A Rare Case of Visceral Arterial Stenoses in Williams-Beuren Syndrome Treated by Complex Revascularization

Williams-Beuren syndrome is a rare neurodevelopmental disorder. We present the case of a 27-year-old patient with Williams-Beuren syndrome and a juxtarenal abdominal aorta coarctation. As arterial hypertension (AHT) was not controlled, bilateral renal artery bypasses were performed at the age of 2 years by means of a hepatorenal bypass and a splenorenal bypass. Twenty years later, the patient presented with abdominal pain, diarrhea, and recurrence of AHT, and severe celiac artery and superior mesenteric artery stenoses were discovered. The distal arterial complications of this syndrome are uncommon. After 5 years of medical treatment, aggravation of the patient's symptoms prompted us to consider possible surgical management. The patient was successfully treated using a complex direct and indirect procedure that consisted of a bypass between the celiac aorta and infrarenal aorta, associated with a celiac artery bypass. Instead of endovascular management, this surgical procedure could be considered effective and long lasting for treating this rare cause of renal AHT.