Abstract
BACKGROUND: Midaortic syndrome (MAS) is a rare vascular anomaly characterized by segmental narrowing of the distal descending thoracic or abdominal aorta. Renal or visceral arteries may also be affected to varying degrees. MAS is often associated with renovascular hypertension, and requires early intervention. When medical therapy and percutaneous interventions fail to control hypertension, surgical treatment is required. We report a case of MAS that failed to respond to bilateral renal artery stenting, but treated with aortic bypass and orthotopic right renal autotransplantation with good outcome. CASE PRESENTATION: A 31-year-old woman presented with headache and poorly controlled hypertension due to severe MAS. She had severe ostial stenoses of renal and visceral arteries. Her hypertension failed to respond to medical therapy (four drugs) and bilateral renal artery stenting. The implanted stent in the right renal artery rendered revascularization of the artery difficult. A one-stage revascularization was performed, which consisted of an aortoaortic bypass (between the suprarenal and infrarenal abdominal aorta) with a prosthetic graft, an orthotopic right renal autotransplantation and an aorto-left renal arterial bypass with autogenous saphenous vein grafts. Her recovery was uneventful. At 1-year follow-up, the patient remained well. Her hypertension improved. A postoperative computed tomography angiography showed that all the grafts were patent with no abnormalities at the anastomosis. CONCLUSION: Multiple bypass surgery with reimplantation of autogenous vein graft onto the prosthetic graft is a feasible and effective procedure in renal artery revascularization for MAS. Orthotopic autotransplantation is the procedure of choice in complex renal artery reconstruction.