Severe bronchial disease (bronchial casts, bronchiectasis) and partial anomalous pulmonary venous drainage in two children with 45, X Turner's syndrome.
F. Counil et al., Severe bronchial disease (bronchial casts, bronchiectasis) and partial anomalous pulmonary venous drainage in two children with 45, X Turner's syndrome., ARCH PED, 6(10), 1999, pp. 1070-1074
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.