Phẫu Thuật Quản Lý Và Điều Trị Dị Thường Mạch Máu

234
Part IV: Anatomic Considerations
Pa t ie n t c
T is 3-year-old boy presented with a large LM o the dorsal hand (A). Postoperative unctional
outcome was excellent (B) compared with the clinical appearance be ore excision. In the operating
room, an L-shaped incision was planned and per ormed under tourniquet control. T e lesion in
situ was care ully dissected rom the overlying dorsal sensory branches o the radial nerve (C).
T e lesion was excised, preserving the dorsal sensory branches o the radial nerve and extensor
tendons to the index nger and thumb (D).
COMPLICATIONS
T e requency o complications is directly proportional to the size and f ow characteristics o the
lesion. Larger and higher-f ow lesions tend to have more complications. T ese include compart-
ment syndrome, bleeding, wound dehiscence, in ection, devascularization o a limb or digit or
the skin, secondary contracture, tendon adherence, and nally pain resulting rom nerve injury.
Adherence to basic surgical principles as discussed in this chapter will minimize but not eliminate
these potential problems. Large vascular anomalies involving any portion o the upper extremity
are not impossible problems. Surgical treatment is o en the best remedy and should not be viewed
as the last resort option.
Fig. 15-5
C

D
A

B

235
Chapter 15: Hand and Upper Extremity
CONCLUSION
Vascular anomalies are a challenging group o diagnoses or the hand surgeon. A multidisciplinary
approach to diagnosis and management is critical to caring or these patients, because many o
the lesions are amenable to nonsurgical treatments. For lesions that can be treated with surgery,
the surgeon must have a systematic approach that involves care ul planning and counseling. T e
surgical points and pearls outlined in this chapter will help to avoid problems and ensure suc-
cess ul outcomes.
SUGGESTED READINGS
Malan E, Puglionisi A. Congenital angiodysplasias o the extremities. I. Generalities and classi cation; venous dys-
plasias. J Cardiovasc Surg ( orino) : , .
Malan E, Puglionisi A. Congenital angiodysplasias o the extremities. II. Arterial, arterial and venous, haemolym-
phatic dysplasias. J Cardiovasc Surg ( orino) : , .
Mulliken JB, Glowacki J. Hemangiomas and vascular mal ormations in in ants and children: a classi cation based
on endothelial characteristics. Plast Reconstr Surg : , .
Szilagyi DE, Smith RF, Elliott JP, et al. Congenital arteriovenous anomalies o the limbs. Arch Surg : , .
de akats G. Vascular anomalies o the extremities: report o ve cases. Surg Gynecol Obstet : , .
Upton J, Coombs C, Mulliken JB, et al. Vascular mal ormations o the upper limb: a review o patients. J Hand
Surg Am : , .
Upton J, Marler JJ. Vascular anomalies o the upper extremity. In Mathes S, ed. Plastic Surgery, vol . Philadelphia:
Saunders Elsevier, .
Upton J, Mulliken JB, Murray JE. Classi cation and rationale or treatment o vascular anomalies in the upper
extremity. J Hand Surg Am ( Pt ): , .

(Trang không có chữ)

237
Samantha A. Spencer, Arin K. Greene, Daniel Hedequist
Vascular anomalies that involve the vertebral column and lower extremity may require manage-
ment by an orthopedic surgeon. Most lesions are isolated vascular mal ormations ( or example,
capillary, lymphatic, venous, or arteriovenous) or overgrowth syndromes ( or example, congen-
ital lipomatous overgrowth, vascular mal ormations, epidermal nevis, spinal/skeletal anomalies/
scoliosis [CLOVES] syndrome, Klippel- renaunay syndrome, or Parkes Weber syndrome). Le-
sions involving the vertebral column can cause osteolysis and collapse o the vertebra, as well
as scoliosis. Vascular anomalies o the lower extremity can result in vertical or circum erential
overgrowth, leg-length discrepancy, hemarthrosis, contractures, pain, decreased ambulation, and/
or an inability to t clothing (see Video 16-1, Intraarticular Venous Mal ormation).
T e key to a treatment plan is a thorough history and physical examination. In the history the
surgeon must assess overall unction and prior treatments. T e patient should be asked i he or she
can walk community or household distances. T e surgeon should inquire i pain is problematic
and inter ering only with sports or with school, sleep, and mood. T e surgeon should also ask
Ver t ebr al Co l u mn
an d Lo w er Ext r emit y
Ch a Pt er 16
KEY POINTS
• The surgeon should understand the particular risks associated with each lesion and ensure
that patients undergo a multidisciplinary evaluation preoperatively.
• A contrast-enhanced MRI should be used to assess the skin-to-bone involvement of the
patient’s anomaly.
• The surgeon should monitor leg length and foot length inequality and angular lower extrem-
ity deformity and use epiphysiodesis/hemiepiphysiodesis before puberty if indicated.
• Intraarticular involvement should be treated aggressively before joint destruction and con-
tracture with sclerotherapy and arthroscopy.

