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Unilateral ovarian agenesis affects approximately 1 in 11,240 women while bilateral agenesis is even rarer. A 22-year-old Sri Lankan single woman and also a university undergraduate presented with a five-year absence of menstruation. She denied sexual activity, contraceptive use, or significant medical history. Physical examination revealed Tanner Stage III breasts and scanty hair. Investigations showed an atrophic uterus, congenital bilateral ovarian anomaly, and elevated FSH levels. Karyotyping performed on follow-up revealed a 46, XX genotype. The management involved a diagnostic laparoscopy, confirming intra-abdominal findings without complications. The post-operative follow-up was unremarkable. This case underscores the rarity of congenital bilateral ovarian anomaly in young women, presenting challenges in diagnosis and management. Despite extensive evaluation, including diagnostic laparoscopy and karyotyping, the exact aetiology remains elusive. Further research is needed to elucidate the underlying mechanisms of this rare condition and guide future management strategies.
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Ganashiam et al. (2024) studied this question.
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