Presentation
Chylothorax presenting as non-immune hydrops is even rarer. We report a case of congenital bilateral chylothorax presenting as non immune hydrops and managed successfully with chemical pleurodesis. [ncbi.nlm.nih.gov]
Entire Body System
- Fever
Given the noninflammatory nature of chyle, pleuritic chest pain or fever should not be expected, with lymphoma being an exception (fever is a “B” symptom). [pulmonologyadvisor.com]
Since the accumulation is non-inflammatory, fever and pleuritic chest pain are not present [14]. Investigations Investigations essentially focus on confirmation of chylothorax by fluid analysis and diagnosis of the cause. [cureus.com]
The child had rare sneezing and coughing but his mother had not noted fevers. The child was not on any medications and had no prior allergies or surgery. The child resided with his mother, father, and grandparents who were all smokers. [thoracic.org]
Fever or chest pain are not usually associated with chylothorax, as chyle does not generate inflammation by itself.[4] On examination, chylothorax may lead to reduced breath sounds on the affected side, associated with a dull sound when the chest is tapped [en.wikipedia.org]
- Collapse
In view of pleural effusion on right Side, computed tomography scan thorax was done that revealed marked right side pleural effusion with collapse of lung and contralateral tracheal and mediastinal shift. [jcnonweb.com]
In chilotoraxul progressive installation can cause physiological disorders by: pulmonary compression, pulmonary collapse, leading to decreased ventilation and perfusion and subsequently with hypoxemia by rights-are left, contralateral mediastinal shift [medicaldb.blogspot.com]
She was immediately placed on a high frequency ventilator to help her breath and maintain positive pressure in her lungs to try and prevent collapsing. Reese developed chylothorax and has had up to three different chest tubes in her little body. [littleleakers.com]
Fluid was pouring into her chest cavity and filled up to a point that it collapsed her lungs. At the rate the fluid was invading her cavity it would quickly start affecting other organs besides her lungs. [raiseyourgarden.com]
Figure 1: Eight hours of life Chest x-ray showing right sided pleural effusionwith underlying collapse and deviation of the mediastinum to the left. [latunisiemedicale.com]
- Transient Hypothyroidism
AIM: To describe a group of neonates with congenital, non-traumatic chylothorax, one of whom developed transient hypothyroidism following treatment with somatostatin. [ncbi.nlm.nih.gov]
The hypothesis is that infants with congenital hypothyroidism may lose thyroxin and other hormones via the chylothorax fluid causing transient hypothyroidism. [clinicaltrials.gov]
Multiple side effects have been reported in up to 14% neonates treated with octreotide including hyperglycaemia, necrotising enterocolitis, transient mild cholestasis, transient hypothyroidism, pulmonary hypertension, and severe hypotension [3]. [hindawi.com]
Gastrointestinal intolerance and clinical presentations suggestive of necrotizing enterocolitis and transient hypothyroidism were reported as side effects. [cochrane.org]
hypothyroidism in newborns. 54 61 Maayan-Metzger A...Kuint J 16188789 2005 9 Octreotide in the treatment of congenital chylothorax. 54 61 Rasiah SV...Lui K 15367160 2004 10 The Practicality of Feeding Defatted Human Milk in the Treatment of Congenital [malacards.org]
- Inflammation
(b) CECT of chest showing right side pleural collection, mediastinal shift with no signs of pleural inflammation. (c) Intercostal drainage bag showing chyle drainage. [jcnonweb.com]
Neutrophils predominate in pleural fluid from patients with pleural inflammation (Table 2921). More than 10% of transudates will also have a predominance of neutrophils, but this has limited clinical significance. [passeidireto.com]
Liquid flow is stopped by irritating the lungs and chest wall with pleural inflammation and closed space. Medication with octreotide proved beneficial and in some cases chilotoraxul stopped after a few weeks. [medicaldb.blogspot.com]
Fever or chest pain are not usually associated with chylothorax, as chyle does not generate inflammation by itself.[4] On examination, chylothorax may lead to reduced breath sounds on the affected side, associated with a dull sound when the chest is tapped [en.wikipedia.org]
