Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex of structural abnormalities of the female urogenital tract. A 17-year-old girl with uterine didelphys associated with OHVIRA syndrome presented with progressive development of cyclic lower abdominal discomfort and a large abdominopelvic mass. We describe the findings from ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), the first case report of this syndrome to examine all three different imaging modalities in a single patient. We also review the literature on OHVIRA syndrome and discuss important considerations relevant to radiologists and other clinicians.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4898179PMC
http://dx.doi.org/10.2484/rcr.v5i1.327DOI Listing

Publication Analysis

Top Keywords

ohvira syndrome
16
uterine didelphys
8
didelphys associated
8
obstructed hemivagina
8
hemivagina ipsilateral
8
ipsilateral renal
8
renal anomaly
8
anomaly ohvira
8
syndrome
5
associated obstructed
4

Similar Publications

Diagnosis and surgical treatment of obstructed hemivagina and ipsilateral renal anomaly in a dog: a case report.

Front Vet Sci

December 2024

Department of Veterinary Surgery, College of Veterinary Medicine, Konkuk University, Seoul, Republic of Korea.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), also called Herlyn-Werner-Wunderlich syndrome, is an extremely rare Müllerian duct anomaly accompanied by Wolffian duct anomalies. A 10-year-old intact female Yorkshire Terrier weighing 3.35 kg was presented with anorexia, depression, vomiting, and abdominal pain.

View Article and Find Full Text PDF

Müllerian (paramesonephric) duct anomalies (MDA) are a rare condition, occurring in 5.5% of female newborns. One of the most complex malformations is represented by Obstructed Hemivagina and Ipsilateral Renal Anomalies (OHVIRA) syndrome, also known as Herlyn -Werner-Wunderlich (HWW) syndrome.

View Article and Find Full Text PDF

: Endometriosis and urogenital malformation with uterus didelphys and renal agenesis might occur concomitantly, and the question arises whether both entities are associated with each other. : A literature search was conducted in PubMed and Web of Science, using the following search terms: "endometriosis and uterine malformation, endometriosis and Herlyn-Werner-Wunderlich syndrome", "endometriosis and OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome" and "uterus didelphys, renal agenesis and endometriosis". : We identified and examined 36 studies, comprising a total of 563 cases with coinciding endometriosis and OHVIRA.

View Article and Find Full Text PDF

CRKL Gene Deletion in Familial Zinner (OSVIRA) and OHVIRA Syndromes.

Pediatrics

January 2025

Department of Paediatric Surgery & Urology, Bristol Royal Hospital for Children, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, United Kingdom.

We present the first description of a family in which 2 siblings show alternative expression of CRKL gene deletion as the phenotypes of Zinner (OSVIRA, obstructed seminal vesicle and ipsilateral renal agenesis) and OHVIRA (obstructed hemivagina with an ipsilateral renal anomaly) syndromes. The male infant with Zinner syndrome and his sister aged 5 years with OHVIRA syndrome both have a paternally inherited 703-kb deletion at chromosome 22q11.21 that includes CRKL.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!