Scoliosis repair in a child with Rett syndrome
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Scoliosis repair in a child with Rett syndrome book
Introduction Scoliosis is derived from the Greek word meaning curvature or crooked. Scoliosis is a coronal plane deformity of the spine that is greater than 10% and has structural rotation at the apical segment. The incidence of scoliosis is 1.8 per 1000 of school age children in the United Kingdom. Rett syndrome is a progressive neurological disorder. It is an inherited condition, prevalent in 1 in 10,000 females,1 the gene is carried on the X chromosome and only affects girls. The incidence of scoliosis in Rett syndrome increases with age affecting 80% of those who reach puberty.2
Scoliosis correction is contemplated in these patients to prevent deterioration of respiratory function or to improve seating position. The anaesthetic challenges of scoliosis correction in a child with Rett syndrome include recognition of the potential problems associated with the syndrome and delivery of an appropriate anaesthetic that takes into consideration the important issues of minimizing homologous blood transfusion and preservation of spinal cord function.