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Paediatric anaesthesia · Sep 2018
Case ReportsAnesthetic considerations for scoliosis surgery in a patient with recessive severe/lethal form of osteogenesis imperfecta.
- Joel W E Chin and Grant M Stuart.
- Department of Anaesthesia, Great Ormond Street Hospital NHS Trust, London, UK.
- Paediatr Anaesth. 2018 Sep 1; 28 (9): 817-818.
AbstractOsteogenesis imperfecta is a heterogeneous genetic disorder characterized by bone fragility, with disease ranging from mild fractures to death in utero. We describe a child with autosomal recessive osteogenesis imperfecta type VIII (severe or lethal phenotype), who successfully underwent posterior spinal fusion, was extubated on postoperative day 1 and discharged home 25 days later. Recently identified recessive forms of osteogenesis imperfecta are associated with severe/lethal phenotype. Special consideration is needed in scoliosis surgery, with challenges arising from prone positioning, neurophysiology monitoring, and blood loss.© 2018 John Wiley & Sons Ltd.
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