Clin Endosc.  2013 Mar;46(2):178-181. 10.5946/ce.2013.46.2.178.

A Case of Enteritis Cystica Profunda in the Ampulla of Vater Mimicking Choledochocele

• You KW
• Park SW
• Lee GS
• Kim DJ
• Moon HC
• Hong GY

• KMID: 1805301
• DOI: http://doi.org/10.5946/ce.2013.46.2.178

Abstract

Enteritis cystica profunda is a very rare disease in which a mucin-filled cystic space is surrounded partially with nonneoplastic columnar epithelium in the submucosa of the small intestine. Most of the cases are accompanied by intestinal diseases, and the disease usually occurs in the jejunum and the ileum and there has been no report of a case that occurred in the ampulla of Vater. A 58-year-old healthy female patient without any particular symptom visited the hospital to get additional examination for a mass found on the ampulla of Vater by accident. In esophagogastroduodenoscopy, a cystic mass showing a positive pillow sign was found on the ampulla of Vater. Endoscopic retrogradecholangiopancreatography was conducted as choledochocele was suspected, but no abnormality was found in the biliary system. In endoscopic ultrasonography, multiseptated cystic structures were detected in the submucosal layer of the duodenum. The lesion was resected completely through endoscopic snare polypectomy and the case was histologically diagnosed as enteritis cystica profunda.

Keyword

Enteritis cystica profunda; Choledochal cyst; Ampulla of Vater; Duodenum

MeSH Terms

Ampulla of Vater
Biliary Tract
Choledochal Cyst
Duodenum
Endoscopy, Digestive System
Endosonography
Enteritis
Epithelium
Female
Humans
Ileum
Intestinal Diseases
Intestine, Small
Jejunum
Rare Diseases
SNARE Proteins
SNARE Proteins

Figure

• 

  Fig. 1 Esophagogastroduodenoscopy finding. It shows 1 cm-sized softly protruding mass lesion on the ampulla of Vater.

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  Fig. 2 Duodenoscopic findings. (A) At the ampulla of Vater, a bulging lesion covered with normal mucosa was found at the proximal part of the major papilla. (B) The pillow sign is positive. (C) The lesion was captured by snare for resection. (D) After snare polypectomy, most of the major papilla was preserved.

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  Fig. 3 Endoscopic retrograde cholangiopancreatography finding. It shows no abnormal finding of the bile duct.

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  Fig. 4 Endoscopic ultrasonography finding (12-MHz miniprobe). About 8 mm-sized, honeycomb-shaped, multiseptated cystic lesion arising from submucosal layer of duodenum (arrow).

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  Fig. 5 Histological finding. Multiple cysts lined mucin-producing glandular cells are located in the submucosa (H&E stain, ×40).

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