Phẫu Thuật Quản Lý Và Điều Trị Dị Thường Mạch Máu

34
Part II: Vascular Tumors
Pa tie n t B
T e ulcerated posterior scalp hemangioma o this patient was dif cult to manage with dressing
changes, because the in ant was laying on the tumor. T e lesion would ultimately require resec-
tion a er it had involuted but was removed during the proli erative phase to improve the patient’s
quality o li e and to take advantage o avorable scalp laxity that acilitated the procedure. T e
ulcerated tumor (A), as well as the wound a er extirpation (B), are seen. T e postoperative ap-
pearance is shown be ore suture removal (C).
Pa tie n t C
T is 3-month-old in ant presented with an enlarging retroauricular lesion. T e lesion was re-
moved during the proli erating phase, because:
• Further growth could have caused a prominent ear de ormity, necessitating otoplasty.
• T e lesion would require resection in the uture or residual bro atty tissue, excess skin,
and alopecia (even i it was treated with pharmacotherapy).
T e lesion is shown be ore resection (A through C), and the wound is shown a er excision (D).
T e retroauricular suture line is seen (E and F).
A

C
B
Fig. 4-2
Fig. 4-3
A

C
B
D

F
E

35
Chapter 4: Infantile Hemangioma
Pa tie n t D
T is girl developed an ulcerated in antile hemangioma o her ace. T e lesion was removed during
in ancy, because the patient would require removal o the lesion in later childhood, and the
aesthetic outcome would be the same. A circular excision and purse-string closure were used to
limit the length o the scar on the child’s ace. T e preoperative view (A) and views a er circular
excision (B) and purse-string closure (C) are shown. T e early postoperative view shows tempo-
rary bunching o skin that resulted rom the purse-string closure (D). T ree months a er surgery,
the skin f attened and the scar was maturing (E). T e patient is shown 2 years a er the procedure
(F). T e parents were pleased with the appearance o the scar and were not interested in a second
stage to convert the circular scar into a line.
Fig. 4-4
A
E
B
C
F
D

36
Part II: Vascular Tumors
Pa tie n t E
T is in ant had an ulcerated lesion o the orehead that caused pain and required dressing changes.
Because the tumor needed excision during the involuting phase or residual bro atty tissue and
the scar would be the same, I per ormed a resection o the lesion during the proli erating phase.
T e hemangioma was located along the hairline, and thus I chose a one-stage lenticular excision,
because the scar would be in a relaxed skin-tension line and camouf aged by hair. I the heman-
gioma was in a more visible area o the orehead, I would have per ormed a two-stage circular
excision and purse-string closure to limit the length o the scar. T e hemangioma is shown be ore
ulceration (A), and the ulcerated lesion is seen be ore excision (B). T e postoperative appearance
is shown (C).
Pa tie n t F
T is in ant presented with a large ulcerated tumor o the arm. Because the ulceration ailed to
heal, the lesion was removed during the proli erative phase. A circular excision was chosen to limit
the length o the scar. T e tumor is shown be ore surgery (A), as well as a er circular excision (B)
and purse-string closure (C). T e arm is seen 4 months a er surgery (D).
Fig. 4-6
A

B
C

D
A

C
B
Fig. 4-5

37
Chapter 4: Infantile Hemangioma
in vo l u t e D in f a n t il e h e Ma n g io Ma : s u c t io n -a s s is t e D l iPe c t o My
Pa tie n t A
T is young child had two de ormities o the posterior trunk rom involuted in antile heman-
giomas. T e caudad tumor le residual skin discoloration and bro atty tissue. T e cephalad
hemangioma was subcutaneous and had bro atty residuum and a contour abnormality. T e
in erior lesion was managed by resection o the abnormal-appearing skin. T rough this incision:
• Subcutaneous bro atty tissue was removed directly.
• A cannula was used to reach the superior hemangioma to f atten the area.
T e preoperative view and outline o superior subcutaneous overgrowth are shown (A and B). T e
marking or the in erior resection o the abnormal skin is shown (C). T e outer circle delineates
the extent o residual subcutaneous bro atty tissue. T e postoperative appearance is seen at the
end o the procedure (D).
Fig. 4-7
A

B
C

D

38
Part II: Vascular Tumors
Pa tie n t B
T is adolescent emale presented with subcutaneous bro atty residuum and a scar rom an
ulcerated in antile hemangioma. Because the skin over the shoulder ullness was normal, it was
f attened with suction-assisted lipectomy rather than the use o a long incision. T e cannula was
placed through the un avorable scar. A er the procedure, the cannula site was closed, which
improved the appearance o the original scar. T e intraoperative markings o the access site in
between areas o ullness are seen (A). T e intraoperative view o suction-assisted subcutaneous
adipose removal is shown (B), along with the lipoaspirate (C). Flattening o areas a er excision
and suction-assisted lipectomy is visible (D). T e 3-month postoperative appearance is seen (E).
Fig. 4-8
A

B
D

E
C

39
Chapter 4: Infantile Hemangioma
in vo l u t e D in f a n t il e h e Ma n g io Ma : s c a l P
Pa tie n t A
Large lesions located on the scalp may result in residual bro atty tissue and alopecia. Because
hemangiomas expand the skin, lesions can be removed with lenticular excision and linear closure.
T e preoperative appearance o this lesion is seen (A). T e intraoperative markings are shown
(B). Note that a subtotal cutaneous excision was designed to acilitate wound closure. Complete
excision was not mandatory; the goal o the procedure was to f atten the area and improve alo-
pecia. T e wound is shown a er removal and subgaleal undermining with scoring (C). Because
o signi cant tension on the wound, it was closed with interrupted, nondissolving sutures. Note
the appearance be ore suture removal (D).
Pa tie n t B
Residual bro atty tissue and alopecia can be improved with resection. In this patient, regressed
in antile hemangioma caused a de ormity (A). T e markings or resection are shown (B). T e
closure with absorbable sutures is seen (C).
Fig. 4-9
A

B
C

D
A

C
B
Fig. 4-10

40
Part II: Vascular Tumors
Pa tie n t C
T is in ant male presented with a large scalp tumor that had completed the proli erative phase.
T e lesion was observed until it had nished regression at 31⁄2 years o age. A er involution, the
tumor was signi cantly smaller, less vascular, and easier to remove. T e appearance in late in ancy
is shown (A). A er involution, the lesion continued to cause a de ormity (B). Note that the ear
became less prominent. T e extent o the hemangioma was visible a er the hair was shaved to
acilitate the procedure (C), and the markings or resection can be seen (D). A er wound closure
and otoplasty, the prominence o the superior ear was decreased (E).
Fig. 4-11
A
C
B
E
D

