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Hydranencephaly
Anna Illner, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • In utero cerebral hemispheric destruction
                • Hemihydranencephaly: Rare unilateral form

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Other Modality Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
                                            2. Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
                                            3. Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
                                            4. Bae JS et al: Prolonged survival to adulthood of an individual with hydranencephaly. Clin Neurol Neurosurg. 110(3):307-9, 2008
                                            5. Merker B: Life expectancy in hydranencephaly. Clin Neurol Neurosurg. 110(3):213-4, 2008
                                            6. Quek YW et al: Hydranencephaly associated with interruption of bilateral internal carotid arteries. Pediatr Neonatol. 49(2):43-7, 2008
                                            7. Tsai JD et al: Hydranencephaly in neonates. Pediatr Neonatol. 49(4):154-7, 2008
                                            8. Vercauteren G et al: Bluetongue virus serotype 8-associated congenital hydranencephaly in calves. Transbound Emerg Dis. 55(7):293-8, 2008
                                            9. Counter SA: Preservation of brainstem neurophysiological function in hydranencephaly. J Neurol Sci. 263(1-2):198-207, 2007
                                            10. Segawa Y et al: A case of partial 14q- with facial features of holoprosencephaly and hydranencephaly. Pediatr Neurol. 37(1):51-4, 2007
                                            11. Mittelbronn M et al: Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria. Hum Pathol. 37(11):1503-7, 2006
                                            12. Altschuler EL et al: Left-hemispheric hydranencephaly with less favorable findings. Pediatrics. 116(6):1603-4; author reply 1604, 2005
                                            13. Ulmer S et al: Living a normal life with the nondominant hemisphere: magnetic resonance imaging findings and clinical outcome for a patient with left-hemispheric hydranencephaly. Pediatrics. 116(1):242-5, 2005
                                            14. Usta IM et al: Early ultrasonographic changes in Fowler syndrome features and review of the literature. Prenat Diagn. 25(11):1019-23, 2005
                                            15. Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
                                            16. Hahn JS et al: Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings. J Child Neurol. 18(5):367-70, 2003
                                            17. Hahn JS et al: Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings. J Child Neurol. 18(5):367-70, 2003
                                            18. Laurichesse-Delmas H et al: First-trimester features of Fowler syndrome (hydrocephaly-hydranencephaly proliferative vasculopathy). Ultrasound Obstet Gynecol. 20(6):612-5, 2002
                                            19. Witters I et al: Two siblings with early onset fetal akinesia deformation sequence and hydranencephaly: further evidence for autosomal recessive inheritance of hydranencephaly, fowler type. Am J Med Genet. 108(1):41-4, 2002
                                            20. Greco F et al: Hemihydranencephaly: case report and literature review. J Child Neurol. 16(3):218-21, 2001
                                            21. Stevenson DA et al: Hydranencephaly in an infant with vascular malformations. Am J Med Genet. 104(4):295-8, 2001
                                            22. Kavaslar GN et al: The novel genetic disorder microhydranencephaly maps to chromosome 16p13.3-12.1. Am J Hum Genet. 66(5):1705-9, 2000
                                            23. Lam YH et al: Serial sonographic features of a fetus with hydranencephaly from 11 weeks to term. Ultrasound Obstet Gynecol. 16(1):77-9, 2000
                                            24. McAbee GN et al: Prolonged survival with hydranencephaly: report of two patients and literature review. Pediatr Neurol. 23(1):80-4, 2000
                                            25. Gschwendtner A et al: Hydranencephaly with renal dysgenesis: a coincidental finding? Case report with review of the literature. Gynecol Obstet Invest. 44(3):206-10, 1997
                                            26. Lubinsky MS: Association of prenatal vascular disruptions with decreased maternal age. Am J Med Genet. 69(3):237-9, 1997
                                            27. Poe LB et al: MR of hydranencephaly. AJNR Am J Neuroradiol. 10(5 Suppl):S61, 1989
                                            28. Hanigan WC et al: MRI and evoked potentials in a child with hydranencephaly. Pediatr Neurol. 4(3):185-7, 1988
                                            29. Herman DC et al: Ophthalmic findings of hydranencephaly. J Pediatr Ophthalmol Strabismus. 25(3):106-11, 1988
                                            30. Bendon RW et al: Recurrent developmental anomalies: 1. Syndrome of hydranencephaly with renal aplastic dysplasia; 2. Polyvalvular developmental heart defect. Am J Med Genet Suppl. 3:357-65, 1987
                                            31. Greene MF et al: Hydranencephaly: US appearance during in utero evolution. Radiology. 156(3):779-80, 1985
                                            32. Najafzadeh TM et al: Etiologic heterogeneity in hydranencephaly. Birth Defects Orig Artic Ser. 18(3B):229-35, 1982
                                            33. Dublin AB et al: Diagnostic image evaluation of hydranencephaly and pictorially similar entities, with emphasis on computed tomography. Radiology. 137(1 Pt 1):81-91, 1980
                                            34. Sutton LN et al: Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery. 6(1):34-8, 1980
                                            35. Lindenberg R et al: "Infantile hydranencephaly"--a report of five cases of infarction of both cerebral hemispheres in infancy. Brain. 90(4):839-50, 1967
                                            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

                                                        • Definitions

                                                          • In utero cerebral hemispheric destruction
                                                          • Hemihydranencephaly: Rare unilateral form