238
Part IV: Anatomic Considerations
about bowel and bladder dys unction, back pain, and headaches, which can be signs o tethering
or spinal cord involvement. Any patient with these symptoms must have an MRI o the neural
axis be ore a surgical plan is made. T e surgeon should ask about bleeding and lymphatic drainage
rom areas o mal ormation and inquire about any prior episodes o blood clots or bleeding and
a amily history o these conditions. Finally, the surgeon must inquire i in ections have occurred
and record the severity and requency.
During the physical examination, a ull-body examination is important. Gait should be assessed
in ambulatory patients in the hallway (walking and running i possible). T e surgeon should
correct leg-length discrepancy by placing blocks under the shorter leg be ore an Adams orward
bend test o the spine is per ormed with a scoliometer or ree app to examine spinal asymmetry.
T e surgeon should determine whether a so tissue mass is creating imbalance or whether it is
truly a bony de ormity. T is can be di cult to determine on examination and usually requires
imaging. Standing pelvic balance and shoulder symmetry should be assessed. T e coronal and
sagittal alignment o the spine and lower extremities should be noted. T e surgeon should mea-
sure side-to-side thigh, cal , and oot circum erence di erence and leg-length discrepancy. Foot
size di erence and macrodactyly, syndactyly, and sandal gap should be noted. All joints should be
assessed or range-o -motion and angular de ormities, which are common. T e surgeon should
per orm a ull neurovascular examination.
Standing PA and lateral spinal lms with any leg-length discrepancy corrected with blocks are
a good start at assessing spinal de ormity. Patients with signi cant scoliosis, back pain, and/or
neurologic symptoms need a ull spine MRI. For those with high-f ow mal ormations ( or ex-
ample, arteriovenous mal ormations), angiography may be needed. Leg-length discrepancy can
be assessed with a standing AP hips-to-ankles radiograph; newer low-dose systems such as EOS
make these lms accurate and provide less radiation exposure. Because oot height and bulk o en
contribute greatly to a signi cant leg-length discrepancy (anything greater than 2 cm/approaching
1 inch) in vascular anomalies, standing lms are pre erred to lying down scanogram measurement
radiographs with a ruler, which do not include the oot.
For patients with so tissue overgrowth, joint contractures, or pain, a contrast-enhanced MRI o
the a ected part is critical or surgical planning. T is can reveal the presence o anomalous vessels,
intraarticular involvement, and the extent o deep disease in the musculature and around nerves.
SURGICAL INDICATIONS
v e r t e b r a l c o l u Mn /s Pin e
Scoliosis in the general pediatric population is generally observed until the curve reaches 25 de-
grees and braced until 40 to 50 degrees. T is is the point at which curvature bracing is ine ective
and curve progression continues a er skeletal maturity, which is why this is the threshold or spi-
nal straightening and instrumented usion. However, vascular mal ormations do not ollow these
rules. Most curves result rom intrathecal involvement (commonly seen in CLOVES syndrome)
or vertebral column structural instability and collapse ( or example, lymphatic mal ormations
and Gorham-Stout disease). T us curve progression is common. Spinal usion stabilization is
considered or progressive curvature o more than 40 degrees. Neurosurgical intervention or
spinal cord detethering, decompression, and resection o intrathecal masses is much more com-
mon in the vascular mal ormation population than in the idiopathic scoliosis population. Spinal
stabilization is also considered or instability and ractures.

239
Chapter 16: Vertebral Column and Lower Extremity
l e g -l e n g t h a n D f o o t -l e n g t h Dif f e r e n c e
A leg-length di erence o greater than 2 cm causes gait disturbance, pelvic imbalance, and sec-
ondary scoliosis and can be a later source o leg and back pain. Although shoe li s are e ective,
this amount o di erence requires a build-on plat orm to each walking shoe. T is is di cult and
tedious to maintain or a li etime, particularly i the plat orm is several inches, which leads to
ankle sprains and di culty with uneven sur aces. During childhood, annual clinical examinations
and shoe li s are recommended. As patients approach puberty, their nal predicted leg length
and oot length can be estimated accurately by widely available tools such as the Green-Anderson
or the Paley Multiplier method. Apps are now available that can also assist. Because major leg
lengthening on the una ected limb or emoral shortening on the overgrown side is much riskier
and morbid in this group, guided growth or epiphysiodesis is the most widely recommended
technique that has good success i used in time on the overgrown part.
a n g u l a r De f o r Mit y
Angular de ormity such as genu valgum/knock knees or genu varum/bow legs can occur. When-
ever possible, guided growth/hemiepiphysiodesis with implantable devices to tether one side o
the physis should be used to avoid the morbidity o osteotomy.
in t r a a r t ic u l a r in vo l v e Me n t
When vascular mal ormations occur around joints, they can invade the capsule and cause recur-
rent hemarthrosis and eventual end-stage arthritis and contracture. I diagnosed early, aggressive
sclerotherapy and synovectomy can stop this cycle. Whenever possible, arthroscopic evaluation
and synovectomy with cauterization o intraarticular mal ormation should be used. Very ew pa-
tients with vascular mal ormations are joint replacement candidates because o the nature o the
so tissue envelope. T ere ore a severely arthritic contracted knee o en warrants an amputation.
Jo in t c o n t r a c t u r e
In young li e early contractures can be managed with physical therapy. O en the contracture is
driven by deep muscle and joint disease, which should be treated aggressively when possible with
interventional radiology injection and cryotherapies and arthroscopic ablation. Botox and serial
casting are not indicated, because the muscles are not spastic. In select cases tendon lengthening
is help ul. I there is ocal muscle involvement with pain and contracture, muscle resection can
be bene cial.
s o f t t is s u e o v e r g r o w t h
By 2 years o age, children with overgrowth mal ormation syndromes ( or example, CLOVES
syndrome or Klippel- renaunay syndrome) should see a multidisciplinary team trained in vas-
cular anomalies. Assessment or anomalous veins should be done with closure in interventional
radiology. T e amily should be educated about compressive wrapping o lymphedema in limbs
and later compression garments where appropriate. For severe cases, oral sirolimus and other
drug therapies should be maximized according to the recommendations o the team’s hematology
and oncology members. Children with CLOVES syndrome should be put on a renal ultrasound
Wilms tumor screening protocol. For patients with massive overgrowth that limits ambulation,
tting o clothing, and use o limbs, debulking is recommended. A staged approach is used, with
the most a ected part worked on rst and in limbs rom distal to proximal. For massively over-
grown, non unctional limbs or or those creating a parasitic limb or cardiac ailure, amputation
is recommended.