[…] other pleural effusions with respiratory distress and dyspnea as chief complaints. 15 Assessing trauma can be particularly challenging, as cases may be asymptomatic for up to 10 days post injury. 2 Iatrogenic delay is attributed to limited food intake, inflammation [sages.org]
- Anemia
New chapters, expanded and updated coverage, increased worldwide perspectives, and many new contributors keep you current on the late preterm infant, the fetal origins of adult disease, neonatal anemia, genetic disorders, and more. "...a valuable reference [books.google.com]
Povidone-iodine pleurodesis for congenital chylothorax of the newborn. 61 Resch B...Reiterer F 26419497 2016 42 [Role of lymphatic scintigraphy and surgery in congenital chylothorax]. 61 Methlouthi J...Hassen S 28154676 2016 43 Life-Threatening Hemolytic Anemia [malacards.org]
[…] after ruling out congenital infections (with maternal serology to toxoplasmosis, rubella, cytomegalovirus, syphilis, herpes and parvovirus B19), immune hydrops (blood type and antibody screen), fetal-maternal hemorrhage (Kleihauer–Betke test), fetal anemia [omicsonline.org]
[…] summarized below4 Cardiac: congenital heart disease leading to congestive heart failure 19-26% Pulmonary: chylothorax, lymphangiectasia, cystic adenomatoid malformation or sequestration 8-10% Genetic: trisomy 21, 18, 13, 15, 16, Turner syndrome 35% Fetal anemia [thoracic.org]
[…] without hydrops fetalis, or patients of acquired chylothorax (post-operative or traumatic chylothorax), of non-idiopathic chylothorax (with an identifiable cause, such as chromosomal anomalies, structural heart defects, immune mediated diseases, severe anemia [frontiersin.org]
Respiratoric
- Respiratory Distress
Congenital chylothorax, a rare cause of respiratory distress in neonate, presented here with respiratory distress from two days of age. Thoracocentesis was performed at six days, revealing a clear fluid with high protein content. [ncbi.nlm.nih.gov]
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. [jcnonweb.com]
We report a case of a full-term baby diagnosed with respiratory distress after 1 week of life. An X-ray of his chest showed a left pleural effusion. [hindawi.com]
Conclusion Chylothorax is a rare cause of respiratory distress in newborns. Its diagnosis is based on clinical, radiological, and pleural fluid biochemical findings. [omicsonline.org]
- Pneumonia
Consult new chapters covering Ultrasound, Innate Immunity, Adaptive Immunity, Deposition and Clearance, Ventilator-Associated Pneumonia. [books.google.com]
Intercurrent upper and lower respiratory tract infections and two cases of pneumonia did not appear to have influenced results of pulmonary function testing. [erj.ersjournals.com]
She received cefazolin for prophylaxis while the chest tube was in place and a one‐time dose of pentamidine as prophylaxis against pneumocystis pneumonia. The patient was discharged on DOL 53 after successfully transitioning to breast milk. [onlinelibrary.wiley.com]
Gastrointestinal
- Vomiting
A complication, specifically vomiting was noted in 1 case during octreotide therapy. Conclusion : In this study, octreotide therapy resulted in a safe and excellent outcome. [kjp.or.kr]
Although I was trying not to get too excited about it, other than the vomiting, Nate was doing so well, that I actually thought it may happen this time. [chylothorax.wordpress.com]
In closed tear duct injuries can occur through different pathogenic mechanisms: hyperextension of the spine especially when the duct is fixed by the pathological process and is full-postprandial-rupture occurs above the diaphragm; effort vomiting, coughing [medicaldb.blogspot.com]
On September 13th, Kristina went into her doctor's office with complaints of abnormal and horrible pain, spotting, vomiting, and contractions. After examining her, the doctor determined she was dilated to 5cm and 90% effaced - she was 36 weeks. [youcaring.com]
Table 1 Reported side effects of octreotide Transient loose stools(15) Nausea/Vomiting (8) Hypoglycemia, Hyperglycemia (8) Recurrent chylothorax (8) Necrotising enterocolitis (10) Pulmonary hypertension (11) Bloody diarrohea (19) Abdominal distension [jpss.eu]
- Nausea
The reported adverse reactions include cramps, flatulence, nausea, diarrhea, necrotizing enterocolitis, hyperglycemia, transient hypothyroidism, and liver dysfunction. [jcnonweb.com]
