A Report of Laurence Moon Biedl Syndrome with Urogenital Sinus Abnormality

Laurence-Moon-Biedl syndrome is one of the rare disorders. Herein we report a case of Laurence-Moon-Biedl syndrome with urogenital sinus anomaly.Case Report: The patient was a 17 years old girl with colicky and periodic abdominal pain since 4 months ago who was referred to the gynecology ward of Rasoul Akram hospital affiliated to Iran University of Medical Sciences. In the physical examination we found an abdomino-pelvic mass at the umbilical region. In examination of the genitalia, the vestibule was not seen and no orifice was found between the urethra and the anus.During the laparotomy we found the mass was a huge vagina full of old blood and pyometra, which were drained. The uterus was enlarged but the fallopian tubes and the ovaries were normal. An incision was performed in the perineum, between the urethra and the anus. Then a petzer drain was inserted from the inside of the uterus to the incision.After operation, the patient was treated with conjugated estrogen 0.625 mg/day for 25 days and medroxy progesterone acetate 10 mg/day for 10 days. After discontinuing the both drugs she menstruated. After the first menstruation the petzer drain was dislodged.