41
Chapter 4: Infantile Hemangioma
B
A

C
Fig. 4-13
in vo l u t e D in f a n t il e h e Ma n g io Ma : f o r e h e a D
Pa tie n t A
Round lesions in the central orehead are resected best with a circular excision and purse-string
closure to limit the length o the scar. T is 3-year-old child had a large involuted tumor (A). T e
hemangioma was removed as a circle to avoid a scar that would have been two to three times the
diameter o the lesion i it were resected with a lenticular design (B). T e purse-string closure
is seen (C). Note that the circular scar was less than 50% o the area o the hemangioma (D). A
second-stage procedure was per ormed to convert the circular scar into a line (E). T e length o
the scar in a relaxed skin-tension line o the orehead was approximately the same as the diameter
o the hemangioma.
Pa tie n t B
Elliptical lesions are usually removed by lenticular excision, because the linear scar approximates
the length o the hemangioma. T is patient is shown be ore (A) and a er resection (B). Residual
telangiectasias along the scar were treated with a pulsed-dye laser (C).
Fig. 4-12
A

C
B
E
D

42
Part II: Vascular Tumors
in vo l u t e D in f a n t il e h e Ma n g io Ma : n o s e
Pa tie n t A
Super cial round hemangiomas o the nose are removed with a circular excision to limit the
length o the scar. T is 12-month-old had a large tumor (A). T e hemangioma was allowed to
involute be ore excision to acilitate the procedure and give the best outcome. At 3 years o age,
the lesion was smaller and less vascular (B and C). Consequently, resection was sa er and easier
and the aesthetic outcome was most avorable. T e patient is shown a er circular excision and
purse-string closure (D). T e area o the circular scar was signi cantly less than the hemangioma
that was resected (E). T e markings or conversion o the circular scar into a line are seen (F),
and the patient is shown a er lenticular excision o the scar (G). T e postoperative appearance
o this patient is shown (H and I).
Fig. 4-14
A
D
B
F
E
C
G

I
H

43
Chapter 4: Infantile Hemangioma
Fig. 4-15
A
C
B
E
D
F

H
G
Pa tie n t B
Subcutaneous nasal tip hemangiomas require open rhinoplasty to improve the contour. T is
in ant had a large lesion o the nose (A). T e tumor was allowed to involute be ore determin-
ing whether operative intervention was necessary. Also, waiting until regression was complete
acilitated the procedure and achieved the best aesthetic outcome. T e lesion was signi cantly
smaller at 3 years o age, but the child had a residual de ormity (B). T e intraoperative view o the
subcutaneous lesion is seen through an open rhinoplasty incision (C). T e lesion is shown a er
removal (D). Note that the hemangioma separated the lower lateral cartilages. T e cartilages were
reapproximated with sutures in the midline (E). Expanded skin a er excision o the underlying
hemangioma can also be seen (F). T e markings illustrate the redundant integument that was
resected be ore closure. T e nose is shown a er wound closure (G). T e patient’s postoperative
appearance is shown (H).

44
Part II: Vascular Tumors
Pa tie n t C
T is patient presented a er the rapid proli erating phase with a large subcutaneous lesion involv-
ing the glabella. T e tumor was allowed to involute be ore intervention. A circular excision was
used to access the entire subcutaneous tissue, remove the expanded skin, and give the shortest
possible scar. T is patient is shown in late in ancy (A). T e lesion was signi cantly smaller at
3 years o age (B). T e outline o the extent o subcutaneous bro atty tissue and circular skin
resection is seen (C). T is patient is shown a er resection o the involuted hemangioma (D) and
purse-string closure (E). T e patient is shown be ore second-stage conversion o the scar to a line
(F). T e markings or lenticular scar excision (G) and linear closure are shown (H).
Fig. 4-16
A
C
B
E
D
F

H
G

45
Chapter 4: Infantile Hemangioma
in vo l u t e D in f a n t il e h e Ma n g io Ma : c h e e k
Pa tie n t A
Residual hemangiomas involving the lateral cheek can be resected with a preauricular incision.
T is patient had a large lesion in in ancy that was treated with prednisone (A). Residual bro atty
tissue, telangiectasias, and anetoderma were visible at 3 years o age (B). A er a preauricular
incision was made, the skin was undermined medially (similar to a aceli ), and the abnormal
integument was excised. T e preauricular incision is di cult to appreciate a er surgery (C).
Pa tie n t B
Round lesions on visible areas o the ace are removed with a circular excision and purse-string
closure. T is patient is shown be ore surgery (A) and a er circular resection (B) and purse-string
closure (C). T e patient’s postoperative appearance is seen (D). T e child’s parents were pleased
with the scar and were not interested in a second-stage to convert it into a line.
A

B
C

D
Fig. 4-18
Fig. 4-17
A

C
B

46
Part II: Vascular Tumors
Pa tie n t C
Large hemangiomas o the cheek are usually resected best with a circular excision and purse-string
closure to provide the most avorable-appearing scar. T is 12-month-old child presented with a
large tumor a er the proli erative phase was nished (A). T e lesion was observed until 31⁄2 years
o age to allow it to decrease in size as much as possible to acilitate the resection (B). Intraoper-
ative outline o the circular excision is seen (C). T is patient is shown a er subtotal removal (D)
and purse-string closure (E). T e circular scar became elliptical along the mesolabial crease (F).
T e outline o the lenticular resection o the scar, which will be converted into a line, is shown
(G). T e early postoperative appearance is seen (H).
Fig. 4-19
A
C
B
E
D
F

H
G

47
Chapter 4: Infantile Hemangioma
Pa tie n t D
T is patient could have been treated with liposuction through a preauricular incision, but in my
experience the resulting improvement in contour is not as avorable as direct excision because:
• It is more di cult to remove the adipose tissue.
• T e skin is not excised.
Although contraction occurs a er liposuction, the contour improvement is not as good as remov-
ing the expanded skin. I per ormed a direct circular excision and later converted the scar into a
line. T e patient has had a avorable improvement in his contour, and the scar on his cheek can
be appreciated only on close inspection. T e preoperative view o le cheek overgrowth rom an
involuted subcutaneous hemangioma is shown (A). T e intraoperative markings are seen (B). T e
outer circle delineates the extent o the lesion, and the inner marking is the cutaneous skin exci-
sion. T rough the circular excision, skin f aps were raised, and the entire area o excess adipose
tissue was sa ely removed by direct excision (C). T e wound was closed with a purse-string closure
(D). T e skin resection helped to reduce his cheek prominence. T e appearance o the circular
scar be ore its conversion into a line is seen (E). T e postoperative results are shown (F and G).
Fig. 4-20
A
B

D
C
E

G
F

48
Part II: Vascular Tumors
in vo l u t e D in f a n t il e h e Ma n g io Ma : u PPe r l iP
Pa tie n t A
Involuted lesions located on the cutaneous portion o the upper lip can be removed with a cir-
cular excision. T is child presented a er in ancy and was observed until 31⁄2 years o age to allow
the lesion to complete involution be ore proceeding with resection. T is patient’s preoperative
appearance is shown (A and B). T e intraoperative marking o the circular excision o bro atty
residuum and expanded skin is visible (C). Residual telangiectasias outside o the skin resection
can be treated with a pulsed-dye laser. T e patient is seen a er rst-stage excision and purse-
string closure (D). T e circular scar is shown be ore the second-stage procedure (E). T e outline
o lenticular resection o residual scar/lesion along the mesolabial crease is visible (F). T e post-
operative appearance is shown be ore pulsed-dye laser treatment o telangiectasias outside o the
scar (G and H).
Fig. 4-21
A
C
B
E
D
F

