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Venous Reconstruction In The Setting Of Congenital Iliac Vein Malformation
Sandra Iskandar, MD1, Michelle Wang
1, Tatiana Hage
2, Vincent Narvaez
1.
1Riverside Community Hospital, Riverside, CA, USA,
2California University of Science and Medicine, Colton, CA, USA.
DEMOGRAPHICS:Phlegmasia cerulea dolens (PCD) is a rare but life-threatening complication of deep vein thrombosis (DVT). It results from extensive venous occlusion causing congestion, and ischemia. Classic presentation includes pain, swelling, and discoloration, with high mortality if untreated. We report a case of PCD in congenital left iliac vein atresia treated with catheter-directed thrombolysis, fasciotomy, and femoral-femoral cross over vein bypass (Palma procedure).
HISTORY:A 42-year-old previously healthy male presented with 24 hours of progressive leg pain and discoloration. On exam, the limb was tense, discolored, tender, with preserved motor, sensory, and pulses (Figure 1). Duplex ultrasound revealed extensive left iliofemoral deep vein thrombosis (DVT). The patient underwent urgent catheter-directed thrombolysis with thrombus removal. Venography demonstrated congenital atresia of the left common iliac vein with lumbar collateral drainage (Figure 2). Calf fasciotomies revealed viable muscle. Subsequent serial washouts with VAC therapy were performed. Due to persistent venous congestion, he underwent a Palma procedure with femoro-femoral GSV transposition to bypass the iliac atresia.
PLAN:The patient tolerated all interventions without hemodynamic instability. Postoperatively, he underwent serial washouts and wound VAC therapy, followed by discharge with continued VAC care. During hospitalization, he was maintained on a therapeutic heparin infusion and transitioned to long-term prophylactic anticoagulation at discharge. At follow-up, he remains asymptomatic with a patent venous bypass and no evidence of venous hypertension.
DISCUSSION: This case presents a serious complication of congenital venous malformation, which can predispose patients to extensive thrombosis and life-threatening sequelae such as phlegmasia cerulea dolens. In these settings, vascular reconstruction plays a central role in restoring venous outflow. The Palma procedure is an effective surgical treatment option for congenital iliac vein atresia that offers high long-term patency rates. In our patient, it provided durable venous drainage with resolution of symptoms and preservation of limb viability.
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