Herlyn-Werner-Wunderlich Syndrome

Herlyn-Werner-Wunderlich Syndrome

Author Name : Dr. V. Hemavathy, Dr. Sathyalathasarathy, R. Sangeetha

ABSTRACT

HWW syndrome is a very rare congenital anomaly of urogenital tract involving Mullerian ducts and mesonephric ducts. It is characterised by a triad of symptoms - uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It presents soon after menarche or shows delayed presentation depending on the type. It can exhibit acute pelvic pain shortly after menarche and may show non-specific and variable symptoms with resultant delay in diagnosis. The most common presentation is pain and dysmenorrhea, and pain and abdominal mass in the lower abdomen secondary to haematocolpos and/or haematometra.

Awareness is necessary in order to diagnose and treat this disorder properly before complications occur. MRI is the preferred modality for the delineation of uterine malformation. When renal anomalies are encountered, a screening should also be made for congenital abnormalities of the reproductive tract and vice versa.

Key words - Uterus didelphys, Vaginal septum, Renal agenesis, Herlyn-Werner-Wunderlich syndrome