Possible side effects: hyperglycaemia, hyperthyroidism, cramps, nausea, diarrhoea, renal impairment. Therefore, patients receiving these medications should have careful monitoring of their blood sugars, urine output and irritability. [rch.org.au]
Side effects of octreotide relate to reduced sperm motility and intestinal secretion: hypertension, malabsorption, nausea, flatulence, hepatic dysfunction and hyperglycemia 9. [scielo.br]
Table 1 Reported side effects of octreotide Transient loose stools(15) Nausea/Vomiting (8) Hypoglycemia, Hyperglycemia (8) Recurrent chylothorax (8) Necrotising enterocolitis (10) Pulmonary hypertension (11) Bloody diarrohea (19) Abdominal distension [jpss.eu]
[…] for octreotide in children has ranged from 0.3 to 1.0 μg/kg per hour. 117, 118 The optimal timing of introduction and duration of treatment is unknown. 2 Side effects of somatostatin and octreotide include hyperglycemia, hypothyroidism, 119 cramps, nausea [pediatrics.aappublications.org]
- Constipation
[…] groups only if a risk-benefit analysis is positive.[6][7] Adverse effects[edit] The most common adverse effects are headache, hypothyroidism, cardiac conduction changes, gastrointestinal reactions (including cramps, nausea/vomiting and diarrhoea or constipation [en.wikipedia.org]
Skin
- Pterygium Colli
The mechanisms involved behind chylothorax in Noonan syndrome may be multifactorial such as congenital heart disease, coagulation-factor deficiency, pterygium colli, and lymphangiomatosis of the pleura, lungs, and chest wall. [academic.oup.com]
Urogenital
- Cesarean Section
She was born at a gestational age of 34 weeks by Cesarean section because of fetal hydrothorax and hydrops fetus, confirmed by ultrasonography at 32 weeks. Emergent resuscitation and immediate thoracentesis were performed soon after birth. [ncbi.nlm.nih.gov]
A cesarean section was planned for the day of delivery following additional pleurocentesis; however, this was converted to emergent cesarean following fetal cardiac decelerations. [onlinelibrary.wiley.com]
- Renal Insufficiency
Polyvidone iodine seems to a good alternative to other chemical pleurodesis after ruling out renal insufficiency. 4. [scirp.org]
Workup
Diagnostic workup in a neonate presenting with chylothorax would be maternal, fetal, and neonatal. The aim is to exclude other causes of hydrothoraces. [ispub.com]
Conversely, increasing levels indicate a worsening condition requiring aggressive workup or treatment. LD iso enzyme analysis may be helpful in diagnosing problematic exudates but is not routinely recommended (Lossos, 1997). [passeidireto.com]
X-Ray
- Left Pleural Effusion
An X-ray of his chest showed a left pleural effusion. Moreover, a thoracentesis combined with a biochemical study of the pleural fluid confirmed the diagnosis of chylothorax. In our case, the conservative therapy was successful. [hindawi.com]
Because of this anatomic arrangement leakage from the lower portion of the duct produces a left pleural effusion. · 1:10,000 births. · Overall mortality 25% (1-3). Unilateral effusion (90%) > bilateral fluid collections. [fetalultrasound.com]
Left pleural effusion resolved on day 14 and right on day 21. Patient went home on strictly medium chain triglyceride feeds on day 30. He was readmitted at 2 months and introduced to regular infant formula. [ispub.com]
On day 22, chemical pleurodesis with povidone-iodine (Betadine 4% Scrub) through the left chest tube was carried out. The left sided pleural effusion resolved over 48 hours, but a parallel increase in the right sided pleural effusion was observed. [fn.bmj.com]
Serum
- Hypogammaglobulinemia
However, long-term insertion of chest tube has been reported to be associated with hypoproteinemia, lymphopenia, hypogammaglobulinemia, infection, and prolonged ventilator use and its associated lung injury, leading to prolonged hospitalization and emotional [jcnonweb.com]
He also developed hypogammaglobulinemia with IgG levels measured at a nadir of 34.7% of the laboratory's lower threshold of normal. He was treated with IVIG infusions on 4 occasions from DOL 12‐30. [onlinelibrary.wiley.com]
[…] dullness to percussion, and poor air entry. 89 Among patients with chronic chylothorax, muscle wasting, weight loss, and other signs of malnutrition can be present. 1 The patients may be compromised immunologically because of lymphocyte depletion and hypogammaglobulinemia [pediatrics.aappublications.org]
