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Congenital Vascular Malformation 385
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The VM in particular has a significant incidence of secondary abnormal longbone growth with subsequent bone length discrepancy. In addition, it is also known
to have a relatively high incidence of combined LM, which is still called
Klippel–Trenaunay syndrome [15, 16].
Of the many clinical clues this patient presented with that suggested VM among
various CVMs, immediate collapse of the bulging soft lesion along the foot upon
elevation was the most important.
Therefore, hemodynamic assessment of the lower extremity along the scattered
soft tissue tumors has to be the starting point for the work-up of proper diagnosis
and treatment of this disease complex; duplex ultrasonographic study provides
most of the essential hemodynamic information and an excellent guideline for
further management (Fig. 41.2). [Q2: C]
a
b
Fig. 41.2. a
system. bSonographic assessment of the VM lesion located superficially in the lower extremity.
Sonographic identification of the communicating/draining vein between VM lesion and deep vein
386 Vascular Surgery
Further study to assess scoliosis with pelvic tilt and/or abnormal long-bone
growth with length discrepancy may be carried out once primary diagnosis of the
vascular malformation has been made. In this case report, the patient presented
a
b
Fig. 41.3. a
form of lesion along the deep vein system as femoral-popliteal venectasia. bET form of the VM lesion, infiltrating into foot muscle structure as well as sole soft tissue.
ET form of the VM in diffuse infiltrating status mostly confined within subcutaneous soft tissue, and T
Congenital Vascular Malformation 387
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recurrent episodes of tender swelling of the left knee following minor trauma. This
was probably due to the bleeding/leaking from the VM lesion near to the knee joint
to the surrounding soft tissue. A detailed evaluation of the knee joint itself can be
deferred until the basic evaluation of VM, presented as soft tissue swelling along the
knee joint, is completed with MRI, WBBPS, and duplex ultrasonography [17, 18].
This approach will delineate the accurate relationship of this VM lesion to the periarticular structure including the joint space, and the potential risk of inducing
hemarthrosis by repeated bleeding following trauma.
Radiological assessment of lumbosacral spine together with long-bone length discrepancy should be made after hemodynamic assessment to identify the extent of
VM, starting with duplex scan as the most basic laboratory test [19].
Although duplex ultrasonographic study can provide most of the crucial first-line
hemodynamic information about vascular malformation, MRI of T1 and T2 images
is the most valuable non-invasive study for clinical diagnosis, and has become the
new gold standard for the diagnosis, especially for the VM [17] (Fig. 41.3). [Q3: C]
MRI study of the soft tissue along the entire left lower extremity extending from
toe to the torso can confirm the clinical diagnosis of VM already made preliminarily
by ultrasonographic study. MRI can provide precise delineation of the anatomical
relationship of the malformation lesion with its surrounding tissues like muscle,
tendon, nerve, vessels, and bone from the foot to the retroperitoneal, pelvic, and
gluteal regions. In addition to the duplex scan and MRI study in this patient,
various non-invasive tests are needed for further differential diagnosis.
Lymphoscintigraphic study based on radioisotope-tagged sulfur colloid is indicated to assess lymphatic function and the lymph-conducting system in general and
rule out chronic lymphedema due to the T form of LM [20, 21].
The extremity involved was felt to be firmer than usual for a VM-affected leg,
with general diffuse swelling throughout the entire length of the lower limb; this
finding suggested primary lymphedema combined with venous stasis so that further
evaluation of the lymphatic function is indicated with radionuclide lymphoscintigraphy. The lymphatic function assessment of this patient with lymphoscintigraphy
has shown the marginal status of the lymphatic system and its vulnerability to
further insult by the ET form of LM.
WBBPS based on radioisotope-tagged red blood cell pooling is also indicated as
one of three basic tests for the diagnosis of VM. This relatively new investigation is
very sensitive in detecting abnormal blood pooling throughout the body (Fig. 41.4).
It can be used not only as a practical test to assess treatment results but also as a
screening test for hidden vascular malformation. It also has a unique role in the differentiation between venous and lymphatic malformation [22, 23].
CT scanning also has practical value in providing information on the relationship
of vascular malformation to its surrounding skeletal and soft tissue of the lower
extremity.
Transarterial lung perfusion scintigraphy (TLPS) can provide crucial information
on possible involvement of a micro-, if not, macro-AVM lesion to the VM
(Fig. 41.5).
AVM involvement is a critical condition for the management strategy of VM; the
VLM in particular is seldom combined with the AVM, especially in micro-AVM,
which can be overlooked by conventional arteriography alone. Positive
confirmation of no existence of micro-AVM is extremely important before the initiation of the treatment to the symptomatic VM lesions, especially when it is combined with LM.
