Derek A. Bruce

Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients.

Treatment options for the craniosynostoses vary from conservative observation until completion of growth to radical remodeling in infancy. To further define the timing and type of treatment necessary in these complex disorders, we have retrospectively analyzed all patients operated on for this deformity during the past 12 years. One-hundred and sixty-four patients with craniosynostosis were analyzed and subgrouped into asymmetrical (predominantly unilateral) and symmetrical (bilateral) deformities, in addition to segregation by age and type of procedure performed. This was done recognizing that no deformity, like no normal human face, is truly symmetrical. Results of treatment were categorized on the basis of the need for additional surgery and varied from no refinements necessary (category I) to major reduplication of the initial procedure (category IV). Analysis of the data led us to conclude that excellent results can be expected in the asymmetrical deformities group treated in infancy by a unilateral approach. Similarly, for the mild symmetrical deformities, treatment at this time by bilateral orbital advancement gives satisfactory results in the majority of patients. By contrast, the more severe symmetrical groups treated in childhood have a high incidence of requiring secondary major reconstructions, and consideration should be given to delaying craniofacial surgery until age 7 or older, although earlier cranial surgery may be advisable.

Intrathecal Baclofen for Management of Spastic Cerebral Palsy: Multicenter Trial

Intrathecal baclofen infusion has demonstrated effectiveness in decreasing spasticity of spinal origin. Oral antispasticity medication is minimally effective or not well tolerated in cerebral palsy. This study assessed the effectiveness of intrathecal baclofen in reducing spasticity in cerebral palsy. Candidates were screened by randomized, double-blind, intrathecal injections of baclofen and placebo. Responders were defined as those who experienced an average reduction of 1.0 in the lower extremities on the Ashworth Scale for spasticity. Responders received intrathecal baclofen via the SynchroMed System and were followed for up to 43 months. Fifty-one patients completed screening and 44 entered open-label trials. Lower-extremity spasticity decreased from an average baseline score of 3.64 to 1.90 at 39 months. A decrease in upper-extremity spasticity was evidenced over the same study period. Forty-two patients reported adverse events. Most common reports were hypotonia, seizures (no new onset), somnolence, and nausea or vomiting. Fifty-nine percent of the patients experienced procedural or system-related events. Spasticity in patients with cerebral palsy can be treated effectively by continuous intrathecal baclofen. Adverse events, although common, were manageable. (J Child Neurol 2000;15:71-77).

Pathophysiology, treatment and outcome following severe head injury in children.

The pathophysiology and outcome following severe head injury in 85 children are presented. The commonest initial CT diagnosis was of acute brain swelling. This swelling was associated with an increased white matter density on the CT scan which decreased to normal concomitant with recovery and increased ventricular size. CBF measurements in 6 of these patients revealed an increased blood flow despite a decreased CMRO2 and clinical coma. This CT pattern of diffuse swelling is believed to be due to acute cerebrovascular congestion and hyperemia and not to edema. Because of this, all children were treated with endotracheal intubation and controlled hyperventilation as part of the initial management. Mass lesions were uncommon, 20%. ICP was monitored in 40 children. The ICP rose above 20 Torr despite therapy in 80% of children with decerebrate or flaccid coma and in only 20% of children with spontaneous motor function. The ICP was at its highest between the second and fifth day. Aggressive therapy to control the ICP, with barbiturates if necessary, was successful in 80% of the patients. The overall results were useful recovery in 87.5% of the children, 3.5% were left vegetative or severely disabled and 9% died.

Pediatric brain stem gliomas.

Sixty‐two pediatric patients with brain stem glioma diagnosed between 1964 and 1978 have been reviewed. Posterior fossa exploration was performed on 53% of the patients. Pathology was obtained by biopsy in 58% of those explored. Fifty‐four patients had definitive radiation therapy with a median dose of 5000 rads. The actuarial five‐year survival for the entire group is 30%.

Narrative Discourse after Closed Head Injury in Children and Adolescents

This study examined narrative discourse in 20 children and adolescents at least 1 year after sustaining a head injury. Narratives were analyzed along the dimensions of language structure, information structure, and flow of information. Severity of impaired consciousness was associated with a significant reduction in the amount of language and information. The most important finding which emerged was the disruption in information structure. This pattern confirms the impression of disorganized discourse in severely injured children. Explanations for the disruption in information structure are explored in terms of the role of vocabulary, memory, and localization of lesion according to magnetic resonance imaging. In view of recent evidence that frontal lobe damage is associated with discourse formulation deficits in adults and is the most common site of focal lesion in closed head injury, we examined discourse patterns in individual patients with frontal lobe lesions. Preliminary data from our single-case studies suggest discourse patterns similar to those reported for adults with frontal lobe injuries.

