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A Rare Case Congenital Megalourethra in Prune Belly Syndrome

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by

Vishnu
in

Introduction

Prune Belly Syndrome (PBS) is a rare congenital disorder affecting approximately 1 in 40,000 male births. It is characterized by a triad of symptoms: abdominal muscle deficiency, undescended testicles, and urinary tract abnormalities. In this case study, we explore an unusual presentation of PBS associated with congenital megalourethra.

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Case Overview

A male neonate born at 38 weeks with a birth weight of 2500g was diagnosed with bilateral hydronephrosis. Physical examination revealed undescended testicles and an enlarged penis. Diagnostic imaging and cystourethroscopy confirmed the presence of a large anterior urethral diverticulum, leading to a diagnosis of PBS.

Key Findings:

  • Bilateral hydronephrosis with renal function impairment
  • Presence of a large diverticulum in the anterior urethra
  • Urosepsis requiring multiple hospitalizations
  • Improvement following vesicostomy and urethrostomy procedures

Clinical Significance

PBS can lead to life-threatening complications, including renal insufficiency and pulmonary hypoplasia. Early diagnosis via prenatal and postnatal ultrasound is essential.

Treatment Approaches:

  • Vesicostomy: Relieves urinary obstruction and prevents renal damage.
  • Cutaneous Urethrostomy: Enhances urinary drainage in cases of severe urethral obstruction.
  • Dorsal Slit Technique: Helps alleviate phimosis, reducing infection risks.

Broader Implications in Urology

The American Urological Association (AUA) highlights the importance of early intervention in congenital urological anomalies to minimize long-term morbidity. Advances in fetal imaging and neonatal surgical techniques have improved outcomes for PBS patients.

Access Full Study

Read the full study at https://doi.com/10.29328/journal.acr.1001005.

For more case studies on congenital urogenital anomalies, explore our case report archives.

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