Long-term follow-up of functioning after spinal surgery in patients with Rett syndrome.

In a prospective study, 23 consecutive girls with Rett syndrome and neuromuscular scoliosis were evaluated for functioning at a long-term follow-up. The patients had mostly improved, which was confirmed by their parents. Rett syndrome is associated with neuromuscular scoliosis and has a typically long C-shaped thoracolumbar kyphoscoliosis. Prospective long-term follow-up studies related to these patients' total situation are sparse. Most studies focus on the Cobb angle of the scoliosis, whereas parents are mainly concerned about the girls' continued functioning. Twenty-three patients with Rett syndrome and neuromuscular scoliosis were evaluated preoperatively from 1993 to 2002. At follow-up, 19 patients remained in the study. Three patients died (not due to surgery), and one patient could not participate because it was too far to travel. Mean follow-up time was 74 months (range 49-99 months). The assessments comprised the sitting balance, seating supports in wheelchair, weight distribution, time used for rest, care given, and angle of scoliosis. Follow-up questionnaires and two-open-ended questions about the positive and negative effects of surgery were sent to parents. Sitting balance, number of seating supports in wheelchair, weight distribution, time used for rest, and the Cobb angle had all improved after surgery. The parents assessed improvement in seating position, daily activities, time used for rest, and cosmetic appearance. We can conclude that the stabilized spine resulted in sufficient strength to keep the body upright with the possibility of looking around at the surroundings more easily. The girls got better seating position with less need for seating adaptations in the wheelchair and with reduced time needed for resting during the day. Finally we can conclude that the indication for surgery is to get a better posture which lead to less risk of pressure sores, and that un upright position lead to better possibility to easily breath with fewer episodes of pneumonia and a better general health as result. The evidence of positive surgical effects for girls with Rett syndrome is of great importance in indication for surgery in the decision-making process.

Long-term follow-up of functioning after spinal surgery in patients with neuromuscular scoliosis.

STUDY DESIGN: A prospective study of 100 consecutive preoperative patients with neuromuscular scoliosis whose activities and function were evaluated in a long-term follow-up. OBJECTIVES: To evaluate long-term follow-up of functioning in patients with neuromuscular scoliosis. SUMMARY OF BACKGROUND DATA: Few studies of patients operated for neuromuscular scoliosis are prospective, with long-term follow-up based on assessments of activities and function. METHODS: The follow-up included 82 patients with neuromuscular scoliosis who were evaluated before surgery from 1992 to 1996. The follow-up time was 84.5 months on average. The assessments comprised sitting, angle of scoliosis, lung function, reaching, pain estimation, activities of daily living (ADL), care given, and time used for resting. A follow-up questionnaire as well two open-ended questions about the positive and negative effects of the surgery were sent to the patients/parents. RESULTS: Improvements after surgery were shown in the Cobb angle, lung function, seating position, ADL, and time used for resting. In a comparison between the 1-year follow-up and the long-term follow-up, there were further improvements in sitting, ADL, and care given but an increased Cobb angle. These results were in line with the assessments reported by patients and relatives. Only minor differences were shown in long-term outcome in subgroups according to understand/not understand verbal instructions. Patient age 21 years or less seemed more improved than those age 22 years and older at the time of surgery. CONCLUSIONS: The comparison of the preoperative and long-term follow-ups showed that patients were mostly improved. Further improvements were shown beyond the 1-year follow-up. The subjective assessments also confirmed the results. The heterogeneity of patients with neuromuscular scoliosis makes it important to evaluate the patients in subgroups and in relation to age.