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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
- Gray matter-lined cleft extending from brain surface to ventricle
- Closed lip (type 1)
- Parallel gray matter lips in contact with each other
- Open lip (type 2)
- Gray matter lips separated by intervening cerebral spinal fluid (CSF)
- CSF extends to underlying ventricle
- Gray matter lips separated by intervening cerebral spinal fluid (CSF)
- Closed lip (type 1)
- Gray matter-lined cleft extending from brain surface to ventricle
IMAGING
General Features
Ultrasonographic Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Shannon P et al: Brain and placental pathology in fetal COL4A1 related disease. Pediatr Dev Pathol. 1093526620984083, 2021
- Maurice P et al: COL4A1 and COL4A2 mutations: when to test a fetus? Ultrasound Obstet Gynecol. ePub, 2020
- Braga VL et al: Schizencephaly: a review of 734 patients. Pediatr Neurol. 87:23-9, 2018
- Griffiths PD: Schizencephaly revisited. Neuroradiology. 60(9):945-60, 2018
- Khalid R et al: COL4A1 and fetal vascular origins of schizencephaly. Neurology. 90(5):232-4, 2018
- Rege SV et al: Bilateral giant open-lip schizencephaly: a rare case report. J Pediatr Neurosci. 11(2):128-30, 2016
- Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
- Kutuk MS et al: Prenatal diagnosis and postnatal outcome of schizencephaly. J Child Neurol. 30(10):1388-94, 2014
- Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol. 35(7):1418-24, 2014
- Lee W et al: Prenatal diagnostic challenges and pitfalls for schizencephaly. J Ultrasound Med. 28(10):1379-84, 2009
- Oh KY et al: Fetal schizencephaly: pre- and postnatal imaging with a review of the clinical manifestations. Radiographics. 25(3):647-57, 2005
- Packard AM et al: Schizencephaly: correlations of clinical and radiologic features. Neurology. 48(5):1427-34, 1997
- Barkovich AJ et al: Schizencephaly: correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol. 13(1):85-94, 1992
- Komarniski CA et al: Prenatal diagnosis of schizencephaly. J Ultrasound Med. 9(5):305-7, 1990
- Barkovich AJ et al: MR imaging of schizencephaly. AJR Am J Roentgenol. 150(6):1391-6, 1988
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
- Gray matter-lined cleft extending from brain surface to ventricle
- Closed lip (type 1)
- Parallel gray matter lips in contact with each other
- Open lip (type 2)
- Gray matter lips separated by intervening cerebral spinal fluid (CSF)
- CSF extends to underlying ventricle
- Gray matter lips separated by intervening cerebral spinal fluid (CSF)
- Closed lip (type 1)
- Gray matter-lined cleft extending from brain surface to ventricle
IMAGING
General Features
Ultrasonographic Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Shannon P et al: Brain and placental pathology in fetal COL4A1 related disease. Pediatr Dev Pathol. 1093526620984083, 2021
- Maurice P et al: COL4A1 and COL4A2 mutations: when to test a fetus? Ultrasound Obstet Gynecol. ePub, 2020
- Braga VL et al: Schizencephaly: a review of 734 patients. Pediatr Neurol. 87:23-9, 2018
- Griffiths PD: Schizencephaly revisited. Neuroradiology. 60(9):945-60, 2018
- Khalid R et al: COL4A1 and fetal vascular origins of schizencephaly. Neurology. 90(5):232-4, 2018
- Rege SV et al: Bilateral giant open-lip schizencephaly: a rare case report. J Pediatr Neurosci. 11(2):128-30, 2016
- Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
- Kutuk MS et al: Prenatal diagnosis and postnatal outcome of schizencephaly. J Child Neurol. 30(10):1388-94, 2014
- Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol. 35(7):1418-24, 2014
- Lee W et al: Prenatal diagnostic challenges and pitfalls for schizencephaly. J Ultrasound Med. 28(10):1379-84, 2009
- Oh KY et al: Fetal schizencephaly: pre- and postnatal imaging with a review of the clinical manifestations. Radiographics. 25(3):647-57, 2005
- Packard AM et al: Schizencephaly: correlations of clinical and radiologic features. Neurology. 48(5):1427-34, 1997
- Barkovich AJ et al: Schizencephaly: correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol. 13(1):85-94, 1992
- Komarniski CA et al: Prenatal diagnosis of schizencephaly. J Ultrasound Med. 9(5):305-7, 1990
- Barkovich AJ et al: MR imaging of schizencephaly. AJR Am J Roentgenol. 150(6):1391-6, 1988
