Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group.

Jang Hee Han,Mi-Jung Lee,Sang Won Han,Yong Seung Lee,Young Jae Im,Sang Woon Kim,Samuel Kim

doi:10.1371/journal.pone.0166776

Jang Hee Han, Mi-Jung Lee + Show 5 more

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https://doi.org/10.1371/journal.pone.0166776

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Journal: PloS one	Publication Date: Nov 18, 2016
Citations: 52	License type: CC BY 4.0

Affiliation: Yonsei University, Seoul National University

Abstract

PurposeObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by Müllerian duct and renal anomalies. It is usually regarded as a disease of adolescence; however, due to a number of possible problems, the management of patients before puberty should not be overlooked. We assessed the clinical course of prepubertal patients to propose appropriate management.Materials and MethodsWe retrospectively assessed 43 prepubertal OHVIRA syndrome patients who were diagnosed and followed up at our institution from July 2004 to June 2015. We reviewed medical records, focusing on presentation, radiologic findings, surgical management, and the overall clinical course.ResultsMedian age at diagnosis was 1.3 months and median follow-up period was 25.5 months. The most common accompanying ipsilateral urologic anomalies were ectopic ureter and ureterocele, while the most common contralateral anomaly was vesicoureteral reflux. During the follow-up period, six patients (14.0%) required surgery at a median age of 31.2 months due to recurrent urinary tract infection, uncontrolled vaginal distention compressing adjacent organs, urinary incontinence, or intractable abdominal pain.ConclusionsWhile OHVIRA syndrome is known as a postpubertal disease, about 13% of prepubertal patients in our study required surgery. When ectopic ureter insertion into the vagina is present, further treatment may be needed to address the complications caused by continuous urine production. Patients should be monitored for complications arising from either obstructed hemivagina or renal anomalies with regular follow-up, especially before the age of five years.

Highlights

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, known as Herlyn-Werner-Wunderlich syndrome, was first reported by Purslow in 1922 [1]
During the follow-up period, six patients (14.0%) required surgery at a median age of 31.2 months due to recurrent urinary tract infection, uncontrolled vaginal distention compressing adjacent organs, urinary incontinence, or intractable abdominal pain
While OHVIRA syndrome is known as a postpubertal disease, about 13% of prepubertal patients in our study required surgery

Summary

Introduction

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, known as Herlyn-Werner-Wunderlich syndrome, was first reported by Purslow in 1922 [1]. Abnormal development of the Wolffian ducts leads to the unilateral renal agenesis and imperforated hemivagina associated with OHVIRA syndrome [2]. This syndrome has been widely recognized due to increasing familiarity with the disorder and improvement in diagnosis [3]. Symptoms occur less frequently in prepubertal patients with this syndrome. Distended hemivagina presenting as a protruding vaginal mass occurs rarely in neonatal patients and regresses after cessation of exposure to maternal estrogen after birth [5,6]. Due to its rare symptomatic presentation, little is known about OHVIRA in prepubertal group

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