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Ahmed, Alshafei, Khan, and Gouda: Neonatal bilateral chylothorax treated by octreotide


Chylothorax is defined as the abnormal accumulation of lymphatic fluid in the pleural space. Chyle escapes to pleura either secondary to leakage from the thoracic duct (during birth trauma or surgery) or as a result of congenital thoracic duct obstruction in utero or anomalies of the pulmonary lymphatics.1

In neonates, congenital idiopathic chylothorax is the most common cause of pleural effusion.2 It carries a high risk of mortality and morbidity due to immunodeficiency, malnutrition and invasive procedures.3

Classic management is conservative, involving feeding with a low-fat, high-protein formula, total parenteral nutrition (TPN), octreotide infusion, or surgical intervention by either shunting or pleurodesis.4

Case report

A full-term female infant was delivered by caesarean section at 37 weeks gestation because of fetal bradycardia and had an Apgar score of 8 at 1 minute and of 9 at 5 minutes. The baby’s weight was 3.36 kg (50th centile), her length was 35 cm (below the 10th centile) and her head circumference was 33 cm (50th centile). The baby was not dysmorphic and an initial examination showed her to be normal except for respiratory distress.

The mother was 26 years old and para3+1. Her blood group was A positive. Antenatal ultrasound showed polyhydramnios, oedema around the fetal chest and moderate bilateral pleural effusion.

The baby was admitted to a neonatal intensive care unit as a result of her respiratory distress and subcostal recessions. Nasal continuous positive airway pressure was applied, resulting in acceptable blood gas concentrations. Chest radiography revealed bilateral moderate pleural effusion (Figure 1). Bedside ultrasound showed significant pleural effusion on the right side and moderate pleural effusion on the left; no ascites or pericardial effusion was seen. Echocardiography was normal and her karyotype was normal female 46,XX.


Bilateral pleural effusion on admission.


Diagnostic thoracentesis extracted 40 ml of clear straw-coloured fluid from the right side and 10 ml of clear straw-coloured fluid from the left side so a right chest tube was inserted. Biochemical and cytological analysis (before feeding) showed 25 mg/dl triglycerides, 122 mg/dl glucose, 2.9 g/dl total protein, 42 mg/dl cholesterol, 4000 red blood cells/mm3 and 1500 white blood cells (WBC)/mm3 comprising 90% lymphocytes and 10% neutrophils. No micro-organisms were found in the cultures. The baby was kept nil by mouth on day 1 and oral feeding was gradually increased until day 5, when oral feeding was withdrawn because of progressive accumulation of pleural effusion and respiratory distress.

Repeated thoracentesis revealed a triglyceride level of 120 mg/dl, cholesterol of 58 mg/dl, total protein of 3.2 g/dl and predominant lymphocytes with negative cultures. The peripheral blood WBC was 14 200 cells/mm3 with 67% neutrophils and 8% lymphocytes (absolute lymphocyte count 1100 cells/mm3). As a result of these findings, the patient was diagnosed with chylothorax and received TPN with gradually increased enteral feeding of an enriched medium-chain triglyceride (MCT) formula [Monogen® (Nutricia, North America)].

A gradual increase in chylothorax and air leaks – with repeated tapping and repositioning of thoracotomy tubes on both sides (Figure 2) – occurred for 2 weeks. Replacement of pleural aspirate by fresh-frozen plasma and albumin was carried out to compensate for protein loss.


Bilateral chest tubes and left-sided surgical emphysema.


As a result of the baby’s poor response to nutritional intervention, at the age of 3 weeks intravenous infusion of octreotide was started at a dose of 2 µg/kg per hour but resulted in minimal improvement. The dose was gradually increased to 6 µg/kg per hour, which resulted in a prompt resolution of pleural effusion and improvement in respiratory functions (Figure 3).


Resolution of chylous effusion.


Chest tubes were removed after 1 week of octreotide infusion with very minimal bilateral effusion detected by chest computerized tomography. The total duration of octreotide infusion was 30 days. Octreotide was tolerated well with no recorded vomiting, hypotension, hypoglycaemia or liver dysfunction. The MCT-based formula was also tolerated well and given for 4 weeks before switching to breast milk and cow’s milk formula until discharge at the age of 3 months.


Neonatal chylothorax is a serious rare disorder of unknown aetiology in the majority of cases.5 Idiopathic chylothorax may be associated with a variety of clinical disorders such as pulmonary lymphangiectasis, lymphangiomatosis or Down, Turner or Noonan syndromes. It is associated with significant morbidities as well as a high mortality rate of up to 50%.6

Chylous effusion is diagnosed primarily on an analysis of pleural fluid with a total WBC count of ≥ 1000 cells/µl, at least 80% lymphocytes and ≥ 110 mg/dl triglycerides.7

The classic treatment strategy for chylothorax includes pleural fluid aspiration and, sometimes, chest tube insertion for diagnostic purposes and the relief of respiratory distress.8

Nutritional support involves a MCT-based formula that is absorbed directly to portal circulation and bypasses intestinal lymphatics.9 Some authors recommend TPN to provide complete gut rest to allow healing of the thoracic duct and to minimize lymph production.9

Surgical interventions include pleuro-peritoneal shunting, thoracic duct ligation or pleurodesis.6 These surgical methods may be helpful when other conservative treatments fail to control effusions.

Octreotide is a long-acting somatostatin analogue that is an effective conservative therapeutic modality that should be considered for congenital idiopathic chylothorax. Its mechanism of action remains uncertain but could be vasoconstriction of splanchnic vasculature, thus reducing intestinal secretions and lymphatic flow.10

There is no established dose of octreotide in neonatal chylothorax, but most physicians start with 0.5 µg/kg per hour and titrate up to 10 µg/kg per hour. In our case, a favourable response was gained at a dose of 6 µg/kg per hour with nearly complete resolution of chylous effusion detected by serial bedside ultrasound imaging.

Octreotide is a generally safe medication and was well tolerated without any complications throughout the treatment period of 30 days. Follow-up consultations with the patient in the clinic until the age of 8 months was satisfactory with no recurrence of pleural collection; the patient was symptom free.

Further trials are required to determine and investigate the proper dose, timing, duration and route of delivery for octreotide. In line with other reports, we find that octreotide remains a promising and effective alternative to surgery in neonatal idiopathic chylothorax when other conservative treatment modalities fail.6,810



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