Pediatric craniofacial surgery and parental attitudes.

A prospective study was designed to explore the effects of attitudes of parents who have children affected with craniofacial deformities. The study focuses on the child's craniofacial deformity and its effect upon the child's mental well being. The effect of surgical reconstruction of the deformity upon these parental attitudes was evaluated as well. The parent actively involved in the child's in- and outpatient care was enrolled in the study (N = 24). The evaluation protocol was administered prior to the surgery and postoperatively at 2 weeks, 3 months, and 1 year. For the majority of these parents, having a child with an obvious facial deformity did not make them less accepting of the children or their childrearing responsibilities. These parents expected the affected children to have positive self-esteem. This was the case for the majority of the children. There was no evidence to suggest that the affected children had a negative impact on the family structure. The child's disfigurement was rarely portrayed in parental drawings of the family, and the affected child could not be distinguished from other siblings. In spite of these positive findings, a majority of older children had significant behavioral problems. The parental attitudes were affected variably and transiently by the event of surgical reconstruction. Most attitudes returned to the preoperative baseline within the year of study.

Hydrocephalus and mental retardation in craniosynostosis.

We prospectively studied craniosynostosis, regardless of neurologic status, by cranial computed tomography or psychometric testing in 56 children. None of the 27 children with simple craniosynostosis (single or multiple suture involvement) had evidence of hydrocephalus on CT scan. Of the 24 patients with simple craniosynostosis who underwent psychometric testing, 17 were of average intelligence; six were in the low average range. The single mentally retarded child had a history of severe perinatal asphyxia. Hydrocephalus occurred more frequently (five of 23 cases) in children with complex craniosynostosis syndromes, including Pfeiffer syndrome, Crouzon syndrome, and kleeblattschädel deformity. More striking than hydrocephalus, however, was the finding of dysmorphic ventricular dilation in eight patients, including the three children with Apert syndrome and four with Crouzon syndrome. Nineteen of the 25 children with complex craniosynostosis syndromes receiving psychometric testing were of normal intelligence. Four children with borderline normal intelligence had either hydrocephalus or ventricular dilation. The two children with mental retardation were sisters with Crouzon syndrome whose family included other retarded individuals. This study indicates that the incidence of hydrocephalus and mental retardation in craniosynostosis is lower than reported previously.

Late effects of radiotherapy on patients with cerebellar medulloblastoma.

Nine long-term survivors of cerebellar medulloblastoma treated with surgery and irradiation were retrospectively examined with a complete battery of neuropsychological tests and the results compared with their nonirradiated siblings. Significant decreased scores were found in the full-scale intelligence quotients (IQ) (P less than 0.001), performance IQ (P less than 0.001), and verbal IQ (P less than 0.02) with all nine irradiated patients scoring below their siblings. Also, educational quotients (EQ) of the irradiated patients were 12 to 17 points below the nonirradiated siblings with arithmetic EQ significantly decreased (P less than 0.01). Most severely affected were those children younger than 8 years at time of irradiation. No correlation was found with whole-brain dose, or objective physical or neurologic findings.

Intelligence in epilepsy: a prospective study in children.

A prospective study tested the stability of the IQ in children with seizure disorders. Seventy-two children with epilepsy underwent psychological evaluations within two weeks of initial diagnosis and yearly thereafter for an average of 4 years. Forty-five of the patients also had a nonepileptic sib evaluated in parallel. The mean IQ for all the children with epilepsy was 99.7 (+/- 20.2, standard deviation) at the time of the initial test, not significantly different from the siblings. This score did not change appreciably with time. Eight of the 72 epileptic patients (11.1%), however, had a persistent decrease in IQ of 10 points or more. These patients had a higher incidence of drug levels in the toxic range (p less than 0.001), their epilepsy was more difficult to control (p less 0.005), and their seizures began at an earlier age (p less than 0.05). Discriminant analysis is revealed that the number of drugs to which the patient became toxic and the age at seizure onset were the two best predictors of ultimate IQ. These two predictors correctly classified 71% of all patients as to whether their IQ would drop by 10 or more points during the test period, remain within 10 points of the initial test score, or increase by more than 10 points. Total number of seizures and seizure control were less good predictors, according to this method of analysis. The findings suggest that, in younger children in particular, total seizure control should not be achieved at the price of repeated episodes of drug toxicity.

A controlled study of siblings of hyperactive children.

A controlled study of siblings of hyperactive boys resulted in the following findings. The hyperactive child syndrome is more common among brothers of hyperactive children than among brothers of controls (26 per cent vs. 9 per cent). Both probands and their brothers presented with more symptoms of depression-anxiety than controls. The probands, but not their sibs, presented with more antisocial symptoms than controls. Although differences in intelligence and achievement scores were found between probands, their sibs, and controls, both intelligence level and academic achievement were within normal limits in all groups.

A follow-up study of Sydenham's chorea.

Twenty-five patients convalescing from Sydenham's chorea were contrasted by clinical examination, electroencephalograms, and psychometric and psychologic tests to 15 siblings and 20 matched rheumatic fever controls. A group of 10 postchoreic patients who had two or more signs could be identified. Patients in this group had all the signs classified as moderate or severe, performed less well than other choreic subjects on the Bender gestalt test, and had a higher percentage of abnormal electroencephalograms but not a higher incidence of behavioral disorders. This subgroup could not be predicted from a review of neurologic history or from analysis of the acute episode of chorea. Our data would suggest that uncomplicated Sydenham's chorea is not necessarily a benign self-limited affliction of the central nervous system and that some patients are left with definite, albeit minimal, neurologic residua.

Prospective evaluation of treatment of Hemophilus influenzae meningitis.

Fifty children with Hemophilus influenzae meningitis have been enrolled in a prospective study. Patients were randomly assigned chloramphenicol or ampicillin treatment; there were no significant differences between groups in other respects. Countercurrent immunoelectrophoresis proved to be a valuable tool for rapid diagnosis of the causative agent even in pretreated patients. Increasing quantities of capsular polyribosephosphate antigen detected in the initial cerebrospinal fluid correlated significantly (r=0.62419; p less than 0.01) with early and late sequelae of meningitis. None of the patients died. Severe and persistent neurologic or intellectual deficits were noted in four (8%) of the children, and an additional 14 (28%) had IQ scores between 70 and 90. The presence of bactericidal antibody in serum was not protective. Anti-PRP antibody generally was not present in acute serum specimens and irrespective of the quantity of antigenic stimulus provided by the disease was nondetectable in 21 of 24 children less than 17 months of age following recovery.