240
Part IV: Anatomic Considerations
SURGICAL MANAGEMENT
v e r t e b r a l c o l u Mn /s Pin e f u s io n
T e surgeon should assess the type o mal ormation and per orm three-dimensional imaging
and planning. T e use o titanium implants will allow a postoperative MRI. Many patients have
signi cant anomalous venous systems, and thus all patients should be evaluated by hematology
and interventional radiology be ore surgery. Many patients need preoperative embolization with
in erior vena cava (IVC) lter placement. Bleeding should be expected and mitigated with preop-
erative embolization, normalization o D-dimer and brinogen (o en with preoperative antico-
agulation with enoxaparin sodium [Lovenox]), and intraoperative use o special cautery devices.
O en a combined team approach with neurosurgery, orthopedics, and plastic surgery is help ul.
e PiPh ys io De s is /h e Mie PiPh ys io De s is
Although epiphysiodesis/hemiepiphysiodesis is a minor operation, these patients still need pre-
operative assessment and closure o anomalous veins ( or example, the lateral marginal vein o
Servelle in patients with Klippel- renaunay syndrome) and maybe perioperative anticoagulation
with a preoperative hematology consultation. Epiphysiodesis involves either percutaneous or
mini-open drilling/curettage o the physis or transphyseal screw placement. Hemiepiphysiodesis
involves partial tethering or closure o one side o the physis, usually with an implanted device
to correct angular de ormity.
T ese techniques should always be per ormed under a tourniquet in patients with vascular anom-
alies. A radiolucent table and intraoperative f uoroscopy are essential. T e small wounds (o en
5 mm or epiphysiodesis and 2 to 3 cm or hemiepiphysiodesis) should be closed with clear PDS
and compressed or 24 hours with a bulky dressing/compressive wrap. T is technique is highly
success ul with minimal morbidity; however, it takes 1 to 3 years to see the e ect as the contra-
lateral part catches up in length.
a r t h r o s c o Py
Supine arthroscopy with a tourniquet is suggested. A pressurized inf ation saline system and
appropriate cautery and ablation tools are essential. I there is extensive involvement, an over-
night drain with a compressive wrap can be used. T e small scope portals can be closed either
with nylon or buried sutures. Multiple small portals can be used to access the mal ormation; or
example, the surgeon can use several suprapatellar portals to access the at pad mal ormation and
gutter mal ormation.
f o o t De f o r Mit y /o v e r g r o w t h
T e ability to wear o -the-shel shoes is o en a stated goal o patient and amilies. o accomplish
this, various techniques may be used. Length may be reduced by metatarsal/proximal phalanx
epiphysiodesis done in mini-open direct curettage ashion. A stapes curette rom the otolaryngol-
ogy surgery kits is help ul. Macrodactylous toes must be assessed or unction; debulking rarely
achieves lasting success, and ray resection is most de nitive. Dorsal overgrowth can be debulked
but may recur. T e best treatment or eet with signi cant toe overgrowth may be amputation
at the metatarsal phalangeal joints, whereas the best treatment or extreme overgrowth with a
dys unctional sole o the oot may be a Syme amputation.

241
Chapter 16: Vertebral Column and Lower Extremity
Mu s c l e r e s e c t io n v e r s u s a MPu t a t io n
T ree-dimensional imaging is essential to assess the extent o deep involvement in a limb. I there
is a ocal lesion or involvement o a single compartment, resection can be highly success ul and
bene cial. Although amputation is always a last resort, it can return a patient to high unctionality.
Patients and amilies must be counseled about the known natural history o the disease and the
current range o treatment options available. Redundant muscles in the lower extremity can be
sa ely resected without the need or reconstruction with ree f aps. For example, the gastrocnemius
or soleus muscle can unction alone, and resection o the gluteus maximus is well tolerated i the
gluteus medius is intact. Patients with extensive involvement, particularly multicompartmental or
with nerve involvement on imaging, are not good candidates or resection. T e surgeon should use
a sterile tourniquet whenever possible, have special cauterization tools such as the Aquamantys
system available, and plan or a wide exposure. Usually the surgeon should resect the entire a -
ected muscle i it is redundant under the tourniquet and then drop the tourniquet and achieve
hemostasis. Intraoperative placement o nerve block catheters is help ul. We suggest a care ul
layered closure over drains with the subdermal and skin layers per ormed with clear, small 3-0
and then 4-0 PDS. T e surgeon should leave the drains in with a compressive wrap until output is
under 15 cc per day and continue compressive dressings or 4 to 6 weeks. Amputation should be
considered or dys unctional, pain ul, or severely overgrown parasitic limbs without other options.
s o f t t is s u e De b u l k in g
Excision o excess so tissue can be per ormed with suction-assisted lipectomy (liposuction)
or excision o skin and subcutaneous tissue. T e surgical approach is determined by the type
o vascular anomaly and the location o overgrown tissue. An MRI is per ormed to con rm the
diagnosis and illustrate what structures are causing the overgrowth ( or example, bone, muscle,
or subcutaneous tissue). Only skin and subcutaneous tissue can be removed; we do not advocate
resection o intra ascial overgrowth because o the morbidity associated with muscle extirpation.
Generally, liposuction is pre erred or lesions that are not extremely vascular and primarily a ect
the subcutaneous tissue ( or example, capillary mal ormation, adipose hypertrophy in CLOVES
syndrome, microcystic lymphatic mal ormation, and lymphedema). Liposuction can only remove
tissue in the subcutaneous plane and is not indicated or lesions that are signi cantly vascular
because o di culty controlling bleeding ( or example, venous mal ormations or arteriovenous
mal ormations). Liposuction is a minimally invasive method to remove subcutaneous tissue, be-
cause it requires only small (1 cm) incisions to insert the cannulas. A er the subcutaneous tissue
is removed, the skin will contract to urther reduce the size o the extremity.
Skin and subcutaneous resection is indicated or patients with signi cant cutaneous involvement,
vascular lesions that require meticulous hemostasis, or or patients with excess skin that requires
removal ( or example, Klippel- renaunay syndrome, venous mal ormations, and arteriovenous
mal ormations). Localized areas can be removed in one stage, and skin f aps do not exceed one
hal o the leg circum erence to prevent skin necrosis. Muscle ascia typically is not removed.
ourniquets are e ective to reduce blood loss. Di use extremity overgrowth requires staged ex-
tirpations. An incision is made on one side o the extremity, skin f aps are raised no more than
one hal the circum erence, tissue between the muscles and skin is removed, and redundant skin
is excised over drains. At least 3 months later, the procedure is repeated along the other side o the
extremity. Compared with liposuction, “open” resection o so tissue is associated with greater
morbidity and postoperative recovery.

