Septate uterus with cervical duplication and longitudinal vaginal septum: a report of five new cases

OBJECTIVE: To describe a unique congenital müllerian anomaly. DESIGN: Case report. SETTING: A university-based reproductive endocrine center. PATIENT(S): Five reproductive-age, nulligravida patients who underwent clinical, radiologic, and surgical work-up. INTERVENTION(S): Retrospective review of prior medical records and studies. MAIN OUTCOME MEASURE(S): Definition of abnormal pelvic anatomy. RESULT(S): Five patients from a university-based, reproductive endocrine center were found to have cervical duplication with a longitudinal vaginal septum, uterine septum, and a normal fundus. The patients most often presented initially to their primary obstetrician-gynecologists with symptomatic complaints secondary to their vaginal septums. Diagnoses were obtained with physical examinations, ultrasound imaging, hysterosalpingograms, magnetic resonance imaging, and surgical evaluation. CONCLUSION(S): These findings call into question the classic hypothesis of unidirectional (caudal to cranial) müllerian development and supports an alternative embryologic hypothesis of Müller et al., which states that fusion and resorption begins at the isthmus and proceeds simultaneously in both the cranial and caudad directions. The high number of cases reported here might be due to the increased accessibility and accuracy of such imaging modalities as magnetic resonance imaging. These patients will be followed longitudinally so that it can be determined whether this anomaly affects fertility and so that the optimal treatment plans can be developed.