Chylothorax in Infants and Children

Tutor, James D.

doi:10.1542/peds.2013-2072

Cited by 171 publications

(216 citation statements)

References 148 publications

(190 reference statements)

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“…The clinical picture of a patient with chylothorax is insidious and develops as fluid accumulates in the pleural cavity; asymptomatic at first, it then progresses with cough and dyspnea1 , 2; fever and pleuritic pain are rare findings 23 On physical examination, there is unilateral or bilateral dullness and decreased breath sounds 3.…”

Section: Discussionmentioning

confidence: 99%

“…, 3 On physical examination, there is unilateral or bilateral dullness and decreased breath sounds 3. Complications of a chylothorax with chronic evolution include protein-calorie malnutrition, immune deficiency by lymphocyte and immunoglobulin depletion1 , 4 and electrolyte disorders 5…”

Section: Discussionmentioning

confidence: 99%

“…, 3 Usually, cellularity is predominantly comprised of lymphocytes (>50%), with a protein content between 2 and 3mg/dL5 and low levels of lactate dehydrogenase 35 If the diagnostic doubt persists, the preferred method is chylomicron analysis in the biological fluid, with a positive result 13 …”

Section: Discussionmentioning

confidence: 99%

“…A review published in 2014 reports more than 35 possible etiologies 1. Among these are congenital malformations of the lymphatic system, such as pulmonary lymphangioma, lymphangiectasia and thoracic duct atresia1 , 2 , 6; chylothorax associated with genetic syndromes, such as Down, Noonan and Turner syndrome,6 among others1 , 6; after head and neck and thoracic surgical procedures (in up to 6% of cardiac surgeries)1 , 7; after other iatrogenic events in the neonatal period, such as birth trauma and superior vena cava thrombosis due to central venous catheterization5 , 6; chylothorax after closed thoracic trauma1; and chylothorax associated with cancer, such as neurogenic neoplasia, teratomas, sarcomas and especially lymphomas, in which the lymph accumulation in the pleural space may be the initial manifestation,1 , 2 in addition to granulomatous infections such as tuberculosis 1. The patient in this case did not have findings that were consistent with congenital malformations, had not suffered trauma or surgery, whereas cancer and infections were ruled out.…”

Section: Discussionmentioning

confidence: 99%

“…, 2 It can lead to significant respiratory morbidity and has an extensive list of causes, with great diagnostic difficulty 12 This study aims to report the case of a child with spontaneous bilateral chylothorax.…”

Section: Introductionmentioning

confidence: 99%

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Bilateral spontaneous chylothorax after severe vomiting in children

Rodrigues

Romaneli

Ramos

et al. 2016

Revista Paulista de Pediatria (English Edition)

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Objective:To report the case of a child with bilateral chylothorax due to infrequent etiology: thoracic duct injury after severe vomiting.Case description:Girl, 7 years old, with chronic facial swelling started after hyperemesis. During examination, she also presented with bilateral pleural effusion, with chylous fluid obtained during thoracentesis. After extensive clinical, laboratory, and radiological investigation of the chylothorax etiology, it was found to be secondary to thoracic duct injury by the increased intrathoracic pressure caused by the initial manifestation of vomiting, supported by lymphoscintigraphy findings.Comments:Except for the neonatal period, chylothorax is an infrequent finding of pleural effusion in children. There are various causes, including trauma, malignancy, infection, and inflammatory diseases; however, the etiology described in this study is poorly reported in the literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Bilateral spontaneous chylothorax after severe vomiting in children

Rodrigues

Romaneli

Ramos

et al. 2016

Revista Paulista de Pediatria (English Edition)

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Persistent chylothorax associated with lymphatic malformation type 6 due to biallelic pathogenic variants in PIEZO1

Kovesi

Rojas

Boycott

2023

American J of Med Genetics Pt A

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PIEZO1 is required for lymphatic valve formation, and several lymphatic abnormalities have been reported to be associated with autosomal recessive PIEZO1 pathogenic variants including neonatal hydrops, lymphedema involving various body regions, and chylothorax. Persistent or recurrent chylothorax has been infrequently described in association with pathogenic variants in the PIEZO1 gene. We present a 4-year-old female with bilateral pleural effusions detected prenatally, who was diagnosed with bilateral chylothoraces post-partum. She subsequently had recurrent pleural effusions involving both pleural cavities, which tended to improve with restriction of her fat intake, and, one occasion, subcutaneous octreotide. She also had bilateral calf, and intermittent cheek swelling. Genetic testing revealed two deleterious variants in PIEZO1: c.2330-2_2330-1del and c.3860G > A (p.Trp1287*), both of which were classified as likely pathogenic. This supported a diagnosis of Lymphatic Malformation Type 6 (OMIM 616843), also known as Hereditary Lymphedema Type III.Hereditary Lymphedema type III can be associated with persistent chylothorax that can vary in size over time.

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Severe generalized edema in a premature neonate: A case report and literature review

Zong,

Huang,

Xiong

et al. 2024

Clinical Case Reports

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Key Clinical MessageWith no family history, and an atypical phenotype, the clinical diagnosing of Noonan syndrome (NS) can be very difficult. The present case emphasized that generalized edema in neonates may be the potential first symptom of NS.AbstractSevere generalized edema is a rare pathological condition with high mortality in newborns, in particular the premature infants. It is characterized by the extensive subcutaneous tissue edema and the accumulation of fluid in neonatal body fluid compartments. The etiology and pathogenesis of hydrops in neonates are quite complex. Generally speaking, hydrops can be divided into immune hydrops and non‐immune hydrops according to the etiology. It is still challenging in treating severe neonatal edema. In this study, we presented a preterm newborn with severe generalized edema after birth, which was finally diagnosed with Noonan syndrome (NS). The infant clinically manifested as severe generalized edema alone, without the involvement of multiple organ malformation. Generalized edema in neonates was probably the first symptom of NS. Therefore, differential diagnosis of NS is necessary for infants developing generalized edema.

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Chylothorax in Infants and Children

Cited by 171 publications

References 148 publications

Bilateral spontaneous chylothorax after severe vomiting in children

Bilateral spontaneous chylothorax after severe vomiting in children

Persistent chylothorax associated with lymphatic malformation type 6 due to biallelic pathogenic variants in PIEZO1

Severe generalized edema in a premature neonate: A case report and literature review

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