[Uterus duplex with hematometrocolpos and ipsilateral agenesis of the kidney]

Malformations of the mullerian-duct-system occur with an incidence of about 0.001-0.005% of the population, and they are frequently associated with unilateral agenesis of the kidney.

A 12-year-old girl presented to our emergency department with cyclic abdominal pain which increased with each of her menses. Ultrasound investigation showed a uterus duplex with obstructed hemivagina and hematometrocolpos as well as an ipsilateral absence of the kidney. Additionally, a cystic expansion in the small pelvis was detected which required further investigations (MRT and MR-angiography) to establish the diagnosis of a hematosalpinx. Excision of the vaginal septum resulted in drainage of the hemato-metrocolpos and the hematosalpinx.

Unilateral hematometrocolpos should be considered as an important differential diagnosis in female adolescents with relapsing lower abdominal pain, even if normal menstruation occurs.