Bronchogenic cyst: Difference between revisions

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==[[Bronchogenic cyst pathophysiology|Pathophysiology]]==
==[[Bronchogenic cyst pathophysiology|Pathophysiology]]==
It is thought that bronchogenic [[cyst]] is the result of abnormal budding of the ventral <ref name="pmid26986156">{{cite journal| author=Han C, Lin R, Yu J, Zhang Q, Zhang Y, Liu J | display-authors=etal| title=A Case Report of Esophageal Bronchogenic Cyst and Review of the Literature With an Emphasis on Endoscopic Ultrasonography Appearance. | journal=Medicine (Baltimore) | year= 2016 | volume= 95 | issue= 11 | pages= e3111 | pmid=26986156 | doi=10.1097/MD.0000000000003111 | pmc=4839937 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26986156  }} </ref> portion of the primitive [[foregut]]<ref name="pmid17859095">{{cite journal| author=Maier HC| title=Bronchiogenic Cysts of the Mediastinum. | journal=Ann Surg | year= 1948 | volume= 127 | issue= 3 | pages= 476-502 | pmid=17859095 | doi=10.1097/00000658-194803000-00010 | pmc=1513836 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17859095  }} </ref><ref name="pmid12809250">{{cite journal| author=Sarper A, Ayten A, Golbasi I, Demircan A, Isin E| title=Bronchogenic cyst. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 2 | pages= 105-8 | pmid=12809250 | doi= | pmc=161894 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12809250  }} </ref><ref name="pmid19015445">{{cite journal| author=Teissier N, Elmaleh-Bergès M, Ferkdadji L, François M, Van den Abbeele T| title=Cervical bronchogenic cysts: usual and unusual clinical presentations. | journal=Arch Otolaryngol Head Neck Surg | year= 2008 | volume= 134 | issue= 11 | pages= 1165-9 | pmid=19015445 | doi=10.1001/archotol.134.11.1165 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19015445  }} </ref>destined to become the [[respiratory tract|tracheobronchial tree]]<ref name="pmid30725658">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume=  | issue=  | pages=  | pmid=30725658 | doi= | pmc= | url= }} </ref> between days 26 - 40 of  [[gestation]]. <ref name="pmid30725658">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume=  | issue=  | pages=  | pmid=30725658 | doi= | pmc= | url= }} </ref> At such, it is often found in relation to the trachea or esophagus.<ref name="pmidhttps://doi.org/10.1186/1749-8090-7-94">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=https://doi.org/10.1186/1749-8090-7-94 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10  }} </ref> On rare occasions, they may be found in the [[Skull|cranial]], [[Neck|cervical]], [[Mediastinum|mediastinal]], and [[abdomen|abdominal]] regions. If  the cell mass maintains its connection with the [[respiratory tract|tracheobronchial tree]], the [[cyst]] becomes intrapulmonary or in close association with the lungs. If separated, there is no contiunity with the bronchia lumen. Such [[cyst]] increases in size gradually due to secretions produced within the cavity, leading to compressive symptoms.<ref name="pmid17859095">{{cite journal| author=Maier HC| title=Bronchiogenic Cysts of the Mediastinum. | journal=Ann Surg | year= 1948 | volume= 127 | issue= 3 | pages= 476-502 | pmid=17859095 | doi=10.1097/00000658-194803000-00010 | pmc=1513836 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17859095  }} </ref><ref name="pmid26002606">{{cite journal| author=Suda K, Sueyoshi R, Okawada M, Koga H, Lane GJ, Yamataka A | display-authors=etal| title=Completely intramural bronchogenic cyst of the cervical esophagus in a neonate. | journal=Pediatr Surg Int | year= 2015 | volume= 31 | issue= 7 | pages= 683-7 | pmid=26002606 | doi=10.1007/s00383-015-3720-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26002606  }} </ref>


==Gross pathology==
==Gross pathology==

Revision as of 18:47, 13 July 2020

Bronchogenic cyst
DiseasesDB 1707
MeSH D009404

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor: Joanna Ekabua, M.D. [2]

