D.  Correction of gait with physical therapy and shoe adjustment

E.  Biopsy of the soft tissue mass

Question 6

Which of the following is not an indication for the treatment of venous malformation?

A.  Lesion located near to the limb threatening region B.  Life threatening lesion

C.  Symptomatic lesion

D.  Lesion with complication

E.  All the lesions regardless of their condition

Question 7

What is the International Society for the Study of Vascular Anomaly (ISSVA) recommended and most popular strategy with respect to limb length discrepancy?

A.  Immediate surgical intervention to the epiphyseal plate to arrest further abnormal growth of the affected bone

B.  Conservative treatment of limb length discrepancy only with physical therapy and shoe adjustment

C.  Hemodynamic control of venous malformation as a priority whenever possible

D.  Corrective surgery of bone for length discrepancy with the unaffected limb as a priority E.  None of the above

Question 8

What is the current trend of therapeutic strategy for venous malformation lesions in the lower extremity?

A.  Surgical excision of the vascular lesions and related procedure only B.  Transarterial embolotherapy only

C.  Transvenous sclerotherapy only

D.  Multidisciplinary approach with surgical therapy and embolosclerotherapy E.  Percutaneous direct puncture sclerotherapy only

Question 9

What is the general consensus on invasive investigations (e.g., arteriography; phlebography) for venous malformation?

A.  There is no indication for invasive investigation for the diagnosis and treatment of venous malformation.

B.  Invasive investigations are indicated in every suspected case of venous malformation for the confirmation of the diagnosis.

C.  Invasive investigation can be reserved for the therapeutic regimen as a road map and/ or occasional differential diagnosis.

D.  Invasive investigation should be used only for the follow-up assessment. E.  None of the above.

Question 10

What is the most important precondition for the treatment of venous malformation in the lower extremity?

A.  History of deep vein thrombosis B.  Combined lymphatic malformation

C.  Vascular-bone syndrome: length discrepancy of the long bone D.  Existence of deep vein system

E.  Skin lesion with ulcer and necrosis

Question 11

What has to be included in the differential diagnosis of venous malformation?

A.  Lymphatic malformation B.  AV shunting malformation C.  Infantile hemangioma D.  Capillary malformation E.  All of the above

44.2  Commentary

Congenital vascular malformation (CVM) is regarded as one of the most difficult diagnostic and therapeutic enigmas in the practice of medicine. Vascular surgeons often take this vascular malformation quite casually without any specific knowledge, and this cavalier approach can end in failure. Clinical presentations of the CVMs are extremely variable, ranging from an asymptomatic birthmark to a life-threatening condition. This variability in the clinical presentation has been a major challenge to even the most experienced clinicians.1,2 Manyattemptstocontrolthisever-challengingproblem,especiallyinthetwentieth century, were led by surgeons alone, but with mostly disastrous results because of poorly planned and over-aggressive surgical treatment carried out on the basis of limited knowledge.3,4 Recently, a multidisciplinary approach was introduced with a new concept based on Hamburg classification.5,6 The Hamburg classification gives excellent clinical applicability with minimum confusion because the new terminology itself provides substantial

information on the anatomico-pathophysiological status of vascular malformation; it has become the most fundamental rationale for the advanced concept of vascular malforma- tion7–9 (Table 44.1). It classifies complex groups of various vascular malformations based on the predominant type: VM, LM, AVM, and combined form which is mostly hemolymphatic malformation (HLM). The VM is the most common type of CVM together with LM and they often combine together to make the clinical condition quite complicated.

When this HLM consists of only two components, that is, VM and LM, it is grouped separately as VLM which is almost equivalent to Klippel–Trenaunay syndrome, where our patient belongs.

ThenewHamburgclassificationprovidescriticalinformationrelatingtorecurrencebased on precise information of embryonal stage when the developmental arrest has occurred.9,10

Table 44.1  Hamburg classification of congenital vascular malformation: 1988 consensus with modification

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 VM17 (Fig. 44.3). [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 assesslymphaticfunctionandthelymph-conductingsystemingeneralandruleoutchronic 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. 44.4). 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. 44.5).

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.

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.

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-threaten- ing regions (e.g., proximity to the joint space) or potentially life/critical function-threaten- ing 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 theepiphysealplatetoarrestfurtherabnormalgrowthoftheaffectedlongbonehasbrought 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 ISSV A members thesedays is to endorsea newstrategy to controlthehemodyamic abnormality of VMfirst, 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]

MostofthediagnosisofVMinthelowerextremityinparticularcanbemadeefficiently 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