Donald A. Simpson

Assessing the conscious level in infants and young children: a paediatric version of the Glasgow Coma Scale

The Glasgow Coma Scale is widely accepted as a measure of impaired consciousness both in clinical practice and in research. In its standard form, the scale is inapplicable to infants and children below the age of 5 years. We have devised a paediatric coma scale, which recognises that the expected normal verbal and motor responses must be related to the patients age. This scale has been routinely used in a paediatric teaching hospital over a 10-year period and has appeared to be satisfactory, although it is less sensitive to changes in the conscious level than the adult scale. We have assessed the consistency of observer evaluation, using video tapes of infants and children exhibiting varying levels of consciousness: when conducted after formal instruction, assessments were reasonably consistent.

Cephaloceles: Treatment, Outcome, and Antenatal Diagnosis

A series of 74 cephaloceles (17 cranial meningoceles and 57 meningoencephaloceles) is reported. Infants born with large meningoencephaloceles containing recognizable cerebral tissue usually did badly despite endeavors to conserve brain function by expanding the cranial capacity (5 cases) or decompressing hydrocephalic ventricles (9 cases). Infants with cranial meningoceles almost all did well, even when there was associated hydrocephalus. The etiological diversity of cephaloceles is emphasized. Frontoethmoidal meningoencephaloceles, which occur with noteworthy frequency in South and Southeast Asia, require separate consideration in both genetic counseling and treatment; the associated facial deformities (hypertelorism and orbital dystopia) can be corrected with a one-stage craniofacial reconstruction. Antenatal diagnosis by ultrasound is now often possible and was achieved in 4 cases; we suggest that neurosurgeons should participate in such antenatal evaluations.

Scaphocephaly: Aesthetic and Psychosocial Considerations

A series of 48 children with scaphocephaly has been reviewed. 44 had synostosis of the sagittal suture alone, and four had additional involvement of other sutures. Although six children showed significant developmental delay, with eventual mental retardation in five cases, it does not appear that this is caused by mechanical constriction of the brain. In the majority of cases, scaphocephaly appears to be a benign variant in cranial development.

The craniosynostoses : causes, natural history, and management

I Causes and Effects.- 1 The Concept of Craniosynostosis and the Evolution of Craniofacial Surgery.- 2 The Growth of the Skull and the Role of the Sutures.- 3 Aetiology and Pathogenesis.- 4 Pathology.- 5 Incidence.- II Symptoms and Strategies.- 6 Symptomatology.- 7 Principles of Investigation.- 8 Principles of Treatment.- III Simple Calvarial Deformities.- 9 Classification.- 10 Scaphocephaly.- 11 Trigonocephaly.- 12 Turricephaly.- 13 Plagiocephaly.- 14 Oxycephaly and Related Conditions.- IV Complex Craniofacial Deformities.- 15 Craniofacial Syndromes.- Crouzon Syndrome.- Apert Syndrome.- Saethre-Chotzen Syndrome.- Pfeiffer Syndrome.- Carpenter Syndrome.- Cohen Syndrome.- Other Craniofacial Syndromes.- 16 Surgical Management.- 17 Results and Complications.- 18 Psychosocial Aspects of Craniofacial Surgery.- Appendices.- A: Organisation of a Craniofacial Unit.- B: Syndromes Associated with Craniosynostosis.- References.

Arachnoid cysts: a critical review of 41 cases.

Fortyone cases of arachnoid cyst are reviewed that were treated at the Adelaide Childrens Hospital and the Royal Adelaide Hospital. The pathogenesis of the cysts is discussed as well as the method of treatment. Certain aspects of this condition are still unsolved.

Premature craniosynostosis-a complication of thyroid replacement therapy.

Three hypothyroid children with premature craniosynostosis are presented, who have been treated intensively with 1-thyroxine from early infancy. It is postulated that the craniosynostosis is the result of iatrogenic hyperthyroidism during a critical period of skull growth. Various regimens of treatment for hypothyroidism are discussed with recommendations for avoiding this potentially hazardous complication of therapy.

Fronto-ethmoidal meningoencephaloceles: morphology and treatment.

Twenty-five cases of fronto-ethmoidal meningoencephaloceles have been studied. The relationship to other sincipital meningoencephaloceles is explored. In all cases the exit holes from the anterior cranial fossa are at the site of the foramen caecum. The facial component of the defect determines the sub-classification: naso-frontal, naso-ethmoidal and naso-orbital. The cranio-facial deformity may consist of hypertelorism, orbital dystopia, elongation of the face and dental malocclusion. These reflect the distorting influence of the extruded intracranial contents on facial growth. Early removal of the meningoencephalocele by the cranio-facial route is recommended to allow normal growth forces to be re-established. In older patients with established deformities translocation of the orbits may be necessary.

CRANIUM BIFIDUM. INVESTIGATION, PROGNOSIS AND MANAGEMENT*

The term cranium bifidum includes the encephaloceles and the cranial meningoceles. Though rarer than spina bifida, these conditions are not uncommon, and their management can be difficult. A series of 14 patients is presented to illustrate the problems, and to show that treatment can produce good results where there are no crippling associated cerebral dysplasias. The value of contrast radiological investigations to demonstrate these dysplasias is emphasized. Some interesting and unexplained variations in the geographical incidence of this condition are discussed.

Surgical treatment of neonatal intracranial teratoma

Two cases of neonatal intracranial teratoma treated by surgical extirpation are reported. Operative treatment of this tumor has been reported rarely. The problems associated with neurosurgery for this condition and reasons for the dismal overall prognosis are discussed.

The Development of Interhemispheric Transfer of Tactile Information in Cases of Callosal Agenesis

Congenital absence of corpus callosum in relatively asymptomatic individuals raises the question of mechanisms responsible for interhemispheric transfer of information. Possible alternative methods of communication between the cerebral hemispheres involve the anterior commissure, subcortical commissural pathways, bilateral representation of functions that are normally predominantly under unilateral control, enhanced transmission of information on the ipsilateral sensory and motor pathways, and behavioral cross-cueing (Jeeves, 1990). Before it is possible to pinpoint a particular mechanism, it is necessary to determine whether subjects with congenital absence of the corpus callosum (ACC) differ from normal individuals when performing tasks that require interhemispheric transfer, and whether transfer deficits, where present, are similar in magnitude to those shown by patients with surgical section (partial or complete) of the corpus callosum. We report a study of seven cases of callosal agenesis using a tactile finger localization task previously shown to be sensitive to extent and location of acquired lesions of the corpus callosum (Geffen et al., 1985). Developmental improvements in interhemispheric transfer in normal children were also elicited using this task (Quinn and Geffen, 1986). However, the proposal that this improvement reflects the concomitant myelination of the corpus callosum between ages 5–11 years has been questioned (Pipe, 1991).