Abstract
Osteogenesis imperfecta (OI) is a genetic collagen disorder characterized by extreme skeletal fragility, complicating perioperative care for pediatric patients with any fractures. Systemic risks include restrictive lung disease, coagulation abnormalities, and predisposition to hypermetabolism and hyperthermia, along with airway challenges from cervical instability. A seven-year-old girl with a femoral shaft fracture was scheduled for titanium elastic nailing. She had multiple prior fractures from trivial trauma, a family history of OI, blue sclerae, and dental caries. After careful premedication, padding, and warming, total intravenous anesthesia was given with propofol-enabled gentle intubation and positioning. Surgery proceeded uneventfully, with stable hemodynamics and no hyperthermia. Multimodal analgesia facilitated smooth recovery. Multidisciplinary planning, meticulous handling, and avoidance of volatile agents ensured safe outcomes in this fragile patient.
References
1. Åström E. Management of osteogenesis imperfecta. Paediatrics and Child Health. 2008;18(2):97-98. DOI: https://doi.org/10.1016/j.paed.2007.10.011
2. Patel A, Aghara AR, Mehta K, Panchal S, Gami VM, Desai D. Anesthetic management of femur fracture in a pediatric patient with osteogenesis imperfecta. Journal of Anesthesia & Pain Medicine. 2024;9(2):1-3. DOI: https://doi.org/10.33140/JAPM.09.02.05
3. Gupta D, Purohit A. Anesthetic management in a patient with osteogenesis imperfecta for rush nail removal in femur. Anesth Essays Res. 2016;10(3):677-9. PMID: 27746572 DOI: https://doi.org/10.4103/0259-1162.184612
4. Oakley I, Reece LP. Anesthetic implications for the patient with osteogenesis imperfecta. AANA J. 2010;78(1):47-53. PMID: 20977129
5. Santos ML, Añez C, Fuentes A, Méndez B, Periñán R, Rull M. Airway management with ProSeal LMA in a patient with osteogenesis imperfecta. Anesth Analg. 2006;103(3):794. PMID: 16931717 DOI: https://doi.org/10.1213/01.ane.0000227155.44286.28
6. Sullivan BT, Margalit A, Garg VS, Njoku DB, Sponseller PD. Incidence of fractures from perioperative blood pressure cuff use, tourniquet use, and patient positioning in osteogenesis imperfecta. J Pediatr Orthop. 2019;39(1):e68-e70. PMID: 29189536 DOI: https://doi.org/10.1097/bpo.0000000000001105
7. Wang H, Huang X, Wu A, Li Q. Management of anesthesia in a patient with osteogenesis imperfecta and multiple fractures: a case report and review of the literature. J Int Med Res. 2021;49(6):3000605211028420. PMID: 34190615 DOI: https://doi.org/10.1177/03000605211028420
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2024 Ahish Raj Dhital, Chetan Bohara, Sanju Regmi