James' adaptation of the Glasgow coma scale (JGCS) was designed for young children. Intubated patients are not allocated a verbal score, however, so important changes in a patient's conscious level may be missed. A grimace score was therefore developed and assessed for use in intubated children.Two observers made a JGCS observation within 15 minutes of each other. One observer was the patient's nurse and the other a trained investigator. Interobserver reliability was determined between the first and second observation for each component of the scale. Reliability was measured using and weighted statistics.Seventy three children had 104 sets of observations. Interobserver reliability was moderate to good for all components, with the grimace score better than the verbal score.It is concluded that the grimace score is more reliable than the verbal score and may be useful in intubated patients in whom the verbal score cannot be used.

This study summaries the current management of scoliosis in patients with Duchenne Muscular Dystrophy.A literature review of Medline was performed and the collected articles critically appraised. This literature is discussed to give an overview of the current management of scoliosis within Duchenne Muscular Dystrophy.Importantly, improvements in respiratory care, the use of steroids and improving surgical techniques have allowed patients to maintain quality of life and improved life expectancy in this patient group.

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The management of spinal deformity in children with univentricular cardiac pathology poses significant challenges to the surgical and anaesthetic teams. To date, only posterior instrumented fusion techniques have been used in these children and these are associated with a high rate of complications. We reviewed our experience of both growing rod instrumentation and posterior instrumented fusion in children with a univentricular circulation. Six children underwent spinal corrective surgery, two with cavopulmonary shunts and four following completion of a Fontan procedure. Three underwent growing rod instrumentation, two had a posterior fusion and one had spinal growth arrest. There were no complications following surgery, and the children undergoing growing rod instrumentation were successfully lengthened. We noted a trend for greater blood loss and haemodynamic instability in those whose surgery was undertaken following completion of a Fontan procedure. At a median follow-up of 87.6 months (interquartile range (IQR) 62.9 to 96.5) the median correction of deformity was 24.2% (64.5° (IQR 46° to 80°) vs 50.5° (IQR 36° to 63°)). We believe that early surgical intervention with growing rod instrumentation systems allows staged correction of the spinal deformity and reduces the haemodynamic insult to these physiologically compromised children. Due to the haemodynamic changes that occur with the completed Fontan circulation, the initial scoliosis surgery should ideally be undertaken when in the cavopulmonary shunt stage.