Objective: To analyze the outcomes of patients undergoing repair of congenital tracheal stenosis. Methods: This was a retrospective review of congenital tracheal stenosis patients treated between 2001 and 2007 at the University of São Paulo School of Medicine Hospital das Clínicas in São Paulo, Brazil. Results: Six boys and one girl (age at diagnosis ranging from 28 days to 3 years) were included. Five of the patients also had cardiac or major vessel malformations. The stenosis length was short in three patients, medium in one and long in three. The techniques used were pericardial patch tracheoplasty in three patients, resection and anastomosis in two, slide tracheoplasty in one and vascular ring correction in one. One patient died during surgery due to hypoxia and hemodynamic instability, and one died from septic shock on postoperative day 11. Other complications included pneumonia, arrhythmia, stenosis at the anastomosis level, residual stenosis, granuloma formation and malacia. The mean follow-up period was 31 months; four patients were cured, and one required the use of a T-tube to maintain airway patency. Conclusions: Congenital tracheal stenosis is a curable disease. However, its repair is complex and is associated with high rates of morbidity and mortality.

Keywords: Tracheal stenosis/congenital; Tracheal diseases; Surgical procedures, operative.

Introduction: The diagnosis of diffuse lung disease is still a challenge for the pediatric intensive care physician. Routine clinical examinations and laboratory tests are frequently negative. The objective of this study was to evaluate the diagnostic potential, the impact on therapy and the rate of complications of open lung biopsy in children with undiagnosed diffuse lung disease, respiratory failure and inappropriate response to initial therapy. Methods: From January 1987 to January 1997, 29 children with diffuse pulmonary disease of unknown etiology, respiratory failure (PaO2 /FiO2 < 300) and no response to previous treatments were considered for open lung biopsy. Newborns, children with known chronic pulmonary disease and children with untreatable shock or coagulopathy were excluded. All biopsies were performed by a thoracic surgeon by a microthoracotomy in the lung shown to be the most affected by X-ray examination. Tissue samples were analyzed in terms of cultures, light microscopy, electron microscopy and immunofluorescence microscopy, according to the pathologist's decision. Results: All biopsies (100%) resulted in at least one histological diagnosis and in 20 patients (68.9%) it was obtained a specific diagnosis. The most frequent histological patterns found were: non-specific interstitial pneumonitis with variable degrees of fibrosis in 18 cases; bronchiolitis in eight cases and pulmonary hypertension in three cases. Regarding the most frequent specific diagnosis, six children were found with cytomegalovirus infection, three with Pneumocystis carinii, three with adenovirus and three with respiratory syncytial virus infection. These data induced a change in therapy in 20 children (68.9%). The most frequent changes in therapy were the use of corticosteroids in 14 children and a review of the antibiotic regimen in six patients. Seven patients (24.1%) presented with complications that were easily resolved. There were 13 deaths, probably due to the critical conditions of these patients, all unrelated to the procedure. Conclusions: Open lung biopsy, though an invasive procedure, should be considered in the evaluation of selected children with undiagnosed diffuse lung disease, respiratory failure and with no satisfactory response to previous therapies.