Surgical and functional results of spine fusion in spinal muscular atrophy.

From 1965 to 1987, 84 spinal muscular atrophy patients were followed at Rancho Los Amigos Medical Center (RLAMC). Twenty-seven patients were excluded from this study due to insufficient medical documentation (16), lack of follow-up (5), and death (6); leaving 57 patients in the general study group. Group I (34 patients) had posterior spinal fusion (PSF) with Harrington rod instrumentation (HRI); mean age at surgery was 12 years, average preoperative curve was 57 degrees, average postoperative correction was 42%, with a loss of correction of 9 degrees. The complication rate in this group was 35%. The average follow-up interval was 9 years (range, 4-19 years). Group II (six patients) underwent PSF with Luque segmental spinal instrumentation (SSI); mean age at surgery was 11 years, preoperative curves averaged 37 degrees, average postoperative correction was 42% with a loss of correction of 3 degrees. The complication rate in this group was 16%. Follow-up was 3.5 years. Physical therapy and occupational therapy evaluations were done preoperatively and postoperatively at 2- and 5-year intervals. Information was gathered in three categories: 1) ambulation, 2) equipment use, and 3) functional activities. After fusion, sitting tolerance was maintained but additional use of mobile arm supports, lapboards, and reaching aides was necessary for all patients. The ability to perform activities such as drinking, self-feeding, and self-hygiene declined during the 2 years immediately following surgery but improved by 5 years. Surgical patients never approached their preoperative skill levels. Therapy evaluations further demonstrated that there were no difference in function between either operative group.

Functional activities in spinal muscular atrophy patients after spinal fusion.

Spinal muscular atrophy patients present with weakness, greater in the proximal muscles, leading to scoliosis and limited upper extremity function. The purpose of this study is to identify unique aspects of these patients and to understand how spinal fusion affects their function. Forty patients underwent Harrington or Luque rod instrumentation with functional evaluations preoperatively and 2 and 5 years postoperatively. Biomechanical assessment of function is important. Flexibility of the spine is functionally advantageous because distal strength is used to align weaker proximal segments. Postoperatively, lack of spinal flexibility resulted in a decline in gross motor function and increased use of UE aids due to a change in the trunk position in the weaker patients. The stronger patients' activities were maintained. Earlier mobilization in patients with Luque procedures did not improve postoperative function.

Cognitive predictors of young children's readiness for powered mobility.

Independent mobility in early childhood has been associated with the development of various cognitive and psychosocial skills. However, children with physical disabilities are not always able to move independently and may be at risk for delays in these areas. Early provision of powered mobility can offer young children an opportunity for independent mobility. Despite this, there is little information to help determine when a young child has the cognitive skills necessary to operate a powered wheelchair safely. This current research aims to identify these skills. A cognitive assessment battery and a wheelchair mobility training and assessment program were developed. Twenty-six children with physical disabilities between the ages of 20 and 36 months were evaluated on the cognitive assessment and participated in the wheelchair training and assessment program. A stepwise regression analysis was used to determine which of the cognitive skills predicted wheelchair mobility performance. The cognitive domains of spatial relations and problem solving were found to be significant and accounted for 57% of the variance in wheelchair skills. Developmental cut-off points on these scales as they relate to wheelchair skills are presented and clinical applications are discussed.

Gait and posture changes in the Duchenne muscular dystrophy child.

As the child with Duchenne muscular dystrophy (DMD) grows older, gait and posture changes occur. This is attributable to the natural progress of muscle weakness secondary to muscle tissue destruction. Bracing improves body alignment and maintains limb stability passively. Surgical correction of contractures allows for bracing and maintenance function and is recommended as an adjunct to the overall care of the ambulatory DMD patient.

Fractures caused by falling from a wheelchair in patients with neuromuscular disease.

This report discusses the occurrence of fractures caused by falling from a wheelchair. 13 distal femur, two proximal humerus and three distal tibia fractures were seen in 13 patients, of whom 11 had Duchenne muscular dystrophy, one had Becker muscular dystrophy and one had spinal muscular atrophy. All were non-ambulant and had been wheelchair-dependent for at least one year before the fall occurred. Six patients sustained fractures between 1976 and 1982, and five more between 1983 and 1985. Eight subsequent fractures from wheelchair falls have occurred since 1986. Although wheelchair design, controls and adaptations have continued to improve, wheelchair safety has not.