Results of Surgery for Scoliosis in Rett Syndrome

Abstract

The British Isles Survey for Rett Syndrome stores longitudinal health data from clinical examinations and postal questionnaires to monitor health and severity in Rett syndrome, including the presence and severity of scoliosis and the effects of corrective surgery. Scoliosis is rarely present at birth (3% before regression) but usually appears by 25 years (87%). The degree tends to increase with growth and by 16 to 20 years, 43% (75 of 173) of cases with classic Rett syndrome reported severe or operated scoliosis. Surgical correction was reported in 91 classic cases. Following initial postoperative recovery, families considered that the operation had improved general well-being for 84% of individuals (42 of 50 classic cases with postoperative health reports). Thirteen of 50 patients walked independently before surgery, and 12 patients did so following surgery; 2 stopped walking, and 1 who had not walked began to do so. Scoliosis surgery usually benefited sitting posture (82% better, 10% worse), chest episodes (52% better, 6% worse), and digestion of food (42% better, 6% worse). However, toilet function was improved in only 10% and deteriorated in 20%. Families reported short-term problems at operation in 48% (24 of 50) and minor recurrence of scoliosis in 22% (11 of 50). Surgery in a specialized unit is satisfactory management for severe scoliosis in Rett syndrome. Recommendations include planning for surgery when the curve passes 40°, ensuring optimal nutrition before and after surgery, robust fixture of the whole spine in two stages, familiarization of the surgical team with the individual and the disorder before the operation, and inclusion of the main carer in the hospital care team. Parents form an important part of the management team. Families also require support during and after this stressful major procedure. ( J Child Neurol 2003;18:703—708).