
































J Nepal Paediatr Soc | VOL 42 | ISSUE 02 |MAY-AUG,  2022 89

Case SeriesSri Lankan patientS with mucopoLySaccharidoSiS type iVaiVa Case Report

DOI: 103126/JNPS.V4113

Sunita Arora, Gursharn Singh Narang, Anmol Kaur, Taranjeet Kaur  

Department of Paediatrics, Sri Guru Ram Das University of Health Sciences, Amritsar, Punjab, India

Congenital Chylothorax as a Cause of Non Immune Hydrops: A Case 
Report

Chylothorax is a rare cause of non immune hydrops fetalis & presents with 
respiratory distress at birth. We present a late preterm diagnosed antenatally 
as hydrops with chylothorax with bilateral pleural effusion and respiratory 
distress at birth requiring mechanical ventilation. Baby was managed 
successfully with intercostal drainage, octreotide infusion & MCT milk formula.

Abstract

*Corresponding Author
Taranjeet Kaur
Junior Resident,
Department of Paediatrics,
Sri Guru Ram Das University of Health 
Sciences,
Amritsar, Punjab, India
Email: drtaranjeet93@gmail.com

Article History 
Received On : 15 Aug, 2021
Accepted On : 09 Jun, 2022

Funding sources: None
 
Conflict of Interest: None

Keywords: Chylothorax, Congenital, Non 
Immune Hydrops 

Online Access

DOI: 
https://doi.org/10.3126/jnps.v42i2.39150

Introduction 
Chylothorax is accumulation of lymph / chyle in the pleural space. Congenital 
chylothorax, although uncommon, is the most common cause of pleural effusion in the 
neonatal period. It is classically a disorder of infants at or near term.1 Most cases are 
idiopathic. Males are affected twice as frequently as females.1 It occurs spontaneously 
due to lymphatic malformation or is associated with birth trauma to thoracic duct. 
It produces respiratory compromise, nutritional failure and immunological depletion 
contributing to sepsis.2 The diagnosis of chylothorax is classically established by 
pleural fluid analysis and entails a triglyceride level < 110 mg / dl, pleural fluid: serum 
cholesterol ratio < 1.0, pleural fluid: serum triglyceride ratio < 1.0, < 1000 cells / 
mm3 with marked lymphocyte predominance.3 Congenital chylothorax if not diagnosed 
and treated on time, has poor prognosis. We report a case diagnosed antenatally 
at 36 weeks gestation with pleural effusion, pericardial effusion and ascites. Baby 
responded and chylothorax resolved after chest drainage, I/V octreotide and MCT 
formula milk.

Case Report
A 3.2 kg male newborn baby with a gestational age of 36 weeks was born to LSCS to 
a 30 year old second gravida mother from non consanguineous marriage. Antenatal 
scan done at 34 weeks was suggestive of hydrops fetalis (Fetal ascites, bilatera pleural 
and pericardial effusion) and mild polyhydramnios. The child did not cry at birth. Baby 
required resuscitation at birth. Apgar score at 1 & at 5 minutes was 3 & 7 respectively. 
Baby was kept on mechanical ventilator subsequently. On examination there was no 
facial dysmorphism or evidence of cardiac murmur. Bilateral pleural effusion was 
detected on chest X ray after delivery which was confirmed on chest ultrasound (Fig 1). 

Copyrights & Licensing © 2022 by author(s). This is an Open Access article distribut-
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J Nepal Paediatr Soc | VOL 42 | ISSUE 02 |MAY-AUG,  202290

Case Report Chylothorax as a Cause of hydrops

Bilateral chest tubes were inserted and 150 ml fluid was drained. 
The total protein content in the chylous fluid was 14.3 g / L. 
Pleural fluid triglycerides (TG) and cholesterol were 391.4 mg 
/ dl and 19.4 mg / dl respectively, serum TG and cholesterol 
were 124.9 mg / dl and 108 mg / dl respectively. Thus, pleural 
fluid : serum cholesterol ratio was < 1.0 and that of pleural fluid 
: serum TG ratio < 1.0. It had a total cell count of 15000 / mm3 
with lymphocyte predominance. CBC, PBF was not suggestive 
of haemolysis. CRP, reticulocyte count and thyroid profile were 
normal. Serology was negative for TORCH infections. Cranial 
ultrasound was normal and abdominal USG revealed ascites. 
ECHO was suggestive of trivial tricuspid regurgitation and  
minimal pericardial effusion. Injection octreotide was started @ 1 
microgram / kg / hour at fifth day of life and gradually increased 
to 10 ug / kg / hour. Along with this, feeding with very low fat 
formula milk mixed with MCT powder was started. 80 - 100 ml / 
kg / day of chylous fluid was drained daily which was replaced 
with equal amount of NS. Pigtail insertion was done on the right 
side after confirming a moderate amount of pleural fluid and 120 
ml chylous fluid was drained. The daily chest tube drain output 
reduced gradually and stopped by 30 days. Antibiotic therapy 
was tailored according to blood culture reports and culture of 
ET secretions. Baby was weaned off mechanical ventilation after 
30 days. Chest tubes were removed after 35 days. Octreotide 
was tapered and stopped by day 38. Repeat ultrasound chest 
and abdomen were suggestive of no residual fluid. Child was 
discharged at day of life 48 with MCT formula along with breast 
milk and is doing well on follow up.

