Esophagectomy for a Rapidly Progressing Esophageal Duplication Cyst Dong Wang, MD, Li-cheng Du, MD, Qiang-xiu Wang, MD, and Zhou Wang, MD Departments of Thoracic Surgery, General Surgery, and Pathology, Shandong Provincial Hospital affiliated to Shandong University, Jinan, China
Esophageal duplication cysts are rare and frequently asymptomatic anomalies of the adult gastrointestinal tract that are often misdiagnosed in clinical practice. Identifying the unique features of esophageal duplication cysts is therefore important. We report a unique case of esophageal duplication cyst in a 52-year-old woman with rapidly progressing chest pain and dysphagia. The cyst was found to share, in part, a remarkably inflammatory and edematous inner lining with the esophagus. Enucleation was not feasible, and therefore, esophagectomy was performed. The only long-term side effect that occurred after 44 months of follow-up examinations was slight acid reflux esophagitis. (Ann Thorac Surg 2015;99:e79–81) Ó 2015 by The Society of Thoracic Surgeons
fever, was evaluated in the Outpatient Department of the Shandong Provincial Hospital affiliated to Shandong University in September 2010. The patient reported that the chest pain began 2 weeks before the evaluation and that the condition became aggravated along with the development of dysphagia. The dysphagia progressed quickly and she could hardly swallow liquid food 10 days after the symptom appeared. The initial evaluation did not identify the cause of the chest pain or dysphagia, and the patient was referred to the Department of Thoracic Surgery for a detailed examination. A roentgenogram barium swallow showed a half-round filling defect in the lower part of the esophagus that resulted in the near blockage of barium passage (Fig 1A). A chest computed tomography (CT) scan revealed a large low-density to high-density paraesophageal mass (Figs 1B and 1C). No small intestinal duplication, esophageal atresia, tracheoesophageal fistula, or spinal abnormality was found, nor was their evidence of infection, bleeding, perforation, or malignancy. We performed a left thoracotomy, using a standard posterolateral incision. The paraesophageal mass was a 10- 6- 4-cm3 cyst attached with high tension to the
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sophageal duplication cysts (EDCs) are infrequent gastrointestinal tract anomalies principally found in children, with a male predominance. EDCs are rarely seen in adults because up to 80% of EDCs are diagnosed in childhood and removed, and less than 7% of EDCs remain asymptomatic until adolescence [1, 2]. EDCs result from vacuolization failure of the primitive esophagus around the fourth to eighth gestational week during embryonic development [1]. EDCs have been associated with other congenital anomalies such as small intestinal duplication, esophageal atresia, tracheoesophageal fistula, and spinal abnormalities [3]. Asymptomatic EDCs are difficult to diagnose in adults, and even cases with clinical symptoms are often mistaken for other more common esophageal conditions because of the nonspecific nature of EDC symptoms [2]. Owing to potential complications associated with an untreated EDC, including bleeding, infection, or conversion to a malignancy, the most common treatment for EDCs is surgical excision even if patients are not symptomatic [1, 2]. Thus, diagnosis of EDCs is important. Here we reported a unique adult case of EDC with rapidly progressing chest pain and dysphagia that was ultimately treated with esophagectomy instead of enucleation. A 52-year-old woman with chest pain and dysphagia, but no history of palpitation, cough, dyspnea, weight loss or
Accepted for publication Jan 13, 2015. Address correspondence to Dr Zhou Wang, Department of Thoracic Surgery, Shandong Provincial Hospital affiliated to Shandong University, Jinan 250021, China; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Fig 1. The esophageal duplication cyst (arrows) is seen on a (A) barium esophagogram and on (B) axial and (C) coronal computed tomography images before the operation. (D) Barium esophagogram after the operation. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.01.047
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CASE REPORT WANG ET AL ESOPHAGECTOMY FOR A RAPIDLY PROGRESSING EDC
Ann Thorac Surg 2015;99:e79–81
Fig 2. (A) Hematoxylin and eosin staining of the cystic mass lesion with hypertrophic smooth muscles in the wall (original magnification 100). (B) Hematoxylin and eosin staining of stratified squamous epithelium lining the cyst (original magnification 400).
lower thoracic esophagus. The cyst extended medially to the esophagus and shared a common muscular coat with the esophagus. We initially tried to dissect the cyst from the inferior pole but soon realized that its adhesion to the esophagus was extremely intimate. Therefore, we exposed the esophageal lumen and found that the cyst shared an extremely edematous inner lining with the esophagus, whereas the mucosal lining was separate. In consideration of the risk of fistula and recurrence, we removed the involved esophageal region and performed a gastroesophageal anastomosis below the aortic arch. After removal, we observed that the cyst contained old clots and brown purulent materials. Pathologic examination confirmed that the mass was an EDC with squamous epithelium (Fig 2). The patient’s postoperative recovery was uneventful, and repeated barium examinations did not show any esophageal abnormalities (Fig 1D). The only long-term postoperative symptom the patient was observed to experience after 44 months of follow-up examinations was slight acid reflux esophagitis, which was resolved with ranitidine (40 mg/d).
