on the severity o the lower extremity, procedures may
be per ormed on an outpatient basis or patients may require inpatient recovery.
I a patient has bilateral lower extremity disease, only one leg is treated at a time to allow the
individual to ambulate a er surgery. T e contralateral extremity is reduced 3 to 6 months later.
Immediately a er the procedure, the patient is encouraged to use the limb as much as possible
and continue pneumatic compression or 1 to 2 hours a day. A er the operative bandages are re-
moved, patients wash the limb once a day and wrap the extremity with Ace wraps or 6 weeks until
the limb has reached a steady-state volume. T e individual is then tted or a new custom- tted
garment.
Rarely, patients with primary lymphedema o the lower extremity and extreme overgrowth are
managed best with staged skin/subcutaneous because:
• Severe brosis o the subcutaneous tissue is less amenable to liposuction.
• Signi cant skin excess requires resection.
ypically, a medial incision is made, skin f aps are raised one third the circum erence o the ex-
tremity, and subcutaneous tissue, muscle ascia, and redundant skin are removed. Several months
later, the procedure is repeated with a lateral incision. A er staged skin/subcutaneous excision,
patients can have signi cant improvement in the extremity unction, but the procedure is more
di cult and associated with greater morbidity compared with liposuction. Penile/scrotal lymph-
edema is not amenable to liposuction and requires staged skin/subcutaneous resection.

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Part III: Vascular Malformations
PATIENT EXAMPLES
Ma c r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : r e s e c t io n a f t e r
s c l e r o t h e r a Py
Pa tie n t A
T is child was born with a large macrocystic LM o the ace and neck. Resection o the lesion
would place the child at signi cant risk or surgical morbidity, poor aesthetic outcome, and un a-
vorable scars. T e child was treated with sclerotherapy. A er collapse o the cysts, the expanded
integument contracted but le redundant skin. He underwent resection o the excess tissue with
a preauricular and postauricular incision. Because the LM was initially treated with sclerotherapy,
less skin required excision, and the area was improved with well-hidden incisions around the ear.
T e appearance in in ancy is shown (A). T is patient had redundant tissue a er collapse o the
cysts rom sclerotherapy (B). T e patient is shown a er removing the extra skin (C).
Fig. 8-2
A
B

C

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Chapter 8: Lymphatic Malformations
Pa tie n t B
T is child was born with macrocystic LMs o the neck and axilla. A er treatment with sclerother-
apy, the skin o the neck did not completely contract, and he was le with a de ormity. Because
he had webbing o his anterior and lateral neck, the incision was placed over the lateral band to
avoid a scar on the ront o his neck. A er resection o the lateral band, the anterior neck was
undermined and tightened, and excess skin was excised. T e childhood appearance is shown (A).
Residual skin excess and webbing o the anterior and lateral neck can be seen a er sclerotherapy
(B and C). T is patient had improved contour a er resection o redundant skin through an inci-
sion placed over the lateral neck band (D and E).
Fig. 8-3
A
B

C
D

E

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Part III: Vascular Malformations
Ma c r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : r e s e c t io n w it h o u t
s c l e r o t h e r a Py
T is 22-month-old child was diagnosed with a macrocystic LM o the upper extremity. T e lesion
was resected rather than treated with sclerotherapy, because it was localized and thus amenable
to complete resection and cure. In addition, the skin was scarred and discolored by the lesion,
which could only be improved by resecting the damaged integument. T e preoperative appearance
o the macrocystic LM is seen (A). Ultrasonography showed macrocysts (B). T e markings or
resection (C) and the intraoperative view o the cyst (D) are shown. T is patient is shown a er
resection (E) and closure o the incision (F).
Fig. 8-4
A

B
C

D
E

F

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Chapter 8: Lymphatic Malformations
Ma c r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : r e c o n s t r u c t io n a f t e r
s c l e r o t h e r a Py
T is woman underwent resection o a macrocystic axillary LM at an outside institution. She
developed a contracture and chronic wound. T e area was reconstructed with scar release,
Z-plasties, and gra ing. T e axillary band and wound are seen (A). She is shown a er scar re-
lease and Z-plasties (B). A split-thickness skin gra is placed into the axilla (C). T e healed gra
and release o the contracture are shown (D).
Mic r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : c a u t e r y
T is adolescent girl had a di use microcystic LM o her le leg. Multiple cutaneous vesicles were
bleeding. T e symptomatic areas were treated with electrocautery. T e preoperative appearance
is seen (A). She is shown a er cauterization (B).
Fig. 8-5
A

