Abnormal development, improper fusion, or failure of resorption of müllerian (paramesonephric) duct structures
Müllerian agenesis or hypoplasia (class I in American Fertility Society classification system)
Complete or segmental agenesis or variable degrees of uterovaginal hypoplasia
Unicornuate uterus (class II)
Partial or complete unilateral hypoplasia
Uterus didelphys (class III)
Duplication of uterus
Bicornuate uterus (class IV)
Incomplete fusion of superior uterovaginal canal
Septate uterus (class V)
Incomplete resorption of uterine septum
Arcuate uterus (class VI)
Near-complete resorption of uterine septum
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Radiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Habiba M et al: The development of the human uterus: morphogenesis to menarche. Hum Reprod Update. 27(1):1-26, 2021
O'Flynn O'Brien KL et al: The prevalence of müllerian anomalies in women with a diagnosed renal anomaly. J Pediatr Adolesc Gynecol. 34(2):154-60, 2020
Pitot MA et al: Müllerian duct anomalies coincident with endometriosis: a review. Abdom Radiol (NY). 45(6):1723-40, 2020
Yen CF et al: Laparoscopic metroplasty: reconstructive surgery for unicornuate uterus with noncommunicating, functional uterine horn. Fertil Steril. 114(5):1119-21, 2020
Ludwin A et al: Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems. Ultrasound Obstet Gynecol. 54(6):800-14, 2019
Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
Jegannathan D et al: Magnetic resonance imaging of classified and unclassified Müllerian duct anomalies: comparison of the American Society for Reproductive Medicine and the European Society of Human Reproduction and Embryology classifications. SA J Radiol. 22(1):1259, 2018
Choussein S et al: Mullerian dysgenesis: a critical review of the literature. Arch Gynecol Obstet. 295(6):1369-81, 2017
Olpin JD et al: MR imaging of müllerian fusion anomalies. Magn Reson Imaging Clin N Am. 25(3):563-75, 2017
Ludwin A et al: Reliability of the European Society of Human Reproduction and Embryology/European Society for Gynaecological Endoscopy and American Society for Reproductive Medicine classification systems for congenital uterine anomalies detected using three-dimensional ultrasonography. Fertil Steril. 104(3):688-97.e8, 2015
Schlomer B et al: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina. J Pediatr Urol. 11(2):77.e1-6, 2015
Santos XM et al: The utility of ultrasound and magnetic resonance imaging versus surgery for the characterization of müllerian anomalies in the pediatric and adolescent population. J Pediatr Adolesc Gynecol. 25(3):181-4, 2012
Marcal L et al: Mullerian duct anomalies: MR imaging. Abdom Imaging. 36(6):756-64, 2011
Mane SB et al: Our 10-year experience of variable Müllerian anomalies and its management. Pediatr Surg Int. 26(8):795-800, 2010
Vallerie AM et al: Update in Müllerian anomalies: diagnosis, management, and outcomes. Curr Opin Obstet Gynecol. 22(5):381-7, 2010
Chandler TM et al: Mullerian duct anomalies: from diagnosis to intervention. Br J Radiol. 82(984):1034-42, 2009
Junqueira BL et al: Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics. 29(4):1085-103, 2009
Capito C et al: Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: a series of 32 cases. Pediatrics. 122(4):e891-7, 2008
Orazi C et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. sonographic and MR findings in 11 cases. Pediatr Radiol. 37(7):657-65, 2007
Troiano RN et al: Mullerian duct anomalies: imaging and clinical issues. Radiology. 233(1):19-34, 2004
Elford KJ et al: The forgotten female: Pediatric and adolescent gynecological concerns and their reproductive consequences. J Pediatr Adolesc Gynecol. 15(2):65-77, 2002
Lang IM et al: MR imaging of paediatric uterovaginal anomalies. Pediatr Radiol. 29(3):163-70, 1999
Abnormal development, improper fusion, or failure of resorption of müllerian (paramesonephric) duct structures
Müllerian agenesis or hypoplasia (class I in American Fertility Society classification system)
Complete or segmental agenesis or variable degrees of uterovaginal hypoplasia
Unicornuate uterus (class II)
Partial or complete unilateral hypoplasia
Uterus didelphys (class III)
Duplication of uterus
Bicornuate uterus (class IV)
Incomplete fusion of superior uterovaginal canal
Septate uterus (class V)
Incomplete resorption of uterine septum
Arcuate uterus (class VI)
Near-complete resorption of uterine septum
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Radiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Habiba M et al: The development of the human uterus: morphogenesis to menarche. Hum Reprod Update. 27(1):1-26, 2021
O'Flynn O'Brien KL et al: The prevalence of müllerian anomalies in women with a diagnosed renal anomaly. J Pediatr Adolesc Gynecol. 34(2):154-60, 2020
Pitot MA et al: Müllerian duct anomalies coincident with endometriosis: a review. Abdom Radiol (NY). 45(6):1723-40, 2020
Yen CF et al: Laparoscopic metroplasty: reconstructive surgery for unicornuate uterus with noncommunicating, functional uterine horn. Fertil Steril. 114(5):1119-21, 2020
Ludwin A et al: Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems. Ultrasound Obstet Gynecol. 54(6):800-14, 2019
Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
Jegannathan D et al: Magnetic resonance imaging of classified and unclassified Müllerian duct anomalies: comparison of the American Society for Reproductive Medicine and the European Society of Human Reproduction and Embryology classifications. SA J Radiol. 22(1):1259, 2018
Choussein S et al: Mullerian dysgenesis: a critical review of the literature. Arch Gynecol Obstet. 295(6):1369-81, 2017
Olpin JD et al: MR imaging of müllerian fusion anomalies. Magn Reson Imaging Clin N Am. 25(3):563-75, 2017
Ludwin A et al: Reliability of the European Society of Human Reproduction and Embryology/European Society for Gynaecological Endoscopy and American Society for Reproductive Medicine classification systems for congenital uterine anomalies detected using three-dimensional ultrasonography. Fertil Steril. 104(3):688-97.e8, 2015
Schlomer B et al: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina. J Pediatr Urol. 11(2):77.e1-6, 2015
Santos XM et al: The utility of ultrasound and magnetic resonance imaging versus surgery for the characterization of müllerian anomalies in the pediatric and adolescent population. J Pediatr Adolesc Gynecol. 25(3):181-4, 2012
Marcal L et al: Mullerian duct anomalies: MR imaging. Abdom Imaging. 36(6):756-64, 2011
Mane SB et al: Our 10-year experience of variable Müllerian anomalies and its management. Pediatr Surg Int. 26(8):795-800, 2010
Vallerie AM et al: Update in Müllerian anomalies: diagnosis, management, and outcomes. Curr Opin Obstet Gynecol. 22(5):381-7, 2010
Chandler TM et al: Mullerian duct anomalies: from diagnosis to intervention. Br J Radiol. 82(984):1034-42, 2009
Junqueira BL et al: Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics. 29(4):1085-103, 2009
Capito C et al: Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: a series of 32 cases. Pediatrics. 122(4):e891-7, 2008
Orazi C et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. sonographic and MR findings in 11 cases. Pediatr Radiol. 37(7):657-65, 2007
Troiano RN et al: Mullerian duct anomalies: imaging and clinical issues. Radiology. 233(1):19-34, 2004
Elford KJ et al: The forgotten female: Pediatric and adolescent gynecological concerns and their reproductive consequences. J Pediatr Adolesc Gynecol. 15(2):65-77, 2002
Lang IM et al: MR imaging of paediatric uterovaginal anomalies. Pediatr Radiol. 29(3):163-70, 1999
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