Ventriculomegaly involving lateral and 3rd ventricles as result of complete or partial obstruction to CSF flow within cerebral aqueduct
AS diagnosis generally refers to congenital form characterized by varying degree of obstruction at level of cerebral aqueduct
However, tumors, germinal matrix hemorrhage, or vascular lesions can obstruct aqueduct as well
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
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):942-9, 2018
Kline-Fath BM et al: Congenital aqueduct stenosis: progressive brain findings in utero to birth in the presence of severe hydrocephalus. Prenat Diagn. 38(9):706-12, 2018
Tonetti DA et al: Clinical outcomes of isolated congenital aqueductal stenosis. World Neurosurg. 114:e976-81, 2018
Yamada S et al: Current and emerging MR imaging techniques for the diagnosis and management of CSF flow disorders: a review of phase-contrast and time-spatial labeling inversion pulse. AJNR Am J Neuroradiol. 36(4):623-30, 2015
Griessenauer CJ et al: Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 13(1):13-20, 2014
Kartal MG et al: Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: an update. Insights Imaging. 5(4):531-41, 2014
Tully HM et al: Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 57(8):359-68, 2014
Ucar M et al: Evaluation of aqueductal patency in patients with hydrocephalus: three-dimensional high-sampling-efficiency technique (SPACE) versus two-dimensional turbo spin echo at 3 Tesla. Korean J Radiol. 15(6):827-35, 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
Muehlmann M et al: Magnetic resonance-based estimation of intracranial pressure correlates with ventriculoperitoneal shunt valve opening pressure setting in children with hydrocephalus. Invest Radiol. 48(7):543-7, 2013
O'Neill BR et al: Rapid sequence magnetic resonance imaging in the assessment of children with hydrocephalus. World Neurosurg. 80(6):e307-12, 2013
Rush ET et al: Four new patients with Gomez-Lopez-Hernandez syndrome and proposed diagnostic criteria. Am J Med Genet A. 161A(2):320-6, 2013
Gallo P et al: The endoscopic trans-fourth ventricle aqueductoplasty and stent placement for the treatment of trapped fourth ventricle: long-term results in a series of 18 consecutive patients. Neurol India. 60(3):271-7, 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
Schroeder C et al: Why does endoscopic aqueductoplasty fail so frequently? Analysis of cerebrospinal fluid flow after endoscopic third ventriculostomy and aqueductoplasty using cine phase-contrast magnetic resonance imaging. J Neurosurg. 117(1):141-9, 2012
Cinalli G et al: Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 27(10):1621-42, 2011
Algin O et al: Phase-contrast MRI and 3D-CISS versus contrast-enhanced MR cisternography on the evaluation of the aqueductal stenosis. Neuroradiology. 52(2):99-108, 2010
Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009
Bateman GA: Magnetic resonance imaging quantification of compliance and collateral flow in late-onset idiopathic aqueductal stenosis: venous pathophysiology revisited. J Neurosurg. 107(5):951-8, 2007
da Silva LR et al: Endoscopic aqueductoplasty in the treatment of aqueductal stenosis. Childs Nerv Syst. 23(11):1263-8, 2007
Koch-Wiewrodt D et al: Success and failure of endoscopic third ventriculostomy in young infants: are there different age distributions?. Childs Nerv Syst. 22(12):1537-41, 2006
Sansone JM et al: Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. 103(5 Suppl):388-92, 2005
Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003
Tisell M et al: Neurological symptoms and signs in adult aqueductal stenosis. Acta Neurol Scand. 107(5):311-7, 2003
Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001
Schroeder HW et al: Endoscopic aqueductoplasty: technique and results. Neurosurgery. 45(3):508-15; discussion 515-8, 1999
Graf WD et al: The pachygyria-polymicrogyria spectrum of cortical dysplasia in X-linked hydrocephalus. Eur J Pediatr Surg. 8 Suppl 1:10-4, 1998
Castro-Gago M et al: Autosomal recessive hydrocephalus with aqueductal stenosis. Childs Nerv Syst. 12(4):188-91, 1996
