(91) OHVIRA with Ectopic Ureter Implanted into the Obstructed Hemivagina: Case Series

Background: OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) most commonly presents in adolescence with pain due to development of hematocolpos after menarche, and is definitively managed with surgical resection of the obstructing hemivaginal septum. When identified at prepubertal ages, OHVIRA is rarely symptomatic and generally surgical management may be deferred. We present two cases of prepubertal, symptomatic OHVIRA in an infant and a two year old who both had hydrocolpos from an ectopic ureter draining into the obstructed hemivagina. Due to persistent symptoms, both cases required surgical management.

Case: Case 1 was a two week old neonate who underwent a diagnostic laparoscopy and decompression of hydrocolpos causing contralateral hydronephrosis. After rapid reaccumulation of hydrocolpos, MRI demonstrated a right-sided ectopic ureter implanting into the obstructed right hemivagina. A Harrison fetal bladder stent was then placed vaginoscopically at three weeks of life to drain the hydrocolpos and removed at five months.
Case 2 presented at two years old with intermittent episodes of bloody vaginal discharge. MRI showed hydrocolpos and a remnant right ureter emptying into the right hemivagina, although no clear renal parenchyma was seen on the right side. Due to the distal location of her vaginal septum, the vaginal septum was able to be resected with resolution of obstruction.

Comments: Surgical management of OHVIRA is traditionally delayed until puberty to reduce risk of restenosis. Implantation of an ectopic ureter into the obstructed hemivagina may necessitate earlier intervention due to development of urocolpos if there is functional renal tissue present. These cases describe two different surgical management approaches applied in individualized ways to address patients’ different presentations and anatomy. We also describe a novel use of a fetal bladder stent used successfully as a vaginal stent in an infant.