Clinical characteristics of nontraumatic chylothorax in pediatric patients

So Hyun Shin; Jun Hyuk Song; Min Jung Kim; Saebeom Hur; Woo Sun Kim; Dong In Suh

doi:10.4168/aard.2019.7.4.206

Journal List > Allergy Asthma Respir Dis > v.7(4) > 1138012

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Shin, Song, Kim, Hur, Kim, and Suh: Clinical characteristics of nontraumatic chylothorax in pediatric patients

Original Article

Allergy Asthma Respir Dis 2019;7(4):206-211.

Published online: 12 October 2019

DOI: https://doi.org/10.4168/aard.2019.7.4.206

Clinical characteristics of nontraumatic chylothorax in pediatric patients

So Hyun Shin¹, Jun Hyuk Song², Min Jung Kim¹, Saebeom Hur³, Woo Sun Kim³, Dong In Suh¹

^¹Department of Pediatrics, Seoul National University Hospital, Seoul Korea

^²Department of Pediatrics, Myongji Hospital, Hanyang University College of Medicine, Goyang

^³Department of Radiology, Seoul National University Hospital, Seoul Korea

Correspondence to: Jun Hyuk Song https://orcid.org/0000-0002-8283-4652 Department of Pediatrics, Myongji Hospital, Hanyang University College of Medicine, 55 Hwasu-ro 14beon-gil, Deogyang-gu, Goyang 10475, Korea Tel: +82-31-810-5170, Fax: +82-31-969-0500, E-mail: stealawayou@hanmail.net

Received 12 February 2019 Revised 26 April 2019 Accepted 31 May 2019

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To evaluate clinical characteristics of pediatric nontraumaitc chylothorax and to suggest appropriate therapeutic manage-ments.

Methods

We retrospectively reviewed medical records of 22 patients with nontraumatic chylothorax from January 2005 to December 2018 in the Children's Hospital of Seoul National University. We analyze their etiology, treatment, complications and outcome.

Results

Of the 22 patients, 16 were diagnosed before 1 year old and 6 after 1 year old. The causes of chylothorax under 1-year-old children were related to congenital factors (n=9), unknown causes (n=5), and high central venous pressure (n=2). The causes of chylothorax over 1-year-old children were related to congenital factors (n=3), unknown causes (n=1), high venous pressure (n=1), and lymphoma (n=1). All patients had dietary modification. Eight of them were cured by dietary modification, but there was no improvement in over 1-year-old children. Medication was added to patients refractory to dietary modification. Four patients with medication were improved and 5 were improved by surgical management. Nutritional, immunological and other complications occurred in many patients. Five death cases were reported. Four patients were under 1 year old and 1 was over 1 year old. The causes of nontraumatic chylothorax in dead patients were high central venous pressure (n=3), congenital (n=1), and unknown (n=1).

Conclusion

Nontraumatic chylothorax more frequently occurs in under 1-year-old children. The most common cause is congenital factors. Stepwise management is effective in many patients, but specific treatment is needed in some cases. The prognosis is related to the onset of age and underlying diseases.

Keywords: Nontraumatic chylothorax, Pediatrics, Diet, Management

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Fig. 1.

Flow chart of the patient selection.

Fig. 2.

(A) Lymphangiography that showed lymphatic leakage to thorax cavity. (B) Magnetic resonance lymphangiography before (left) and after (right) administration of sirolimus.

Table 1.

The characteristics of the study subjects (n=22)

Characteristic	Value
Sex
Male:female	16 (72.7):6 (27.3)
Age (mo)	28±55.5 (1 day–192 months)
<1 yr:≥1 yr	16 (72.7):6 (27.3)
Preterm	9 (40)
Patients with underlying diseases	14 (63.6)
Symptom of dyspnea	19 (86.4)
Computed tomography	13 (59)
Lymphangiography	7 (31.8)
Magnetic resonance lymphangiography	2 (9)
Patients required respiratory support	19 (86.4)
Low flow oxygen supplementation	8 (36.4)
Mechanical ventilation	11 (50)

Values are presented as number (%) or mean±standard deviation (range).

Table 2.

The causes of nontraumatic chylothorax

Cause	<1 Year	≥1 Year
Congenital	9 (56)	3 (50)
	Fetal hydrops 7 (44)	Gorham stout sidease 2 (33)
	Down syndrome 2 (13)	Noonan syndrome 1 (17)
Unknown	5 (31)	1 (17)
High central venous pressure	2 (13)	1 (17)
Lymphoma	–	1 (17)
Total	16	6

Values are presented as number (%).

Table 3.

Outcomes of different treatments according to cause of chylothorax

Causes of chylothorax	Age	Treatment method
Causes of chylothorax	Age	Diet	Diet+med	Diet+med+op	Diet+op
Congenital	<1 yr (n=9)	5/6	–	3/3	–
	≥1 yr (n=3)	–	3*/3	–	–
Venous thrombosis	<1 yr (n=2)	0/1	0/1	–	–
	≥1 yr (n=1)	0/1	–	–	–
Lymphoma	<1 yr (n=0)	–	–	–	–
	≥1 yr (n=1)	–	–	–	1/1
Unknown	<1 yr (n=5)	3/3	1/1	0/1	–
	≥1 yr (n=1)	–	–	–	1/1

Values are presented as number of chylothorax improvement/number of treated patients.

Diet, dietary modifications; diet+med, dietary modifications plus somatostatin and analogues (octreotide) or sirolimus; diet+med+op, dietary modifications plus somatostatin and analogues (octreotide) or sirolimus plus surgical treatment; diet+op, dietary modifications plus surgical treatment.

*Amount of chylothorax was decreased partially but was no longer produced.

Table 4.

Summary of complications (n=22)

Age (yr)	Growth retardation	Immunodeficiency	Infection	Electrolyte imbalance	Thrombosis	ARDS
<1	5	1	7	1	1	2
≥1	3	0	3	0	0	0

ARDS, acute respiratory distress syndrome.

Table 5.

Summary of the dead patients (n=5)

Case	Sex/age at diagnos	is Underlying disease	Cause of chylothorax	Treatment method	Treatment period	d Cause of death
1	M/3 mo	DMD, ASD, PS	Unknown	Diet+med+op	2 mo	ARDS
2	M/10 day	SCID, gastroschisis	Venous thrombosis	Diet	4 mo	Septic shock
3	F/1 mo	B cell deficiency	Occlusion of right subclavian ve and superior vena cava	in Diet+med	3 mo	Sepsis
4	M/2.5 yr	Midgut volvulus, CKD, coagulapthy	Venous thrombosis	Diet	3 mo	Sepsis, renal failure
5	M/0 day	HCMP, PS, AS	Fetal hydrops	Diet	23 day	Sepsis, heart failure

DMD, duchenne muscular dystrophy; ASD, atrial septal defect; PS, pulmonary stenosis; ARDS, acute respiratory distress syndrome; diet, dietary modifications; diet+med, dietary modifications plus somatostatin and analogues (octreotide) or sirolimus; diet+med+op, dietary modifications plus somatostatin and analogues (octreotide) or sirolimus plus surgical treatment; diet+op, dietary modifications plus surgical treatment; SCID, severe combined immunodeficiency; CKD, chronic kidney disease; HCMP, hypertrophic car-diomyopathy; AS, aortic stenosis.

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