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Schizencephaly revisited
1Academic Unit of Radiology, University of Sheffield, Floor C, Glossop Road, Sheffield, England, S10 2JF, UK. p.griffiths@sheffield.ac.uk.
Insights
This study reviews 32 cases of schizencephaly in children and fetuses, proposing a new classification system and suggesting an acquired destructive cause for this brain malformation.
Area of Science:
- Neuroscience
- Developmental Biology
- Pediatric Neurology
Background:
- Schizencephaly is a rare congenital brain malformation characterized by a cleft in the cerebral hemisphere.
- Understanding its diverse presentations and underlying causes is crucial for diagnosis and management.
Purpose of the Study:
- To report the range of schizencephaly appearances in pediatric and fetal cases over a 10-year period.
- To detail classification systems for different forms of schizencephaly.
- To re-assess potential etiological and mechanistic causes.
Main Methods:
- A retrospective review of 21 children and 11 fetuses diagnosed with schizencephaly between 2007 and 2016.
- Analysis of schizencephaly type, location, and associated brain abnormalities, including the septum pellucidum and fornices.
Main Results:
- Schizencephaly type 2 was the most common form in children (67%) and fetuses (45%).
- Type 3 was more prevalent in fetuses (55%) than children (24%).
- Associated brain abnormalities were frequent, present in 67% of children and 55% of fetuses.
Conclusions:
- A novel classification system for schizencephaly is proposed, integrating literature definitions.
- The study describes the appearances and associations of pediatric and fetal schizencephaly.
- Evidence suggests an acquired destructive etiology for most cases, with a proposed mechanism for associated cortical abnormalities.
Purpose:
In this paper, I will report the range of appearances of schizencephaly in children and fetuses by reviewing a 10-year experience from a single centre and detail classification systems for the different forms of schizencephaly. This will lead to re-assessment of possible aetiological and mechanistic causes of schizencephaly.
Methods:
All cases of pediatric and fetal schizencephaly were located on the local database between 2007 and 2016 inclusive. The studies were reviewed for the presence, location and type of schizencephaly, as well as the state of the (cavum) septum pellucidum, the location of the fornices and the presence of other brain abnormalities.
Results:
The review included 21 children and 11 fetuses with schizencephaly. Schizencephaly (type 1) was found in 9% of children but no fetuses, schizencephaly (type 2) was present in 67% of the pediatric cases and in 45% of fetuses, whilst schizencephaly (type 3) was present in approximately 24% of children and 55% of fetuses. Other brain abnormalities were found in 67% of children and 55% of fetuses.
Conclusion:
I have proposed a new system for classifying schizencephaly that takes into account all definitions of the abnormality in the literature. Using that approach, I have described the appearances and associations of pediatric and fetal cases of schizencephaly from a single centre. Review of the current literature appears to favour an acquired destructive aetiology for most cases of schizencephaly, and I have proposed a mechanism to explain the cortical formation abnormalities found consistently in and around areas of schizencephaly.
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