OHVIRA syndrome

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Classification according to ICD-10
Q51.2 Other forms of the uterus duplex
ICD-10 online (WHO version 2019)

The OHVIRA syndrome , acronym for O bstruierte H emi V agina and I psilaterale R enale anomaly , is a very rare malformation syndrome with the main features of a twin system of the uterus , vagina and renal .

Synonyms are: uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis; English Double uterus-hemivagina-renal agenesis syndrome; Double uterus and obstructed hemivagina syndrome; Herlyn-Werner syndrome; Obstructed hemivagina and ipsilateral renal anomaly; Wunderlich syndrome

Not to be confused is the Wunderlich syndrome (common in English-speaking countries) for spontaneous, nontraumatic kidney bleeding.

distribution

The frequency is given as less than 1 in 1,000,000, the inheritance mechanism is not known.

root cause

This malformation syndrome is based on a disturbance in the embryonic development in the development of the Müllerian and Wolffian ducts.

Clinical manifestations

Clinical criteria are:

diagnosis

The suspected diagnosis is usually made by sonography , the diagnosis is confirmed by laparoscopy . Possible complications of obstruction of the hemivagina are endometriosis , adhesions , hematometra , hematosalpinx , ascending inflammation .

treatment

To surgical procedures which are resection of Vaginalseptums and the hemi hysterectomy available.

history

The first description comes from 1976 by the gynecologist Alvin F. Wiersma and colleagues.

literature

  • A. Mandava, RR Prabhakar, S. Smitha: OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. In: Journal of pediatric and adolescent gynecology , Volume 25, No. 2, April 2012, pp. E23 – e25, doi: 10.1016 / j.jpag.2011.11.004 , PMID 22421561 .
  • Hansa Dhar, Yasser A. Razek, Ilham Hamdi: Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report. In: Oman Medical Journal , Volume 26, 2011, p. 447, doi: 10.5001 / omj.2011.114 .
  • Boram Han, Christopher N. Herndon, et al. a .: Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. In: Radiology Case Reports , Volume 5, 2010, p. 327, doi: 10.2484 / rcr.v5i1.327 .

Web links

Individual evidence

  1. a b c d Uterine doubling - hemivagina - renal agenesis. In: Orphanet (Rare Disease Database).
  2. AF Wiersma, LF Peterson, EJ Justema: Uterine anomalies associated with unilateral renal agenesis. In: Obstetrics and gynecology , Vol. 47, No. 6, June 1976, pp. 654-657, PMID 934555 .