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Aqueductal Stenosis
Paula J. Woodward, MD
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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

                                  1. Etchegaray A et al: Prenatal genetic considerations in congenital ventriculomegaly and hydrocephalus. Childs Nerv Syst. 36(8):1645-60, 2020
                                  2. Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020
                                  3. 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
                                  4. 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
                                  5. 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
                                  6. Pisapia JM et al: Fetal ventriculomegaly: diagnosis, treatment, and future directions. Childs Nerv Syst. 33(7):1113-23, 2017
                                  7. 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
                                  8. Emery SP et al: Accuracy of prenatal diagnosis of isolated aqueductal stenosis. Prenat Diagn. 35(4):319-24, 2015
                                  9. Emery SP et al: Fetal therapy for isolated aqueductal stenosis. Fetal Diagn Ther. 38(2):81-5, 2015
                                  10. 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
                                  11. 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
                                  12. 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
                                  13. Spennato P et al: Endoscopic third ventriculostomy for idiopathic aqueductal stenosis. World Neurosurg. 79(2 Suppl):S21.e13-20, 2013
                                  14. Sacko O et al: Endoscopic third ventriculostomy: outcome analysis in 368 procedures. J Neurosurg Pediatr. 5(1):68-74, 2010
                                  15. D'Addario V et al: Sonographic diagnosis of fetal cerebral ventriculomegaly: an update. J Matern Fetal Neonatal Med. 20(1):7-14, 2007
                                  16. 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

                                                                  1. Etchegaray A et al: Prenatal genetic considerations in congenital ventriculomegaly and hydrocephalus. Childs Nerv Syst. 36(8):1645-60, 2020
                                                                  2. Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020
                                                                  3. 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
                                                                  4. 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
                                                                  5. 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
                                                                  6. Pisapia JM et al: Fetal ventriculomegaly: diagnosis, treatment, and future directions. Childs Nerv Syst. 33(7):1113-23, 2017
                                                                  7. 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
                                                                  8. Emery SP et al: Accuracy of prenatal diagnosis of isolated aqueductal stenosis. Prenat Diagn. 35(4):319-24, 2015
                                                                  9. Emery SP et al: Fetal therapy for isolated aqueductal stenosis. Fetal Diagn Ther. 38(2):81-5, 2015
                                                                  10. 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
                                                                  11. 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
                                                                  12. 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
                                                                  13. Spennato P et al: Endoscopic third ventriculostomy for idiopathic aqueductal stenosis. World Neurosurg. 79(2 Suppl):S21.e13-20, 2013
                                                                  14. Sacko O et al: Endoscopic third ventriculostomy: outcome analysis in 368 procedures. J Neurosurg Pediatr. 5(1):68-74, 2010
                                                                  15. D'Addario V et al: Sonographic diagnosis of fetal cerebral ventriculomegaly: an update. J Matern Fetal Neonatal Med. 20(1):7-14, 2007
                                                                  16. Kenwrick S et al: X linked hydrocephalus and MASA syndrome. J Med Genet. 33(1):59-65, 1996