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Enteritis cystica profunda (ECP), a rare and benign condition, is defined as the displacement of the glandular epithelium into the submucosa and more profound layers of the small intestinal wall leading to the formation of mucin-filled cystic spaces. ECP frequently occurs in the ileum or jejunum and is associated with diseases such as Crohn disease and Peutz-Jeghers syndrome. ECP also develops in the absence of known pathology. ECP in the duodenum is very rare and mostly occurs without associated conditions. In this report, we present a rare case of ECP without an associated disease, in the second portion of the duodenum distal to the ampulla of Vater and coexisting with lipoma within the polypoid lesion.

Enteritis cystica profunda is a rare and benign disease defined as the invagination of the intestinal epithelium into the submucosa and more profound layers of intestinal wall leading to the formation of mucin-filled cystic spaces. We reported the case of a 45-year-old female, suffering from a Crohn's disease, with a Koenig's syndrome, diarrhea, abdominal pain and weight loss. The colonoscopy and the abdominopelvic scan showed a terminal ileal stenosis, with parietal calcifications. A surgical ileocecal resection was decided. Gross examination of the ileocecal resection showed a thickening of the ileal wall, with many mucin-filled cysts measuring 1mm to 2cm, with some calcifications. The ileal mucosa was ulcerated, and showed a stenotic sector extending over 3cm. Histological examination showed acute ulcerated ileitis lesions, with chronic ileitis lesions and stenosis, compatible with the known diagnosis of Crohn's disease. There were also many cysts into the ileal wall. They were lined with a regular ileal epithelium. The cysts contained mucus, with some calcifications. Some cysts were ruptured, with extravasation of mucus within the wall. Cystica profunda can be found anywhere along the digestive tract. The physiopathology is not yet well understood, but it seems to be favored by chronic aggression of the intestinal wall. This pathology most often coexists with Crohn's disease. The main differential diagnosis is mucinous adenocarcinoma. Cystica profunda does not require any specific treatment., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Enteritis cystica profunda (ECP) is a rare benign disease first described in the colonic epithelium. This pathology is developed as cystic lesions filled with mucinous material delineated by an epithelium of columnar characteristic in the mucosa of the small intestine. A 61-year-old patient without history of previous surgical procedures was admitted to the emergency room with one day of evolution of abdominal pain associated with anorexia, no bowel movements, multiple emetic episodes, and oral intolerance. A diagnosis of intestinal symptomatic management was performed and then a diagnostic laparoscopy was performed with intestinal resection, and primary anastomosis and the surgical specimen was obtained for histopathological study. ECP is a pathology whose pathophysiology is poorly understood, which is commonly accepted as the development of an ulcerative process with the consequent development of a cyst as a repair method. The final diagnosis is made through an anatomopathological study. The scarce literature suggests that this condition can be managed by surgery in order to resect the affected tissue and provide adequate primary anastomosis. Enteritis cystica profunda is a rare disease associated with pathologies such as Crohn's disease. Surgery is the preferred treatment and obtaining a surgical specimen is mandatory for histopathological analysis. • Enteritis cystica profunda is an extremely rare benign disease whose pathophysiology has been scarcely reported. • Enteritis cystica profunda can develop symptoms related to intestinal obstruction. • Surgery is the cornerstone treatment and obtaining a surgical specimen is mandatory for histopathological diagnosis. [ABSTRACT FROM AUTHOR]

We report the case of a 3-month-old male infant with small bowel intussusception caused by enteritis cystica profunda (ECP). The baby was admitted because he was refusing to feed, and was passing “redcurrant jelly” -like stools. A palpable mass was identified, and abdominal ultrasonography showed a mass with a lumen and lumen appearance. We performed laparotomy and resected the segment of bowel containing the mass. The resected segment had enteritis cystica profunda, which was considered to have precipitated the intussusception. A review of the English medical literature revealed only three other cases of children with similar symptoms in the last 30 years. [ABSTRACT FROM AUTHOR]

The hereditary condition known as Peutz-Jeghers syndrome is characterized by mucosal pigmentation and gastrointestinal polyps. The polyps, usually pedunculated hamartomas, are significant only for the symtoms they cause. Intramural lesions also have been described with gross microscopic features that are often interpreted as malignant. Careful evaluation may show most of these lesions to be enteritis cystica profunda. These rarely diagnosed but benign tumors have very different implications for treatment and prognosis. [ABSTRACT FROM AUTHOR]

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.