eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Bronchogenic Cyst

Updated: Oct 6, 2008

Introduction

Background

Bronchogenic cysts, although relatively rare, represent the most common cystic lesion of the mediastinum. Shanti and Klein (2008) studied a series of 236 patients undergoing pulmonary resection for cystic lung lesions.¹ Bronchogenic cysts constituted 20% of this group. Of these 47 cases, 20 involved a lobar location, which required lobectomy, and 27 cases were extralobar and were treated with resection of the cyst.

In infants and small children, these cysts can be life threatening when they compress vital structures. In particular, subcarinal cysts can pose life-threatening airway compromise. In infants, the initial presentation may be respiratory distress. More than one half of patients are asymptomatic.

The cysts are usually found using antenatal ultrasonography and routine chest radiography and during evaluations for GI or cardiac symptomatology. Bronchogenic cysts are the result of anomalous development of the ventral foregut; they are usually single but may be multiple and can be filled with fluid or mucus. They have been found all along the tracheoesophageal course, in perihilar or intraparenchymal sites, with a predilection for the area around the carina. Those in the mediastinum frequently attach to, but do not communicate with, the tracheobronchial tree. Bronchogenic cysts have also been described in more remote locations, including the interatrial septum, neck, abdomen, and retroperitoneal space.

Pathophysiology

Symptomatic patients usually present with symptoms related to cyst infection or compression of adjacent structures. Bronchogenic cysts can be diagnosed in infants with respiratory distress because of the frequency at the level of the carina. Partial obstruction of the trachea or bronchus with resultant emphysema may occur, and some reports have noted cases in which Swyer-James syndrome and asthma were initially considered. Less frequently, communications may develop between the cyst and airway. Both adenocarcinoma and rhabdomyosarcoma have been reported to arise. Cysts located within the abdomen may also produce symptoms because of infection or compression of adjacent structures, and hemorrhage has been reported in cysts with gastric mucosa.

Frequency

United States

Although rare, bronchogenic cysts are the second most common subtype of foregut cysts found in the middle mediastinum. Enterogenous cysts are the most common subtype of foregut cysts, representing as much as 70%; bronchogenic cysts represent 7-15% of the cystic lesions of the foregut. Reported prevalence rates are 1 case per 42,000 population and 1 case per 68,000 population, respectively.²

Mortality/Morbidity

Ribet et al's 1995 series reported a postoperative morbidity of 13.4%.³ This series included 45 adult and 24 pediatric cases over a 25-year period at the University of Lille in France.

Sex

No sex predilection is apparent.

Age

More than 50% of cases are diagnosed in patients older than 15 years.

Clinical

History

Chest pain and dysphagia are the most common symptoms in adults with bronchogenic cysts; in infants, symptoms are most often produced as a result of airway or esophageal compression.
In 1996, Aktogu and colleagues reported superior vena cava syndrome, tracheal compression, pneumothorax, pleurisy, and pneumonia in a series of 30 adult patients.⁴
Bronchogenic cysts should be considered in patients with recurrent pulmonary infections.
Intra-abdominal cysts are rare. As with the mediastinal variety, most are asymptomatic; however, hemorrhage, infection, and compression of adjacent structures can be observed.
The presence of symptoms is important in preoperative assessment because symptomatic patients are more likely to have perioperative difficulties.
In 1995, Ribet and colleagues reported that 70.8% of children were symptomatic because 75% of the cysts were in a critical area around the level of the carina.³ Approximately 60% of adults in this series were symptomatic, and 53% of those mediastinal cysts were at or above the carina.

Physical

Location is more important than cyst volume in its association with symptoms of compression.
Signs of airway compression are more frequently observed in infants and small children than in adults and may include cough, wheeze, dyspnea, and respiratory distress.
Secondary infection of the cyst may occur and may present with fever and respiratory symptoms.
Gastric symptoms, including abdominal pain may be observed. Peptic ulceration may be identified in cysts containing gastric mucosa.
Limaiem et al (2008) reported 33 cases of bronchogenic cysts and identified 4 categories of presentation: asymptomatic (6%); general symptoms including anorexia, weight loss and fever; respiratory symptoms including chest pain, hemoptysis, cough, dyspnea; and other symptoms, including dysphagia and back pain.²

Causes

Cysts are believed to result from abnormal development of the ventral foregut and lung budding during the first trimester.