Comment EDCs occur most frequently in children, occur more frequently in males than in females, and are the second most common duplication of the alimentary tract, after the ileum, comprising approximately 20% of the cases [2]. Dysphagia, epigastric discomfort, and retrosternal pain are the most common symptoms of EDCs [3]. In the current case, the patient reported the rapid development of pain and subsequent dysphagia. We believe these symptoms were caused by the rapid growth of the EDC [1, 2]. The diagnosis of EDCs, usually performed by CT and endoscopic ultrasonography [1], is challenging. EDCs usually appear as homogenous, round or oval and sharply marginated masses in the posteroinferior mediastinum. A chest roentgenogram may appear completely normal in some patients who have small-sized and midline EDCs. In this patient, a chest CT scan revealed a smooth, cystic, and well-defined low-density to highdensity mediastinal mass without effective enhancement. Other imaging modalities commonly used to diagnose EDCs include magnetic resonance imaging (MRI) and transesophageal endoscopic ultrasonography (EUS). MRI
usually shows the thin walls and the fluid contents of the lesion, giving a relatively specific diagnosis [4]. EUS is considered the best radiologic diagnostic modality for EDCs. EUS can distinguish EDCs from other mediastinal masses by demonstrating the relationship between the muscularis of the esophagus and the EDC muscle layer [5]. However, because of the patient’s quickly escalating chest pain and dysphagia, the operation was performed without EUS. Surgical examination and histopathology of the resected lesion are required to differentiate EDCs definitively from other mediastinal cysts. Thus, even with strong imaging evidence, a definite diagnosis of EDCs can only be established by pathologic evaluation after surgical resection. Symptoms and complications occur in approximately 70% of patients with untreated asymptomatic EDCs [5]. Once symptoms develop, surgical resection may become more complicated. Although en bloc resection of EDCs is generally advocated for patients with symptomatic or asymptomatic EDCs, open surgical resection through a posterolateral thoracotomy is the conventional approach because EDCs may extend to the opposite side through the prevertebral space [4]. Enucleation has been widely advocated but may not be suitable in many cases. In recent years, video-assisted thoracoscopic surgery has been increasingly applied in EDC removal. Compared with the conventional approach, thoracoscopic surgery reduces postoperative pain and results in better cosmetic outcomes [6]. The cyst in this patient partially shared the inner lining of the esophagus. The shared lining was remarkably inflammatory and hydropic, and thus, we determined that enucleation was not feasible. We therefore performed an esophagectomy to remove the cyst and the associated region of the esophagus. The operation successfully resolved the patient’s chest pain and dysphagia. After 44 months of follow-up, the only notable long-term side effect was a slight acid reflux esophagitis, which was successfully treated with ranitidine. Our findings indicate that esophagectomy was an efficacious and safe treatment strategy for the EDC in this patient. Nevertheless, we will continue to monitor this patient to observe whether other potential postoperative complications, including esophageal fistula, pyothorax, esophageal stricture, and diverticulum, occur in this patient.
Ann Thorac Surg 2015;99:e79–81
This work was supported by Natural Science Foundation of China (N0.81172161).
References 1. Bagheri R, Asnaashari AM, Afghani R. Esophageal duplication cyst. Asian Cardiovasc Thorac Ann 2015;23:332–4. 2. Castelijns PS, Woensdregt K, Hoevenaars B, et al. Intraabdominal esophageal duplication cyst: a case report and review of the literature. World J Gastrointest Surg 2014;6:112–6.
CASE REPORT WANG ET AL ESOPHAGECTOMY FOR A RAPIDLY PROGRESSING EDC
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3. Obasi PC, Hebra A, Varela JC. Excision of esophageal duplication cysts with robotic-assisted thoracoscopic surgery. JSLS 2011;15:244–7. 4. McMaster WG Jr, Mukherjee K, Parikh AA. Surgical management of a symptomatic foregut duplication cyst. Am Surg 2012;78:E306–7. 5. Zhang Z, Jin F, Wu H, et al. Double esophageal duplication cysts, with ectopic gastric mucosa: a case report. J Cardiothorac Surg 2013;8:221. 6. Takemura M, Yoshida K, Morimura K. Thoracoscopic resection of thoracic esophageal duplication cyst containing ectopic pancreatic tissue in adult. J Cardiothorac Surg 2011;6:118.
