Anesthesia for orthopedic surgery can offer distinct challenges for the pediatric anesthesiologist. In addition to the potential for life-threatening surgical blood loss or even management of tourniquet pain, the pediatric anesthesiologist may be faced with significant coexisting diseases which include but are not limited to cardiopulmonary, oncologic, or other systemic diseases. Coexisting diseases Cardiopulmonary disease Managing the patient with significant cardiac disease presents a unique comorbidity when delivering anesthesia for pediatric orthopedic surgery. For example, 25% of patients with idiopathic scoliosis can also have mitral valve prolapse. Where cardiac disease usually comes into play with orthopedic surgery is when pulmonary dysfunction from restrictive lung disease promotes pulmonary hypertension. In this section we will present some of the more common patient conditions with cardiopulmonary disease that may be encountered when delivering anesthesia to pediatric patients for orthopedic surgery. Marfan syndrome is a variable, autosomal dominant connective tissue disorder. It involves mutation in the fibrillin 1 gene on chromosome 15. Marfan syndrome patients usually present in orthopedic surgery with spine pathology such as kyphoscoliosis, spondylolisthesis, and atlantoaxial subluxation (1). Other surgical presentations include neonatal hip dislocation, protrusio acetabulae, pes planus, hand/wrist instability, and shoulder/knee instability (2). The currently accepted diagnostic tool are the Ghent criteria, which include skeletal, ocular, cardiovascular, pulmonary, and integumentary organ systems (3). A diagnosis of Marfan syndrome requires at least two major criteria in two systems and involvement of a third. Major criteria include dilation of ascending aorta, dissection of ascending aorta, lens dislocation, dural ectasia, proven mutation, family history, and > four skeletal findings (which include pectus carinatum, pectus excavatum requiring surgery, wrist and thumb signs, scoliosis >20 degrees or spondylolisthesis, reduced elbow extension, pes plenus, and protrusion acetabulae).
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