Nephron sparing management of Fraley's syndrome with a pyelopyelostomy

Purpose: We describe a new technique for nephron sparing management of functionally significant vascular compression of the upper pole infundibulocaliceal system (Fraley's syndrome). Materials and Methods: Two patients evaluated for debilitating, narcotic dependent flank pain had significant upper pole infundibulocaliceal obstruction resulting from aberrant major arterial branches entering the hilum dorsally. Reconstructive surgery consisted of dismembered pyelopyelostomy with transposition of the crossing vessels in both patients. Results: Pain relief was immediate and complete in both patients. Followup radiographic evaluation at 1 and 12 months, respectively, has also documented resolution of the obstruction. Conclusions: Vascular compression causing obstruction of the superior infundibulum can cause debilitating pain. Pathophysiologically operative intervention can be nephron sparing and gratifying for the surgeon and the patient.