Unusual Presentation of Fusiform Megalourethra and Anterior Urethral Valves as a Transient Fetal Cystic Perineal Mass
Lauren E. Corona* and John M. Park
University of Michigan, Department of Urology, Ann Arbor, Michigan
A 28 year old G1P1 was referred with a 22 week 46 XY fetus showing a perineal midline fluid filled structure, confirmed by ultrasound and MRI. Moderately dilated right pelvis was the only other abnormality. A follow up ultrasound repeated at 25 weeks showed resolution of this cystic structure and hydronephrosis.
Male infant was born at 41 weeks. Neonatal exam findings included bilateral non-palpable intraabdominal testes (confirmed by ultrasound), penoscrotal transposition, large phallus with dorsal curvature, ventral subcoronal urethrocutaneous fistula (along with patent orthotopic urethral meatus), partially formed prepuce, and imperforate anus. The newborn ultrasound showed an unremarkable bladder and mild right hydronephrosis. He underwent colostomy and mucous fistula creation on day of life (DOL) 1. He subsequently stopped voiding, and ultrasound on DOL 2 showed severe bilateral hydronephrosis with marked bladder distension. Unsuccessful attempts at catheterization led to bedside suprapubic tube (SPT) placement. Voiding cystourethrogram performed via SPT showed right renal duplication, grade 4 lower pole reflux, non-trabeculated large capacity bladder and obstruction in the anterior urethra.
Diagnostic cystoscopy at 3 weeks of age revealed a massively dilated anterior urethra consistent with fusiform megalourethra and 3-French caliber anterior urethral valve near the bulbar urethral region. Successful direct vision internal urethrotomy (DVIU) was performed 1 week later. Thereafter, the patient voided normally per urethra. He was placed on prophylactic antibiotics for right lower pole reflux. He awaits urethral and phallic reconstruction along with orchidopexy
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