H
G

49
Chapter 4: Infantile Hemangioma
Pa tie n t B
T is patient presented with a hemangioma in an un avorable location that was treated with in-
tralesional corticosteroid during in ancy. His residual de ormity was managed with a combined
method o resection and pulsed-dye laser. T e appearance o the hemangioma in in ancy is shown
(A). T e patient is seen at 3 years o age a er completion o involution (B). Fibro atty tissue and
excess skin were resected with a vertical incision along the location o the missing right philtral
column (C). A er resection, the area is f at and redundant skin has been removed (D). T e resid-
ual skin containing telangiectasias was managed with a pulsed-dye laser. T is patient is shown
a er two pulsed-dye laser treatments (E).
Fig. 4-22
A
C
B
E
D

50
Part II: Vascular Tumors
Pa tie n t C
Residual tumors along the mucocutaneous junction may be removed with a lenticular resection
with placement o the linear scar along the white roll. T is child had an involuted hemangioma
o the upper lip that underwent a one-stage lenticular excision (A). T e postoperative appearance
with a linear scar placed at the mucocutaneous junction is seen (B).
Pa tie n t D
Residual lesions a ecting the vermilion or mucosa are excised with a transverse mucosal resection
and linear closure. T is 3-year-old emale had a residual tumor causing a de ormity (A). Markings
or a transverse mucosal excision are shown (B). Linear closure at the junction o the keratinized
and nonkeratinized vermilion is shown (C). T e patient had improved contour a er surgery (D).
Note that the transverse scar on the vermilion is hidden.
Fig. 4-23
B
A
Fig. 4-24
B
A
D
C

51
Chapter 4: Infantile Hemangioma
Pa tie n t E
Lesions involving the median tubercle can be improved with a transverse mucosal resection. T e
preoperative appearance o this patient at 3 years o age is shown (A). T e markings or resection
are visible (B). T is patient is shown a er excision (C) and wound closure (D). T e early postop-
erative result is seen (E).
Pa tie n t F
Destroyed anatomic structures o the upper lip can be reconstructed a er the tumor has involuted.
T is patient was managed at an outside institution during in ancy and had anetoderma o the up-
per lip and the absence o the le Cupid’s bow. T e preoperative view (A) and markings (B) or the
skin excision where the vermilion will be advanced to re-create the Cupid’s bow are shown. T e
inset o the vermilion f ap was advanced superiorly (C). T e postoperative appearance is seen (D).
A

C
B

D
Fig. 4-26
Fig. 4-25
A
C
B
E
D

52
Part II: Vascular Tumors
in vo l u t e D in f a n t il e h e Ma n g io Ma : l o w e r l iP
Pa tie n t A
Involuted lesions o the lower lip are resected similar to the upper lip. T is 2-week-old in ant is
shown be ore the development o an in antile hemangioma (A). T e tumor is seen at the end o
the proli erative phase (B) and a er completion o involution (C). T e appearance o the lower
lip is shown a er transverse lenticular resection (D).
Pa tie n t B
Residual bro atty tissue located on the cutaneous portion o the lip is removed by placing an
incision along the mucocutaneous junction. T e appearance o the hemangioma o this patient
at 12 months o age is seen (A). Residual bro atty tissue was caudal to the vermilion at 3 years o
age (B). T e intraoperative markings are shown (C). T e in erior line delineates the area o excess
subcutaneous adipose tissue. Along the white roll a skin excision was marked to remove excess
expanded skin. T e suture line was placed along the mucocutaneous junction a er excision (D).
T e patient’s appearance is shown 3 months a er surgery (E).
Fig. 4-28
A

B
C

E
D
A

C
B

D
Fig. 4-27

53
Chapter 4: Infantile Hemangioma
Pa tie n t C
Large labial lesions may require staged resection. T is 3-year-old male is seen be ore rst-stage
excision (A). T e marking or a transverse mucosal resection (B) and linear closure (C) are shown.
Second-stage resection o residual bro atty tissue involved the cutaneous portion o the lower
lip (D). A mucocutaneous skin excision was per ormed to remove expanded skin and acilitate
access to the subcutaneous broadipose tissue delineated by the outer marking. T e incision was
closed along the white roll (E). T e early postoperative result is shown be ore pulsed-dye laser
or residual telangiectasias (F).
Fig. 4-29
B
A
D
C
F
E

54
Part II: Vascular Tumors
in vo l u t e D in f a n t il e h e Ma n g io Ma : e a r
Pa tie n t A
umors involving the ear can cause overgrowth and de ormation requiring otoplasty, in addi-
tion to resection o the residual lesion. T is child had an involuted hemangioma that caused a
prominent lobule (A). T is patient is shown a er resection o the lesion (B). Note the markings
or a posterior lobule skin excision to set back the ear lobe. T e patient is seen a er lobule skin
resection (C). T e postoperative appearance is shown (D).
Pa tie n t B
Residual bro atty tissue and damaged skin are removed similar to other areas, typically with a
lenticular resection and linear closure. T is patient had excess tissue, a scar, and overgrowth o the
lower third o the ear (A). T e markings or vertical resection are shown (B). T e postoperative
appearance is seen (C).
Fig. 4-30
B
A
D
C
Fig. 4-31
A

C
B

55
Chapter 4: Infantile Hemangioma
in vo l u t e D in f a n t il e h e Ma n g io Ma : n e c k
Pa tie n t A
T e neck is a avorable location or lenticular excision, because linear scars can be placed in
relaxed skin-tension lines. Neck lesions are also more likely to be elliptical and less amenable to
circular excision. T is male had redundant skin, bro atty tissue, and anetoderma rom an in-
voluted hemangioma (A). T e intraoperative view is shown (B). T e appearance is seen 4 weeks
a er surgery (C and D).
Pa tie n t B
Although this hemangioma was amenable to a circular excision, I removed it in one stage with
a lenticular resection, because a linear scar in a relaxed skin-tension line o the posterior neck is
more avorable than a circular scar (limiting the length o the scar in this location is also not crit-
ical). T e preoperative view o this patient is shown (A), ollowed by the outline o the resection
(B). T e patient is seen a er wound closure (C).
A
C
B
D
Fig. 4-32
Fig. 4-33
A

C
B

56
Part II: Vascular Tumors
Pa tie n t C
Hemangiomas located along the lateral neck can be removed with a lenticular excision placed
in a relaxed skin-tension line. T is patient had an involuted hemangioma o the neck (A). T e
3-month postoperative view is shown (B).
in vo l u t e D in f a n t il e h e Ma n g io Ma : t r u n k
Pa tie n t A
Most residual lesions o the trunk are removed with a one-stage lenticular resection, because
linear scars in relaxed skin-tension lines are avorable, and it is not critical to limit the length o
the scar. T is patient had a lesion o the lateral abdomen (A). T e intraoperative markings are
shown (B). T e early appearance o the scar a er resection is seen (C).
A

B
Fig. 4-34
Fig. 4-35
A

B

C

57
Chapter 4: Infantile Hemangioma
Fig. 4-37
A

B

C

D
Fig. 4-36
A

B

C

D
Pa tie n t B
A circular excision was chosen or this emale patient to avoid a long scar between her breasts.
She had an involuted hemangioma o the chest (A). Circular excision (B) and purse-string closure
(C) are shown. T e appearance is shown 4 weeks a er surgery (D).
Pa tie n t C
Large involuted lesions o the trunk can be removed by serial excision. T is patient had a residual
hemangioma o the posterior trunk (A). T e patient is seen a er rst-stage excision (B) and be ore
second-stage resection (C). Wound closure is shown a er complete excision (D).

