Fluorescent Ureteral Catheter-Assisted Laparoscopic Nephrectomy for a Pediatric Patient With Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome.

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital disorder characterized by Müllerian duct malformation and ipsilateral renal abnormalities. Variant forms associated with hypoplastic kidneys and ectopic ureteral insertion in the vagina may require nephrectomy because of persistent urinary incontinence. In such cases, urinary drainage through a vaginal opening permits direct ureteral access via vaginoscopy without the size limitations associated with transurethral cystoscopy. Therefore, retrograde catheter placement is technically feasible in patients with OHVIRA syndrome. Consequently, OHVIRA is a particularly suitable indication for ureteral catheterization. Fluorescent ureteral catheters, which were recently introduced in surgery for adults, enable real-time intraoperative visualization of the ureter and may further enhance surgical safety. We report a case involving laparoscopic nephrectomy for OHVIRA syndrome with an ectopic ureter and severely hypoplastic kidney in a pediatric patient. The ectopic ureteral orifice was successfully identified during vaginoscopy when the patient was 5 years of age; therefore, retrograde placement of a fluorescent ureteral catheter was feasible. Intraoperative fluorescence enabled the reliable identification of both the ureter and small renal remnant, thus facilitating safe dissection and resection. This case demonstrates that retrograde placement of a fluorescent ureteral catheter via vaginoscopy is feasible and useful for pediatric patients with OHVIRA syndrome who require nephrectomy. This technique may improve intraoperative ureteral visualization and surgical safety; therefore, it should be considered for selected patients with ectopic ureteral drainage.