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PubMed This is a summary of 13 peer-reviewed journal articles Updated
Gynecology · Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

Subtypes and Anatomy: Type I vs. Type II MRKH

At a Glance

MRKH syndrome is categorized into Type I, which only affects the reproductive system, and Type II, which also involves the kidneys and spine. Comprehensive screening is essential to check kidney and bone health, and to monitor for painful uterine remnants that respond to monthly hormones.

While all individuals with MRKH syndrome share the absence of a fully developed uterus and upper vagina, the condition is categorized into two subtypes based on whether other parts of the body are involved. Understanding which “type” you have is a crucial step in your care, as it determines which specialists should be on your medical team [1][2].

Type I: Isolated MRKH

In Type I, the condition is “isolated.” This means the developmental changes are limited strictly to the reproductive system—the Müllerian ducts [1][2]. If you have Type I, your kidneys, spine, and hearing are typically unaffected. Your body followed a standard developmental path in every way except for the formation of the uterus and the upper vaginal canal [2].

Type II: Beyond the Reproductive System

Type II is more complex because it involves other organ systems that were developing at the same time as the reproductive tract [2][3]. This type is sometimes referred to as MURCS association, an acronym that helps doctors remember the most common features:

  • MU: Müllerian duct aplasia (absence of the uterus/upper vagina) [4].
  • R: Renal anomalies (kidney issues) [4].
  • CS: Cervical Somite anomalies (spinal or vertebral defects) [4].

Why Doctors Check Your Kidneys and Spine

Because the kidneys and the spine develop alongside the reproductive system in the womb, a disruption in one often signals a disruption in the others [5][6]. This is why screening is a mandatory part of the diagnostic process for every patient [6][7].

Common Renal (Kidney) Anomalies:

  • Renal Agenesis: Being born with only one kidney [8].
  • Ectopic Kidney: A kidney that is located in the “wrong” place, such as down in the pelvis rather than the upper abdomen [8][9].

Common Skeletal (Spine) Anomalies:

  • Vertebral Defects: Small changes in the shape or fusion of the bones in the spine, particularly in the neck (cervical) or chest (thoracic) area [10][4].
  • Scoliosis: A curvature of the spine [10].

Identifying these early is important because a missing or misplaced kidney needs to be monitored to prevent long-term issues like chronic infections or decreased kidney function [8][9].

Functional Uterine Remnants and Cyclic Pain

While the uterus is described as “absent” in MRKH, some patients have small “nubbins” or functional uterine remnants [11]. These are small patches of uterine tissue that may contain a lining (endometrium) that responds to your monthly hormones [11].

Because there is no path for the blood to exit, it can build up inside the remnant—a condition called hematometra [11]. This often causes sharp or cramping cyclic pelvic pain that occurs once a month [11].

If you experience this, you should schedule an appointment with your gynecologist to investigate. While usually not a medical emergency, doctors can use a “gold standard” MRI to locate the remnants and may recommend a minimally invasive laparoscopic surgery to remove them and stop the pain [12][13].

Feature Type I (Isolated) Type II (MURCS)
Uterus/Vagina Absent or underdeveloped Absent or underdeveloped
Ovaries Normal and functional Normal and functional
Kidneys Typical May be missing or misplaced
Spine/Vertebrae Typical May have fusion or curvature
Hearing/Heart Typical Occasionally affected

Knowing your subtype isn’t just a label; it’s a roadmap that ensures every part of your body is getting the specific care and monitoring it needs [2][6].

Common questions in this guide

What is the difference between Type I and Type II MRKH?
Type I MRKH is 'isolated,' meaning it only affects the development of the uterus and upper vagina. Type II involves additional organs that develop at the same time in the womb, most commonly the kidneys and the spine.
Why do doctors check my kidneys and spine if I have MRKH?
Because the kidneys, spine, and reproductive system develop together during fetal growth, a change in one often signals a change in the others. Early screening helps identify missing or misplaced kidneys and spinal curvatures so they can be properly monitored.
Can I still have period pain if I don't have a fully developed uterus?
Yes. Some patients have functional uterine remnants, which are small patches of tissue that respond to your monthly hormones. Because there is no path for the blood to exit, it can build up and cause cyclic cramping or sharp pelvic pain.
What does MURCS association mean?
MURCS association is an acronym doctors use to describe Type II MRKH. It stands for Müllerian duct aplasia (absent uterus/vagina), Renal anomalies (kidney issues), and Cervical Somite anomalies (spinal or vertebral defects).

