Mayer-Rokitansky-Küster-Hauser syndrome

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is an anomaly that belongs to class I Mullerian duct anomalies.

There are two different form of this syndrome:

  • the typical form (type A) of this syndrome is characterised by the congenital absence of the uterus and upper 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


It has a reported incidence of ~1:4000-5000 female live births.

Clinical presentation

Clinical presentation is characterised by primary amenorrhoea, with normal hormonal levels guaranteed by fully functional gonads. At times cyclic pelvic pain may be present in the post-puberal period due to an accumulation of haemorrhagic material within uterine buds with a functioning endometrium.


The anomaly is thought to arise during embryogenesis, with arrested development of the paramesonephric ducts at ~7 weeks after fertilisation.

The MRKH syndrome is generally characterised by normal external genitalia and absence or reduced development of the uterus and upper two-thirds of the vagina.

The fallopian tubes, uterus, cervix and upper ¾ of vagina develop from Müllerian ducts between the 8th-12th gestational week. A developmental defect occurring at this stage leads to agenesis of Müllerian structures. The development of kidneys, ureter, and bladder occurs concomitantly at around 6th-12th week of gestation. The presence of residual components such as a blind vaginal pouch and a rudimentary uterus (non-functioning myometrial tissue), which are noted in significant proportion of patients.


The syndrome is often associated with alterations in the urinary or skeletal system which include:

See also

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Article Information

rID: 10786
System: Gynaecology
Tag: syndrome
Synonyms or Alternate Spellings:
  • Mayer-Rokitansky-Küster-Hauser
  • MRKH
  • MRK
  • Mayer-Rokitansky-Küster
  • MRKH syndrome
  • Mayer-Rokitansky-Küster syndrome
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    Case 6: with crossed fused renal ectopia
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