Case Study
Esophageal duplication cyst Reza Bagheri1, Amir Mohammad Hashem Asnaashari2 and Reza Afghani1
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(3) 332–334 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314530977 aan.sagepub.com
Abstract Esophageal duplication cyst is a rare congenital mediastinal cyst. Most of these cysts become symptomatic in childhood and only rare cases remain asymptomatic until adolescence. They may produce symptoms due to esophageal and respiratory system compression, rupture, and infection. A 25-year-old man presented with pulmonary infection and bronchiectasis that did not improve with medical treatment. A diagnosis of esophageal duplication cyst was made intraoperatively.
Keywords Duplication cyst, bronchogenic cyst, mediastinal cyst
Introduction Esophageal duplication cyst is a very rare mediastinal cyst that is commonly diagnosed during childhood, and very rare cases remain asymptomatic until adolescence. These cysts result from failure of vacuolization of the primitive esophagus during embryologic development around the fourth to eight gestational week.1 Duplication cysts need intervention and treatment due to potential symptoms. We describe a patient with pulmonary infection and bronchiectasis that did not improve with medical treatment; the diagnosis of esophageal duplication cyst was an intraoperative finding.
Case report The patient was a 25-year-old man who presented with intermittent low grade fever and productive cough. After taking his history and a physical examination, a posteroanterior chest radiograph was obtained (Figure 1). Medical treatment was started, but his symptoms did not resolve. Thoracic computed tomography revealed a consolidation in the right hemithorax with fullness of the right hilum (Figure 2). Fiberoptic bronchoscopy showed only a mucopurulent secretion in the right main bronchus. The patient underwent surgery through a right posterolateral thoracotomy. After opening the right thoracic cavity, a bronchiectatic
segment of the right lower lobe was found to be accompanied by a cystic lesion just adhered to the esophagus wall (Figure 3). The cyst had fistulized to the right main bronchus, and after resection, it was found to be full of purulent secretions. The bronchiectatic segment was resected and the bronchus repaired. After homeostasis, aerostasis, and insertion of 2 chest tubes, we closed the thorax. The pathologic finding was esophageal duplication cyst (Figure 4).
Discussion Esophageal cysts are the third most common esophageal mass after leiomyoma and benign polyps, although they are less rare than bronchogenic cysts. Among all mediastinal cysts, 5%–14% are of esophageal origin. They are more common in the lower one third of the esophagus, and the male to female ratio is 2:1.2 1 Cardiothoracic Surgery & Transplant Research Center, Emam Reza Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 2 COPD Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
Corresponding author: Reza Afghani, Cardiothoracic Surgery & Transplant Research Center, Emam Reza Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. Email: [email protected]
Downloaded from aan.sagepub.com at University of Ulster Library on April 17, 2015
Bagheri et al.
333
Figure 1. Posterolateral chest radiography on admission.
Figure 3. The specimen after resection.
Figure 2. Conventional computed tomography scan in mediastinal view.
Failure of vacuolization of the primitive esophagus during embryologic development around the 4th to 8th gestational week results in formation of an esophageal cyst. These cysts may be adherent to the esophagus or intramural. The cyst is covered with nonkeratinizing squamous or ciliated columnar epithelium, and the cyst wall contains two layers of smooth muscle. These findings, accompanied by a lack of cartilage in the cyst wall, can differentiate an esophageal duplication cyst from a bronchogenic cyst.1,2 However, differentiation of these two cysts has no practical significance because they have similar features and require the same treatment. Up to 80% of these cysts are diagnosed in childhood, and the majority become symptomatic; less than 7% remain asymptomatic until adolescence.1 Pain and dysphagia are the most common symptoms in adults, while respiratory symptoms due to compression are more obvious in children. Hemorrhage into the cyst may occur. The cyst may rupture into the pleura or
Figure 4. Pathological findings of the resected specimen, showing esophageal squamous epithelium covered by a smooth muscle layer. Hematoxylin and eosin stain, original magnification 100.
