Major Limb Amputations in Patients with Congenital Vascular Malformations.

ABSTRACT

BACKGROUND: The need for major amputations in patients with vascular malformations is rare. This study reviews our contemporary experience with major amputations in patients with vascular malformations. METHODS: 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 analyzed to identify those requiring either a transfemoral or transhumeral amputation. This cohort was investigated for clinical course, surgical procedures, and outcomes. RESULTS: Five patients (0.5%) underwent major amputation, including 3 transhumeral and 2 above-knee amputations. The median age was 37.8 years (interquartile range (IQR) 25.4-40.2), and 2 (40%) were male. Four (80%) patients had high-flow arteriovenous malformations, including 1 (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, with the amputation being at the level of residual malformation. As such, amputation had been initially felt to be high risk 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, 20 direct stick embolizations, 3 debulking procedures, 38 debridements, 6 skin grafts or muscle flaps, and 4 minor amputations. The median time course of treatment before amputation was 117 months (IQR 44-171). Indications for major amputation included chronic pain and recurrent bleeding in all 5 (100%) patients, loss of function in 2 (40%), nonhealing wounds in 2 (40%), and sepsis in 1 (20%) patient. There were no perioperative deaths. The median blood loss was 1,000 mL (IQR 650-2,750). All patients required transfusion of packed red blood cells with a mean of 1.6 units (standard deviation 0.54). Transhumeral amputation was facilitated by transcatheter embolization in 1 (33%) and an occlusion balloon within the subclavian artery in 2 (66%) patients. The median length of stay was 6 days (IQR 5-13). The median length of follow-up was 132 months (IQR 68-186) from initial intervention and 12 months (IQR 8-31) 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, 1 patient underwent 3 transcatheter embolization procedures before revision and 1 underwent 1 embolization after revision. CONCLUSIONS: Although rare, successful amputation at the level of residual malformation can be performed in select patients with refractory complications of vascular malformations including intractable pain, bleeding, or nonhealing wounds. Specific preoperative and intraoperative measures may be critical to achieve satisfactory outcomes, and endovascular techniques continue to play a role after amputation.