Differential Diagnoses

Workup

Imaging Studies

Prenatal studies: Bronchogenic cysts are prenatally identified in 70% of cases using ultrasonography. Conventional 2-view chest radiography and barium swallow are often sufficient to support a preoperative diagnosis. Additional clarification may be obtained using chest CT and MRI studies. Ultrafast MRI provides additional anatomic detail. For prenatally identified lesions, chest CT scanning is recommended after birth regardless of the radiography findings at that time.
Conventional 2-view chest radiography
- This is the first diagnostic study indicated in a child with respiratory distress.
- This typically shows a sharply demarcated spherical mass of variable size, most commonly located in the middle mediastinum around the carina.
- When the cyst is infected or contains secretions, it may appear as a solid tumor or may demonstrate an air fluid level.
Esophagography: A barium swallow helps to define the mass and its effect on adjacent structures.
Chest CT scan: Cysts appear as lesions with smooth borders and thin walls and may contain secretions, pus, or blood. Calcifications may also be observed.
CT or MRI
- A homogeneous mass of moderate-to-bright intensity is observed on T2-weighted MRI.
- On T1-weighted images, lesions may vary in their intensity because of their protein content.
- The finding of a cystic lesion at the level of the carina on CT scan or MRI is most frequently associated with a bronchogenic cyst.
- In all other locations, diagnosis cannot be as reliably forecast.

Procedures

Limaiem et al (2008) reported the results of 23 patients with bronchogenic cysts who underwent bronchoscopic evaluation.² They demonstrated evidence of extrinsic compression of the tracheobronchial tree in 4 cases and identified a fistula in 8 cases.

Histologic Findings

Surgical specimens of excised bronchogenic cysts reveal cystic lesions lined by respiratory epithelium.
Occasionally, cysts may contain gastric mucosa or bronchial cartilage.
Differentiation between congenital and acquired cysts may be difficult, if not impossible, in the presence of coexisting infection.
Media file 4 shows the surgical pathology of a cystic lesion. The left side is a low-power view of a bronchogenic cyst showing cartilage and smooth muscle within the wall and cyst lined by ciliated columnar epithelium. The right side is a high-power view (40X) of the same slide, showing ciliated columnar epithelial lining of the cyst.

Treatment

Surgical Care

Surgical resection of all symptomatic bronchogenic cysts is recommended.
In newborns with asymptomatic bronchogenic cysts, surgical intervention is recommended at age 3-6 months, allowing for compensatory lung growth.
Thoracoscopic resection has major advantages that include less pain, better cosmesis, and decreased risk of rib fusion.⁵
Temporizing or palliative procedures such as transparietal, transbronchial, or mediastinal puncture and aspiration may be considered in cases in which patients are symptomatic and the situation for complete resection is not optimal.

Consultations

Preoperative anesthesia consultation is recommended, particularly in infants, because of concern with airway compromise and monitoring.

Medication

Drug therapy is not currently a component of the standard of care for this condition. See Treatment.

Follow-up

Further Outpatient Care

Late recurrences may occur in patients who have undergone partial resection. Long-term follow-up is necessary due to possible recurrence.

Transfer

Maternal transfer: Infants with large prenatally diagnosed bronchogenic cysts should be delivered in a tertiary center with pediatric surgical capabilities. Although they may not require immediate intervention, evaluation and close follow-up are important in the newborn period.
Infant transfer: Symptomatic newborns with cystic lesions should be stabilized and expeditiously transferred to a tertiary center with pediatric surgical capabilities for evaluation and treatment.

Complications

Mass effect
- Compression of the GI tract can result in dysphagia.
- Compression of the airway, especially if cyst is just below the carina, can result in life-threatening respiratory distress.
- Compression of the heart and great vessels can result in dysrhythmias and obstruction of the vena cava.
Cyst-related complications: Infection, rupture, bleeding, and compression are common. A risk of malignant degeneration is also noted.
Other: Other reported complications include airway-cyst fistula, ulceration, and hemorrhage.

Prognosis

In general, postoperative outcomes after resection of bronchogenic cysts are excellent. Thoracoscopic resections are minimally invasive and have advantage of a smaller incision, less post operative pain and better cosmesis.
Poor outcomes have been reported in patients with life-threatening complications preoperatively.
Limaiem et al reported the following perioperative and postoperative complications (no postoperative deaths were reported):²
- Perioperative rupture of the cyst
- Postoperative pneumothorax
- Hemorrhage
- Pleural effusion
- Seizure
No long-term complications have been reported with complete resection.⁶
Late recurrences have been reported with partial excisions.
Left untreated, the most common complications include tracheobronchial compression and pulmonary infection.

