Rare Müllerian Anomaly: Complete Septate Uterus with Simultaneous Longitudinal and Transverse Vaginal Septa

We present a patient with primary amenorrhea and a rare combination of anomalies. She was found to have a septate uterus, double cervix, and a longitudinal and a low transverse vaginal septum.

An 18-year-old girl with primary amenorrhea presented with severe monthly pelvic pain. Examination and imaging revealed a thin transverse vaginal septum, complete septate uterus, double cervix, and a longitudinal vaginal septum. The transverse and longitudinal vaginal septa were excised and repaired.

Although repetitive pregnancy loss and preterm birth are associated with various Müllerian duct anomalies, clinicians should also be suspicious of the presented anomaly in cases of primary amenorrhea and cyclic pelvic pain. To our knowledge, this is the only case of simultaneous septate uterus with longitudinal and transverse vaginal septum and the second case of combined longitudinal and transverse septum, which caused primary amenorrhea. This rare anomaly further supports the bidirectional regression theory of Müllerian development.