In turn, thoracostomy can lead to loss of lymphocytes, proteins, coagulation factors, and antibodies as well as lymphatic fluid, resulting in increased risk of occurrence of complications like hypoproteinemia, coagulopathy, lymphopenia, hypogammaglobulinemia [ncbi.nlm.nih.gov]
- Hypoalbuminemia
Adverse reactions are mild and include abdominal distension, hypoalbuminemia and rarely may contribute to septicemia through its inhibitory role on immune responses [4]. [cureus.com]
The loss of lymphatic fluid causes hypoalbuminemia, hydroelectrolyte alterations, coagulation and immunodeficiency 4 with important involvement of clinical condition of the child and increased morbidity and mortality. [scielo.br]
Treatment
Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. [kjp.or.kr]
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. [nature.com]
The average daily drain output within 3 days post-treatment (median 62 mL, range 10-651 mL) was significantly lower than that before treatment (median 133 mL, range 70-620 mL) (P = .002). [ncbi.nlm.nih.gov]
Prognosis
Morbidity of congenital chylothorax (CC) is high, and prognosis is very poor if chylothorax is associated with hydrops fetalis. The optimal treatment of CC has not been defined. [ncbi.nlm.nih.gov]
Appropriate and effective treatment of patients with chylothorax may contribute to the prognosis and neonatal survival. [gorm.com.tr]
Etiology
Chylothorax may have primary or secondary etiologies. [nature.com]
[…] could then be used to draft a prospective multicenter trial, we propose three flow-charts showing three algorithms that could be used to: 1) obtain a definitive diagnosis of pleural chylous effusion; 2) specifically focus on chyle leakage evolution and etiology [ncbi.nlm.nih.gov]
Currently, the etiology of chylothorax is poorly understood and treatment options are limited. [onlinejacc.org]
Epidemiology
Acquired Disease. 61 Slater BJ...Rothenberg SS 25629239 2015 49 Congenital chylothorax treated with oral sildenafil: a case report and review of the literature. 61 Malleske DT...Yoder BA 25917021 2015 50 Congenital chylothorax: a prospective nationwide epidemiological [malacards.org]
Congenital chylothorax: a prospective nationwide epidemiological study in Germany. Arch Dis Child - Fetal Neonatal Ed [Internet]. 2015;100(2):F169– 72. Available from: http://fn.bmj.com/lookup/doi/10.1136/ archdischild-2014-307274. 4. Rocha G. [revistas.uis.edu.co]
Congenital chylothorax: a prospective nationwide epidemiological study in Germany. Arch Dis Child Fetal Neonatal Ed 100: 169-72. Downie L, Sasi A, Malhotra A (2014) Congenital chylothorax: associations and neonatal outcomes. [omicsonline.org]
& Wolfe, R., 1 Jan 2019, In : Epidemiology (Cambridge, Mass.). 30, 1, p. 38-47 10 p. [monash.edu]
Pathophysiology
Autopsy findings from one of these cases and others previously described suggest that the pathophysiological mechanisms involved may be variable. [ncbi.nlm.nih.gov]
Pathogenesis: The main causes of fistula Chile are effusion thoracic duct obstruction at different levels or channel its repercussions on waging tributarelor pathophysiological mechanisms depending on the level where it appears. [medicaldb.blogspot.com]
However, we speculated that the pathophysiology of pneumothorax after thoracocentesis in these infants might involve so called “pneumothorax ex vacuo.” [frontiersin.org]
This review aims to summarize the current literature regarding pathophysiology, clinical manifestations, and management options for CC. [dovepress.com]
Question 3 Which of the following statements regarding the pathophysiology of chylothorax is INCORRECT? Chylothoraces in premature babies can occur when there is compression of the main thoracic duct. [thoracic.org]
Prevention
Pulmonary hypoplasia was successfully prevented by OK-432. [ncbi.nlm.nih.gov]
XENical in the prevention of diabetes in obese subjects (XENDOS) study: a randomized study of orlistat as an adjunct to lifestyle changes for the prevention of type 2 diabetes in obese patients. Diabetes Care 2004: 27(1):155-161. [books.google.es]
Page 223 - American Thoracic Society/Centers for Disease Control and Prevention/Infectious Diseases Society of America: treatment of tuberculosis. Am J Respir Crit Care Med 2003;167:603-662. [books.google.com]