Supraglottic Mass Management in a Pediatric Patient with Proteus Syndrome

Proteus Syndrome is a rare genetic condition that is characterized by vascular, lymphatic malformation and asymmetric overgrowth of skin, bone, central nervous system, and adipose tissue.^1,2  We present a computed tomography image of an asymmetrically enlarged left tonsil with near complete obstruction of the oropharynx in a 5-year-old boy with Proteus Syndrome (left image). The mass can also be visualized, with rigid bronchoscopy, touching the base of the tongue in the image on the right.

Airway management in patients with Proteus Syndrome is often challenging because of facial asymmetry or mandibular or cervical deformity.²  Direct laryngoscopy in these patients is often unsuccessful with grade III or IV views. Additionally, these patients may also present with obstructive, soft tissue airway masses that are not well visualized on simple x-ray.³  Computed tomography imaging should be considered early as part of the preoperative assessment. Given the risk of complete airway obstruction due to pharyngeal muscle relaxation during general anesthesia, the preferred option for a definitive airway securement in these pediatric patients is maintenance of spontaneous ventilation under general anesthesia during airway instrumentation. Flexible fiberoptic intubation has been successful in patients with Proteus Syndrome, but this may not be the case for patients with large supraglottic masses due to bleeding and inability to navigate around the obstruction.³  For these patients, rigid bronchoscopy for examination and intubation by otolaryngology is a superior technique with a likely higher chance of success. The main advantage of using a rigid bronchoscope is to better displace the mass and reach the glottis.