Midaortic syndrome and hypertension in childhood

Midaortic syndrome is a form of fibromuscular hyperplasia that involves the abdominal aorta including the renal and visceral branches. Symptomatology depends on the degree and location of vessel narrowing. This report updates the authors' experience and details 17 operative cases, the largest reported series. Seventeen of the 45 patients who have been operated on for severe renovascular hypertension have had this syndrome. Ages have ranged from 5 months to 15 years (average, 9.7 years). Signs and symptoms have included various manifestations of malignant hypertension, congestive heart failure, oliguric renal failure, and claudication. Intestinal angina has not been noted despite celiac and/or superior mesenteric arterial involvement. Although balloon angioplasty was performed in two patients, lasting results were not achieved. One primary nephrectomy was performed. The other 16 patients had vascular reconstructions including aortoaortic bypass grafting (n = 12), with bilateral renal artery bypasses (n = 9) or unilateral renal bypass (n = 3) or bilateral renal bypass alone (n = 4). The majority had associated visceral artery narrowing, but excellent collaterals have been present so no visceral reconstructions have been required. All renal artery bypasses have been with reinforced saphenous vein.

In the average follow-up period of 48 months, 12 of the 17 patients have been cured of hypertension, and the other five have improved. Claudication, congestive failure, and renal failure have been alleviated. Thus far, reinforcement of the saphenous vein grafts has prevented aneurysmal degeneration and graft loss.

These results indicate that aggressive single-stage reconstruction is the best approach for these children.