58
Part II: Vascular Tumors
Pa tie n t D
T is patient had a large, di use in antile hemangioma o the le breast during in ancy. T e tumor
expanded the skin, causing ptosis and anetoderma. She underwent mastopexy to improve her
breast symmetry. She is seen be ore surgery (A) and a er periareolar mastopexy (B).
Pa tie n t E
T is young male presented with an involuted lesion o the le chest. T e lesion was removed in
two stages, and the nipple and areola were preserved. T e appearance o the lesion in early child-
hood is shown (A). T e lesion was smaller a er it had involuted when the patient was 5 years
o age (B). T e markings or rst-stage resection o subcutaneous bro atty tissue are visible (C
and D).
A

B
Fig. 4-38
Fig. 4-39, A-D
A

B
C

D

59
Chapter 4: Infantile Hemangioma
T e intraoperative view at the end o the procedure is shown (E). T e appearance is shown be ore
second-stage periareolar mastopexy to remove excess skin and decrease the circum erence o the
areola (F and G). T e appearance a er circular skin resection (H) and purse-string closure (I) is
seen. T e postoperative result is shown (J).
Fig. 4-39, E-J
E

F
G

H
I

J

60
Part II: Vascular Tumors
in vo l u t e D in f a n t il e h e Ma n g io Ma : e x t r e Mit y
Pa tie n t A
Large, round lesions o the arm can be removed with circular excision to limit the length o the
scar. T e preoperative appearance o the lesion is seen (A). T is patient underwent circular re-
moval (B) and purse-string closure (C). T e scar’s appearance is shown a er the rst stage (D).
T e intraoperative view is shown a er lenticular excision o the scar and linear closure (E). T e
postoperative result is seen (F).
Fig. 4-40
A

B
C

D
E

F

61
Chapter 4: Infantile Hemangioma
Pa tie n t B
T is patient presented at 3 years o age with a large involuted lesion o the upper extremity. Be-
cause the hemangioma was elliptical and not round, it was managed by serial lenticular resection.
T e preoperative appearance (A) and markings or rst-stage excision (B) are shown. T e linear
suture line is seen at the end o the procedure (C). T e residual scar and hemangioma are shown
be ore second-stage excision (D). Intraoperative markings are shown (E). T e patient is shown
a er wound closure (F). T e linear scar is seen 3 months a er surgery (G).
Fig. 4-41
A

B
C

D
E

F
G

62
Part II: Vascular Tumors
Pa tie n t C
Lower extremity hemangiomas are typically removed with lenticular resection and linear closure.
Large lesions may require serial excision. T is patient is shown be ore surgery (A). T e outline o
rst-stage serial excision (B) is seen, and the wound is shown a er resection (C). T e appearance
and markings or second-stage removal are shown (D and E). T e suture line is seen at the end
o the procedure (F).
Fig. 4-42
A

B
C

D
E

F

63
Chapter 4: Infantile Hemangioma
COMPLICATIONS
T e two most common complications a er extirpation o an in antile hemangioma are bleeding
and suture line dehiscence. T e risk o hematoma is greatest during the proli erative phase when
the tumor is vascular. Resection o bro atty tissue a er the tumor has involuted has a lower risk
o postoperative hemorrhage. A er excising an in antile hemangioma, the wound edges that are
sutured together should not contain residual hemangioma i possible. T e tumor causes ischemia
as a result o shunting past the capillary circulation. T us approximation o tissues containing
the lesion has a higher risk o in ection and incision line breakdown. Serial excision o in antile
hemangioma should be avoided i possible; it is pre erable to remove the entire area. I skin edges
containing the in antile hemangioma must be sutured together, interrupted sutures should be
placed, because these are the least ischemic, and they should be le in place longer compared
with placement into a nonischemic site.
Circular excision and purse-string closure have a slightly higher risk o dehiscence compared with
linear closure. Strategies to reduce the wound opening are to:
• Place interrupted cutaneous sutures, in addition to the purse-string suture
• Use nondissolvable sutures
• Rein orce the area with Steri-Strips i interrupted cutaneous absorbable sutures are placed
I a circular incision opens, it will contract into a circular scar that may not appear signi cantly
di erent a er it has matured.
CONCLUSION
Operative management o in antile hemangiomas can be challenging. Variables that inf uence
management include:
• Age o the patient
• Location o the lesion
• ype o de ormity
Generally, resection during in ancy is avoided, because operative morbidity is highest, and the
outcome/scar is usually better i the tumor is removed a er it has regressed and is much smaller.
T e indications to remove a lesion during the proli erating phase are:
• Failure/contraindication to pharmacotherapy
• T e scar/outcome would be the same as intervention during the involuting phase
Because in antile hemangiomas expand the skin, a er their excision almost all wounds can be
reconstructed by approximating local tissues. Circular lesions in noticeable locations can be re-
moved by circular excision and purse-string closure to limit the length o a linear scar. I pre er to
intervene surgically or a hemangioma between 3 and 4 years o age because:
• umors do not improve signi cantly a er 31⁄2 years o age.
• Long-term memory and sel -esteem begin to orm around 31⁄2 years o age.

64
Part II: Vascular Tumors
SUGGESTED READINGS
Couto JA, Maclellan RA, Greene AK. In antile hemangioma: treatment rate during the proli erating phase. J Cra-
nio ac Surg : , .
Couto JA, Maclellan RA, Greene AK. Management o vascular anomalies and related conditions using suction-
assisted tissue removal. Plast Reconstr Surg : e, .
Couto RA, Maclellan RA, Zurakowski D, et al. In antile hemangioma: clinical assessment o the involuting phase
and implications or management. Plast Reconstr Surg : , .
Couto JA, Sullivan JE, Greene AK. Facial wound closure in children using a - absorbable suture dressing. J Cra-
nio ac Surg : , .
Greene AK. Management o hemangiomas and other vascular tumors. Clin Plast Surg : , .
Hassanein AH, Couto JA, Greene AK. Circular excision and purse-string closure or pediatric acial skin lesions. J
Cranio ac Surg : , .
Mulliken JB, Rogers GF, Marler JJ. Circular excision o hemangioma and purse-string closure: the smallest possible
scar. Plast Reconstr Surg : , .

