Bronchogenic cyst of the interatrial septum
© Jiang et al.; licensee BioMed Central Ltd. 2013
Received: 10 August 2012
Accepted: 3 July 2013
Published: 9 July 2013
Bronchogenic cyst is considered as an uncommon congenital anomaly. It can be mostly found in mediastinum or lung. Intracardiac bronchogenic cyst is very rare. We found 2 cases in more than 20000 cardiac surgical cases in our department. The 2 cases bronchogenic cyst arose from interatrial septum (IAS), the preoperative diagnosis were myxoma, but the histological diagnosis were bronchogenic cyst in both cases. Although it is very rare, it should be considered while intracardiac tumor is diagnosed. It is recommended to complete resection of any bronchogenic cyst for primarily diagnostic and potentially therapeutic reasons, and every effort should be made to prevent complications due to injury to nearby tissues.
KeywordsCongenital anomaly Bronchogenic cyst Interatrial septum Diagnosis Therapy
Bronchogenic cyst is considered as an uncommon congenital anomaly. It can be mostly found in mediastinum or lung. Intracardiac bronchogenic cyst is very rare. We found 2 cases in more than 20000 cardiac surgical cases in our department. The 2 cases bronchogenic cyst arose from interatrial septum (IAS), the preoperative diagnosis were myxoma, but the histological diagnosis were bronchogenic cyst in both cases. It is always misdiagnosed as the other intracardiac tumor.
Postoperative course was uneventful, the patient was discharged 10 days post-operation. Echocardiography revealed no recurrence during the 3 years follow-up.
The location of the bronchogenic cyst can be explained by embryogenesis. Bronchogenic cysts are believed to represent a localized portion of the tracheobronchial tree that becomes separated from normal airways during the branching process and does not undergo further development. Most probably develop between the 26th and the 40th day of intrauterine life, during the most active period of airway development. Cardiac primordia exist in a place very near to the foregut or primitive tracheobronchial tree. At this time, abnormal budding of the tracheobronchial tree may migrate to a myocardial location; bronchogenic cysts arise from such budding. Thus, cysts are usually at the pericardium, and it is very rare that tumors are in the interatrial septum [1, 2].
Intracardiac bronchogenic cysts are rare and are located on the epicardial surface or within the myocardium projecting into one of the cardiac cavities . In the only review dealing with a larger number of intracardiac bronchogenic cysts, the majority were found in the right side of the heart, and only a few were located in the left side or their localization was not specified [3, 4]. The location of the cyst in the two cases we reported was in the IAS, one protruding into the right atrium, the other projecting into the left atrium.
A bronchogenic cyst is a benign tumor. Many cases are asymptomatic with the lesions incidentally detected. Symptoms of intracardiac bronchogenic cysts such as chest pain, shortness of breath and arrhythmias can vary according to the location of the cyst, its size and compression of heart and vessels [1, 5].
The tumor of the 1st case we presented was larger than the ASD, the symptoms may come from the occlusion by the mass. But the ASD was bigger than the cyst in the 2nd case, the symptoms may due to the ASD.
Most intracardiac bronchogenic cysts were found by transthoracic and transesophageal echocardiography, the diagnosis was made by contrast-enhanced chest helical CT and cardiac MRI and later was approved intraoperatively [1, 6].
While considering an intracardiac tumor, myxoma, papillary fibroelastoma, thrombus, metastasis, etc. is usually considered. For a cystic tumor in the cardiac chamber, bronchogenic cyst should be in the differential diagnosis list [3, 6]. The preoperative diagnosis of the two cases we reported were myxomas because bronchogenic cyst is rarely seen.
Bronchogenic cyst is a benign tumor, but definitive diagnosis should be made by pathology study. It is not possible to perform biopsy in the cardiac chamber because pulmonary or cerebral embolism may be resulted from the fluid contained. Bronchogenic cysts may develop into malignancy [7–9]. St-Georges et al. recommended all presumed bronchogenic cysts seen in the adult be resected because the majority will ultimately become symptomatic or complicated .
Although bronchogenic cyst of interatrial septum is very rare, it should be considered while intracardiac tumor is diagnosed. It is recommended to complete resection of any bronchogenic cyst for primarily diagnostic and potentially therapeutic reasons, and every effort should be made to prevent complications due to injury to nearby tissues.
Written informed consent was obtained from the patient for the publication of this report and any accompanying images.
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