Phẫu Thuật Tạo Hình (Tập 6): Bàn Tay Và Chi Trên – Neligan (Ấn bản 4)

680
SECTION IV
CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
Fig. 30.13  Reconstruction of the proximal humerus at 5-year follow-up. The
transferred proximal fibula underwent significant remodeling and hypertrophy as
well as enough longitudinal growth to prevent limb length discrepancy.
Fig. 30.14  The anterior tibial artery supplies the growth plate and proximal
epiphysis by means of a recurrent epiphyseal branch and the proximal two-thirds of
the diaphysis by means of musculoperiosteal perforator branches.
Periosteal branches to fibula
Epiphyseal recurrent branch
Anterior tibial artery
acceptable blood supply to the growth plate based solely on
the peroneal artery. Although this is probably anatomically
possible if enough soft tissue is left around the fibular neck,
further experiences151,156 have had disappointing results on the
growth rate of grafts supplied by the peroneal artery alone.
The use of a bipedicled graft has been suggested,147,149,151
with the purpose of providing two separate blood supplies to
the epiphysis and the diaphysis. This option, however, is
technically demanding, time-consuming, and requires two
anastomoses at the recipient site. In addition, the physis is
very susceptible to ischemia, and normal growth can be
expected only if ischemia time is less than 3 h.168 For these
reasons, a single-pedicled graft is definitely a better choice.
The anterior tibial system has proven to be adequate in
supplying both the epiphysis and the shaft and it is therefore
the preferred pedicle for such a graft (Fig. 30.14). A potential
disadvantage to the use of the anterior tibial vascular system
is that the pedicle is very short (the distance between the
division of the popliteal artery and the origin of the recurrent
branch at the fibular neck). To overcome this issue, it has been
suggested to use a reverse-flow pedicle.151,154,161 In this way,
long vessels are provided, allowing comfortable anastomosis
at the recipient site.
Harvest technique of the proximal fibula based on
the tibialis anterior artery (Video 30.1 )
The flap includes the proximal fibular epiphysis and a vari­
able amount of the adjoining diaphysis. The epiphyseal
articular surface is oriented upward and medially to form the
proximal tibiofibular joint. The biceps femoris tendon and
the lateral collateral ligament are inserted in the lateral and
proximal aspect of the epiphysis, which is also where the
origins of the peroneus longus and extensor digitorum longus
muscles are located. The common peroneal nerve crosses the
fibular neck, moving toward the anterior compartment of
the leg. The superficial branch is located in the space between
the extensor digitorum longus and peroneus longus muscles,
while the deep branch reaches the interosseous membrane
and joins the anterior tibial vessels, giving several motor
branches to the surrounding muscles. Proceeding proximally,
the dissection of the vascular pedicle with preservation of the
motor branches becomes increasingly demanding because of
the increasing number of motor branches surrounding the
vessels.
The harvest technique (Video 30.1
) has been refined
over the years and has been finally described in detail169,170
with the purpose of standardizing it and making it reproduc­
ible. The aim of the procedure is to harvest the proximal

681
Treatment/surgical technique
Fig. 30.15  (A,B) An anterolateral approach is used for the harvest of the proximal
fibula based on the anterior tibial vascular system. The skin incision is on the
projection of the intermuscular space between the tibialis anterior and extensor
digitorum longus muscles. The incision is prolonged proximally and posteriorly
over the tendon of biceps femoris muscle.
Incision
Peroneal nerve
A
B
Fig. 30.16  During the diaphyseal dissection of the pedicle, great care must be
paid to isolating the peroneal nerve and its motor branches to the muscles of the
anterior compartment.
fibula, preserving the blood supply to both the epiphysis
and diaphysis with the least possible local morbidity. The
following step-by-step description is the optimal surgical
sequence according to the experience of the senior author
(Video 30.1
).
Skin incision
The surgical approach is developed in the plane between tibi­
alis anterior and extensor digitorum longus muscles because
this allows for direct visualization of the neurovascular
bundle. The skin incision is therefore located in the anterolat­
eral aspect of the leg and is extended proximally up to the
neck of the fibula where it proceeds posteriorly and proxi­
mally over the biceps femoris tendon (Fig. 30.15).
Exposure of the anterior tibial pedicle
The anterior tibial artery and veins lie on the interosseous
membrane. The peroneal nerve surrounds the vessel accord­
ing to a very intricate tridimensional pattern and delivers
several motor branches to the muscles of the anterior
compartment of the leg (Fig. 30.16). These motor branches are
more numerous in the proximal part of the leg and it is there­
fore recommended to start the dissection distally and proceed
proximally, maintaining the same plane to the fibular neck.
Great care should be taken not only in dissecting the nerve
from the vessels but also in preserving the small perforator
branches of the anterior tibial artery that pierce the extensor
digitorum longus and peroneus longus muscles, interposed
between the vascular pedicle and the fibular shaft, supplying
the periosteum of the proximal two-thirds of the diaphysis.
Dissection of the peroneal nerve at the fibular neck
The point where the peroneal nerve approaches the fibular
neck is an important anatomic landmark. The nerve is in close
contact with the bone and is covered by the proximal portion
of extensor digitorum longus and peroneus longus muscles,
which must be sharply divided in order to expose and protect
the nerve and its motor branches (Fig. 30.17). Medially, at the
same level, the recurrent epiphyseal branch of the anterior
tibial artery rises from the main artery and pierces the mus­
cular cuff and feeds the epiphysis. Its direct dissection is
unnecessary and potentially dangerous; it is therefore recom­
mended to preserve the proximal insertion of the muscles
attached to the proximal epiphysis in order to protect the
fragile epiphyseal vascular network.
Section of the interosseous membrane
and distal osteotomy
The interosseous membrane must be sharply detached from
the tibia, and all the soft tissue interposed between it and the
vascular bundle must be carefully protected (Fig. 30.18). The
perforator vessels to the shaft of the fibula branches off later­
ally and pierces the of extensor digitorum longus and peroneus
longus muscle bellies. In order to maintain the vascular con­
nection between the artery and bone, a longitudinal strip of
muscle approximately 1 cm wide should be preserved around
the harvested fibula (Fig. 30.19). The fibula is cut distally at
the desired length, and an extra portion of periosteum is left
in order improve healing capacity at the recipient site. The
anterior tibial artery and veins are ligated as distally as pos­
sible to obtain a long reverse-flow pedicle.

682
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CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
Fig. 30.17  (A,B) The peroneal nerve is an important landmark in proximal
dissection. The nerve is exposed in its intramuscular portion by means of sharp
section of the extensor digitorum longus and peroneus longus muscles. The
muscular cuff proximal to the section must be left intact because it protects the
recurrent branch to the growth plate of the anterior tibial artery.
Peroneal nerve
Fibular epiphysis
Biceps femoris tendon
Tibialis anterior
Anterior tibial
artery
Extensor
digitorum
longus
Peroneus
longus
A
B
Fig. 30.18  Sharp detachment of the interosseous membrane from the tibia. Note
the location of the vascular pedicle and its relationship to the peroneal nerve.
Fig. 30.19  The perforator branches to the diaphysis are too small and fragile to
allow a direct intramuscular dissection. It is therefore suggested that a strip of
muscle containing the perforators be left intact between the vascular pedicle and
the bone.
Fibula
Tibialis anterior
Tibia
Anterior tibial
artery and vein
Extensor
digitorum
longus
Peroneus longus
Harvest of the biceps femoris tendon and capsulotomy of
the proximal tibiofibular joint
The insertions of the biceps femoris tendon and the lateral
collateral ligaments are almost at the same point at the apex
of the fibula. The tendon should be divided in two strips
(Fig. 30.20): the posterior portion is harvested with the fibula
to provide additional soft tissue useful for stabilization of the
recipient joint; the anterior half is used to reinforce the lateral
collateral ligament after its reinsertion on the lateral aspect of
tibial metaphysis by means of staples or transosseous sutures.
Gentle external rotation of the fibula allows for the division
of the medial and posterior capsule of the proximal tibiofibu­
lar joint after coagulation of the lateral inferior genicular
artery.

683
Postoperative care
Final dissection of the proximal portion of
the vascular pedicle
The anterior tibial artery should be dissected up to the divi­
sion of the popliteal artery (Fig. 30.21). The recurrent branch
rises from the anterior tibial artery approximately 2 cm distal
to its origin, and is often difficult to identify. For this reason,
it is advisable to preserve all the small vessels, which pierce
the muscular cuff toward the fibular epiphysis. After release
of the tourniquet, the muscular cuff surrounding the epiphy­
sis, the diaphyseal periosteum, and the medullary canal are
carefully inspected. After a few seconds, bleeding should be
observed at the three levels, confirming the successful harvest
of the proximal fibula, based on the anterior tibial artery
pedicle (Fig. 30.22). Due to low tolerance to ischemia, it is
suggested to wait 20–30 min before clipping and dividing the
proximal pedicle.
Details regarding the selection of flap vena comitans is
given in Box 30.1; osteosynthesis in Box 30.2; and selection of
recipient vessels in Box 30.3.
Postoperative care
Donor site
In order to protect the reconstructed lateral collateral liga­
ment, a cast at 30° flexion of knee and neutral ankle position
is applied. In children below the age of 8 years, the cast is
maintained for 4 weeks; in older and compliant children, the
cast may be substituted after 2 weeks with a brace of appro­
priate size, for a total of 4 weeks of immobilization. The
rehabilitation program is started at 1 month postoperatively
with the aim of recovering full motion of the knee without
compromising stability (Box 30.4).
Recipient site
A long spica cast or a shoulder brace is applied for 4 weeks,
respectively, after distal radius and proximal humerus
Fig. 30.20  (A,B) The biceps femoris tendon is longitudinally divided in two strips.
One is harvested with the bone flap and may be used to improve the stability of the
joint after the transfer. The second one is fixed to the lateral aspect of the tibia to
reinforce the lateral collateral ligament.
A
Tibialis anterior
Biceps femoris tendon
Extensor
digitorum
longus
Peroneus longus
Head of fibula
B
Fig. 30.21  After release of tourniquet and 20–30 min of reperfusion of the flap,
the tibialis anterior artery is ligated and severed just distal to its emergence from
the popliteal artery.

684
SECTION IV
CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
Fig. 30.22  At the end of the dissection, before severing the vascular pedicle, the
tourniquet is released to reset the ischemia time. Bleeding should be observed from
both the epiphysis and the medullar canal after a few minutes. 1, posterior articular
capsule is severed; 2, bleeding from the bone is observed.
Tibialis anterior
Deep peroneal nerve
Superficial
peroneal nerve
Biceps femoris
tendon (cut)
Fibula
(cut)
Extensor
digitorum
longus
Peroneus longus
Head of fibula
1
2
BOX 30.1  Selection of flap vena comitans
The proximal epiphyseal fibular transfer based on the anterior tibial
artery is a reverse-flow flap; however, an adequate venous return is
guaranteed by the presence of several small shunts that interconnect
the two venae comitantes, allowing the reverse venous flow to
bypass the valves. Due to this intricate pattern of valves and shunts,
usually, only one of the two venae comitantes has adequate return
flow: it is instrumental, for a good outcome, to identify which one is.
After 20–30 min with the tourniquet down, the two venae
comitantes are ligated and divided proximally leaving intact the flow
through the artery. Within a couple of minutes, the venous flow is
inverted and bleeding is evident from one of the two venae
comitantes. The bleeding vein is tagged to be anastomosed at the
recipient site. (Fig. 30.23).
During the dissection of the vascular bundle care must be taken
not to interrupt these delicate venous shunts.
Fig. 30.23  (A) Pattern of valves and shunts of venae comitantes. (B) When the
flow is inverted, bleeding is usually evident from one of the two venae comitantes.
Normal flow
Venous
valve
Venous
shunt
Proximal
Distal
Inverted flow
Bleeding
comitans
A
B
BOX 30.2  Osteosynthesis
Humerus reconstruction
The arm is a single bone segment with a high mechanical stress,
particularly in case of rotation against resistance. For this reason, a
stable bone fixation must be provided; long plates are therefore
preferred over less invasive options such as K-wires and screws.
However, the implant cannot be too stiff for risk of fracture at the
level of the most proximal screw, which is the weakest point of the
implant (Fig. 30.24A). In order to provide a more elastic and less
invasive bone fixation, we advocate the use of locking compression
plates (LCP). The fibula is usually placed for a couple of centimeters
in the medullary canal of the distal stump of the humerus and a
periosteal flap is wrapped around the bony junction. Then a long
LCP plate is placed. Three bicortical screws are used in the humeral
portion while few unicortical screws are used in the fibular portion
(see Fig. 30.24B). This type of assemblage provides a very stable
bone fixation at the junction between fibula and humerus and a
minimally invasive and elastic fixation in the fibular segment.
Radius reconstruction
Similarities in size and shape between the radius and the fibula
facilitates osteosynthesis in the forearm. Also in this case, LCP
plates are the preferred method of bone fixation. As an alternative,
lag screws and step-cut osteotomy may be used. The wrist joint is
temporarily stabilized with a K-wire which is usually removed 1
month after surgery, in addition the strip of biceps femoris tendon is
woven in the residual capsule in order to provide additional stability
to the joint. By contrast, the distal radioulnar joint is intentionally left
lax without any type of stabilization in order to maintain full range of
pronation and supination.

685
Postoperative care
Fig. 30.24  (A) Humerus implant: the weakest portion of the implant is at the passage between the hardware and the bone, with higher risk of fracture at these levels
especially in case of rigid implants. (B) More elastic and less invasive bone fixation.
A
B
BOX 30.3  Selection of recipient vessels
The harvest of the fibula based on a reverse-flow anterior tibial
artery pedicle allows for a long distal pedicle, which may be easily
anastomosed to the recipient vessels.
In humeral reconstruction, the deep brachial artery, satellite of
the radial nerve, is usually preferred for end-to-end anastomosis
with the fibular vessels. If the venae comitantes are too small, the
cephalic vein or one of the venae comitantes of the brachial artery
may be chosen for venous anastomosis.
In case of radius reconstruction, there are more choices. When
the radial artery is resected together with radius, the proximal stump
can be easily anastomosed with the fibular pedicle end to end:
Alternatively, the anterior interosseous artery may be used end to
end because its sacrifice has a negligible impact on hand blood
supply.
BOX 30.4  Postoperative rehabilitation: donor site
• After 4 weeks of immobilization, the cast or the splint are
removed and passive range of motion of knee and ankle is
initiated.
• The use of crutches is virtually impossible because of the
surgery involving the upper limb and is therefore avoided.
• During the therapy sessions, the child is also helped to get
acquainted with the upright position standing only on the
non-operated leg.
• The splint is repositioned only at night for 4 more weeks.
• A transient palsy of the peroneal nerve occurs in almost all
cases. Great care is taken to avoid equinus deformity of the foot
with Achilles tendon retraction. A Codivilla spring is used
routinely in all cases of peroneal palsy and maintained until
complete recovery. Passive mobilization of the ankle is taught to
parents and performed 3–5 times per day.
• Full weight-bearing is usually possible at 2 months after surgery.
• The rehabilitation program is eventually tailored to each patient
considering their clinical condition and the necessity of adjuvant
chemotherapy.