Patient Education

Complete surgical excision is recommended as the treatment of choice.
Fatal complications of air embolism in an airline passenger and a fatal myocardial infarction secondary to compression of the left main coronary artery have been reported.
The more common complications of untreated bronchogenic cysts include pulmonary infection and symptoms of tracheobronchial compression.

Miscellaneous

Medicolegal Pitfalls

Failure to diagnose
Failure to anticipate potential for expansion of the cyst or subsequent infection if not resected

Multimedia

Media file 1: Plain chest radiograph showing a left paraspinal mass. Photo courtesy of A Fruauff, MD.

Media file 2: Esophagogram shows an anterior and lateral mass effect in the distal portion of the esophagus corresponding to the paraspinal mass in Media file 1. Photo courtesy of A Fruauff, MD.

Media file 3: Chest CT scan shows an increased soft tissue density in the left paraesophageal area. Photo courtesy of A Fruauff, MD.

Media file 4: The left side shows a low-power view of a bronchogenic cyst showing cartilage and smooth muscle within the wall and the cyst lined by ciliated columnar epithelium. The right side shows a high-power view (40X) of a bronchogenic cyst demonstrating the ciliated columnar epithelial lining. Photo courtesy of A Schuss, MD.

Media file 5: Extrapulmonary bronchogenic cyst. Unilocular cystic mass attached to mediastinal structures by a short pedicle. The inner surface is trabeculated, and the cyst contains mucinous material. The cyst measures 15 cm X 5 cm. Photo courtesy of K Kenigsberg, MD.

References

Shanti CM, Klein MD. Cystic lung disease. Semin Pediatr Surg. Feb 2008;17(1):2-8. [Medline].
Limaïem F, Ayadi-Kaddour A, Djilani H, Kilani T, El Mezni F. Pulmonary and mediastinal bronchogenic cysts: a clinicopathologic study of 33 cases. Lung. Jan-Feb 2008;186(1):55-61. [Medline].
Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J Thorac Cardiovasc Surg. May 1995;109(5):1003-10. [Medline].
Aktogu S, Yuncu G, Halilçolar H, Ermete S, Buduneli T. Bronchogenic cysts: clinicopathological presentation and treatment. Eur Respir J. Oct 1996;9(10):2017-21. [Medline].
Langenburg SE, Lelli JL. Minimally invasive surgery of the lung: lung biopsy, treatment of spontaneous pneumothorax, and pulmonary resection. Semin Pediatr Surg. Feb 2008;17(1):30-3. [Medline].
Laje P, Liechty KW. Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn. Jul 2008;28(7):612-8. [Medline].
Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int. Jun 2008;24(6):643-57. [Medline].
Duwe BV, Sterman DH, Musani AI. Tumors of the mediastinum. Chest. Oct 2005;128(4):2893-909. [Medline]. [Full Text].
Eber E. Antenatal diagnosis of congenital thoracic malformations: early surgery, late surgery, or no surgery?. Semin Respir Crit Care Med. Jun 2007;28(3):355-66. [Medline].
Monrigal JP, Granry JC. Excision of bronchogenic cysts in children using an ultrathin fibreoptic bronchoscope. Can J Anaesth. Jul 1996;43(7):694-6. [Medline].
Philippart A. Benign mediastinal cysts and tumors. In: O'Neil JA, Rowe M, Grosfeld JL, et al, eds. Pediatric Surgery. 5^th ed. St. Louis, MO: Mosby-Year Book Inc; 1998.
Rahmani MR, Filler RM, Shuckett B. Bronchogenic cyst occurring in the antenatal period. J Ultrasound Med. Dec 1995;14(12):971-3. [Medline].
Sarper A, Ayten A, Golbasi I, Demircan A, Isin E. Bronchogenic cyst. Tex Heart Inst J. 2003;30(2):105-8. [Medline].
Takeda S, Miyoshi S, Inoue M, et al. Clinical spectrum of congenital cystic disease of the lung in children. Eur J Cardiothorac Surg. Jan 1999;15(1):11-7. [Medline].
Takeda S, Miyoshi S, Minami M, et al. Clinical spectrum of mediastinal cysts. Chest. Jul 2003;124(1):125-32. [Medline]. [Full Text].

Keywords

bronchogenic cyst, lung bud cyst, subcarinal cysts, mediastinal cyst, dysphagia, respiratory distress, pulmonary cyst, airway compromise, respiratory distress, emphysema, Swyer-James syndrome, asthma, adenocarcinoma, rhabdomyosarcoma, superior vena cava syndrome, tracheal compression, pneumothorax, pleurisy, pneumonia

Contributor Information and Disclosures

Author

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Medical Editor

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center
Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, and Christian Medical & Dental Society
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

Further Reading