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my imaging, do I have Type I or Type II MRKH?
  2. 2.Do I have 'functional uterine remnants,' and if so, do they contain endometrial tissue that could cause pain?
  3. 3.If my ultrasound showed a missing or misplaced kidney, should I consult with a urologist to monitor my long-term kidney health?
  4. 4.Are my vertebral or spinal findings part of the MURCS association, and do I need to see an orthopedic specialist?
  5. 5.Given the risk of end-stage renal disease in some Type II cases, what is the long-term plan for monitoring my kidney function?

Questions For You

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References

References (13)
  1. 1

    Prevalence of urinary, prolapse, and bowel symptoms in Mayer-Rokitansky-Küster-Hauser syndrome.

    Pennesi CM, English EM, Bell S, et al.

    American journal of obstetrics and gynecology 2021; (225(1)):70.e1-70.e12 doi:10.1016/j.ajog.2021.02.020.

    PMID: 33621544
  2. 2

    Mayer-Rokitansky-Küster-Hauser syndrome with inguinal hernia, left renal fusion, and malrotation: a rare case.

    Li C, Yang H, Xiao H, Yan J

    Therapeutic advances in urology 2025; (17()):17562872251398912 doi:10.1177/17562872251398912.

    PMID: 41328177
  3. 3

    Transumbilical Vaginoplasty Through Fractionated Miniports: A Sutureless Procedure.

    El Saman AM, Abou-Taleb H, Khalaf M, et al.

    Obstetrics and gynecology 2019; (134(1)):44-48 doi:10.1097/AOG.0000000000003307.

    PMID: 31188326
  4. 4

    Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature.

    Bjørsum-Meyer T, Herlin M, Qvist N, Petersen MB

    Journal of medical case reports 2016; (10(1)):374 doi:10.1186/s13256-016-1127-9.

    PMID: 28003020
  5. 5

    Mayer-Rokitansky-Küster-Hauser syndrome type II. Case report.

    Calle JJR

    JBRA assisted reproduction 2025; (29(4)):827-833 doi:10.5935/1518-0557.20250057.

    PMID: 41165215
  6. 6

    Mayer-Rokitansky-Kuster-Hauser syndrome.

    Novoa CCT, Leite MTC, Sartori MGF

    Revista brasileira de ginecologia e obstetricia : revista da Federacao Brasileira das Sociedades de Ginecologia e Obstetricia 2025; (47()) doi:10.61622/rbgo/2025FPS4.

    PMID: 40406045
  7. 7

    ACOG Committee Opinion No. 728: Müllerian Agenesis: Diagnosis, Management, And Treatment.

    Obstetrics and gynecology 2018; (131(1)):e35-e42 doi:10.1097/AOG.0000000000002458.

    PMID: 29266078
  8. 8

    Living-Donor Kidney Transplant in a Patient With Type B Mayer-Rokitansky-Küster-Hauser Syndrome, Reconstructed Vagina, and Abnormal Pelvic Vessels: A Case Report.

    Campise M, Ferraresso M, Favi E, et al.

    Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation 2019; (17(2)):266-268 doi:10.6002/ect.2016.0220.

    PMID: 28540840
  9. 9

    Zinner and Mayer-Rokitansky-Küster-Hauser syndromes: when unilateral renal agenesis meets genital anomalies.

    Briosa F, Valsassina R, Mira C, Zagalo A

    BMJ case reports 2019; (12(5)) doi:10.1136/bcr-2018-229034.

    PMID: 31061196
  10. 10

    Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic.

    La Maida GA, Della Valle A, Gallazzi E, et al.

    Brain & spine 2023; (3()):101753 doi:10.1016/j.bas.2023.101753.

    PMID: 37197562
  11. 11

    A case of Mayer-Rokitansky-Küster-Hauser syndrome in a low-resource tertiary hospital in Douala, Cameroon.

    Egbe TO, Kobenge FM, Junette Arlette MM, et al.

    SAGE open medical case reports 2019; (7()):2050313X19830817 doi:10.1177/2050313X19830817.

    PMID: 30800315
  12. 12

    Mayer-Rokitansky-Kuster-Hauser Syndrome: From Radiological Diagnosis to Further Challenges-Review and Update.

    Schiau C, Csutak C, Ciurea AI, et al.

    Diagnostics (Basel, Switzerland) 2026; (16(1)) doi:10.3390/diagnostics16010138.

    PMID: 41515635
  13. 13

    Hernia uterine inguinale associated with Mayer-Rokitansky-Küster-Hauser syndrome: Three case reports and literature review.

    Dai Y, Qin C, Zhu L, Luo G

    Medicine 2023; (102(5)):e32802 doi:10.1097/MD.0000000000032802.

    PMID: 36749224

This page explains MRKH syndrome subtypes and anatomy for educational purposes only. Always consult your gynecologist and specialist team for medical advice, imaging, and personalized care.

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