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

Autor: Kudela G; Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland. Electronic address: gkudela@sum.edu.pl., Wiernik A; Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland., Drosdzol-Cop A; Department of Woman's Health, School of Health Sciences in Katowice, Medical University of Silesia, Katowice, Poland., Machnikowska-Sokołowska M; Department of Diagnostic Imaging, Medical University of Silesia, Katowice, Poland., Gawlik A; Department of Pediatrics and Pediatric Endocrinology, Medical University of Silesia, Katowice, Poland., Hyla-Klekot L; Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland., Gruszczyńska K; Department of Diagnostic Imaging, Medical University of Silesia, Katowice, Poland., Koszutski T; Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland.
Jazyk: angličtina
Zdroj: Journal of pediatric urology [J Pediatr Urol] 2021 Oct; Vol. 17 (5), pp. 653.e1-653.e9. Date of Electronic Publication: 2021 Jun 30.
DOI: 10.1016/j.jpurol.2021.06.023
Abstrakt: 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 Systematic 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 didelphys (82.9%), and ipsilateral renal agenesis (92.2%). Vaginal septectomy was the most common surgical approach (86.5%). Hemivaginectomy (2.2%), hemihysterectomy (4.2%), or total hysterectomy (0.7%) were also performed in several patients. Some subjects required salpingectomy (3.3%) or oophorectomy (1.8%). 7.5% of patients, mainly infants, did not require surgery due to the spontaneous resolution 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 isolated hematocolpos and a thick vaginal septum between 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.
(Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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