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Perinatal/Neonatal Case Presentation

Severe Congenital Chylothorax Treated With Octreotide

Abstract

We report a neonate with severe congenital chylothorax. Subcutaneous octreotide was added to the standard treatment regime. The chylothorax resolved with no observed side effects.

Introduction

Chylothorax, defined as a collection of lymph in the pleural space, is a serious cause of respiratory distress in the neonate. Chylothorax may have primary or secondary etiologies. Secondary chylothorax is most commonly associated with trauma, thoracic surgery, and great vessel thrombosis.1 Primary or congenital chylothorax arises due to an abnormality of the lymphatic duct and may occur spontaneously or in association with conditions such as polyhydramnios, hydrops fetalis, intrathoracic mass lesions, superior caval vein obstruction, or Noonan's, Turner's and Down's syndromes.1 The incidence of congenital chylothorax is reported as 1 in 2000 NICU admissions.2

Regardless of the etiology, chylothorax produces detrimental respiratory, nutritional, and immunological consequences.3 Mortality rates range from 20% to 60% depending upon associated findings, gestational age, and the duration and severity of the chylothorax.3

We report on a neonate with severe congenital chylothorax. Subcutaneous octreotide was added to the standard treatment regime with no observed side effects. To our knowledge, use of octreotide for treatment of congenital chylothorax had not been previously reported.

CASE REPORT

A 3.7 kg female neonate was delivered to a First Nations Canadian, 16-year-old primigravida mother via cesarean section for failure to progress following induction of labor at 40 2/7 weeks gestation. There was no significant maternal medical history. However there was a history of neonatal deaths of unknown etiology within the paternal family.

A routine ultrasound at 15 weeks noted nuchal fold thickening and a sonolucent collection of fluid in the chest diagnosed as a cystic hygroma. An amniocentesis performed at this time revealed normal chromosomes. A fetal echocardiogram at 20 weeks was reported as normal. An ultrasound at 37 weeks demonstrated resolution of the fluid collection.

Resuscitation included intubation with assisted ventilation, placement of an umbilical venous line, and one dose of intratracheal epinephrine. Apgar scores were documented as 1, 4, and 5 at 1, 5, and 10 minutes. A chest-radiograph revealed a massive right pleural effusion. Diagnostic thoracentesis produced 5 ml of straw-colored fluid with 97% lymphocytes, confirming the diagnosis of chylothorax. Transport to a Level III Neonatal Intensive Care Unit at approximately 12 hours of age was indicated with increasing ventilatory requirements. A repeat chest radiograph demonstrated a right pleural effusion under tension and soft tissue swelling lateral to the right chest wall (see Figure 1). The tissue swelling was thought to be chylous leakage through the thoracentesis site.

Figure 1
figure1

Right pleural effusion under tension. Right lateral soft tissue swelling secondary to chylous leakage.

Physical exam revealed posteriorly rotated ears, down-slanted palpebral fissures, redundant neck skin, and a fluctuant mass on the right side of the chest. A cranial ultrasound was normal. An echocardiogram showed a small apical muscular ventricular septal defect. Urine organic acids were normal; plasma amino acids showed a slightly elevated methionine level thought to be suggestive of immature liver function.

Trisomy 21, Noonan's syndrome, Turner's syndrome, Frynes syndrome, familial congenital chylothorax, and an inborn error of metabolism were all ruled out based on the physical exam, chromosome analysis, and echocardiogram results. A genetic consult stated that given the in utero history of cystic hygroma or congenital chylothorax, there was evidence to suggest a prenatal lymphatic disorder which resulted in an isolated case of congenital chylothorax.

The patient was extubated on day 4. From days 4 to 7 multiple, frequent thoracenteses were required with increasing frequency, resulting in larger volumes being obtained with each successive tap. On day 7 a pneumothorax was diagnosed, necessitating placement of a chest tube, reintubation, and a morphine infusion. The chest drain was clamped on day 13 and discontinued on day 14. Chylous leakage was noted from the thoracentesis site through the dressing. Severe respiratory distress on day 16 required reinsertion of the chest drain to be left in situ for another 7 days.

