Management challenges of a large upper extremity vascular malformation in a patient with Capillary Malformation/Arteriovenous Malformation (CM-AVM) syndrome.

We describe a 17-year-old boy with Capillary Malformation-Arteriovenous Malformation (CM-AVM) syndrome and a massive vascular malformation of the right chest wall, shoulder, and upper arm. Persistent growth of the malformation caused cutaneous ulcerations and recurrent massive bleeding episodes. We proceeded with a modified shoulder disarticulation preceded by ligation of the subclavian artery and innominate vein by median sternotomy. After a staged debulking resection of the residual chest wall AVM with rotational transverse rectus abdominis myocutaneous flap coverage, the patient discharged home safely. This report demonstrates a multidisciplinary approach is critical to manage life threatening complications in CM-AVM patients.