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Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) with rudimentary uterine horns

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Primary amenorrhea.

Patient Data

Age: 25 years
Gender: Male

Congenital absence of the uterus and upper 2/3 vagina with normal ovaries containing small follicles. Two rudimentary uterine horns are noted connected by a thin horizontal hypointense fibrotic band well-visualized on axial and coronal T2 sequences.

Minimal physiological effusion in Douglas pouch.

Normal appearance and location of both kidneys on coronal FIESTA sequence.

Annotated images showing the rudimentary uterine horns (white arrows) and the horizontal fibrotic band (green arrow).

Case Discussion

Case of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) with bilateral rudimentary uterine horns (rare form).

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