Synonyms and keywords: Bronchial cyst

Overview

Bronchogenic cyst is a rare benign congenital malformation of the tracheobronchial tree derived from the ventral aspect of the foregut. It most frequently occurs in the middle mediastinum, in the early stages of gestation or in the lungs, at the later stages of gestation. Atypical locations may be explained by its embryologic origin. Patients with bronchogenic cyst may be asymptomatic or present with respiratory distress, increasing stridor, feeding difficulties, chest pain, cough, progressive dysphagia, odynophagia, purulent sputum, dyspnea, anorexia and/orweight loss. Radiologic findings are useful to differentiate bronchogenic cysts from other cysts but may they not always confirm the diagnosis. Diagnosis is confirmed by surgical excision, which is curative, and histological findings of ciliated columnar epithelial lining of the cyst.

Historical Perspective

There is limited information about the historical perspective of bronchogenic cyst.

Classification

Pathophysiology

Gross pathology

Bronchogenic cyst appear as single or multiple smooth spherical pink masses which may be unlobulated or lobulated. [1][2] The lobules could be of various sizes, communicating or noncommunicating. The wall is relatively thin and frequently trabeculated,[2] even though there may be thicker portions. Cyst often appear to have check valves,[3] leading to their enlargement from secretions by its clilated columnar epithelium. Secretions may be a thick, white mucoid material if uninfected, and a dirty brown mucoid to purulent material if infected.[2] Occassionally, secretions could be hemorrhagic or pneumatic.[4]

Bronchogenic cyst. (Picture courtesy: Doctorlib)


Interior surface of a bronchogenic cyst showing some thick, white mucoid material at the top right corner (Yellow arrowhead). Case courtesy of Dr Yale Rosen (Picture courtesy: Wikimedia)


Histopathology

Bronchogenic cysts are usually lined by ciliated columnar epithelium of respiratory type, leading to distention as a result of secretions produced within the cavity. Cyst wall may be composed of cartilage, smooth muscle, fibrous tissue, chronic inflammatory infiltrates, necrotic tissue, focal areas of squamous metaplasia, vascular proliferation, bronchial gland, and mucous glands.[1] [2] [5] [6][7]

High magnification micrograph of a medisatinal bronchogenic cyst using H&E stain. Case courtesy of Assoc Prof Frank Gaillard (picture courtesy: Radiopaedia)


High magnification micrograph of a medisatinal bronchogenic cyst using H&E stain showing cilia. Case courtesy of Assoc Prof Frank Gaillard (picture courtesy: Radiopaedia)


Causes

The cause of bronchogenic cysts is undetermined.

Differentiating Bronchogenic cyst from other Diseases

Bronchogenic cyst must be differentiated from lung abcess, thymic cyst, and esophageal duplication cysts

