Bilateral infundibulopelvic stenosis without renal insufficiency: is surgery necessary?

The following case describes the clinical course of a patient with bilateral infundibulopelvic stenosis from her initial presentation at age 2 through the age of 14 years. This condition is associated with hypoplasia of segments of the upper collecting system and is characterized by dilated calyces that drain through stenotic infundibulae. Our patient is unique in that, although her renal function has slowly deteriorated with time, she has a persistently non-obstructive pattern on dynamic imaging studies. Only a minority of patients reported in the literature with this condition progress to renal insufficiency or failure. Although some patients have undergone surgery for presumed obstruction, surgical intervention has no proven benefit. Patients are at risk for hyperfiltration injury and should be followed for the development of hypertension, proteinuria and renal insufficiency. Without evidence of obstruction, medical management remains the cornerstone of treatment.