Major limb amputations in patients with congenital vascular malformations

Abstract Objective 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 (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-Trenaunay 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 due to the proximal extent of the lesions. Prior to 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 prior to 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%), non-healing wounds in 2 (40%), and sepsis in 1 (20%) patient. There were no perioperative deaths. Median blood loss was 1000 mL (IQR: 650-2750). All patients required transfusion of packed red blood cells with a mean of 1.6 units (SD: 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). 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 prior to 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 non-healing wounds. Specific pre- and intraoperative measures may be critical to achieve satisfactory outcomes, and endovascular techniques continue to play a role after amputation.