Tracheal bronchus: association with respiratory morbidity in childhood.

An aberrant right upper lobe (RUL) bronchus arising from the trachea (tracheal bronchus) can be responsible for recurrent pneumonia. In this hospital, 2% of children requiring bronchoscopy for respiratory symptoms are found to have a tracheal bronchus, which is frequently thought to be an incidental finding. We reviewed findings in 18 patients to determine when a tracheal bronchus is of clinical significance. The age at presentation ranged from 1 day to 54 months (mean 17 months). The children had recurrent pneumonia (nine), stridor (six), respiratory distress (two) and a thoracic mass (one). Other congenital abnormalities were present in 14, including Down syndrome (two), tracheoesophageal fistula (two), and fused or hypoplastic first and second ribs (four). Recurrent RUL pneumonia was present in five. Bronchiectasis or bronchial stenosis was shown by bronchography in four of five; in all five the right upper lobe was surgically resected, with resolution of the recurrent pneumonias. The presence of a clinically significant tracheal bronchus should be considered in every child with recurrent RUL pneumonia, especially in children with Down syndrome or rib abnormalities; if bronchiectasis or bronchial stenosis is found, surgical resection should be performed.

Pneumothorax in cystic fibrosis: management and outcome.

We reviewed our experience over the past 12 years to determine the best method of management, to determine the morbidity and the physiologic outcome of medical vs surgical treatment of pneumothorax complicating CF, and to assess the influence of age, sex, and Shwachman scores on survival. Sixty-five patients, ages ranging from 5 to 32 years (mean 18 years). Shwachman scores ranging from 25 to 87 (mean 57), and a male-female ratio of 1:1, experienced 170 pneumothoraces, 93 first episodes, and 77 recurrences, requiring 211 trials of management. All methods of management except needle aspiration resulted in a fair rate of resolution (70 to 100%), but recurrence rates were high for observation (60%), needle aspiration (79%), trocar thoracotomy (63%), tetracycline sclerosis (86%), and silver nitrate sclerosis (43%). The recurrence rates were 12.5% for quinacrine sclerosis and 0% for parietal pleurectomy. Quinacrine sclerosis and parietal pleurectomy were the most effective methods of management. There was no significant difference in pulmonary function before pneumothorax and after pleural sclerosis or parietal pleurectomy. Age, sex, and severity of pulmonary disease were all independent variables influencing prognosis. Severity of disease, rather than the occurrence of a pneumothorax, appears to be the major cause of death. We recommend that quinacrine sclerosis should be considered for management of the first pneumothorax, and parietal pleurectomy if it fails.

The lung following repair of congenital diaphragmatic hernia.

To determine the effects of the pulmonary hypoplasia present at birth in infants with congenital diaphragmatic hernia upon subsequent development of the lung, 19 patients who had undergone surgical repair before the age of one year were studied at ages 6 to 18 years. Total lung capacity and vital capacity averaged 99% of predicted value. Diffusing capacity for carbon monoxide was normal. Forced expiratory volume in one second averaged 89% of predicted value and 80% of vital capacity. Total respiratory system conductance and maximum expiratory flow volume curves obtained during air and helium-oxygen breathing were normal. Xenon 133 radiospirometry performed in nine patients revealed equal distribution of lung volumes on the two sides. Ventilation to the hernia side was reduced in only two patients. Blood flow to the hernia side was reduced in all nine patients. Chest radiographs supported the physiologic observations. These findings are consistent with the persistence of a reduction in the number of branches or generations of pulmonary arteries and bronchi on the side of the hernia. Since a substantial part of the vascular resistance resides in peripheral vessels, this developmental abnormality influences the distribution of pulmonary blood flow, although it has little effect on tests reflecting airway resistance or the distribution of ventilation.