388 Vascular Surgery
Fig. 41.4.
lower extremity.
Fig. 41.5.
the AVM lesion involved to the VM lesion. Normal TLPS finding with no evidence of micro-AV shunting can rule
out AVM without further investigation by arteriography.
Extensive abnormal blood pooling by the ET lesions and T lesion of the VM, diffusely involving entire
TLPS investigation of arteriovenous (AV) shunting status in lower extremity to assess potential risk of
The TLPS can therefore provide necessary guidance for the further invasive study
of arteriography [24, 25].
However, classical lymphangiography (or lymphography) using oil-based contrast material is no longer performed for the screening lymphatic function because
of the potential risk of further damaging the lymphatic vessel with the procedure.
[Q4: E]
Once the final diagnosis of a combination of VM and LM has been made, then the
next decision should be whether treatment is indicated. In view of the abnormal
long-bone growth involvement to this vascular malformation, immediate treatment
of this particular VM is generally preferred.
Congenital Vascular Malformation 389
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Treatment priority should be given to the primary etiology, i.e. vascular malformation. Therefore, the control of abnormal hemodynamic status of the lower
extremity secondary to the VM should have priority [26, 27]. [Q5: C]
All the other clinical problems secondary to this primary lesion, including scoliosis with pelvic tilt, abnormal long-bone growth with bone length discrepancy, and
abnormal gait, can be deferred while treatment is aimed at the VM itself [5, 6, 26].
Not all the VM lesions are indicated or feasible for treatment. In general, VM
lesions located near limb-threatening regions (e.g. proximity to the joint space) or
potentially life/critical function-threatening regions (e.g. proximity to the airway),
symptomatic lesions, and/or lesions with complications are generally considered for
treatment [5, 11]. [Q6: E]
There is significant controversy over how to manage limb-length discrepancy as
the secondary phenomenon of the VM in the lower extremity. Surgical intervention
directly to the epiphyseal plate to arrest further abnormal growth of the affected
long bone has brought mixed results, with further controversy on its long-term
value [13, 14]. Therefore, general the consensus on this issue of vascular-bone syndrome accepted by most ISSVA members these days is to endorse a new strategy to
control the hemodyamic abnormality of VM first, since hemodynamic impact/stimulation by the VM lesions to the intraosseous tissue along the epiphyseal plates is
known to be the cause of abnormal long-bone growth [14, 26]. The strategy based
on conservative treatment only with physical therapy and shoe adjustment until the
long-bone growth is completed is also not acceptable due to increasing morbidity in
gait and spine, as well as the unpredictable outcome of late correction. Meanwhile,
too aggressive an approach with early correction of long-bone discrepancy has also
been abandoned due to significant difficulty in achieving good long-term results.
[Q7: C]
The traditional surgical approach of removing the entire lesion is still theoretically acceptable if the lesion is located in a surgically accessible area and localized
enough to be completely removable with limited or no morbidity. However, this
condition is generally very rare and for most VM lesions there will be significant
morbidity with a surgical approach aimed at complete removal of the lesion.
Therefore, a multidisciplinary approach that combines traditional surgical
therapy with newly introduced embolosclerotherapy utilizing various emboloscleroagents is the treatment strategy of choice [5, 6, 8]. This can substantially reduce
overall treatment-related morbidity with good long-term therapy results [11, 12].
A lesion located along the surgically inaccessible area and/or with prohibitively
high surgical morbidity is generally treated with sclerotherapy alone. The current
trend in the management of VM of the lower extremity involves a multidisciplinary
approach combining surgical therapy, sclerotherapy, and/or embolotherapy, whenever feasible [5, 27]. [Q8: D]
Most of the diagnosis of VM in the lower extremity in particular can be made
efficiently on the basis of non-invasive studies. However, classical invasive studies,
including arteriography and phlebography, are still considered to be the gold standard for the management of all vascular malformations, but they are generally
reserved for use as a road map for the final therapeutic regimen (Fig. 41.6). These
invasive imaging techniques are also used to rule out hidden micro-AVM combined
with the VM, especially when TLPS findings indicate a high possibility of a microAV shunting condition [6, 8, 25]. [Q9: C]
Numerous emboloscleroagents have been tested for the treatment of VM; most
recently, absolute ethanol has been accepted as the scleroagent of choice not only
for VM but also for AVM, with excellent long-term outcome with no recurrence
390 Vascular Surgery
Fig. 41.6
Percutaneous direct puncture phlebographic findings of the ET-form lesions of VM in the thigh; it may
become a road map for the subsequent endovascular management with embolo/sclerotherapy.
