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Renal hypertrophic infundibular stenosis.

Randall Craver1, Rebecca Boyd, Kenneth Ward

  • 1Department of Pathology, Louisiana State University Health Sciences Center, Children's Hospital of New Orleans, 200 Henry Clay Ave., New Orleans, LA 70118, USA. rcrave@lsuhsc.edu

Fetal and Pediatric Pathology
|August 13, 2005
PubMed
Summary

Bilateral hydrocalycosis, caused by infundibular stenosis, led to a left nephrectomy in a young man. The stenosis resulted from smooth muscle hyperplasia containing rare CD117 cells, suggesting a unique muscular condition.

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Area of Science:

  • Urology
  • Pathology
  • Gastrointestinal Physiology

Background:

  • Bilateral hydrocalycosis can necessitate kidney removal.
  • Infundibular stenosis is a potential cause of urinary tract obstruction.

Observation:

  • An 18-year-old male presented with bilateral hydrocalycosis.
  • Radiographic imaging revealed bilateral infundibular stenosis.
  • A left nephrectomy was performed due to the condition.

Findings:

  • The infundibular narrowing was attributed to marked submucosal smooth muscle hyperplasia.
  • Rare, spindled CD117-positive interstitial cells were observed within the hyperplastic muscle.
  • The presence of CD117 cells suggests muscular hyperplasia, distinct from leiomyomatosis.

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Implications:

  • This case highlights a rare cause of obstructive uropathy.
  • The presence of interstitial cells may offer insights into smooth muscle regulation in the urinary tract.
  • The unknown stimulus for this hyperplasia warrants further investigation.