LONG-TERM STUDIES OF METOPIC SYNOSTOSIS - FREQUENCY OF COGNITIVE IMPAIRMENT AND BEHAVIORAL DISTURBANCES

Although the occurrence of cognitive impairment and behavioral disturb ances in patients with metopic synostosis has been described, the inci dence of this dysfunction has not been established. The records of 36 consecutive children with metopic synostosis followed at one craniofac ial center from 1978 to 1993 were reviewed and parental questionnaires were completed to establish the frequency of mental retardation, lear ning disabilities, and behavioral problems associated with this synost osis. Documentation of syndromes, abnormal karyotype, and central nerv ous system anomalies also tvas done. The study group consisted of 27 m ales and 9 females. The average age at most recent follow-up was 7 yea rs and 1 month (range 6 months to 22 years). Two patients had chromoso mal abnormalities (9p syndrome and trisomy 21). On the basis of CT and MRI scans, intracranial anomalies were identified for only one patien t having an absent corpus callosum. Thirty-two of the study patients h ad adequate information for longitudinal assessment. Twenty patients h ave normal development without apparent disability. Of these, those of school age are at appropriate grade level. Eight patients have mild t o moderate learning disabilities or behavioral problems, including att ention deficit/hyperactivity disorder and impaired language developmen t. Four patients have significant mental impairment. Impaired cognitiv e development was not limited to children with abnormal karyotype or c entral 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 dev elopmental and neurologic evaluation for infants with metopic synostos is.