Double uterus-hemivagina-renal agenesis

Double Uterus-Hemivagina-Renal Agenesis (DUHRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome (HWWS), is a rare congenital anomaly characterized by a triad of reproductive and urinary tract malformations. This condition involves the presence of a double uterus, a hemivagina (half of the vagina is obstructed), and renal agenesis (absence of one kidney). DUHRA syndrome is a result of abnormal development of the Müllerian ducts and the mesonephric ducts during fetal development.

Symptoms and Diagnosis

Individuals with DUHRA syndrome may not exhibit symptoms until puberty, when menstrual irregularities, such as dysmenorrhea (painful menstruation) and menstrual cramps, become apparent due to the accumulation of menstrual blood in the obstructed hemivagina. This can also lead to endometriosis and infections. The presence of a double uterus may not cause symptoms on its own and is often discovered during investigations for other conditions or during routine gynecological examinations.

Diagnosis of DUHRA syndrome typically involves a combination of ultrasound, MRI (Magnetic Resonance Imaging), and sometimes laparoscopy. These imaging techniques help in visualizing the double uterus, the obstructed hemivagina, and the absence of one kidney.

Treatment

Treatment for DUHRA syndrome is usually surgical and aims to relieve symptoms and prevent complications. The surgical approach may involve creating an opening in the obstructed hemivagina to allow menstrual blood to flow out freely. In cases where there is significant discomfort or risk of recurrent infections, more extensive surgical interventions may be considered.

Etiology

The exact cause of DUHRA syndrome is unknown, but it is believed to be related to disruptions in the development of the Müllerian ducts and mesonephric ducts during the early stages of fetal development. Genetic factors may play a role, but specific genetic mutations associated with the syndrome have not been identified.

Epidemiology

DUHRA syndrome is extremely rare, with only a few hundred cases reported in the medical literature. It affects females exclusively, given its involvement of the female reproductive system.

Prognosis

With appropriate surgical treatment, individuals with DUHRA syndrome can lead normal lives, although they may have an increased risk of infertility and recurrent urinary tract infections. Regular follow-up with a healthcare provider specializing in reproductive health is recommended to manage and monitor potential complications.