686
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CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
reconstruction. The rehabilitation program follows the
immobilization period and must take into account different
variables such as type and stability of bone fixation, amount
of muscles excised during the resection, and age and compli­
ance of the patient.
Outcomes, prognosis, and
complications
Analysis of the results takes into account the survival of the
grafts, as well as their consolidation, longitudinal growth, and
remodeling. In a personal series of 27 cases of upper limb
skeletal reconstruction after bone sarcoma resection with
epiphyseal transplant, all the transferred bones except one
survived and healed with the recipient bone in a period of
time ranging from 1 to 2 months. This variability depended
on the age of the child and the length of the reconstructed
segment. In some cases, the viability of the transplant was
confirmed by bone scan.
Significant axial growth, calculated by the progressive
increase in the distance between the tip of the metal plate used
for bone fixation and the apex of the epiphysis of the trans­
ferred fibula, has been observed in approximately 70% of
cases monitored. Its extent was variable depending on factors
that are only partially known, amongst which the patient’s
age was predominant. The annual growth trend varied
between 0.7 and 1.35 cm (Fig. 30.25), and in all cases there was
prevention of future length discrepancy with the opposite
limb. As far as the radius is concerned, symmetrical growth
was observed in the neoradius in all cases, not only with
respect to the opposite limb, but also to the adjacent ulna,
which confirms the integration of the fibula into its new
anatomical site. The average flexion of the wrist was 75°,
while the average extension was 63°. The overall range of
movement was equal to on average 70% of the opposite hand,
which allowed for excellent functional recovery. The prosupi­
nation range was also restored.
Fig. 30.25  Postoperative radiograph and 4-year follow-up of one case of distal
radius reconstruction. In this case, the average growth per year was 0.9 cm.
In cases of humeral reconstruction, the functional outcomes
were not as positive. The morphological and dimensional
discrepancy between the fibular head and the glenoid fossa
may lead to proximal migration of the fibular epiphysis and,
in some cases, subacromial displacement. Moreover, the
extent of soft tissue involved in the neoplasia and conse­
quently removed further hinders functional recovery. In all
monitored cases, however, shoulder abduction between 70°
and 100° was observed.
Premature ossification of the growth plate and consequent
end of growth were observed in five cases of humeral recon­
struction. Two of these transplants were based on peroneal
vessels, which, in our opinion, are not as reliable in ensuring
correct epiphyseal perfusion. In the other three cases, the
epiphyseal artery was probably damaged during stabilization
in the glenohumeral joint. Five fractures of the neohumerus
and two of the neoradius were observed. All the fractures but
one healed conservatively in due time. Replacement of an
inadequate fixation was performed in one case.
In four cases of proximal humerus reconstruction, incorrect
alignment between the fibula head and glenoid fossa was
observed. In these cases, the epiphysis migrated subacromi­
ally. The reasons for this complication are the anatomical
mismatching between fibular head and glenoid fossa and the
extensive oncological excision of muscles and ligaments with
consequent joint stability. All the four cases, however, recov­
ered an acceptable range of motion.
With transosseous suture of the lateral collateral ligament
at the donor site, no residual instability of the knee was ever
observed. Neuropraxia of the peroneal nerve occurred in
approximately two-thirds of cases. This was probably caused
by stretching of small motor branches during the dissection.
This deficit resolved spontaneously within 1 year in all but
two cases. One of the patients had a permanent residual
paralysis of the tibialis anterior and one of the extensor digi­
torum longus muscles.
Secondary procedures
Secondary procedures may be necessary at both the donor
and the recipient site.
Donor site
In case of permanent palsy of the peroneal nerve, a tendon
transfer is indicated to restore the function of the tibialis
anterior muscle. In our series, we observed no cases of knee
joint instability.
Recipient site
A feared complication is the total loss of the graft due to
failure to restore its blood supply. We did not observe this
complication but its treatment would require the use of a
massive frozen allograft as salvage procedure. In the majority
of cases, the fate of an osteoarticular allograft in the medium/
long term is a massive resorption of the articular cartilage,
which should be treated with a prosthetic replacement of the
allogenic humeral head.
In our experience, we have observed premature growth
arrest, probably due to vascular damage to the physis, in five
cases of proximal humerus reconstruction. Despite the limb

687
Future directions
majority of cases, either the reconstruction of the proximal
humerus or the distal radius. In the future, new recipient sites
might be added to the classic ones, taking advantage of the
high remodeling potential of the immature fibular head. We
have recently used this technique for mandible reconstruction
on a child affected by osteoblastoma of the mandibular
condyle, with encouraging results. In selected cases, other
joints might benefit from reconstruction with vascularized
proximal epiphyseal transfer.
Congenital differences at the wrist level, such as radial and
ulnar dysplasia, are now successfully treated with this proce­
dure. A few cases of radial dysplasia have been, indeed,
reported recently with good results.160 These cases, though,
need a longer follow-up to evaluate the growth rate. These
initial results are very encouraging, and epiphyseal transfer
of the proximal fibula may be a valid alternative to the pro­
cedure described by Vilkki,171 which is, at present, the only
alternative to the conventional techniques.
length discrepancy, all patients have an acceptable function
and no further surgery was required.
A relatively frequent complication is graft fracture in proxi­
mal humerus reconstruction. In our series, this event was
usually successfully treated conservatively with immobiliza­
tion in a cast. Nonetheless, revision of the hardware and
cancellous bone grafting may be considered in cases of dis­
placed fractures.
Future directions
Although epiphyseal transfer proved to be a reliable and
effective reconstructive option in case of skeletal disorders
involving an immature epiphysis, this procedure is not widely
used yet. The technique is well standardized, and several
papers are now available in the literature reporting, in the vast
3. Rauch F. Bone growth in length and width: the yin and yang of
bone stability. J Musculoskelet Neuronal Interact. 2005;5:194–201. This
entertaining article presents in a very approachable manner the
morphophysiology of the growth plate and the mechanisms that regulate
bone growth.
27. Canavese F, Charles YP, Dimeglio A. Skeletal age assessment from
elbow radiographs. Review of the literature. Chir Organi Mov.
2008;92:1–6. A useful method to assess skeletal age during puberty is
presented in this article. The paper also offers an accurate description of
growth during puberty and contains a review of the literature of other
methods used for skeletal age assessment in growing individuals.
85. Sailhan F, Chotel F, Guibal AL, et al. Three-dimensional MRI
imaging in the assessment of physeal growth arrest. Eur Radiol.
2004;14:1600.
98. Birch JG. Surgical technique of physeal bar resection. Instr Course
Lect. 1992;41:445.
147. Tsai TM, Ludwig L, Tonkin M. Vascularized fibular epiphyseal
transfer: a clinical study. Clin Orthop. 1986;210:228–234. In this
article, a clinical series of eight pediatric patients who underwent joint
reconstruction by means of fibular epiphyseal transplant is reported.
Four of the patients showed continued growth of the transplanted bone.
A double-pedicled flap was described for the first time in this pioneering
report.
148. Pho RW, Patterson MH, Kour AK, et al. Free vascularised
epiphyseal transplantation in upper extremity reconstruction.
J Hand Surg Am. 1988;13:440–447.
156. Innocenti M, Delcroix L, Romano GF, et al. Vascularized
epiphyseal transplant. Orthop Clin North Am. 2007;38:95–101.
165. Taylor GI, Wilson KR, Rees MD, et al. The anterior tibial vessels
and their role in epiphyseal and diaphyseal transfer of the fibula:
experimental study and clinical applications. Br J Plast Surg.
1988;41:451–469. This is the first article reporting both an experimental
and clinical experience in proximal fibular epiphyseal transfer based on
the anterior tibial vascular system. The most remarkable finding
consisted of assessing the ability of the anterior tibial artery to supply
not only the proximal growth plate but also a considerable amount of
fibular shaft. This improvement in the knowledge of the vascularity of the
proximal fibula allowed reliable use of a fibula flap with a single pedicle.
170. Innocenti M, Delcroix L, Manfrini M, et al. Vascularized proximal
fibular epiphyseal transfer for distal radial reconstruction. J Bone
Joint Surg Am. 2005;87(suppl 1):237–246. In this article, the operative
technique for harvesting the proximal fibula supplied by the anterior
tibial vascular system is reported in detail. A step-by-step description of
the procedure is provided and supported by anatomical drawings and
related clinical pictures. Recommendations and pitfalls may help the
reader in approaching this reconstructive technique.
Access the complete reference list online at

687.e1
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SECTION IV • Congenital Disorders
31 
Vascular anomalies of the upper extremity
Joseph Upton III and Amir Taghinia
SYNOPSIS
■ Vascular anomalies of the upper limb are divided into tumors
(including hemangiomas) and malformations.
■ Hemangiomas have a biphasic growth phase consisting of proliferation
and spontaneous involution; malformations tend to expand slowly
throughout life.
■ Hemangiomas are usually treated conservatively, whereas vascular
malformations can be managed by observation, sclerotherapy,
embolization, and/or surgical resection.
■ Surgical resections can be predictably performed if basic principles
are followed; venous malformations, lymphatic malformations, and
combined lesions are most amenable. Many technical refinements
have made these operations safer and more predictable.
■ Fast-flow lesions with or without fistulae comprise the most difficult
group of lesions; their natural history is one of gradual progression.
■ New discoveries in the biochemical pathways responsible for these
lesions hold promise for future medical therapy.
Introduction
During the past five decades, there has been a steady, almost
exponential increase in our knowledge of vascular anomalies.
A biological classification system has evolved and is under
constant refinement.1 A careful physical examination aug­
mented with advanced imaging will yield an accurate diag­
nosis from the vast myriad of possibilities. A multidisciplinary
team can offer individualized treatment with some degree of
predictability. Improvements in minimally invasive radiologi­
cal procedures have dramatically transformed treatment of
these lesions. With discovery of biochemical agents and
pathways responsible for these lesions, widening application
of medical therapies show promising outcomes. However, in
many patients, surgical treatment is often the best remedy and
should not be viewed as the last resort. A carefully planned
and well-orchestrated procedure and rehabilitation can yield
remarkable outcomes.2
Classification and Overview
The etiology of vascular birthmarks has long stimulated the
fertile imagination of humans. Historically, classification
systems have reflected a balance between folklore and science.
Well into the 19th century, the doctrine of maternal impres­
sions dictated that a gravid mother’s craving for strawberries,
sight of an accident, or emotional longing could imprint a
vascular blemish, a naevus maternus, on her unborn child.3,4
With the development of the microscope, a vast number of
histological terms have been introduced. Despite these aca­
demic advances, most lesions are still inaccurately labeled
as hemangiomas. Study of the histopathology and natural
history of these lesions spawned intriguing embryological
classifications,5,6 which seemed logical at the time but failed to
distinguish involuting from non-involuting lesions and offered
little guidance in management. In the late 1970s, Mulliken
and Folkman proposed a working hypothesis that these cells
could be distinguished by their cellular characteristics and
Clinical tip
Vascular anomalies are complex problems and are best
approached by a multidisciplinary team. In our center, we have
a weekly conference and a separate weekly clinic where the
team meets to discuss individual patients. Representatives
from radiology, general (pediatric) surgery, hematology,
anesthesiology, plastic surgery, dermatology, and scientists
from the laboratory all attend. Other specialists such as
neurosurgeons, orthopedic surgeons, oral surgeons,
ophthalmologists, endocrinologists, and head and neck
surgeons are called as needed. Thus, a comprehensive
approach to patient care can be undertaken, with immediate
availability of ultrasound imaging and expert opinion. This
approach has been monumental in the correct diagnosis and
treatment of hundreds of patients.
  Access video content for this chapter online at expertconsult.com

689
Classification and Overview
subsequently surgical specimens were analyzed by selected
histological stains, electron microscopy, and autoradiography
(uptake of tritiated thymidine into DNA). On the basis of
these studies, two types of lesions were present: those that
showed endothelial hyperplasia, called hemangiomas, and
those that did not, called malformations. A binary schema
based on biologic activity was proposed1 and accepted by
the International Society for the Study of Vascular Anomalies
(ISSVA) in 1996.7 This system has been corroborated by both
radiological studies8,9 and immunohistological staining.10
However, “no system is carved in stone and must be written
on recyclable paper” and is in a constant state of revision.
For example, the initial category of “hemangiomas” has been
changed to “vascular tumors” to include all vascular tumors
in all age groups. A particular vascular lesion may be moved
from one category to another or may be discarded completely
with expansion of clinical experience and basic science inves­
tigation. For the past 30 years, this system has been very
helpful to the upper limb surgeon in the proper diagnosis and
management of these lesions (Table 31.1). Most frustrating are
Table 31.1  ISSVA classification of vascular anomalies
Vascular tumors
Vascular malformations
Slow-flow vascular malformations
Hemangioma:
Infantile hemangioma (IH)
Congenital (CH)
Non-involuting
congenital
hemangioma (NICH)
Rapidly involuting
congenital
hemangioma (RICH)
Hemangiomatosis
Pyogenic granuloma
Kaposiform
hemangioendothelioma
(KHE)
Rare tumors:
Hemangioendothelioma
Infantile fibrosarcoma
Hemangiopericytoma
Giant cell angioblastoma
Capillary malformations (CM):
Port-wine stain
Telangiectasias
Angiokeratoma
Venous malformations (VM):
Common sporadic VM (VM)
Blue rubber bleb syndrome
(BRBS) or Bean syndrome
Glomuvenous malformation (GVM)
Familial cutaneous and mucosal
venous malformation (VMCM)
Maffucci syndrome
Diffuse VM (Bockenheimer)
Lymphatic malformations (LM):
Common lymphatic malformation
(LM)
Lymphangiomatosis
Gorham–Stout syndrome
Fast-flow vascular malformations
Arterial malformation (AM)
Arteriovenous malformation (AVM)
Arteriovenous fistula (AVF)
Complex-combined vascular
malformations
CVM, CLM, LVM, CLVM (Klippel–
Trénaunay),
CM-AVM (Parkes Weber), AVM-LM
Proteus, hemihypertrophy,
capillary–lymphatic–overgrowth–
vascular–epidermal nevus
(CLOVE)
ISSVA, International Society for the Study of Vascular Anomalies.
the large number of eponyms with and without abbreviations,
which have been attached to these lesions. These terms are
often used indiscriminately without specific reference to cell
type, flow characteristics, and growth. Adding to the confu­
sion is the common association of growth disturbances. Both
overgrowth and undergrowth with and without dysplastic
fat deposition may accompany vascular anomalies. The
summary in Table 31.2
helps delineate our present state of
knowledge of this nosologic quagmire. The recent discovery
that somatic activating mutations in the phosphatidylinositol-
3-kinase (PIK3CA)/mammalian target of rapamycin (mTOR)
pathway appear to underlie a number of overgrowth pheno­
types (termed PIK3CA-related overgrowth spectrum, PROS)11
promises to provide some degree of molecular differentiation
to these disorders.12–14 Table 31.2 appears online only.
A correct diagnosis impacts directly upon appropriate
treatment and an incorrect diagnosis may result in unneces­
sary or harmful remedies. Most upper limb surgeons designate
all fast-flow anomalies with or without arteriovenous fistulae
as Parkes Weber syndrome (PWS). However, PWS patients
comprise a very small percentage of the fast-flow group,
which comprises about 15% of upper extremity fast-flow
malformations.15 Not all slow-flow combined malformations
should be called Klippel–Trénaunay syndrome (KTS), an
eponym we reserve for combined capillary–lymphatico-
venous malformation (CLVM) lesions. The venous malforma­
tion (VMs) collectively comprise the largest clinical group of
patients evaluated by the hand surgeon. The majority are
small lesions involving all portions of the upper limb and/or
ipsilateral chest wall. Other subgroups have emerged. The
diffuse VMs involving all structures within the upper limb
including bone have been designated Bockenheimer lesions,16
originally described as “diffuse genuine phlebectasias”, and
are not amenable to surgery with the exception of pathologic
fractures and localized areas of symptomatic thromboses.
Maffucci patients commonly get skeletal enchondromas but
many of the skeletal lucencies may be clusters of VMs, which
are also found in abundance within the soft-tissue planes.17,18
The glomuvenous (GVM) lesions occur in clusters with a
characteristic appearance, are painful, have been localized to
chromosome 1p21–22, and in the past were called glomus
tumors or glomangiomas.19 They have been renamed to stress
the fact that they are malformations and not tumors (as sug­
gested by the suffix “oma”). The VMs seen in the blue rubber
bleb nevus syndrome (BRBNS) also have a characteristic
cutaneous appearance, are also familial and often confused
with GVM, Maffucci and capillary malformations–venous
malformations (CM–VM).20 The associated gastrointestinal
lesions, often localized to the small bowel, are often the cause
of anemia and iron deficiencies (see Table 31.7).
Lymphatic malformations (LMs) often occur in combina­
tion with other vascular elements but the most common
varieties are either isolated or diffuse LMs, which may involve
any portion of the upper limb and/or ipsilateral chest wall.15
Lymphangiomatosis conditions are not of primary concern to
the upper limb surgeon as they involve visceral organs and/
or the lungs and in the long run are life endangering. Never­
theless, a microsurgical approach to treatment of central
lymphatic conduction anomalies may be of some utility.21 LMs
in the Gorham–Stout syndrome involve skeletal structure
including the humerus, radius, ulna and tubular bones of the
hand.22 Pathologic fractures can occur.