Diseases Clinical manifestations Para-clinical findings Gold standard
Symptoms Physical examination
Lab Findings Imaging Histopathology
Symptom 1 Symptom 2 Symptom 3 Physical exam 1 Physical exam 2 Physical exam 3 Lab 1 Lab 2 Lab 3 Imaging 1 Imaging 2 Imaging 3
Lung abcess[8] Fever Chest pain night sweats Dullness to percussion coarse inspiratory crackles Decreased breath sounds Leukocytosis Increased inflamatory markers (ESR, CRP) Anemia of chronic disease
Frontal view of CXR showing lung abscess with air-fluid level. Case courtesy of Dr Abu-Rahmeh Zuhair (Picture courtesy: Radiopaedia)
Lateral view 0f CXR showing lung abscess with air-fluid level. Case courtesy of Dr Abu-Rahmeh Zuhair (Picture courtesy: Radiopaedia)
Non-contrast CT showing lung abscess with air-fluid levels. Case courtesy of Dr Elsayed Mohamed Elsayed (Picture courtesy: Radiopaedia)
H&E stain showing lung abscess. Arrowhead – pink necrotic tissue with necrotic granulocytes and bacteria. Arrow – chronic inflammatory cells and dilated blood vessels (Picture courtesy: Webpath)
CT with intravenous contrast
Thymic cyst[9] Cough Chest pain Dyspnea Some patients may present with a soft compressible neck mass - - - - -
x-ray scan showing a thymic mass (yellow arrowhead). Case courtesy of Dr. Stefan Ludwig (Picture courtesy: Radiopaedia)
CT scan showing a thymic mass corresponds to a cystic lesion (yellowarrow head). Case courtesy of Dr. Stefan Ludwig (Picture courtesy: Radiopaedia)
CT scan showing a calcified thymic mass corresponds to a cystic lesion (yellow arrowhead). Case courtesy of Dr Bruno Di Muzio (Picture courtesy: Radiopaedia)
H&E stain of thymic cyst showing bland squamous epithelium and some thymic tissue in its wall. Case courtesy of Dr Hanni Gulwani (Picture courtesy: Pathologyoutlines)
Biopsy withhistopathology and cytology
Esophageal duplication cysts[9][10][9] Retrosternal and thoracic back pain Epigastric discomfort Dysphagia - - - - - -
endoscopic view of an esophageal duplication cyst (Picture courtesy: Endoscopic ultrasound)
X-ray post barium swallow showing an oval mass compressing the esophagus. Case courtesy of Dr Michael P Hartung (Picture courtesy: Radiopaedia)
CT showing an esophageal duplication cyst. Case courtesy of Assoc Prof Craig Hacking (Picture courtesy: Radiopaedia)
Histologic image of an esophageal duplication cyst showing two-layered muscle coat like the GIT, and epithelium lining of pseudo-ciliated columnar cells. (Picture courtesy: National Center for Biotechnology Information)
Endoscopic ultrasound
  • Anechoic or hypoechoic, homogenous lesions
  • Regular margins originating from the submucosal layer or outside of the gut wall(adventitia)

Epidemiology and Demographics

Incidence

Prevalences

  • The prevalence of bronchogenic cyst is estimated at 1 per 42,000 and 1 per 68,000 admissions in two hospital series.[12]

Case fatality rate/mortality rate

Age

  • Bronchogenic cyst can be found in people of all age groups.[3]

Race

  • There is no racial predilection to bronchogenic cyst.

Gender

  • Bronchogenic cyst is slightly more prevalent in men and often remain undetected till the third or fourth decade of life.[14][7]

Risk Factors

There are no established risk factors for bronchogenic cyst.

Screening

There is insufficient evidence to recommend routine screening for bronchogenic cyst as it is usually an incidental finding.

Natural History, Complications and Prognosis

Natural History

The symptoms of bronchogenic cyst usually develop in the third to fourth decade of life and start with symptoms such as

Complication

Common complications of bronchogenic cyst include

Prognosis

Prognosis is generally good,[6] a Morbidity & Mortality rate of 20% has been recorded in older patients treated for bronchogenic cyst.[13]

Diagnosis

History and Symptoms

The majority of patients with bronchogenic cyst are asymptomatic and are diagnosed during investigations for other pathologies.[2][28][34]

When symptomatic, clinical presentation of bronchogenic cyst depends on the age of the patient, size and the location of the cyst.[2][35]

In infants bronchogenic cyst presents with [6][3][36][35][37][38][39]

In children and adults, bronchogenic cyst presents with

Physical Examination

Physical examination of patients with bronchogenic cyst is usually normal. On rare occasions, the following signs can be seen

Laboratory Findings

  • Some patients with bronchogenic cyst may have elevated serum tumor makers CA125 and CA19-9, which is usually suggestive of progression/complication.[32][46]

Despite enhanced imaging modalities, a definitive diagnosis of bronchogenic cyst is challenging preoperatively.[32]

Chest X Ray

An x-ray with esophagography may be helpful in the diagnosis of bronchogenic cyst. Findings on an x-ray suggestive of bronchogenic cyst include:[3][4]

  • Homogeneous non-calcified soft tissue density mass.
  • Round to oval opacity
  • Secondary excavation
  • Cavity with or without levels
  • Atelectasis
  • Hydropneumothorax
Frontal Chest X-ray showing left bronchogenic cyst. Case courtesy of Dr Kamel Mahmoud Alkhateeb (Picture courtesy: Radiopedia)


Lateral Chest X-ray showing left bronchogenic cyst. Case courtesy of Dr Kamel Mahmoud Alkhateeb (Picture courtesy: Radiopedia)


