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Pseudoexstrophy with epispadias.

J K Mahajan1, Sunita Ojha, K L N Rao

  • 1Department of Pediatric Surgery, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. jkmahajanjk@sify.com

Urologia Internationalis
|May 18, 2005
PubMed
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This study details a rare case of pseudoexstrophy with epispadias, a congenital anomaly. Surgical repair achieved continence, highlighting successful management of this complex condition.

Area of Science:

  • Pediatric Urology
  • Congenital Anomalies
  • Surgical Reconstruction

Background:

  • Exstrophy variants are rare congenital anomalies characterized by external genitalia malformations.
  • These variants share features with classical bladder exstrophy but often have a more intact urinary tract.
  • Pseudoexstrophy is an uncommon exstrophy variant, with rare co-occurrence of epispadias.

Observation:

  • A case of pseudoexstrophy with epispadias is presented.
  • The intact bladder was initially covered by a mucous membrane that subsequently epithelialized.
  • The epispadias was surgically corrected using penile disassembly, repositioning the bladder neck and urethra ventrally and posteriorly.

Findings:

  • The surgical repair resulted in a hypospadiac urethral meatus.

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  • Despite the hypospadias, the child achieved dry intervals.
  • Occasional stress incontinence was noted post-operatively.

    • Implications:

    • This case demonstrates a successful surgical approach for a rare pseudoexstrophy with epispadias.
    • The penile disassembly technique offers a viable option for complex epispadias reconstruction.
    • Management strategies can lead to improved urinary continence in these challenging congenital conditions.