tracheobronchial tree and cause symptoms. Progression to malignancy has been reported.2–4 Diagnosis is usually made by computed tomography and endoscopic ultrasonography.4 Endoscopic ultrasound examination can help in the differentiation of solid from cystic lesions, and can also delineate which layer of the esophagus is involved.2,5 In barium swallow, duplication cyst is seen as a mass with a pressure effect on the esophagus; but with esophagoscopy, we cannot differentiate duplication cyst from liomyoma.6 Because of
Downloaded from aan.sagepub.com at University of Ulster Library on April 17, 2015
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Asian Cardiovascular & Thoracic Annals 23(3)
ectopic epithelium of the stomach, a Tc-99 m scan is positive in 50% of cases.2,3 There are some reports recommending follow-up in asymptomatic cysts.4 Overall, observation is specially suggested for patients at high risk for surgery. In symptomatic cases, complete resection is considered.2,7 Resection can be performed through open thoracotomy or video-assisted thoracoscopic surgery.1,3,4 The surgical approach is similar to that for bronchogenic cysts. Usually, there is no communication between the cyst and the wall of esophagus, so care should be taken not to open the esophageal mucosa. Partial resection of the cyst can be performed in difficult cases.6 Biopsy and aspiration of the cyst is not recommended because of the potential for complications and cyst infection.2 Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Pisello F, Geraci G, Arnone E, Sciutto A, Modica G and Sciume C. Acute onset of esophageal duplication cyst in adult. Case report. G Chir 2009; 30: 17–20. 2. Patterson GA, Cooper JD, Deslauriers JD, Lerut AEMR, Luketich JD, Rice TW. Pearson’s Thoracic & Esophageal Surgery: Mediastinal Cysts and Duplications in Adults. 3rd ed. 2008:1584–1585. 3. Takemura M, Yoshida K and Morimura K. Thoracoscopic resection of thoracic esophageal duplication cyst containing ectopic pancreatic tissue in adult. J Cardiothorac Surg 2011; 6: 118. 4. Wiechowska-Kozlowska A, Wunsch E, Majewski M and Milkiewicz P. Esophageal duplication cysts: endosonographic findings in asymptomatic patients. World J Gastroenterol 2012; 18: 1270–1272. 5. Bhutani MS, Hoffman BJ and Reed C. Endosonographic diagnosis of an esophageal duplication cyst. Endoscopy 1996; 28: 396–397. 6. Shields TW, locicero III J, Reed CE, Feins RH. General Thoracic Surgery: Foregut Cysts of the Mediastinum. 7th ed. 2009;2527–2528. 7. Nobuhara KK, Gorski YC, La Quaglia MP and Shamberger RC. Bronchogenic cysts and esophageal duplications: common origins and treatment. J Pediatr Surg 1997; 32: 1408–1413.
Downloaded from aan.sagepub.com at University of Ulster Library on April 17, 2015
Esophageal duplication cyst Reza Bagheri1, Amir Mohammad Hashem Asnaashari2 and Reza Afghani1
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(3) 332–334 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314530977 aan.sagepub.com
Abstract Esophageal duplication cyst is a rare congenital mediastinal cyst. Most of these cysts become symptomatic in childhood and only rare cases remain asymptomatic until adolescence. They may produce symptoms due to esophageal and respiratory system compression, rupture, and infection. A 25-year-old man presented with pulmonary infection and bronchiectasis that did not improve with medical treatment. A diagnosis of esophageal duplication cyst was made intraoperatively.
Keywords Duplication cyst, bronchogenic cyst, mediastinal cyst
Introduction Esophageal duplication cyst is a very rare mediastinal cyst that is commonly diagnosed during childhood, and very rare cases remain asymptomatic until adolescence. These cysts result from failure of vacuolization of the primitive esophagus during embryologic development around the fourth to eight gestational week.1 Duplication cysts need intervention and treatment due to potential symptoms. We describe a patient with pulmonary infection and bronchiectasis that did not improve with medical treatment; the diagnosis of esophageal duplication cyst was an intraoperative finding.
Case report The patient was a 25-year-old man who presented with intermittent low grade fever and productive cough. After taking his history and a physical examination, a posteroanterior chest radiograph was obtained (Figure 1). Medical treatment was started, but his symptoms did not resolve. Thoracic computed tomography revealed a consolidation in the right hemithorax with fullness of the right hilum (Figure 2). Fiberoptic bronchoscopy showed only a mucopurulent secretion in the right main bronchus. The patient underwent surgery through a right posterolateral thoracotomy. After opening the right thoracic cavity, a bronchiectatic
segment of the right lower lobe was found to be accompanied by a cystic lesion just adhered to the esophagus wall (Figure 3). The cyst had fistulized to the right main bronchus, and after resection, it was found to be full of purulent secretions. The bronchiectatic segment was resected and the bronchus repaired. After homeostasis, aerostasis, and insertion of 2 chest tubes, we closed the thorax. The pathologic finding was esophageal duplication cyst (Figure 4).
