Congenital infundibulopelvic stenosis

Indications for intervention, surgical technique, and review of literature

Christopher E. Bayne, Craig A Peters

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Introduction 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. Materials and 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. Discussion 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.[Figure presented]

Original languageEnglish (US)
Pages (from-to)389.e1-389.e5
JournalJournal of Pediatric Urology
Volume12
Issue number6
DOIs
StatePublished - Dec 1 2016

Fingerprint

Pathologic Constriction
Kidney
Renal Insufficiency
Multicystic Dysplastic Kidney
Azotemia
Kidney Pelvis
Hydronephrosis
Urography
Case Management
Urology
Pituitary Gland
Nephrectomy
Ultrasonography
Magnetic Resonance Spectroscopy
Pediatrics
Wounds and Injuries

Keywords

  • Calicocalicostomy
  • Infundibulopelvic dysgenesis
  • Infundibulopelvic stenosis
  • Multicystic dysplastic kidney
  • Ureterocalicostomy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Urology

Cite this

Congenital infundibulopelvic stenosis : Indications for intervention, surgical technique, and review of literature. / Bayne, Christopher E.; Peters, Craig A.

In: Journal of Pediatric Urology, Vol. 12, No. 6, 01.12.2016, p. 389.e1-389.e5.

Research output: Contribution to journalArticle

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abstract = "Introduction 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. Materials and 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. Discussion 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.[Figure presented]",
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N2 - Introduction 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. Materials and 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. Discussion 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.[Figure presented]

AB - Introduction 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. Materials and 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. Discussion 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.[Figure presented]

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