Staged Surgical and Endovascular Management of Parkes-Weber Syndrome to Preserve Limb Function: A Case Report

Parkes-Weber Syndrome (PWS) is a rare congenital vascular disorder of unknown etiology for which no established curative treatments currently exist. High-flow arteriovenous malformations (AVMs) in PWS can lead to severe complications, including the need for major limb amputation.

A woman in her 50s presented with progressive swelling, severe pain, and impaired ambulation in her right thigh. She was diagnosed at age 14 with varicose veins in her right lower limb and a 1.5-cm limb-length discrepancy, with the right leg longer than the left, and later developed an arteriovenous fistula at age 23. By age 54, examination revealed extensive swelling and hardening of the thigh, with CT imaging showing numerous abnormal vessels forming a nidus and a ruptured hematoma measuring 17 × 14 × 18 cm. She was referred to our facility after unsuccessful attempts to remove hematoma removal at a previous institution and was diagnosed with PWS. Preoperative embolization of multiple niduses was performed to reduce blood flow, although perfusion to other niduses persisted. Hematoma removal was conducted in 2 stages. The first surgery involved securing arterial flow with a stent graft and partial excision of the hematoma (13 h 39 min; blood loss: 9105 mL). Nineteen days later, a second surgery was performed to remove approximately 90% of the hematoma while preserving the remaining wall to avoid complications (8 h 14 min; blood loss: 5533 mL). The patient recovered without complications and was discharged 2 weeks following the second surgery. Minor delays in wound healing resolved within 6 months, and she remains recurrence-free at 48 months.

This case highlights the complexity of managing PWS and underscores the importance of individualized, multidisciplinary care.