Abstract
Rokitansky-Küster-Hauser syndrome (RKH), also known as Müllerian agenesis, represents a rare congenital disorder characterized by the absence or underdevelopment of the müllerian duct structures in phenotypic females. This condition manifests as primary amenorrhea, with a typical constellation of associated anomalies encompassing the urogenital and skeletal systems. Despite its rarity, RKH poses significant physical, psychological, and reproductive challenges to affected individuals. This comprehensive article endeavors to elucidate the complex clinical spectrum, pathogenesis, diagnostic modalities, and multidisciplinary management strategies for Rokitansky-Küster-Hauser syndrome.
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