Arin K. Greene
65
A pyogenic granuloma (PG) is also re erred to as a lobular capillary hemangioma. It is unique in
the eld o vascular anomalies, because almost all PGs are treated surgically. Many other types o
vascular anomalies are managed by observation, pharmacotherapy (topical, intralesional, or sys-
temic), laser, sclerotherapy, and/or embolization. A PG is red and may be sessile or pedunculated.
Lesions average 6 mm in diameter, and the mean age o onset is 6 years. PGs most commonly
a ect the head/neck (two thirds), ollowed by the trunk, upper extremity, and lower extremity.
T e integument or mucous membranes are involved. Numerous treatments have been described:
curettage, cryotherapy, ull-thickness skin excision, laser, shave excision, and super cial cautery;
pharmacotherapy is not e ective. Because PGs involve the reticular dermis, interventions that
do not treat the entire depth o the skin have an approximately 50% recurrence rate ( or example,
curettage, shave excision, super cial cautery, and laser therapy).
Pyo g en ic Gr an u l o ma
Ch a Pt er 5
KEY POINTS
• Most pyogenic granulomas require treatment, because they are at risk for bleeding.
• A barrier dressing (for example, a Band-Aid) is used to cover the lesion to avoid incidental
trauma and hemorrhage.
• Interventions that do not treat the entire depth of the dermis have a higher recurrence rate.
• Full-thickness skin excision is used for large lesions.
• Small pyogenic granulomas or those with a narrow base can be treated with needle-tip cau-
tery through the dermis.

66
Part II: Vascular Tumors
SURGICAL INDICATIONS
Surgical management o a PG is usually mandatory. Unlike many other types o vascular anom-
alies that are o en removed to improve the patient’s appearance, PGs have a signi cant risk o
ulceration and bleeding. Consequently, asymptomatic lesions are extirpated to obviate the risk
o hemorrhage. A secondary indication to remove a PG is to improve the patient’s sel -esteem,
which can be lowered i the tumor is located in a visible area.
T e phenotypes and morbidity o PGs are demonstrated. A large sessile lesion o the posterior
trunk is seen (A). A pedunculated PG o the lip causing psychosocial morbidity is shown (B).
A small lesion o the cheek is seen (C). An intraoral PG is visible (D). A PG that developed in
a capillary mal ormation is seen (E). An ulcerated lesion (F) and a bleeding PG (G) are shown.
Fig. 5-1
A
C
B
E
D
F

G

67
Chapter 5: Pyogenic Granuloma
SURGICAL MANAGEMENT
t iMin g o f in t e r v e n t io n
A PG requires intervention, regardless o the age o the patient. Most individuals present a er
the lesion has bled; many have been managed in an emergency department or hemorrhage be-
ore their re erral. Although the amount o blood loss is not harm ul to the patient, bleeding can
cause signi cant anxiety or the individual and amily. Consequently, the procedure is scheduled
as soon as possible. Lesions that have not yet bled can be scheduled less acutely. Rarely a small
and f at PG may exist or a long period without a history o hemorrhage. Consideration is given
to treating the lesion be ore 4 years o age, when the patient’s long-term memory and sel -esteem
begin to orm. In older children, adolescents, and adults, the onset o a PG can cause psychosocial
morbidity, and the patient will seek intervention to improve his or her appearance (see Video 5-1,
Pyogenic Granuloma).
While awaiting treatment, a PG will occasionally “ all o ” as a result o incidental trauma, ul-
ceration, or bleeding. T e lesion usually reenlarges, because the component in the deep dermis
remains. I the PG has “disappeared,” I examine the patient, and i a cutaneous component is
present, I will o er treatment to eliminate the risk o regrowth (i the lesion enlarges and begins
to bleed again, the morbidity and scar will be worse). I I am unable to identi y the PG a er it has
allen o , the patient is observed.
c o n t r o l o f t h e l e s io n u n t il s u r g ic a l t r e a t Me n t
A PG typically hemorrhages rom incidental trauma and does not bleed spontaneously. Patients
and amilies are counseled to cover the lesion with a Band-Aid or protection until it is treated. I
a PG bleeds, amilies are instructed to cover the lesion with a Band-Aid and then apply pressure
with a washcloth soaked in ice water over the Band-Aid. I a washcloth is touching the PG, the
lesion will rebleed a er pressure is discontinued, because the lesion/clot will be adherent to the
washcloth.
t y Pe o f a n e s t h e s ia
Determining whether a PG can be treated with local or general anesthesia depends on the age o
the patient, the location o the lesion, and the size o the PG. A young child with a large lesion on
the ace is more likely to require general anesthesia than an older individual with a small PG on
the trunk. Generally, removal o acial lesions cannot be per ormed with the patient under local
anesthesia be ore adolescence. PGs located on the trunk or extremity may be able to be excised
in the o ce in older children. I I am com ortable removing a lesion with either a local or general
anesthetic, I will let the patient/ amily chose which method they pre er.
f u l l -t h ic k n e s s s k in e xc is io n
My rst-line treatment or PGs is excision o the skin involving the lesion, because it eliminates
the risk o recurrence. An additional advantage o resection is that the specimen can be sent or
histopathologic examination. T e borders o the PG are marked, and the surgical site is in ltrated

68
Part II: Vascular Tumors
with 0.25% bupivacaine with epinephrine. I pre er a long-acting local anesthetic to give the pa-
tient a greater duration o pain control. Epinephrine can be administered anywhere in the body
(including ngers and toes); it acilitates the procedure by reducing blood loss. I the PG is sessile,
the excision borders are at the junction o the lesion and normal surrounding tissue. A margin
o normal skin should not be harvested, because the PG is benign and the length o the scar will
increase. Most PGs have a small base attached to the skin with a larger, super cial component
(similar to a mushroom). It is important to li the PG and outline only the base or excision.
Usually a much smaller incision and scar are necessary than was initially expected. Lesions can
be removed with a No. 67 Beaver blade or a No. 11 blade. A er resection to the subcutis, the base
o the wound is cauterized to injure any possible remaining lesional tissue to reduce the risk o
recurrence.
A er excision o a PG, the wound can be closed in three ways:
. Linearly
. As a circle
. Allowed to heal secondarily
Linear closure is used or lesions located on the trunk or extremity or head/neck in a noncon-
spicuous location ( or example, the scalp) or or noncircular lesions. In children I place absorb-
able sutures to avoid removal, which can be traumatic or the patient and parents. PGs that are
circular and located in un avorable locations on the ace ( or example, the nose, lip, or cheek) are
best managed by circular excision and purse-string closure. Although a circular excision limits
the length o the scar, a disadvantage is that a second procedure may be necessary to convert the
round scar into a line. I a second stage is per ormed, the length o the scar will be approximately
the same size as the diameter o the original lesion. More than one hal o patients are satis ed
with the appearance o the circular scar and are not interested in a second stage to convert it into
a line. T e circular scar can appear similar to a chicken pock or acne scar. Occasionally I allow
small circular PGs located in un avorable locations ( or example, the mucocutaneous junction
o the lip and nasal tip) to heal secondarily. Placing sutures into very small openings can be
di cult and lengthen the scar; allowing a circular area to contract also minimizes distortion o
surrounding structures.
c a u t e r y
Small sessile PGs (less than or equal to 2 mm in diameter) can be treated with ull-thickness
cautery instead o excision; this technique yields the smallest possible scar. Young children re-
quire sedation, and older patients are managed with a local anesthetic. I use electrocautery with
a needle tip and a low coagulation setting. T e needle is placed ull-thickness through the PG
extending to the subcutis. T e area is covered with bacitracin. Cautery can also be used or large
pedunculated PGs that have a small base less than or equal to 2 mm in diameter. T e cutaneous
component o the lesion is removed with a scissors, and the needle-tip Bovie is used to cauterize
the remaining lesion in the dermis.

