kyphoscoliosis repair and anesthesia

Congenital contractural arachnodactyly (CCA) is a rare connective tissue disorder that has several phenotypic similarities to Marfan syndrome. Among the phenotypic characteristics of patients with CCA, severe kyphoscoliosis and thoracic cage abnormalities are commonly reported. In this case report, we describe a patient with coexisting CCA and severe pectus excavatum requiring multiple surgical repairs. The impact severe scoliosis and pectus excavatum in isolation have on cardiopulmonary anatomy and physiology can be significant, and their effects can be profound concomitantly...

The presence of kyphoscoliosis can adversely affect gas exchange because of restriction to gas flow and reduction of lung volume. The effects become more exaggerated during positive-pressure ventilation due to the uneven distribution of ventilation. The use of a double-lumen tube helps to reduce the ventilation perfusion mismatch that occurs because of positive-pressure ventilation. We report a patient with severe kyphoscoliosis who underwent repair of an atrial septal defect, in whom a double-lumen tube was used for ventilation and the conduct of general anesthesia...

An 18-year-old male with Marfan's syndrome underwent surgery for repair of an ascending aortic dissection. The clinical features of this congenital syndrome and the acute management of its complications are described. As elective surgery is frequently required for ocular, musculo-skeletal or cardiovascular problems in people with Marfan's syndrome, patients must be carefully assessed preoperatively for specific complications. Significant pulmonary problems include restriction of lung function due to pectus excavatum or kyphoscoliosis and intrinsic pulmonary involvement with emphysema, bronchogenic cysts and "honeycomb lung...