Bronchogenic cysts are congenital malformations of the bronchial tree (type of bronchopulmonary foregut malformation). They can present as a mediastinal mass that may enlarge and cause local compression. It is also considered the commonest of foregut duplication cysts.
Bronchogenic cysts are rare congenital lesions accounting for only 5-10% of paediatric mediastinal masses 8. The incidence of mediastinal cysts is equal between the sexes whereas intrapulmonary cysts are reported to have a male predilection 8.
In many instances, bronchogenic cysts are asymptomatic and are found incidentally when the chest is imaged 5. When large, mass effect may result in bronchial obstruction leading to air trapping and respiratory distress. An alternative presentation may occur when the cyst becomes infected.
Bronchogenic cysts form as a result of abnormal budding of the bronchial tree during embryogenesis (between 4th-6th weeks) 1, and as such they are lined by secretory respiratory epithelium (cuboid or columnar ciliated epithelium) 1,4. The wall is made up of tissues similar to that of the normal bronchial tree, including cartilage, elastic tissues, mucous glands and smooth muscle 1. They do not usually communicate with the bronchial tree, and are therefore typically not air filled. Rather, they contain fluid (water), variable amounts of proteinaceous material, blood products, and calcium oxalate 4. It is the latter three components that result in increased attenuation mimicking solid lesions. They are rarely multiple.
They can occur in the mediastinum or be intrapulmonary. The most common location is the middle mediastinum (65-90%). The distribution of locations can be quite varied:
- mediastinal (~70%) 5
- usually does not communicate with the tracheobronchial tree
- subcarinal, right paratracheal and hilar locations most common
- approximate incidence includes
- carinal area: ~50%
- paratracheal area: ~20%
- oropharangeal wall: ~15%
- retrocardiac area: ~10%
- parenchymal (intrapulmonary)
- typically perihilar
- predilection for lower lobes 1
- other uncommon locations
- cutaneous 2
- pericardium 5
- extending across the diaphragm and appearing dumb-bell shaped 1
- retroperitoneal 3
Although bronchogenic cysts are usually fluid filled, occasionally a communication may develop following infection or intervention, resulting in an air-filled cystic structure +/- an air-fluid level 1,5.
The cysts usually appear as soft-tissue density rounded structures, sometimes with compression of surrounding structures. Occasionally such compression can lead to air-trapping and a hyperlucent hemithorax 5. As the cysts may contain calcium oxalate, dependent layering of calcific density material (milk of calcium) may on occasion be seen 5,10.
Typically appear as well circumscribed spherical or ovoid masses of variable attenuation 1,4 with variable fluid composition explaining the different CT attenuations observed.
Approximately 50% are fluid density (0-20 HU), however a significant proportion are of soft tissue density (>30 HU) or even hyperdense to surrounding mediastinal soft tissues 4. CT is better able to detect calcium oxalate (milk of calcium) layering dependently 10. Usually, there is no solid contrast enhancement.
Sometimes performed for confirmation
- variable signal intensity, from low (similar to fluid) to high (due to protein content)
- fluid fluid level has also been reported, attributed to layering of variable fluid content 4-5
- T2: usually high signal intensity due to fluid contents
Treatment and prognosis
The choice of treatment is somewhat controversial. Some authors advocate surgical excision of all cysts given their tendency to become infected or rarely, to undergo malignant transformation 5. Increasingly, these lesions are treated with transbronchial or percutaneous aspiration under CT guidance to both confirm the diagnosis and to treat them. Small lesions can be followed, however they do have a tendency to increase in size over time, sometimes rapidly 7.
- fistula formation with the bronchial tree
- ulceration of the cyst wall
- secondary bronchial atresia
- superimposed infection
- malignant transformation is very rare (0.7% risk 11), but reported, with primaries including 5:
General imaging differential considerations for uncomplicated cysts include:
- congenital cysts and malformations
- intrathoracic pancreatic pseudocyst (for retroperitoneal bronchogenic cysts or for pancreatic pseudocysts that extend intra thoracically through the aortic hiatus or oesophageal hiatus)
If complicated, e.g. with infection or haemorrhage, also consider:
- enlarged lymph nodes (especially if centrally necrotic
- pulmonary masses
- focal haematoma: different clinical context
- 1. Yoon YC, Lee KS, Kim TS et-al. Intrapulmonary bronchogenic cyst: CT and pathologic findings in five adult patients. AJR Am J Roentgenol. 2002;179 (1): 167-70. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Pradeep KE. Cutaneous bronchogenic cyst: an under-recognised clinicopathological entity. J. Clin. Pathol. 2009;62 (4): 384. doi:10.1136/jcp.2008.057695 - Pubmed citation
- 3. Onol FF, Baytekin F, Dikbas O et-al. A retroperitoneal bronchogenic cyst mimicking adrenal tumour in an adult: is differential diagnosis truly possible? J. Clin. Pathol. 2009;62 (2): 187-9. doi:10.1136/jcp.2008.061077 - Pubmed citation
- 4. Lyon RD, Mcadams HP. Mediastinal bronchogenic cyst: demonstration of a fluid-fluid level at MR imaging. Radiology. 1993;186 (2): 427-8. Radiology (abstract) - Pubmed citation
- 5. Lucaya J, Baert AL, Strife JL. Pediatric Chest Imaging, Chest Imaging in Infants and Children. Springer Verlag. (2007) ISBN:3540326758. Read it at Google Books - Find it at Amazon
- 6. Shields TW. General Thoracic Surgery. Lippincott Williams & Wilkins. (2009) ISBN:0781779820. Read it at Google Books - Find it at Amazon
- 7. Naidich DP, Srichai MB, Krinsky GA. Computed tomography and magnetic resonance of the thorax. Lippincott Williams & Wilkins. (2007) ISBN:0781757657. Read it at Google Books - Find it at Amazon
- 8. Staatz G, Honnef D, Piroth W et-al. Pediatric Imaging. George Thieme Verlag. (2007) ISBN:1604060832. Read it at Google Books - Find it at Amazon
- 9. Grainger & Allison's diagnostic radiology essentials. Churchill Livingstone. ISBN:0702034487. Read it at Google Books - Find it at Amazon
- 10. McAdams HP, Kirejczyk WM, Rosado-de-Christenson ML et-al. Bronchogenic cyst: imaging features with clinical and histopathologic correlation. Radiology. 2000;217 (2): 441-6. doi:10.1148/radiology.217.2.r00nv19441 - Pubmed citation
- 11. Kirmani B, Kirmani B, Sogliani F. Should asymptomatic bronchogenic cysts in adults be treated conservatively or with surgery?. Interact Cardiovasc Thorac Surg. 2010;11 (5): 649-59. doi:10.1510/icvts.2010.233114 - Pubmed citation