Abstract
INTRODUCTION AND IMPORTANCE: Report of an extremely rare case of triple Mullerian anomaly consisting of cervical agenesis, partial vaginal agenesis and complete bicorporeal uterus with functioning endometrium associated with adenomyosis and pelvic endometriosis in a young girl managed with hysterectomy of both uterine horns and excision of pelvic endometriosis. PRESENTATION OF CASE: A 20-year-old young woman presented with primary amenorrhoea and severe cyclical pain abdomen. She was diagnosed with a rare triple Mullerian anomaly consisting of cervical agenesis, partial vaginal agenesis and complete bicorporeal uterus with functioning endometrium associated with adenomyosis and pelvic endometriosis. She had undergone laparoscopic hematosalpinx drainage in an outside setting which provided her a temporary relief from symptoms. After an accurate pre-operative diagnosis of her condition, she underwent hysterectomy of both uterine horns as both the horns were grossly adenomyotic with hematometra. DISCUSSION: This case is unique as two different developmental anomalies agenesis and lateral fusion defect were found together leading to a triple Mullerian anomaly with co-existing adenomyosis and endometriosis. Conservative surgery in this particular case had high probability for developing obstruction, sepsis or pelvic abscess later or repeat procedures/surgeries leading to increased morbidity. She was given the option for oocyte freezing along with gestational surrogacy if she desired fertility in future. CONCLUSION: Early diagnosis and tailored intervention of Mullerian anomalies is essential to improve patients' quality of life. Definitive surgery in the form of hysterectomy should be considered if there are associated pathologies such as adenomyosis and endometriosis and findings such as hematometra.