Challenges in the management of refractory bilateral idiopathic congenital chylothoraces in a newborn

Chylothorax was first described in 1917 by Pisek and is the most common cause of pleural effusion during the neonatal period. Primary (congenital) chylothorax is rare and often idiopathic; it occurs more commonly in males and is more likely to affect the right side of the chest. There are some known associations of congenital chylothorax such as Down's syndrome, Noonan's syndrome, and Turner's syndrome as well as polyhydraminos and hydrops fetalis [6]. Congenital chylothorax typically presents at birth, or within the first week of life. The presentation is one of respiratory distress and the diagnosis is typically confirmed by chest radiographs and the aspiration of a milky, lymphocyte-rich fluid from the pleural space.

The management of congenital chylothorax typically involves the use of chest drains together with either a low fat, high protein diet supplemented with medium-chain triglycerides, or total parenteral nutrition (TPN). In the majority of cases this management is sufficient to result in resolution of the chylothorax. In some instances however, chylothorax can persist. In these cases various treatment methods have been tried, including the use of the somatostatin analogue (octreo­tide) and a variety of surgical techniques. Surgical interventions such as pleurodesis, thoracic duct ligation and pleural peritoneal shunts are usually reserved for the most refractory cases with limited success.

In this case report we discuss the evidence base for the management of congenital chylothoraces and describe the successful resolution of refractory bilateral congenital idiopathic chylothorax in a neonate using a combination of bleomycin pleurodesis, surgical pleurodesis and thoracic duct ligation.