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.
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| References |
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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. 5th 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].
Further Reading
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
