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O&G TopicsReproductive endocrinology & infertility

O&G · Reproductive endocrinology & infertility

Congenital uterine anomalies (Müllerian duct anomalies)

Also known as Müllerian duct anomalies · Müllerian anomalies · Congenital uterine anomalies · Septate uterus · Bicornuate uterus · Uterus didelphys · Unicornuate uterus · Müllerian agenesis · ASRM MAC2021 · ESHRE/ESGE CONUTA classification

Exam-exhaustive FRANZCOG fellowship reference on congenital uterine (Müllerian duct) anomalies. Reproduces the three competing classifications — the 1988 American Fertility Society system, the 2013 ESHRE/ESGE (CONUTA) anatomy-based U0–U6 classes, and the 2021 ASRM Müllerian Anomalies Classification — with the septate diagnostic criteria (ASRM, ESHRE/ESGE, CUME) that produce wildly different prevalence figures. Covers Müllerian duct embryology, the ESHRE/ESGE Thessaloniki diagnostic tier (3D ultrasound first-line, MRI for complex), hysteroscopic septum resection with the limits of its observational evidence (Cochrane, TRUST trial, Wu meta-analysis), and Strassman metroplasty for the bicornuate uterus. RANZCOG-primary, globally tagged.

medium16 referencesUpdated 1 Aug 202612 min readVerification in progress

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FRANZCOGMRCOGABOGFRCSCMRCPI

Red flags

  • Adolescent with cyclical pelvic pain and primary amenorrhoea, or pain with a normal cycle, and a pelvic mass — think an obstructive anomaly (OHVIRA, transverse vaginal septum, imperforate hymen); image and relieve the obstruction to protect fertility
  • A uterus labelled 'septate' by one set of criteria but not another — the ASRM/CUME vs ESHRE/ESGE discordance is wide; confirm with 3D ultrasound before any surgery to avoid operating on a normal or arcuate uterus
  • A 'septate uterus' with an external fundal cleft — that is a bicornuate uterus, not a septum; hysteroscopic resection of a bicorporeal fundus is the classic irreversible error
  • Recurrent second-trimester loss or preterm labour in a woman never imaged — a Müllerian anomaly is on the differential and 3D ultrasound is cheap and definitive
  • Any Müllerian anomaly found on imaging without a renal tract assessment — Müllerian and renal development share embryological proximity; screen the kidneys
  • Müllerian agenesis (MRKH) mistaken for imperforate hymen or androgen insensitivity — karyotype and assess the uterus before any decision about gonadectomy or vaginal creation
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Your progress

Saved on this device.

Practise this topic8 MCQs with explanations

Target exams

FRANZCOGMRCOGABOGFRCSCMRCPI

Red flags

  • Adolescent with cyclical pelvic pain and primary amenorrhoea, or pain with a normal cycle, and a pelvic mass — think an obstructive anomaly (OHVIRA, transverse vaginal septum, imperforate hymen); image and relieve the obstruction to protect fertility
  • A uterus labelled 'septate' by one set of criteria but not another — the ASRM/CUME vs ESHRE/ESGE discordance is wide; confirm with 3D ultrasound before any surgery to avoid operating on a normal or arcuate uterus
  • A 'septate uterus' with an external fundal cleft — that is a bicornuate uterus, not a septum; hysteroscopic resection of a bicorporeal fundus is the classic irreversible error
  • Recurrent second-trimester loss or preterm labour in a woman never imaged — a Müllerian anomaly is on the differential and 3D ultrasound is cheap and definitive
  • Any Müllerian anomaly found on imaging without a renal tract assessment — Müllerian and renal development share embryological proximity; screen the kidneys
  • Müllerian agenesis (MRKH) mistaken for imperforate hymen or androgen insensitivity — karyotype and assess the uterus before any decision about gonadectomy or vaginal creation
One-line fellowship answer

A congenital uterine (Müllerian) anomaly is a structural deviation of the uterus, cervix and/or upper vagina from abnormal paramesonephric duct development. Diagnose it with 3D transvaginal ultrasound as first-line in symptomatic or high-risk women (ESHRE/ESGE Thessaloniki tier), reserving MRI for complex anomalies and dilemmas. The septate uterus is the commonest clinically significant anomaly and the one you operate on — by hysteroscopic metroplasty — but its prevalence swings wildly (1.4% to 13.5%) depending on which criteria you apply, and the evidence for resection is observational. Match the operation to the anomaly: hysteroscopic for a septum, Strassman for a bicornuate uterus, drainage for an obstruction.[2][3][4][7]

A 33-year-old sits across the desk from you with three first-trimester losses and a 3D ultrasound report describing "a fundal indentation." The number that decides whether she has surgery is not the size of the indentation — it is which definition of septate you adopt, and the same uterus can be septate, arcuate or unclassifiable depending on the rule you read. Congenital uterine anomalies reward the candidate who knows the classifications, the imaging tier, and the limits of the evidence; they punish the one who resects first and asks later.[7][8]

Overview and definition

Congenital uterine anomalies — also called Müllerian duct anomalies — are structural deviations of the uterus, cervix and/or upper vagina arising from abnormal development of the paired paramesonephric (Müllerian) ducts. They range from clinically silent to profoundly disruptive, and their reproductive burden falls on miscarriage, preterm birth and subfertility rather than on mortality.[2][5]

