Experience in the surgical treatment of Fraley’s Syndrome

OBJECTIVE: To report our 20-year experience with nephron-sparing surgery in the treatment of Fraley's syndrome. MATERIALS AND METHODS: From September 1976 to July 1996, 6 women, 18-43 years old, underwent surgical treatment. All patients had been suffering debilitating right flank pain for at least 6 months before the operation. Diagnosis was in call cases made with the use of intravenous pyelography and renal arteriography, which showed a vascular impression on the superior infundibulum with secondary dilatation of the upper pole calyx. This was localized only on the right side in 5 cases, while in 1 it was bilateral though more severe on the right. Ipsilateral nephroptosis was observed in 2 of the patients. In 2 cases in whom the superior infundibulum was sufficiently long, an infundibulo-infundibulostomy with everted flaps was performed. In a case in whom a short infundibulum was compressed between a venous and an arterial branch, Fraley's infundibulopyelostomy was carried out. In 1 patient in whom the infundibulum was compressed by the anterior-superior segmental artery, a Heineke-Mikulicz-type infundibulorrhaphy was combined to vasopexy. In the remaining 2 cases, the infundibulum was not cut: in one case, a simple vasopexy of two vascular branches was carried out, while in the other, a minor arterial branch was ligated and divided. In 4 patients, nephropexy was also performed. RESULTS: No significant intra- or postoperative complications were observed. Follow-up averaged 102.5 months. Pain relief was complete in 5 cases, in whom disappearance of the vascular impression was also radiologically demonstrated. Only 1 patient, who underwent infundibulorrhaphy and vasopexy, experienced occasional flank pain and urinary infection after the operation, with just a slight improvement in the excretory urogram. CONCLUSIONS: Surgical treatment of Fraley's syndrome is indicated only in symptomatic and/or complicated cases; in relation to the type of obstruction and the anatomy of the intrarenal structures, whether excretory or vascular, several effective nephron-sparing techniques can be selected.