69
Chapter 5: Pyogenic Granuloma
PATIENT EXAMPLES
f u l l -t h ic k n e s s s k in e xc is io n : l in e a r c l o s u r e
Pa tie n t A
Most PGs are managed by ull-thickness skin excision and linear closure. T e preoperative ap-
pearance o the sessile lesion is seen (A and B). T is patient is shown a er resection without
margins (C).
Pa tie n t B
PGs can enlarge over time. T is patient was managed by linear excision, because the lesion was
located in a avorable location along a relaxed skin-tension line o the lower eyelid. T e appearance
o the PG during the initial consultation is seen (A). T e increased size o the PG on the day o
surgery is shown (B). T e linear scar was hard to appreciate a er surgery (C).
A

C
B
Fig. 5-2
A

C
B
Fig. 5-3

70
Part II: Vascular Tumors
Pa tie n t C
A er excision o this sessile PG in this patient, it was closed linearly, because the scar was in
a avorable location hidden behind the ear. T is patient is shown be ore surgery (A) and a er
wound closure (B).
Pa tie n t D
T is large PG was sessile and located along the hairline. It was closed linearly, because: (1) the
scar would be camouf aged by hair, and (2) the location was in an un avorable area or circular
excision and purse-string closure (round scars in hair-bearing areas can cause visible alopecia).
T e PG is shown be ore resection (hair was shaved in the area to acilitate the procedure) (A).
Linear wound closure is seen (B).
A

B
Fig. 5-4
A

B
Fig. 5-5

71
Chapter 5: Pyogenic Granuloma
Pa tie n t E
T e base o a PG is o en narrower than the diameter o the rest o the lesion. Consequently, the
PG can be removed with an incision shorter than the lesion. A large PG that was ulcerated and
bleeding is seen (A). T e lesion was pedunculated on a stalk, and thus the base was smaller than
the rest o the PG. A er excision the suture line was shorter than the diameter o the extracuta-
neous component o the lesion (B).
Pa tie n t F
T is lesion was sessile and involved a large portion o the ala. Removal o the entire PG required
a skin gra or local f ap to reconstruct the area. Consequently, I per ormed a subtotal resection
and planned to remove the remainder o the lesion serially during a second stage. However, a er
the rst procedure, the residual lesion (or erythema rom inf ammation) resolved, and this patient
did not require urther intervention. T e preoperative appearance is seen (A). A er resection the
residual redness remained (B). T e erythema resolved a er surgery (C).
A

B
Fig. 5-6
A

C
B
Fig. 5-7

72
Part II: Vascular Tumors
f u l l -t h ic k n e s s s k in e xc is io n : c ir c u l a r c l o s u r e
Pa tie n t A
T is young child had a sessile PG in an un avorable location on the nose. o limit the length o
the scar, a circular excision and purse-string closure were per ormed. T is patient is shown be ore
surgery (A) and a er purse-string closure (B).
Pa tie n t B
T is PG was adjacent to the mucocutaneous junction o the lower lip. Lenticular excision would
have resulted in a vertical scar at least twice the diameter o the lesion that could have crossed
the mucocutaneous junction. Consequently, the lesion was excised as a circle and closed with a
purse-string suture to avoid violating the white roll. T is patient is seen be ore surgery (A) and
2 weeks a er surgery (B).
A

B
Fig. 5-8
A

B
Fig. 5-9

73
Chapter 5: Pyogenic Granuloma
f u l l -t h ic k n e s s s k in e xc is io n : c l o s u r e by s e c o n Da r y in t e n t io n
Lesions involving the distal extremity are in a avorable location or healing by secondary inten-
tion. Closure o the wound would have increased the length o the scar, required immobilization,
and been at risk or suture line dehiscence. Instead topical antibiotic was applied, and this patient
was allowed to resume ull activity. T is PG involved the small nger (A). A er excision the
wound was le open to heal secondarily (B). T e postoperative appearance o the small nger is
shown (C).
f u l l -t h ic k n e s s c a u t e r y
Small PGs do not require ull-thickness excision and can be treated with electrocautery. Resection
o this lesion would have created a linear scar that may have crossed the mucocutaneous junction.
Full-thickness mucosa was cauterized with needle-tip cautery on a low setting. T e preoperative
appearance is seen (A). Needle-tip cautery used is shown (B). T e intraoperative view a er treat-
ment is seen (C). T e postoperative appearance is shown (D).
A

C
B
Fig. 5-10
Fig. 5-11
A

B
C

D

74
Part II: Vascular Tumors
Pa r t ia l e xc is io n a n D f u l l -t h ic k n e s s c a u t e r y
Pa tie n t A
T is young child developed a pedunculated labial PG that was managed by excision and cautery
o the base o the lesion. Preoperative views are shown (A and B). Full-thickness cautery o the
lip is shown a er excision (C and D). T e postoperative appearance is seen (E and F).
Pa tie n t B
T e stalk o this PG was excised, and the remaining lesion involving the deep dermis was treated
with cautery. T is patient is shown be ore (A) and a er (B) excision and ull-thickness cautery.
T e early postoperative view is seen (C).
Fig. 5-12
A

C
B
D

F
E
A

C
B
Fig. 5-13

75
Chapter 5: Pyogenic Granuloma
Pa tie n t C
T is boy presented with a PG o his right cheek. T e preoperative appearance o the PG is shown
(A). T e potential length o a linear incision to remove the circular PG is seen (B). T is patient
is shown af er circular excision (C) and ull-thickness cautery o the base o the lesion (D). T e
patient is seen 6 weeks af er surgery (E).
COMPLICATIONS
I have not experienced the recurrence o a PG af er per orming a ull-thickness skin excision or
treating the entire depth o the integument with cautery. Postoperative bleeding is rare, because
the lesions are small, and the area is cauterized during the procedure. In ection is also very rare.
Circular excision and purse-string closure have a slightly higher risk o wound dehiscence com-
pared with lenticular resection and linear closure.
CONCLUSION
A PG is a small but problematic lesion because it requently bleeds, causing distress to the patient
and amily. I the tumor is located in a visible location, it can also lead to psychosocial morbidity.
Almost all PGs require intervention either to treat or prevent hemorrhage. Lesions are covered
to prevent incidental trauma that can cause bleeding. Large sessile PGs or pedunculated lesions
with a wide base are removed with a ull-thickness skin excision; margins are unnecessary. Small
PGs or those with a narrow base can be treated with needle-tip cautery through the entire dermis.
Fig. 5-14
A

