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

Abstract

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.

Keywords: MRI; OHVIRA; hematosalpinx; hysteroscopy; laparoscopy; pyocolpos; ultrasound.

Figure 1

— MRI images showing the hematometra along with the hematocolpos

Figure 2

— Laparoscopic image showing the hematocolpos, dilated left horn, and left hematosalpinx.

Figure 3

— MRI image showing the bicornuate uterus and the hematocolpos

Figure 4

— Laparoscopic image showing a symmetrical bicornuate uterus.

Figure 5

— Drainage of the pyocolpos.

Figure 6

— Vaginoplasty and marsupialization of the vaginal edges.