when treated properly [11, 12, 25, 28–30]. However, this has significant side effects,
resulting in various acute and/or chronic complications/morbidity, such as deep
vein thrombosis, pulmonary embolism, nerve palsy, and various degrees of skin to
soft tissue damage from bullae to full thickness necrosis. Therefore, the selection of
ethanol as the scleroagent to treat VM has to be based on the risk involved of recurrence, acute morbidity during the therapy, and long-term sequelae of the treatment
[6]. In order to treat VM of the lower extremity safely, careful hemodynamic assessment of the deep vein system is also mandatory, including confirmation of the existence of a normal deep vein system. This is crucial before treatment of the T-form
lesion of VM, the marginal (lateral embryonic) vein in particular. Once the
deep vein system is properly documented, proper treatment of VM can be initiated.
[Q10: D] However, all the other issues raised in Question 10, including history of
deep vein thrombosis, combined LM, and history of skin damage during previous
sclerotherapy, will also require careful assessment to improve overall safety of the
planned treatment.
Differential diagnosis with other forms, T or ET forms, as well as other kinds of
vascular malformation, VM. LM, VLM, or AVM, is mandatory, in view of their different behavior with different clinical impact. This is particularly important for the
ET form of various vascular malformations whose behavior is totally unpredictable.
The ET form retains the original evolutional ability of mesenchymal cells, in contrast to the T form, so that it can grow when the condition/stimulation should meet
(e.g. trauma, surgery, pregnancy, hormone therapy) [10]. Regarding the VM of the
lower extremity, precise differential diagnosis of other conditions such as LM or
AVM is extremely important, because the treatment strategy is substantially different [6, 27]. Besides, initial differential diagnosis for VM, like any vascular malfor-
Congenital Vascular Malformation 391
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mation, should start from the infantile (neonatal) hemangioma which also belongs
to the vascular anomaly together with the vascular malformation. Hemangioma is a
true vascular tumor and not a vascular malformation, possessing distinctively different pathophysiology, anatomico-histology, and clinical behavior [1, 31]. [Q11: E]
The clinical significance of capillary malformation is not understood properly yet,
but it should be included in the evaluation of any vascular malformation although
the modified Hamburg classification did not include it in the classification of
various CVMs, due to the lack of clinical significance for the vascular surgeon [32].
References
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4. Szilagyi DE, Smith RF, Elliott JP, Hageman JH. Congenital arteriovenous anomalies of the limbs.
Arch Surg 1976;111:423–29.
5. Lee BB, Bergan JJ. Advanced management of congenital vascular malformations: a multidisciplinary
approach. J Cardiovasc Surg 2002;10(6):523–33.
6. Lee BB. Critical issues on the management of congenital vascular malformation. Ann Vasc Surg
2004;18(3):380–92.
7. Belov St. Anatomopathological classification of congenital vascular defects. Semin Vasc Surg
1993;6:219–24.
8. Lee BB. Advanced management of congenital vascular malformation (CVM). Int Angiol
2002;21(3):209–13.
9. Belov St. Classification of congenital vascular defects. Int Angiol 1990;9:141–6.
10. Bastide G, Lefebvre D. Anatomy and organogenesis and vascular malformations. In: Belov St, Loose
DA, Weber J, editors. Vascular malformations. Reinbek: Einhorn-Presse Verlag, 1989;20–2.
11. Lee BB, Kim DI, Huh S, Kim HH, Choo IW, Byun HS, Do YS. New experiences with absolute ethanol
sclerotherapy in the management of a complex form of congenital venous malformation. J Vasc Surg
2001;33:764–72.
12. Lee BB, Do YS, Byun HS, Choo IW, Kim DI, Huh SH. Advanced management of venous malformation with ethanol sclerotherapy: mid-term results. J Vasc Surg 2003;37(3):533–8.
13. Mattassi R. Differential diagnosis in congenital vascular-bone syndromes. Semin Vasc Surg
1993;6:233–44.
14. Belov St. Correction of lower limbs length discrepancy in congenital vascular-bone disease by vascular surgery performed during childhood. Semin Vasc Surg 1993;6:245–51.
15. Lee BB: Klippel–Trenaunay syndrome and pregnancy. Int Angiol 2003;22(3):328.
16. Servelle M. Klippel and Trenaunay’s syndrome. Ann Surg 1985;201:365–73.
17. Lee BB, Choe YH, Ahn JM, Do YS, Kim DI, Huh SH, Byun HS. The new role of MRI (magnetic resonance imaging) in the contemporary diagnosis of venous malformation: can it replace angiography?
