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[Malformations of the central nervous system: analysis of 157 pediatric autopsies]
L De Noronha1, F Medeiros, V D Martins
1Departamento de Patologia Médica, Universidade Federal do Paraná, Curitiba, Brasil. luno@uol.com.br
Insights
Central nervous system malformations occurred in 2.69% of pediatric autopsies. Neural tube defects, including anencephaly and myeloencephaloceles, were the most common congenital anomalies.
Area of Science:
- Neurology
- Developmental Biology
- Pathology
Context:
- Central nervous system (CNS) malformations are a significant concern in pediatric health.
- Previous studies have documented the prevalence and types of these congenital anomalies.
Purpose:
- To analyze the prevalence and spectrum of central nervous system malformations in a large pediatric autopsy cohort.
- To identify the most frequent types of CNS malformations and their distribution.
Summary:
- This study reviewed 5837 pediatric autopsies from 1960-1995, identifying 157 cases (2.69%) of CNS malformations.
- Neural tube defects were most common (61%), including anencephaly and myeloencephaloceles.
- Other significant findings included prosencephalic (8%) and posterior fossa malformations (3%), with higher mortality in the neonatal period.
Impact:
- Provides epidemiological data on CNS malformations over a 35-year period.
- Highlights the burden of neural tube defects and other structural brain anomalies in neonates.
- Informs public health strategies and clinical management of congenital neurological disorders.
Abstract:
The malformations of the central nervous system affect about 5 to 10 children per 1000 births. We studied the central nervous system malformations in 5837 pediatric autopsies performed in the Sector of Anatomic Pathology, Hospital de Clínicas-UFPR, between 1960 and 1995. There were 157 central nervous system malformations (2.69%), the commonest were neural tube defects (61%): 47 cases of anencephaly and 45 cases classified in the group of myeloencephaloceles. The anomalies of the prosencephalic evagination corresponded to 8% of all central nervous system malformations, with seven cases of holoprosencephaly. Posterior fossa malformations occurred in 3%, with three cases of Arnold-Chiari. In the present study, the mortality rate due to central nervous system malformations was higher in the neonatal period.