Anesthetic management in a patient with osteogenesis imperfe
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Abstract
Osteogenesis imperfecta (OI) is a rare genetically inherited syndrome involving connective tissues, resulting in anatomic and physiologic abnormalities, which results in any form of anesthesia, a challenging task. We hereby report a case of OI type I presented with distinctively blue sclera, hearing loss, kyphoscoliosis, and mild pulmonary restrictive disease who underwent rush nail removal in the femur.

CASE REPORT
A 15-year-old female presented in preanesthetic checkup clinic for rush nail removal of femur. She was a known case of OI type I and history of recurrent fractures for which she had been operated three times uneventfully under general anesthesia. There was no history of OI in the family.On general examination, she was short statured with a height of 103 cm, weighing 49 kg, blue sclera, fragile bones, progressive hearing loss, kyphoscoliosis. Her blood pressure was 130/80 mmHg, and heart rate was 80 beats/min. There was no pallor, icterus, cyanosis, clubbing, lymphadenopathy, or pedal edema. Respiratory system revealed barrel-shaped chest with bilateral equal air entry, and cardiovascular system revealed normal heart sounds. Electrocardiogram (ECG), liver function test, renal function test, and coagulation profile were normal. Chest X-ray was normal except mediastinal widening; X-ray of the spine revealed dorsolumbar kyphoscoliosis to the left. Pulmonary function test was suggestive of mildly restrictive disease. Airway assessment showed acceptable flexion and extension at neck with adequate mouth opening and normal dentition, Mallampatti Class III. The patient was accepted for surgery as American Society of Anesthesiologists Grade III; in view odontoaxial dislocation and normal coagulation profile, we decided to proceed with a spinal anesthetic technique. Thorough operating room preparation was completed including difficult airway equipment, and measures made ready to deal with hyperthermias if any.....

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5062238/?report=reader
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