Discussion Esophageal cysts are the third most common esophageal mass after leiomyoma and benign polyps, although they are less rare than bronchogenic cysts. Among all mediastinal cysts, 5%–14% are of esophageal origin. They are more common in the lower one third of the esophagus, and the male to female ratio is 2:1.2 1 Cardiothoracic Surgery & Transplant Research Center, Emam Reza Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 2 COPD Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
Corresponding author: Reza Afghani, Cardiothoracic Surgery & Transplant Research Center, Emam Reza Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. Email: [email protected]
Downloaded from aan.sagepub.com at University of Ulster Library on April 17, 2015
Bagheri et al.
333
Figure 1. Posterolateral chest radiography on admission.
Figure 3. The specimen after resection.
Figure 2. Conventional computed tomography scan in mediastinal view.
Failure of vacuolization of the primitive esophagus during embryologic development around the 4th to 8th gestational week results in formation of an esophageal cyst. These cysts may be adherent to the esophagus or intramural. The cyst is covered with nonkeratinizing squamous or ciliated columnar epithelium, and the cyst wall contains two layers of smooth muscle. These findings, accompanied by a lack of cartilage in the cyst wall, can differentiate an esophageal duplication cyst from a bronchogenic cyst.1,2 However, differentiation of these two cysts has no practical significance because they have similar features and require the same treatment. Up to 80% of these cysts are diagnosed in childhood, and the majority become symptomatic; less than 7% remain asymptomatic until adolescence.1 Pain and dysphagia are the most common symptoms in adults, while respiratory symptoms due to compression are more obvious in children. Hemorrhage into the cyst may occur. The cyst may rupture into the pleura or
Figure 4. Pathological findings of the resected specimen, showing esophageal squamous epithelium covered by a smooth muscle layer. Hematoxylin and eosin stain, original magnification 100.
tracheobronchial tree and cause symptoms. Progression to malignancy has been reported.2–4 Diagnosis is usually made by computed tomography and endoscopic ultrasonography.4 Endoscopic ultrasound examination can help in the differentiation of solid from cystic lesions, and can also delineate which layer of the esophagus is involved.2,5 In barium swallow, duplication cyst is seen as a mass with a pressure effect on the esophagus; but with esophagoscopy, we cannot differentiate duplication cyst from liomyoma.6 Because of
Downloaded from aan.sagepub.com at University of Ulster Library on April 17, 2015
334
Asian Cardiovascular & Thoracic Annals 23(3)
ectopic epithelium of the stomach, a Tc-99 m scan is positive in 50% of cases.2,3 There are some reports recommending follow-up in asymptomatic cysts.4 Overall, observation is specially suggested for patients at high risk for surgery. In symptomatic cases, complete resection is considered.2,7 Resection can be performed through open thoracotomy or video-assisted thoracoscopic surgery.1,3,4 The surgical approach is similar to that for bronchogenic cysts. Usually, there is no communication between the cyst and the wall of esophagus, so care should be taken not to open the esophageal mucosa. Partial resection of the cyst can be performed in difficult cases.6 Biopsy and aspiration of the cyst is not recommended because of the potential for complications and cyst infection.2 Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Pisello F, Geraci G, Arnone E, Sciutto A, Modica G and Sciume C. Acute onset of esophageal duplication cyst in adult. Case report. G Chir 2009; 30: 17–20. 2. Patterson GA, Cooper JD, Deslauriers JD, Lerut AEMR, Luketich JD, Rice TW. Pearson’s Thoracic & Esophageal Surgery: Mediastinal Cysts and Duplications in Adults. 3rd ed. 2008:1584–1585. 3. Takemura M, Yoshida K and Morimura K. Thoracoscopic resection of thoracic esophageal duplication cyst containing ectopic pancreatic tissue in adult. J Cardiothorac Surg 2011; 6: 118. 4. Wiechowska-Kozlowska A, Wunsch E, Majewski M and Milkiewicz P. Esophageal duplication cysts: endosonographic findings in asymptomatic patients. World J Gastroenterol 2012; 18: 1270–1272. 5. Bhutani MS, Hoffman BJ and Reed C. Endosonographic diagnosis of an esophageal duplication cyst. Endoscopy 1996; 28: 396–397. 6. Shields TW, locicero III J, Reed CE, Feins RH. General Thoracic Surgery: Foregut Cysts of the Mediastinum. 7th ed. 2009;2527–2528. 7. Nobuhara KK, Gorski YC, La Quaglia MP and Shamberger RC. Bronchogenic cysts and esophageal duplications: common origins and treatment. J Pediatr Surg 1997; 32: 1408–1413.
Downloaded from aan.sagepub.com at University of Ulster Library on April 17, 2015
