Superior vena cava syndrome complicating calcific uremic arteriolopathy in an ESRD male patient on maintenance hemodialysis following failed kidney transplantation

We described the unusual presentation of right unilateral facial swelling in a 48-year old end-stage renal disease (ESRD) male patient on maintenance hemodialysis following a failed kidney transplant. This was subsequently confirmed to be secondary to the extrinsic compression of the superior vena cava (SVC) by a large lobulated amorphous extra-osseous right axillary mass lesion that extended into the upper right thoracic outlet. Superior vena cava venogram and balloon angioplasty led to symptomatic relief. The factors involved in the pathogenesis of calciphylaxis are discussed and the available therapeutic options for this rare albeit debilitating disease are reviewed.