689.e1
Classification and Overview
Continued
Table 31.2  Diagnostic work-up of vascular anomalies
Klippel–
Trénaunay (KT)
Parkes Weber
(PW)
Lipomatous
CLOVES
Hemihypertrophy
Proteus
Maffucci
Capillary stain
Present, deep
purple
Present, pink, often
diffuse
Rare
Present, pink,
diffuse, truncal
Uncommon
Present, pink, often diffuse
Absent (rarely
reported)
Progressive
overgrowth
Present
Present
Present, symmetrical
Present, often
extensive
Either overgrowth or
undergrowth
Absent at birth, haphazard,
unrelenting,
disproportional
Secondary to
enchondroma
growth, moderate
to massive
Hemodynamics
Slow flow
Fast flow
Slow flow
Slow flow
Slow flow
Slow flow
Slow flow
Vascular
anomalies
VM, LVM
AVM, AVFs
Absent
VM, LVM, LM
Absent
VM, LM, CM
VM including within
bone, hand
common
Associated
anomalies
Common: GI, GU,
genitalia
Hypoplastic
lymphatics
Deep venous
anomalies
Rare, Cobb
syndrome
Absent
No visceral
Skin, CNS, heart, GU,
dental, others
Cerebriform soles/palms,
linear nevi, dysregulated
fat, lung cysts,
neoplasm, facial
phenotype
None
Limb
enlargement
Moderate,
disproportionate
digits and toes
common
Arm, leg length
discrepancy
Moderate, nerve-
territory oriented
Moderate
Macrodactyly,
syndactyly,
polydactyly, club feet
Major, asymmetrical
Disproportionate
Limb affected
Upper 5%, lower
95%
Upper 23%, lower
77%
Equal
Equal
Arms, hands equally
Arms/legs, hands/feet
equal
All
Skeletal
changes
Unusual, can occur
with large lesions
Common, direct
involvement,
demineralization
Common,
symmetrical
Marked scoliosis,
lower limb
Hip dysplasia, scoliosis,
increased bone age
Skull hyperostosis,
vertebral
megaspondylodysplasia
Gross distortion,
asymmetry, limb
length
discrepancy,
phalanges
common
scoliosis, short
stature, fractures
Macrodactyly
Diffuse
enlargement
Minimal to
moderate
Moderate to gigantic
Diffuse or longer
with flexion
contractures
Digits longer with
flexion or extension
contractures
Asymmetrical, moderate to
gigantic
Digits longer with
asymmetrical
masses,
increased size
due to tumors
Dysregulated fat Present within
malformation
Present
Common, entire limb
and axilla
Present
Multiple lipomas
Lipomas, regional fat
absence
Present occasionally
Nerve
involvement
Absent
Absent
Intermittent
Intermittent
Absent, compression
neuropathies with
contractures
Absent
Absent

689.e2
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
Table 31.2  Diagnostic work-up of vascular anomalies—cont’d
Klippel–
Trénaunay (KT)
Parkes Weber
(PW)
Lipomatous
CLOVES
Hemihypertrophy
Proteus
Maffucci
Muscle
anomalies
Displaced by VM,
LVM
Displaced by
malformation
Absent
Absent
Common, atavistic
intrinsic and extrinsic
muscles
Absent
None
Associated
neoplasms
Absent
Absent
Absent
Absent
Renal, adrenal and
CNS
Ovarian, parotid
Chondrosarcoma
(17.5–30%), many
others less
frequently,
ovarian, CNS
Coagulopathy
Present
Normal
Normal
Normal
Normal
Normal
None
Increased
cardiac
output, CHF
No
Yes
No
No
No
No
No
Clinical
prognosis
Stable
Often progressive
deterioration
Steal phenomenon
Worse with CHF or
amputations
Good, related to size
and weight of limb
Progressive
deterioration with
extensive limb
involvement
Progressive
contractures (flexion
or extension, intrinsic)
Progressive growth of
affected regions
Good with local Rx;
Ollier’s patients
increased
problems
Risks
DVT, risk increased
postoperatively
Infection, CHF,
gangrene
Secondary arthritis
Scoliosis,
pulmonary
compromise
Neoplasms:
pheochromocytoma,
Wilms,
hepatoblastoma
Neoplasms, PE,
depression
Pathologic fractures
Genetics
Unknown
Unknown
Unknown
Unknown
Unknown
Unknown
Unknown
AVF, Arteriovenous fistula; AVM, arteriovenous malformation; CHF, congestive heart failure; CLOVES, congenital lipomatous overgrowth with vascular malformations, epidermal nevi, and skeletal anomalies; CM, capillary
malformation; CNS, central nervous system; DVT, deep vein thrombosis; GI, gastrointestinal; GU, genitourinary; LM, lymphatic malformation; LVM, lymphatico-venous malformation; PE, pulmonary embolism; Rx,
treatment; VM, venous malformation.

690
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
There are many conditions in which vascular anomalies,
overgrowth or undergrowth coexist and with our present state
of knowledge, it is difficult to draw clear lines due to the
increasing amounts of growth factors found in the omnipres­
ent dysplastic fat tissue within these extremities. Often at their
vascular clinic reviews, the authors encounter lesions that do
not fit into any specific category and are designated as
“PUVA”, for provisionally unique vascular anomalies. With time,
this large group of “unknowns” will become more precisely
defined. Several examples of recent breakthroughs in clinical
diagnosis and biochemical markers include: discovery of
PIK3CA12,13,23 in overgrowth conditions, and the identification
of CLOVES syndrome (congenital lipomatous overgrowth
with vascular malformations, epidermal nevi, and skeletal
anomalies) as well as fibro-adipose vascular anomaly (FAVA).24
Children with CLOVES present with large extremity and
truncal CMs, moderate to marked overgrowth, large fatty
tumors, either LMs or VMs, and epidermal nevi.25,26 They have
characteristic upper extremity anomalies including macrodac­
tyly, syndactyly, joint contractures, wide spade-like hands,
with hyperextension/hyperabduction of the thumb. Previ­
ously, most of these cases had been called “macrodactylies”,
“gigantism”, or Proteus and did not fit the criteria for the
Proteus syndrome (see Table 31.2
).27,28
FAVA was recently described as an entity similar to but
distinct from venous malformation.24 This lesion is dense and
does not contain large vascular channels amenable to tradi­
tional sclerotherapy or steroid injections. It usually affects
muscles in the extremities. Pain is the hallmark symptom and
surgical resection the only known effective treatment.
Another group of confusing lesions can be found in the
fast-flow group, where all lesions should not be called Parkes
Weber (PWS) anomalies. Most fast-flow lesions are easily
diagnosed by their clinical characteristics, palpable bruits,
shunts, and progressive growth. A small group of these chil­
dren seen by the extremity surgeon may have mild hypotonia,
fast-flow deeper lesions and frontal bossing. Males have a
nevus on the glans of the penis. In the past they were placed
in the Bannayan–Riley–Ruvalcaba syndrome (BRRS) or the
Cowden syndrome. Genetic testing is diagnostic and they are
now called the PTEN hamartoma-tumor syndrome (PHTS)
(see Table 31.8).29,30 The hand surgeon may also see the capil­
lary malformation–arteriovenous malformation (CM–AVM)
syndrome, which initially appears to be an innocent CM,
which are pinkish red and surrounded by a small halo. One-
third of these patients may have an existing AVM and 12%
may evolve into a PWS and intracranial AVMs should be
considered. This condition is hereditary and is caused by a
mutation in RASA1.31,32 The PWS describes a diffuse AVM of
an overgrown extremity with an overlying CM.33 In contrast
to lesions with direct arterial–venous shunting (AVMs with
arteriovenous fistulas [AVFs]), PWS lesions show diffuse
involvement of all soft-tissue structures with fast-flow arteri­
oles in the initial absence of large draining veins. Large shunts
develop secondarily. The lower extremity is involved much
more frequently than the upper limb.
Diagnosis/patient presentation
Evaluation begins with a detailed history and physical
examination. In contrast to the rapid growth and slow
involution that characterize infantile hemangioma (IH),
vascular malformations usually continue to grow. Although
vascular malformations are by definition present at birth,
many are inconspicuous and some do not appear until late
childhood. Dermal ectasias, such as the relatively common
capillary malformation (“port-wine stain”), are usually noted
in the newborn nursery. Rarely, later in life will it become
obvious that some of these capillary stains overlie a slow-flow
(venous or lymphatic) anomaly or a fast-flow (arteriovenous)
malformation. Venous and lymphatic malformations often
coexist and may be quite insidious in their initial presentation.
Venous malformations may not manifest until late childhood
or adolescence; large lesions are usually obvious within the
first 4–8 years of life. Fortunately, the vast majority of vascular
malformations are correctly diagnosed by clinical examina­
tion alone.4,15,34,35 It is important to obtain baseline functional
data including length and circumference of the affected and
unaffected limbs early in life and to follow these children as
they grow. The clinical characteristics of the more common
malformations will be described with the individual case
presentations.
The diagnostic work-up varies for each type of vascular
anomaly (Table 31.3). Ultrasonography is the “go-to” initial
radiological test for these lesions, and can easily confirm the
initial distinction between a vascular tumor and vascular
malformation.36 In our center, the ultrasound is typically done
in the clinic room with a portable machine. This diagnostic
tool not only pinpoints the location of the lesion but also
enables its identification as either slow-flow or fast-flow.
Expensive diagnostic studies are unnecessary in the case of a
diffuse or localized, asymptomatic slow-flow malformation or
a small fast-flow lesion amenable to simple excision and
closure. When a patient is symptomatic or if an operation is
being considered for a large vascular anomaly, one should
learn as much as possible about the size, extent, depth, and
three-dimensional (3D) characteristics of the lesion. Magnetic
resonance imaging (MRI) with contrast (gadolinium) is the
“gold standard”. Fat-suppressed T1-weighted sequences
with contrast will differentiate between a vascular tumor and
malformation.37 MRI can also help distinguish between lesions
similar to VM such as FAVA24 or intramuscular hemangioma
(Enzinger type).38
Computed tomography (CT) can be used to detect interos­
seous involvement or skeletal distortion caused by a vascular
malformation and help delineate soft-tissue planes. Large
cystic cavities in an LM are clearly depicted. New 3D render­
ing of both fast-flow and slow-flow malformations using CT
technology provides very impressive views of the entire
malformation and its relationship to neighboring soft tissue
and skeletal structures. Three-dimensional rendering of com­
puted tomography angiography (CTA) can be invaluable
preoperatively. Plain radiology of the affected region provides
very little information other than showing the presence of
phleboliths and interosseous involvement or other skeletal
distortions. Angiography has very little use in the diagnosis
of slow-flow malformations, although it is indispensable in
the diagnosis and assessment of fast-flow lesions.15 The angio-
architecture and 3D anatomy of the malformation can be
precisely determined and the arterial suppliers and draining
veins clearly identified. Indirect and direct phlebography
techniques are sometimes used for diagnostic and therapeutic
purposes for VM in the extremity.

691
Treatment/surgical technique
to treat large, difficult lesions with promising outcomes.39
Despite many advancements, however, a subset of these
lesions is still best treated with surgery.
Surgical treatment of vascular anomalies is not an endeavor
for the timid or the arrogant (Video 31.1
). A well-balanced
approach that incorporates calculated risk and cautious execu­
tion is necessary – one that can only be cultivated with experi­
ence. The surgeon who devotes a practice to the treatment of
these lesions is obligated to document meticulously and
analyze critically – habits that will serve one well for life-long
learning. Most lesions can never be absolutely eradicated.
Re-expansion or recurrence is the rule, despite significant
Treatment/surgical technique
The treatment of vascular anomalies has evolved significantly.
Although many of these lesions were treated surgically in the
past, advanced knowledge and minimally invasive techniques
have changed our approach. Now, most of these lesions are
best treated by interventional radiologists. As we further
elucidate the molecular basis of vascular anomalies, medical
therapy continues to improve. Examples include steroids and
beta-blockers for infantile hemangioma. Moreover, sirolimus
(also known as rapamycin) is being used more frequently
Table 31.3  Vascular anomalies and overgrowth conditions
Diagnostic work-up
Condition
Hemangioma
CM
VM
Physical examination
++++
++++
++++
Ultrasound
+++
−
+
MRI, MRV, MRA
++
−
+++
Radiographs
−
−
++ phleboliths bone
CT
+
−
+
Angiography
−
−
−
Biopsy
+
−
+
LM
Combined
AVM
Physical examination
++++
++++
++++
Ultrasound
+
+
+++
MRI, MRV, MRA
+++
+++
+++
Radiographs
+ bone
+ bone
+ bone
CT
+
+
+
Angiography
−
−
++
Biopsy
+
+
+
AVM, Arteriovenous malformation; CM, capillary malformation; CT, computed tomography; LM, lymphatic malformation; MRA, magnetic resonance angiography; MRI,
magnetic resonance imaging; MRV, magnetic resonance venography; VM, venous malformation.

