Obstructed hemivagina and ipsilateral renal agenesis/anomaly (OHVIRA) syndrome is a very rare condition affecting girls. The time of diagnosis varies, from cases of prenatal diagnosis up to adulthood, including pregnancy or delivery. Most commonly, it is recognised during puberty and usually as an acute condition. We present a case report of an adolescent girl who underwent the treatment because of acute abdominal pain. The case is interesting due to a previous diagnosis of one-side renal agenesis. It appears to be useful to perform a diagnostic pelvic imaging at the time of diagnosis of renal agenesis or to plan to perform it at the beginning of puberty, to prevent the need for urgent surgery. This management may allow the planning of proper follow-up, minimising the risk of possible complications.
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http://dx.doi.org/10.3390/jcm12237284 | 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 PDFTaiwan J Obstet Gynecol
January 2025
Department of Obstetrics and Gynecology, Taipei Chang Gung Memorial Hospital, Taipei, Taiwan; School of Medicine, Chang Gung University, Taoyuan, Taiwan. Electronic address:
Objective: To describe a rare case of a retroperitoneal Müllerian cyst in a teenage girl with a protruding uterus and associated urogenital anomalies, and to discuss the challenges faced in differential diagnosis and management of such cases.
Case Report: We present the case of a 14-year-old girl presented with a protruding uterus for several weeks, with a history of twin-twin transfusion syndrome at birth. Initial ultrasonography identified a large pelvic cystic tumor.
J 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 PDFCureus
December 2024
Radiodiagnosis, MNR Medical College and Hospital, Sangareddy, IND.
Zinner syndrome is an extremely uncommon congenital anomaly of the male urogenital tract. It is attributed to an embryological anomaly that arises in the distal segment of the mesonephric or Wolffian duct. It is the inadequate migration of the ureteric bud that contributes to the failure of differentiation of the metanephric blastema, which ultimately results in ipsilateral renal agenesis and atresia of the ejaculatory duct.
View Article and Find Full Text PDFJ Med Genet
December 2024
John T. Macdonald Foundation Department of Human Genetics, University of Miami Miller School of Medicine, Miami, Florida, USA
Introduction: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder classically associated with multiple basal cell carcinomas, odontogenic keratocysts and skeletal anomalies. However, its significant phenotypic heterogeneity often delays the diagnosis. Here, we undertake the first comprehensive characterisation of NBCCS and congenital urinary tract anomalies.
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