MRKH Type II

Case Discussion

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), also known as Müllerian agenesis, is a congenital anomaly characterized by vaginal agenesis associated with, in the majority of cases, a spectrum of other genitourinary tract abnormalities. MRKH syndrome belongs to class I Mullerian duct anomalies. Two different forms are described: • the typical form (type A) of this syndrome is characterized by the congenital absence of the uterus and upper 2/3 vagina with normal ovaries and fallopian tubes • the atypical form (type B) of the syndrome includes associated abnormalities of the ovaries and fallopian tubes and renal anomalies Arrested development of the paramesonephric ducts at ~7 weeks after fertilization, during embryogenesis. associated with alterations in the urinary or skeletal system which include: • vertebral anomalies: may be present in ~10% of cases • renal anomalies: such as renal agenesis, ectopic kidney, fused kidney, renal hypoplasia, and horseshoe kidney may be seen in 30-40% of patients with the syndrome

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