692
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
to contracture. If a palmar approach is chosen, a zigzag
incision is preferred, utilizing existing skin creases. If
multiple debulking procedures are contemplated in the
upper arm or forearm, each incision should be planned
carefully to avoid unnecessary scarring. The medial
surface of the arm, elbow and forearm is the least
conspicuous. Incisions in the weight-bearing plantar
surfaces of the foot should be avoided. The placement
of incisions in the medial arch, along the borders of the
foot, and on the dorsal surfaces is safe. Scars in the
webspaces or along eponychial and/or paronychial
folds will contract and are likely to become problematic.
4. Magnification, either loupe or microscopic, makes a
tremendous difference in the identification and
preservation of normal neurovascular structures. Small
vessels, such as vincular pedicles to the flexor tendons
and nutrient vessels to the carpal bones, should be
saved if uninvolved. Often, vascular anomalies will
displace, but not typically invade, neighboring soft
tissues.
5. Avoid vascular compromise. Only one-half of a digit
should be dissected at a time (see Table 31.6). If possible,
at least one or two large dorsal veins per finger should
be preserved to enhance venous drainage, especially
when excising a diffuse lymphatic lesion in the hand or
foot. The superficial venous system should not be
resected unless a deep system has been identified. When
a critical arterial segment to a digit, hand, or foot is
removed, this segment should be reconstructed with a
vein graft so that at least one digital artery is preserved
per digit and one major artery supplies the hand or foot
with a functional intact palmar or plantar arch.
6. Avoid intraneural dissection whenever possible, despite
gross involvement. Although many vascular
malformations, particularly VMs and PTEN lesions, are
entangled in nerves, dissection often leaves
in-continuity neuromas with partial or complete loss of
distal sensory or motor function. The symptoms are
usually much worse than the initial ones.
7. Avoid partial dissections within large muscle groups.
Removal of the entire muscle en bloc is preferred to
avoid a secondary contracture of the entire
musculotendinous unit. If more than half of a skeletal
muscle is excised piecemeal, a secondary contracture is
likely. VMs, mixed CLVMs, capillary malformation-
arteriovenous malformation (CM-AVM) and
lymphatico-venous malformations (LVMs) are the most
problematic lesions. The best treatment for large FAVA
lesions appears to be en bloc excision of the entire
involved muscle.
8. Replace abnormal or ischemic skin with a skin graft or a
flap. Tissue with questionable viability can be observed
and resected later, if necessary. It is usually best to
remove and replace skin that is heavily scarred from a
previous procedure, chronically infected and ulcerated,
populated by lymphatic vesicles or deprived by a
proximal steal phenomenon.
9. Drains should be used liberally, and delayed primary
closure of the wound should be considered. Persistent
postoperative bleeding is usually best treated with
direct pressure, elevation, and immobilization, rather
extirpative operations. This is an important consideration
`in preoperative planning, patient counseling, and future
interventions.
Surgical principles gain acceptance with time and must be
periodically reassessed and refined, particularly in this rapidly
advancing field. Principles should not be confused with
techniques that change frequently, and are modulated to a
great extent by technology. Many of the following surgical
principles have evolved from the early operative experience
with vascular malformations (especially the fast-flow types),
which was punctuated with complications (Table 31.4).2,3
1. Preoperative planning should include correlation of the
size, extent, and involvement of structures with physical
examination and imaging. All studies should be
reviewed preoperatively; serial studies of growing
children are often invaluable in demonstrating the true
extent of involvement. A thorough explanation of all
potential complications must be given to the patient
and/or parents.
2. The surgeon should mentally outline the extent of the
resection and abide by it. Use of the pneumatic tourniquet
and complete exsanguination is necessary to visualize
normal and abnormal structures clearly, regardless of
the size of the malformation. Once the operative field
becomes bloodstained, the identification of nerves,
tendons, intrinsic muscles, and other structures is more
difficult.
3. Placement of incisions is important – particularly in
children. A high midaxial incision in the digit is
preferred, as it can be used again and is hidden. With
growth, palmar scars within or near the palm can lead
Table 31.4  Principles of management
1. Clear explanation of surgery and complications. Give parents
time to make decisions
2. Carefully planned and thorough dissection within a well-
defined region
3. Meticulous hemostasis under tourniquet control, bloodless
field
4. Fine instruments, magnification, good assistance
5. Judicious incision placement. Avoid dorsum of hand or digit
6. Identification of all structures, preservation tendons, joints,
and nerves
7. Dissect no more than half of a digit or limb at a time
8. Avoid vascular compromise. One side at a time. One vessel
intact
9. Avoidance intraneural dissections. Neuromas are a nemesis
10. Quick operating rehearsed with assistance if possible
11. Resect marginal skin. Tension free closures. Good tissue
coverage
12. Thoughtful, meticulous closures. Subcuticular techniques best
13. Liberal use of drains, tissue sealants, and delayed closures
14. Appropriate early immobilization and subsequent early joint
range of motion
15. Aggressive treatment of complications. Amputation of
functionless extremities
16. Conscientious, regular follow-up for those with significant
malformations
17. A full explanation of potential complications to parents and
patients

693
Vascular tumors
than by re-exploration. Baseline coagulation studies
should be performed for large/diffuse VMs and
LVMs. Liberal use of tissue sealant products is
encouraged.
10. Immobilize the operated extremity in a young child or
adolescent. In the treatment of children with vascular
anomalies, the postoperative lack or loss of
immobilization is the single most important cause
of wound dehiscence, maceration, and chronic
infection.
11. The surgeon who has the courage to treat difficult
fast-flow lesions should also be prepared to amputate a
symptomatic non-functional or painful digit or extremity
following unsuccessful attempts at palliation. The
reconstructive surgeon need not view amputation as a
failure.
12. Follow-up evaluation should be performed compulsively
at yearly intervals. The true extent of the lesion can
never be absolutely eradicated short of an amputation.
Early childhood, adolescence, and pregnancy are times
when change may occur in vascular malformations due
to hormonal influences. Malformations may expand
dramatically during pregnancy, with use of high-
estrogen anti-ovulation medication, or following
trauma. Large AVMs, LMs or mixed lesions may be well
tolerated as young children only to become burdensome
as teenagers due to their expansion, bulk, ulceration,
and appearance or steal symptoms.
13. Give the patient and parents adequate time to make their
decision. The diagnosis and treatment of vascular
anomalies is a rapidly changing field. It is important to
keep both parents and patients at the appropriate age
clearly informed about the natural history of their
particular malformation, options for treatment
and new types of therapies. These patients and
parents often have questions that are not easily
answered by their family physician. It is axiomatic that
the surgeon provide a clear explanation of all potential
complications and expected outcomes prior to any
surgical treatment.
Vascular tumors
Infantile hemangioma (IH)
Basic science/disease process
IH is usually single and involves the upper limb in 15% of
cases. The median time of appearance is at age 2 weeks. When
dermis is involved, the skin initially appears pink but can
evolve to deep red. Deeper lesions may cause a pale, bluish
discoloration (Fig. 31.1). This lesion grows very fast during
the first 9 months (proliferating phase), begins to shrink by 12
months (involuting phase), and involution is complete by 5
years (involution phase) leaving residual telangiectasias,
fibrofatty residuum and redundant, atrophic skin.34,37,40
Diagnosis/patient presentation
Some 90% of IH diagnoses are made by history and physical
examination. Handheld Doppler demonstrates fast flow.
Ultrasonography shows fast flow, elevated resistance and
increased venous drainage.36 On MRI scans, hemangiomas are
isointense on T1, hyperintense on T2, and enhance during
proliferating phase. Rarely is biopsy needed. Immunostaining
for GLUT1, a glucose transporter specific to IH, will differenti­
ate this lesion.10
The majority of IH involve the head and neck, lumbosacral
region, and liver. The PHACES association includes IH on the
face with at least one of the following: posterior fossa brain
malformation, hemangioma, arterial cerebrovascular anomaly,
coarctation of the aorta and cardiac defects, eye/endocrine
abnormalities, and sternal clefting or a supraumbilical raphe.
These children need the appropriate specialty consultations.
Treatment/surgical technique
Most IH are managed by observation because 90% are small,
localized, and do not impair function. To protect against
ulceration and/or maceration during the proliferating phase,
topical antibiotic cream and a petrolatum gauze barrier may
be used. Ulcerated wounds are treated with local wound care
and rarely need biologic coverage such as skin grafts. Those
Clinical tips
In large lesions, it is usually best to do a staged excision.
Thorough dissection limited to a specified area should be
performed so that subsequent re-entry into a densely scarred
bed will be unnecessary. Limited staged (but thorough)
excisions are usually better than one extensive dissection that
leaves behind abnormal tissue. This way, the surgeon can
minimize likelihood of re-entering an area of previous
dissection. For example, the digits (including the thumb)
should be debulked in stages. We typically debulk one side of
a digit through a midlateral incision (half of the dorsal and half
of the volar surface), and return several months later to debulk
the other side. A single procedure is usually best for the
dorsum of the hand, wrist, and palm of the same extremity in
a patient with a diffuse LM or VM. In these patients, it usually
best to start distal to minimize swelling of involved digits once
the more proximal structures are debulked.
Clinical tips
Caveats of palmar dissections
Multiple technical refinements have made these dissections,
once considered impossible, both predictable and safe. These
are summarized in Table 31.5.
Clinical tips
Caveats of thumb/digital dissections
Surgical approaches to the thumb and digit are similar to
those summarized above and have become much safer
with microvascular techniques. These are summarized in
Table 31.6.

Table 31.5  Caveats of palmar dissections
1. Anatomy complex. Have three-dimensional perspective.
2. Compulsive hemostasis under tourniquet. Avoid inkwell phenomenon.
3. Proximal to distal dissection of nerves. Look for epineural fat and follow epineural connective tissue plane.
4. Identify deep motor branch of ulnar nerve in Guyon’s canal and mid-palm.
5. Median and ulnar nerves often involved. Take your time.
6. Although displaced, arterial is anatomy normal. Follow adventitial connective tissue plane. Save palmar arches.
7. Completely resect involved intrinsics to avoid contracture.
8. Try to preserve first dorsal interosseous and adductor pollicis muscles.
9. Simple muscle splitting for localized calcified thrombi.
10. Tissue sealants and drains to avoid bleeding
11. Postoperative compression of thumb and digits easy with Coban
12. Postoperative compression of palm difficult. Wrapping ineffective. Uniform pressure with vacuum-assisted closure device for open
wounds or dorsal/palmar splints for closed wounds.
13. Stay within a well-defined region. Tendency to be too aggressive
14. Resect and replace marginal skin.
Diffuse VM palm
Motor branch of the ulnar nerve
Dissection complete
Table 31.6  Caveats of thumb/digital dissections
1. Anatomy much easier than palm; outline region to be debulked before exsanguination
2. Incisions: midaxial or midlateral, avoid zigzag incisions in glabrous skin to avoid hypertrophic scars; utilize dorsal extension
creases if necessary
3. Tourniquet control: digital for distal half and regular proximal digit and thumb
4. Subdermal flap elevation 1st, deeper dissection later
5. Decompress Grayson and Cleland ligaments to identify neurovascular bundle; these structures distorted but always present
6. One side of digit (palmar and dorsal at a time; leave one bundle untouched and do not dissect more than 270° around digit.
7. Identify and dissect along paratenon of extensor; remove malformation between extensor and bone.
8. Preserve vincular vessels at joint space level and dorsal sensory branches of digital nerves: dissect along adventitial planes
(arteries) and epineural plane (nerves); keep neurovascular bundle intact.
9. Complete excision of malformation; extend along vincular vessels if necessary.
10. Palmar dissection restricted to ipsilateral side of digit or thumb
11. Palmar pulp dissection: “berry pick” VM, LM, or LVM out of septal pockets and keep nerve attachments to skin intact
12. Extend dissection into palm to level of metacarpophalangeal joint and distal palmar flexion crease.
13. Tissue sealants and drains to avoid bleeding
14. Drape redundant skin over digit and release tourniquet before resection; tension free closure.
15. Postoperative compression dressing held with Coban
Diffuse VM
Ulnar neurovascular bundle
Radial neurovascular bundle
Closure

695
Vascular tumors
be excised in one stage, whereas larger ones require a staged
approach.
Congenital hemangioma
Basic science/disease process
The upper limb surgeon must be familiar with a variant
known as congenital hemangioma (CH).45 In contrast to
infantile hemangioma, congenital hemangioma are rare
lesions fully grown at birth that do not exhibit the prolifera­
tion and regression so typical of IH. These lesions appear
differently with a red-violaceous color, coarse telangiectasias,
a central pallor, and a peripheral halo. There are two varia­
tions of CH: the rapidly involuting congenital hemangioma
(RICH)46 and the non-involuting congenital hemangioma
(NICH)47 (Fig. 31.3). Both lesions are commonly seen in the
extremities and have an equal sex distribution. RICH invo­
lutes rapidly after birth; 50% of the lesion is gone by 7 months
and involution is complete by 14 months of age. Fibrofatty
deposits are not present. NICH does not regress and persists
as raised, lumpy, plaque-like lesions with a characteristic
peripheral halo. A persistent fast-flow component remains
unchanged.
that are small but cause functional problems can be injected
with steroids.41
Problematic lesions too large to treat with intralesional
corticosteroid injections are first managed with oral predni­
sone started at 3 mg/kg per day for 1 month and then tapered
until it is discontinued 10–12 months later.37 Complications of
long-term corticosteroid therapy are well known and usually
disappear with discontinuation of therapy. Recently, pro­
pranolol has been used but its efficacy and safety compared
to corticosteroids is debated.42,43 Atenolol may have a better
risk profile than propranolol, and its use is gaining substantial
support.44 Alternatively, the child may be switched to vincris­
tine; interferon is no longer used in those under 12 months of
age due to spastic diplegia.
Surgery in the upper limb (during the proliferation phase)
is indicated only for problematic ulcerations or large lesions
interfering with function. The particular child in Fig. 31.2 has
a large, chronically ulcerated lesion that envelopes the neuro­
vascular structures. Excision and neuroplasty through a high
midaxial incision both enabled functional use of the digit and
avoided secondary contracture of the digit.
After involution, the hemangioma can leave redundant
skin with fibrofatty soft tissue. Though these lesions pose no
functional problem, they can be unsightly. Small lesions can
A
B
C
Fig. 31.1  Infantile hemangioma (IH). (A) This well-localized, solitary IH on the dorsal forearm has a bluish hue. (B) The MRI shows a multilobular, isointense lesion on
T2-sequence. (C) Excision included extensive dorsal tenosynovectomy. (D) Two weeks later active extension is initiated.
D

