Herlyn-Werner-Wunderlich syndrome presented with amenorrhea: A rare urogenital anomaly in females

A 13-year-old prepubertal girl had with primary amenorrhea and severe lower abdominal pain. Pelvic ultrasound showed double uterine cavity and a fluid collection in left uterine cavity with diffuse low level internal echoes, which appeared contiguous with the endocervix. Hymen could not be evaluated because the patient could not be taken to the gynecological table the patient was initially diagnosed as imperforate hymen, gynecologic examination was performed with sedation, and hymen has a normal patency. Magnetic resonance imaging (MRI) revealed two uterus and two cervix, normal right endometrial cavity and 17 mm fluid in the left endometrial cavity and compatible with hematometra. Hematocolpos was observed in the left half of the vagina. There was no left kidney and the right kidney was normal. Based on these findings, the patient was diagnosed as Herlyn-Werner-Wunderlich syndrome and vaginal septum resection was performed as treatment. Evaluation of the genital tract by means of MRI scanning or ultrasonography should be recommended in all girls with known renal abnormalities before the onset of menstruation. Key Words: Herlyn-Werner-Wunderlich syndrome, amenorrhea, renal agenesis, hematometra, uterus didelphis.