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December 1997

Complications Following Airway Surgery in Noonan Syndrome

Author Affiliations

From the Division of Pediatric Otolaryngology, Department of Otolaryngology, University of Pittsburgh School of Medicine, and Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, Pittsburgh, Pa.

Arch Otolaryngol Head Neck Surg. 1997;123(12):1341-1343. doi:10.1001/archotol.1997.01900120091015

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

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