Duplicated uterus and hemivaginal or hemicervical atresia with ipsilateral renal agenesis: an institutional clinical series of 52 cases

ObjectivesTo retrospectively review cases of unilateral vaginal or cervical atresia with ipsilateral renal agenesis at our institution and to analyze the clinical presentation, diagnostic pitfalls, management, and embryological implications for the vaginal origin that arise from this syndrome.Study designA retrospective observational study that included 52 patients diagnosed with this syndrome between 1998 and 2008 at Peking Union Medical College Hospital.ResultsThe median age at diagnosis was 21.5 years, and the median time between the first onset of symptoms and diagnosis was 12 months. The most common presenting complaints were dysmenorrhea, purulent discharge and irregular spotting, despite the wide spectrum of symptoms at referral. Patients with and without a communication between the two hemivaginas or hemiuteri had different clinical characteristics. Of the patients, 59.6% had an obstruction on the right side. Of patients who had received a check-up prior to referral, 92.9% (n = 28) had been misdiagnosed, and 53.9% had received inappropriate surgery as therapy. The pathology of the resected septum showed squamous epithelium in 13 samples, while 5 samples had epithelium with paramesonephric characteristics.ConclusionKnowledge of the origins and clinical presentation of this syndrome is the foundation for correct and timely diagnosis and treatment. Moreover, this unique anomaly may offer essential clues for determining the embryological origins of the vagina and cervix.