242
Part IV: Anatomic Considerations
PATIENT EXAMPLES
s Pin a l De f o r Mit y
T is 10-year-old boy with CLOVES syndrome had progressive severe scoliosis with a neuro-
genic bladder and an acquired club oot (A). He had undergone previous detethering and truncal
debulking. Paraspinal vascular mal ormations had appropriate embolization and repeat spinal
cord detethering, and an IVC lter was placed preoperatively (B). During initial spinal usion,
acute hypotension and desaturation occurred, and the procedure was aborted. A workup deter-
mined that a pulmonary embolus had occurred through anomalous veins o the chest wall. T ese
were treated. Eventually, spinal usion was success ul 5 months later. Subsequently, the patient
required so tissue release o the acquired club oot de ormity, revision o spinal instrumentation
as a result o junctional kyphosis, and multiple embolizations and treatments o urther upper
extremity and truncal vascular mal ormations.
Fig. 16-1, A and B
A

B

243
Chapter 16: Vertebral Column and Lower Extremity
Seven years a er surgery, a PA spinal usion radiograph showed the coils in the anomalous axil-
lary veins (C), and a lateral standing radiograph demonstrated good sagittal plane balance (D).
Le ankle club oot release is seen, but the oot is plantigrade again and unctional (E). He is
short-distance community ambulatory with some weakness, wears o -the-shel clothes/shoes,
has a partially neurogenic bladder (incomplete emptying success ully managed with intermit-
tent sel -catheterization), is on li elong war arin anticoagulation but attends a university inde-
pendently, and has a good quality o li e.
ake-home points in spinal de ormity in vascular mal ormation syndromes include the ollowing:
• T e surgeon should always obtain MRI imaging o the entire spine and have neurosurgery
review it.
• Bracing can be tried but is usually not e ective or progressive spinal de ormity.
• Repeated neurologic examinations are essential. A decline in unction mandates urther
workup or intrathecal disease/spinal cord tethering. T is is not always completely treatable.
• Anomalous ectatic veins should be closed preoperatively with IVC lter placement in those
with a signi cant clotting risk.
• During spinal usion, neuromonitoring should be placed be ore surgical incision. Prepara-
tions or high blood loss should be made. Appropriate blood products should be available
with appropriate IV access and an arterial line in place, Cell Saver, special cautery devices,
and direct anticoagulation agents (such as Gel oam).
• Postoperatively ICU management is suggested initially.
• Patients should be counseled regarding the additional surgical risks because o their vascular
mal ormation, in addition to the standard spinal usion risks.
C

D

E
Fig. 16-1, C-E

244
Part IV: Anatomic Considerations
f o o t o v e r g r o w t h
T is 6-year-old boy had CLOVES syndrome and severe bilateral ore oot overgrowth with mac-
rodactyly (A through C). Shoe wearing was di cult (only open-toe adult-size sandals or cus-
tom shoes) with marked toe overgrowth. T is was treated by bilateral amputation o all toes
through the metatarsal phalangeal joints and concomitant metatarsal epiphysiodesis (D through
G). Postoperative cast immobilization or children provides a sterile dressing and compression
and prevents equinus (H). T e goals o this surgery were walkable, shoeable eet and prevention
o urther overgrowth, which were achieved. I possible, through-joint amputations are pre erred
in children to prevent bony stump overgrowth and spikes.
Fig. 16-2
D

E

F

G
H
A

B

C

245
Chapter 16: Vertebral Column and Lower Extremity
in t r a a r t ic u l a r v a s c u l a r Ma l f o r Ma t io n
Intraarticular venous mal ormation should be treated to prevent recurrent hemarthrosis and
arthritis. T is can be per ormed with arthroscopic cautery devices.
T is 10-year-old girl had a venous mal ormation o the lower extremity with intraarticular in-
volvement. T e patella had synovitis and a venous mal ormation in the suprapatellar pouch (A).
Healthy patello emoral articulation, patella (top,) and trochlea (bottom) are seen (B). She had
extensive synovitis and a venous mal ormation in the intercondylar notch and at pad (C and D),
which obscured the medial and lateral compartments. A medial emoral condyle with a venous
mal ormation in the medial gutter abutting the medial meniscus was seen (E). A cautery tool was
inserted through the medial parapatellar portal (F).
Fig. 16-3, A-F
A

B
C

D
E

F

246
Part IV: Anatomic Considerations
An intraarticular venous mal ormation is shown be ore treatment (G). T e appearance is seen
a er treatment (H). T e superior aspect o the Ho a at pad is shown be ore treatment (I and J).
T e Ho a at pad and intercondylar notch are seen midtreatment with arthroscopic cauterization
and synovectomy (K and L). T e intercondylar notch is shown midtreatment (M and N). T e
intercondylar notch is demonstrated a er treatment (O).
Fig. 16-3, G-O
G

H
I

J
K

L
M

N

O

247
Chapter 16: Vertebral Column and Lower Extremity
Synovitis in the in rapatellar at pad is seen (top) with a minor amount o venous mal ormation
(P and Q). T e trochlear groove is visible (bottom). T e in rapatellar at pad is shown af er treat-
ment (R). T e patella is superior, and the trochlear groove is in erior. T e clear lateral compart-
ment view is seen af er treatment (S). T e emur (top), the lateral meniscus (central), and lateral
tibial plateau (inferior) are seen. T e clear, normal medial compartment view is shown af er
treatment (T). T e emur (top), medial meniscus to the lef , and tibial plateau at bottom o the
photo are seen. T e treated synovium is seen (U). T e cautery tool is shown against a normal at
pad af er removal o the venous mal ormation (V).
Fig. 16-3, P-V
P

Q
R

S
T

U

V

248
Part IV: Anatomic Considerations
in t r a Mu s c u l a r v a s c u l a r Ma l f o r Ma t io n
T is 18-year-old male had an extensive broadipose vascular anomaly o the leg, mostly extra as-
cial posterior cal and extensive gastrocnemius involvement. A er sclerotherapy with glue and coil
embolization ailed, he underwent resection. T e procedure was per ormed in the prone position
under a sterile tourniquet (A and B). Exposure o the mal ormation showed extensive super cial
veins, which were treated with Aquamantys bipolar cautery (C and D).
Fig. 16-4, A-D
A
B
C