Esophageal duplication cysts are rare and frequently asymptomatic anomalies of the adult gastrointestinal tract that are often misdiagnosed in clinical practice. Identifying the unique features of esophageal duplication cysts is therefore important. We report a unique case of esophageal duplication cyst in a 52-year-old woman with rapidly progressing chest pain and dysphagia. The cyst was found to share, in part, a remarkably inflammatory and edematous inner lining with the esophagus. Enucleation was not feasible, and therefore, esophagectomy was performed. The only long-term side effect that occurred after 44 months of follow-up examinations was slight acid reflux esophagitis. (Ann Thorac Surg 2015;99:e79–81) Ó 2015 by The Society of Thoracic Surgeons
fever, was evaluated in the Outpatient Department of the Shandong Provincial Hospital affiliated to Shandong University in September 2010. The patient reported that the chest pain began 2 weeks before the evaluation and that the condition became aggravated along with the development of dysphagia. The dysphagia progressed quickly and she could hardly swallow liquid food 10 days after the symptom appeared. The initial evaluation did not identify the cause of the chest pain or dysphagia, and the patient was referred to the Department of Thoracic Surgery for a detailed examination. A roentgenogram barium swallow showed a half-round filling defect in the lower part of the esophagus that resulted in the near blockage of barium passage (Fig 1A). A chest computed tomography (CT) scan revealed a large low-density to high-density paraesophageal mass (Figs 1B and 1C). No small intestinal duplication, esophageal atresia, tracheoesophageal fistula, or spinal abnormality was found, nor was their evidence of infection, bleeding, perforation, or malignancy. We performed a left thoracotomy, using a standard posterolateral incision. The paraesophageal mass was a 10- 6- 4-cm3 cyst attached with high tension to the
E
sophageal duplication cysts (EDCs) are infrequent gastrointestinal tract anomalies principally found in children, with a male predominance. EDCs are rarely seen in adults because up to 80% of EDCs are diagnosed in childhood and removed, and less than 7% of EDCs remain asymptomatic until adolescence [1, 2]. EDCs result from vacuolization failure of the primitive esophagus around the fourth to eighth gestational week during embryonic development [1]. EDCs have been associated with other congenital anomalies such as small intestinal duplication, esophageal atresia, tracheoesophageal fistula, and spinal abnormalities [3]. Asymptomatic EDCs are difficult to diagnose in adults, and even cases with clinical symptoms are often mistaken for other more common esophageal conditions because of the nonspecific nature of EDC symptoms [2]. Owing to potential complications associated with an untreated EDC, including bleeding, infection, or conversion to a malignancy, the most common treatment for EDCs is surgical excision even if patients are not symptomatic [1, 2]. Thus, diagnosis of EDCs is important. Here we reported a unique adult case of EDC with rapidly progressing chest pain and dysphagia that was ultimately treated with esophagectomy instead of enucleation. A 52-year-old woman with chest pain and dysphagia, but no history of palpitation, cough, dyspnea, weight loss or
Accepted for publication Jan 13, 2015. Address correspondence to Dr Zhou Wang, Department of Thoracic Surgery, Shandong Provincial Hospital affiliated to Shandong University, Jinan 250021, China; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Fig 1. The esophageal duplication cyst (arrows) is seen on a (A) barium esophagogram and on (B) axial and (C) coronal computed tomography images before the operation. (D) Barium esophagogram after the operation. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.01.047
e80
CASE REPORT WANG ET AL ESOPHAGECTOMY FOR A RAPIDLY PROGRESSING EDC
Ann Thorac Surg 2015;99:e79–81
Fig 2. (A) Hematoxylin and eosin staining of the cystic mass lesion with hypertrophic smooth muscles in the wall (original magnification 100). (B) Hematoxylin and eosin staining of stratified squamous epithelium lining the cyst (original magnification 400).
lower thoracic esophagus. The cyst extended medially to the esophagus and shared a common muscular coat with the esophagus. We initially tried to dissect the cyst from the inferior pole but soon realized that its adhesion to the esophagus was extremely intimate. Therefore, we exposed the esophageal lumen and found that the cyst shared an extremely edematous inner lining with the esophagus, whereas the mucosal lining was separate. In consideration of the risk of fistula and recurrence, we removed the involved esophageal region and performed a gastroesophageal anastomosis below the aortic arch. After removal, we observed that the cyst contained old clots and brown purulent materials. Pathologic examination confirmed that the mass was an EDC with squamous epithelium (Fig 2). The patient’s postoperative recovery was uneventful, and repeated barium examinations did not show any esophageal abnormalities (Fig 1D). The only long-term postoperative symptom the patient was observed to experience after 44 months of follow-up examinations was slight acid reflux esophagitis, which was resolved with ranitidine (40 mg/d).