B
C

E
D

76
Part II: Vascular Tumors
SUGGESTED READINGS
Greene AK. Management o hemangiomas and other vascular tumors. Clin Plast Surg : , .
Greene AK. Pyogenic granuloma. In Greene AK, ed. Vascular Anomalies: Classi cation, Diagnosis, and Manage-
ment. St Louis: Quality Medical Publishing, .
Gupta A, Kozakewich H. Histopathology o vascular anomalies. Clin Plast Surg : , .
Lee J, Sinno H, ahiri Y, et al. reatment options or cutaneous pyogenic granulomas: a review. J Plast Reconstr
Aesthet Surg : , .
Mills SE, Cooper PH, Fechner RE. Lobular capillary hemangioma: the underlying lesion o pyogenic granuloma. A
study o cases rom the oral and nasal mucous membranes. Am J Surg Pathol : , .
Patrice SJ, Wiss K, Mulliken JB. Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study o
cases. Pediatr Dermatol : , .

Arin K. Greene
77
Several types o rare vascular tumors may be managed by resection. T e most requently encoun-
tered are (in order o requency): congenital hemangiomas, kaposi orm hemangioendotheliomas
(KHE), in antile myo bromas, arteriovenous acral tumors, and Enzinger intramuscular heman-
giomas. Because o the rarity o these lesions, their management will be collectively discussed in
this single chapter.
SURGICAL INDICATIONS
T e major variable that dictates whether a rare vascular tumor is treated surgically is the type o
lesion (see Video 6-1, Rare umor). Be ore surgical intervention is considered, the tumor must be
diagnosed. Although more than 90% o vascular anomalies can be diagnosed by history and phys-
ical examination, uncommon lesions are more di cult to identi y. Consequently, rare vascular
tumors are likely to require imaging or biopsy or diagnosis. A er the type o lesion is known, its
Rar e Vas c u l ar Tu mo r s
Ch a Pt er 6
KEY POINTS
• Management of a rare vascular tumor is dependent on the type of lesion.
• Rapidly involuting congenital hemangiomas and kaposiform hemangioendotheliomas rarely
require surgical intervention.
• Noninvoluting congenital hemangiomas, infantile myofibromas, Enzinger intramuscular he-
mangiomas, and arteriovenous acral tumors are commonly resected.
• Surgical intervention for infantile myofibromas, Enzinger intramuscular hemangiomas, and
arteriovenous acral tumors is usually required to accurately diagnose these lesions by his-
topathologic examination.
• Rare vascular tumors are best managed in an interdisciplinary vascular anomalies center.

78
Part II: Vascular Tumors
natural history and potential treatments are considered. Some rare vascular tumors regress ( or
example, congenital hemangiomas and in antile myo bromas), and thus they may not require
immediate intervention. KHEs respond to chemotherapy and are seldom treated surgically. Other
lesions are managed primarily with excision ( or example, Enzinger intramuscular hemangiomas
and arteriovenous acral tumors).
SURGICAL MANAGEMENT
c o n g e n it a l h e Ma n g io Ma
T ere are two types o congenital hemangiomas:
. Rapidly involuting congenital hemangioma (RICH)
. Noninvoluting congenital hemangioma (NICH)
Both types are ully ormed at birth and are located on the head/neck (about 45%), extremity
(about 45%), or trunk (about 10%). Lesions are red purple, well de ned, and usually surrounded
by a pale halo. Unlike in antile hemangiomas, congenital hemangiomas do not have postnatal
growth. A RICH begins to involute immediately a er birth and has completely regressed by ap-
proximately 12 months o age. A NICH does not improve and maintains the same appearance.
Ra p id ly Invo lu tin g Co n ge n ita l He m a n g io m a
In contrast to an in antile hemangioma, which o en leaves behind excess broadipose tissue, a
RICH can cause subcutaneous atrophy a er involution. A patient may have a cutaneous depres-
sion or residual telangiectasias that causes psychosocial morbidity. I the skin is relatively normal,
adipose atrophy can be reconstructed with at gra ing. elangiectasias, scarring, or anetoderma o
the integument may be improved by excising the area. I the region is large, serial resection might
be required. A de ormity in a visible location ( or example, the ace or hands) can be improved
Table 6-1 Management o Rare Vascular Tumors
Tumor
Natural History
Morbidity
Treatment
RICH
Regression
Subcutaneous atrophy
Cutaneous discoloration
Observation
Resection
Fat grafting
NICH
Static
Cutaneous discoloration
Observation
Resection
KHE
Partial regression
Thrombocytopenia
Pain
Contractures
Cutaneous discoloration
Vincristine
Sirolimus
Resection
Infantile myofibroma
Regression or static
Subcutaneous hypertrophy
Observation
Resection
Enzinger intramuscular
hemangioma
Progression
Subcutaneous hypertrophy
Resection
Arteriovenous acral
tumor
Progression
Subcutaneous hypertrophy
Cutaneous discoloration
Resection