CT

CT scan may be helpful in the diagnosis of bronchogenic cyst. The density of bronchogenic cysts is variable from water density to high density due to blood, anthracotic pigment, increased calcium content, or increased protein content of the fluid.[11][47] Findings on CT scan suggestive of bronchogenic cyst is an

Non-contrast enhanced axial CT of mediastinal bronchogenic cyst showing a well-defined anterior mediastinal mass with incomplete peripheral thin linear calcification.. Case courtesy of Dr Mostafa El-Feky (picture courtesy: Radiopaedia)


Non-contrast enhanced coronal CT of bronchogenic cyst showing air-fluid levels. Case courtest of Dr Muhammad Shoyab (picture courtesy:https://radiopaedia.org/cases/mediastinal-bronchogenic-cyst-1?lang=gb Radipaedia])


MRI

MRI may be helpful in the diagnosis of bronchogenic cyst. After contrast injection, enhancement of the cyst is frequently absent.[17][32][11][47] Findings on MRI suggestive of bronchogenic cyst include

  • T1-weighted images show ipointense signal
  • T2-weighted images show hyperintense signal.
MRI of the chest at the level of T1 demonstrating a broncogenic cyst. Case courtesy of Dr Kamel Mahmoud Alkhateeb (Picture courtesy: Radiopedia)


MRI of the chest at the level of T1 demonstrating a broncogenic cyst. Case courtesy of Dr Kamel Mahmoud Alkhateeb (Picture courtesy: Radiopedia)


Ultrasound

ultrasound may be helpful in the diagnosis of bronchogenic cyst. Finding on an ultrasound suggestive of bronchogenic cyst include

Fetal Ultrasound (Picture courtesy: Fetalultrasound)


Fetal Ultrasound (Picture courtesy: Fetalultrasound)



Other Diagnostic Studies

Other diagnostic studies for bronchogenic cyst include:

Treatment

Surgery

Surgery is the mainstay of treatment for bronchogenic cyst even when the patients are asymptomatic,[28]conservative approach is not approved.[7] Surgery is imperative with symptoms such as [4]

Most commonly used approaches for complete excision of bronchogenic cysts are[4][32][28][41]

Other procedures include

Complete surgical resection is curative.[6][34] Risk during surgery is usually low, complications include but are not limited to persistent air leak,[4] pulmonary and vascular compressions, vascular, pleural, tracheobronchial, pulmonary, mediastinal and diaphragmatic adhesions,[7] inflammatory stenosis,[38] and thrid degree heart block.[17]

