Temporary Perineal Urethrostomy for External Sphincter Dilation in a Male Patient With High Risk Myelomeningocele

Abstract

We describe our use of perineal urethrostomy to facilitate serial external sphincter dilations in a male child with high risk myelomeningocele. A 5-month-old boy with lumbar myelomeningocele presented with poor bladder compliance, increased detrusor leak point pressure and high grade vesicoureteral reflux. Urodynamic parameters failed to improve with clean intermittent catheterization and anticholinergic therapy. Given the persistent risk to the upper urinary tract, perineal urethrostomy was performed to facilitate external sphincter dilation in a serial fashion. The patient underwent 2 aggressive external sphincter dilations (30Fr) under direct vision. The second dilation was performed in the outpatient clinic without anesthesia. Subsequent cystometrographic evaluation revealed dramatic improvement in bladder storage properties with improved compliance and a safe detrusor leak point pressure. Moreover, followup imaging demonstrated complete resolution of vesicoureteral reflux. Creation and maintenance of perineal urethrostomy is a valuable innovation in the treatment of some boys with high risk myelomeningocele. This easily reversible diversion allows aggressive, sequential external sphincter dilation and produces substantial improvements in bladder storage properties.