Photoclinic

 

 

 

 

Figure 1. Endosonography showing a 27×28 mm anechoic mass lesion originating from the  submucosa layer of the esophagus containing a fluid level with faint echogenic material inside.

 

 

 

Rasoul Sotoudehmanesh MD•*, Mohammad Behgam-Shadmehr MD**, Raika Jamali MD*

 

Authors’ affiliations: *Digestive Diseases Research Center, Shariati Hospital, Tehran University of Medical Sciences, **Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Disease, Shaheed Beheshti University of  Medical Sciences, Tehran, Iran.

•Corresponding author and reprints: Rasoul Sotoudehmanesh MD, Digestive Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Tel: +98-218-801-2992, Fax:+98-218-801-2992, E-mail: setoodeh@ams.ac.ir

 

Accepted for publication: 24 April 2008

 

 

A

 51-year-old woman presented with progressive dysphagia to solids and liquids and heartburn of two months' duration. She also reported recent weight loss of six kilograms. On endoscopic examination, there was a submucosal mass lesion with intact mucosa, narrowing the esophageal lumen   from   the   submucosa    in   the    posterior  aspect of the esophageal wall in the distal part of the esophagus extending to the lower esophageal sphincter. Endosonography showed a 27×28 mm anechoic mass lesion originating from the wall adjacent to the aorta, with a fluid level containing faint echogenic material inside.

 

 

 

 

What is Your Diagnosis?

 See the page 432 for diagnosis

 

 

 

Photoclinic Diagnosis:                   Esophageal Duplication Cyst

 

Figure 2. Thoracotomy resection view of the esophageal duplication cyst.

 

 

Because the cyst wall originated from the submucosal layer in the Endoscopic Ultrasound (EUS) study, the diagnosis of duplication cyst was considered preoperatively. Duplication cysts are rare congenital anomalies that arise during early embryonic development. They are most frequently found in the proximal small intestine, although they can also be found in the esophagus, stomach, and colon. The cysts are usually incidentally discovered through radiologic studies or at endoscopy, since they are mostly asymptomatic. Dysphagia, abdominal pain, bleeding, and pancreatitis (when located near the ampulla of Vater) have been reported.1 Reports of malignant transformation (adenocarcinoma and squamous cell carcinoma), bleeding, and infection in cysts exist.2–6 Duplication cysts appear as a submucosal mass lesion with normal overlying mucosa or as a diverticulum that can vary in size from several millimeters up to 5 cm at endoscopy.

They are most commonly diagnosed by CT scan or MRI since they are infrequently seen endoluminally. Endosonography usually shows anechoic homogeneous lesions with regular margins arising from the submucosa (the third layer) or extrinsic to the gastrointestinal wall. The diagnosis can usually be made through the characteristic endoscopic and endosonographic features. Endoscopic ultrasound-guided fine-needle aspiration (EUS-guided FNA) has been used to establish the diagnosis of an esophageal cyst7 although this is not necessary and has the risk of causing infection.8

Although most duplication cysts in adults are incidentally found on imaging studies or during endoscopy and can be managed expectantly, this large symptomatic cyst was removed completely in an open surgery with no postoperative complications. Although the progressive symptoms in the adult patient raised the possibility of complications, the excised cyst was lined with a ciliated pseudostratified columnar epithelium with scattered mucus-secreting cells without any signs of dysplasia and it merely contained mucoid fluid.

 

References

 

1         Faigel DO, Burke A, Ginsberg GG, Stotland BR, Kadish SL, Kochman ML. The role of endoscopic ultrasound in the evaluation and management of foregut duplications. Gastrointest Endosc. 1997; 45: 99 – 103.

2         Coit DG, Mies C. Adenocarcinoma arising within a gastric duplication cyst. J Surg Oncol. 1992; 50: 274 – 277.

3         Lee MY, Jensen E, Kwak S, Larson RA. Metastatic adenocarcinoma arising in a congenital foregut cyst of the esophagus: a case report with review of the literature. Am J Clin Oncol. 1998; 21: 64 – 66.

4         Singh S, Lal P, Sikora SS, Datta NR. Squamous cell carcinoma arising from a congenital duplication cyst of the esophagus in a young adult. Dis Esophagus. 2001; 14: 258 – 261.

5         Lee HS, Jeon HJ, Song CW, Lee SW, Choi JH, Kim CD, et al. Esophageal duplication cyst complicated with intramural hematoma—case report. J Korean Med Sci. 1994; 9: 188 – 196.

6         Zdenek K, Vladimír P, Markéta H, Petr K. Partial laparoscopic resection of inflamed mediastinal esophageal duplication cyst. Surg Laparosc Endosc Percutan Tech. 2007; 17: 311 – 312.

7         van Dam J, Rice TW, Sivak, MV Jr. Endoscopic ultrasonography and endoscopically-guided needle aspiration for the diagnosis of upper gastrointestinal tract foregut cysts. Am J Gastroenterol. 1992; 87: 762 – 765.

8         Wildi SM, Hoda RS, Fickling W, Schmulewitz N, Varadarajulu S, Roberts SS, et al. Diagnosis of benign cysts of the mediastinum: the role and risks of EUS and FNA. Gastrointest Endosc. 2003; 58: 362 – 368.

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