Anesthetic management of a child with a laryngeal mass associated with Laryngo-onycho-cutaneous syndrome

Abstract

With fewer than 50 reported cases, Laryngo-onycho-cutaneous syndrome is a rare condition presenting in childhood with skin and conjunctival ulcerations and laryngeal pathology. A 4-year-old girl presented with recent onset aphonia. Airway endoscopy revealed a laryngeal mass. Tracheostomy was advised but parents denied consent. Two weeks later, she developed severe stridor warranting emergent tracheostomy. The child was diagnosed as Laryngo-onycho-cutaneous syndrome, also known as Shabbir syndrome. Subsequently, she was taken up for laser excision of the mass. Here, we describe the anesthetic management in such airway procedures. Total intravenous anesthesia and local anesthetic in the form of vocal cord spray are safe and environment-friendly alternatives to inhalational anesthesia for airway endoscopies.