Pleuroperitoneal shunt in the management of chylothorax caused by thoracic lymphatic dysplasia

Three cases of intractable chylothorax secondary to thoracic lymphatic dysplasia were treated by pleuroperitoneal shunt insertion. These cases included on with Gorham's syndrome, and one case with a bilateral chylothorax and chylous ascites. Pleuroperitoneal shunts allowed an adequate internal drainage in all cases, alleviating protein and lymphocyte losses caused by recurrent pleural taps. In the third case with chylous ascites, valved shunts were used to avoid reflux between the peritoneal cavity and the pleural space. Such palliative therapy did not change the bad prognosis of these patients with lymphatic disorders but improved the children's quality of life.