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.

The results of spinal fusion in patients with paralytic deformities are usually presented as the correction of the deformity. When evaluating the surgical results in such patients, it is advantageous to classify the patients into subgroups because of the varying dysfunction and disabilities. The aim of this study was to evaluate the effect of spinal fusion in patients with paralytic scoliosis in relation to function in terms of Impairments, activities in terms of Disabilities, and dependence in terms of Handicaps 1 year postoperatively, with emphasis on subgroups. A total of 94 patients with paralytic scoliosis and 18 different diagnoses were evaluated 1 year after surgery. The patients were classified according to whether or not they could understand verbal instructions. The patients were also grouped according to the Scoliosis Research Society classification of diagnoses. A set of instruments was used with the variables classified according to WHO's International Classification of Impairments, Disabilities, and Handicaps (ICIDH). This consists of the patient's/relative's motives for surgery, persistent skin discoloration, ambulating or use of wheelchair, use of a brace, sitting balance, weight distribution on a sitting surface, angle of scoliosis, reaching, pain estimation, activities of daily living (ADL) Klein and Bell, care given, time spent resting, and seating supports. The set of instrument also included a follow-up questionnaire comprising 12 different areas, where the patients/relatives assessed the results of surgery. The study showed that spinal fusion in paralytic scoliosis led to showed improvements in the whole group of patients at the Impairment level in the angle of scoliosis, sitting balance, weight distribution, and reduced number of patients with persistent skin discoloration, and at the Handicap level in reduced time for resting during the day, reduced number of seating supports in the wheelchair, and in the use of a brace. Most of the parameters were unchanged. The results in the subgroups were almost the same as in the whole group, although pain and reaching at the Impairment level, and ADL at the Disability level, could not be measured in the patients who were unable to understand verbal instructions. The subjectively assessed results showed that seating posture was ranked positively irrespective of the motive for surgery. The study showed that the patients with paralytic scoliosis maintained or improved their function and level of independence in terms of Impairment and Handicap 1 year postoperatively. The subjective results assessed by the patients/relatives also showed a positive outcome of surgery. Weight distribution on a seating surface was improved, but still uneven, and with respect to better sitting balance and increasing time sitting in a wheelchair, this can involve a risk for pressure sores and needs further investigation. When introducing outcomes including the Disability level, one must take the importance of homogeneity in the groups into consideration.

Surgical treatment for neuromuscular scoliosis is effective for most patients. Although those afflicted constitute a heterogeneous group, the aim of surgical treatment is approximately the same for all patients: a spine balanced in the coronal and sagittal planes over a level pelvis. Surgery results in a more stable and straighter spine, which should in turn improve performance in different activities. Previous evaluations of surgery for neuromuscular scoliosis reported in the literature have focused primarily on Cobb angles; there are very few studies dealing with the ability to perform various activities. A new tool for evaluation was developed in several steps, starting with a telephone interview with patients who had undergone surgery and a literature search. The evaluation instrument was then developed, followed by a pilot study and validation of new parts of the instrument. The instrument focuses on performance components and on activity performance. Eight items are evaluated before and after surgery. These data are complemented by a questionnaire administered to the patient or relatives at follow-up. The new parts of the instrument were developed specifically for patients with neuromuscular scoliosis, and the data obtained have been shown to have a high correlation with established measures of activities of daily living of daily living). They should therefore provide us with useful information concerning functional gains as a result of surgery as well as the effect of surgery on activity performance.

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