Kadowaki C et al: Cine magnetic resonance imaging of aqueductal stenosis. Childs Nerv Syst. 11(2):107-11, 1995
Villani R et al: Long-term outcome in aqueductal stenosis. Childs Nerv Syst. 11(3):180-5, 1995
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KEY FACTS
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TERMINOLOGY
Abbreviations
Aqueductal stenosis (AS)
Definitions
Ventriculomegaly involving lateral and 3rd ventricles as result of complete or partial obstruction to CSF flow within cerebral aqueduct
AS diagnosis generally refers to congenital form characterized by varying degree of obstruction at level of cerebral aqueduct
However, tumors, germinal matrix hemorrhage, or vascular lesions can obstruct aqueduct as well
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
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):942-9, 2018
Kline-Fath BM et al: Congenital aqueduct stenosis: progressive brain findings in utero to birth in the presence of severe hydrocephalus. Prenat Diagn. 38(9):706-12, 2018
Tonetti DA et al: Clinical outcomes of isolated congenital aqueductal stenosis. World Neurosurg. 114:e976-81, 2018
Yamada S et al: Current and emerging MR imaging techniques for the diagnosis and management of CSF flow disorders: a review of phase-contrast and time-spatial labeling inversion pulse. AJNR Am J Neuroradiol. 36(4):623-30, 2015
Griessenauer CJ et al: Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 13(1):13-20, 2014
Kartal MG et al: Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: an update. Insights Imaging. 5(4):531-41, 2014
Tully HM et al: Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 57(8):359-68, 2014
Ucar M et al: Evaluation of aqueductal patency in patients with hydrocephalus: three-dimensional high-sampling-efficiency technique (SPACE) versus two-dimensional turbo spin echo at 3 Tesla. Korean J Radiol. 15(6):827-35, 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
Muehlmann M et al: Magnetic resonance-based estimation of intracranial pressure correlates with ventriculoperitoneal shunt valve opening pressure setting in children with hydrocephalus. Invest Radiol. 48(7):543-7, 2013
O'Neill BR et al: Rapid sequence magnetic resonance imaging in the assessment of children with hydrocephalus. World Neurosurg. 80(6):e307-12, 2013
Rush ET et al: Four new patients with Gomez-Lopez-Hernandez syndrome and proposed diagnostic criteria. Am J Med Genet A. 161A(2):320-6, 2013
Gallo P et al: The endoscopic trans-fourth ventricle aqueductoplasty and stent placement for the treatment of trapped fourth ventricle: long-term results in a series of 18 consecutive patients. Neurol India. 60(3):271-7, 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
Schroeder C et al: Why does endoscopic aqueductoplasty fail so frequently? Analysis of cerebrospinal fluid flow after endoscopic third ventriculostomy and aqueductoplasty using cine phase-contrast magnetic resonance imaging. J Neurosurg. 117(1):141-9, 2012
Cinalli G et al: Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 27(10):1621-42, 2011
Algin O et al: Phase-contrast MRI and 3D-CISS versus contrast-enhanced MR cisternography on the evaluation of the aqueductal stenosis. Neuroradiology. 52(2):99-108, 2010
Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009
Bateman GA: Magnetic resonance imaging quantification of compliance and collateral flow in late-onset idiopathic aqueductal stenosis: venous pathophysiology revisited. J Neurosurg. 107(5):951-8, 2007
da Silva LR et al: Endoscopic aqueductoplasty in the treatment of aqueductal stenosis. Childs Nerv Syst. 23(11):1263-8, 2007
Koch-Wiewrodt D et al: Success and failure of endoscopic third ventriculostomy in young infants: are there different age distributions?. Childs Nerv Syst. 22(12):1537-41, 2006
Sansone JM et al: Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. 103(5 Suppl):388-92, 2005
Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003
Tisell M et al: Neurological symptoms and signs in adult aqueductal stenosis. Acta Neurol Scand. 107(5):311-7, 2003
Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001