title: Successful treatment of laryngeal stenosis in laryngo-onycho-cutaneous syndrome with topical mitomycin C.
abstract: Laryngo-onycho-cutaneous syndrome is a very rare entity found in Punjabi families. It affects the skin, nails, and larynx. Laryngeal involvement may cause lethal airway obstruction, and has in the past proved very difficult to treat. Mitomycin C is an antibiotic that acts as an alkylating agent, inhibiting DNA synthesis. It reduces fibroblast proliferation, and has previously been used to treat choanal atresia and laryngeal stenosis. We report an 18-year-old man with complete transglottic laryngeal stenosis secondary to laryngo-onycho-cutaneous syndrome. An airway was established by dissection with a bougie and sickle knife, and was initially maintained by the upper limb of a Montgomery T-tube. Laryngeal granulation tissue present on removal of the T-tube was treated with topical mitomycin C (2 mg/mL) applied for 4 minutes on two occasions with an interval of 1 month. A year later, the airway remained patent, with no granulation tissue.
authors.affiliation: Department of Pediatric ENT, Great Ormond Street Hospital for Children, London, United Kingdom. seamusphillips@hotmail.com
authors.forename: P Seamus
authors.initials: PS
authors.lastname: Phillips
authors.forename: Kwame
authors.initials: K
authors.lastname: Amonoo-Kuofi
authors.forename: Ian D B
authors.initials: ID
authors.lastname: Hore
authors.forename: David J
authors.initials: DJ
authors.lastname: Atherton
authors.forename: David M
authors.initials: DM
authors.lastname: Albert
mesh_terms: D000287:Administration, Topical; D000293:Adolescent; D000402:Airway Obstruction / Q000209:etiology*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005500:Follow-Up Studies; D006801:Humans; D007828:Laryngoscopy / Q000379:methods; D007829:Laryngostenosis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D016685:Mitomycin / Q000627:therapeutic use*; D014009:Onychomycosis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D018570:Risk Assessment; D012720:Severity of Illness Index; D012872:Skin Diseases, Vesiculobullous / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D016685:Mitomycin