Chondrodysplasia punctata presenting with tracheal obstruction.

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.

Management of postintubation tracheal stenosis: appropriate indications make outcome differences.

BACKGROUND: Laryngotracheal stenosis is difficult to treat and its etiologies are multiple; nowadays, the most common ones are postintubation or posttracheostomy stenoses. OBJECTIVE: To provide an algorithm for the management of postintubation laryngotracheal stenoses (PILTS) based on the experience of a tertiary care referral center. METHODS: A retrospective study was conducted on all patients treated for PILTS over a 10-year period. Patients were divided into a surgically and an endoscopically treated group according to predefined criteria. The characteristics of the two groups were analyzed and the outcomes compared. RESULTS: Thirty-three consecutive patients were included in the study: 14 in the surgically treated group and 19 in the endoscopically treated group. Our candidates for airway surgery were healthy patients presenting with complex tracheal stenoses, subglottic involvement or associated tracheomalacia. The endoscopic candidates were chronically ill patients presenting with simple, strictly tracheal stenoses not exceeding 4 cm in length. Stents were placed if the stenosis was associated with tracheomalacia or exceeded 2 cm in total length. In the surgically treated group, 2/14 patients needed more than one procedure versus 8/19 patients in the endoscopically treated group. At the end of the intervention, 50% of the patients were decannulated in the surgically treated group versus 84.2% in the endoscopically treated group (p = 0.03). However, the decannulation rates at 6 months and the symptomatology at rest and on exertion on the last follow-up visit were comparable in the two groups. CONCLUSION: Our experience in the management of PILTS demonstrates that both surgery and endoscopy yield excellent functional outcomes if the treatment strategy is based on clear, predefined objective criteria.