Herlyn-Werner-Wunderlich Syndrome: A Rare Case Report

Müllerian anomalies are a complex spectrum of congenital defects of the female reproductive tract caused by an interruption in the normal development of Müllerian ducts and their associated structures. The clinical presentation of these anomalies varies and includes acute presentations. In this report, a case of a 16-year-old girl who attended the emergency department with severe colicky abdominal pain and nausea has been presented. The patient was on the fifth day of the menstrual cycle, and her pain was not completely relieved by the administration of non-steroidal anti-inflammatory drugs. The patient had menarche at the age of 14 years and used to have a regular menstrual cycle every 30 days. Her menstrual flow was average with a cycle duration of five days. An abdominal ultrasound examination was performed to rule out ovarian torsion, which demonstrated normal appearance and ovary size, and a large heterogeneous collection in the rectouterine pouch with unclear uterine morphology. Subsequently, magnetic resonance imaging revealed a duplication of the uterus, cervix, and vagina. The right hemivagina and right endometrial cavity were distended, consistent with hematometrocolpos. Right renal agenesis with compensatory hypertrophy of the left kidney was observed. The constellation of uterus didelphys obstructed hemivagina, and ipsilateral renal agenesis represented the diagnosis of Herlyn-Werner-Wunderlich syndrome. Herlyn-Werner-Wunderlich syndrome is a rare and complex type of congenital uterine abnormality. Although rare, this syndrome should be considered in the differential diagnosis of severe dysmenorrhea in adolescent girls with renal anomalies. In this case, the patient underwent vaginoplasty with resection of the vaginal septum to relieve the obstruction. At follow-up visits, she did not have a recurrence of symptoms.