Aqueductal stenosis (AS): Lateral & 3rd ventriculomegaly due to complete or partial obstruction of CSF flow through cerebral aqueduct
Diagnosis of AS generally refers to congenital form characterized by varying degrees of obstruction at cerebral aqueduct
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
Mirsky DM et al: Imaging of fetal ventriculomegaly. Pediatr Radiol. 50(13):1948-58, 2020
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
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
Severino M et al: MR imaging diagnosis of diencephalic-mesencephalic junction dysplasia in fetuses with developmental ventriculomegaly. AJNR Am J Neuroradiol. 38(8):1643-6, 2017
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
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
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
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
Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001
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TERMINOLOGY
Definitions
Aqueductal stenosis (AS): Lateral & 3rd ventriculomegaly due to complete or partial obstruction of CSF flow through cerebral aqueduct
Diagnosis of AS generally refers to congenital form characterized by varying degrees of obstruction at cerebral aqueduct
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
Mirsky DM et al: Imaging of fetal ventriculomegaly. Pediatr Radiol. 50(13):1948-58, 2020
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
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
Severino M et al: MR imaging diagnosis of diencephalic-mesencephalic junction dysplasia in fetuses with developmental ventriculomegaly. AJNR Am J Neuroradiol. 38(8):1643-6, 2017
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
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
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
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
Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001
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