Three ideas anchor the whole topic. First, classification is contested: three systems coexist (the 1988 American Fertility Society classification, the 2013 ESHRE/ESGE CONUTA classification, and the 2021 ASRM Müllerian Anomalies Classification), and they do not agree on what counts as an anomaly. Second, the septate uterus dominates the clinical conversation because it is the commonest clinically significant anomaly and the only one routinely operated on. Third, imaging shows the cavity and the contour, and the external fundal contour is the feature that separates a septum (operable hysteroscopically) from a bicornuate uterus (not).[3][7][15]

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References16ShowHide
  1. [1]The American Fertility Society The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions Fertility and sterility, 1988.PMID 3371491
  2. [2]Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, Li TC, Tanos V, Brölmann H, Gianaroli L, Campo R The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies Human reproduction (Oxford, England), 2013.PMID 23771171
  3. [3]Pfeifer SM, Attaran M, Goldstein J, Lindheim SR, Petrozza JC, Rackow BW, Siegelman E, Troiano R, Winter T, Zuckerman A, Ramaiah SD ASRM müllerian anomalies classification 2021 Fertility and sterility, 2021.PMID 34756327
  4. [4]Grimbizis GF, Di Spiezio Sardo A, Saravelos SH, Gordts S, Exacoustos C, Van Schoubroeck D, Bermejo C, Amso NN, Nargund G, Timmermann D, Athanasiadis A, Brucker S, De Angelis C, Gergolet M, Li TC, Tanos V, Tarlatzis B, Farquharson R, Gianaroli L, Campo R The Thessaloniki ESHRE/ESGE consensus on diagnosis of female genital anomalies Gynecological surgery, 2016.PMID 26918000
  5. [5]Chan YY, Jayaprakasan K, Zamora J, Thornton JG, Raine-Fenning N, Coomarasamy A The prevalence of congenital uterine anomalies in unselected and high-risk populations: a systematic review Human reproduction update, 2011.PMID 21705770
  6. [6]Homer HA, Li TC, Cooke ID The septate uterus: a review of management and reproductive outcome Fertility and sterility, 2000.PMID 10632403
  7. [7]Ludwin A, Ludwin I, Coelho Neto MA, Nastri CO, Bhagavath B, Lindheim SR, Martins WP Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems Ultrasound in obstetrics & gynecology, 2019.PMID 30977223
  8. [8]Carriles I, Brotons I, Errasti T, Ruiz-Zambrana A, Ludwin A, Alcazar JL Prevalence of Septate Uterus in a Large Population of Women of Reproductive Age: Comparison of ASRM 2016 and 2021, ESHRE/ESGE, and CUME Diagnostic Criteria: A Prospective Study Diagnostics (Basel), 2024.PMID 39335698
  9. [9]Bermejo C, Martínez Ten P, Cantarero R, et al. Three-dimensional ultrasound in the diagnosis of Müllerian duct anomalies and concordance with magnetic resonance imaging Ultrasound in obstetrics & gynecology, 2010.PMID 20052665
  10. [10]Karthik SDS, Kriplani A, Mahey R, Kachhawa G Successful Reproductive Outcome After Laparoscopic Strassmann's Metroplasty Journal of human reproductive sciences, 2017.PMID 29142454
  11. [11]Joosse MI, Kostova EB, Rikken JF, Mol BWJ, Goddijn M, van Wely M Septum resection for women of reproductive age with a septate uterus The Cochrane database of systematic reviews, 2025.PMID 41268817
  12. [12]Wu X, Zhang M, Sun P, Jiang JJ, Yan L Pregnancy and Adverse Obstetric Outcomes After Hysteroscopic Resection: A Systematic Review and Meta-Analysis Frontiers in surgery, 2022.PMID 35832500
  13. [13]Rikken JFW, Kowalik CR, Emanuel MH, Bongers MY, Spinder T, de Kruif JH, Bloemenkamp KWM, Jansen FW, Veersema S, Mulders AGMGJ, Thurkow AL, Hald K, Mohazzab A, Khalaf Y, Clark TJ, Farrugia M, van Vliet HA, Stephenson MS, van der Veen F, van Wely M, Mol BWJ, Goddijn M The randomised uterine septum transsection trial (TRUST): design and protocol BMC women's health, 2018.PMID 30290803
  14. [14]Bender Atik R, Christiansen OB, Elson J, et al. ESHRE guideline: recurrent pregnancy loss: an update in 2022 Human reproduction open, 2023.PMID 36873081
  15. [15]Dixit R, Duggireddy CS, Pradhan GS Mullerian anomalies: revisiting imaging and classification Insights into imaging, 2025.PMID 39962011
  16. [16]Ludwin A, Ludwin I, Kudla M, et al. Diagnostic accuracy of three-dimensional sonohysterography compared with office hysteroscopy and its interrater/intrarater agreement in uterine cavity assessment after hysteroscopic metroplasty Fertility and sterility, 2014.PMID 24581576

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PreviousAnovulation & ovulation induction: WHO classification (FIGO HyPO-P 2023), letrozole first-line, gonadotropins, and OHSS preventionReproductive endocrinology & infertilityNextFertility preservation (oncofertility, oocyte, embryo and ovarian tissue cryopreservation)Reproductive endocrinology & infertility