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Spinal bleed osteogenesis imperfecta

Munish Garg, Manish Jain, Amit Gupta
Sometimes in practice of anaesthesia, anaesthesiologist encounters patients with rare congenital diseases. To anaesthesiologist, these patients are a challenge due to inherent complications associated with the disease. Here, we are reporting a case of osteogenesis imperfecta who was posted for the surgery for vesical calculus. All investigations were done to rule out any cardio-respiratory abnormalities, bleeding disorders, which are commonly associated with these patients. Caudal epidural was chosen as anaesthesia technique of choice as spinal anaesthesia was anticipated to be difficult due to associated kyphoscoliosis...
February 2009: Indian Journal of Anaesthesia
Deanna Sasaki-Adams, Abhaya Kulkarni, James Rutka, Peter Dirks, Michael Taylor, James M Drake
Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by disruption of normal collagen formation resulting in varying degrees of skeletal vulnerability, ligamentous laxity, and scleral discoloration. Children with OI may suffer from complex neurosurgical problems affecting the brain and spine. The authors sought to determine the neurosurgical implications of OI in a cohort of patients treated at a quaternary care center for pediatrics. The authors reviewed the case histories of 10 children with OI treated by the neurosurgical service at the Hospital for Sick Children in Toronto between January 1988 and March 2007...
March 2008: Journal of Neurosurgery. Pediatrics
K Sperry
Osteogenesis imperfecta (OI) is an uncommon inherited systemic disorder of the connective tissues characterized primarily by varying degrees of bony fragility. Consequently, individuals affected by this condition frequently suffer severe skeletal injuries from otherwise innocuous traumatic events. This syndrome has other associated abnormalities, including hydrocephalus and brain stem compression on the basis of cranial developmental defects (platybasia), cardiac and vascular problems, respiratory disease from spinal deformities, vascular fragility, a bleeding disorder caused by an apparent platelet function abnormality, and anesthesia-related hyperpyrexia...
March 1989: American Journal of Forensic Medicine and Pathology
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