Two children with Noonan syndrome underwent airway surgery and both experienced spontaneous chylothorax after surgery. The Noonan syndrome phenotype may include defects that significantly increase the risk of complications associated with surgery. Manifestations of Noonan syndrome may include congenital heart disease; coagulation factor deficiency; pterygium colli; and lymphangiomatosis of the pleura, lungs, and chest wall that can lead to life-threatening chylothorax. In this article, the 2 cases are presented and the relevant literature is reviewed to increase awareness of the potential problems that may be encountered in patients with Noonan syndrome. Recommendations are made regarding preoperative investigations and perioperative management to prevent complications.
Arch Otolaryngol Head Neck Surg. 1997;123:1341-1343
Yellon RF. Complications Following Airway Surgery in Noonan Syndrome. Arch Otolaryngol Head Neck Surg. 1997;123(12):1341-1343. doi:10.1001/archotol.1997.01900120091015