Resection of a Large Innominate Vein Aneurysm in a Patient with Neurofibromatosis Type 1

Background Venous aneurysms are exceedingly rare manifestations of neurofibromatosis type 1 (NF1). There are only a handful of cases reported, and no prior cases describing treatment of mediastinal venous aneurysms in this patient population exist. Case Report A 58-year-old woman with NF1 presented with a right neck mass. The mass had recently doubled in size and was associated with cough, hoarseness of voice, and pain. Her pertinent medical history included untreated obstructive sleep apnea, severe pulmonary hypertension, and a recent hospital admission for pneumonia. On physical examination, numerous cutaneous neurofibromas were noted. The mass encompassed her right neck and supraclavicular area with marked respiratory variation. Computed tomography showed a complex 7-cm venous aneurysm including her right innominate, internal jugular, and subclavian veins. Surgical approach involved median sternotomy with right cervical extension and a right infraclavicular counter incision. Extracorporeal circulation was established through the left groin. Ligation of the right internal jugular vein was required. The aneurysm was completely excised, and venous reconstruction consisted of cryopreserved femoral vein anastomosed to right innominate and infraclavicular subclavian veins. Intraoperatively, her preexisting pulmonary hypertension resulted in acute right heart failure requiring placement of a right ventricular assist device (RVAD). She subsequently returned to the operating room for RVAD weaning and sternal closure. Her postoperative course was lengthy; however, many of her aneurysm-related symptoms resolved. Conclusions This case represents management of the only innominate vein aneurysm in the setting of NF1 described in the literature. Vascular reconstruction is possible, however difficult. Careful preoperative planning and use of extracorporeal circulation was necessary in this case.