1/6. Combination of renal agenesis with respiratory and alimentary tract atresia results in normal lung development.The VACTERL complex comprises renal agenesis and atresias of the alimentary and respiratory tracts. We report on a case with this combination causing severe oligohydramnios but with normal lung development. The likely protective mechanism for pulmonary development was an increase in alveolar pressure and reduced alveolar fluid loss due to the esophageal-tracheal malformation. This suggests the possible treatment of oligohydramnios by tracheal occlusion.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/6. Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm.OBJECTIVES: To refine the classic definition of, and provide a working definition for, congenital high airway obstruction syndrome (CHAOS) and to discuss the various aspects of long-term airway reconstruction, including the range of laryngeal anomalies and the various techniques for reconstruction. DESIGN: Retrospective chart review. patients: Four children (age range, 2-8 years) with CHAOS who presented to a single tertiary care children's hospital for pediatric airway reconstruction between 1995 and 2000. CONCLUSIONS: To date, CHAOS remains poorly described in the otolaryngologic literature. We propose the following working definition for pediatric cases of CHAOS: any neonate who needs a surgical airway within 1 hour of birth owing to high upper airway (ie, glottic, subglottic, or upper tracheal) obstruction and who cannot be tracheally intubated other than through a persistent tracheoesophageal fistula. Therefore, CHAOS has 3 possible presentations: (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a tracheoesophageal fistula, and (3) near-complete high upper airway obstruction. Management of the airway, particularly in regard to long-term reconstruction, in children with CHAOS is complex and challenging.- - - - - - - - - - ranking = 0.4keywords = atresia (Clic here for more details about this article) |
3/6. Bronchial atresia associated with spontaneous pneumothorax: report of a case.A 32-yr-old male patient with recurrent pneumothorax associated with bronchial atresia of the subsegmental branch of the posterior segmental bronchus of the right upper lobe was successfully treated with right upper lobectomy. Before surgery, the bronchial atresia with pneumothorax was suspected on the chest radiograph and CT scans, which showed the findings of bronchocele with localized hyperinflation of the right upper lobe. The examination of surgical specimen from the resected right upper lobe suggests that the cause of the recurrent pneumothorax was the rupture of the subpleural bullae in the hyperinflated lung segment distal to the atretic bronchus.- - - - - - - - - - ranking = 1.2keywords = atresia (Clic here for more details about this article) |
4/6. Tracheal agenesis: management of the first 10 months of life.Tracheal agenesis is a potentially lethal congenital anomaly, appearing only at birth. We describe a newborn preterm infant who presented with immediate respiratory distress and no audible cry. There was almost complete tracheal agenesis with a very short segment of distal trachea (only two tracheal rings) arising from the anterior wall of the esophagus, before dividing into the mainstem bronchi. The anomaly was unsuspected prenatally, as the scan showed pyloric atresia and complex congenital cardiac disease. Despite the patient's difficult course, with correction of the rare-associated malformations (cardiac and gastrointestinal tract anomalies), the fact that the child is lively and neurologically normal for her age, requires that we now consider the patency of the airway and the possibility of surgical correction, in accordance with a good quality of life.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
5/6. prenatal diagnosis and management of mainstem bronchial atresia.The prenatal diagnosis, natural history and management of mainstem bronchial atresia have not been described previously. We report two cases of prenatally diagnosed proximal bronchial atresia. The first patient presented at 18 weeks with sonographic and MRI findings consistent with bronchial atresia with fetal hydrops. The mother developed the mirror syndrome and labor was induced. A non-viable fetus was delivered at 25 weeks. The second patient presented at 16 weeks gestation with evidence of an intrathoracic mass that was subsequently prenatally diagnosed as a right mainstem bronchial atresia. The right lung increased rapidly in size and was associated with the onset of fetal hydrops. At 24 weeks, fetal pneumonectomy was performed but the fetus expired intraoperatively due to cardiovascular collapse. Post-mortem findings in both cases confirmed the presence of an atretic mainstem bronchus with massive enlargement of the lung. Bronchial atresia involving the mainstem bronchus is associated with a poor prognosis.- - - - - - - - - - ranking = 1.8keywords = atresia (Clic here for more details about this article) |
6/6. Congenital bronchial atresia associated with spontaneous pneumothorax.A 36-year-old woman presented with left chest pain and frequent symptoms of upper respiratory infection. Chest roentgenograms revealed a left pneumothorax and apical bulla, and hyperlucency in the left pulmonary field. She was diagnosed with congenital bronchial atresia associated with a left spontaneous pneumothorax. A thoracoscopy-assisted left superior segmentectomy was performed. There was no recurrence of the pneumothorax or symptoms of recurrent upper respiratory infection at the 1-year follow-up examination. Bulla formation was believed to have resulted from emphysematous changes in the peripheral lung due to congenital bronchial atresia. The pneumothorax may have occurred due to rupture of the bulla.- - - - - - - - - - ranking = 1.2keywords = atresia (Clic here for more details about this article) |