PEDIATRICS Vol. 31 No. 3 March 1963, pp. 416-425
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CONGENITAL DILATATION OF THE PULMONARY LYMPHATICS

S. N. Javett M.D.1, I. Webster B.Sc., M.B., B.Ch.1, and J. L. Braudo M.D., M.R.C.P.1

1 The Transvaal Memorial Hospital for Children, Johannesburg, South Africa

A case of congenital dilation of the pulmonary lymphatics is described, the infant being still alive at the age of 9 months. The distinctive clinical features dating from the age of 3 days were marked distension of the thoracic cage, dyspnea with inspiratory recession, prolonged expiration without adventitious sounds, and the absence of cough. X-rays of the chest showed voluminous lungs which altered little on expiration. At thoracotomy, the lungs were found to have a peculiar sponge rubber consistency, deflating tardily to compression and leaving visible finger imprints. There were no cysts large enough to be seen with the naked eye. Biopsies of the lungs taken at an interval of 6 months showed persistent and unaltered gross dilatation of the subpleural, perivascular, and septal lymphatics. No other pathology was present in the lungs. Later the infant became subject to spontaneous attacks of wheezing, associated with cough. Despite the troubled course, the patient gained weight reasonably. The cushioning of the lungs by the dilated lymphatics is the probable factor responsible for the restricted movement and all other associated respiratory signs. Infection and cor pulmonale are the complications most to be feared.

Submitted on May 15, 1962
Accepted on September 7, 1962




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