Pediatric Mayer-Rokitansky-Küster-Hauser Syndrome

Pediatric Mayer-Rokitansky-Küster-Hauser Syndrome



Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome causes the vagina and uterus to be underdeveloped or completely missing.

Expanded overview

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a disorder that affects females reproductive system. Females born with this condition have normal external genitalia, but have underdeveloped or completely missing vagina and uterus. Females with this condition generally do not menstruate, but they often have normal breast and pubic hair development. Because females with MRKH do not have a functional uterus, they cannot bear children.


The causes of MRKH is unknown, but is thought to be due to genetics.


There are two types of MRKH syndrome:

  • MRKH syndrome type 1 – only affects the reproductive organs
  • MRKH syndrome type 2 – causes abnormalities in other areas of the body (such as kidneys, spine or heart), as well as the reproductive system


In many cases, this condition shows no symptoms during childhood. The main symptom of this condition is:

  • Teenaged female who hasn't begun menstruating

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