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Bronchogenic Cysts: A Diagnostic Dilemma in Adults

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posted on 2021-02-26, 22:30 authored by Taylor Garza, Melissa Taylor, Gal Levy

These foregut-derived congenital malformations are usually discovered prenatally on ultrasound and can be seen at any point along the tracheobronchial tree. So you might be thinking to yourself, what’s the dilemma? Well, when it comes to adults, bronchogenic cysts can be seen throughout the chest and abdomen, but, in general, they rarely occur and even more rarely occur in the abdomen. Adding to this, very few cases have been recorded in literature and most cysts are found incidentally on radiographs because patients tend to present with nonspecific symptoms. The objective of this video is to walk through a case report and briefly discuss facts regarding bronchogenic cysts.

A 60-year-old female was referred to the authors’ clinic for evaluation of an esophageal mass. She had initially been evaluated for epigastric pain with radiation to her back, during which a chest CT displayed a well-circumscribed 76 mm nonenhancing solid lobular mass with scattered internal calcifications abutting the left hemidiaphragm and indiscernible from the gastric fundus. Further workup with an endoscopic ultrasound revealed an intramural subepithelial lesion in the fundus, concerning for a stromal lesion. A biopsy was then conducted and revealed mucinous debris.

This was followed by an MRI, which displayed a lobulated T2 hyperintense and T1 hypointense mass seen between the posterior aspect of the gastric fundus and left diaphragmatic crus with benign features consistent with esophageal duplication cyst.

An upper GI study showed a noncommunicating cyst presumed to be a gastric duplication cyst. No gastroesophageal reflux was noted and there was normal emptying. Bravo pH tests were normal with DeMeeseter scores of 0.3. Manometry studies were also normal with a DCI value of 2774 (mmHg-cm-s) and IRP value of 13.8. On EGD, the submucosal lesion was seen at the fundus on retroflexion without secondary inflammation. On laparoscopy, the mass was well circumscribed along the fundus and retrogastric space. An endoscopic ultrasound image-guided fine needle aspiration showed mixed bacteria and mucinous debris, but no malignant cells were identified.

Using an energy device, the pars flaccida was divided, preserving the left hepatic artery. The left crus was thickened and inflamed. There was a clear plane between the mass and the stomach. The tissue was further dissected to access the cyst, which was nestled on top of the hiatus of the stomach. During dissection, the cyst was opened, and thick white debris was aspirated, allowing the cyst to be more easily mobilized. The origin of the cyst appeared to be at the angle of His. The base of the cyst was transected with a robotic stapling device. Completion of endoscopy confirmed no injury to the gastric wall or esophagus.

A final pathology report described a cyst lined with respiratory type epithelium with underlying smooth muscle and mature cartilage consistent with a benign bronchogenic cyst. No malignancy was identified.

How do these cysts originate? As the primitive foregut divides into a dorsal portion (forming the esophagus) and a ventral portion (forming the tracheobronchial tree) in the embryo, a spectrum of abnormalities can arise. Notably, bronchogenic cysts are thought to be ventral foregut-derived errors that arise from epithelial cells budding off the tracheobronchial tree. As the diaphragm develops, these lesions may arise above the diaphragm (most commonly in the mediastinum) or migrate below (most commonly retrogastric). Most of these cysts are located near the trachea and attached by a stalk. Rarely, they may have a patent communication which is complicated by infection.

In order to be classified as a true bronchogenic cyst, there needs to be ciliated epithelium present along with either bronchial mucous glands or cartilage. However, these criteria can make it difficult to differentiate bronchogenic cysts from esophageal duplication cysts, which are composed of ciliated or non-ciliated epithelium between two layers of smooth muscle without mucous glands or cartilage. As previously stated, most patients with bronchogenic cysts present with nonspecific symptoms ranging from coughing or wheezing to flank or abdominal pain, thus making it difficult to narrow down a differential. While the risk of malignancy associated with bronchogenic cysts is low, most cases require surgical resection to prevent any future complications. This can be easily accomplished with laparoscopic excision as the preferred method of choice due to its minimally invasive advantages. Overall, bronchogenic cysts rarely occur and, when they do, can pose a diagnostic challenge in adults.

References

Ubukata, H., Satani T, Motohashi G, Konishi S, Goto Y, Watanabe Y, et al. Intra-abdominal bronchogenic cyst with gastric attachment: Report of a case. Surg Today. 2011;41:1095-1100.
Liang, M., Yee H, Song J, Marks J. Subdiaphragmatic bronchogenic cysts: A comprehensive review of the literature. Am Surg. 2005;71(12):1034- 1041.
Inaba K., Sakurai Y, Umeki Y, Kanaya S, Komori Y, Uyama I. Laparoscopic excision of subdiaphragmatic bronchogenic cyst occurring in the retroperitoneum: Report of a case. Surg Laparosc Endosc Percutan Tech. 2010; 20(6): e199-e203.
Oermann, C. (2020). Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula. UpToDate. Retrieved July 6, 2020, from https://www-uptodate-com.libux.utmb.edu/contents/congenital-anomalies-of-the-intrathoracic-airways-and-tracheoesophageal-fistula