Computed Tomography of Pediatric Head Trauma: Acute General Cerebral Swelling

General cerebral swelling was the most common CT finding in 100 pediatric patients with acute head injury, and was demonstrated by CT as absence or compression of the lateral and third ventricles and perimesencephalic cisterns. Up to 50% of the children who die from head injury are conscious on admission. The most common pathological findings are diffuse cerebral swelling, loss of cerebrospinal fluid spaces, and venous congestion of the cortex.

Computed Tomography of Craniocerebral Injury in the Abused Child

Computed tomography (CT) was performed in 26 infants and children with craniocerebral trauma related to abuse during a 41-month period. The pattern of brain injury differed from that seen in the nonabused traumatized infant or child. Parieto-occipital acute interhemispheric subdural hematoma (AIHSH) with associated parenchymal injury was the most frequent finding (58%). Follow-up by CT in patients with AIHSH demonstrated infarction in half and cerebral atrophy in all.

Dimensions of cognition measured by the tower of London and other cognitive tasks in head‐injured children and adolescents

To determine the factor structure of executive functioning in head‐injured (n = 81) and normal children (n = 102), we administered tests of concept formation and problem solving, plus planning, verbal fluency, design fluency, memory (to evaluate semantic organization), and response modulation using a Go/No‐Go task. The children who sustained closed head injury (CHI) were divided into subgroups who sustained severe (n = 39) and mild/moderate (n = 42) injury. The CHI groups and normal controls were also grouped according to age at the time of testing (6–8, 9–12, and 13–16). The principal components analysis disclosed a five‐factor solution that accounted for 79% of the variance: Conceptual‐Productivity (Factor 1), Planning (Factor 2), Schema (Factor 3), Cluster (Factor 4), and Inhibition (Factor 5). Age had a significant effect on Factors 1, 2, and 5, whereas severity of CHI affected Factors 1,2,4, and 5. Using hierarchial regression in which the Glasgow Coma Scale score, age, and their interaction were ent...

Magnetic resonance imaging in relation to functional outcome of pediatric closed head injury: A test of the Ommaya-Gennarelli model

OBJECTIVE To characterize late neuropathological findings of pediatric closed head injury (CHI), to assess depth of brain lesion in relation to acute severity, and to assess long-term outcome to test the Ommaya-Gennarelli model. METHODS Magnetic resonance imaging (MRI) at least 3 months postinjury in a prospective sample (n 5 169) and at least 3 years after CHI in a retrospective sample (n 5 82) was studied. Lesion volume was measured by planimetry. Acute CHI severity was measured by the Glasgow Coma Scale. Patients were classified according to the depth of the deepest parenchymal lesion into no lesion, subcortical, and deep central gray/brain stem groups. The outcomes were assessed by the Glasgow Outcome Scale and the Vineland Adaptive Behavior Scale, which were performed at the time of the MRI in the retrospective sample and up to 3 years postinjury in the prospective sample. RESULTS Focal brain lesions were present in 55.4% of the total sample. Depth of brain lesion was directly related to severity of acute impairment of consciousness and inversely related to outcome, as measured by both the Glasgow Outcome Scale and the Vineland Adaptive Behavior Scale. A rostrocaudal gradient of hemispheric lesion frequency was observed, whereas the posterior lesions of the corpus callosum were particularly common. Total lesion volume could not explain the depth of lesion effect. CONCLUSION Our findings extend support for the Ommaya-Gennarelli model to pediatric CHI, indicating that depth of brain lesion is related to functional outcome. The relative frequency of focal brain lesions revealed by late MRI is higher than that of previous findings using acute computed tomography. Future investigations could explore whether depth of lesion observed using late MRI is sensitive to neuroprotective interventions.

Observations on the Use of Rigid Fixation for Craniofacial Deformities in Infants and Young Children

With the introduction of smaller plating systems, many surgeons have adopted their use for craniofacial procedures in infants and small children. We have encountered some previously undescribed problems associated with the use of plates and screws in infants and children. These problems can be classified into three general areas: 1) Plate displacement. This occurs after placement of the metal plates on the outside of the infant calvaria. With subsequent growth, these plates and screws may be found along the inner cortex of the skull with screw points embedded through dura. 2) Plate and screw isolation. In certain locations in young children, plates placed on the outer cortex of bone may later be found to be more prominent, isolated on a peninsula of bone. 3) Plate placement. We have also encountered plates that have been placed along the inner cortex of the skull during a previous operative procedure. This makes subsequent surgery extremely difficult and may increase the risk for secondary surgery. We propose that a previously described model for growth, involving areas of bony resorption and deposition, may be responsible for the observed plate displacement.