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KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Müllerian duct anomalies (MDA)
Synonyms
- Uterine fusion anomalies
- Subtype: Obstructed hemivagina & ipsilateral renal agenesis (OHVIRA syndrome)
Definitions
- 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
- Müllerian agenesis or hypoplasia (class I in American Fertility Society classification system)
- Abnormal development, improper fusion, or failure of resorption of müllerian (paramesonephric) duct structures
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
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Müllerian duct anomalies (MDA)
Synonyms
- Uterine fusion anomalies
- Subtype: Obstructed hemivagina & ipsilateral renal agenesis (OHVIRA syndrome)
Definitions
- 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
- Müllerian agenesis or hypoplasia (class I in American Fertility Society classification system)
- Abnormal development, improper fusion, or failure of resorption of müllerian (paramesonephric) duct structures
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
