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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/05/09 18:21:08」(JST)

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A chylothorax (or chyle leak) is a type of pleural effusion. It results from lymphatic fluid (chyle) accumulating in the pleural cavity.

Causes

Its cause is usually leakage from the thoracic duct or one of the main lymphatic vessels that drain to it. The most common causes are lymphoma and trauma caused by thoracic surgery. If the patient is on a normal diet, the effusion can be identified by its white and milky appearance, as it contains high levels of triglycerides. This chyle composition of triglycerides is mostly in the form of chylomicrons.

The condition is rare but serious, and appears in all mammals. In animals, chylothorax usually results from diseases that cause obstruction to the thoracic duct, preventing lymph from draining normally into the venous system. Examples include tumors, heartworm disease, right-sided cardiac failure, or idiopathic lymphangiectasia.

Treatment

Since the mechanism behind chylothorax is not well understood, treatment options are limited. Drainage of the fluid out of the pleural space is essential to obviate damage to organs, especially the inhibition of lung function by the counter pressure of the chyle. Another treatment option is pneumoperitoneal shunting (creating a communication channel between pleural space and peritoneal cavity). By this surgical technique loss of essential triglycerides that escape the thoracic duct can be prevented. Omitting fat (in particular FFA) from the diet is essential. Either surgical or chemical pleurodesis are options: the leaking of lymphatic fluids is stopped by irritating the lungs and chest wall, resulting in swelling and closure of the pleural space. The medication octreotide has been shown to be beneficial and in some cases will stop the chylothorax after a few weeks.^[1]^[2]

In animals, the most effective form of treatment until recently has been surgical ligation of the thoracic duct combined with partial pericardectomy.^[3] There is at least one case report (in a cat) of clinical response to treatment with rutin.^[4]

References

^ Dalokay Kilic, MD, Ekber Sahin, MD, Oner Gulcan, MD, Bulent Bolat, MD, Riza Turkoz, MD, Ahmet Hatipoglu, MD (2005). "Octreotide for Treating Chylothorax after Cardiac Surgery". Texas Heart Institute Journal 32 (3): 437–39. PMC 1336729. PMID 16392238.
^ Marcia L. Buck, Pharm.D., FCCP (2004). "Octreotide for the Management of Chylothorax in Infants and Children". Pediatric Pharmacotherapy 10 (10).
^ Birchard SJ, Smeak DD, McLoughlin MA (March 1998). "Treatment of idiopathic chylothorax in dogs and cats". J. Am. Vet. Med. Assoc. 212 (5): 652–7. PMID 9524635.
^ http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1180424/

External links

00085 at CHORUS
University of Wisconsin: http://www.vetmed.wisc.edu/dss/ChyloThoraxTrial/page5.php
Morris Animal Foundation: http://www.morrisanimalfoundation.org/ada_studies.php?col=study&val=179

UpToDate Contents

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1. 乳糜胸症の病因、臨床像、および診断 etiology clinical presentation and diagnosis of chylothorax
2. 乳糜胸症のマネージメント management of chylothorax
3. 食道切除術：合併症およびマネージメント esophagectomy complications and management
4. 乳糜胸症の臨床像、診断、およびマネージメント clinical presentation diagnosis and management of cholesterol effusions
5. 心臓手術後の胸水 pleural effusions following cardiac surgery

English Journal

RASA1 somatic mutation and variable expressivity in capillary malformation/arteriovenous malformation (CM/AVM) syndrome.

Macmurdo CF1, Wooderchak-Donahue W2,3, Bayrak-Toydemir P2,3, Le J2, Wallenstein MB1, Milla C1, Teng JM4, Bernstein JA1, Stevenson DA1.
American journal of medical genetics. Part A.Am J Med Genet A.2016 Jun;170(6):1450-4. doi: 10.1002/ajmg.a.37613. Epub 2016 Mar 11.
Germline mutations in RASA1 are associated with capillary malformation-arteriovenous malformation (CM-AVM) syndrome. CM-AVM syndrome is characterized by multi-focal capillary malformations and arteriovenous malformations. Lymphatic anomalies have been proposed as part of the phenotype. Intrafamilial
PMID 26969842

[The Use of Octreotide Acetate in the Management of Refractory Chylothorax Following Surgical Treatment for Lung Cancer].

Kawakami T1, Ishida I, Sugawara T, Oura H.
Kyobu geka. The Japanese journal of thoracic surgery.Kyobu Geka.2016 Jun;69(6):429-32.
A 78-year-old man underwent right upper lobectomy with systemic lymph node dissection for lung cancer. On the 1st operative day, chylothorax was suspected by a large amount of yellowish-white fluid through the chest tubes. In spite of stopping the oral intake as a conservative therapy, a lot of chyl
PMID 27246125

Endothelial Mitogen-Activated Protein Kinase Kinase Kinase Kinase 4 Is Critical for Lymphatic Vascular Development and Function.

Roth Flach RJ1, Guo CA2, Danai LV2, Yawe JC2, Gujja S2, Edwards YJ2, Czech MP1.
Molecular and cellular biology.Mol Cell Biol.2016 May 31;36(12):1740-9. doi: 10.1128/MCB.01121-15. Print 2016 Jun 15.
The molecular mechanisms underlying lymphatic vascular development and function are not well understood. Recent studies have suggested a role for endothelial cell (EC) mitogen-activated protein kinase kinase kinase kinase 4 (Map4k4) in developmental angiogenesis and atherosclerosis. Here, we show th
PMID 27044870