Combined agenesis of uterus, cervix, and upper portion of vagina most common
Presents as primary amenorrhea
Unicornuate uterus (20%)
Single uterine horn; may have rudimentary horn
Uterus didelphys (5%)
2 separate, noncommunicating horns with 2 cervices
Bicornuate uterus (10%)
Concave or heart-shaped external contour
2 horns with variable degree of fusion
May have 1 cervix (bicornis unicollis) or 2 cervices (bicornis bicollis)
Septate uterus (55%)
Normal external contour
Septum may extend for variable lengths
Complete (to external os) or partial (subseptate)
Arcuate uterus
Argued whether this should be classified as congenital anomaly or anatomic variant
Smooth, broad, fundal indentation into cavity with normal external contour
IMAGING
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Akhtar MA et al: Reproductive implications and management of congenital uterine anomalies: scientific impact paper No. 62 November 2019. BJOG. 127(5):e1-13, 2020
Karami M et al: The association between mullerian anomalies and IUGR: a meta-analysis. J Matern Fetal Neonatal Med. 32(14):2408-11, 2019
Khazaei S et al: The association of mullerian anomalies and placenta abruption: a meta-analysis. J Matern Fetal Neonatal Med. 32(3):512-6, 2019
Ludwin A et al: Reproductive surgery for müllerian anomalies: a review of progress in the last decade. Fertil Steril. 112(3):408-16, 2019
Theodoridis TD et al: Surgical management of congenital uterine anomalies (including indications and surgical techniques). Best Pract Res Clin Obstet Gynaecol. 59:66-76, 2019
Committee on Adolescent Health Care.: ACOG committee opinion No. 728: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 131(1):e35-42, 2018
Friedman MA et al: Screening for mullerian anomalies in patients with unilateral renal agenesis: leveraging early detection to prevent complications. J Pediatr Urol. 14(2):144-9, 2018
Robbins JB et al: Müllerian duct anomalies: embryological development, classification, and MRI assessment. J Magn Reson Imaging. 41(1):1-12, 2015
Sadro CT: Pictorial review of complications of uterine anomalies. Can Assoc Radiol J. 66(4):368-76, 2015
Acién P et al: Reproductive outcome in uterine malformations with or without an associated unilateral renal agenesis. J Reprod Med. 59(1-2):69-75, 2014
Dreisler E et al: Müllerian duct anomalies diagnosed by saline contrast sonohysterography: prevalence in a general population. Fertil Steril. 102(2):525-9, 2014
Paradisi R et al: The techniques and outcomes of hysteroscopic metroplasty. Curr Opin Obstet Gynecol. 26(4):295-301, 2014
Lewis AD et al: Pregnancy complications in women with uterine duplication abnormalities. Ultrasound Q. 26(4):193-200, 2010
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Müllerian duct anomaly (MDA)
Definitions
Spectrum of congenital uterine malformations
Agenesis/hypoplasia (10%)
Combined agenesis of uterus, cervix, and upper portion of vagina most common
Presents as primary amenorrhea
Unicornuate uterus (20%)
Single uterine horn; may have rudimentary horn
Uterus didelphys (5%)
2 separate, noncommunicating horns with 2 cervices
Bicornuate uterus (10%)
Concave or heart-shaped external contour
2 horns with variable degree of fusion
May have 1 cervix (bicornis unicollis) or 2 cervices (bicornis bicollis)
Septate uterus (55%)
Normal external contour
Septum may extend for variable lengths
Complete (to external os) or partial (subseptate)
Arcuate uterus
Argued whether this should be classified as congenital anomaly or anatomic variant
Smooth, broad, fundal indentation into cavity with normal external contour
IMAGING
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Akhtar MA et al: Reproductive implications and management of congenital uterine anomalies: scientific impact paper No. 62 November 2019. BJOG. 127(5):e1-13, 2020
Karami M et al: The association between mullerian anomalies and IUGR: a meta-analysis. J Matern Fetal Neonatal Med. 32(14):2408-11, 2019
Khazaei S et al: The association of mullerian anomalies and placenta abruption: a meta-analysis. J Matern Fetal Neonatal Med. 32(3):512-6, 2019
Ludwin A et al: Reproductive surgery for müllerian anomalies: a review of progress in the last decade. Fertil Steril. 112(3):408-16, 2019
Theodoridis TD et al: Surgical management of congenital uterine anomalies (including indications and surgical techniques). Best Pract Res Clin Obstet Gynaecol. 59:66-76, 2019
Committee on Adolescent Health Care.: ACOG committee opinion No. 728: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 131(1):e35-42, 2018
Friedman MA et al: Screening for mullerian anomalies in patients with unilateral renal agenesis: leveraging early detection to prevent complications. J Pediatr Urol. 14(2):144-9, 2018
Robbins JB et al: Müllerian duct anomalies: embryological development, classification, and MRI assessment. J Magn Reson Imaging. 41(1):1-12, 2015
Sadro CT: Pictorial review of complications of uterine anomalies. Can Assoc Radiol J. 66(4):368-76, 2015
Acién P et al: Reproductive outcome in uterine malformations with or without an associated unilateral renal agenesis. J Reprod Med. 59(1-2):69-75, 2014
Dreisler E et al: Müllerian duct anomalies diagnosed by saline contrast sonohysterography: prevalence in a general population. Fertil Steril. 102(2):525-9, 2014
Paradisi R et al: The techniques and outcomes of hysteroscopic metroplasty. Curr Opin Obstet Gynecol. 26(4):295-301, 2014
Lewis AD et al: Pregnancy complications in women with uterine duplication abnormalities. Ultrasound Q. 26(4):193-200, 2010
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