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. Radiography, ultrasonography, and computed tomography revealed uterine didelphys, obstructed hemivagina, a cystic structure around the right uterus, and right renal agenesis, leading to the diagnosis of OHVIRA syndrome. An ovariohysterectomy and decompression of the obstructed right hemivagina were performed, and the cystic structure near the right uterine horn was removed by resection, along with the right uterus and ovary. After the surgical intervention, the patient's symptoms including abdominal pain, anorexia, and depression were immediately resolved. The patient was followed up for 1 month postoperatively with ultrasonography at 2-week intervals, which revealed the progression of mild fluid retention in the right hemivagina. However, no additional urogenital findings were identified, and the patient continued to exhibit no overt clinical symptoms. This case report describes the diagnosis and surgical treatment of the first documented case of OHVIRA syndrome in an animal. Unlike in human medicine, where vaginal septectomy is performed to prevent dilatation of the obstructed hemivagina and thereby resolve clinical symptoms, performing ovariohysterectomy combined with fluid aspiration from the obstructed hemivagina showed a favorable postoperative prognosis in the dog.
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http://dx.doi.org/10.3389/fvets.2024.1488107 | DOI Listing |
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 PDFMedicina (Kaunas)
November 2024
Department of Anatomy and Embryology, "Iuliu Hatieganu" University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania.
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 PDFJ Clin Med
December 2024
Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany.
: 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 PDFPediatrics
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 PDFBackground: Complete bicorporeal uterus, double cervix and obstructive longitudinal vaginal septum (classified as U3bC2V2 according to ESHRE/ESGE classification) is a rare congenital anomaly of the genital tract. This condition is typically associated with ipsilateral renal agenesis and is known as Herlyn-Werner-Wunderlich syndrome or OHVIRA (Obstructed HemiVagina and Ipsilateral Renal Anomaly) syndrome. The primary symptoms include dysmenorrhea and pelvic pain, which usually manifest after menarche due to haematocolpos in the obstructed hemivagina.
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