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Rehabilitation of children and infants with osteogenesis imperfecta. A program for ambulation
L H Gerber1, H Binder, J Weintrob
1Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892.
Insights
A comprehensive rehabilitation program, including bracing and surgery, significantly improves functional mobility in children with osteogenesis imperfecta (OI). This approach reduces fracture rates and enhances the quality of life for affected children.
Area of Science:
- Pediatric Orthopedics
- Rehabilitation Medicine
- Genetics
Background:
- Osteogenesis imperfecta (OI) presents significant challenges in managing bone fragility and deformities in children.
- Children with OI often experience recurrent fractures and impaired mobility, necessitating specialized care.
Purpose of the Study:
- To evaluate the effectiveness of a comprehensive management program for children with osteogenesis imperfecta (OI).
- To assess the impact of rehabilitation, bracing, and surgical interventions on functional outcomes and fracture rates in pediatric OI patients.
Main Methods:
- An eight-year retrospective study of 12 children with OI.
- Implementation of a program including strengthening exercises, aquatic therapy, supportive seating, long leg bracing, and femoral plating/rodding when indicated.
- Fracture rates and ambulation status were tracked before and after bracing.
Main Results:
- Following bracing, fracture rates decreased from 1.5 to 0.83 fractures per year in children who experienced fractures.
- Nine out of twelve children became functional ambulators, and three were home ambulators.
- Long leg bracing showed positive effects on tibial bowing, with decreases observed in most cases.
Conclusions:
- A multidisciplinary approach combining rehabilitation, bracing, and surgery can lead to high functional activity levels in children with OI.
- This comprehensive management strategy appears to reduce fracture incidence and improve mobility with manageable risks.
Abstract:
Management of children and infants with osteogenesis imperfecta (OI) poses difficult decisions for pediatricians, orthopedists, and physiatrists. These children are frequently frail with disabling bone and joint deformities and fractures. In an eight-year cumulative management of 12 children with OI, a comprehensive program included strengthening exercises to the pelvic girdle and lower extremity muscles, in addition to pool exercises and molded seating to support upright posture. Long leg braces were fitted when the children were able to sit unsupported. All 12 were fitted with braces; nine were functional ambulators, and three were home ambulators. Six children required femoral plating or rodding, two of whom subsequently had the metal removed. Lower extremity fractures averaged one and one-half per year prior to bracing for nine children who had fractures. There was 0.83 fracture per year for the ten children who had fractures after bracing. The degree of femoral bowing increased in four, decreased in four, and remained unchanged in four, while the degree of tibial bowing increased in two, decreased in nine, and remained unchanged in one during the observation period. A comprehensive rehabilitation program and long leg bracing with surgical operations on the femur result in a high level of functional activity for children with OI with an acceptable level of risk for fracture.