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Long-term studies of metopic synostosis: frequency of cognitive impairment and behavioral disturbances
E J Sidoti1, J L Marsh, L Marty-Grames
1Division of Plastic and Reconstructive Surgery, St. Louis Children's Hospital, Mo, USA.
Insights
Metopic synostosis, a skull malformation, is linked to cognitive and behavioral issues in at least one-third of affected children. Early developmental and neurological evaluations are crucial for early detection and intervention.
Area of Science:
- Pediatric Neurosurgery
- Developmental Pediatrics
- Genetics
Background:
- Metopic synostosis is a premature fusion of the metopic suture, potentially impacting brain development.
- Previous studies have suggested a link between metopic synostosis and neurodevelopmental deficits, but incidence data is limited.
Purpose of the Study:
- To determine the frequency of cognitive impairment and behavioral disturbances in children with metopic synostosis.
- To identify associated factors such as chromosomal abnormalities and central nervous system anomalies.
Main Methods:
- Retrospective review of 36 children with metopic synostosis treated at a craniofacial center.
- Parental questionnaires to assess developmental outcomes, learning disabilities, and behavioral problems.
- Documentation of genetic and neurological findings.
Main Results:
- Cognitive and behavioral abnormalities were identified in at least 33% of patients.
- Learning disabilities, behavioral problems (e.g., ADHD), and significant mental impairment were observed.
- Impaired cognitive development occurred even in patients without chromosomal or CNS anomalies.
Conclusions:
- Cognitive and behavioral abnormalities are common in metopic synostosis, occurring in at least one-third of cases.
- These abnormalities can be subtle, necessitating ongoing developmental and neurological monitoring.
- Longitudinal evaluation is essential for early identification and management of neurodevelopmental issues in infants with metopic synostosis.
Abstract:
Although the occurrence of cognitive impairment and behavioral disturbances in patients with metopic synostosis has been described, the incidence of this dysfunction has not been established. The records of 36 consecutive children with metopic synostosis followed at one craniofacial center from 1978 to 1993 were reviewed and parental questionnaires were completed to establish the frequency of mental retardation, learning disabilities, and behavioral problems associated with this synostosis. Documentation of syndromes, abnormal karyotype, and central nervous system anomalies also was done. The study group consisted of 27 males and 9 females. The average age at most recent follow-up was 7 years and 1 month (range 6 months to 22 years). Two patients had chromosomal abnormalities (9p syndrome and trisomy 21). On the basis of CT and MRI scans, intracranial anomalies were identified for only one patient having an absent corpus callosum. Thirty-two of the study patients had adequate information for longitudinal assessment. Twenty patients have normal development without apparent disability. Of these, those of school age are at appropriate grade level. Eight patients have mild to moderate learning disabilities or behavioral problems, including attention deficit/hyperactivity disorder and impaired language development. Four patients have significant mental impairment. Impaired cognitive development was not limited to children with abnormal karyotype or central nervous system anomaly. Cognitive and behavioral abnormalities occur in at least a third of patients with metopic synostosis. The, at times, subtle nature of these abnormalities mandates longitudinal developmental and neurologic evaluation for infants with metopic synostosis.