OHVIRA

OHVIRA
Other namesObstructed hemivagina and ipsilateral renal anomaly, Herlyn–Werner–Wunderlich syndrome
Medical specialtyGynecology, medical genetics
SymptomsVaried; abdomen and pelvic pain, irregular menstrual cycles
Usual onsetPrenatal
TreatmentSurgery
Frequency1 in 1,000,000

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA or OHVIRA syndrome), also known as Herlyn–Werner–Wunderlich syndrome, is a very rare genetic disorder. It affects the way females develop their genitalia. People with OHVIRA have two vaginas, two uteruses, and one kidney. Symptoms include pain in the abdomen and pelvis and issues with menstrual cycles or fertility. About one in one million females have OHVIRA and it is usually diagnosed during puberty.

Symptoms

The symptoms of OHVIRA are broad and vary. There is no general criteria for a diagnosis.[1] Most people with the disorder have pains in their abdomen or pelvis. This pain often comes during menstruation. They may have issues with urination and have discharge (liquid substance) coming out of the vagina. Issues with fertility are common.[2] Unusual menstrual cycles, such as bleeding too much or too little, is also common. Many people with OHVIRA have more painful menstrual cycles. Some people may also have vomiting, constipation, or fevers.[3]

People with OHVIRA who get pregnant usually have high-risk pregnancies. They may have bleeding in their genitals and need to be hospitalized.[4]

Diagnosis

Most people with OHVIRA are diagnosed in puberty when symptoms, such as pain with menstrual cycles, begin. An ultrasound or MRI (magnetic resonance imaging) is usually used to look at the internal genitalia (uterus and vagina).[3]

Treatment

Many people with OHVIRA will get a vaginoplasty. This is a surgery that fixes and repairs the vagina.[1] Another type of surgery is laparoscopic hemi hysterectomy. This surgery removes one of the two uteruses. This type of surgery is usually safer and more effective.[5][6]

If someone is diagnosed late and does not get treatment right away, then they may develop problems like infertility, endometriosis, pyometra, or pelvic inflammatory disease.[7]

Prevalence

About one in one million females are estimated to have OHVIRA.[8]

References

  1. ↑ 1.0 1.1 Smith, Nicole A.; Laufer, Marc R. (April 2007). "Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up". Fertility and Sterility. 87: 918–922.
  2. ↑ Malarazhagan, Vaishnavi; Veerasingham, Mayooran; Sivanesan, Kanapathippillai (2020-04-01). "Right-sided obstructed hemivagina ipsilateral renal agenesis (OHVIRA): A case report". Case Reports in Women's Health. 26: e00185. doi:10.1016/j.crwh.2020.e00185. ISSN 2214-9112.
  3. ↑ 3.0 3.1 "Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis - OHVIRA | Texas Children's". www.texaschildrens.org. Retrieved 2025-01-12.
  4. ↑ Muraoka, Ayako; Tsuda, Hiroyuki; Kotani, Tomomi; Kikkawa, Fumitaka (2016). "Severe Hemoperitoneum During Pregnancy with Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report". The Journal of Reproductive Medicine. 61 (5–6): 290–294. ISSN 0024-7758. PMID 27424375.
  5. ↑ Moufawad, Graziella; Ayed, Amal; Sleiman, Zaki (2023-04-11). "Reconsidering the best minimally invasive approach for patients with herlyn-werner-wunderlich syndrome: Should we push the frontiers for a better outcome?". Frontiers in Surgery. 10. ISSN 2296-875X.
  6. ↑ Huseyin, Kiyak; Lale, Turkgeldi Susan; Burak, Yucel; Tolga, Karacan; Pinar, Kadirogullari; Kerem, Seckin Doga (2019-07-01). "A case of obstructed hemivagina and ipsilateral renal anomaly syndrome complicated with pyometra: tips and tricks for laparoscopic hemihysterectomy". Fertility and Sterility. 112 (1): 177–179. doi:10.1016/j.fertnstert.2019.04.016. ISSN 0015-0282.
  7. ↑ Cox, Deven; Ching, Brian (2012-03-17). "Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos". Journal of Radiology Case Reports. 6 (3). doi:10.3941/jrcr.v6i3.877. ISSN 1943-0922. PMC 3370711. PMID 22690286.
  8. ↑ Zaborowska, L. Z.; Tudorache, S.; Ludwin, I.; Ludwin, A. (2023). "EP33.06: Overview of patients with OHVIRA syndrome: diagnosis delay, misdiagnoses and errors in management". Ultrasound in Obstetrics & Gynecology. 62 (S1): 308–309. doi:10.1002/uog.27236. ISSN 1469-0705.