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Perioperative Care of an Eleven-Year-Old Child With Osteogenesis Imperfecta Type II During Posterior Spinal Fusion
Alaa Soliman1, Marwan Hillis1, Allen Kadado2
1Department of Anesthesiology & Pain Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
Insights
Osteogenesis imperfecta (OI) is a rare genetic disorder causing bone fragility and multi-system issues. Anesthetic management for OI patients requires careful consideration of airway, positioning, respiratory, and coagulation risks during surgery.
Area of Science:
- Medical Genetics
- Anesthesiology
- Orthopedic Surgery
Background:
- Osteogenesis imperfecta (OI) is an autosomal dominant inherited connective tissue disorder.
- OI presents with bone fragility, skeletal deformities, and potential cardiovascular, respiratory, and hematologic system involvement.
- Perioperative management of OI patients is complex due to risks like difficult airways, fracture, restrictive lung disease, and coagulation issues.
Abstract:
Osteogenesis imperfecta (OI) is an autosomal dominant inherited condition involving connective tissue associated with not only bone fragility, but multi-system involvement with perioperative implications. OI is characterized by bone fragility, skeletal deformities, and variable involvement of the cardiovascular, respiratory, and hematologic systems. Perioperative care poses unique challenges related to the potential for difficult airway management, fracture risk during positioning, restrictive lung disease, coagulation disturbances, and potential hyperthermic reactions. We describe the anesthetic management of an 11-year-old child with OI type II presenting for posterior spinal fusion for progressive scoliosis. The basic cellular mechanisms responsible for OI are reviewed, clinical phenotypic categories and end-organ involvement discussed, and options for intraoperative anesthetic care presented.
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