696
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
A
Fig. 31.2  Infantile hemangioma (IH). (A) An expanding IH with chronic ulceration
and bleeding in a 9-month-old child. (B) Excision was performed through a high
midaxial incision and normal skin creases.
B
Diagnosis/patient presentation
Diagnosis is made by physical examination, clinical course
and ultrasonography. CH lesions do not stain for GLUT1.
Treatment/surgical technique
RICH does not require operative management early in life,
as it undergoes accelerated regression. Only large lesions in
other parts of the body may need oral corticosteroid or second-
line drug therapy. NICH may require intralesional corticoste­
roid injection in problematic regions of the upper limb. Large,
lumpy masses within the palm of the hand, interdigital web­
spaces, or those causing muscle or tendon impairment are best
excised. Large lesions on the arm, forearm, and within the
antecubital fossa are excised later in childhood.
Pyogenic granuloma
Basic science/disease process
Pyogenic granuloma (PG) is a solitary red papule that grows
rapidly, forming a stalk. It has been called a lobular capillary
hemangioma.48 It is small with an average diameter of 6.5 mm.
The male to female ratio is 2 : 1. It commonly involves skin
and, in the upper extremity, it is most problematic on the
glabrous surfaces of the digits, in nail folds (Fig. 31.4), within
webspaces and flexion creases. It appears as a red papule
growing on the end of a stalk. Persistent bleeding and constant
ulceration are common. PG is distributed all over the body
including the mucous membranes; cheeks, lips, oral cavity,
eyelids and forehead. Histological examination will differenti­
ate PG from hemangioma.49
Diagnosis/patient presentation
Diagnosis is made by physical examination because the
appearance is classic.
Treatment/surgical technique
Numerous treatments have been described for PG: curettage,
shave excision, laser therapy, or excision. Complete excision
is preferred in the hand and upper limb. Skin graft coverage
may be necessary for large resulting defects.
Vascular malformations
Capillary malformations
Basic science/disease process
CM consists of dilated capillaries in the superficial dermis.
CM is most often solitary, but has a wide range in size and
may occur in any region of the extremity (Figs. 31.5A,B &
31.5C–F
). With time and aging, these pink lesions will often
darken and develop some fibrovascular overgrowth. They can
be associated with deeper vascular lesions, both slow-flow
(Klippel– Trénaunay, CLOVES) and fast-flow (Parkes Weber,
CM–AVM, PTEN) (see Tables 31.2 & 31.8
) and with skeletal
and soft tissue hypertrophy. A CM on the face in one or
more of the trigeminal nerve distributions is indicative of

697
Vascular malformations
awake and with topical anesthesia. More dramatic outcomes
are obtained in the head and neck region and face than in the
trunk and extremities. Multiple treatments are often neces­
sary. Outcomes in younger children are better than adults;
results show 90% lightening of lesions, 50–90% improvement,
and 20% minimal change. Many will re-darken with time.53
Occasionally, a dark purple, overgrown extremity CM will be
resected or replaced with a graft.
Lymphatic malformations
Basic science/disease process
LM results from an error in the embryonic development of the
lymphatic system. Since lymphatic and venous systems have
Fig. 31.3  Congenital hemangioma (CH).
(A) Extensive skin ulceration may occur
during proliferation and involution. CH
requires local wound care. (B) A
non-involuting congenital hemangioma
(NICH) shows coarse telangiectasias, hard
nodules surrounded by a peripheral halo.
(C) A rapidly involuting congenital
hemangioma (RICH) shows typical
enlargement and coarse telangiectasias
during infancy. (D) Several years later with
no treatment.
A
B
C
D
Sturge–Weber syndrome.50 These patients are at risk for sei­
zures, retinal detachment, glaucoma, and stroke. Fig. 31.5C–F
appears online only.
Diagnosis/patient presentation
Diagnosis is made by physical examination. Deeper
lesions must be studied with radiographs, scans, and
ultrasonography.
Treatment/surgical technique
No treatment is necessary for extremity CMs. Pulsed dye laser
(585 nm) can effectively decrease the intensity of the cutane­
ous blush.51,52 Patients (including infants) are often treated

698
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
and this skin may be thick and exhibit cutaneous puckering
much like the peau d’orange skin seen in lymphedema. Cutane­
ous vesicles may coalesce, drain clear fluid, bleed, and lead to
chronic wounds and sepsis.
LM may be located anywhere within the upper limb and
is characteristically macrocystic in the cervicofacial and neck
(“cystic hygroma”), mediastinum, and axilla (Figs. 31.6 &
31.7) and microcystic in the distal forearm, wrist, and hand.
LMs may be accompanied by increased amounts of dysplastic
fat adding to the bulk of the malformation. It is unusual for
LMs to directly involve muscle tissue as they are primarily
found within the subcutaneous tissue planes. The most prob­
lematic regions are within intertriginous skin folds, interdigital
webspaces, nail folds and within open wounds caused by
vesicle leakage.
There is no characteristic pattern of skeletal involvement
with LM. The long and tubular bones of the extremities and
joints are rarely affected. The eponym “Gorham disease” is
used for skeletal involvement.56 Extensive LM may be associ­
ated with skeletal disuse atrophy. Smaller lesions confined to
the distal extremities can present with overgrowth of the hand
or foot. LMs and combined lesions containing lymphatic
components (CLVM, LVM) can progress to gigantism. Joints
are rarely affected but can become severely restricted by the
bulky LM (see Table 31.2
).
Diagnosis/patient presentation
Some 90% of LMs are diagnosed by history and physical
examination. Small, superficial lesions do not require further
diagnostic work-up.34 Large or deep lesions may require MRI
scans to confirm the diagnosis, define the 3D characteristics
a common origin, combined lymphatico-venous malforma­
tions (LVM) occur frequently.54,55 LM is characterized by the
size of the malformed channels: microcystic, macrocystic, or
combined. Most lymphatic lesions are noted at birth or within
the first 2 years of life. Large macrocystic lesions may be
slightly compressible and those with smaller channels are
rubbery, a characteristic distinguishing feature. They do not
decompress with overhead elevation. Overlying skin may
appear normal, have a bluish hue or contain clear or pink
vesicles. Dark vesicles are indicative of intralesional bleeding.
Dermal involvement often overlies a large lymphatic lesion
Fig. 31.4  Pyogenic granuloma (PG). A periungual PG with typical stalk will
continue to bleed unless completely removed with curettage or excision. In the
young child, this must be differentiated from an unreduced Salter I injury of the
distal phalanx (Seymour fracture).
A
B
Fig. 31.5  Capillary malformation (CM)
variations. (A) Solitary CM over the thenar
region in a teenager; stain-darkened during
the adolescent growth spurt. (B) Localized
CM plus large arteriovenous malformation
(AVM) with multiple arteriovenous fistulas
in a young child with a palpable bruit.

698.e1
Vascular malformations
C
D
F
E
Fig. 31.5, cont’d (C) CM plus overgrowth of thumb, long and ring digits in a child with CLOVES. (D) Extensive CM
plus lipomatous overgrowth, syndactyly, and no skeletal overgrowth. (E) Limb of child with CM–AVM. (F) CM in an
older man. Note variable effect of laser treatment on the dorsal forearm. CM darkened with cobblestone appearance
during the adult years.

699
Vascular malformations
of the LM and plan treatment. These scans show characteristic
fluid-filled spaces, multiple septations of variable thickness,
hyperintense T2-weighted sequences due to water content,
and slight rim enhancement following contrast administra­
tion. Diffuse enhancement is not present. Direct puncture
of macrocystic lesions further delineates the architecture of
the lesion prior to sclerotherapy. Large venous channels are
often present and bleeding into a large macrocyst is the rule
rather than the exception.57 Ultrasonography (US) shows
multiloculated macrocysts with “rim enhancement” and
hypo-echoic microcystic lesions with little flow. Histologic
confirmation of LM is rarely necessary. LM characteristically
shows abnormally walled channels filled with eosinophilic,
protein-rich fluid and collections of lymphocytes. Positive
immunostaining is seen with lymphatic markers D2-40 and
LYVE-1.58
Treatment/surgical technique
LM is a benign condition, and intervention for small asymp­
tomatic lesions is not necessary. More aggressive management
is reserved for symptomatic lesions that cause pain, infection,
compression neuropathies, significant deformity, or functional
impairment. An infected LM within the extremity can be con­
trolled with local wound care, oral antibiotics, and occasionally
intravenous antimicrobial therapy. The rapidly advancing
“wild fire” infections seen in these lesions can cause sepsis but
will regress as rapidly as they progressed with appropriate
treatment. Sclerotherapy is the first-line management for large,
problematic macrocystic LMs. Several sclerosants have been
used: doxycycline,59 sodium tetradecyl sulfate (SDS), ethanol,
bleomycin,60 and OK-432.61 SDS is the most widely used.
Ethanol has the highest complication rate and is particularly
toxic to neurovascular structures (see Fig. 31.14). OK-432 is
very effective but not widely available. Multiple injection ses­
sions are required for all but the smallest lesions. Swelling,
compartment syndromes and skin extravasation are the most
common complications encountered in the upper limb. We
prefer sclerotherapy for the macrocystic LMs primarily located
in the head and neck, axilla, and proximal portions of the upper
extremity. Microcystic lesions are best treated surgically.
Resection is reserved for: (1) symptomatic LM causing
bleeding or fluid loss, infection, bleeding, pain, or obstruction
of function; (2) lesions (microcystic) that cannot be adequately
treated with sclerotherapy; and (3) small, well-localized lesions
that can be resected for cure. Surgical principles and technical
caveats have been outlined. The neonate in Fig. 31.6 presented
a good example. Despite sclerotherapy, issues such as bleed­
ing, fluid shifts, and metabolic derangement could not be
adequately managed. Resection included a careful identifica­
tion of all neurovascular structures within and beyond the
brachial plexus and excision of large amounts of involved
tissue. Characteristically, the LM in this baby was confined to
the subcutaneous tissue planes and extended only between
muscle groups. Staged debulking of large upper extremity
LMs is much safer than large radical resections. We often start
with the bulkiest chest wall, axillary, and arm regions before
extending more distal into the forearm and hand (see Fig.
31.7). Areas of involved skin are preferentially removed and
only one side of a forearm, wrist, hand, or digit are debulked
at one operation. Flap compromise, wound dehiscence, and
infection are early problems and tendon adherence, neuromas,
and joint stiffness may be encountered later.
Because of its complex anatomy, the hand is the most dif­
ficult region to dissect for an LM or any other type of vascular
malformation. There are very few natural fascial planes within
the indurated lymphatic tissue, which may have been scarred
by previous inflammation. However, smooth, areolar connec­
tive tissue planes can usually be found around both nerves
and arteries and should be followed meticulously. Of all
surgical principles outlined previously, limited and thorough
dissection is most important for LM in the hand (see Table
31.4). In contrast to the digits and thumb, postoperative edema
is almost impossible to control, and may affect joint mobility
and tendon gliding. At this level, all LMs are microcystic
and are accompanied by a large amount of adipose tissue,
with the consistency ranging from soft to hard rubber (see
Fig. 31.8B). Resection in the hand is reserved for those lesions
causing functional impairment. These procedures are lengthy
A
Fig. 31.6  Macrocystic axillary lymphatic malformation (LM) in a neonate. (A) This
extensive LM was diagnosed by prenatal ultrasonography; child born by caesarean
section. Constant fluid shifts and intralesional bleeding complicated initial
management. (B) At 6 months, an aggressive debulking was performed with
isolation and preservation of all neurovascular structures. Surgery took 6 h with a
blood loss of <100 cc. A dramatic result was obtained with compression wrapping.
B

700
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
surfaces of the palm or digit have a predilection for hypertro­
phic scarring. Intradigital commissures and webspaces must
be closed in their normal configuration. Coban™ effectively
controls postoperative edema with circumferential wrapping.
Fig. 31.9B,C appears online only.
Outcomes, prognosis, and complications
The degree of functional gain and appearance is proportional
to the extent of the malformation. The use of the pneumatic
tourniquet in limb surgery significantly decreases the recur­
rence rate seen in the head and neck region.62,63 Frequency of
complications such as persistent drainage, wound dehiscence,
and skin loss is less than 8%; secondary neuromas and
hypertrophic scarring are much more common (20%).15 With
appropriate postoperative therapy and splinting, joint stiff­
ness can be minimized. Upper extremities with large, heavy,
edematous hands will always be “helping extremities” and
digits with limited range of motion will never be normal due
to the scar from pervious surgeries and residual LM within
the remaining soft tissue. However, extremities with well-
localized LM can be expected to function normally following
well-planned and executed surgery.
and tedious. Incisions through glabrous skin must be well
designed, as there is a tendency for hypertrophic scar forma­
tion, particularly when there is dermal involvement. With
dorsal debulking, several large dorsal veins should be pre­
served in-continuity with the cephalic drainage system of the
forearm. Nevertheless, removal of these veins does not pre­
dictably cause venous congestion (see Table 31.5).
Dorsal LMs in the thumb and fingers are common and are
the easiest in the limbs to debulk (see Table 31.6). Muscle is
usually not involved, and the LM usually does not extend
below or penetrate through flexor or extensor tendons. The
entire dorsal area can be debulked, along with removal of
one-half of any involved palmar portion in one procedure.
Liposuction, even with ultrasonic technique, is not a good
option because of the firm, rubbery consistency of microcystic
LM and potential for injury to neurovascular structures.
Involved dorsal skin should be excised and replaced by thick
split-thickness or full-thickness skin grafts. Multiple digits
can be debulked during one operation, and the dorsum of the
hand at another stage. Midaxial incisions are preferred (Figs.
31.8 & 31.9A–C
). A large dorsal vein in the hand or digit is
preserved. Vincular vessels at the joint space level are pre­
served whenever possible. Incisions through the glabrous
A
B
Fig. 31.7  Chest wall and forearm lymphatic malformations (LM). (A) A rubbery, hard LM involving the entire arm and chest wall with a normal hand in a 16-month-old
child. (B,C) Staged resections started with the chest wall where pectoralis major, latissimus dorsi, and serratus anterior muscles have been preserved. The forearm was
debulked in two additional stages. (D) Three months postoperatively, following three operations.
C
D