J Am Coll Surg 2004;198(4):549–58.
18. Lee BB: Current concept of venous malformation (VM). Phlebolymphology 2003;43:197–203.
19. Lee BB, Mattassi R, Choe YH, Vaghi M, Ahn JM, Kim DI, et al. Critical role of duplex ultrasonography for the advanced management of venous malformation (VM). Phlebology, March 2005 (in
press).
20. Lee BB, Seo JM, Hwang JH, Do YS, Kim DI, Byun HS, et al. Current concepts in lymphatic malformation (LM). J Vasc Endovasc Surg 2005;39(1) 67–81.
21. Lee BB, Kim DI, Whang JH, Lee KW. Contemporary management of chronic lymphedema –
personal experiences. Lymphology 2002;35(Suppl):450–5.
22. Lee BB, Mattassi R, Kim BT, Kim DI, Ahn JM, Choi JY. Contemporary diagnosis and management of
venous and AV shunting malformation by whole body blood pool scintigraphy (WBBPS). Int Angiol
April 2005 (in press).
23. Lee BB, Kim BT, Choi JY, Cazaubon M. Prise en charge des malformations vasculaires congénitales
(MVC) en 2003: rôle de la scintigraphy corps entier dans las surveillance évolutive. Angeiologie
2003;55(3):17–26.
392 Vascular Surgery
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2005;24(2):173–84.
25. Lee BB, Do YS, Yakes W, Kim DI, Mattassi R, Hyun WS, Byun HS. Management of arterial-venous
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42. Deep Venous Thrombosis
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Fahad S. Alasfar, Dwayne Badgett and Anthony J. Comerota
A 67-year-old male had a history of a right calf deep venous thrombosis (DVT)
following a flight from California to New York. He was treated on that occasion
with anticoagulation with unfractionated heparin then Coumadin for 3 months.
Recently, he was diagnosed with sigmoid cancer. He is now on postoperative day
three from exploratory laparotomy, sigmoid colectomy and extensive lysis of
adhesions. Although he was haemodynamically stable, he required a transfusion
of three units of blood. DVT prophylaxis for the perioperative period included
graded knee-high compressive stockings and intermittent pneumatic compression (IPC).
Question 1
What are the risk factors that predispose to DVT?
Question 2
What is the clinical presentation of a patient with anti-thrombin III (ATIII)
deficiency?
Question 3
Regarding antiphospholipid antibody (APA) syndrome, which of the following is
not correct?
A. Procainamide has been associated with the development of APA syndrome.
B. Thrombotic complications associated with APA syndrome are limited to the
venous system.
C. Long-term anticoagulation has been recommended in managing APA syn-
drome, maintaining the international normalised ratio (INR) at 3 or higher.
D. Recurrent venous and arterial thrombosis is a major feature of the APA
syndrome.
395
396 Vascular Surgery
Question 4
Regarding Factor V Leiden gene mutation, which of the following is/are correct?
A. Factor V Leiden mutation is an important risk factor for pulmonary embolism
and DVT during pregnancy or use of oral contraceptives.
B. Factor V Leiden mutation is associated with an increased risk of myocardial
infarction and angina.
C. Hyperhomocystinaemia increases the risk of Factor V Leiden carriers having
any Venous Thromboembolic Episodes (VTE) from two per cent to ten per cent.
D. A single-point mutation in the gene coding for coagulation Factor V results in
the formation of a Factor V molecule that is not inactivated properly by activated protein C (APC).
Question 5
Which of the following statements are true concerning prophylaxis for DVT?
A. There are many prospective randomised studies supporting the efficacy
of graded compression stockings in preventing DVT in patients with
malignancy.
B. IPC is as effective as low-dose unfractionated heparin (LDUH) in reducing the
risk of DVT.
C. LDUH and low-molecular-weight heparin (LMWH) are most effective in pre-
venting DVT.
D. Dextran is an excellent alternative to LDUH in preventing DVT.
On the fifth postoperative day, the patient began complaining of mild left calf
pain and swelling. On physical examination, his lower extremities were warm with
normal pulses. The left calf was mildly swollen with slight tenderness. A venous
duplex of the lower extremity revealed thrombosis of the left popliteal, posterior
tibial and peroneal veins.
Question 6
Which of the following statements regarding perioperative DVT is/are correct?
A. In general surgery, the overall incidence of DVT as assessed by labelled fibrino-
gen uptake (FUT) is 25 per cent.
B. In surgical patients with malignant disease, the incidence of postoperative DVT
is 60 per cent.
C. The incidence of postoperative DVT after total hip replacement is 45–55%.
D. Major trauma patients have a low risk for DVT.
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