Staged male urethroplasty transferring megalourethra tissue as free graft dorsal inlay to proximal urethral atresia in VACTERL association

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Abstract

Megalourethra is a rare spectrum of urologic malformations of penile corporal structures frequently associated with multiple congenital anomalies, such as prune belly syndrome or vertebral, anorectal, cardiac, trachea-esophageal, renal, and limb (VACTERL association) defects. A 6-year-old boy with VACTERL association and proximal urethral atresia with distal fusiform megalourethra underwent staged reconstruction, including appendicovesicostomy, perineal urethrostomy, and first-stage urethroplasty with a dorsal inlay free graft of megalourethra tissue to the proximal urethral atretic region, followed by second-stage urethroplasty. At 2.6 years of follow-up, he was continent, voids per urethra without postvoid residual urine volume, and no longer performs clean intermittent catheterization by way of the appendicovesicostomy.

Original languageEnglish (US)
Pages (from-to)1417-1419
Number of pages3
JournalUrology
Volume78
Issue number6
DOIs
StatePublished - Dec 1 2011

ASJC Scopus subject areas

  • Urology

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