Congenital Chylothorax

Authors


Poster Presentation

Background

Chylothorax is caused by chyle containing lymphatic fluid draining into the pleural cavity. Chylothorax is the most common type of pleural effusion during the neonatal period, may cause significant respiratory compromise, and is associated with substantial mortality and morbidity. This report summarizes the course of a 35 week-gestation neonate with bilateral congenital chylothorax with a review of etiology, radiographic and laboratory diagnosis, and successful management of chylothorax with octreotide.

Case

A preterm male infant was born weighing 4,695 grams at 35 weeks gestation to a 33-year-old mother by cesarean due to weight gain of 20 pounds in 2 weeks, limb edema, hypertension, polyhydramnios, fetal distress, and face presentation. There was no significant maternal medical history and 8 and 20 weeks gestation ultrasounds were reported as normal. Resuscitation at delivery included intubation with assisted ventilation, placement of umbilical lines, and intratracheal epinephrine and surfactant. Apgar scores were documented as 0, 1, 3, 7 at 1, 5, 10, and 15 min. A chest radiograph revealed complete white-out suggestive of massive pleural effusions and bilateral chest tube placements produced 200 ml of straw-colored pleural fluid.

Composition of pleural fluid with predominance of lymphocytes and chylomicrons with no evidence of infections, genetic or metabolic anamolies and cardiac disease suggested congenital chylothorax. Chest tube output initially went up to a maximum of 900 ml/day and dropped slowly to 300 ml/day by day 10. The patient was extubated on day 11 but continued to put out 200 to 300 ml/day of chyle requiring the chest tubes to be left in situ. Octreotide was administered intravenously initially at 1 mcg/kg/hr titrating to a maximum dose of 7 mcg/kg/hr from days 23 to 32. With octreotide, chest tube output dropped significantly with no side effects and both chest tubes were discontinued on day 30. Slowly advancing feedings with a protein-vitamin-mineral formula were commenced on day 4 of octreotide. The infant was fully bottle and breast fed with resolution of chylothorax and respiratory distress on day 40.

Conclusion

Congenital chylothorax, an uncommon cause of respiratory distress in neonates, is diagnosed initially by prenatal ultrasound or postnatal x-ray and definitely by evaluation of pleural fluid in the pleural space. Standard management of neonatal chylothorax usually entails prolonged hospitalization requiring multiple procedural or surgical interventions. Nutritional status, along with fluid and electrolytes, needs to be monitored closely. Administration of octreotide in our case led to a more rapid resolution of pleural drainage, no recurrence, and early hospital discharge.

Ancillary