A microscopic deletion of chromosome 22q11.2 has been identified in most patients with the DiGeorge, velocardiofacial syndrome, conotruncal anomaly face syndrome, and in some patients with isolated conotruncal cardiac anomalies. This study presents the neurodevelopmental outcome, including cognitive development, language development, speech, neuromuscular development, and behavioral characteristics of 40 preschool children (ages 13 to 63 months) who have been diagnosed with the 22q11.2 deletion. The impact of cardiac disease, cardiac surgery, and the palatal anomalies on this population was also studied. In the preschool years, children with a 22q11.2 deletion are most commonly found to be developmentally delayed, have mild hypotonia, and language and speech delays. The more significantly delayed children are at high risk to be subsequently diagnosed with mild or moderate mental retardation. The global delays and the variations in intelligence found are directly associated with the 22q11.2 deletion and are not explained by physical anomalies such as palatal defects or cardiac defects, or therapeutic interventions such as cardiac surgery. Our findings demonstrate that there is a pattern of significant speech disorders within this population. All of the children had late onset of verbal speech. Behavioral outcomes included both inhibition and attention disorders. Early intervention services are strongly recommended beginning in infancy to address the delays in gross motor skills, speech and language, and global developmental delays.

Nonaccidental injury accounts for nearly one quarter of all hospital admissions for head injury in infancy, and is associated with significant morbidity and mortality. Long-term outcome in survivors, however, has been incompletely studied. In this series, 84 infants 2 years of age and younger with the shaking-impact syndrome consecutively admitted to a single hospital between 1978 and 1988 were identified. A questionnaire detailing current medical, developmental, and behavioral status was developed, and attempts were made to locate the 62 children surviving the acute injury. Family instability and strict confidentiality restrictions precluded locating the majority of children, but 14 children with demographic and injury characteristics similar to those of the overall group were contacted at an average of 9 years after injury. Seven children were severely disabled or vegetative, 2 were moderately disabled, and 5 had a good outcome. Of the latter group, 3 had repeated grades and/or required tutoring. Acute factors associated with poor outcome included unre-sponsiveness on admission, need for intubation, age less than 6 months, and bilateral or unilateral diffuse hypodensity on CT scan. All children with bilateral diffuse hypodensity and loss of gray-white differentiation on CT scan remained blind, retarded, nonverbal, and nonambulatory in spite of aggressive medical and surgical management. This study suggests that the majority of children surviving the shaking-impact syndrome suffer major permanent morbidity, and that acute factors predicting long-term outcome may help guide aggressiveness of care.

Ten patients with intracranial lesions, anaesthetized with thiopentone and nitrous oxide (70%) in oxygen (30%) received etomidate 0.2 mg kg-1 i.v. Ventilation was controlled in each patient. Intracranial pressure (i.c.p.) and mean arterial pressure (m.a.p.) were recorded. I.c.p. decreased significantly in all patients (0.01 greater than P greater than 0.001). Although PaCO2 decreased during the period of measurement, the extent and time-course of this change suggested that it was not mainly responsible for changes in i.c.p. M.a.p. decreased in most patients, but the decrease was statistically significant only at 3 and 4 min after the administration of etomidate (0.05 greater than P greater than 0.02). The changes in cerebral perfusion pressure (c.p.p.) and heart rate were not clinically or statistically significant. We conclude that etomidate can be used for the induction of anaesthesia in patients with intracranial space-occupying lesions without increasing i.c.p. or seriously reducing c.p.p.

We have compared codeine and tramadol in a prospective, double-blind study of postoperative analgesia in 75 patients after elective intracranial surgery. Twenty-five patients received codeine 60 mg, tramadol 50 mg or tramadol 75 mg i.m. Patients receiving codeine had significantly lower pain scores over the first 48 h after operation (P < 0.0001). Although there was no difference in visual analogue scale (VAS) scores between the three groups at 24 h, the codeine group had significantly lower scores at 48 h (P < 0.0001). The tramadol 75 mg group had significantly higher scores for both sedation and nausea and vomiting (P < 0.0001 for both scores). We conclude that codeine 60 mg i.m. provided better postoperative analgesia than tramadol after craniotomy and that tramadol 75 mg should be avoided because of its side effects of increased sedation and nausea and vomiting.