Abstract

The need for major amputations in patients with vascular malformations is rare. This study reviews our experience with major amputations in patients with vascular malformations. A retrospective review from April 2014 to November 2018 identified 993 patients undergoing management of a vascular malformation involving the upper or lower extremity at a tertiary center. This population was investigated for clinical course, surgical procedures, and outcomes. Five patients (0.5%) underwent major amputation, including three transhumeral and two above-knee amputations. The median age was 37.8 years (interquartile range [IQR], 25.4-40.2 years), and two (40%) were male. Four (80%) patients had high-flow arteriovenous malformations, including one (20%) with Parkes Weber syndrome. One (20%) patient had a low-flow venous malformation associated with Klippel-Trénaunay syndrome. All patients had malformation extending into the chest or pelvis, and amputation was initially thought to be unfeasible because of the proximal extent of the lesions. Before amputation, a median of 11 procedures (IQR, 4-39) were performed per patient. This included 29 transarterial embolizations, 4 transvenous embolizations, 19 direct stick embolizations, 3 debulking procedures, 38 débridements, 6 skin grafts or muscle flaps, and 4 minor amputations. The median time course of treatment before amputation was 117 months (IQR, 44-171 months). Indications for major amputation included chronic pain and recurrent bleeding in all five (100%) patients, loss of function in two (40%), nonhealing wounds in two (40%), and sepsis in one (20%) patient. There were no perioperative deaths. Median blood loss was 1000 mL (IQR, 650-2750 mL). All patients required transfusion of packed red blood cells with a mean of 1.6 units (standard deviation, 0.54 unit). Transhumeral amputation was facilitated by transcatheter embolization in one (33%) and an occlusion balloon within the subclavian artery in two (66%) patients. The median length of stay was 6 days (IQR, 5-13 days). Median length of follow-up was 132 months (IQR, 68-186 months) from initial intervention and 12 months (IQR, 8-31 months) from amputation. Two patients (40%) who had undergone transhumeral amputation required revision of the amputation site for recurrent ulceration at 2 and 38 months. Of these, one patient underwent three transcatheter embolization procedures before revision, and one underwent one embolization after revision. Although rare, successful amputation can be performed in select patients with refractory complications of vascular malformations including intractable pain, bleeding, and nonhealing wounds. Specific preoperative and intraoperative measures may be critical to achieve satisfactory outcomes, and endovascular techniques continue to play a role after amputation.

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