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Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50

Daniel G Wong1, Michelle K Arevalo1, Niccolo Maria Passoni1,2

  • 1Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

BJU International
|August 17, 2018
PubMed

Insights

A new scoring system, RUBACE, effectively grades prune belly syndrome (PBS) severity and categorizes patients. This tool aids in understanding genotype-phenotype correlations and future treatment studies for PBS.

Area of Science:

  • Urology
  • Pediatric Surgery
  • Medical Genetics

Background:

  • Prune Belly Syndrome (PBS) presents with diverse phenotypic severity, complicating accurate assessment and classification.
  • Existing scoring systems may lack comprehensive phenotyping across all age groups.

Purpose of the Study:

  • To develop and validate a novel, pragmatic scoring system, RUBACE (Renal, Ureter, Bladder/Outlet, Abdominal wall, Cryptorchidism, Extra-genitourinary), for assessing PBS phenotypic severity.
  • To apply the RUBACE system to a pilot cohort and compare its efficacy with the established Woodard criteria.

Main Methods:

  • The RUBACE scoring system was designed based on six organ/system sub-scores (0-31 total).
  • A prospective cohort of 50 PBS patients (2000-2017) was analyzed cross-sectionally.
  • RUBACE scores were calculated, and patients were categorized into isolated PBS, syndromic PBS, and PBS-plus variants. Scores were compared to Woodard categories.

Main Results:

  • The pilot cohort included 48 males and 2 females with a mean RUBACE score of 13.8.
  • The cohort comprised 76% isolated PBS, 12% syndromic PBS, and 10% PBS-plus cases.
  • Significant differences in mean RUBACE scores were observed across Woodard categories (P < 0.001).

Conclusions:

  • The RUBACE system provides a practical, organ/system-level tool for grading PBS severity and classifying patient subtypes.
  • This standardized phenotyping approach is expected to facilitate genotype-phenotype correlations.
  • RUBACE is poised to support future multicenter studies on PBS treatment outcomes.
Abstract

Keywords:
Prunebelly syndromecryptorchidismmegacystisseverity score

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