Octreotide was administered via a subcutaneous port, initially at a dose of 40 mcg/kg/day to a maximum dose of 70 mcg/kg/day, from days 2 to 18. As recommended,11 we monitored closely for evidence of cholelithiasis, liver and renal impairment, and glucose tolerance. Plasma glucose was monitored daily. Liver function tests, creatinine, bilirubin, and complete blood counts were monitored weekly. An abdominal ultrasound was conducted every 2 weeks. No side effects were noted. Feedings of Portagen were commenced on day 3 and then held on day 7 because of increasing volumes of chylous fluid obtained via thoracentesis. Enteral feedings were reintroduced on day 19 with a 50% Portagen, 50% expressed breast milk regimen. The baby was fully bottle and breast fed with resolution of the chylothorax upon back transfer to the Level II nursery on day 21.

DISCUSSION

Management of congenital chylothorax has focused on the treatment of respiratory compromise, nutritional support, and closure of the chylous leak.4 Appropriate cardiopulmonary support may include endotracheal intubation and mechanical ventilation for those infants who present with severe respiratory failure. Both conventional and high-frequency ventilatory modes have been used successfully.5

In addition to diagnosis confirmation, the initial thoracentesis also evacuates chyle from the involved hemithorax. Small chylothoraces have been described as resorbing spontaneously.6 A thoracostomy tube is placed for continuous drainage if the chyle reaccumulates and requires frequent intermittent aspirations to keep the pleural space empty.4 Opinions vary regarding the duration of the period of conservative management. An extended period of conservative management may result in severe immunological and nutritional disturbances.7

Nutritional support directly influences the amount of chyle formation. The diet may be manipulated with the use of enteric rest with parenteral nutrition8 or medium-chain trigylceride feedings or elemental formulas.3 However, there may be no difference in chylothorax resolution whether breast or formula feedings are given.9 Protein replacement, adequate caloric intake, and electrolyte supplementation are all part of standard management. The chyle also contains prothrombin, fibrinogen, immunoglobulins, and lymphoctes, which place the neonate at risk for coagulopathy and immunodeficiency.3

Surgical intervention may be considered if, after conservative management, the daily loss of chyle exceeds 100 ml/day for a 5-day period, or chyle production fails to diminish after a 14-day period, or nutritional complications present.10 Surgical procedures for refractory chylothoraces include thoracotomy and thoracic duct ligation, apical pleurectomy, pleural abrasion, chemical pleurodesis or pleural-to-peritoneal shunts.7 Early intervention in refractory neonatal pleural effusions may minimize morbidity and shorten the length of hospital stay.7

When faced with a severe congenital chylothorax, our team reviewed the literature and identified that Octreotide might provide a novel and clinically efficacious addition to the standard conservative therapy. Octreotide, a somatostatin analog, is prescribed as an antisecretory agent. Somatostatin reduces the intestinal absorption of fats and decreases gut motility. It is described as an unlabeled treatment for a variety of conditions, including acromegaly, secretory diarrhea, esophageal varices, breast cancer, cryptosporidiosis, Cushing's syndrome, insulinomas, small bowel fistulas, postgastrectomy dumping syndrome, chemotherapy-induced diarrhea, and Zollinger–Ellison syndrome.11 Octreotide has also been used in the treatment of severe neonatal hypoglycemia.12

Octroetide has been used in the treatment of postoperative chylothorax in pediatric patients requiring cardiac surgery.13,14 Octreotide reduces thoracic duct flow and the triglyceride level of the chyle.14,15 There is evidence in the literature to support no long-term effects on thyroid hormone or growth hormone when used continuously for periods longer than 5 years.12

Although we could not find evidence of efficacy in the literature, the neonate in whom we used Octreotide for 17 days had resolution of her congenital chylothorax without complications.

SUMMARY

We describe our experience with the use of Octreotide in the management of a neonate with severe chylothorax. Although it is impossible to determine the rate at which the chylothorax would have resolved without the use of Octreotide, we found it to be a safe and effective adjunctive treatment. Further study is required to determine the effectiveness of this drug in a larger number of patients with neonatal congenital chylothorax.

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Correspondence to Sandra Young RNC, MN, NNP.

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Young, S., Dalgleish, S., Eccleston, A. et al. Severe Congenital Chylothorax Treated With Octreotide. J Perinatol 24, 200–202 (2004). https://doi.org/10.1038/sj.jp.7211053

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