A chylothorax refers to the presence of chylous fluid in pleural space often as a result of obstruction or disruption to thoracic duct. It may be congenital or acquired.
It can be due to direct injury to the lymphatic ducts or due to obstruction of the ducts and fragile collateral formations which are prone to spontaneous rupture:
- iatrogenic: post thoracic and abdominal surgery in particular oesophagectomy and block dissection of the neck considered the most common aetiology in modern days
- lymphoma: generally thought to be the most common non-traumatic cause
- non-surgical trauma, e.g. thoracic duct laceration due to thoracic vertebrae fractures
- idiopathic/cryptogenic: most common in neonatal period
- congenital thoracic duct ectasia
- fibrosing/sclerosing mediastinitis
- nephrotic syndrome
- tuberous sclerosis
- sarcoidosis (rare) 3
- Gorham disease (rare; shoulder girdle/thoracic bone osteolysis) 6
In a neonate:
- congenital heart disease
- Turner syndrome
- Noonan syndrome
- Down syndrome
- pulmonary sequestration (extralobar)
- tracheo-oesophageal fistula
Increased density of hemithorax with ipsilateral pleural effusion (most common on the left). Less frequently bilateral.
Simple appearing pleural effusion without debris or septations.
Most of the time, it appears as a simple fluid collection of near water density.
Low output (less than 1000ml/day) is treated conservatively with a low-fat diet. High output usually treated with open or video-assisted ligation. Alternatively, thoracic duct embolization can be performed by an interventional radiologist with a comparable outcome to surgical ligation.
- 1. Talwar A, Lee HJ. A contemporary review of chylothorax. Indian J Chest Dis Allied Sci. 50 (4): 343-51. Pubmed citation
- 2. Fairfax AJ, McNabb WR, Spiro SG. Chylothorax: a review of 18 cases. Thorax. 1986;41 (11): 880-5. doi:10.1136/thx.41.11.880 - Free text at pubmed - Pubmed citation
- 3. Jarman PR, Whyte MK, Sabroe I et-al. Sarcoidosis presenting with chylothorax. Thorax. 1995;50 (12): 1324-5. Thorax (link) - Free text at pubmed - Pubmed citation
- 4. Büttiker V, Fanconi S, Burger R. Chylothorax in children: guidelines for diagnosis and management. Chest. 1999;116 (3): 682-7. doi:10.1378/chest.116.3.682 - Pubmed citation
- 5. Doerr CH, Allen MS, Nichols FC et-al. Etiology of chylothorax in 203 patients. Mayo Clin. Proc. 2005;80 (7): 867-70. doi:10.4065/80.7.867 - Pubmed citation
- 6. Collins J. Case 92: Gorham syndrome. Radiology. 2006;238 (3): 1066-9. doi:10.1148/radiol.2383032126 - Pubmed citation