RESUMO
Chondrodysplasia punctata is a group of congenital bone and cartilage disorders characterized by erratic calcification during development. Laryngeal and tracheal calcification and subsequent stenosis, while being reported in several cases of chondrodysplasia punctata, are not frequent findings and there are no proposed management techniques. We describe here a case of an infant with chondrodysplasia punctata associated to tracheal stenosis that was successfully treated with balloon dilation, and with long term follow-up.
Assuntos
Condrodisplasia Punctata/diagnóstico , Estenose Traqueal/etiologia , Condrodisplasia Punctata/complicações , Humanos , Lactente , MasculinoRESUMO
We conducted a retrospective study to analyze various aspects of tracheotomy in children with abnormal laryngotracheal anatomy, a congenital cardiac anomaly, both, or neither. Our study population consisted of 53 children who had undergone tracheotomy performed by a single otolaryngologist; 12 patients had abnormal laryngotracheal anatomy and 9 had a cardiac anomaly. Mean operative times were significantly longer in both of these groups than they were in children with normal anatomy (69 vs. 50 min; p < 0.0001) and in children with no cardiac anomaly (65 vs. 53 min; p < 0.05). Post-tracheotomy survival at 1 and 2 years for children with a cardiac anomaly was significantly worse than survival for those without (44 vs. 91%; p < 0.001). Likewise, children who had been intubated for 10 days or more had significantly worse survival than did those who were intubated for less than 10 days (55 vs. 95%; p < 0.001). Finally, we found that the patients with prolonged intubation, respiratory failure, and a cardiac surgical history had higher mortality rates associated with tracheotomy.