CASE PRESENTATION: We report a case of a patient with unsuspected müllerian duct anomaly in a term pregnancy. A 33-year-old Malay woman with previously uninvestigated involuntary primary infertility for 4 years presented with acute right pyelonephritis in labor at 38 weeks of gestation. She has had multiple congenital anomalies since birth and had undergone numerous surgeries during childhood. Her range of congenital defects included hydrocephalus, for which she was put on a ventriculoperitoneal shunt; imperforated anus; and tracheoesophageal fistula with a history of multiples surgeries. In addition, she had a shorter right lower limb length with limping gait. Her physical examination revealed a transverse scar at the right hypochondrium and multiple scars at the posterior thoracic region, levels T10-T12. Abdominal palpation revealed a term size uterus that was deviated to the left, with a singleton fetus in a nonengaged cephalic presentation. The cervical os was closed, but stricture bands were present on the vagina from the upper third until the fornices posteriorly. She also had multiple rectal prolapses and strictures over the rectum due to previous anorectoplasty. An emergency cesarean delivery was performed in view of the history of anorectoplasty, vaginal stricture, and infertility. Intraoperative findings showed a left unicornuate uterus with a communicating right rudimentary horn.
CONCLUSION: Most cases of müllerian duct anomaly remain undiagnosed due to the lack of clinical suspicion and the absence of pathognomonic clinical and radiological characteristics. Because it is associated with a wide range of gynecological and obstetric complications, it is vital for healthcare providers to be aware of its existence and the role of antenatal radiological investigations in its diagnosis. The presence of multiple congenital abnormalities and a history of infertility in a pregnant woman should warrant the exclusion of müllerian duct anomalies from the beginning. Early detection of müllerian duct anomalies can facilitate an appropriate delivery plan and improve the general obstetric outcome.