Uncommon presentations of an uncommon entity: OHVIRA syndrome with hematosalpinx and pyocolpos


OHVIRA, pyocolpos, hematosalpinx, hysteroscopy, laparoscopy, MRI, ultrasound.

Published online: Feb 16 2018

Z. Sleiman1, T. Zreik1, R. Bitar1, R. Sheaib1, A. Al Bederi2, V. Tanos3

1Lebanese American University, Department of Obstetrics and Gynecology, Beirut, Lebanon, 2Tiba IVF center, Hilla, Irak., 3St. George’s Medical School, Nicosia University, Nicosia, Cyprus


Müllerian malformations result from defective fusion of the Müllerian ducts during development of the female reproductive system. The least common form of these malformations is Herlyn-Werner-Wunderlich syndrome characterized by obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). The most common presentation of this syndrome is a mass secondary to hematocolpos, pain, and dysmenorrhea. Clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan.

We report two cases of this syndrome, featuring two very rare clinical presentations: hematosalpinx and pyocolpos. The clinical course of the pathology is not standard and each patient is treated accordingly.