V7-14: Robot-Assisted Laparoscopic Nephroureterectomy in a 9-year-old Girl with Obstructed Hemivagina Ipisilateral Renal Anomaly

Video

Introductions and Objectives
Obstructed hemivagina ipsilateral renal anomaly (OHVIRA) is a rare Mullerian duct abnormality with renal manifestations (renal agenesis, hypoplasia, ectopic ureter, multicystic dysplastic kidney). Classically, this presents after menarche with abdominal pain, but because of its rarity, diagnosis is often delayed. There have been less than 20 reported cases of prepubertal OHVIRA, with most involving neonates. In the infant female, diagnosis can sometimes be made with unilateral renal agenesis on prenatal ultrasound and vaginal bulge after birth. Rarely do girls present between the newborn and pubertal period. There are no definitive guidelines for treatment of prepubertal girls with OHVIRA.

Methods
We present a case of OHVIRA diagnosis and management in a 9-year-old girl, which came to light via continuous incontinence that developed after incorrect diagnosis and treatment for imperforate hymen.

Results
A 9-year-old female presented to an outside emergency room with abdominal pain. Abdominal ultrasound showed hydrocolpos and an absent left kidney. The working diagnosis was imperforate hymen with blockage of menses and she underwent “hymenotomy”. Since this procedure, she has had continuous vaginal leakage. Genitourinary exam showed Tanner 1 genitalia, pooling of fluid within the vagina, a circumferentially intact hymenal ring and a deeper area of tissue within the vagina that had previously been incised. There were no abdominal masses and the urethra was normal. Cross sectional imaging revealed uterus didelphys, a normal right kidney and a small left pelvic kidney that took up and excreted contrast into a hydronephrotic left ureter. We took the patient to the operating room with a presumptive diagnosis of OHVIRA with a left atrophic kidney with a presumed ectopic ureter into the left hemivagina that had been incised. We explored the pelvis robotically and identified the dilated left ureter within the pelvis with insertion into the left vagina. Proximally, there was an atrophic pelvic kidney with a series on anomalous vessels. We performed a left nephroureterectomy. The distal ureter was amputated at its entry point into the vagina. The patient recovered uneventfully and went home on post-operative day 1. She was immediately dry after the procedure.

Conclusions
OHVIRA is a rare diagnosis that requires a high index of suspicion. When presented with a prepubertal patient with renal abnormalities and a pelvic mass careful work up is important prior to surgical intervention to ensure accurate diagnosis and treatment in the same setting.

Funding: None