TY - JOUR
T1 - Upper airway complications of junctional epidermolysis bullosa
AU - Ida, Jonathan B.
AU - Livshitz, Irina
AU - Azizkhan, Richard G.
AU - Lucky, Anne W.
AU - Elluru, Ravindhra G.
PY - 2012/4
Y1 - 2012/4
N2 - Objective: To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB). Study design: 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. Results: 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. Conclusion: 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.
AB - Objective: To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB). Study design: 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. Results: 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. Conclusion: 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.
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U2 - 10.1016/j.jpeds.2011.09.029
DO - 10.1016/j.jpeds.2011.09.029
M3 - Article
C2 - 22050875
AN - SCOPUS:84858332413
VL - 160
SP - 657-661.e1
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 4
ER -