Bronchoscopic concerns in Proteus syndrome: a case report.

Area of Science:

• Anesthesiology
• Medical Genetics
• Pediatric Surgery

Background:

• Proteus syndrome (PS) is a rare congenital disorder characterized by segmental overgrowth.
• Clinical variability in PS often leads to challenging anesthetic management.
• Airway complications in PS patients undergoing anesthesia are not well-documented.

Purpose of the Study:

• To describe the anesthetic challenges in a pediatric patient with Proteus syndrome.
• To illustrate the utility of fiberoptic bronchoscopy in managing difficult airway in PS.
• To contribute to the understanding of airway anomalies in Proteus syndrome.

Main Methods:

• Case report of an 11-year-old girl with Proteus syndrome undergoing ear surgery.
• Detailed assessment of airway-related features complicating intubation.
• Management of general anesthesia utilizing fiberoptic bronchoscopy.

Main Results:

• The patient presented with facial asymmetry, dental anomalies, limited neck mobility, and scoliosis.
• These features posed significant challenges for endotracheal intubation.
• Fiberoptic bronchoscopy facilitated successful airway management and intubation.

Conclusions:

• Proteus syndrome necessitates careful preoperative evaluation for potential airway difficulties.
• Fiberoptic bronchoscopy is an invaluable tool for managing complex airways in PS patients.
• Further research on anesthetic considerations for Proteus syndrome is warranted.