701
Vascular malformations
gradual dilatation and expansion with time and growth.3
Although VMs are present at birth, they are not always
obvious. Within the first 5–6 years of life, all but the smallest
or deepest lesions will become clinically evident. Less than
10% are clinically indolent until adolescence. Approximately,
40% of all VMs involve the extremities and more than half of
these involve deeper structures including muscle, bone, nerve
and connective tissue planes. They grow commensurately
with the child, and slowly expand during the adolescent
growth spurt. VM is typically sporadic and solitary and half
have a somatic mutation in the endothelial receptor TIE2.64
VM may range from small, localized skin lesions to diffuse
malformations involving all tissue planes including bones
and joints. These lesions become engorged when the limb is
in a dependent position and decompress when the limb is
held above the level of the heart (Fig. 31.10). Most VMs are
located in the subcutaneous plane superficial to the muscular
fascia, but in contrast to LM, VM may involve muscles
directly.65 The majority of VMs are solitary and asymptomatic.
Often they are multiloculated, incompletely decompressed
Fig. 31.8  Lymphatic malformation (LM) hand. (A) A 4-year-old child presented with an extensive
microcystic LM of the entire forearm, wrist, and hand. The dorsum was approached through an ulnar incision
with preservation of dorsal venous system, nerves, and extensor tendons. (B) The LM along the digits is
microcystic, indurated and envelops all structures. Joint spaces are not involved. (C) Multiple digits and
thumb are debulked during a single stage. (D) With continued compression wrapping and exercise, contour
and function have been maintained 15 years later.
A
B
C
D
Fig. 31.9  Lymphatic malformation (LM) digit. (A) LM along the side of a digit.
A
Venous malformation
Basic science/disease process
VM is the most common vascular anomaly. It is comprised of
thin walls and abnormal smooth muscle cells, arranged in
clumps instead of in a concentric orientation. VMs show

701.e1
Vascular malformations
(B) Lymphatic microcysts and small venous thromboses are
studded within dysplastic adipose tissue. (C) The neurovascular bundle has been
dissected with preservation vincular branches at the joint levels.
B
C
Fig. 31.9, cont’d

702
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
be followed carefully through adolescence and subsequent
pregnancies.15,35
Functional problems are related to the size, weight, and
location of the VM. Pain and paresthesias are usually the
result of local inflammation around intralesional thrombi or
nerve compression, usually at the elbow, wrist, hand and/or
tarsal tunnel of the foot. Areas of phlebothrombosis or local
hemorrhage become swollen, firm and painful, especially
when compressive garments are applied. Most symptoms are
aggravated after exercise involving repetitive movements
such as lifting, gripping, running, or kicking. Many VMs will
be surprisingly large with minimal, if any, symptoms in young
children.
Syndromes with VM
There is an emerging group of syndromes which contain VM
and which have become clinically distinct (Table 31.7
). Less
than 20% of all VM patients will have a syndromic designa­
tion with a very specific pattern of malformation including
with elevation, and have palpable regions of scar from previ­
ous inflammation around intralesional thrombi, which feel
like small peas in a sac.
The configuration, size, and caliber of venous channels in
VMs have no pattern, such as the characteristic microcystic
and macrocystic cavities and channels of an LM. Large-caliber
veins may be located on a digit, foot, and leg or within the
axilla. Often these large, dilated, redundant draining veins of
extremities place the patient at risk for clot formation and
subsequent pulmonary embolism.
VMs do not progress from a slow-flow to fast-flow state;
they grow (expand) commensurately with the child. Any
enlargement following a partial resection represents redi­
rection of flow into adjacent anomalous channels. There is
definitely hormonal modulation with medium and large
lesions. They often increase in size during the adolescent
growth spurt, menses, while on anti-ovulant medication,
and during pregnancy. In women who experience an exac­
erbation of their symptoms during pregnancy, there is no
postpartum reduction to the previous size. Patients must
Fig. 31.10  Venous malformation (VM)
hand. (A) In a dependent position, the hand
with a diffuse VM becomes engorged; bulk
and weight become a functional
impediment. (B) Calcified phleboliths are
often present. (C) Functional motion is
impaired only by the malformation.
(D) After elevation there is no pain,
fullness, or congestion. This patient has an
extensive VM involving the entire
hemithorax with a large stagnant venous
collecting system in the axilla.
Consequently, she is on long-term
anticoagulation.
A
B
C
D

703
Vascular malformations
glomuvenous malformation (GVM)66,67 and capillary malfor­
mation venous malformation (CMVM).68 In the blue rubber
bleb nevus syndrome (BRBNS),69,70 small blue nodules are
present in isolation or with extensive connections. A small
number of upper limb patients present with combined
lymphatic-venous malformations with or without capillary
staining: LVM, CLVM (Klippel–Trénaunay) Understandably,
this group of lesions can be confusing but the management is
similar. Table 31.7 appears online only.
Complications of untreated VMs include pain, swelling,
bulk, and contour deformity and psychosocial morbidity.
Intralesional thromboses are the most frequent cause for pain.
VM can cause leg-length discrepancy, hypoplasia due to
disuse atrophy, pathologic fracture, hemarthrosis, and degen­
erative arthritis. VM of muscle may result in pain, weakness,
fibrosis leading to joint contracture and subsequent disabil­
ity.65 Phlebothromboses within large, stagnant venous chan­
nels in the arm, axilla and ipsilateral chest wall have been the
focus for pulmonary embolism. A coagulation profile should
be obtained in all patients with an extensive VM, with or
without capillary or lymphatic components, particularly if
there is any history of bleeding, ecchymosis or hemarthrosis.
High-risk patients should be placed on anticoagulation
therapy (see below).
Diagnosis/patient presentation
At least 90% of VMs are diagnosed by history and physical
examination. Dependent position of the anomaly will confirm
the diagnosis: VM will enlarge due to reduced venous return
in the extremity (Fig. 31.10C,D). Small, superficial, asymptom­
atic lesions require no further work-up. However, larger and/
or deeper lesions should be evaluated by MRI to: (1) confirm
diagnosis; (2) define the 3D extent of the lesion, and (3) plan
long-term treatment. VM is hyperintense on T2-weighted
sequences. In contrast to LM, VM will enhance with contrast,
show phleboliths as signal voids, and may involve muscle. US
may be used for some localized lesions; characteristic findings
show slow-flow anechoic–hypoechoic channels separated by
more solid regions of variable echogenicity. Phleboliths are
hyperechoic with acoustic shadowing. Magnetic resonance
venography (MRV) is helpful for large lesions of the arm,
forearm, and chest wall. CT scans are used to assess skeletal
VM.57,71 Histological diagnosis of VM is rarely necessary, but
may be indicated if there is a suspicion of malignancy or if
the imaging is not confirmatory. Arteriography is not helpful
(see Table 31.2
).
Treatment/surgical technique
Compression garments are the first-line of treatment and are
used to reduce blood stagnation within the lesion, thereby
reducing the chance of expansion, localized intralesional
coagulopathy (LIC), phlebolith formation, and pain. Those
with pain and large lesions are given daily aspirin (81 mg) to
prevent clotting. Large lesions are at risk for the vicious cycle
of blood stagnation, stimulation of thrombin, and conversion
of fibrinogen to fibrin. Subsequent fibrinolysis results in LIC.
A chronic coagulopathy can cause either thrombosis (phlebo­
lith formation) or bleeding. Low molecular-weight heparin
(LMWH) is considered for those children and adults with
significant LIC or those at risk for disseminated intravascular
coagulation (DIC). Those with large VMs and low fibrinogen
levels pre-sclerosis are given LMWH 14 days before and after
the procedure. Anticoagulation is held for 24 h perioperatively
(12 h before and after the intervention) to prevent bleeding
complications. Patients who develop a serious thrombotic
problem, such as a pulmonary embolus, require long-term
anticoagulation or a vena caval filter.
Sclerotherapy is the next treatment option for symptomatic
lesions that cause pain, functional impairment, a mass effect,
or major contour problems.15,72 In the arm, elbow, and proxi­
mal forearm, sclerotherapy is safer and more effective than
resection (Fig. 31.11
). Symptomatic regions must be specifi­
cally targeted. Although sclerotherapy reduces the size of
the malformation, it generates a tremendous amount of scar
and does not remove the malformation. The VM usually
re-expands, and multiple sessions are invariably needed,
sometimes for a lifetime. Direct puncture sclerotherapy is
used for VM. Most patients are managed under anesthesia
using US guidance. The most common complications are skin
ulceration (10–15%), local extravasation, compartment syn­
drome in the arm or forearm and secondary contracture (see
Fig. 31.14A).(15) Sclerotherapy in the palm or near large
nerves can result in painful neuropathy and should be
avoided. Fig. 31.11 appears online only.
Post-treatment monitoring is required for large lesions with
an extremity surgeon on-call for problems. Large VMs of the
axilla and arm contain anomalous veins too large for sclero­
therapy; these should be embolized. Sclerotherapy is most
effective for small and large VM in the axilla, arm, elbow, and
proximal forearm where muscle may be directly involved.
Surgical resection is preferred in the distal forearm, wrist,
thumb, and digits, where there is little muscle tissue (Figs.
31.12
& 31.13). The secondary scar generated by sclero­
therapy in these regions is detrimental to nerve function,
tendon and muscle gliding and joint motion. Large vascular
channels respond better to sclerotherapy than small nests or
clusters of VM commonly seen in GVMs or Maffucci patients.
Surgical resection is preferred. We prefer to avoid sclerother­
apy prior to resection, as it can cause scarring and complicate
the dissection field. Fig. 31.12 appears online only.
Resection is reserved for VMs that are well localized in a
single muscle group (such as the intrinsic muscles of the
hand), lesions with thromboses, and those causing neurologic
impairment or compressive problems (see Table 31.5). In
the distal extremity, resection of the entire involved muscle
group is preferred, especially for a small muscle–tendon
unit. Examples include the intrinsic muscles for which other
muscles can substitute or the extrinsic flexors for which
tendon transfers are possible. Partial resection of these small
muscle–tendon units cannot be recommended; this fails to
improve function. Flexion contractures followed by prolonged
rehabilitation often occurs after subtotal resection of muscles.
The indications for surgical resection are primarily functional,
but can also be aesthetic.
The principles of treatment are summarized in Table 31.4.
The advantages of surgical resection in the upper extremity
are: (1) a pneumatic tourniquet is used to provide a bloodless
field; (2) all anatomic structures can be identified; (3) only the
malformation is removed; (4) nerves, muscles, and tendons
may be left injured; and (5) well-localized lesions can be
completely removed. However, the recovery time is longer;
the rehabilitation more difficult; and there is always a chance
of local VM re-expansion in adjacent regions.

703.e1
Vascular malformations
Table 31.7  Variations of venous malformations (VM)
Common VM
BRBS
GVM
CMVM
Maffucci
Diffuse VM
Name, eponym
Venous malformation
Blue rubber bleb
syndrome; bean
Glomuvenous
malformation;
glomangioma
Capillary malformation,
venous malformation
Maffucci syndrome
Genuine phlebectasis of
Bockenheimer; diffuse VM
Capillary stain
Absent
Absent
Absent
Present; involves
mucous membranes;
purple older
Absent
Absent
Skin
appearance
Bluish discoloration,
present at birth; wide
distribution
Dark blue spots
present at birth
Thickened and purple
early; present at birth;
cobblestone
appearance
Skin, mucous
membranes
Bluish to purple;
thickened; nests and
nodules
Diffuse; large, tortuous
venous channels
Genetics
50% mutation in
endothelial TIE2
Unknown
Mutation glomulin gene;
chromosome 1
p21–22
Mutation TIE2,
chromosome 9p21;
possible 2nd hit
mutation
Unknown
Unknown
Pattern
Sporadic; common;
sexes equal
AD; sexes equal
AD; sexes equal;
AD; 1% all VMs; sexes
equal
Sporadic; sexes equal;
rare
Sporadic; sexes equal
Hemodynamics
Slow-flow
Slow-flow
Slow-flow
Slow-flow
Slow-flow
Slow-flow
Limb affected
All parts of body
All parts of body
All parts of body
Skin, mucosa, muscle
All limbs
All limbs
Skeletal
involvement
Present, unusual
None
None
None
90% hands and feet;
enchondromas
Present, diffuse, pathologic
fractures
Visceral
involvement
Yes
Yes: particularly
bowel
anemia common
None
None
None
None
Pathology
Deficient smooth muscle
in vessel walls; dilated
vessels; thrombus
formation
Similar to VM, well
delineated,
thin-walled dilated
vessels
Cuboidal glomus cells
Similar to common VM
Similar to VM; thick
spindle cells
Similar to common VM
AD, Autosomal-dominant inheritance.

704
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
involve the dorsal interosseous system along the entire length
of the interosseous membrane and may extend into the volar
compartments. Surgical decompression of the cubital tunnel,
radial tunnel as well as the volar side of the median nerve
usually relieves neurogenic pain, which may also be second­
ary to bleeding, thrombophlebitis, or phleboliths within the
VM. Neural wrapping with acellular dermal matrix, autoge­
nous vein, other alloplastic materials or local uninvolved
tissue is a helpful adjunct to any neuroplasty in this region.
The distal forearm, wrist, palm, thumb, and digits are more
amenable to surgical extirpation because less muscle mass
and more solid tendinous and neural structures occupy the
region. Frequently, what appears to be a superficial lesion in
the distal forearm and wrist extend deep to and through the
interosseous membrane and involve the rete system. Resec­
tions in this region must be thorough, and usually includes
removal of the interosseous membrane. Denervation of both
The proximal forearm presents one of the most difficult
operative fields for the extremity surgeon due to the high
density of important anatomic structures in a compact space.
Subcutaneous VMs are not problematic in this area. Intramus­
cular lesions are difficult to resect because the potential for
damage to adjacent muscle and injury to the many small
motor branches of the median nerve, and particularly the
posterior interosseous nerve on the dorsal forearm. Localized
lesions deep within the flexor pronator region are difficult to
approach without damage to the wrist flexors and deep digital
and thumb flexors. Sclerotherapy is the first line of treatment
(see Fig. 31.11
). On the dorsal surface, it is much easier to
identify and decompress the radial nerve through the Arcade
of Frohse; however, the risk for injury to extensively involved
extensor muscle bellies is high. VMs in this region are usually
diffuse and extend well beyond the fascial planes into the
muscles. Commonly, VMs and other vascular anomalies
Fig. 31.13  Venous malformation (VM) palm. (A) This 6-year-old child with a diffuse VM of the palm could not wear garments due to painful intralesional thrombi. (B) The
coronal T2-weighted images show multiple signal voids within a diffuse, through-and-through VM. The mid-palm was most symptomatic. (C) The dissection begins with
identification of the palmar arch (red loops) and median and ulnar nerves (yellow loops). VM contains multiple thrombi admixed with dysplastic fat. (D) A complete
proximal-to-distal resection was completed with preservation of all neurovascular structures, flexor tendons, and lumbrical muscles, including their motor branches. (E) Five
years later, normal sensation and motion has been maintained. VM remains but is not symptomatic.
A
B
C
D
E

