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Porencephalic Cyst
Gregory L. Katzman, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Porencephaly
            • Definitions

              • Various definitions of porencephaly
                • Congenital/acquired CSF-filled cavity that usually communicates with ventricular system
                  • Deep, unilateral/bilateral cavities or excavations
                  • Lined by reactive gliosis/astrocytic proliferation
                • Presence of cysts/cavities in brain parenchyma
                  • Communicating by "pore" with arachnoid space
                • Cavities arising in fetal life or early infancy
                  • Brain destruction during perinatal period
                  • Frequently communicate with subarachnoid space &/or lateral ventricles

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nonvascular Interventions

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Colin E et al: Fetal intracerebral hemorrhage and cataract: think COL4A1. J Perinatol. 34(1):75-7, 2014
                                              2. Kutuk MS et al: Is there relation between COL4A1/A2 mutations and antenatally detected fetal intraventricular hemorrhage? Childs Nerv Syst. 30(3):419-24, 2014
                                              3. Numata Y et al: Brain magnetic resonance imaging and motor and intellectual functioning in 86 patients born at term with spastic diplegia. Dev Med Child Neurol. 55(2):167-72, 2013
                                              4. Yoneda Y et al: Phenotypic spectrum of COL4A1 mutations: porencephaly to schizencephaly. Ann Neurol. 73(1):48-57, 2013
                                              5. Verbeek E et al: COL4A2 mutation associated with familial porencephaly and small-vessel disease. Eur J Hum Genet. 20(8):844-51, 2012
                                              6. Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
                                              7. Vermeulen RJ et al: Fetal origin of brain damage in 2 infants with a COL4A1 mutation: fetal and neonatal MRI. Neuropediatrics. 2011 Feb;42(1):1-3. Epub 2011 Apr 15. Erratum in: Neuropediatrics. 42(2):e1, 2011
                                              8. de Vries LS et al: COL4A1 mutation in two preterm siblings with antenatal onset of parenchymal hemorrhage. Ann Neurol. 65(1):12-8, 2009
                                              9. Gul A et al: Prenatal diagnosis of porencephaly secondary to maternal carbon monoxide poisoning. Arch Gynecol Obstet. 279(5):697-700, 2009
                                              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

                                                        • Synonyms

                                                          • Porencephaly
                                                        • Definitions

                                                          • Various definitions of porencephaly
                                                            • Congenital/acquired CSF-filled cavity that usually communicates with ventricular system
                                                              • Deep, unilateral/bilateral cavities or excavations
                                                              • Lined by reactive gliosis/astrocytic proliferation
                                                            • Presence of cysts/cavities in brain parenchyma
                                                              • Communicating by "pore" with arachnoid space
                                                            • Cavities arising in fetal life or early infancy
                                                              • Brain destruction during perinatal period
                                                              • Frequently communicate with subarachnoid space &/or lateral ventricles

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Nonvascular Interventions

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Colin E et al: Fetal intracerebral hemorrhage and cataract: think COL4A1. J Perinatol. 34(1):75-7, 2014
                                                                                          2. Kutuk MS et al: Is there relation between COL4A1/A2 mutations and antenatally detected fetal intraventricular hemorrhage? Childs Nerv Syst. 30(3):419-24, 2014
                                                                                          3. Numata Y et al: Brain magnetic resonance imaging and motor and intellectual functioning in 86 patients born at term with spastic diplegia. Dev Med Child Neurol. 55(2):167-72, 2013
                                                                                          4. Yoneda Y et al: Phenotypic spectrum of COL4A1 mutations: porencephaly to schizencephaly. Ann Neurol. 73(1):48-57, 2013
                                                                                          5. Verbeek E et al: COL4A2 mutation associated with familial porencephaly and small-vessel disease. Eur J Hum Genet. 20(8):844-51, 2012
                                                                                          6. Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
                                                                                          7. Vermeulen RJ et al: Fetal origin of brain damage in 2 infants with a COL4A1 mutation: fetal and neonatal MRI. Neuropediatrics. 2011 Feb;42(1):1-3. Epub 2011 Apr 15. Erratum in: Neuropediatrics. 42(2):e1, 2011
                                                                                          8. de Vries LS et al: COL4A1 mutation in two preterm siblings with antenatal onset of parenchymal hemorrhage. Ann Neurol. 65(1):12-8, 2009
                                                                                          9. Gul A et al: Prenatal diagnosis of porencephaly secondary to maternal carbon monoxide poisoning. Arch Gynecol Obstet. 279(5):697-700, 2009