The role of bronchoscopy in the management of patients with severe craniofacial syndromes.
OBJECTIVE
The purpose of this study is to assess the incidence of airway anomalies in children with severe craniofacial syndromes and to establish the role of bronchoscopy in the care of these patients.


METHODS
Consecutive children with craniofacial syndromes, including both bony deformities of the skull and face, in which a bronchoscopy was performed between 1995 and 2010 were retrospectively reviewed.


RESULTS
Thirty-six patients (22 boys, 14 girls; mean age, 39 months) were studied. Craniofacial synostosis was present in 21 patients (Crouzen syndrome, 11; Apert syndrome, 7, Pfeiffer syndrome, 3) and craniofacial dysostosis in 15 (Goldenhart syndrome, 8; Treacher Collins syndrome, 7). In 30 patients (83.3%), bronchoscopy was performed because of respiratory symptoms (apneic episodes, 22; respiratory distress, 13; stridor, 6; cyanosis, 1) and, in the remaining 6 (asymptomatic children), during guided tracheal intubation before a surgical procedure. Airway anomalies were found in 69.4% of patients (70% in symptomatic patients). Management consisted of tracheotomy in 13 patients, adenoidectomy/tonsillectomy in 13, glossopexy in 5, antireflux surgery in 3, and supraglottoplasty in 2.


CONCLUSIONS
Airway anomalies occurred in 70% of children with severe craniofacial syndromes and respiratory symptoms. Bronchoscopy should be performed routinely in this selected group of patients, and the entire airway must be examined. Treatment should be tailored to each individual patient.
Pediatric Airway Unit, Division of Pediatric Surgery, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, E-28041, Spain. janton.hdoc@salud.madrid.org
Juan L
JL
Antón-Pacheco
Carmen
C
Luna Paredes
Antonio
A
Martínez Gimeno
Gloria
G
García Hernández
Rubén
R
Martín de la Vega
Ana
A
Romance García
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery; D000233:Adenoidectomy; D000293:Adolescent; D000402:Airway Obstruction / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D000465:Algorithms; D001999:Bronchoscopy; D002648:Child; D002675:Child, Preschool; D002754:Choanal Atresia / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D019465:Craniofacial Abnormalities; D019468:Disease Management; D017558:Elective Surgical Procedures; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007442:Intubation, Intratracheal; D007819:Laryngeal Edema / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D015619:Respiratory System Abnormalities / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery; D012189:Retrospective Studies; D014059:Tongue / Q000601:surgery; D014068:Tonsillectomy; D014140:Tracheotomy