Bone Abstracts

Introduction: Although previously babies with genetic type II Osteogenesis Imperfecta (OI) would not have expected to survive, they are now surviving beyond the neonatal period. We describe two such children who have survived beyond infancy.

Aim & methods: To identify differences in motor developmental progress between a typical severe (type III) OI child vs two Type II OI children and suggest possible causes. Medical, nursing and therapy (physiotherapy and occupational therapy) notes were reviewed and compared for the 3 patients. Significant medical events (respiratory, neurosurgical interventions and fractures) and developmental milestones were identified. Contact time with Multi-Disciplinary Team [MDT] (excluding doctors) was recorded from the hospital activity system.

Results: Motor outcomes were grossly delayed in the Type II vs Type III baby despite intense input from physiotherapy, mean hours (73.75 vs 30.25) in the first 10 months of life. Children (A & B) with type II OI achieved active head turns in supine at age 9 and 4 months respectively vs age 3 months in child C (type III). Independent rolling -supine to prone- was achieved age 18 months in child A, not yet achieved in child B but achieved by age 14 months in child C. Ability to lift head in prone was achieved age 3 months in child C but at age 18 months in child A and not yet achieved in child B (now age 19 months). Number of fractures in all 3 babies in the first year of life was low A=2, B=1 vs C=2. Significant medical events affecting respiratory, feeding and neurological function were more frequent in the Type II babies. Similarly, inpatient time in the first year of life was greater in the Type II vs Type III OI (mean 312.5 vs 62 days).

Conclusion: Babies with type II OI are likely to have complex medical problems which affect their motor development. Although bone fragility precautions are important, other factors seem to have a greater impact. They are also very likely to require much higher long term therapy input which should be factored into care planning.

Disclosure: The authors declared no competing interests.