Phlegmasia Cerulea Dolens in a Patient with Ulcerative Colitis Treated by Iliofemoral Venous Thrombectomy

Abstract

The authors present the case of a 17-year-old woman with ulcerative colitis who presented with phlegmasia cerulea dolens (PCD) of the right leg. On examination, the compartments of her right leg were tense, sensory and motor function were greatly diminished, and pedal pulses were absent. Venous duplex revealed extensive venous thrombosis from the tibial veins to the external iliac vein. Treatment consisted of anticoagulation with systemic heparinization, and she was brought to the operating room for intraoperative venography, inferior vena caval filter placement, and four-compartment fasciotomy. Arterial pulses did not return; therefore, venous thrombectomy was performed by extrusion of distal thrombus with an Esmarch bandage, and fluoroscopic guided balloon catheter thrombectomy of the distal inferior vena cava and iliac veins proximally. Completion venography revealed the absence of residual thrombus. Postoperatively, the patient developed a lower gastrointestinal hemorrhage and anticoagulation was terminated. The fasciotomy skin incisions underwent delayed closure on postoperative day 6, and the patient was subsequently discharged with compression stockings. Total abdominal colectomy with creation of a J-pouch was performed 1 month following discharge. After 1 year, the patient had no complaints related to her leg. On examination, no evidence of venous insufficiency was present. Venous duplex demonstrated a patent deep venous system with no evidence of reflux. Uncomplicated PCD responds to heparin therapy in 50-80% of patients, but the risk for developing the postphlebitic syndrome is high. Venous thrombectomy has been reported to provide better long-term functional outcome. Catheter-directed thrombolysis has the added theoretical benefit of preserving endothelial and valvular function, and high technical success rates have been reported. No long-term functional outcome results for thrombolysis are available. Thrombolysis is contraindicated if compartment syndrome or venous gangrene is present. The aggressive surgical management of PCD may result in improved outcome.