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Other Modality Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
                                                                                      2. Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
                                                                                      3. Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
                                                                                      4. Bae JS et al: Prolonged survival to adulthood of an individual with hydranencephaly. Clin Neurol Neurosurg. 110(3):307-9, 2008
                                                                                      5. Merker B: Life expectancy in hydranencephaly. Clin Neurol Neurosurg. 110(3):213-4, 2008
                                                                                      6. Quek YW et al: Hydranencephaly associated with interruption of bilateral internal carotid arteries. Pediatr Neonatol. 49(2):43-7, 2008
                                                                                      7. Tsai JD et al: Hydranencephaly in neonates. Pediatr Neonatol. 49(4):154-7, 2008
                                                                                      8. Vercauteren G et al: Bluetongue virus serotype 8-associated congenital hydranencephaly in calves. Transbound Emerg Dis. 55(7):293-8, 2008
                                                                                      9. Counter SA: Preservation of brainstem neurophysiological function in hydranencephaly. J Neurol Sci. 263(1-2):198-207, 2007
                                                                                      10. Segawa Y et al: A case of partial 14q- with facial features of holoprosencephaly and hydranencephaly. Pediatr Neurol. 37(1):51-4, 2007
                                                                                      11. Mittelbronn M et al: Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria. Hum Pathol. 37(11):1503-7, 2006
                                                                                      12. Altschuler EL et al: Left-hemispheric hydranencephaly with less favorable findings. Pediatrics. 116(6):1603-4; author reply 1604, 2005
                                                                                      13. Ulmer S et al: Living a normal life with the nondominant hemisphere: magnetic resonance imaging findings and clinical outcome for a patient with left-hemispheric hydranencephaly. Pediatrics. 116(1):242-5, 2005
                                                                                      14. Usta IM et al: Early ultrasonographic changes in Fowler syndrome features and review of the literature. Prenat Diagn. 25(11):1019-23, 2005
                                                                                      15. Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
                                                                                      16. Hahn JS et al: Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings. J Child Neurol. 18(5):367-70, 2003
                                                                                      17. Hahn JS et al: Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings. J Child Neurol. 18(5):367-70, 2003
                                                                                      18. Laurichesse-Delmas H et al: First-trimester features of Fowler syndrome (hydrocephaly-hydranencephaly proliferative vasculopathy). Ultrasound Obstet Gynecol. 20(6):612-5, 2002
                                                                                      19. Witters I et al: Two siblings with early onset fetal akinesia deformation sequence and hydranencephaly: further evidence for autosomal recessive inheritance of hydranencephaly, fowler type. Am J Med Genet. 108(1):41-4, 2002
                                                                                      20. Greco F et al: Hemihydranencephaly: case report and literature review. J Child Neurol. 16(3):218-21, 2001
                                                                                      21. Stevenson DA et al: Hydranencephaly in an infant with vascular malformations. Am J Med Genet. 104(4):295-8, 2001
                                                                                      22. Kavaslar GN et al: The novel genetic disorder microhydranencephaly maps to chromosome 16p13.3-12.1. Am J Hum Genet. 66(5):1705-9, 2000
                                                                                      23. Lam YH et al: Serial sonographic features of a fetus with hydranencephaly from 11 weeks to term. Ultrasound Obstet Gynecol. 16(1):77-9, 2000
                                                                                      24. McAbee GN et al: Prolonged survival with hydranencephaly: report of two patients and literature review. Pediatr Neurol. 23(1):80-4, 2000
                                                                                      25. Gschwendtner A et al: Hydranencephaly with renal dysgenesis: a coincidental finding? Case report with review of the literature. Gynecol Obstet Invest. 44(3):206-10, 1997
                                                                                      26. Lubinsky MS: Association of prenatal vascular disruptions with decreased maternal age. Am J Med Genet. 69(3):237-9, 1997
                                                                                      27. Poe LB et al: MR of hydranencephaly. AJNR Am J Neuroradiol. 10(5 Suppl):S61, 1989
                                                                                      28. Hanigan WC et al: MRI and evoked potentials in a child with hydranencephaly. Pediatr Neurol. 4(3):185-7, 1988
                                                                                      29. Herman DC et al: Ophthalmic findings of hydranencephaly. J Pediatr Ophthalmol Strabismus. 25(3):106-11, 1988
                                                                                      30. Bendon RW et al: Recurrent developmental anomalies: 1. Syndrome of hydranencephaly with renal aplastic dysplasia; 2. Polyvalvular developmental heart defect. Am J Med Genet Suppl. 3:357-65, 1987
                                                                                      31. Greene MF et al: Hydranencephaly: US appearance during in utero evolution. Radiology. 156(3):779-80, 1985
                                                                                      32. Najafzadeh TM et al: Etiologic heterogeneity in hydranencephaly. Birth Defects Orig Artic Ser. 18(3B):229-35, 1982
                                                                                      33. Dublin AB et al: Diagnostic image evaluation of hydranencephaly and pictorially similar entities, with emphasis on computed tomography. Radiology. 137(1 Pt 1):81-91, 1980
                                                                                      34. Sutton LN et al: Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery. 6(1):34-8, 1980
                                                                                      35. Lindenberg R et al: "Infantile hydranencephaly"--a report of five cases of infarction of both cerebral hemispheres in infancy. Brain. 90(4):839-50, 1967