Comment EDCs occur most frequently in children, occur more frequently in males than in females, and are the second most common duplication of the alimentary tract, after the ileum, comprising approximately 20% of the cases [2]. Dysphagia, epigastric discomfort, and retrosternal pain are the most common symptoms of EDCs [3]. In the current case, the patient reported the rapid development of pain and subsequent dysphagia. We believe these symptoms were caused by the rapid growth of the EDC [1, 2]. The diagnosis of EDCs, usually performed by CT and endoscopic ultrasonography [1], is challenging. EDCs usually appear as homogenous, round or oval and sharply marginated masses in the posteroinferior mediastinum. A chest roentgenogram may appear completely normal in some patients who have small-sized and midline EDCs. In this patient, a chest CT scan revealed a smooth, cystic, and well-defined low-density to highdensity mediastinal mass without effective enhancement. Other imaging modalities commonly used to diagnose EDCs include magnetic resonance imaging (MRI) and transesophageal endoscopic ultrasonography (EUS). MRI
usually shows the thin walls and the fluid contents of the lesion, giving a relatively specific diagnosis [4]. EUS is considered the best radiologic diagnostic modality for EDCs. EUS can distinguish EDCs from other mediastinal masses by demonstrating the relationship between the muscularis of the esophagus and the EDC muscle layer [5]. However, because of the patient’s quickly escalating chest pain and dysphagia, the operation was performed without EUS. Surgical examination and histopathology of the resected lesion are required to differentiate EDCs definitively from other mediastinal cysts. Thus, even with strong imaging evidence, a definite diagnosis of EDCs can only be established by pathologic evaluation after surgical resection. Symptoms and complications occur in approximately 70% of patients with untreated asymptomatic EDCs [5]. Once symptoms develop, surgical resection may become more complicated. Although en bloc resection of EDCs is generally advocated for patients with symptomatic or asymptomatic EDCs, open surgical resection through a posterolateral thoracotomy is the conventional approach because EDCs may extend to the opposite side through the prevertebral space [4]. Enucleation has been widely advocated but may not be suitable in many cases. In recent years, video-assisted thoracoscopic surgery has been increasingly applied in EDC removal. Compared with the conventional approach, thoracoscopic surgery reduces postoperative pain and results in better cosmetic outcomes [6]. The cyst in this patient partially shared the inner lining of the esophagus. The shared lining was remarkably inflammatory and hydropic, and thus, we determined that enucleation was not feasible. We therefore performed an esophagectomy to remove the cyst and the associated region of the esophagus. The operation successfully resolved the patient’s chest pain and dysphagia. After 44 months of follow-up, the only notable long-term side effect was a slight acid reflux esophagitis, which was successfully treated with ranitidine. Our findings indicate that esophagectomy was an efficacious and safe treatment strategy for the EDC in this patient. Nevertheless, we will continue to monitor this patient to observe whether other potential postoperative complications, including esophageal fistula, pyothorax, esophageal stricture, and diverticulum, occur in this patient.
Ann Thorac Surg 2015;99:e79–81
This work was supported by Natural Science Foundation of China (N0.81172161).
References 1. Bagheri R, Asnaashari AM, Afghani R. Esophageal duplication cyst. Asian Cardiovasc Thorac Ann 2015;23:332–4. 2. Castelijns PS, Woensdregt K, Hoevenaars B, et al. Intraabdominal esophageal duplication cyst: a case report and review of the literature. World J Gastrointest Surg 2014;6:112–6.
CASE REPORT WANG ET AL ESOPHAGECTOMY FOR A RAPIDLY PROGRESSING EDC
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3. Obasi PC, Hebra A, Varela JC. Excision of esophageal duplication cysts with robotic-assisted thoracoscopic surgery. JSLS 2011;15:244–7. 4. McMaster WG Jr, Mukherjee K, Parikh AA. Surgical management of a symptomatic foregut duplication cyst. Am Surg 2012;78:E306–7. 5. Zhang Z, Jin F, Wu H, et al. Double esophageal duplication cysts, with ectopic gastric mucosa: a case report. J Cardiothorac Surg 2013;8:221. 6. Takemura M, Yoshida K, Morimura K. Thoracoscopic resection of thoracic esophageal duplication cyst containing ectopic pancreatic tissue in adult. J Cardiothorac Surg 2011;6:118.