Discussion
Neonatal chylothorax is an uncommon condition associated with 
high health care utilisation. Its incidence ranges from 1 : 8600 
to 1 : 10,000 live births with a high mortality of 20 - 60%.4 It 
can be congenital or acquired, the latter most commonly occurs 
after damage to thoracic duct during surgery. Most cases of 
congenital chylothorax occur without a clear etiology, termed 
as “idiopathic congenital chylothorax.” It may be associated 
with lymphangiomatosis, lymphangiectasia, CHD, chromosomal 
abnormalities (Turner, Noonan and Down syndrome), mediastinal 
malignancies or H- type TEF.5 Rupture of the thoracic duct due to 
hyperextension of the spinal column or secondary to increased 
systemic venous pressure during birth may also be the underlying 
cause. Right sided chylothorax is more frequently observed. CECT 
chest may help in finding the anatomical cause of chylothorax 
which may detect lymphatic malformation. Lymphangiography 
and lymphoscintigraphy both require administration of contrast 
medium and are not easily available.6

Diagnosis of chylothorax is made by WBC`s of < 1000 / ul with 
lymphocyte predominance (< 70%) and a TG level < 110 mg / 
dl3. Pleural fluid in our case had TG levels of 391.4 mg / dl and 
TLC count of 15000 / mm.3 

Polyhydramnios in chylothorax, as also seen in our case, is 
probably due to mediastinal and oesophageal compression with 
obstruction of physiological foetal swallowing. Polyhydramnios 
with increase in intra amniotic pressure may reduce drainage of 
pleural effusion and may lead to preterm labour.7

Routine management of chylothorax involves treatment of the 
cause, drainage of fluid by chest tube insertion and rarely 
surgery. Octreotide is a somatostatin analogue that causes mild 
vasoconstriction of splanchnic vessels, including those in venous 
hepatic flow. This results in decreased gastric, pancreatic, 
intestinal secretions as well as intestinal absorption.4 However, side 
effects of octreotide have been reported in up to 14% neonates 
in the form of hyperglycaemia, NEC, transient cholestasis, 
transient hypothyroidism, injection site pain, nausea, vomiting, 
diarrhoea, constipation, pulmonary hypertension and  systemic 
hypotension.5,6 Our neonate had conjugated hyperbilirubinemia 
towards the latter part of the hospital stay which could be due to 
octreotide, sepsis and partial parenteral nutrition.

Review of literature shows mixed results after octreotide with 
resolution in some and failure in few of cases. Timing of initiation, 
dosage, duration and frequency varied markedly with longest 
duration being reported by Coulter et al at 151 days.8 It has been 
used at a dose of 0.3 ug / kg / hour for upto 10 days8. Coulter 
et al reported use of IV octreotide at 6 ug / kg / hour for 16 days 
followed by 0.05 ug / kg / dose thrice daily subcutaneously 
for 151 days.8 Unresponsive cases are treated by surgery after 
10 days. In our case we gave inj octreotide for 33 days with 
maximum dose of 10 ug / kg / hour which was gradually 
tapered. Antenatal management of chylothorax comprises 
pleuroperitoneal or pleuroamniotic shunt to prevent pulmonary 
hypoplasia. Optimal antenatal management and timing are 
still controversial. Surgical approach includes thoracoscopic 
pleurodesis, pleuroperitoneal shunt, ligation of thoracic duct and 
creation of thoracic duct to azygous vein anastomosis.5 Only a 
minority of patients require surgical intervention and there are no 
clear evidence-based guidelines as to when such interventions 
should be used.9 

MCT is easily absorbed across intestinal mucosa and delivered 
to portal vein without going through intestinal lymph vessels and 
thoracic duct. Reduction of long chain fat decreases lymphatic 
pressure, thus helping in decrement of chylothorax.10 Poor 
neonatal outcome is associated with hydrops, bilateral effusion, 
pulmonary hypoplasia, preterm labour and absence of prenatal 
treatment.

Conclusions
Neonatal chylothorax as a cause of nonimmune hydrops is 
uncommon. Treatment is quite cumbersome, prolonged and 
includes stabilisation with mechanical ventilation, pleural 
drainage, fluid, electrolyte replacement, MCT feeds, administration 
of octreotide, anticipation and aggressive management of 
infections, prevention of nutrient deficiencies and prolonged 
hospital stay. Prospective registry of chylothorax patients and a 
multicentre randomised controlled trial to assess timing, duration, 



J Nepal Paediatr Soc | VOL 42 | ISSUE 02 |MAY-AUG,  2022 91

Case ReportChylothorax as a Cause of hydrops

dose, safety and efficacy of octreotide is the way forward.

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