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- Title
Anesthetic considerations for scoliosis surgery in a patient with recessive severe/lethal form of osteogenesis imperfecta.
- Authors
Chin, Joel W. E.; Stuart, Grant M.
- Abstract
Summary: Osteogenesis 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.
- Subjects
SCOLIOSIS treatment; SCOLIOSIS in children; PEDIATRIC anesthesia; OSTEOGENESIS imperfecta; PEDIATRIC surgery; GENETIC disorders in children; BONE growth
- Publication
Pediatric Anesthesia, 2018, Vol 28, Issue 9, p817
- ISSN
1155-5645
- Publication type
Article
- DOI
10.1111/pan.13461