D

249
Chapter 16: Vertebral Column and Lower Extremity
A Cell Saver was used. T e diseased gastrocnemius muscle was dissected ree down to the main
neurovascular pedicle, which was stick tied, cauterized, and then cut (E). A healthy soleus muscle
had drains in place (F). Large round Blake drains were placed. A sciatic nerve catheter wire was
placed or postoperative pain control. Final multilayer closure with PDS was used or subdermal
and subcuticular layers, because tissue-containing vascular mal ormations can delay healing and
lead to late dehiscence (G). T e wound was dressed with a bulky Jones dressing and splint to
prevent equinus until the drains were removed (less than 15 cc/day, o en 1 to 2 weeks postopera-
tively) (H). T e patient then transitioned to a boot. Gastrocnemius muscle resection injected in
pathology showed extensive vasculature (I).
Fig. 16-4, E-I
E
G

H

I
F

250
Part IV: Anatomic Considerations
Dif f u s e l o w e r e x t r e Mit y o v e r g r o w t h
T is 23-year-old man had progressive, massive le lower leg and oot lymphaticovenous over-
growth consistent with Klippel- renaunay syndrome (A and B). He was wheelchair bound and
unable to wear o -the-shel clothing or shoes. He requested an attempt at debulking (C), which
ailed because o f ap necrosis and bleeding (D). Preoperative closure o anomalous large veins
and IVC lter placement were done because o the clotting risk. Preoperative and postoperative
enoxaparin was given. He then agreed to above-knee amputation. Initially a loose closure with
a central open area was done with large simple sutures; this was closed 1 week later. Healing
or prosthetic tting took 3 months. T e postoperative course was complicated by heart ailure,
which resolved completely with medical support. T is was presumed to be the result o rebound
hypertension rom his previous parasitic limb and enlarged blood volume. T e healed stump
with silicone sleeve and prosthetic is shown 6 months a er surgery (E). He was able to ambulate
independently a er a prosthetic tting and to wear normal-sized clothes and shoes (F).
Fig. 16-5
A
B
C

D
E

F

251
Chapter 16: Vertebral Column and Lower Extremity
COMPLICATIONS
c l o t t in g v e r s u s b l e e Din g
o avoid complications, the surgeon must understand the underlying vascular anomaly. In certain
syndromes such as CLOVES and Klippel- renaunay, anomalous veins are a prominent eature
and increase the risk o deep vein thrombosis and pulmonary embolism. T us preoperative em-
bolization, IVC lters, and preoperative and postoperative anticoagulation are help ul. However,
even with these precautions, complications can still happen. T e clotting risk must be balanced
with bleeding, which can be substantial. Most invasive procedures in these patients require an ICU
stay. Hematology input on trans usion parameters is help ul, because most o the blood products
end up in the mal ormation and not centrally (the parasitic limb phenomenon).
w o u n D b r e a k Do w n a n D in f e c t io n
In patients with skin involvement, most will have some amount o breakdown in larger operations
with extensive incisions. T e use o PDS in skin closure, wide based f aps without tension that
are adequately drained to prevent underlying hematoma-causing skin necrosis, and compressive
wraps/compression stockings are help ul. Most breakdown can be managed by switching to wet-
to-dry dressings, with debridement reserved or large amounts o devitalized tissue or in ection.
Patients and amilies must be counseled that the healing by secondary intention is slow and
may take weeks but can usually be achieved with outpatient wound care. T e use o incisional
vacuum-assisted closure sponges can be help ul over closed wounds or compression/drainage
but should be used with caution because o deep bleeding risk. In persistent leaking lymphatic
vesicles near wounds, a CO2 laser can be help ul.
r e c u r r e n t Dis e a s e
All the techniques discussed may ultimately ail i the vascular mal ormation recurs. Although it
can be di cult to estimate this or a particular patient and amily, this must be mentioned. For
example, in a more di usely involved limb undergoing muscle resection or ocal pain or knee
arthroscopy or intraarticular disease, recurrence that may later require amputation can occur.
It is o en help ul to mention that recurrence will be addressed, with a urther multidisciplinary
evaluation o all treatment options.
CONCLUSION
Vascular mal ormations and overgrowth syndromes commonly a ect the vertebral column and
lower extremity. With proper assessment o the exact type o mal ormation, the degree o deep
structure involvement, and the amount o morbidity, excellent surgical treatment options exist.
T e overall goals are: (1) a neurologically intact stable spinal column with minimal de ormity,
(2) a balanced pelvis in stance/gait, (3) well-aligned unctional legs/ eet, and (4) the ability to t
clothing.

252
Part IV: Anatomic Considerations
SUGGESTED READINGS
Alomari AI, Spencer SA, Arnold RW, et al. Fibro-adipose vascular anomaly: clinical-radiologic-pathologic eatures
o a newly delineated disorder o the extremity. J Pediatr Orthop : , .
Hedequist D, Spencer S, Richards BS, et al. Surgical treatment o spinal de ormity in patients with CLOVES syn-
drome: a report o cases. J Pediatr Orthop : , .
Kulungowski AM, Fishman SJ. Management o combined vascular mal ormations. Clin Plast Surg : , .
Spencer SA, Sorger J. Orthopedic issues in vascular anomalies. Semin Pediatr Surg : , .
Uller W, Fishman SJ, Alomari AI. Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg
: , .

253
c r eDit s
Chapter 1
Table 1-2 Data rom Wasse M, Blei F, Adams D, et al. Vascular anomalies classif cation: rec-
ommendations rom the International Society or the Study o Vascular Anomalies. Pediatrics
136:3203, 2015.
Chapter 3
Table 3-1 Adapted rom Mulliken JB, Anupindi S, Ezekowitz RA. Case 13-2004: a newborn girl
with a large cutaneous lesion, thrombocytopenia and anemia. N Engl J Med 350:1764, 2004.
Chapter 11
Table 11-1 Data rom Spetzler RF, Martin NA. A proposed grading system or arteriovenous
mal ormations. J Neurosurg 65:476, 1986.
Chapter 12
Fig. 12-2 Courtesy o John B. Mulliken, MD. From Mulliken JB, Burrows PB, Fishman SJ, eds.
In Mulliken and Young’s Vascular Anomalies: Hemangiomas and Mal ormations, ed 2. New York:
Ox ord University Press, 2013. By permission o Ox ord University Press.
Chapter 13
Fig. 13-2 From Rahbar R, Rowley H, Perez-Atayde AR, et al. Delayed presentation o lymphatic
mal ormation o the cervico acial region: role o trauma. Ann Otol Rhinol Laryngol 111:828, 2002.
Chapter 14
Fig. 14-5 From Fishman SJ. Truncal, visceral, and genital vascular mal ormations. In Mulliken
JB, Burrows PE, Fishman SJ, eds. Mulliken and Young’s Vascular Anomalies: Hemangiomas and
Mal ormations, ed 2. New York: Ox ord University Press, 2013. By permission o Ox ord Univer-
sity Press.

