Cardiopulmonary bypass in surgery for complex-combined vascular malformation of the lower limb: case report

Abstract

A 16-year-old boy was referred with features of Parkes Weber syndrome (PWS) involving the right lower limb. He had presented at birth with cutaneous vascular malformations (VM) in the right thigh and at the age of 7 years developed congestive cardiac failure, which was controlled with drugs. He received alpha interferon and steroids during this period without any benefit. He defaulted follow-up and at 12 years of age presented with further enlargement of the VM in the right thigh and leg with skin and soft tissue thickening. At this stage, embolization and subsequent excision of the VM were tried, but the surgery was abandoned because of massive hemorrhage. Over the next 4 years, the boy became totally bedridden because of massive increase in the size of the limb, repeated hemorrhages, and secondary infection of the VM. Right hip disarticulation was considered the best option to improve his quality of life. To prevent uncontrollable hemorrhage during surgery, the disarticulation was done under cardiopulmonary bypass with low circulatory flow. Postoperatively, the patient required intensive care nursing for a week. He is presently ambulatory with crutches. Cardiopulmonary bypass with low flow has been used for treating posttraumatic arteriovenous malformations. However, its use in surgery for PWS has not been reported earlier.

Diagnosis and management of extensive vascular malformations of the lower limb

“…The main complication of Parkes Weber syndrome and StewarteBluefarb syndrome (SBS) is an increase in cardiac output secondary to the A-VM, which can cause ischemia-induced skin ulceration and heart failure in the most severe cases. 37 Whether or not the vascular malformation involves the trunk, many patients with extensive vascular malformations in the limb present with pulmonary hypertension caused by recurrent pulmonary embolisms 38,39 or a proliferation of new vessels, possibly related to neoangiogenesis.…”

Parkes Weber syndrome—Diagnostic and management paradigms: A systematic review

“…Right leg Primary angiosarcoma of the affected leg with systematic metastases Ismail et al 24 16 39 16/M Asymptomatic Right leg Positive family history for PWS; no genetic evaluation Schaal et al 40 29/F Limb enlargement during pregnancy…”

“…13,18,41,44,45,48 PWS and high-output heart failure Fifteen (31.3%) cases had high-output heart failure due to significant AV shunting. 13,21,24,26,29,30,33,34,38,41,48,49 There was no significant gender predominance (female-male ratio: 1:1), whereas 10 (66.7%) cases 23 46/M Limb amputation with chemotherapy Two months after died from pulmonary and cerebral metastases Ismail et al 24 16/M Hip disarticulation under low-flow cardiopulmonary bypass…”

“…Twelve (25.0%) cases were presented with chronic venous ulcerations of the affected limb 13,22,24,28,30,32,33,35,44,46,49,50 and four (8.3%) manifested distal arterial ischemia. 37,38,43,50 PWS and spinal AVM…”

Treatment of Large Arteriovenous Malformation in Right Lower Limb

“…CPB has been used for managing massive vascular lesions when vascular control cannot be achieved by conventional methods. There was one case of vascular malformation of the lower limb undergoing hip disarticulation under CPB; this case has been reported in the literature [5]. CPB enables the resection to proceed in a bloodless field and the surgeon to identify the adjacent structures, thereby minimizing the risk of damage to these structures and enabling a more complete resection.…”