Clinical Evaluation

This patient underwent a thorough investigation of the nature and extent of the congenital vascular malformation (CVM) involved.

A combination of various non- to minimally- invasive studies were performed to confirm the clinical impression of venolymphatic malformation (VLM): duplex ultrasonography, whole-body blood-pool scintigraphy (WBBPS), magnetic resonance image (MRI) study, transarterial lung perfusion scintigraphy (TLPS), and/or radionuclide lymphoscintigraphy.

The primary hemodynamic impact and the secondary musculoskeletal impact of the venous malformation (VM) were assessed as the main CVM lesion in addition to the extent/degree of each component of the VM, truncular (T) and extratruncular (ET) form, involved in the extremity.

A thorough skeletal evaluation of the long-bone growth discrepancy of the lower extremity and the degree of pelvic tilt with its compensatory scoliosis was also made with conventional bone X-rays.

The TLPS assessment was performed substituting arteriographic investigation of the lower extremity for the possible hidden micro-arteriovenous malformation (AVM) lesion, which was marginally indicated due to an unusually increased venous flow by the isolated VM lesion alone on the duplex scan under the normally developed and functioning deep vein system.

An ascending phlebography was also performed together with the percutaneous direct-puncture phlebography as a therapeutic guide; mandatory confirmation of the presence of a normal deep vein system of the lower extremity was made before starting the treatment to the infiltrating ET-form lesion of the VM.

The final diagnosis confirmed extensive involvement of the VM as an infiltrating type of the ET form causing serious clinical impact directly to the venous system hemodynamically as well as to the skeletal system to induce abnormal long-bone growth of the left lower extremity. A moderate degree of venectasia as a T form of VM along the left femoral-popliteal vein segment was also found, by WBBPS, MRI and duplex scan, and subsequently confirmed by separate ascending phlebography.

A venectasia of the femoral vein was assessed to have a limited clinical significance at this stage in comparison to the ET-form lesions of the VM.

The lymphatic malformation (LM) component which is mixed with the ET form of VM, was confirmed as the ET form, giving minimum and limited clinical impact so that a conservative management/observation was instituted for this LM component.

Therefore, the ET-form lesions of VM along the knee region were selected for active treatment as a priority; this was followed by the ankle and foot lesions.

The primary indication to initiate the treatment immediately was that these lesions were potentially limb-threatening (e.g. hemarthrosis) due to their proximity to the joints with increased vulnerability to repeated trauma, especially as a cause of her knee symptoms.

The treatment was further indicated to arrest/slow down their impact on abnormal long-bone growth.

Multiple infiltrating ET lesions of the VM along the knee region, which is surgically not amenable, were selected for ethanol sclerotherapy as independent therapy. Multisession ethanol sclerotherapy was given using 100-80 per cent absolute ethanol in calculated dosage - not exceeding 1.0 mg per kg of body weight as maximum dose per session - by direct puncture technique under general anesthesia. Close cardiopulmonary monitoring during the procedure was ensured to control and/or prevent transient pulmonary hypertension by the unavoidable spillage of ethanol into the systemic circulation from the lesion during treatment.

The symptomatic lesions along the knee with recurrent painful swelling following minor injuries were controlled well without complication/morbidity and substan-

tially reduced the risk of intra-articular bleeding and subsequent hemarthrosis. Subsequently, the ET-form VM lesions at the foot and ankle underwent surgical excision following preoperative multisession ethanol and N-butyl cyanoacrylic glue embolosclerotherapy with much reduced perioperative morbidity to improve foot function.

Following successful control of multiple VM lesions along the knee, ankle, and foot with priority as a potentially limb-threatening condition, other VM lesions, scattered throughout the lower extremity, were also treated with absolute ethanol to assist further attempts to arrest the abnormal long-bone growth of the lower extremity. The abnormal long-bone growth is attributed to these VM lesions scattered within the muscular structure of the lower extremity in the extensive infiltrating type of ET, with significant impact on the venous circulation along the epiphyseal plate.

In addition to the multisession embolosclerotherapy as independent and/or adjunct perioperative therapy to the VM lesions, the conservative supportive measures to improve and/or maintain overall venous function have been supplemented with the use of a graded compression above-knee stocking to prevent chronic venous insufficiency.

The final decision for the T-form lesion was left femoral-popliteal venectasia, but it was decided to defer treatment until urgent treatment of the ET form of the VM was finished, but to keep it under close observation. It might eventually require treatment (e.g. venorrhaphy, venous bypass) to prevent development of venous thromboembolism when significant venous flow/volume reduction should occur following successful control of the ET form of VM lesions. The hemodynamic consequences of the treatment of such extensive ET-form lesions directly affect total venous blood volume through the deep vein system.

The LM component in this patient was treated only with complex decongestive therapy (CDT) in order to prevent full development of lymphedema. The infiltrating ET form of LM detected together with the ET form of VM has been shown to put extra burden on the marginally normal lymph-conducting system on lymphoscinti-graphic evaluation. Therefore, continuous surveillance for aggressive preventive measurement of local to systemic cellulitis along this ET form of LM lesions is mandated.

This patient will continue to be managed by the multidisciplinary team of the CVM Clinic at regular intervals for her entire lifetime, through periodical follow-up assessment of the treatment results and the natural course of the untreated lesions.

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