Pediatric Bronchogenic Cyst

Updated: Jan 18, 2017
  • Author: Mary E Cataletto, MD; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
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Overview

Background

Bronchogenic cysts, although relatively rare, represent the most common cystic lesion of the mediastinum. [1] Shanti and Klein (2008) studied a series of 236 patients undergoing pulmonary resection for cystic lung lesions. [2] 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. Today most bronchogenic cysts are diagnosed antenatally.

See chest radiograph below illustrating a bronchogenic cyst.

Plain chest radiograph showing a left paraspinal m Plain chest radiograph showing a left paraspinal mass. Photo courtesy of A Fruauff, MD.

In infants and small children, bronchogenic cysts, especially those that compress vital structures, can present with respiratory distress. In particular, subcarinal cysts can pose life-threatening airway compromise. Symptomatic older children may present with recurrent infection. Despite this many, infants and children are asymptomatic and the cystic lesion is found incidentally during routine chest radiography and during evaluations for GI or cardiac symptomatology. [3] Improvements in fetal ultrasonography have provided prenatal identification of many cystic lung lesions, with early diagnosis and surgical intervention. Error rates vary among studies.

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, as depicted below. 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.

Extrapulmonary bronchogenic cyst. Unilocular cysti 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.
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Pathophysiology

Bronchogenic cysts occur within the spectrum of foregut duplication cysts and arise from abnormal ventral budding of the tracheobronchial tree. [4] Infants may present with respiratory distress secondary to compression of adjacent airway structures. Recurrent infection (of the cyst) may be the presenting complaint in older children.

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Epidemiology

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. [5]

Mortality/Morbidity

Ribet et al's 1995 series reported a postoperative morbidity of 13.4%. [6] This series included 45 adult and 24 pediatric cases over a 25-year period at the University of Lille in France.

Sex

Bronchogenic cysts are more common in males. [2]

Age

Antenatal diagnosis is made in over 60% of cases. [7]

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Prognosis

Casagrande and Pederiiva conducted a systematic review of the association of congenital lung malformations and malignant lung tumors in both children and adults. In the adults identified with congenital pulmonary malformations, bronchogenic cysts were most frequently associated with malignant lung tumors. Based on their findings they highlight that all congenital pulmonary malformations need follow-up. No age dependent risk was identified for malignant progression nor did they identify a time limit for progression from diagnosis of the congenital lesion to the malignant one. [8]

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