Effmann Type IIA2 Urethral Duplication with Anorectal Malformation Complicated by Posterior Urethral Diverticulum: A Staged Minimally Invasive Approach.

introductionUrethral duplication associated with anorectal malformation (ARM) is rare and can make both diagnosis and surgical reconstruction difficult. CASE PRESENTATION: A male infant was born at 31 weeks' gestation, weighing 1362 g, with multiple congenital anomalies, including duodenal atresia, right renal agenesis, a tethered cord with a terminal filar lipoma, and a ventricular septal defect. He developed neonatal urinary retention; urethral drainage was possible only with a fine intravenous catheter, and when this became obstructed, a percutaneous cystostomy was placed. Contrast studies demonstrated intermediate-type ARM with a rectobulbar urethral fistula, an enlarged prostatic utricle, and complete sagittal urethral duplication (Effmann type IIA2). Distal to the bulbar urethra, the urethra divided into a severely stenotic dorsal channel and a ventral channel that carried voiding but was also stenotic. At 8 months of age, laparoscopic-assisted anorectoplasty (LAARP) was performed with excision of the prostatic utricle; the rectourethral fistula was divided on the rectal side to avoid urethral injury, and the retained remnant formed an acquired posterior urethral diverticulum (PUD). The residual stenosis and the PUD were then managed endoscopically by balloon dilation of the ventral channel and holmium:yttrium-aluminum-garnet laser ablation of the PUD. The dilation restored spontaneous voiding and allowed cystostomy removal at 21 months of age without open urethral reconstruction. At the most recent follow-up (age 28 months), the patient maintains spontaneous voiding with preserved renal function.

conclusionsWe encountered ARM associated with urethral duplication in which both urethral channels were stenotic and caused neonatal urinary retention. Early urinary diversion allowed accurate anatomic assessment, and the functional ventral channel was preserved by keeping the fistula dissection on the rectal side during LAARP. Staged endoscopic management of the residual stenosis and the acquired PUD achieved stable spontaneous voiding without extensive open urethral reconstruction.