Asrm mullerian anomaly classification 2021 woth categories

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ASRM 2021 Mullerian anomaly classification categories

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Here is the complete ASRM Mullerian Anomalies Classification 2021 (MAC2021):

ASRM Mullerian Anomalies Classification 2021 (MAC2021)

Published in Fertility & Sterility, Vol. 116, No. 5, November 2021. MAC2021 classifies Mullerian anomalies into 9 categories using descriptive terminology (not numbered like the old 1988 AFS system). Anomalies may appear in more than one category because they represent a continuum of development with combined elements. Diagnosis is based on ultrasound (2D/3D), MRI, HSG, hysteroscopy, and/or laparoscopy.

Category 1: Mullerian Agenesis

Also known as: MRKH syndrome, Mayer-Rokitansky-Kuster-Hauser syndrome, vaginal agenesis
Subcategories:
  • Mullerian agenesis (classic) - widely divergent atrophic uterine remnants, absent cervix and upper vagina
  • Mullerian agenesis with bilateral atrophic uterine remnants with functional endometrium
  • Mullerian agenesis with R/L (unilateral) atrophic uterine remnant with functional endometrium
Key features: No palpable uterus/cervix; vaginal canal absent or shallow dimple; evaluate for renal anomalies; presents with primary amenorrhea

Category 2: Cervical Agenesis

Subcategories:
  • Cervical agenesis (complete)
  • Distal cervical agenesis
Key features: Uterus present but cervix absent or partially absent; often presents with cyclic pelvic pain and hematometra

Category 3: Unicornuate Uterus

Subcategories:
  • R/L unicornuate uterus (isolated)
  • R/L unicornuate uterus with R/L distal atrophic uterine remnant (no endometrium)
  • R/L unicornuate uterus with R/L distal uterine remnant with functional endometrium
  • R/L unicornuate uterus with R/L associated atrophic uterine remnant (communicating or non-communicating)
  • R/L unicornuate uterus with R/L uterine horn communicating at the level of the cervix

Category 4: Uterus Didelphys

Subcategories:
  • Uterus didelphys with longitudinal vaginal septum (complete)
  • Uterus didelphys with +/- longitudinal vaginal septum of variable length
  • Uterus didelphys with obstructed R/L hemivagina (Herlyn-Werner-Wunderlich syndrome variant)

Category 5: Bicornuate Uterus

Subcategories:
  • Bicornuate uterus - serosal indentation >1 cm (measured from the bicornual line)
  • Bicornuate uterus with R/L communicating tract
  • Uterus bicornuate bicollis (two cervices)
  • Combined bicornuate septate uterus - serosal indentation >1 cm

Category 6: Septate Uterus

Subcategories:
  • Septate uterus (complete) - septum reaches internal os
  • Partial septate uterus - septum length >1 cm AND septum angle <90°
  • Normal/arcuate uterus - septum length <1 cm AND septum angle >90° (MAC2021 combines arcuate with normal, reflecting limited clinical significance)
  • Robert's uterus - asymmetric complete septum with one obstructed hemicavity
  • Complete septate uterus with duplicated cervices and longitudinal vaginal septum
  • Complete septate uterus with septate cervix and longitudinal vaginal septum
  • Complete septate uterus with duplicated cervices and obstructed R/L hemivagina

Category 7: Longitudinal Vaginal Septum

Subcategories:
  • Longitudinal vaginal septum of variable length (isolated)
  • Longitudinal vaginal septum + uterus didelphys
  • Longitudinal vaginal septum + complete septate uterus with duplicated cervix
  • Obstructed R/L hemivagina + uterus didelphys
  • Obstructed R/L hemivagina + complete septate uterus with duplicated cervices

Category 8: Transverse Vaginal Septum

Subcategories:
  • Mid vaginal septum
  • Distal vaginal septum (Proximal/upper vaginal septum also recognized)

Category 9: Complex Anomalies

For anomalies that do not fit neatly into a single category or involve combined elements:
Subcategories:
  • Bicornuate uterus with bilateral obstructed endometrial cavities
  • Uterus didelphys with communicating hemiuteri and unilateral R/L cervicovaginal atresia
  • Obstructed R/L hemivagina, hemiuterus and single cervix with separate contralateral patent hemiuterus, cervix and vagina
  • Bicornuate uterus with R/L communicating tract and transverse vaginal septum
  • Uterus isthmus agenesis

Key Differences from AFS 1988 Classification

FeatureAFS 1988MAC2021
CategoriesNumbered I-VIIDescriptive names (9 categories)
Cervical anomaliesNot includedIncluded
Vaginal anomaliesNot includedIncluded (2 dedicated categories)
Arcuate uterusSeparate categoryCombined with normal
Diagnostic criteriaVagueStandardized (e.g., septum >1 cm, angle <90°)
FormatPrintable tableInteractive digital tool + printable
Complex anomaliesNot addressedDedicated category

Educational Elements per Category (MAC2021 Interactive Tool)

Each category includes 5 elements:
  1. Presentation - symptoms, exam findings, differential diagnosis
  2. Imaging - MRI, ultrasound, HSG findings
  3. Associated anomalies - renal, skeletal, etc.
  4. Treatment - surgical and medical options
  5. Reproductive outcomes - fertility and pregnancy data

Reference: Pfeifer S et al. ASRM Mullerian Anomalies Classification 2021. Fertil Steril. 2021;116(5):1238-52. PMID: 34756294. An erratum was published in 2023 (Fertil Steril. 2023;119(6):1088).
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