Upper airway complications of junctional epidermolysis bullosa.

[junctional epidermolysis bullosa]

To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB ).We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010 .Twenty -five patients with JEB were identified , and 12 patients were seen in the otolaryngology clinic (age range , 2 months -15 years ; 8 male , 4 female ). Of the 12 patients , 8 underwent rigid laryngoscopy and bronchoscopy for upper respiratory tract symptoms ; 7 of these patients displayed laryngeal pathology , and 5 of them underwent surgical intervention with successful resolution of symptoms . Furthermore , none of these patients had any short - or long -term complications from their surgery . A strict protocol and a precise problem-focused cold surgical technique were used in these cases to protect skin and mucus membranes .With appropriate precautions , endoscopic laryngeal surgery can be safe and effective in patients with JEB and larygnotracheal disease . Endoscopic laryngeal surgery is feasible when indicated for these patients .