B

C

D
Fig. 8-6
A

B

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Part III: Vascular Malformations
Mic r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : c a r b o n Dio x iDe l a s e r
T is adolescent girl had a large area o bleeding and leaking vesicles o her lower extremity. Re-
section would require serial excision or skin gra ing. Her condition was managed with a carbon
dioxide laser. She is shown be ore treatment (A) and immediately a er laser treatment (B). A er
therapy the vesicles were replaced by brosis (C).
Mic r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : c o MPl e t e r e s e c t io n
Pa tie n t A
T is patient developed bleeding rom a super cial, cutaneous LM. Because the area was well lo-
calized and caused a de ormity, it was excised with minimal margins. T is patient is shown be ore
surgery (A) and a er excision (B).
Fig. 8-8
A

B
Fig. 8-7
A

B

C

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Chapter 8: Lymphatic Malformations
Pa tie n t B
T is child developed pain in a localized microcystic LM o the lower extremity. Because o the size
and location o the lesion, it required serial excision or removal. T e appearance o the lesion is
seen (A). T e outline o the rst-stage excision (B) and markings or the second-stage resection
(C) are shown. T e appearance is shown 2 years a er the second-stage procedure (D). T e child
was placed into a knee immobilizer or 2 weeks postoperatively a er each procedure.
Fig. 8-9
A

B
C

D

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Part III: Vascular Malformations
Pa tie n t C
T is child presented with an enlarging LM that had been present since birth. She was unhappy
with the appearance o the lesion. Because the LM was located on her ace, it was resected with
a circular excision and purse-string closure to limit the length o the scar. T e preoperative ap-
pearance is shown (A). T e outer marking delineates the extent o the subcutaneous component
o the lesion (B). T e inner outline is the cutaneous excision. T e entire lesion was removed with
a circular incision (C). T is patient is seen a er purse-string closure o the wound (D). One year
a er surgery, the patient was pleased with the appearance o the circular scar and did not want
to undergo a second-stage procedure to convert it into a line (E).
Pa tie n t D
T is child developed pain in his le lower extremity and was diagnosed with a primarily mi-
crocystic LM. Because the area was symptomatic and localized, it was excised or possible cure.
T e preoperative appearance (A) and outline o the subcutaneous lesion (B) are shown. An MRI
showed the extent o the LM (C). T is patient is seen a er resection (D).
Fig. 8-10
C

D
A

B
E
Fig. 8-11
A

B

C

D

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Chapter 8: Lymphatic Malformations
Mic r o c ys t ic l y MPh a t ic Ma l f o r Ma t io n : s u b t o t a l r e s e c t io n
Pa tie n t A
T is adolescent boy presented with progressive enlargement o his le cheek. He was previously
diagnosed with an LM and had undergone resection at another institution through a medial
cheek incision. T is patient’s preoperative appearance is shown (A and B). An MRI showed the
LM (C). T e markings o the extent o the lesion and the medial cheek incision incorporating the
scar rom his previous surgery are seen (D). T e excised specimen is seen (E). T e cheek is shown
a er resection and closure o the incision (F).
Fig. 8-12
C

D
A

B
E

F

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Part III: Vascular Malformations
Pa tie n t B
T is adolescent boy had an LM on the right side o the ace that caused a de ormity and psycho-
social distress. He was treated with bleomycin sclerotherapy and had minimal improvement. Be-
cause the LM was primarily in the subcutaneous plane, a cutaneous excision was per ormed that
would also elevate his right upper lip. T e preoperative appearance is seen (A). An MRI showed
a microcystic LM o the right side o the ace (B). T e intraoperative markings are shown (C).
T e outer line delineates the extent o the lesion. A circular skin excision was planned along the
mesolabial crease. Subcutaneous tissue was removed through the circular incision (D).
Fig. 8-13, A-D
C

D
A

B

125
Chapter 8: Lymphatic Malformations
T e wound was closed with a purse-string closure (E), and excised tissue is seen (F). T e post-
operative appearance is shown (G and H). T e patient underwent a second stage to convert the
scar into a line and to li and excise additional tissue rom the lip (I). T e marking outlines the
subcutaneous LM that was excised. T e early postoperative view a er the second-stage resection
is shown (J).
Fig. 8-13, E-J
G

H
E

F
I

J

126
Part III: Vascular Malformations
Pa tie n t C
T is 3-year-old boy had a microcystic LM o the right side o the ace. o improve the symmetry,
he underwent buccal at pad resection o his right cheek. T is intervention avoided the placement
o a cutaneous incision and removed tissue in the most prominent overgrown area o his ace. T e
preoperative appearance is shown (A and B). An MRI illustrated the LM (C). T e intraoperative
view o the buccal at pad (D) and the excised specimen (E) are seen. Improvement in symmetry
is visible 4 months a er surgery (F and G).
Fig. 8-14
C

