Pediatric Bronchogenic Cyst 

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

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.

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.

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]

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]

 

Presentation

History

With the advent of improved antenatal diagnosis, many infants are identified prior to the development of symptoms.

  • Chest pain and dysphagia are the most common symptoms in adults with bronchogenic cysts.

  • Recurrent infections may be the clinical presentation in some children

  • In infants, symptoms are most often produced as a result of airway or esophageal compression.

  • Superior vena cava syndrome, tracheal compression, pneumothorax, pleurisy, and pneumonia were reported in a series of 30 adult patients.[9]

  • 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.[6] Approximately 60% of adults in this series were symptomatic, and 53% of those mediastinal cysts were at or above the carina.

  • 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, and dyspnea; and other symptoms including dysphagia and back pain.[5]

  • Jiang et al (2015) reported on 16 pediatric cases of bronchogenic cyst. Of those with palpable lesions in the neck or on the skin, only one was symptomatic (mild stridor). In the remaining eight cases, seven were located in the mediastinum and one was pulmonary; 75% of these children were symptomatic. These findings were in contrast to the 23 adults studied where 56% of the overall adult group were asymptomatic.[10]

Physical

See the list below:

  • 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.

Causes

See the list below:

  • Cysts are believed to result from abnormal development of the ventral foregut and lung budding during the first 4-6 weeks of gestation.

Complications

Airway compromise

Infection

 

 

DDx

Diagnostic Considerations

Imaging studies help to develop your differential diagnosis.

Fetal ultrasound may be the first indication that a cystic lesion is present. Chest films can provide a presumptive diagnosis based on the location of the lesion.

Contrast medium enhanced CT can define calcifications and is generally considered superior to MRI, unless there are concerns about additional anomalies.

 

Workup

Imaging Studies

See the list below:

  • Prenatal studies: Bronchogenic cysts are prenatally identified in 70% of cases using high-resolution ultrasonography. Conventional 2-view chest radiography and barium swallow are often sufficient to support a preoperative diagnosis. However, contrast enhanced CT has largely replaced the esophagogram and can provide information about calcification.

  •   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, as shown below.

      Plain chest radiograph showing a left paraspinal m Plain chest radiograph showing a left paraspinal mass. Photo courtesy of A Fruauff, MD.
    • 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 (see the image below).

    Esophagogram shows an anterior and lateral mass ef Esophagogram shows an anterior and lateral mass effect in the distal portion of the esophagus corresponding to the paraspinal mass (see the radiographic image above). Photo courtesy of A Fruauff, MD.
  • 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.

    • A study of preoperative MDCT concluded that axial MDCT images accurately diagnose the types, location, associated mass effect, and anomalous arteries of congenital lung anomalies.[11, 12]

     

Procedures

See the list below:

  • Limaiem et al (2008) reported the results of 23 patients with bronchogenic cysts who underwent bronchoscopic evaluation.[5] They demonstrated evidence of extrinsic compression of the tracheobronchial tree in 4 cases and identified a fistula in 8 cases.

Histologic Findings

See the list below:

  • 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.

  • The image below 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.

    The left side shows a low-power view of a bronchog 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.
 

Treatment

Surgical Care

See the list below:

  • 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. A publication by Polites et al demonstrated that the use of thoracoscopic resection for congenital cystic lung disease is on the rise. The authors did not find any difference in postoperative length of stay nor in complication rates once the data had been adjusted for patient complexity and the amount of resection performed.[13]

  • 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.

  • A study by Jung et al evaluated the feasibility and safety of video-assisted thoracic surgery in the management of bronchogenic cysts and reported that video-assisted thoracic surgery was safe and effective.[14]

 

Consultations

Preoperative anesthesia consultation is recommended, particularly in infants, because of concern with airway compromise and monitoring. Other members of the multidisciplinary teams caring form infants and children with bronchogenic cysts may include the following:

  • Pediatric surgeon

  • Pediatric anesthesiologist

  • Pediatric intensivist

  • Pediatric gastroenterologist and nutritionist

 

Medication

Medication Summary

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

 

Follow-up

Further Outpatient Care

See the list below:

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

  • Long term follow-up is recommended due to the potential risk of malignancy.

Transfer

See the list below:

  • 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

See the list below:

  • 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)[5] :

  • Perioperative rupture of the cyst

  • Postoperative pneumothorax

  • Hemorrhage

  • Pleural effusion

  • Seizure

No long-term complications have been reported with complete resection.[7]

Late recurrences have been reported with partial excisions.

Left untreated, the most common complications include tracheobronchial compression and pulmonary infection.

Patient Education

See the list below:

  • 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.

  • Long term follow-up is recommended.