Severe bronchial disease (bronchial casts, bronchiectasis) and partial anomalous pulmonary venous drainage in two children with 45, X Turner's syndrome.

The prevalence of cardiovascular malformation is high in Turner's syndrome. We report two cases of partial anomalous pulmonary venous drainage associa ted with a severe bronchial disease. Case reports. - Case 1: a 5-year-old girl with monosomy X presented with ac ute respiratory failure. The chest X-ray showed an atelectasis of the left lower lobe. The patient improved following bronchoscopic removal of a bronc hial cast. Dr(ring hospitalization, apr anomalous pulmonary venous drainage of the right lung was discovered In spire of complete surgical repair of t he cardiac malformation, several episodes of respiration failure occurred s everal years after the operation. Case 2: a 9-year-old girl with monosomy X and coarctation of the aorta underwent pre-operative cardiac catheterizati on. She had a history, of bronchiectasis, leading to a lobectomy. Her respi ratory condition improved following surgery. A partial anomalous pulmonary venous return was discovered during the cardiac angiography. The coarctatio n was repaired surgically. The correction of the left-to-right shunt was no t necessary. Conclusion. - The partial anomalous pulmonary venous return is frequently r eported in Turner's syndrome. The possible respiratory disease associated w ith this cardiac malformation is usually described us (1 consequence of the increase in pulmonary blood flow. Our two cases did not match this hypothe sis. (C) 1999 Editions scientifiques et medicales Elsevier SAS.