Mayer-Rokitansky-Kuster-Huster syndrome

Case contributed by Fateme Hosseinabadi
Diagnosis almost certain

Presentation

Primary amenorrhea

Patient Data

Age: 17 Years
Gender: Female

Congenital absence of the uterus and cervix and upper 2/3 vagina with associated absence of bilateral ovaries and fallopian tubes. External genitalia appears normal.

Case Discussion

Mayer-Rokitanski-Kuster-Hauster syndrome (MRKH) type B or atypical type is a congenital anomaly characterized by vaginal agenesis and congenital absence of uterus, associated with absent ovaries and fallopian tubes.

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