D
A

B
F

G
E

127
Chapter 8: Lymphatic Malformations
Pa tie n t D
T is 3-year-old boy had overgrowth o his right upper lip rom a microcystic LM. T e asymmetry
was improved be ore his sel -esteem and long-term memory began to orm. T e preoperative
view is shown (A). An MRI delineated the extent o the lesion (B). T e intraoperative markings
o a transverse mucosal resection are shown (C). T is patient is seen a er excision (D). T e post-
operative appearance is seen (E).
Fig. 8-15
C

D
A

B
E

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Part III: Vascular Malformations
Pa tie n t E
T is adolescent girl presented with an enlarging lesion o her lower lip, causing psychosocial
morbidity. o improve her appearance, she underwent a subtotal resection through a transverse
mucosal incision. Her preoperative appearance is seen (A). T e outline o a transverse mucosal
resection is shown (B). T e junction o the keratinized and nonkeratinized border was tattooed
and is the center o the excision margins. T e outer line represents the mucosa and vermilion that
was resected. T e incision was closed with interrupted 5-0 Vicryl sutures ollowed by interrupted
6-0 chromic sutures. T e patient was given dexamethasone (Decadron) intraoperatively to help
reduce postoperative swelling and brosis that may add ullness to the lip. o prevent dehiscence,
the patient was maintained on a ull-liquid diet or 2 weeks. T e suture line at the end o the pro-
cedure is shown (C). T e postoperative appearance is seen (D).
Fig. 8-16
C

D
A

B

129
Chapter 8: Lymphatic Malformations
Pa tie n t F
T is young boy was born with an LM o the chin, causing a de ormity. T e resection was per-
ormed at 3 years o age be ore his sel -esteem and long-term memory developed. o access the
entire area with as minimal a scar as possible, a circular excision was centered underneath his
chin. T e skin then was undermined and subcutaneous disease was removed. T e preoperative
view o the chin is seen (A). An MRI showed a microcystic LM o the chin (B). T e circular skin
excision allowed access to a wide area o subcutaneous disease (C). Purse-string closure o the
wound is seen (D). T e postoperative view o the scar is shown (E). T e patient had improved
chin contour a er the procedure (F).
Fig. 8-17
C

D
A

B
E

F

130
Part III: Vascular Malformations
Pa tie n t G
T is child had an enlarging area o his le medial thigh that inhibited his ability to t into cloth-
ing. An MRI showed a microcystic LM primarily located in the subcutaneous tissue. T e pre-
operative view is seen (A). An MRI delineated the lesion (B). T e patient had improved contour
a er resection (C).
Pa tie n t H
T is adolescent girl was born with a di use microcystic LM o her right lower extremity. T e
cutaneous component progressively worsened, and she developed a chronic wound. Despite non-
surgical management, the wound ailed to heal. o close the area, the ulcer and phalanges were
debrided to the metatarsal heads. Dorsal and plantar f aps were closed with interrupted 2-0 Ethi-
bond sutures. T e preoperative appearance o the wound is shown (A and B). T e intraoperative
view a er debridement is seen (C). T is patient is shown a er closure o the so tissue f aps (D).
T e healed wound is shown (E and F).
Fig. 8-18
A

B

C
Fig. 8-19
A

B

C

D
E

F

131
Chapter 8: Lymphatic Malformations
Pa tie n t I
T is young boy had a di use LM o his buttock, causing pain, bleeding, and lymphorrhea. He
underwent previous resections at an outside hospital with a recurrence. He also ailed carbon
dioxide laser treatment. Because previous incisions complicated the use o local skin f aps, the
LM was resected, and the wound was allowed to heal secondarily or maximum contraction.
T e preoperative appearance is seen (A). T e area is shown a er excision (B). A er rst-stage
resection the patient’s symptoms signi cantly improved (C). He developed recurrent bleeding
and underwent another resection o the area (D). T e periphery o the wound was closed linearly
and the center healed secondarily. T e appearance o the area is shown 6 weeks a er surgery (E).
Fig. 8-20
B

C
A
D

E

132
Part III: Vascular Malformations
Pa tie n t J
T is young boy was born with a di use LM o the lower extremity. Cutaneous vesicles were
causing bleeding and lymphorrhea. He underwent wide resection and reconstruction o the area
with a skin gra . A split-thickness gra was chosen to maximize contraction o the wound and
to increase the likelihood o take. Although the gra was placed on a recipient site that contained
the LM, it was success ully revascularized. T ere has not been a recurrence o vesicles through
the gra 7 years a er surgery. T e preoperative appearance is shown (A). T e wound is shown
a er resection o the diseased integument (B). T e split-thickness skin gra is seen a er inset
(C). T e healed gra is seen (D).
Fig. 8-21
A

B
C

D