(Trang không có chữ)

255
In d ex
A
Abdomen, vascular anomalies
complications o ,
overview o ,
patient example o ,
surgical indications or, -
surgical management o , -
Acral tumor, arteriovenous, , -
Activated partial thromboplastin time (aP ), , ,

Airway, vascular mal ormations
complications with,
overview o ,
patient examples o , -
surgical indications or,
surgical management o , -
Amputation
o auricle,
o lower limb, , , , , ,
o upper extremity,
Angiomas, cavernous; see Cavernous mal ormation(s)
Angular de ormity,
Anticoagulation,
Arteriovenous acral tumor, , -
Arteriovenous mal ormation(s) (AVM), -
o central nervous system
complications with,
grading o , ,
overview o ,
patient examples o ,
surgical indications or,
surgical management o , -
classi cation o ,
o cranio acial bones, , ,
di use, , - , -
o ear, ,
embolization o , ,
acial, - , -
o orehead,
o hand,
o lip, , ,
localized, , -
o lower extremity, ,
management o ,
mutations associated with,
natural history o , -
o neck,
nidus o ,
o nose,
overview o , -
recurrence o , ,
regional, - , -
o scalp, - ,
stages o , ,
surgical intervention or, -
complications o , -
indications or, -
patient examples o , -
principles o , ,
timing o , -
treatment algorithm or,
types o ,
Arthroscopy,
B
Bleeding, laboratory assessment o , -
Bleeding risk, management o perioperative, -
Blood f ow, slowed,
Blue rubber bleb nevus syndrome (BRBNS),
venous mal ormations o abdomen in, , , ,

C
Capillary hemangioma,
lobular; see Pyogenic granuloma
Capillary mal ormation(s) (CM)
o cheek, ,
classi cation o ,
clinical eatures o ,
complications with,
o cranio acial bones, ,
o extremity, , -
o ace, , -
o lip, - , -
management o ,
mutations associated with, ,
natural history o ,

256

Index
CM; see Capillary mal ormation(s); Cavernous mal or-
mation(s)
CNS (central nervous system), venous mal ormation
arteriovenous, -
cavernous, -
overview o , -
Coagulopathy
consumptive,
dilutional,
laboratory assessment o , -
in vascular anomalies, -
Coblation or cervico acial vascular mal ormation,
-
Congenital hemangioma
classi cation o , ,
management o ,
noninvoluting, , -
patient examples o , -
rapidly involuting, - , -
surgical intervention or, , -
terminology or,
Congenital pulmonary lymphangiectasia,
Consumptive coagulopathy,
Contraception, hormonal,
Cranio acial bones, vascular anomalies
arteriovenous mal ormation as, , ,
capillary mal ormation as, ,
complications with,
lymphatic mal ormation as, , - ,
overview o ,
patient examples o , -
summary o ,
surgical indications or,
surgical management o , -
vascular tumor as, , ,
venous mal ormation as, - , - ,
Cranium; see Cranio acial bones
Cutaneomucosal venous mal ormation, , ,

Cystic hygroma,
D
D-dimer, , ,
Developmental venous anomaly (DVA),
Diagnosis and management,
Di use phlebectasia o Bockenheimer,
Dilutional coagulopathy,
“Disappearing bone disease,” ,
E
Ear
arteriovenous mal ormations o , ,
in antile hemangioma o , ,
Capillary mal ormation(s) (CM) —cont’d
overview o , -
patient examples o , -
with pyogenic granuloma, ,
o scalp,
surgical indications or, -
surgical intervention or, , -
o trunk, ,
Carbon dioxide laser
or cervico acial vascular mal ormation,
or microcystic lymphatic mal ormation,
Cautery
or microcystic lymphatic mal ormation,
or pyogenic granuloma, , -
Cavernomas; see Cavernous mal ormation(s)
Cavernous mal ormation(s) (CM)
o central nervous system
complications with,
overview o ,
patient example o ,
surgical indications or, -
surgical management o ,
classi cation o ,
Central nervous system (CNS), venous mal ormation
arteriovenous, -
cavernous, -
overview o , -
Cerebral cavernous mal ormation,
Cervico acial vascular mal ormations
complications with,
overview o ,
patient examples o , -
surgical indications or,
surgical management o , -
Cheek
capillary mal ormations o , ,
in antile hemangioma o , , , -
microcystic lymphatic mal ormations o , o cheek,
, -
overgrowth syndromes o , ,
venous mal ormations o ,
Chest, vascular anomalies o
complications o ,
overview o ,
surgical indications or,
surgical management o ,
“Circling the wagons,”
Classi cation o vascular anomalies, -
Clitoris, vascular anomalies o ,
CLOVES syndrome
perioperative thrombotic risk with, -
vertebral column involvement in,
patient examples o , ,
surgical indications or, ,

