Bronchogenic cyst

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

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 they may 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.

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

Bronchogenic cyst can be classified based on location; pulmonary and extrapulmonary.

It is thought that bronchogenic cyst is the result of abnormal budding of the ventral portion of the primitive foregut between days 26 – 40 of gestation.

The cause of bronchogenic cysts is undetermined.

Differentiating bronchogenic cyst from lung abcess, thymic cyst, and esophageal duplication cysts.

There is no racial predilection to bronchogenic cyst. Bronchogenic cyst is slightly more prevalent in men and often remain undetected until the third or fourth decade of life.

There are no established risk factors for bronchogenic cyst.

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

Natural History

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

• Dyspnea
• Chronic dry cough
• Rhinitis
• Chest discomfort/pain
• Neck pain
• Flu-like symptoms and Fever
• Purulent sputum
• Abdominal discomfort
• Limb weakness
• Paroxysmal supraventricular tachycardia
• Atrioventricular block
• Memory impairment
• Asthenia
• Seizure and loss of consciousness
• Dizziness
• Lower back pain
• Urinary incontinence

Complication

Common complications of bronchogenic cyst include

• Tracheal compression
• Esophageal compression
• Cough
• Infection/abscess
• Hemoptysis
• Superior vena cava syndrome
• Pneumothorax
• Pleurisy
• Pneumonia
• Lymphangioma
• Haemorrhage
• Neoplasia(adenocarcinoma, mucoepidermoid carcinoma, anaplastic carcinoma, and leiomyosarcoma)
• Rupture
• Air embolism
• Cardiac tamponade
• Ventricular septal defect
• syringomyelia

Prognosis

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

Excision and Hematoxylin and Eosin staining.

Cillated columar epithelia cells is definitive for broncogenic cyst.

Cystic mass-producing compressive symptoms.

The majority of patients with bronchogenic cyst are asymptomatic and are diagnosed during investigations for other pathologies.

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

In infants bronchogenic cyst presents with

• respiratory distress
• Increasing stridor
• Reccurrent croup
• Feeding difficulties.
• Palpable neck mass
• Non-bilious emesis

In children and adults, bronchogenic cyst presents with

• Dull chest pain with radiation to the shoulder in diaphragmatic broncogenic cyst.
• Cough
• Progressive dysphagia
• Odynophagia
• wheezing
• Adobminal bloating
• Fever
• Palpable neck mass
• purulent sputum
• Hemoptysis
• Dyspnea
• Anorexia and/or weight loss
• Dizziness
• Paroxysmal supraventricular tachycardia
• Atrioventricular block
• Atrial and ventricular premature complexes
• Memory impairment
• Asthenia
• Limping
• Seizure and loss of consciousness
• Sciatica
• Back pain
• Neuralgia
• Urinary incontinence

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

• Cough
• wheezing
• Stridor
• Hemoptysis
• Dyspnea
• Tracheal deviation
• Fluid-thrill
• Anorexia and/or weight loss
• Fever
• Asthenia
• Foot drop
• Atrophy
• Heart murmur
• Tenderness
• Cutanous pigmentation
• Negative knee and ankle reflexes
• Numbness

An elevated serum tumor makers CA125 and CA19-9, which is usually suggestive of progression/complication.

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. Findings on CT scan suggestive of bronchogenic cyst is an

• Cysic mass with air-fluid levels.
• ovoid and well-defined encapsulated low-density cystic mass with no contrast enhancement.
• Thin subtle walls.

MRI may be helpful in the diagnosis of bronchogenic cyst. After contrast injection, enhancement of the cyst is frequently absent. Findings on MRI suggestive of bronchogenic cyst include

• T1-weighted images show ipointense signal
• T2-weighted images show hyperintense signal.

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

• On endobronchial ultrasound, an anechoic or hypoechoic fluid-filled solid mass.
• On transthoracic echocardiography revealing intracardiac cystic mass.
• On endoscopic ultrasonography, which demonstartes abdominal anatomic relations of the cyst.

Other diagnostic studies for bronchogenic cyst include: Excision and Hematoxylin and Eosin staining, which demonstrates ciliated columar epithelia cells is definitive for bronchogenic cyst

• Immunohistochemical staining of bronchogenic cyst of the esophagus , which demonstrates a positive expression of CA199 and CA125.
• Bronchoscopy, which demonstrates pink cystic mass with bronchial attachment
• Gastroendoscopy which demonstartes abdominal anatomic relations of the cyst.
• fluorodeoxyglucose–positron emission tomography to confirm cyst infection.

The mainstay of treatment for bronchogenic cyst is surgery.

Surgery is the mainstay of treatment for bronchogenic cyst even when the patients are asymptomatic, a conservative approach is not approved.

There are no established measures for the primary prevention of bronchogenic cyst.

There are no established measures for the secondary prevention of bronchogenic cyst.

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

Bronchogenic cyst is a rare benign congenital malformation of the tracheobronchial tree derived from the ventral aspect of the foregut.