79
Chapter 6: Rare Vascular Tumors
with surgical intervention between 3 and 4 years o age, be ore the child’s sel -esteem and long-
term memory begin to orm. Because most RICHs are located on the trunk and extremities, a
residual de ormity can usually be camouf aged with clothing. Patients o en present in early ado-
lescence or surgical improvement when their appearance begins to be more important to them.
No n invo lu tin g Co n g e n ita l He m a n g io m a
A NICH changes minimally a er birth and o en causes psychosocial distress. Although laser
treatment may be able to improve its color, the skin and deep dermis are abnormal, and thus a
de ormity will remain. T e most common intervention or NICHs is resection, which can require
serial excisions i it is large. Similar to RICHs, many are located outside o the head/neck, and
patients typically seek intervention in early adolescence to improve their appearance. Congen-
ital hemangiomas are benign, and negative margins in the resection specimen are unnecessary.
Lesions are not at increased risk o intraoperative bleeding.
k a Po s if o r M h e Ma n g io e n Do t h e l io Ma
A KHE is a locally aggressive neoplasm that does not metastasize. It a ects 1 in 100,000 persons,
and 50% are present at birth; the rest usually develop in early childhood. KHEs appear reddish
purple and do not have well-de ned borders. Lesions a ect the head/neck (40%), trunk (30%),
or extremity (30%). Seventy percent cause li e-threatening thrombocytopenia (Kasabach-Merritt
phenomenon). KHEs typically undergo partial regression in early childhood but do not com-
pletely involute. Patients can develop contractures and chronic pain rom brosis. Surgical treat-
ment is rare or two reasons: (1) Lesions are almost always di use, involving multiple tissue planes,
and (2) e ective pharmacotherapy is available.
During in ancy patients usually are treated with either intravenous vincristine or oral sirolimus to
shrink the tumor, treat thrombocytopenia, reduce the risk o chronic pain, and prevent contrac-
tures rom brosis. Surgical intervention or KHEs is reserved or: (1) localized lesions that are
amenable to resection and (2) secondary reconstruction or brosis/contractures. Consideration
should be given to excision in the rare circumstance that the KHE is localized and amenable to
complete (or near-total) resection. Removing the lesion in in ancy will obviate the morbidity
o systemic pharmacotherapy. I possible, the surgeon should wait until the child is older than
6 months o age to reduce the anesthetic risk. Platelet unction must be maximized be ore sur-
gical intervention; patients may require vincristine or sirolimus be ore surgery to normalize their
platelets.
KHEs do not metastasize and have minimal risk o recurrence a er extirpation. Consequently,
achieving normal margins is not mandatory. It can be di cult to distinguish the lesion rom
normal surrounding skin because o edema, brosis, and erythema. As much o the tumor as
possible should be removed; it is not critical to include normal margins i it would complicate
reconstruction. Even a subtotal resection will signi cantly reduce the patient’s symptoms and is
likely to obviate the need or drug treatment.
Surgical intervention or KHEs during the acute phase in early childhood is less requent than
reconstruction o the area or chronic brosis. KHEs may leave behind damaged integument that
can be improved surgically ( or example, contracture, discoloration, and abnormal skin contour).
Reconstruction is based on the type o de ormity. Contractures are released by local tissue rear-
rangements and/or skin gra ing. Abnormal areas o integument can be serially excised. u ed
angioma is closely related to a KHE and treated with the same pharmacotherapy; the lesion is
o en localized and thus more amenable to resection.

80
Part II: Vascular Tumors
in f a n t il e Myo f ib r o Ma
An in antile myo broma is a benign brous tumor, also re erred to as an in antile hemangioperi-
cytoma. T e lesion can be solitary, multi ocal (in antile myo bromatosis), or generalized, a ecting
the viscera. Most are present at birth, and 80% are diagnosed be ore 2 years o age. Lesions can
enlarge during in ancy and range rom 0.5 to 70 cm. In antile myo bromas usually involve the
integument and subcutis. Super cial lesions may be red purple and ulcerate. Subcutaneous tumors
can have normal overlying skin color. An in antile myo broma is di cult to diagnose clinically
and is usually mistaken or another lesion ( or example, an in antile hemangioma, a vascular
mal ormation, a brosarcoma, or a teratoma). Imaging ndings are nonspeci c, and a diagnosis
is typically made by histopathologic examination a er an excisional biopsy has been per ormed.
Almost all in antile myo bromas are excised, because the diagnosis is unclear. T e surgeon and
amily want the lesion identi ed histopathologically to ensure that it is not malignant. I an in-
antile myo broma is recognized clinically and radiographically, it does not need to be removed,
because it is benign, and 50% will undergo partial regression. A er 3 years o age, i a de ormity
remains, consideration is given to excision be ore the child’s memory and sel -esteem begin to
orm. Rarely a patient can have generalized in antile myo bromatosis with lesions involving the
heart, lungs, and gastrointestinal tract. T e mortality rate is high, and management o the vis-
ceral lesions includes resection, chemotherapy, and/or radiation. In antile myo bromas are more
vascular than other types o rare tumors, but bleeding is easily controlled during the resection.
a r t e r io v e n o u s a c r a l t u Mo r
An arteriovenous acral tumor is a benign lesion that appears similar to a pyogenic granuloma, an
arteriovenous mal ormation, or an Enzinger intramuscular hemangioma. T e lesion can develop
in children or adults; the median age o onset in children is 7 years. An arteriovenous acral tumor
is localized and has an average diameter o 0.5 mm; one ourth will bleed. Lesions are most com-
monly located on the lip or eyelid. An arteriovenous acral tumor is pinkish red and involves the
skin and subcutis. Almost all arteriovenous acral tumors are excised without margins, because
they are thought to be pyogenic granulomas, which are at risk o bleeding and causing a de ormity.
A diagnosis is typically made by the pathologist; reexcision to negative margins is unnecessary. I
the lesion is considered an arteriovenous acral tumor based on history and physical examination,
treatment is excision to prevent hemorrhage and improve the patient’s appearance. Resection o
an arteriovenous acral tumor is not associated with increased bleeding. Negative margins are
unnecessary, but the lesion can reenlarge i a subtotal excision is per ormed.
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 benign tumor o skeletal muscle is also re erred to as an Enzinger hemangioma, small-vessel
type, or an intramuscular hemangioma, capillary type. One ourth o lesions develop in the pediat-
ric population (median age at presentation is 25 years). T e tumor a ects the trunk (32%), head/
neck (30%), upper limb (23%), or lower extremity (15%). T e lesion presents as a painless so
tissue enlargement with normal overlying skin. Like in antile myo broma, almost all Enzinger
intramuscular hemangiomas are excised to obtain a histopathologic diagnosis, because they are
di cult to identi y clinically or radiographically. Patients also request resection to improve their
appearance. Enzinger intramuscular hemangiomas are not malignant, and thus negative margins
are not mandatory. T e extirpation and reconstruction should not leave a worse de ormity than
the appearance o the lesion. A er resection, there is approximately a 20% recurrence rate. Lesions
are not at increased risk o perioperative bleeding. I tumor removal is problematic, embolization
may reduce its size but is associated with a high recurrence rate.

81
Chapter 6: Rare Vascular Tumors
Fig. 6-2
A

B
PATIENT EXAMPLES
r a PiDl y 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
Most RICHs do not leave a residual de ormity a er they have completed regression at 1 year o age.
T is newborn in ant had a lesion o the trunk (A). At 6 months o age, the tumor has undergone
involution (B). An abnormality is di cult to appreciate at 12 months o age (C).
Pa tie n t B
Unlike in antile hemangiomas, which can leave excess subcutaneous adipose tissue a er involu-
tion, RICHs may cause at atrophy. Intervention or this tumor is rarely required during in ancy,
because it begins to regress immediately a er birth. T is newborn had a RICH o the upper
extremity (A). T e tumor regressed signi cantly at 6 months o age (B). I a child has a residual
de ormity, it can be improved with resection or at gra ing when he or she is older.
Fig. 6-1
A

B

C

82
Part II: Vascular Tumors
Pa tie n t C
T is boy had residual telangiectasias and at atrophy rom a RICH. He previously underwent
partial excision by another surgeon. Because the skin was a ected, his de ormity was improved
by serial excision. T e preoperative appearance (A) and intraoperative markings or the excision
(B) are shown. T e patient is seen a er wound closure (C). T e appearance is seen be ore another
staged resection to remove the remaining lesion and narrow the scar (D).
Fig. 6-3
A

B
C

D