257
Index
dissection in bloodless eld in,
overview o , -
patient counseling or,
patient examples o , -
patient ollow-up or, -
placement o incisions or, -
postoperative immobilization or,
preoperative planning or,
surgical indications or,
surgical instruments or, -
surgical management o , -
tagging normal structures with,
thorough excision o , -
wound closure or,
Hemangioendothelioma
epithelioid,
kaposi orm
classi cation o , ,
o cranio acial bones, , ,
epidemiology o ,
hematologic abnormalities in, ,
management o ,
natural history o ,
patient examples o , -
platelet trans usion in,
surgical intervention or, ,
terminology or,
Hemangioma(s)
capillary,
lobular; see Pyogenic granuloma
cavernous, ; see also Cavernous mal ormation(s)
o chest cavity and abdomen,
congenital
classi cation o , ,
management o ,
noninvoluting, , -
patient examples o , -
rapidly involuting, - , -
surgical intervention or, , -
terminology or,
Enzinger intramuscular, ,
hepatic,
in antile; see In antile hemangioma
strawberry,
Hemangiomatosis,
Hemangiopericytoma, in antile, ,
Hematologic abnormalities in vascular anomalies, -
Hemiepiphysiodesis,
Hemostasis, -
Hepatic hemangiomas,
Hepatic venous mal ormations,
Hormonal contraception,
Hygroma, cystic,
Hypercoagulability, ,
Hyper brinolysis,
Embolization, -
o arteriovenous mal ormations, ,
o cervico acial vascular mal ormation,
preoperative,
Endoscopic surgery or cervico acial vascular mal or-
mation,
Endothelial damage, -
Enzinger intramuscular hemangioma, ,
Epiphysiodesis,
Epithelioid hemangioendothelioma,
Ex utero intrapartum treatment (EXI ) o cervico a-
cial vascular mal ormations, ,
F
Face
capillary mal ormations o , , -
microcystic lymphatic mal ormations o , - ,
overgrowth syndromes o , , -
venous mal ormations o , , , ,
Facial bones; see Cranio acial bones
Familial congenital primary lymphedema, mutations
associated with,
Fast-f ow lesions, ,
Female genitalia, vascular mal ormations o , ,
Fibrinogen, , ,
Fibrinolysis,
Fibroadipose vascular anomaly, , ,
Foot de ormity/overgrowth, , ,
Foot-length di erence,
Forehead
arteriovenous mal ormations o ,
in antile hemangioma o , , ,
G
Gastrointestinal venous mal ormations, ,
Generalized lymphatic anomaly, ,
Genital lymphedema,
Genitalia, vascular anomalies
complications o ,
overview o ,
patient examples o , ,
surgical indications or,
surgical management o ,
Glomuvenous mal ormation, , ,
Gorham-Stout disease, ,
Grading o arteriovenous mal ormations, ,
Granuloma, pyogenic; see Pyogenic granuloma
H
Hand, vascular anomalies
avoiding intraneural dissection with,
avoiding partial dissection with,
complications with,

258

Index
In antile myo broma, ,
Intraarticular venous mal ormation, , -
Intralesional pharmacotherapy,
Intramuscular hemangioma, capillary type, ,
Intramuscular vascular mal ormation, -
J
Joint contracture,
K
Kaposi orm hemangioendothelioma (KHE)
classi cation o , ,
o cranio acial bones, , ,
epidemiology o ,
hematologic abnormalities in, ,
management o ,
natural history o ,
patient examples o , -
platelet trans usion in,
surgical intervention or, ,
terminology or,
Kasabach-Merritt phenomenon, , , ,
Klippel- renaunay syndrome, , , , , , - ,

L
Labia, vascular anomalies o , ,
Laser surgery,
or cervico acial vascular mal ormation,
Leg-length di erence,
Lip
arteriovenous mal ormations o , , ,
capillary mal ormations o , - , -
in antile hemangioma o , - , - , -
microcystic lymphatic mal ormations o , -
overgrowth syndromes o , - , -
venous mal ormations o , ,
Lobular capillary hemangioma; see Pyogenic granuloma
Lower extremity, vascular anomalies o , -
arteriovenous mal ormations as, ,
complications with,
in antile hemangioma as, ,
microcystic lymphatic mal ormations as, - ,
, , -
overview o , -
patient examples o , -
surgical indications or,
surgical management o , -
venous mal ormations as, , , ,
Lymphangiectasia, congenital pulmonary,
Lymphangioma,
Lymphangiomatosis, ,
I
In antile hemangioma
o cheek,
involuted, -
proli erating,
classi cation o , ,
o ear, ,
epidemiology o ,
o orehead,
involuted,
proli erating,
involuted,
o cheek, -
o ear,
o orehead,
o lip, -
o neck, -
o nose, -
o scalp, -
o trunk, - , -
o upper extremity, -
o lip, -
lower, -
upper, -
o lower extremity, ,
management o ,
natural history o ,
o neck, , -
nonsurgical treatment o ,
o nose, , -
proli erating, - , -
o scalp, , -
involuted, -
proli erating, -
surgical indications or,
surgical intervention or, , -
anatomic considerations with, -
with circular excision, -
complications o ,
with lenticular excision, -
patient examples o , -
principles o ,
with serial excision,
with suction-assisted lipectomy, , -
timing o , -
terminology or,
o trunk,
involuted, -
suction-assisted lipectomy or,
o upper extremity,
involuted, -
proli erating,
suction-assisted lipectomy or,
In antile hemangiopericytoma, ,

259
Index
primary
amilial congenital,
overview o ,
patient example o ,
surgical indications or,
surgical management o , -
M
Male genitalia, vascular mal ormations o ,
Mal ormations, ,
Management
diagnosis and,
morbidity and,
options or, -
physician pre erence and,
principles o , -
variables that determine, -
Mediastinal lymphatic mal ormations,
Morbidity and management,
Muscle resection o lower limb,
Mutations associated with vascular anomalies,
Myo broma, in antile, ,
N
Neck
arteriovenous mal ormations o ,
in antile hemangioma o , , -
Neodymium-doped yttrium aluminum garnet
(Nd:YAG) laser or cervico acial vascular mal-
ormation,
Nidus,
Noninvoluting congenital hemangioma (NICH), ,
-
Nose
arteriovenous mal ormations o ,
in antile hemangioma o , , -
O
Observation,
Oral cavity, vascular mal ormations
complications with,
overview o ,
patient examples o , -
surgical indications or,
surgical management o , -
Overgrowth syndromes
rom capillary mal ormations, -
o cheek, ,
classi cation o ,
clinical eatures o ,
complications with,
o ace, , -
Lymphatic anomaly, generalized, ,
Lymphatic mal ormation(s) (LM), -
abdominal, , -
cervico acial, -
o chest, , ,
classi cation o ,
combined, ,
complications with,
o cranio acial bones, , - ,
generalized lymphatic anomaly as,
genital,
Gorham-Stout disease as,
o hand,
intraabdominal,
macrocystic
overview o ,
patient examples o , -
reconstruction a er sclerotherapy or,
resection a er sclerotherapy or, -
resection without sclerotherapy or,
surgical indications or,
surgical management o ,
management o ,
microcystic
carbon dioxide laser or,
cautery or,
o cheek, , -
o chin,
complete resection o , -
o ace, - ,
intraoral, ,
o lip, -
o lower extremity, - , , , -
overview o ,
patient examples o , -
subtotal resection o , -
surgical indications or,
surgical management o , -
o upper extremity,
mutations associated with,
overview o , -
patient examples o , -
phenotypes o ,
primary lymphedema as
overview o ,
patient example o ,
surgical indications or,
surgical management o , -
surgical indications or, -
surgical intervention or, , -
Lymphedema
genital,
penile, ,

