Narrowing or occlusion at aqueduct of Sylvius causing obstructive hydrocephalus
Hydrocephalus vs. ventriculomegaly
Hydrocephalus
Increased intraventricular pressure
Increased ventricular size
Increased head size
Noncommunicating (obstructive)
CSF flow blocked within ventricular system
Communicating
Failure of CSF resorption
Ventriculomegaly
Result of abnormal parenchymal development or destructive process
Normal intraventricular pressure
Increased ventricular size
Head size normal or small
Lateral ventricles measured at atria
Normal: < 10 mm
Mild dilation: 10-12 mm
Moderate dilation: 12-15
Severe dilation: > 15 mm
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Etchegaray A et al: Prenatal genetic considerations in congenital ventriculomegaly and hydrocephalus. Childs Nerv Syst. 36(8):1645-60, 2020
Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020
Alhousseini A et al: Familial hydrocephalus and dysgenesis of the corpus callosum Associated with Xp22.33 duplication and stenosis of the aqueduct of sylvius with X-linked recessive inheritance pattern. Gynecol Obstet Invest. 84(4):412-6, 2019
Heaphy-Henault KJ et al: Congenital aqueductal stenosis: findings at fetal MRI that accurately predict a postnatal diagnosis. AJNR Am J Neuroradiol. 39(5):942-8, 2018
Ouyang YS et al: Adducted thumb as an isolated morphologic finding: an early sonographic sign of impaired neurodevelopment: A STROBE compliant study. Medicine (Baltimore). 97(38):e12437, 2018
Pisapia JM et al: Fetal ventriculomegaly: diagnosis, treatment, and future directions. Childs Nerv Syst. 33(7):1113-23, 2017
Corral E et al: Prenatal three-dimensional ultrasound detection of adducted thumbs in X-linked hydrocephaly: two case reports with molecular genetic studies. Case Rep Obstet Gynecol. 2015:561713, 2015
Emery SP et al: Accuracy of prenatal diagnosis of isolated aqueductal stenosis. Prenat Diagn. 35(4):319-24, 2015
Emery SP et al: Fetal therapy for isolated aqueductal stenosis. Fetal Diagn Ther. 38(2):81-5, 2015
Perlman S et al: Prenatal diagnosis of fetal ventriculomegaly: Agreement between fetal brain ultrasonography and MR imaging. AJNR Am J Neuroradiol. 35(6):1214-8, 2014
Serikawa T et al: Prenatal molecular diagnosis of X-linked hydrocephalus via a silent C924T mutation in the L1CAM gene. Congenit Anom (Kyoto). 54(4):243-5, 2014
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
Spennato P et al: Endoscopic third ventriculostomy for idiopathic aqueductal stenosis. World Neurosurg. 79(2 Suppl):S21.e13-20, 2013
Sacko O et al: Endoscopic third ventriculostomy: outcome analysis in 368 procedures. J Neurosurg Pediatr. 5(1):68-74, 2010
D'Addario V et al: Sonographic diagnosis of fetal cerebral ventriculomegaly: an update. J Matern Fetal Neonatal Med. 20(1):7-14, 2007
Kenwrick S et al: X linked hydrocephalus and MASA syndrome. J Med Genet. 33(1):59-65, 1996
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
Aqueductal stenosis (AS)
Definitions
Narrowing or occlusion at aqueduct of Sylvius causing obstructive hydrocephalus
Hydrocephalus vs. ventriculomegaly
Hydrocephalus
Increased intraventricular pressure
Increased ventricular size
Increased head size
Noncommunicating (obstructive)
CSF flow blocked within ventricular system
Communicating
Failure of CSF resorption
Ventriculomegaly
Result of abnormal parenchymal development or destructive process
Normal intraventricular pressure
Increased ventricular size
Head size normal or small
Lateral ventricles measured at atria
Normal: < 10 mm
Mild dilation: 10-12 mm
Moderate dilation: 12-15
Severe dilation: > 15 mm
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Etchegaray A et al: Prenatal genetic considerations in congenital ventriculomegaly and hydrocephalus. Childs Nerv Syst. 36(8):1645-60, 2020
Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020
Alhousseini A et al: Familial hydrocephalus and dysgenesis of the corpus callosum Associated with Xp22.33 duplication and stenosis of the aqueduct of sylvius with X-linked recessive inheritance pattern. Gynecol Obstet Invest. 84(4):412-6, 2019
Heaphy-Henault KJ et al: Congenital aqueductal stenosis: findings at fetal MRI that accurately predict a postnatal diagnosis. AJNR Am J Neuroradiol. 39(5):942-8, 2018
Ouyang YS et al: Adducted thumb as an isolated morphologic finding: an early sonographic sign of impaired neurodevelopment: A STROBE compliant study. Medicine (Baltimore). 97(38):e12437, 2018
Pisapia JM et al: Fetal ventriculomegaly: diagnosis, treatment, and future directions. Childs Nerv Syst. 33(7):1113-23, 2017
Corral E et al: Prenatal three-dimensional ultrasound detection of adducted thumbs in X-linked hydrocephaly: two case reports with molecular genetic studies. Case Rep Obstet Gynecol. 2015:561713, 2015
Emery SP et al: Accuracy of prenatal diagnosis of isolated aqueductal stenosis. Prenat Diagn. 35(4):319-24, 2015
Emery SP et al: Fetal therapy for isolated aqueductal stenosis. Fetal Diagn Ther. 38(2):81-5, 2015
Perlman S et al: Prenatal diagnosis of fetal ventriculomegaly: Agreement between fetal brain ultrasonography and MR imaging. AJNR Am J Neuroradiol. 35(6):1214-8, 2014
Serikawa T et al: Prenatal molecular diagnosis of X-linked hydrocephalus via a silent C924T mutation in the L1CAM gene. Congenit Anom (Kyoto). 54(4):243-5, 2014
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
Spennato P et al: Endoscopic third ventriculostomy for idiopathic aqueductal stenosis. World Neurosurg. 79(2 Suppl):S21.e13-20, 2013
Sacko O et al: Endoscopic third ventriculostomy: outcome analysis in 368 procedures. J Neurosurg Pediatr. 5(1):68-74, 2010
D'Addario V et al: Sonographic diagnosis of fetal cerebral ventriculomegaly: an update. J Matern Fetal Neonatal Med. 20(1):7-14, 2007
Kenwrick S et al: X linked hydrocephalus and MASA syndrome. J Med Genet. 33(1):59-65, 1996
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