Sunday, June 24, 2012

Title: Congenital Chylothorax

Woodrow Wilson (Gaylord National Harbor)
Nagendra Monangi, MD , Neonatal-Perinatal Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Discipline: Newborn Care (N)

Learning Objectives:
  1. Summarizes the clinical course of chylothorax in the neonatal period
  2. Review etiopathogenesis, diagnosis and management of congenital chylothorax
  3. Outline the prognosis and outcomes of congenital chylothorax
Submission Description:
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 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:  

Preterm male infant was born weighing 4695 grams at 35 week-gestation to a 33 year–old mother by cesarean section due to weight gain of 20 lb 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 umblical lines and intratracheal epinephrine and surfactant. Apgar scores were documented as 0, 1, 3, 7 at 1, 5, 10 and 15 minutes. A chest radiograph revealed complete white-out suggestive of massive pleural effusions and bilateral chest tube placements produced 200ml of straw-coloured pleural fluid.

Composition of pleural fluid with predominance of lymphocytes and chylomicrons with no evidence of infections, genetic or metabolic anamolies and cardiac disease suggest congenital chylothorax as the diagnosis. Chest tube output initially went up to a maximum of 900 ml/day, dropped slowly to 300 ml/day by day 10. The patient was extubated on Day11 however continued to put out 200-300 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 23-32. On octreotide, chest tube out put dropped significantly with no side effects and both chest tubes were discontinued on day 30. Slowly advancing feedings with Provimin were commenced on day 4 of octreotide. The baby 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 neonate, 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.

Keywords: Chylothorax, Octreotide, pleural fluid, chest tube