260

Index
surgical management o , , -
anesthesia or,
cautery or, , -
circular closure in, ,
closure by secondary intention in, ,
complications o ,
control o lesion prior to,
ull-thickness skin excision or, - , -
linear closure in, , -
partial excision and ull-thickness cautery or,
-
patient examples o , -
timing o intervention or,
terminology or, ,
R
Radio requency ablation (RFA) or cervico acial vas-
cular mal ormation, ,
Rapidly involuting congenital hemangioma (RICH),
- , -
S
Scalp
arteriovenous mal ormations o , - ,
capillary mal ormations o ,
in antile hemangioma o , , - , - , -
overgrowth syndromes o ,
venous mal ormations o , , ,
Sclerotherapy,
or cervico acial vascular mal ormation, ,
or macrocystic lymphatic mal ormations
indications or,
reconstruction a er,
resection a er, -
resection without,
timing o ,
or venous mal ormations, , ,
Scoliosis, , -
Scrotum, vascular anomalies o ,
Sinus pericranii,
Skull; see Cranio acial bones
Slow-f ow lesions, ,
hematologic abnormalities in, ,
So tissue debulking o lower limb,
So tissue overgrowth; see Overgrowth syndromes
Spetzler-Martin AVM grading scale, ,
Spinal usion stabilization, , , -
Spine, vascular anomalies
complications with,
overview o , -
patient examples o , -
surgical indications or,
surgical management o ,
Overgrowth syndromes—cont’d
o lip, - , -
o lower extremity
patient examples o , , , ,
surgical indications or,
surgical management o , ,
management o ,
mutations associated with, ,
natural history o ,
overview o , -
patient examples o , -
with pyogenic granuloma, ,
o scalp,
surgical indications or, -
surgical intervention or, , -
o trunk, ,
P
Parkes Weber syndrome, , ,
Penis, vascular anomalies o , ,
Perioperative hematologic management, -
o bleeding risk, -
undamentals o hemostasis and thrombosis or,
-
or hematologic abnormalities in vascular anoma-
lies, -
laboratory assessment o coagulopathy in, -
o thrombotic risk, -
PG; see Pyogenic granuloma
Pharmacotherapy,
Phlebectasia,
di use, o Bockenheimer,
Physician pre erence and management,
Platelet(s),
Platelet trans usion,
Platelet trapping within vascular tumors, ,
Port-wine stain,
Pregnancy,
Primary lymphedema
amilial congenital,
overview o ,
patient example o ,
surgical indications or,
surgical management o , -
Prothrombin time (P ), , ,
Pulmonary lymphangiectasia, congenital,
Pyogenic granuloma (PG), -
capillary mal ormation with, ,
classi cation o , ,
clinical eatures o ,
management o ,
overview o ,
surgical indications or,

261
Index
thorough excision o , -
venous mal ormations as, ,
wound closure or,
V
Vascular mal ormations, ,
Vascular tumors,
o cranio acial bones, , ,
in antile hemangioma as; see In antile hemangioma
pyogenic granuloma as; see Pyogenic granuloma
rare, -
complications with,
patient examples o , -
surgical indications or, -
surgical management o , -
Venous anomaly, developmental,
Venous ectasia, ,
Venous mal ormation(s) (VM), -
o abdomen, ,
blue rubber bleb nevus syndrome as,
o central nervous system
arteriovenous, -
cavernous, -
overview o , -
cerebral cavernous mal ormation as,
o cheek,
classi cation o ,
o CNS,
complications with,
o cranio acial bones, - , - ,
cutaneomucosal, , ,
di use, , , , -
di use phlebectasia o Bockenheimer as,
o ace, , , ,
broadipose vascular anomaly as, , ,
gastrointestinal, ,
glomuvenous, , ,
hepatic,
intraarticular, , -
o lip, ,
localized, , , -
o lower extremity, , , ,
management o , ,
mutations associated with,
overview o , -
palmar,
patient examples o , -
phenotypes o ,
phlebectasia as,
o scalp, , ,
sclerotherapy or, , ,
sinus pericranii as,
surgical indications or, -
Strawberry hemangioma,
Sturge-Weber syndrome, ,
Suction-assisted lipectomy
or in antile hemangioma, , -
o lower limb,
or primary lymphedema, ,
Surgical intervention,
Systemic pharmacotherapy,
T
erminology or vascular anomalies,
T rombocytopenia, ,
T rombophilia, -
T rombosis, -
T rombotic risk
management o perioperative, -
other considerations or, -
opical pharmacotherapy,
rans usions,
runk
capillary mal ormations o , ,
in antile hemangioma o , , , -
overgrowth syndromes o , ,
venous mal ormations o ,
umors,
o cranio acial bones, , ,
in antile hemangioma as; see In antile hemangioma
pyogenic granuloma as; see Pyogenic granuloma
rare, -
complications with,
patient examples o , -
surgical indications or, -
surgical management o , -
U
Upper extremity, vascular anomalies
avoiding intraneural dissection with,
avoiding partial dissection with,
complications with,
dissection in bloodless eld in,
in antile hemangioma as, , , , -
microcystic lymphatic mal ormations as,
overview o , -
patient counseling or,
patient examples o , -
patient ollow-up or, -
placement o incisions or, -
postoperative immobilization or,
preoperative planning or,
surgical indications or,
surgical instruments or, -
surgical management o , -
tagging normal structures with,

262

Index
Verrucous venous mal ormation, , , -
Vertebral column, vascular anomalies
complications with,
overview o , -
patient examples o , -
surgical indications or,
surgical management o ,
VM; see Venous mal ormation(s)
Volume maintenance,
Venous mal ormation(s) (VM)—cont’d
surgical intervention or, -
complete resection as, , - ,
principles o ,
subtotal resection as, , -
timing o , -
o trunk,
o upper extremity, ,
verrucous, , , -