ABSTRACT
Background: Embryological maldevelopment of the Mullerian or paramesonephric duct results in congenital uterine anomalies. A rare developmental anomaly is a septate uterus with cervical duplication and a longitudinal vaginal septum. This anomaly cannot be explained by the classical unidirectional Mullerian developmental theory and does not fit into American Society for Reproductive Medicine (ASRM) classification of uterine anomalies. The bidirectional Mullerian developmental theory holds good for this type of anomalies and the new ESHRE/ESGE classification for female urogenital tract anomaly (CONUTA) is more suitable for effective categorization, diagnosis, and treatment.Case Report: Two cases of the complete uterine septum with double cervix and longitudinal vaginal septum who presented with dysmenorrhea, dyspareunia, and infertility were diagnosed and managed by hysteroscopy and laparoscopy. The vaginal and the uterine septum were resected. Both the patients had an uneventful post-operative recovery period and were followed up for a period of 1 year. The symptoms such as dysmenorrhea and dyspareunia improved significantly without any effect on the fertility outcome.Conclusion:Very few of such cases are reported in the literature. This rare anomaly is explained by the bidirectional Mullerian developmental theory and the application of the new ESHRE/ESGE classification of the urogenital tract (CONUTA) is more suitable for such anomalies for effective categorization, diagnosis, and treatment.