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

Template:WH Template:WS CME Category::pulmonology

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

Bronchogenic cyst can be classified based on location as follows^[1][2]

1. Pulmonary
   1. Plural^[3]
      1. Visceral
      2. Parietal
   2. Tracheobronchial tree^[1]
      1. Paratracheal
      2. Carinal
      3. Hilar
2. Extra-pulmonary
   1. Cranial^[4][5]
   2. Cervical^[2][6][7]
   3. Mediastinal^[8]
      1. Esophageal^[9][10][11]
      2. Cardiac^{[1][12][13][14]}
   4. Abdominal^[2][15]
      1. Intraperitoneal
         1. Dipgragmatic^[16][17][18]
         2. Gastric^[19][20]
      2. Retroperitoneal
         1. Spinal^{[1][2][21][22][23]}
         2. Adrenal^[24][25][26]
         3. Pancreatic^[27][28]
   5. Perianal^[29]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Joanna Ekabua, M.D. [2] Kalsang Dolma, M.B.B.S.[3]

It is thought that bronchogenic cyst is the result of abnormal budding of the ventral ^[1] portion of the primitive foregut^[2][3][4]destined to become the tracheobronchial tree^[5] between days 26 – 40 of gestation. ^[5] At such, it is often found in relation to the trachea or esophagus.^[6] On rare occasions, they may be found in the cranial, cervical, mediastinal, and abdominal regions. If the cell mass maintains its connection with the 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.^[2][7]

Bronchogenic cyst appear as single or multiple smooth spherical pink masses which may be unlobulated or lobulated. ^[5][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,^[8] 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.^[3]

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

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.^{[5] [2] [9] [4][10]}

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

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

Bronchogenic cyst is a rare benign congenital malformation of the tracheobronchial tree derived from the ventral aspect of the foregut.

The cause of bronchogenic cysts is undetermined.

Template:WH Template:WS

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

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

Differentiating bronchogenic cyst 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[1]	Fever	Chest pain	night sweats	Dullness to percussion	coarse inspiratory crackles	Decreased breath sounds	Leukocytosis	Increased inflamatory markers (ESR, CRP)	Anemia of chronic disease					CT with intravenous contrast
Thymic cyst[2]	Cough	Chest pain	Dyspnea	Some patients may present with a soft compressible neck mass	–	–	–	–	–					Biopsy withhistopathology and cytology
Esophageal duplication cysts[2][3][2]	Retrosternal and thoracic back pain	Epigastric discomfort	Dysphagia	–	–	–	–	–	–					Endoscopic ultrasound Anechoic or hypoechoic, homogenous lesions Regular margins originating from the submucosal layer or outside of the gut wall(adventitia)

Template:WH Template:WS CME Category::Cardiology

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

Incidence

• Bronchogenic cyst accounts for 10% to 15% of mediastinal tumors and 50 to 60% of all mediastinal cysts.^[1]

Prevalences

• The prevalence of bronchogenic cyst is estimated at 1 per 42,000 and 1 per 68,000 admissions in two hospital series.^[2]

Case fatality rate/mortality rate

• In 2011, broncogenic cyst was shown to have a case-fatality rate/mortality rate of 20%.^[3]

Age

• Bronchogenic cyst can be found in people of all age groups.^[4]

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.^[5][6]

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

There are no established risk factors for bronchogenic cyst.

There are no established risk factors for bronchogenic cyst.

Template:WH Template:WS CME Category::Cardiology

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

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

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

Template:WH Template:WS CME Category::Cardiology

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

The symptoms of bronchogenic cyst usually develop in the third to fourth decade of life.

Natural History

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

• Dyspnea^[1][2][3][4]
• Chronic dry cough^[1][2][4]
• Rhinitis^[5]
• Chest discomfort/pain^[1][6][2][3]
• Neck pain^[7]
• Flu-like symptoms and Fever^[1][2][3]
• Purulent sputum^[1]
• Abdominal discomfort
• Limb weakness
• Paroxysmal supraventricular tachycardia^[8]
• Atrioventricular block^[9]
• Memory impairment^[10]
• Asthenia^[10][11][12]
• Seizure and loss of consciousness^[13]
• Dizziness^[14]
• Lower back pain^[11]
• Urinary incontinence^[11]

Complication

Common complications of bronchogenic cyst include

• Tracheal compression^[15][16]
• Esophageal compression^[1]
• Cough^[15]
• Infection/abscess^{[15][17][18][5]}
• Hemoptysis^[1][15]
• Superior vena cava syndrome^[16][19][20]
• Pneumothorax^[1][16]
• Pleurisy^[16]
• Pneumonia^[1][16]
• Lymphangioma^[17]
• Haemorrhage^[17][18]
• Neoplasia(adenocarcinoma, mucoepidermoid carcinoma, anaplastic carcinoma, and leiomyosarcoma)^{[17][21][22][18]}
• Rupture^[1][21][18]
• Air embolism^[13]
• Cardiac tamponade^[3]
• Ventricular septal defect^[23]
• syringomyelia^[7]

Prognosis

Prognosis is generally good,^[17] a Morbidity & Mortality rate of 20% has been recorded in older patients treated for bronchogenic cyst.^[24]

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