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Year : 2017  |  Volume : 6  |  Issue : 3  |  Page : 205-207

Congenital idiopathic chylothorax: A very rare case

Department of Pediatric Surgery, King George's Medical University, Lucknow, Uttar Pradesh, India

Date of Web Publication11-Jul-2017

Correspondence Address:
Sudhir Singh
Department of Pediatric Surgery, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_42_17

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Chylothorax is a very rare cause of respiratory distress in the neonates, defined as a collection of chyle in the pleural space. Causes of chylothorax in neonates are congenital lymphatic malformations, associated syndrome or birth trauma. Here, we are presenting a neonate presented with severe respiratory distress require intubation and ventilator support and on evaluation no cause of chylothorax found. Baby managed with intercostal drainage and octreotide, improved and asymptomatic in 8 months of follow-up.

Keywords: Chylothorax, neonate, octreotide

How to cite this article:
Rawat J D, Singh S, Singh G, Chaubey D. Congenital idiopathic chylothorax: A very rare case. J Clin Neonatol 2017;6:205-7

How to cite this URL:
Rawat J D, Singh S, Singh G, Chaubey D. Congenital idiopathic chylothorax: A very rare case. J Clin Neonatol [serial online] 2017 [cited 2022 Dec 2];6:205-7. Available from: https://www.jcnonweb.com/text.asp?2017/6/3/205/210143

  Introduction Top

Chylothorax is a rare disease, with 1%–2% of pleural effusions and a prevalence of between 1/8600 and 1/15,000 live births.[1] Congenital chylothorax generally presents within the 1st week of life with respiratory distress and the diagnosis typically confirmed by chest radiography and pleural aspiration of a milky fluid. Congenital chylothorax is associated with abnormalities of the lymphatic system such as lymphangiomatosis and lymphangiectasia, congenital heart disease, mediastinal malignancies, and chromosomal abnormalities (trisomy 21, Turners and Noonan syndromes).[2] Many cases of chylothorax had no clear etiology and considered as idiopathic congenital chylothorax like the case presented here.

  Case Report Top

A 4-day-old neonate presented to our emergency department with refusal to feed and severe respiratory distress. The baby was full term, first born girl child delivered normal vaginally with a birth weight of 3.1 kg. There were no abnormality recorded in mother, on regular antenatal check-up and on antenatal sonography. There was no history of birth trauma. The baby was asymptomatic until day thirds of life with normal feed. The baby developed refusal to feed from the 4th day, thereafter she developed progressive respiratory distress. On examination, the baby was in severe respiratory distress with decrease air entry on the right side of the chest, no others any associated congenital anomalies were found. Baby intubated and kept on ventilator due to severe respiratory distress. Chest X-ray revealed right pleural effusion with mediastinal shift toward left, on Contrast Enhanced Computed Tomography (CECT) thorax right pleural collection found with no contrast leak in the plural cavity, there were no signs of pleural inflammation, no any lymphatic malformation was found [Figure 1]a and [Figure 1]b. Pleural tap done which revealed milky white aspirate. On biochemical analysis of pleural fluid suggest chyle with triglyceride level was 120 mg/dl, pleural fluid to serum cholesterol ratio was <1.0 and chylomicron found. Cytological examination revealed absolute cell count 1100 cells/L, lymphocytes 800/mm 3, and erythrocytes 150/mm 3. Patient managed with intercostal drainage with the output of 300 ml initially, followed by 200 ml daily for 5 days [Figure 1]c. In view of persistent drainage in the tube in spite of diet, contacting low fat and containing medium chain triglycerides patient managed with long-acting somatostatin analog (octreotide) in dose of two micro gram/kg/subcutaneously six hourly. Response to octreotide noted on the first day of the start of therapy with gradual decreasing in drain output. On the 7th day of octreotide therapy, drainage stops and intercostal tube removed in view of satisfactory chest condition in radiograph [Figure 1]d. During octreotide therapy, monitoring done for liver and renal function, hyperglycemia, and electrolyte imbalance. In our patient, no significant alteration occurred in above-mentioned parameters and baby discharged in satisfactory condition on 20th days of hospitalization.
Figure 1: (a) Chest radiograph showing right side opacity with mediastinal shift toward left suggestive of right pleural collection. (b) CECT of chest showing right side pleural collection, mediastinal shift with no signs of pleural inflammation. (c) Intercostal drainage bag showing chyle drainage. (d) Chest radiograph after drainage of right chylothorax showing expanded lung

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  Discussion Top

The chyle transported to the cisterna chyli, which overlies the anterior surface of the second lumbar vertebra. The duct passes through the esophageal hiatus of the diaphragm into the thoracic cavity, ascends extra-pleural in the posterior mediastinum to the right of the vertebral column. At the fourth to sixth thoracic vertebrae, the duct crosses to the left of the vertebral column and continues cephalic to enter the superior mediastinum between the aortic arch, the subclavian artery, and the left side of the esophagus. The duct terminate near the subclavian veins into the left jugular vein.[3]

