Clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis

AIM: To analyze the clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis. METHODS: This retrospective study reviewed the medical records of 17 patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis admitted to the Third Xiangya Hospital of Central South University between June 2007 and December 2019. RESULTS: The median age at surgery was 23 years. The most common presenting complaint was infertility. Seven (41.2%) patients were misdiagnosed previously. The obstruction was complete in five patients. All 17 patients underwent vaginoplasty, in which seven adolescent girls underwent vaginoscopic vaginal septum resections. Eight patients underwent hysteroscopic resections of the uterine septum for infertility or spontaneous miscarriage. Laparoscopy was performed in seven patients for specific indications, and only one patient was found to have pelvic endometriosis during the laparoscopy. During the follow-up, seven patients wished to conceive, and there were five living infants (four cesarean deliveries at term and one preterm vaginal delivery). CONCLUSION: Complete septate uterus with double cervix, obstructed hemivagina, and ipsilateral renal agenesis is an variant of obstructed hemivagina and ipsilateral renal agenesis syndrome. Consequently, healthcare providers should be aware of this potential variant.