Upper airway complications of junctional epidermolysis bullosa.
J Pediatr
; 160(4): 657-661.e1, 2012 Apr.
Article
in En
| MEDLINE
| ID: mdl-22050875
ABSTRACT
OBJECTIVE:
To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB). STUDYDESIGN:
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Tracheal Diseases
/
Laryngeal Diseases
/
Epidermolysis Bullosa, Junctional
Type of study:
Guideline
/
Observational_studies
/
Risk_factors_studies
Limits:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Language:
En
Journal:
J Pediatr
Year:
2012
Type:
Article
Affiliation country:
United States