Current management of musculo-skeletal complications in children with mucopolysaccharidosis andosteogenesis imperfecta

The clinical condition and survival rate of children with mucopolysaccharidosis (MPS) as well as the functional capabilities of children with osteogenesis imperfecta (OI) are significantly improved by introducing new pharmaceutical agents: supplemental enzymatic therapy for MPS and bisphosphonates for OI children. This therapy slows the progression of chronic orthopaedic problems. New techniques in orthopaedic treatment can provide easier and faster rehabilitation and functionality recovery of the locomotor system. In MPS patients, orthopaedic problems in the upper extremity are treated well with decompressions in a carpal tunnel and trigger finger releases. MRI evaluation of the spinal deformity shows whether operative or conservative treatment is necessary. Hip dysplasia and knee valgus deformity can be treated with corrective osteotomy. In OI patients, intramedullary fixation is the method of choice in fracture treatment, long bone deformity correction, but also as a method of (re)fracture prevention. Progressive scoliosis in type III OI patients compromises pulmonary function, and surgical treatment of the spinal deformity is often necessary. Potential orthopaedic problems recognized on time, a multidisciplinary treatment approach, and appropriate surgical techniques can significantly improve the functional status of the musculo-skeletal system in children with MPS and in children with OI.
Category: Review
Volume: Vol. 49, No 3, july - september 2005
Authors: D. Antičević, M. Bergovec, T. Đapić
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