Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome-one clinical center case series and the systematic review of 734 cases

Introduction Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare female urogenital tract malformation. Study objective To present 10 patients with OHVIRA treated at the clinical center. To perform a systematic review of OHVIRA case series related to the prevalence of anatomical variants, surgical interventions and endometriosis, and to compare them with our case series. Materials and methods Medical records from 10 OHVIRA patients treated between 2016 and 2020 were retrospectively reviewed. For the systematic review, PubMed and Web of Science were used to search for relevant studies. The Preferred Reporting Items for System-atic Reviews and Meta-Analyses (PRISMA) guidelines were strictly followed. Results The most common anatomical variant includes left obstructed hemivagina (50.7%) with isolated hematocolpos or hydrocolpos (55.9%), uterus didel-phys (82.9%), and ipsilateral renal agenesis (92.2%). Vaginal septectomy was the most common surgical approach (86.5%). Hemivaginectomy (2.2%), hemi-hysterectomy (4.2%), or total hysterectomy (0.7%) were also performed in several patients. Some sub-jects required salpingectomy (3.3%) or oophorec-tomy (1.8%). 7.5% of patients, mainly infants, did not require surgery due to the spontaneous resolu-tion of hydrocolpos. Endometriosis was fortuitously found in 13.6% of the selected cases who underwent laparoscopy or laparotomy. Discussion The most common variant of OHVIRA includes iso-lated hematocolpos and a thick vaginal septum be-tween adjacent hemivaginas. Endometriosis was present in approximately 14% of OHVIRA patients, but this number is probably underestimated. Routine laparoscopy is not required. However, all patients need further monitoring due to a higher risk of endometriosis. Based on the analyzed studies and our case series, vaginal septectomy is a sufficient surgical technique to relieve symptoms and prevent possible complications in most OHVIRA patients.

Automated summary

OHVIRA syndrome is a rare female urogenital tract malformation, with common anatomical variants including left obstructed hemivagina and ipsilateral renal anomaly. Vaginal septectomy is the most common surgical approach, and approximately 14% of patients have endometriosis.