Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by agenesis or aplasia of the uterus. Patients usually present with primary amenorrhea. MRKH can be isolated (type I) or accompanied by other malformations (type II) involving the kidney, skeletal, and vascular systems. Magnetic resonance imaging (MRI) is the mainstay of imaging modality in evaluating this syndrome. A 20-year-old woman presented with cyclic abdominal pain and primary amenorrhea. Secondary sexual characteristics and hormone evaluation were normal. Ultrasound and MRI were conducted and revealed no normal uterus structure with ectopic ovarium and crossed fused ectopic renal.

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