Herlyn-Werner-Wunderlich syndrome, also known as OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) is an extremely rare syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs. It is a type of abnormality of the Müllerian ducts. In most cases, OHVIRA presents as a double uterus with unilateral obstructed (or blind) hemivagina and ipsilateral

renal agenesis Renal agenesis is a medical condition in which one (unilateral) or both (bilateral) fetal kidneys fail to develop. Unilateral and bilateral renal agenesis in humans, mice and zebra fish has been linked to mutations in the gene GREB1L. It has also ...

. It can also affect the urethra, urethral sphincter, ureters, bladder and spleen. Although the true incidence is unknown, it has been reported to be between 0.1% and 3%.

Symptoms

Although there are no specific symptoms for this condition, common complains include progressively increasing pelvic pain during menstruation and hematocolpos due to the buildup of blood in the body. Other symptoms may include swelling of the abdomen, nausea and vomiting during menstruation, and pelvic pain. Fertility may also be affected. The condition often leads to an increased need for C-sections due to the smaller uteruses.

Diagnosis

The condition is often diagnosed through an

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...

ultrasound Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies ...

. Consulting a specialist (in this case a

gynecologist Gynaecology or gynecology (see spelling differences) is the area of medicine that involves the treatment of women's diseases, especially those of the reproductive organs. It is often paired with the field of obstetrics, forming the combined ...

) is recommended.

Treatment

Symptoms can be ameliorated with medication and surgery. In most cases, the blind hemi-vagina is opened, and the fluid drained. In adolescents particularly, vaginoscopic incision of the oblique vaginal septum is a viable option. Pregnancies in women with OHVIRA are categorized as high-risk due to the size and shape of the uteri and cervices as well as the reduced kidney function. In addition, women with undiagnosed OHVIRA can experience genital bleeding during pregnancy and may require hospitalization.{{Cite journal, last1=Muraoka, first1=Ayako, last2=Tsuda, first2=Hiroyuki, last3=Kotani, first3=Tomomi, last4=Kikkawa, first4=Fumitaka, date=May 2016, title=Severe Hemoperitoneum During Pregnancy with Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report, url=https://pubmed.ncbi.nlm.nih.gov/27424375/, journal=The Journal of Reproductive Medicine, volume=61, issue=5–6, pages=290–294, issn=0024-7758, pmid=27424375 Expectant mothers are often managed with cervical sutures and C-sections to prevent fetal distress during labour.

References

Gynaecologic disorders Uterus