Case Studies

Case#1


Template:WikiDoc Sources CME Category::Cardiology

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  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 Maier HC (1948). "Bronchiogenic Cysts of the Mediastinum". Ann Surg. 127 (3): 476–502. doi:10.1097/00000658-194803000-00010. PMC 1513836. PMID 17859095.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Ribet ME, Copin MC, Gosselin BH (1996). "Bronchogenic cysts of the lung". Ann Thorac Surg. 61 (6): 1636–40. doi:10.1016/0003-4975(96)00172-5. PMID 8651761.
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  5. Mehta RP, Faquin WC, Cunningham MJ (2004). "Cervical bronchogenic cysts: a consideration in the differential diagnosis of pediatric cervical cystic masses". Int J Pediatr Otorhinolaryngol. 68 (5): 563–8. doi:10.1016/j.ijporl.2003.12.015. PMID 15081229.
  6. 6.00 6.01 6.02 6.03 6.04 6.05 6.06 6.07 6.08 6.09 6.10 Teissier N, Elmaleh-Bergès M, Ferkdadji L, François M, Van den Abbeele T (2008). "Cervical bronchogenic cysts: usual and unusual clinical presentations". Arch Otolaryngol Head Neck Surg. 134 (11): 1165–9. doi:10.1001/archotol.134.11.1165. PMID 19015445.
  7. 7.00 7.01 7.02 7.03 7.04 7.05 7.06 7.07 7.08 7.09 7.10 7.11 Aktoğu S, Yuncu G, Halilçolar H, Ermete S, Buduneli T (1996). "Bronchogenic cysts: clinicopathological presentation and treatment". Eur Respir J. 9 (10): 2017–21. doi:10.1183/09031936.96.09102017. PMID 8902460.
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  16. 16.0 16.1 16.2 16.3 16.4 Yildiz H, Reichwein R, Poncelet A, Lacroix V, D'abadie P, Ghaye B; et al. (2019). "An unusual case of cardiac tamponade: Bronchogenic cyst infection due to Salmonella bredeney". J Infect Chemother. 25 (2): 151–153. doi:10.1016/j.jiac.2018.07.012. PMID 30098916.
  17. 17.0 17.1 17.2 17.3 17.4 Olsen M, Mitchell TA, Percival TJ, Helsel BS (2015). "Interatrial Bronchogenic Cyst Resection". Ann Thorac Surg. 100 (2): 709–11. doi:10.1016/j.athoracsur.2014.10.025. PMID 26234845.
  18. 18.0 18.1 DiBardino DM, Brenner K (2017). "Bronchogenic Cyst Abscess". J Bronchology Interv Pulmonol. 24 (3): 31–32. doi:10.1097/LBR.0000000000000375. PMID 28696968.
  19. 19.0 19.1 Chumakova M, Morris-Stiff G, Khachaturov V, Ibrahim S (2016). "Bronchogenic cyst arising from the crus of the left hemidiaphragm". BMJ Case Rep. 2016. doi:10.1136/bcr-2015-213658. PMC 4840741. PMID 27068723.
  20. 20.0 20.1 20.2 Chen F, Marx S, Zhang C, Cao J, Yu Y, Chen D (2019). "Intramedullary bronchogenic cyst in the foramen magnum region accompanied with syringomyelia: A case report and literature review". Medicine (Baltimore). 98 (5): e14353. doi:10.1097/MD.0000000000014353. PMC 6380722. PMID 30702626.
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  22. 22.0 22.1 22.2 Miwa E, Tani T, Okada Y, Furukawa Y (2017). "A rare cardiac tumor: Bronchogenic cyst of interatrial septum". Echocardiography. 34 (3): 474–475. doi:10.1111/echo.13445. PMID 28139004.
  23. 23.0 23.1 23.2 23.3 23.4 23.5 Bian B, Yu M, Liu S, Li S, Wei Y, Zhang B (2019). "A suprasellar bronchogenic cyst: A case report". Medicine (Baltimore). 98 (30): e16650. doi:10.1097/MD.0000000000016650. PMC 6709100 Check |pmc= value (help). PMID 31348319.
  24. 24.00 24.01 24.02 24.03 24.04 24.05 24.06 24.07 24.08 24.09 24.10 24.11 Zou MX, Hu JR, Kang YJ, Li J, Lv GH, She XL (2015). "Bronchogenic cyst of the conus medullaris with spinal cord tethering: a case report and review of the literature". Int J Clin Exp Pathol. 8 (4): 3937–42. PMC 4466965. PMID 26097578.
  25. 25.0 25.1 25.2 25.3 25.4 25.5 Lee HD, Han SH, Park SB, Ko Y, Lee KH (2017). "An intradural extramedullary bronchogenic cyst in the thoracolumbar spine: A case report". Medicine (Baltimore). 96 (50): e9263. doi:10.1097/MD.0000000000009263. PMC 5815783. PMID 29390371.
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  32. 32.00 32.01 32.02 32.03 32.04 32.05 32.06 32.07 32.08 32.09 32.10 32.11 32.12 32.13 32.14 32.15 32.16 32.17 32.18 Han C, Lin R, Yu J, Zhang Q, Zhang Y, Liu J; et al. (2016). "A Case Report of Esophageal Bronchogenic Cyst and Review of the Literature With an Emphasis on Endoscopic Ultrasonography Appearance". Medicine (Baltimore). 95 (11): e3111. doi:10.1097/MD.0000000000003111. PMC 4839937. PMID 26986156.
  33. 33.0 33.1 33.2 Blesneac C, Horvath E, Muntean I, Benedek T, Toganel R (2018). "Intracardiac bronchogenic cyst associated with ventricular septal defect: an extremely rare feature in children". Eur Heart J Cardiovasc Imaging. 19 (9): 1074. doi:10.1093/ehjci/jey078. PMID 29897508.
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