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Hydranencephaly
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Absence of cerebral hemispheres with intact thalamus, brainstem, cerebellum, skull/meninges
                  • Associated space filled with CSF
                  • Probably caused by in utero vascular occlusion, infection, or trauma after 1st trimester
                • 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. Kim SY et al: Endoscopic coagulation of choroid plexus in hydranencephaly. J Korean Neurosurg Soc. 55(6):375-8, 2014
                                            2. Pavone P et al: Hydranencephaly: cerebral spinal fluid instead of cerebral mantles. Ital J Pediatr. 40(1):79, 2014
                                            3. Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
                                            4. Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
                                            5. Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
                                            6. Bae JS et al: Prolonged survival to adulthood of an individual with hydranencephaly. Clin Neurol Neurosurg. 110(3):307-9, 2008
                                            7. Merker B: Life expectancy in hydranencephaly. Clin Neurol Neurosurg. 110(3):213-4, 2008
                                            8. Quek YW et al: Hydranencephaly associated with interruption of bilateral internal carotid arteries. Pediatr Neonatol. 49(2):43-7, 2008
                                            9. Tsai JD et al: Hydranencephaly in neonates. Pediatr Neonatol. 49(4):154-7, 2008
                                            10. Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
                                            11. Sutton LN et al: Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery. 6(1):34-8, 1980
                                            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

                                                          • Absence of cerebral hemispheres with intact thalamus, brainstem, cerebellum, skull/meninges
                                                            • Associated space filled with CSF
                                                            • Probably caused by in utero vascular occlusion, infection, or trauma after 1st trimester
                                                          • 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. Kim SY et al: Endoscopic coagulation of choroid plexus in hydranencephaly. J Korean Neurosurg Soc. 55(6):375-8, 2014
                                                                                      2. Pavone P et al: Hydranencephaly: cerebral spinal fluid instead of cerebral mantles. Ital J Pediatr. 40(1):79, 2014
                                                                                      3. Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
                                                                                      4. Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
                                                                                      5. Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
                                                                                      6. Bae JS et al: Prolonged survival to adulthood of an individual with hydranencephaly. Clin Neurol Neurosurg. 110(3):307-9, 2008
                                                                                      7. Merker B: Life expectancy in hydranencephaly. Clin Neurol Neurosurg. 110(3):213-4, 2008
                                                                                      8. Quek YW et al: Hydranencephaly associated with interruption of bilateral internal carotid arteries. Pediatr Neonatol. 49(2):43-7, 2008
                                                                                      9. Tsai JD et al: Hydranencephaly in neonates. Pediatr Neonatol. 49(4):154-7, 2008
                                                                                      10. Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
                                                                                      11. Sutton LN et al: Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery. 6(1):34-8, 1980