Outcome of Therapies for Metabolic Bone Disease in Children

Outcome After Surgery

• Advanced symptoms = poor prognosis: Children with advanced neurological symptoms at the time of intervention have a worse prognosis.
• Decompression successful for most: Craniovertebral decompression ameliorates symptoms of cervicomedullary compression (neurological and respiratory) in most children with achondroplasia (3).
• Fusion successful for 90%: Successful fusion can be anticipated in up to 90% of children wtih skeletal dysplasia (1, 7).
• Instability adjacent to fusion for 1/3: Junctional instability below a previous successful fusion has been reported in up to one third of children with Morquio syndrome, thereby underscoring the importance of long-term follow-up (7).