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Brain
Diagnosis
Anatomy-Based Diagnoses
Ventricles and Cisterns
Hydrocephalus
Aqueductal Stenosis
Aqueductal Stenosis
Usha D. Nagaraj, MD; Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              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

                                          1. Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020
                                          2. 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
                                          3. 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
                                          4. 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
                                          5. Tonetti DA et al: Clinical outcomes of isolated congenital aqueductal stenosis. World Neurosurg. 114:e976-81, 2018
                                          6. 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
                                          7. 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
                                          8. Kartal MG et al: Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: an update. Insights Imaging. 5(4):531-41, 2014
                                          9. Tully HM et al: Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 57(8):359-68, 2014
                                          10. 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
                                          11. 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
                                          12. 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
                                          13. O'Neill BR et al: Rapid sequence magnetic resonance imaging in the assessment of children with hydrocephalus. World Neurosurg. 80(6):e307-12, 2013
                                          14. 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
                                          15. 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
                                          16. 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
                                          17. 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
                                          18. Cinalli G et al: Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 27(10):1621-42, 2011
                                          19. 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
                                          20. 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
                                          21. 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
                                          22. da Silva LR et al: Endoscopic aqueductoplasty in the treatment of aqueductal stenosis. Childs Nerv Syst. 23(11):1263-8, 2007
                                          23. 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
                                          24. Sansone JM et al: Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. 103(5 Suppl):388-92, 2005
                                          25. 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
                                          26. Tisell M et al: Neurological symptoms and signs in adult aqueductal stenosis. Acta Neurol Scand. 107(5):311-7, 2003
                                          27. Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001
                                          28. Partington MD: Congenital hydrocephalus. Neurosurg Clin N Am. 12(4):737-42, ix, 2001
                                          29. Schroeder HW et al: Endoscopic aqueductoplasty: technique and results. Neurosurgery. 45(3):508-15; discussion 515-8, 1999
                                          30. 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
                                          31. Castro-Gago M et al: Autosomal recessive hydrocephalus with aqueductal stenosis. Childs Nerv Syst. 12(4):188-91, 1996
                                          32. Kadowaki C et al: Cine magnetic resonance imaging of aqueductal stenosis. Childs Nerv Syst. 11(2):107-11, 1995
                                          33. Villani R et al: Long-term outcome in aqueductal stenosis. Childs Nerv Syst. 11(3):180-5, 1995
                                          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

                                                    • Diagnostic Checklist

                                                      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

                                                                                  1. Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020
                                                                                  2. 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
                                                                                  3. 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
                                                                                  4. 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
                                                                                  5. Tonetti DA et al: Clinical outcomes of isolated congenital aqueductal stenosis. World Neurosurg. 114:e976-81, 2018
                                                                                  6. 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
                                                                                  7. 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
                                                                                  8. Kartal MG et al: Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: an update. Insights Imaging. 5(4):531-41, 2014
                                                                                  9. Tully HM et al: Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 57(8):359-68, 2014
                                                                                  10. 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
                                                                                  11. 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
                                                                                  12. 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
                                                                                  13. O'Neill BR et al: Rapid sequence magnetic resonance imaging in the assessment of children with hydrocephalus. World Neurosurg. 80(6):e307-12, 2013
                                                                                  14. 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
                                                                                  15. 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
                                                                                  16. 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
                                                                                  17. 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
                                                                                  18. Cinalli G et al: Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 27(10):1621-42, 2011
                                                                                  19. 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
                                                                                  20. 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
                                                                                  21. 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
                                                                                  22. da Silva LR et al: Endoscopic aqueductoplasty in the treatment of aqueductal stenosis. Childs Nerv Syst. 23(11):1263-8, 2007
                                                                                  23. 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
                                                                                  24. Sansone JM et al: Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. 103(5 Suppl):388-92, 2005
                                                                                  25. 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
                                                                                  26. Tisell M et al: Neurological symptoms and signs in adult aqueductal stenosis. Acta Neurol Scand. 107(5):311-7, 2003
                                                                                  27. Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001
                                                                                  28. Partington MD: Congenital hydrocephalus. Neurosurg Clin N Am. 12(4):737-42, ix, 2001
                                                                                  29. Schroeder HW et al: Endoscopic aqueductoplasty: technique and results. Neurosurgery. 45(3):508-15; discussion 515-8, 1999
                                                                                  30. 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
                                                                                  31. Castro-Gago M et al: Autosomal recessive hydrocephalus with aqueductal stenosis. Childs Nerv Syst. 12(4):188-91, 1996
                                                                                  32. Kadowaki C et al: Cine magnetic resonance imaging of aqueductal stenosis. Childs Nerv Syst. 11(2):107-11, 1995
                                                                                  33. Villani R et al: Long-term outcome in aqueductal stenosis. Childs Nerv Syst. 11(3):180-5, 1995