Chylothorax Associated with Noonan Syndrome

Abstract

Treatment of chylous pleural effusions with chest tube thoracostomy and pleurodesis has had limited success despite the sclerosing agent used. It also usually requires prolonged drainage, which could lead to nutritional and immune compromise. We report a case of a 41-year-old man with a recurrent chylothorax secondary to Noonan syndrome that was treated successfully with medical thoracoscopic talc pleurodesis. He presented initially with symptoms suggestive of pneumonia, and a chest radiograph showed a right effusion. Pleural studies revealed the effusion was a chylous exudate with negative cytology and cultures. He was treated initially with antibiotics, but the effusion recurred and he required five subsequent thoracenteses for symptomatic relief. He eventually un-derwent medical thoracoscopy with talc pleurodesis for definitive management and is without recurrence 4 months postoperatively. Spontaneous chylothorax associated with Noonan syndrome is a rare complication that typically affects children and can be managed easily with enteric rest. This option is less feasible in an active adult patient, and therapy should include medical thoracoscopic talc pleurodesis because it is more likely to be successful than chest tube thoracostomy pleurodesis.