INFUNDIBULOPELVIC STENOSIS - A LONG-TERM FOLLOW-UP

We followed 21 patients with congenital infundibulopelvic stenosis, a rare obstructive disorder of the intrarenal collecting system, for a m edian of 11 years (range 2 to 28 years). Of these patients 19 (90%) ha d evidence of bilateral renal disease. In particular, 10 patients had bilateral infundibular pelvic stenosis, 6 a contralateral cystic dyspl astic kidney and 3 a congenitally absent kidney. Extended observations of the patients with this disorder revealed that end stage renal dise ase or renal insufficiency developed in 8 patients (37%), all with bil ateral renal anomalies. Renal biopsies in patients with end stage rena l failure revealed widespread areas of renal dysplasia proximal to the stenotic infundibulum with focal or global glomerulosclerosis of the glomeruli, which was not involved in the dysplastic process. These pat hological findings are consistent with the presence of a renal hyperfi ltration injury. Our data suggest that the prognosis of infundibulopel vic stenosis is dependent upon the extent of total renal mass involved with the disease process and the duration of followup.