Midline fusion of cerebellar hemispheres with partial or complete loss of vermis
IMAGING
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Haratz KK et al: Fourth ventricle index: sonographic marker for severe fetal vermian dysgenesis/agenesis. Ultrasound Obstet Gynecol. 53(3):390-5, 2019
Pertl B et al: The fetal posterior fossa on prenatal ultrasound imaging: normal longitudinal development and posterior fossa anomalies. Ultraschall Med. 40(6):692-721, 2019
Aldinger KA et al: Rhombencephalosynapsis: fused cerebellum, confused geneticists. Am J Med Genet C Semin Med Genet. 178(4):432-9, 2018
Kline-Fath BM et al: Prenatal aqueduct stenosis: association with rhombencephalosynapsis and neonatal outcome. Prenat Diagn. 38(13):1028-34, 2018
Kalane U et al: Rhombencephalosynapsis: a rare cerebellar malformation associated with aqueductal stenosis and obstructive hydrocephalus. Neurol India. 64(6):1381-3, 2016
Kobayashi Y et al: Gómez-López-Hernández syndrome in a Japanese patient: a case report. Brain Dev. 37(3):356-8, 2015
Passi GR et al: Rhombencephalosynapsis. Pediatr Neurol. 52(6):651-2, 2015
Poretti A et al: Fetal diagnosis of rhombencephalosynapsis. Neuropediatrics. 46(6):357-8, 2015
Whitehead MT et al: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. Pediatr Radiol. 44(7):849-56, 2014
Cagneaux M et al: Severe second-trimester obstructive ventriculomegaly related to disorders of diencephalic, mesencephalic and rhombencephalic differentiation. Ultrasound Obstet Gynecol. 42(5):596-602, 2013
Tully HM et al: Persistent figure-eight and side-to-side head shaking is a marker for rhombencephalosynapsis. Mov Disord. 28(14):2019-23, 2013
Weaver J et al: Rhombencephalosynapsis: embryopathology and management strategies of associated neurosurgical conditions with a review of the literature. J Neurosurg Pediatr. 11(3):320-6, 2013
Barth PG: Rhombencephalosynapsis: new findings in a larger study. Brain. 135(Pt 5):1346-7, 2012
Bekiesińska-Figatowska M et al: Rhombencephalosynapsis - isolated anomaly or complex malformation? Pol J Radiol. 77(3):35-8, 2012
Ishak GE et al: Rhombencephalosynapsis: a hindbrain malformation associated with incomplete separation of midbrain and forebrain, hydrocephalus and a broad spectrum of severity. Brain. 135(Pt 5):1370-86, 2012
Koprivsek KM et al: Partial rhombencephalosynapsis: prenatal MR imaging diagnosis and postnatal follow up. Acta Neurol Belg. 111(2):157-9, 2011
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Rhombencephalosynapsis (RES)
Definitions
Midline fusion of cerebellar hemispheres with partial or complete loss of vermis
IMAGING
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Haratz KK et al: Fourth ventricle index: sonographic marker for severe fetal vermian dysgenesis/agenesis. Ultrasound Obstet Gynecol. 53(3):390-5, 2019
Pertl B et al: The fetal posterior fossa on prenatal ultrasound imaging: normal longitudinal development and posterior fossa anomalies. Ultraschall Med. 40(6):692-721, 2019
Aldinger KA et al: Rhombencephalosynapsis: fused cerebellum, confused geneticists. Am J Med Genet C Semin Med Genet. 178(4):432-9, 2018
Kline-Fath BM et al: Prenatal aqueduct stenosis: association with rhombencephalosynapsis and neonatal outcome. Prenat Diagn. 38(13):1028-34, 2018
Kalane U et al: Rhombencephalosynapsis: a rare cerebellar malformation associated with aqueductal stenosis and obstructive hydrocephalus. Neurol India. 64(6):1381-3, 2016
Kobayashi Y et al: Gómez-López-Hernández syndrome in a Japanese patient: a case report. Brain Dev. 37(3):356-8, 2015
Passi GR et al: Rhombencephalosynapsis. Pediatr Neurol. 52(6):651-2, 2015
Poretti A et al: Fetal diagnosis of rhombencephalosynapsis. Neuropediatrics. 46(6):357-8, 2015
Whitehead MT et al: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. Pediatr Radiol. 44(7):849-56, 2014
Cagneaux M et al: Severe second-trimester obstructive ventriculomegaly related to disorders of diencephalic, mesencephalic and rhombencephalic differentiation. Ultrasound Obstet Gynecol. 42(5):596-602, 2013
Tully HM et al: Persistent figure-eight and side-to-side head shaking is a marker for rhombencephalosynapsis. Mov Disord. 28(14):2019-23, 2013
Weaver J et al: Rhombencephalosynapsis: embryopathology and management strategies of associated neurosurgical conditions with a review of the literature. J Neurosurg Pediatr. 11(3):320-6, 2013
Barth PG: Rhombencephalosynapsis: new findings in a larger study. Brain. 135(Pt 5):1346-7, 2012
Bekiesińska-Figatowska M et al: Rhombencephalosynapsis - isolated anomaly or complex malformation? Pol J Radiol. 77(3):35-8, 2012
Ishak GE et al: Rhombencephalosynapsis: a hindbrain malformation associated with incomplete separation of midbrain and forebrain, hydrocephalus and a broad spectrum of severity. Brain. 135(Pt 5):1370-86, 2012
Koprivsek KM et al: Partial rhombencephalosynapsis: prenatal MR imaging diagnosis and postnatal follow up. Acta Neurol Belg. 111(2):157-9, 2011
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