The incidence of congenital chylothorax reported as one in 2000 neonatal Intensive Care Unit (NICU) admissions.[4] In idiopathic congenital chylothorax, neonates have a weak thoracic duct or lymphatic anomalies. Therefore, any increase in venous pressure may cause a break of the congenitally weakened thoracic duct. The mechanism thought to be either traumatic, with rupture of the thoracic duct by hyperextension of the spinal column or secondary to increased systemic venous pressure during birth, especially in complicated deliveries. Right-side chylothorax more frequently observed in comparison to the left side (55% vs. 35%) as found in our cases.[5] Etiology is unknown in the majority of neonatal chylothorax cases as in our case in spites of relevant investigations. CECT may help in finding the anatomical cause of chylothorax, as lymphatic malformations can be detected. In cases of traumatic chylothorax, significant contrast leak may found in the pleural cavity. Lymphangiography and lymphoscintigraphy both require the administration of a contrast agent may help in find out the site of lymphatic leaks, in our cases, the baby was on ventilator and facility of these investigations not available in NICU setup. The idiopathic congenital chylothorax is usually a transient condition that resolves by conservative treatment with the dietary elimination of the long-chain fatty acids or replacement of oral feeding with total parenteral nutrition. Mortality rates range from 20% to 60% depending on associated findings, gestational age, and the duration and severity of the chylothorax.[6] In the case of continuation of drainage despite total parenteral nutrition, advocated to perform surgery like ligation of the thoracic ductus, pleuroperitoneal shunt, pleurectomy, or pleurosis not required in our case.[7]

Octreotide, a somatostatin analog, prescribed as an anti-secretory agent. Somatostatin reduces the intestinal absorption of fats and decreases gut motility. Its mechanism of action in treating chylothorax is not clear, but a possibility is reduction of splanchnic vascular tone, eventually leading to a decreased flow of chyle through the thoracic duct. During therapy with octreotide, patient should be monitor for hyperglycemia, hypothyroidism, diarrhea, renal impairment, necrotizing enterocolitis, and liver dysfunction.[8] In our case, we started the Octreotide in a dose of two microgram subcutaneously every six hourly and response noted on day 1 of therapy. In pediatric and neonatal cases, we can follow the algorithm given by Tutor.[9] First, we should diagnose and determine the etiology of chylothorax, put an intercostal drainage tube for symptomatic improvement. In case drainage >100 ml/year/day, proceeds to surgical intervention and if drainage is less <100 ml/year/day, medical treatment can be tried. In cases were chylothorax persist for more than 2–4 weeks in spite of medical treatments, shift to surgical interventions.

  Conclusion Top

Congenital chylothorax is a very rare cause of respiratory distress in neonates. Diagnosis of this condition based on clinical, radiological, and pleural fluid biochemical findings. Conservative therapy should be tried first before a surgical procedure might be considered. Octreotide is emerging agent in conservative therapy need a randomized, controlled, multicentre trial to assess the safety and efficacy in the treatment of chylothorax.

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There are no conflicts of interest.

  References Top

Epaud R, Dubern B, Larroquet M, Tamalet A, Guillemot N, Maurage C, et al. Therapeutic strategies for idiopathic chylothorax. J Pediatr Surg 2008;43:461-5.  Back to cited text no. 1
Soto-Martinez M, Massie J. Chylothorax: Diagnosis and management in children. Paediatr Respir Rev 2009;10:199-207.  Back to cited text no. 2
Sassoon CS, Light RW. Chylothorax and pseudochylothorax. Clin Chest Med 1985;6:163-71.  Back to cited text no. 3
Van Aerde J, Campbell AN, Smyth JA, Lloyd D, Bryan MH. Spontaneous chylothorax in newborns. Am J Dis Child 1984;138:961-4.  Back to cited text no. 4
Sivasli E, Dogru D, Aslan AT, Yurdakok M, Tekinalp G. Spontaneous neonatal chylothorax treated with octreotide in Turkey: A case report. J Perinatol 2004;24:261-2.  Back to cited text no. 5
Dubin PJ, King IN, Gallagher PG. Congenital chylothorax. Curr Opin Pediatr 2000;12:505-9.  Back to cited text no. 6
van Straaten HL, Gerards LJ, Krediet TG. Chylothorax in the neonatal period. Eur J Pediatr 1993;152:2-5.  Back to cited text no. 7
Mitanchez D, Walter-Nicolet E, Salomon R, Bavoux F, Hubert P. Congenital chylothorax: What is the best strategy? Arch Dis Child Fetal Neonatal Ed 2006;91:F153-4.  Back to cited text no. 8
Tutor JD. Chylothorax in infants and children. Pediatrics 2014;133:722-33.  Back to cited text no. 9


  [Figure 1]

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1 Chylothorax: A Stepwise Approach to Diagnosis and Treatment
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