704.e1
Vascular malformations
A
B
C
Fig. 31.11  Sclerotherapy. Radiographic studies of the vulnerable proximal third of the forearm show a venous malformation (VM), which is ideal for sclerotherapy:
(A) Isointense to adjacent skeletal muscle on axial T1 sequence; (B) enhances on axial T1 plus gadolinium contrast; (C) shows signal voids on axial T2 representing
phleboliths; (D) fills with direct puncture and injection of contrast; (E) shows small calcified phleboliths on radiographs; (F) has multilobulated structure with signal voids
on sagittal T2 sequences.
D
E
F

704.e2
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
Fig. 31.12  Venous malformation (VM) distal forearm. (A,B) A middle-aged man presented with carpal tunnel symptoms. Coronal T1 (above) shows a lesion with signal
voids and T2 sequences (below) demonstrate enhancement, both consistent with VM. (C,D) Enhanced T1-weighted sequences show extension across the interosseous
membrane.
A
B
C
D

704.e3
Vascular malformations
E
F
(E) Excision included the pronator quadratus muscle. (F) Full function returned 6 weeks postoperatively.
Fig. 31.12, cont’d

705
Vascular malformations
dissection beneath the palmar arch will often expose the deep
motor branch of the ulnar nerve (see Fig. 31.13).
What appears to be localized VM in the palm and dorsum
of the hand is always much more extensive than it appears
clinically. The margins of many VMs may not be evident on
MRI or MRV scans. Dissection in this region is difficult and
general guidelines must be strictly observed (see Table 31.5).
Sclerotherapy in this portion of the hand has many potential
complications due to the high concentration of neurovascular
structures, gliding tendons, and delicate intrinsic muscles
(Fig. 31.14).
and anterior and posterior interosseous nerves will alleviate
postoperative wrist pain (see Fig. 31.12
). The median and
ulnar nerves are frequently involved in this region, but intra­
neural dissection is indicated only when there are symptoms.
Secondary neuromas within the main nerves or their sensory
branches at the wrist level can be severely disabling for both
children and adult patients. A deep palmar VM commonly
enlarges within the first webspace and extends along the
adductor pollicis muscle beneath the first dorsal interosseous
muscle. Both muscles are important in pinch and should
be preserved if they are not extensively involved. Deep
Fig. 31.14  Complications of interventional radiology. Intrinsic compartment releases were necessary following
sclerosis of this diffuse venous malformation of the entire palm of the hand. Ethanol was used to embolize this
arteriovenous malformation with a nidus within the palm. The distal portions of the thumb, index, and long digits
were lost.
A
B
C
D
E

706
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
CM may be the only initial sign of an AVM, which may not
become evident until later in childhood. Vascular thrills and
bruits are not always marked. In some patients, a pulsating
mass and accompanying thrill may be psychologically dis­
turbing. Many patients seem to tolerate symptoms for a life­
time (see Fig. 31.15). AVM may be present at the dermal level.
Cutaneous mottling and atrophy, progressing to ulceration,
are secondary to the “steal phenomenon”, i.e., blood is shunted
to the proximal portion of the limb, resulting in peripheral
vascular insufficiency, usually first evident in the fingers, toes,
hands, and feet (Fig. 31.16). Chronic infection, ulceration,
recurrent bleeding, and intractable pain are the end-stage
problems in these unfortunate patients. Paradoxical increased
blood flow but decreased tissue perfusion hampers wound
healing. These malformations are regional and are rarely
found with associated fast-flow anomalies elsewhere in the
body. Patients with an AVM involving the entire extremity
and ipsilateral chest wall or those involving the entire lower
extremity and pelvic region often demonstrate a positive
Nicoladoni sign, a decrease in pulse rate with application of
a pneumatic tourniquet to the affected extremity. A large AVM
in an extremity can cause cardiopulmonary overload (Fig.
31.17 & see Fig. 31.21). Some children develop extraordinary
postures to alleviate their pain (Fig. 31.18
). AVMs do not
cause disseminated intravascular coagulopathy. Intralesional
bleeding can cause compartment compression. Bony hyper­
trophy and elongation are present in more than half of these
affected limbs. Skeletal involvement carries an unfavorable
prognosis but is not an absolute contraindication to surgical
intervention. Fig. 31.18 appears online only.
Diagnosis/patient presentation
AVM can mimic other extremity malformations and there are
many vascular conditions containing fast-flow malformations
(see Table 31.8
). Most lesions are diagnosed by history and
physical examination, and fast-flow characteristics are con­
firmed by Doppler probe in the clinic. MRI may be obtained
to: (1) confirm the diagnosis; (2) outline the true extent of the
lesion; and (3) plan management. The T2-weighted sequences
show tortuous, dilated, arteries and large draining veins,
enhancement, and flow voids. The “nidus” is identified as a
blush within the central portion of the lesion (Fig. 31.17A &
see Fig. 31.19B). Magnetic resonance angiography (MRA) and
CTA scans demonstrate the 3D characteristics and are pre­
ferred over angiogram by most interventional radiologists.33
Extremity surgeons prefer angiography to show the caliber
and flow dynamics of the shunts prior to any surgery.2,15
Anatomic abnormalities are common in the upper limb and
include: giant feeding vessels; persistent interosseous vessels;
corkscrew configuration of arteries; large median artery;
duplicated brachial systems; and large draining veins. Palmar
arches are often obliterated with active shunting, and muscle
groups may be completely replaced by active AVFs. CT scans
are indicated for skeletal involvement. Although critical in the
evaluation of other malformations, histopathological diagno­
sis of AVM is rarely necessary but can be important to rule
out rare malignancies such as infantile fibrosarcoma.
Treatment/surgical technique
The puzzling question with fast-flow lesions is their unpre­
dictable natural history and rate of progression. Simple
Outcomes, prognosis, and complications
Few outcome studies are available due to the extremely vari­
able presentation of VMs within the upper extremity and the
many syndromic designations and confusion in terminology.
VMs can be resected with predictable results. Early complica­
tions often beget later problems and less than optimal out­
comes. Problems occur in up to 20% of patients and are seen
early (swelling, hematoma, delayed wound healing, skin
necrosis) or late (neuroma, scar contracture, joint stiffness).
Wrist and forearm flexion contractures following partial
intermuscular resections are common for proximal forearm
lesions. Symptomatic neuromas develop in 35% of those who
have intraneural dissections.
Arteriovenous malformation
Arteriovenous malformation (AVM) results from an error in
vascular development during embryogenesis in which an
absent capillary bed causes shunting of blood directly from
an artery into a venous system. This shunt occurs through a
direct arteriovenous connection (fistula) or abnormal feeding
vessels between an artery and a vein (nidus). This observation
of Halsted in 1919 may explain why these AV connections are
common in the central nervous system, where apoptosis is
rare.73,74 A number of genetic abnormalities have been
described (Table 31.8
). These lesions are present at birth
only in one-third of patients; 15% involve the extremities.
Almost 80% become clinically evident before the termination
of adolescence and the natural history is one of slow progres­
sive growth and expansion! These malformations worsen
with time and are staged by the Schobinger system (Table
31.9). It is not unusual for a lesion to progress from quiescent
through destructive phases. The timing of progression is not
predictable when first seen. Table 31.8 appears online only.
Not all fast-flow lesions in the upper limb should be labeled
as PWS. We have separated these lesions into four categories
in which the arteriovenous flow is related to: (1) a direct AVM
(Fig. 31.15); (2) flow through a nidus (see Figs. 31.14B & 31.19);
(3) flow through a vascular tumor (see Fig. 31.17) and finally;
(4) diffuse high-flow vessels perfusing all tissue planes (see
Figs. 31.18
& 31.21
). PWS is rare and should be restricted
to the last condition.
Basic science/disease process
The cardinal features of AVM in a limb are warmth, pain,
paresthesias, hyperhidrosis, and compression neuropathy. A
Table 31.9  Schobinger clinical staging system
Stage I
Quiescent
AVM mimics a capillary
malformation or involuting
hemangioma
Stage II
Expansion
Lesion larger, warmer, throbbing
with thrill or bruits
Stage III
Destruction
Stage II plus ulcers, hemorrhage,
persistent pain, tissue necrosis
and bone destruction
Stage IV
Decompensation
Stage III plus increased cardiac
output and cardiac failure

Table 31.8  Combined and fast-flow malformations
Parkes Weber
(PWS)
Ostler–Rendu–
Weber
CM–AVM
PTEN–AVM
CLM
Klippel–
Trénaunay
Eponym/ name
Parkes Weber; AVM
with AVF
Hereditary
hemorrhagic
telangiectasia
(HHT)
CM–AVM
PTEN-
hamartoma
syndrome
(PTHS)
Cowden, BRRS
CLM;
CM–LM
confused
with
CLOVES
KT; CLVM
Vascular
malformation
Diffuse AVM with AVFs
lymphedema
CM, visceral
AVMs in liver
and lungs
CM –small, pink
Multiple AVMs,
VM
CM, LM
CM; VM (varicose
veins); LM
Skin
appearance
Pseudo CM on skin
lymphedema
Telangiectasia
lips, fingers
mucosal-nasal
epistaxis
Pinkish lesion
with halo
CM; possible VM
bluish
discoloration
CM
Capillary stain,
bluish pink,
vesicles,
maceration
Hemodynamics
Fast-flow
Fast-flow AVFs
Fast-flow in 30%
Fast-flow AVM in
50%
Slow-flow
Slow-flow
Cardiac
compromise
Common
Rare, anemia
Possible
None
None
Possible
Arteriovenous
fistuli
Multiple, often
aneurismal
None
Multifocal
None
None
Lateral/deep
venous
anomalies
None
None
None
Clusters
abnormal veins
Tortuous
draining
veins
Common; vein of
Servelle lateral
arm as in leg;
varicose veins
Coagulopathy
None
None
None
Unusual
None
Present
Pattern
Lower → upper
extremities
Lips, intraoral
Legs > arms/hand
Extremities
equal
Lower > upper
extremities
Genetics
Unknown
HHT1 mutation
of endoglin
HHT2 mutation
ALK1
Mutation RASA1;
positive family
history
PTEN mutation
None
Unknown
Associated
anomalies
Rare pelvic AVM;
Cobb syndrome
AVM cerebral,
spinal, liver
and pulmonary
Cerebral AVM
7%
Macrocephaly;
penile freckling
(males);
lipomas
None
Common: GI,
GU, genital
pelvic organs
Limb
hypertrophy
Major, disproportionate
osteolysis can occur
None
Absent
Absent
Uncommon;
can occur
Minor to
moderate
Symptoms
Warmth, pain,
hypertrophy,
sweating
Nasal epistaxis
GI bleeding
anemia
Warmth, pain,
mass
Pain, mass
effect; function
Cellulitis,
size, bulk
Ulcers, cellulitis,
weight and
bulk
Venous stasis,
skin ulcers
Ischemic changes;
pseudo Kaposi
changes
Telangiectasia ±
bleeding
None
Absent
Vesicular
maceration
Common
Clinical
prognosis
Poor; progressive
expansion
Good
Progressive with
AVM
Progressive
Good
Stable; severe
overgrowth
common
AVF, Arteriovenous fistula; AVM, arteriovenous malformation; BRRS, Bannayan–Riley–Ruvalcaba syndrome; CLOVES, congenital lipomatous overgrowth with vascular
malformations, epidermal nevi, and skeletal anomalies; CLVM, capillary–lymphatico-venous malformation; CM, capillary malformation; GI, gastrointestinal; GU,
genitourinary; LM, lymphatic malformation.

706.e2
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
Fig. 31.18  Parkes Weber syndrome (PWS). (A) A 6-year-old child with a capillary malformations (CM) of the wrist and hand, and a pulsatile mass of her distal forearm.
Diagnosis of PWS was made and she was treated with a compression garment. Flexion contractures gradually developed in the wrist and all digits. (B) Angiogram at age 7
showed diffuse hypervascularity and multiple microfistuli without large draining veins. (C) Pain gradually increased as she grew. She learned to control this pain by
dislocating her glenohumeral joint, a maneuver that compressed the massively enlarged subclavian artery and vein. She often slept in this position.
A
B
C

Fig. 31.15  Natural history of fast-flow
arteriovenous malformation (AVM). (A) A
67-year-old adult executive complained of
gradual swelling and pulsation in the hand
since teenage years. Attempted surgical
removal of symptomatic regions had been
aborted due to excessive bleeding.
(B) Early angiographic sequences showed
large, tortuous macro-fistulous shunts
involving radial and ulnar arterial systems
and both palmar arches; very little flow to
the distal digits.
A
B
Fig. 31.16  Fast-flow arteriovenous malformation (AVM) with steal phenomenon. (A) Angiogram of a 21-year-old patient who presented with an asymptomatic bruit of the
wrist shows an AVM involving the radial and interosseous systems. (B) Inverted arteriographic images taken 20 years later show large tortuous radial and interosseous
vessels leading to large aneurysms with shunting in the distal metacarpal region. (C) Tip discoloration of the thumb and long digit caused by steal phenomenon. Distal
ischemia of the thumb will ultimately progress to tissue necrosis.
A
B
C

708
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
Fig. 31.17  CLOVES with arteriovenous malformation (AVM). (A) A 6-month-old baby was referred with large AVM of right arm and forearm, failure to thrive, and refractory
cardiac failure. Arm amputation was recommended by many consultants but refused by the family. (B) Angiography showed nidus located at level of elbow and forearm.
(C) Massively enlarged draining veins were encountered. Dissection proceeded toward the center of the mass. (D) Individual branches of axillary and brachial artery were
mapped and surgically ligated. The dysplastic radial artery (red loop) was replaced with a graft; median, ulnar and radial nerves (yellow loops) were preserved despite
massive stretching. (E) Incision healed well and patient maintained full elbow flexion/extension and sensation. There were also truncal capillary malformations, overgrowth
of multiple limbs, and epidermal nevi. He was later diagnosed as CLOVES.
A
B
C
D
E
AVMs involving multiple tissue planes, but all lesions will
re-expand to some degree, especially if the central nidus has
not been obliterated.
It is important that the extremity surgeon understand the
rationale of embolization, which is often used as the primary
treatment for these lesions. Inert substances are injected
into the nidus to obliterate the abnormal vascular channels,
promote ischemia and ultimate scarring of the malformation.
Substances used for this process may be liquid (ethanol, n-
butyl cyanoacrylate (n-BCA), onyx or solid (polyvinyl alcohol
particles, PVA), or coils of many varieties. It is critical that
these materials are delivered directly to the nidus of the AVM.
Obstruction of the inflow vessels is the major problem with
selective embolization and will result in: (1) collateralization
of vessels; (2) expansion of the malformation; and (3) aggrava­
tion of symptoms.75 More recently, radiologists at our center
observation is the initial treatment in all but the most symp­
tomatic AVMs. Compression garments may be of some help
(see Fig. 31.18C
). The goal of treatment is to control the
progression of the malformation and to alleviate symptoms:
pain, ulceration, bleeding, distal discoloration, or functional
problems. Management options include embolization, surgi­
cal resection or a combination (Fig. 31.19). Surgical resection
offers the best chance for long-term control, but re-expansion
rate following extirpation is high and the deformity created
by the resection may be significant. Radical extirpation may
often create a deformity worse than the malformation itself.
Amputation is the definitive cure, which may be achieved
with functional limitations (see Fig. 31.21). Therefore,
embolization is the first line of intervention for type A and B
lesions in the Schobinger I (quiescence) or II (expansion)
stages. Well-localized lesions respond better than diffuse

