link
Bookmarks
Encephalomalacia, Porencephaly
Anne M. Kennedy, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
14
2
5

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Encephalomalacia
                • Regional brain parenchymal damage
              • Porencephaly
                • Focal cavitary lesion in communication with ventricle
              • Etymology (all from Greek)
                • Encephalo = brain, malacia = softening, leuko = white, clasto = broken
                • Encephalomalacia = softening of brain
                • Periventricular leukomalacia = death of white matter near ventricles

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Giorgio E et al: Two families with novel missense mutations in COL4A1: When diagnosis can be missed. J Neurol Sci. 352(1-2):99-104, 2015
                                      2. Takenouchi T et al: Porencephaly in a fetus and HANAC in her father: variable expression of COL4A1 mutation. Am J Med Genet A. 167A(1):156-8, 2015
                                      3. Williams T et al: Antenatal diagnosis of intracranial haemorrhage and porencephalic cyst. BMJ Case Rep. 2015, 2015
                                      4. Bennett-Back O et al: Magnetoencephalography helps delineate the extent of the epileptogenic zone for surgical planning in children with intractable epilepsy due to porencephalic cyst/encephalomalacia. J Neurosurg Pediatr. 14(3):271-8, 2014
                                      5. Colin E et al: Fetal intracerebral hemorrhage and cataract: think COL4A1. J Perinatol. 34(1):75-7, 2014
                                      6. 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
                                      7. Spruijt M et al: Cerebral injury in twin-twin transfusion syndrome treated with fetoscopic laser surgery. Obstet Gynecol. 120(1):15-20, 2012
                                      8. Vanderbilt DL et al: Prevalence and risk factors of cerebral lesions in neonates after laser surgery for twin-twin transfusion syndrome. Am J Obstet Gynecol. 207(4):320.e1-6, 2012
                                      9. Kokkinos V et al: Hemispherotomy or lobectomy? The role of presurgical neuroimaging in a young case of a large porencephalic cyst with intractable epilepsy. Pediatr Neurosurg. 47(3):204-9, 2011
                                      10. Gul A et al: Prenatal diagnosis of porencephaly secondary to maternal carbon monoxide poisoning. Arch Gynecol Obstet. 279(5):697-700, 2009
                                      11. Aygun C et al: Diffuse multicystic encephalomalacia in a preterm baby due to homozygous methylenetetrahydrofolate reductase 677 C-->T mutation. J Child Neurol. 23(6):695-8, 2008
                                      12. Kim DH et al: Diffusion-weighted imaging of the fetal brain in vivo. Magn Reson Med. 59(1):216-20, 2008
                                      13. Lee YM et al: Twin chorionicity and the risk of stillbirth. Obstet Gynecol. 2008 Feb;111(2 Pt 1):301-8. Erratum in: Obstet Gynecol. 111(5):1217, 2008
                                      14. Ehehalt S et al: Prenatal multicystic encephalomalacia due to anomaly of the aortic arch. Pediatr Neurol. 37(1):67-9, 2007
                                      15. Barkovich AJ: Congenital malformations of the brain and skull. In Pediatric Neuroimaging. Philadelphia: Lippincott Williams and Wilkins. 291-439, 2005
                                      16. Garel C et al: Contribution of fetal MR imaging in the evaluation of cerebral ischemic lesions. AJNR Am J Neuroradiol. 25(9):1563-8, 2004
                                      17. de Laveaucoupet J et al: Fetal magnetic resonance imaging (MRI) of ischemic brain injury. Prenat Diagn. 21(9):729-36, 2001
                                      18. Thauvin-Robinet C et al: Familial orofaciodigital syndrome type I revealed by ultrasound prenatal diagnosis of porencephaly. Prenat Diagn. 21(6):466-70, 2001
                                      19. Nowaczyk MJ et al: Antenatal and postnatal findings in encephalocraniocutaneous lipomatosis. Am J Med Genet. 91(4):261-6, 2000
                                      20. Koch CA et al: Fenestration of porencephalic cysts to the lateral ventricle: experience with a new technique for treatment of seizures. Surg Neurol. 49(5):524-32; discussion 532-3, 1998
                                      21. Bealer JF et al: The incidence and spectrum of neurological injury after open fetal surgery. J Pediatr Surg. 30(8):1150-4, 1995
                                      22. Eller KM et al: Porencephaly secondary to fetal trauma during amniocentesis. Obstet Gynecol. 85(5 Pt 2):865-7, 1995
                                      23. Erasmus C et al: Infantile multicystic encephalomalacia after maternal bee sting anaphylaxis during pregnancy. Arch Dis Child. 57(10):785-7, 1982
                                      Related Anatomy
                                      Loading...
                                      Related Differential Diagnoses
                                      Loading...
                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Definitions

