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Inherited, systemic disease characterised by ossification of cartilage, ligaments and intervertebral discs. Primary concerns related to articular manifestations are the risk of difficult intubation, difficult neuraxial anesthesia, and chronic pain. Concerns related to extraarticular manifestations include cardiovascular involvement (cardiomyopathy, conduction or valvular defects), and reduced chest wall compliance.

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General airway assessment, paying particular attention to neck mobility, cricoarytenoid/laryngeal involvement (hoarse voice), and involvement of TMJ (poor mouth opening)

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Consider pulmonary function tests if significant kyphosis is present. Reduced vital capacity may indicate the need for disposition to a high dependency unit following surgery. #ref

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Consider transthoracic echocardiogram if patient has symptoms/signs suggestive of cardiac involvement.

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ECG may reveal conduction defects (eg sinoatrial blocks) that need to be managed by a cardiologist prior to proceeding. #ref

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Neuraxial anesthesia may be difficult, but still consider attempting if indicated.

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Aug 15, 2011
70% success rate in this Canadian case series. refl.in/de
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Take note of any musculoskeletal deformities to optimise intraoperative positioning.

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Taylor's approach for spinal anesthesia via the lumbosacral foramin may be an approach that works if standard approach fails. refl.in/dd

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