title: Congenital infundibulopelvic stenosis: Indications for intervention, surgical technique, and review of literature.
abstract: BACKGROUND
  Congenital infundibulopelvic stenosis (IFPS) is a rare renal dysmorphism marked by dilated calyces proximal to diminutive infundibulum and renal pelvises. The entity is theorized to exist on the spectrum of congenital obstructive renal diseases between ureteropelvic junction obstruction and multicystic dysplasia.
  
  
  OBJECTIVE
  This case series sought to review and present the surgical management of three cases of IFPS with progressive renal insufficiency.
  
  
  METHODS
  After Institute Review Board approval, we reviewed three cases of IFPS treated by a single surgeon over a 20-year period. All cases displayed evidence of progressive renal decline. The first case of a 16-year-old female had IFPS in a solitary kidney, with previous contralateral nephrectomy of a multicystic dysplastic kidney. The second case, a 17-year-old male, presented after blunt trauma to the ipsilateral flank. The third case of a 10-year-old female presented with microhematuria, mild azotemia and bilateral IFPS.
  
  
  RESULTS
  Our current surgical management has evolved from renal sinus exposure and multiple infundibuloplasties in the first case to preoperative three-dimensional mapping of the dilated calyceal system with intraoperative ultrasonography for selective calicocalicostomies draining to a lower pole ureterocalicostomy in the third case. Our first case, treated with multiple infundibuloplasties experienced slow renal decline into her 20s, and the later cases, treated with calicocalicostomies, have experienced stable to mild loss of renal function with ongoing nephrological follow-up.
  
  
  CONCLUSIONS
  IFPS is a complex pediatric urology problem without a clear treatment algorithm. The goal of surgical intervention is to offer an opportunity to halt or reduce progressive renal insufficiency. Our current surgical approach utilizes preoperative three-dimensional modeling via magnetic resonance urography to guide placement of multiple calicocalicostomies draining to a lower pole ureterocalicostomy.
  
  
  CONCLUSIONS
  Not all cases of IFPS require surgical intervention. We have intervened in cases of IFPS with progressive hydronephrosis or worsening renal insufficiency with acceptable results.
authors.affiliation: Department of Urology, The George Washington University, Washington, DC, USA.
authors.forename: Christopher E
authors.initials: CE
authors.lastname: Bayne
authors.affiliation: Pediatric Urology, Children's Health System Texas, University of Texas Southwestern, Dallas, TX, USA. Electronic address: craig.peters@childrens.com.
authors.forename: Craig A
authors.initials: CA
authors.lastname: Peters
mesh_terms: D000293:Adolescent; D002648:Child; D003251:Constriction, Pathologic / Q000151:congenital / Q000601:surgery; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000151:congenital* / Q000601:surgery*; D007682:Kidney Pelvis / Q000002:abnormalities* / Q000601:surgery*; D008297:Male; D013520:Urologic Surgical Procedures / Q000379:methods
keywords: Calicocalicostomy; Infundibulopelvic dysgenesis; Infundibulopelvic stenosis; Multicystic dysplastic kidney; Ureterocalicostomy