2008 Annual Meeting Abstracts
Congenital Infundibulopelvic Stenosis With Renal Impairment: Surgical Management
Craig A Peters
University of Virginia, Charlottesville, VA
Introduction:
Infundibulopelvic stenosis (IPS) is a rare condition affecting development of the renal pelvis and infundibuli that can produce renal deterioration. There is no well-defined surgical management strategy. Based on 3 cases over 10 years, a novel surgical approach is described.
Methods:
Three patients (4 renal units) with IPS and renal deterioration have been surgically managed. All presented with azotemia or loss of unilateral function demonstrated by renal scan. The initial patient was managed with medial infundibuloplasties and a uretero-calicostomy. Subsequent patients underwent lateral nephrotomies with multiple calico-calicostomies and medial uretero-calicostomy.
Results:
Patient #1 underwent multiple infundibuloplasties and a uretero-calicostomy at age 16 presenting with proteinuria and azotemia following nephrectomy at age 1 for MCDK. Her renal deterioration slowed, but she had incomplete decompression of her upper pole. She has been stable for 10 years. Patient #2 was diagnosed incidentally at 17 years with severe right IPS and minimal left. His split function deteriorated from 43 to 34 percent over 1 year. He had satisfactory decompression and stabilization of renal function with 3 years of follow-up. Patient #3 presented at age 10 with proteinuria, azotemia and hematuria with severe bilateral IPS. She underwent staged bilateral multiple calico-calicostomies and uretero-calicostomy. Her creatinine has decreased and both kidneys show improved drainage.
Conclusions:
IPS is a rare condition that may produce renal deterioration. When surgical correction is appropriate, lateral nephrotomies through the thinned renal parenchyma with multiple calico-calicostomies and medial uretero-calicostomy can produce satisfactory renal drainage and is well tolerated.
