MIDAORTIC SYNDROME PRESENTING IN CHILDHOOD

Mid-aortic syndrome (MAS) is an uncommon condition characterized by se gmental narrowing of the proximal abdominal aorta and ostial stenosis of its major branches. It is usually diagnosed in young adults, but ma y present in childhood as a challenging problem. Over the past 20 year s 13 patients with MAS have presented to this institution. All had hyp ertension, four had associated neurofibromatosis, three persistent eos inophilia and three had Williams syndrome. In all cases arteriography showed a smooth segmental narrowing of the abdominal aorta with concom itant stenosis at the origins of the renal arteries. Six children were successfully treated with antihypertensive medication alone. Percutan eous transluminal angioplasty was attempted in two cases with poor res ult. Surgery was indicated in seven children with refractory hypertens ion and progressive renal impairment. Techniques used to revascularize the kidneys included thoracoabdominal to infrarenal aortic bypass wit h renal artery reimplantation, splenorenal bypass, gastroduodenal to r enal bypass, aortorenal bypass and autotransplantation.