                                                  • Encephalomalacia
                                                    • Regional brain parenchymal damage
                                                  • Porencephaly
                                                    • Focal cavitary lesion in communication with ventricle
                                                  • Etymology (all from Greek)
                                                    • Encephalo = brain, malacia = softening, leuko = white, clasto = broken
                                                    • Encephalomalacia = softening of brain
                                                    • Periventricular leukomalacia = death of white matter near ventricles

                                                IMAGING

                                                • General Features

                                                  • Ultrasonographic Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Giorgio E et al: Two families with novel missense mutations in COL4A1: When diagnosis can be missed. J Neurol Sci. 352(1-2):99-104, 2015
                                                                          2. Takenouchi T et al: Porencephaly in a fetus and HANAC in her father: variable expression of COL4A1 mutation. Am J Med Genet A. 167A(1):156-8, 2015
                                                                          3. Williams T et al: Antenatal diagnosis of intracranial haemorrhage and porencephalic cyst. BMJ Case Rep. 2015, 2015
                                                                          4. Bennett-Back O et al: Magnetoencephalography helps delineate the extent of the epileptogenic zone for surgical planning in children with intractable epilepsy due to porencephalic cyst/encephalomalacia. J Neurosurg Pediatr. 14(3):271-8, 2014
                                                                          5. Colin E et al: Fetal intracerebral hemorrhage and cataract: think COL4A1. J Perinatol. 34(1):75-7, 2014
                                                                          6. 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
                                                                          7. Spruijt M et al: Cerebral injury in twin-twin transfusion syndrome treated with fetoscopic laser surgery. Obstet Gynecol. 120(1):15-20, 2012
                                                                          8. Vanderbilt DL et al: Prevalence and risk factors of cerebral lesions in neonates after laser surgery for twin-twin transfusion syndrome. Am J Obstet Gynecol. 207(4):320.e1-6, 2012
                                                                          9. Kokkinos V et al: Hemispherotomy or lobectomy? The role of presurgical neuroimaging in a young case of a large porencephalic cyst with intractable epilepsy. Pediatr Neurosurg. 47(3):204-9, 2011
                                                                          10. Gul A et al: Prenatal diagnosis of porencephaly secondary to maternal carbon monoxide poisoning. Arch Gynecol Obstet. 279(5):697-700, 2009
                                                                          11. Aygun C et al: Diffuse multicystic encephalomalacia in a preterm baby due to homozygous methylenetetrahydrofolate reductase 677 C-->T mutation. J Child Neurol. 23(6):695-8, 2008
                                                                          12. Kim DH et al: Diffusion-weighted imaging of the fetal brain in vivo. Magn Reson Med. 59(1):216-20, 2008
                                                                          13. Lee YM et al: Twin chorionicity and the risk of stillbirth. Obstet Gynecol. 2008 Feb;111(2 Pt 1):301-8. Erratum in: Obstet Gynecol. 111(5):1217, 2008
                                                                          14. Ehehalt S et al: Prenatal multicystic encephalomalacia due to anomaly of the aortic arch. Pediatr Neurol. 37(1):67-9, 2007
                                                                          15. Barkovich AJ: Congenital malformations of the brain and skull. In Pediatric Neuroimaging. Philadelphia: Lippincott Williams and Wilkins. 291-439, 2005
                                                                          16. Garel C et al: Contribution of fetal MR imaging in the evaluation of cerebral ischemic lesions. AJNR Am J Neuroradiol. 25(9):1563-8, 2004
                                                                          17. de Laveaucoupet J et al: Fetal magnetic resonance imaging (MRI) of ischemic brain injury. Prenat Diagn. 21(9):729-36, 2001
                                                                          18. Thauvin-Robinet C et al: Familial orofaciodigital syndrome type I revealed by ultrasound prenatal diagnosis of porencephaly. Prenat Diagn. 21(6):466-70, 2001
                                                                          19. Nowaczyk MJ et al: Antenatal and postnatal findings in encephalocraniocutaneous lipomatosis. Am J Med Genet. 91(4):261-6, 2000
                                                                          20. Koch CA et al: Fenestration of porencephalic cysts to the lateral ventricle: experience with a new technique for treatment of seizures. Surg Neurol. 49(5):524-32; discussion 532-3, 1998
                                                                          21. Bealer JF et al: The incidence and spectrum of neurological injury after open fetal surgery. J Pediatr Surg. 30(8):1150-4, 1995
                                                                          22. Eller KM et al: Porencephaly secondary to fetal trauma during amniocentesis. Obstet Gynecol. 85(5 Pt 2):865-7, 1995
                                                                          23. Erasmus C et al: Infantile multicystic encephalomalacia after maternal bee sting anaphylaxis during pregnancy. Arch Dis Child. 57(10):785-7, 1982