709
Vascular malformations
A
B
C
Fig. 31.19  Arteriovenous malformation (AVM) of hand. (A) Professional wrestler presented with painful mass in thenar eminence. Thrill first noted during adolescence. (B)
Angiography demonstrated AVM nidus at superficial palmar arch, supplied by radial, interosseous and ulnar vessels. Distal digital and thumb arterial vaso-architecture was
normal. Embolization performed with N-butylcyanoacrylate (NBCA). (C) Post-embolization study showed obliteration of most of AVM with residual large draining veins. (D)
Two days after embolization, the mass was explored: yellow vessel loops mark recurrent motor branch and multiple sensory branches of the median nerve. (E) All nerves,
flexor tendons, and uninvolved thenar muscles were preserved during resection. (F) Five years later, he continues to demonstrate good function. Palmar abduction is present
(abductor pollicis muscle), but key pinch is weak due to subtotal loss of the adductor pollicis muscle.
D
E
F
have focused on obliteration of the outflow in high-flow
lesions that have only a few large outflow venous channels.
This approach has shown promising results but longer-term
studies are needed to corroborate initial findings.
For
temporary
preoperative
embolization,
Gelfoam
powder, PVA, and Embosphere are used instead of liquid
agents. Over the past decade, our interventional radiologists
have preferred onyx, an ethylene-vinyl alcohol copolymer.
At the time of resection, the embolized tissue with its atten­
dant inflammation will have a mass effect, which facilitates
resection. Excision should be completed within 36–72 h post-
embolization, before recanalization restores blood flow to the
region of the lesion. The major complications to embolization
are skin ulceration, local compartment syndrome, and rarely,
distal ischemia (see Fig. 31.14B). Extremity surgeons must
be available following these procedures in interventional
radiology.
Resection is indicated whenever the AVM leads to chronic
ulceration or bleeding, compartment compression, nerve
compression, uncontrolled congestive heart failure (CHF),

710
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
Treatment/surgical technique
Although sclerotherapy has been used to control large tortu­
ous veins and isolated clusters, surgical resection is the defini­
tive treatment of choice. Extirpation may be more difficult if
prior sclerotherapy has been performed. Resections in the
arm, forearm, and hand often involve microvascular dissec­
tions of involved neurovascular structures.
Parkes Weber syndrome (PWS)
PWS is an eponym denoting a fast-flow malformation in
association with soft tissue and/or skeletal overgrowth and
overlying CM.32,33 The term is incorrectly used in its associa­
tion with KTS and should not be used to describe all fast-flow
lesions within the extremities.
Basic science/disease process
No genetic foci or mutations have been identified to date.
Diagnosis/patient presentation
The diagnosis is made by physical examination and ultra­
sound. The lower extremity is involved much more frequently
than the upper. Diffuse or localized CMs are often present;
other skin changes include pseudo Kaposi lesions, lymph­
edema, and skin ulceration late in the progression of the
malformation. Lymphedema is common. The angiograms in
a classic PWS do not initially show specific AVFs with large
draining veins. These early studies show diffuse high-flow
vessels infiltrating the involved soft-tissue and skeletal struc­
tures without large draining veins so characteristic of shunts.
The CM is often diffuse and irregular and covers the region
of underlying hypervascularity (Fig. 31.21
& see Fig. 31.18
). PWS should be distinguished from other fast-flow lesions:
PTEN-AVA, other vascular tumors, AVM with AVFs, and
CM–AVM. MRI, MRA, ultrasound, and biopsy are warranted
with an increase in symptoms. The clinical prognosis is one
of progressive expansion and destructions. Fig. 31.21 appears
online only.
Treatment/surgical technique
Management is identical with the indications and principles
previously presented for fast-flow lesions. Initial treatment is
always conservative, but these children should be followed
through adolescence and adulthood. Symptomatic regions
respond to selective embolization. Cardiac compromise may
be insidious; cardiologists must evaluate these children yearly.
Regional resections are warranted for selective cases but must
be carefully planned and executed with the realization that
these lesions are often progressive. Amputation of non-
functional digits, hands, and arms is usually delayed; pain is
often the precipitating factor for an amputation. Experience
has taught us that amputations of parasitic parts is often
unnecessarily delayed either by the reluctant surgeons or
anxious parents with unrealistic expectations.
Outcomes, prognosis, and complications
Children must be monitored both for cardiac symptoms and
overgrowth issues. The progress may be slow and not predict­
able early in life. All patients have some degree of pain,
gangrene, or unrelenting pain. The procedure must be tailored
to the patient’s needs. In all but the most localized lesions,
resection for AVM in a limb is palliative. The surgeon who
undertakes resection of an AVM in a limb must have specific
objectives that can be accomplished during a safe tourniquet
time. The general principles of treatment outlined earlier for
slow-flow anomalies also apply to fast-flow anomalies (see
Table 31.4). Problems arising during these procedures can get
out of control; these are well known to all surgeons who have
written about operating on these anomalies. Dramatic out­
comes can be achieved with a well-planned and executed
procedure (Fig. 31.20
). There is no predictable procedure
that when performed in childhood will prevent subsequent
expansion of an AVM, short of a limb amputation. In few other
areas of hand surgery is careful explanation of potential com­
plications so important. Amputation of a very symptomatic,
almost parasitic part is encouraged. Severe pain and bleeding
are often the precipitating symptoms (Fig. 31.21
). Figs. 31.20
and 31.21 appear online only.
The same anatomic concerns outlined with the slow-flow
malformations apply to this group of very dynamic lesions.
Nerves and tendons should be preserved. Heavily involved
muscle groups should be resected. Revascularization of the
hand, digit or thumb should be performed if both axial vessels
have been excised. Similarly, marginal skin requires resection
and appropriate resurfacing.
PTEN-associated vascular anomaly
(PTEN-AVA)
Basic science/disease process
The PTEN (phosphatase and tensin homologue) gene
encodes a tumor suppressor lipid phosphatase.29 Children
with these PTEN mutations have the PTEN hamartoma-
tumor syndrome (PHTS). This is an autosomal-dominant
condition, which in the literature has previously been called
the Cowden syndrome or the Bannayan–Riley–Ruvalcaba
syndrome (BRRS).30,76 The PTEN mutation is associated
with multiple benign and malignant tumors that require
surveillance.
Males and females are equally affected. The lower extremi­
ties, especially the thighs and calf musculature, are involved
much more frequently than the upper extremities, where the
arm and forearm regions are more involved than the wrist
and hands. Half (54%) of these patients have fast-flow mal­
formations with arteriovenous shunting and are designated
PTEN-AVA. These children have distinctive macrocephaly;
males have penile freckling and these masses can be large and
multiloculated. Large amounts of adipose tissue surround the
shunts and the mass effect of the affected limb may result
in functional compromise. The lesions directly involve the
muscles, surround neurovascular tissues, and extend within
intramuscular fascial planes. Bone is usually not involved.
Lesions may grow to massive proportions.
Diagnosis/patient presentation
The diagnosis is made by physical examination and imaging
(Fig. 31.22
), and confirmed by histological and genetic
analysis. Histology shows tortuous arteries with mural hyper­
plasia. Fig. 31.22 appears online only.

A
B
C
D
E
Fig. 31.20  Arteriovenous malformation (AVM) with arteriovenous fistuli of thumb. (A) A 60-year-old man
presented with pulsatile bleeding from excoriated regions of his right thumb. He first noted swelling as a young
child, and lived with periodic bleeding and ulceration his entire life. (B) Arteriography showed extensive
macro-fistulous AVM involving radial side of his dominant hand. (C) Congenital absence of his left hand made
him completely dependent on right upper limb. (D) Following resection of scarred and unstable pulp surface and
debulking entire thumb, resurfacing accomplished with neurovascular island flap from ulnar side of long finger.
(E) Flap extended to nail plate distally and included entire working pulp surface of thumb. (F) This grateful
patient has a stable functional thumb, which he perceives is on ulnar side of the long finger.
F

710.e2
SECTION IV
CHAPTER 31 • Vascular anomalies of the upper extremity
A
B
C
D
E
F
Fig. 31.21  Parkes Weber syndrome. (A,B) Teenager with type C arteriovenous malformation, and his parents wanted to save the hand. Index ray had been resected for
uncontrollable pulsatile bleeding. The thumb was ulcerated; patient was suicidal because of unrelenting pain. (C) Angiography showed massively tortuous and enlarged
axial vessels feeding microfistulous shunts within forearm and hand with involvement of all soft-tissue and skeletal structures. (D) Later sequence demonstrates extent of
shunting and lack of distal digital perfusion. (E) Chest radiograph and electrocardiogram confirmed congestive heart failure (Schobinger Stage IV). (F) Below-elbow
amputation followed by predictable recovery, normal adolescent growth spurt, and good function with his prosthesis.

710.e3
Vascular malformations
A
B
Fig. 31.22  High-flow PTEN lesion of the forearm. (A) This teenager has a large lesion of the forearm but has very little to no functional deficit. (B) Sagittal T2-weighted
MRI images show the extensive nature of the lesion that involves the entire volar forearm down to the interosseous membrane. (C) PTEN lesions densely surround and
involve large nerves. In this case, the median nerve is completely involved (white arrow), although the patient does not have any nerve symptoms at this point.
C

711
Vascular malformations
be present including skin vesicles, coalesced vesicles causing
maceration and ulcers, macrocysts in the proximal portion of
the upper limb, and microcysts in the more distal regions. All
lesions are accompanied by large amounts of adipose tissue.
Occasionally VM predominate; the upper limb equivalent of
the lateral “vein of Servelle” is present and does communicate
with the deep venous drainage system along the axial vessels
to the limb. Because the genitourinary and gastrointestinal
systems are commonly involved, these patients may be very
symptomatic. Similar to the foot, massive enlargement of the
wrist and hand with splaying of the digits with or without
ulnar deviation may occur.
Treatment/surgical technique
Management is initially conservative and depends upon the
predominance of the venous or lymphatic components. Man­
agement of the venous components is initially conservative.
Epiphysiodesis to control bilateral growth symmetry is not as
essential in the arm as it is in the lower extremity. Nerve
compressions and lymphatic components respond very well
to surgery. Surgical principles are the same. In the upper limb,
resection is performed to improve function, relieve compres­
sion neuropathies, and restore motion and contour. Those
with large lesions, chest wall involvement and those with
large stagnant venous collecting systems in the arm and axilla
are at risk for pulmonary embolus and are maintained on
anticoagulants. Staged contour resections are difficult but
well worth the effort.
Outcomes, prognosis, and complications
Clinical outcomes vary with the size and extent of the LVM
and are similar to those for large isolated VMs and LMs.
Outcome studies for upper limb KTS patients do not exist.
CLOVES syndrome
Congenital lipomatosis overgrowth, vascular malformations,
epidermal nevi, and scoliosis syndrome (CLOVES) constitutes
the components of a recently described overgrowth condi­
tion.12,25,26,28 This disease entity has been linked to somatic
activating mutations in the PIK3CA gene.11,12,25
Basic science/disease process
The upper extremity is usually involved. Previously, many of
these children were diagnosed as Proteus patients but did not
satisfy the criteria for inclusion. CLOVES patients do not have
uncontrolled progressive skeletal overgrowth, and the soft-
tissue enlargement consists primarily of dysplastic adipose
tissue. Truncal CMs are present in all patients. Patients may
also have fast-flow AVMs (see Fig. 31.17). Overgrowth in the
limb is similar to that seen with LMs. It may or may not be
oriented along a nerve territory (see Table 31.2
).
Typical upper extremity findings include polydactyly,
syndactyly, macrodactyly, and stiffness. Fatty overgrowth
may extend proximally to the hand and forearm. Many
patients have a large, spade-like hand (Fig. 31.23
), an
appearance that is accentuated when there is fatty overgrowth
in the palm. In about half of these patients, the thumb is
positioned in a hyperextended and hyper-abducted posture
(see Fig. 31.22
). Fig. 31.23 appears online only.
swelling, overgrowth, and nerve compression at some ana­
tomic level. Selective embolization successfully controls
symptoms in 70% of patients. We have never documented
regression of this type of malformation.
Capillary malformation–arteriovenous
malformation (CM–AVM)
Basic science/disease process
This is an autosomal-dominant condition caused by a loss-of-
function mutation in the RASA1 gene.31,33 Patients usually
present with a positive family history of similar lesions.
Diagnosis/patient presentation
The CM is evident by physical examination and is distin­
guished by a faint halo around the lesion. We have seen these
in the upper extremity. Almost 10% of patients have an under­
lying fast-flow AVM involving the central nervous system, a
very frequent location for AVMs. A full neurologic examina­
tion is indicated with a cranial MRI. Extracranial AVMs are
rare in patients. Lesions are common in the lips or mouth.
Treatment/surgical technique
No treatment is necessary for the upper extremity CM. AVMs
are followed symptomatically and treated as previously
outlined.
Klippel–Trénaunay syndrome (KTS)
KTS is an eponym denoting a slow-flow capillary–lymphatico-
venous malformation designated as CLVM and is readily
distinguished from PWS by the slow-flow dynamics of the
malformation.
Basic science/disease process
No positive genetic associations have been made.
Diagnosis/patient presentation
An accurate physical examination will determine the pres­
ence of both lymphatic and venous components, as the
CM is obvious at birth. There is a wide range in clinical
presentation.77–79 There are no major hemodynamic alterations
in the cardiovascular system. The lower extremities (95%) are
involved much more frequently than the upper (5%) and the
trunk and pelvis are rarely affected. The limb hypertrophy
is not as profound as in patients with the Proteus syndrome,
CLOVES syndrome, or in hemihypertrophy patients.
However, upper limb involvement may be accompanied by
massive soft-tissue enlargement of not only the upper limb
but also the chest wall. In 10% of these limbs, hypoplasia
may be present. Kyphoscoliosis and pulmonary compromise
often develop as the child grows. Leg-length discrepancy
is common. KTS patients are not at risk for Wilms tumors
and do not need to be screened with abdominal ultrasound
examination.80 The VM pattern is similar to phlebectasias with
large draining veins in the arm and axilla. There are usually
diffuse clusters of large veins and sinuses involving muscle,
fascial planes, and occasionally bone. All forms of LM may