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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 (ECP) is an uncommon benign condition arising after mucosal damage. We describe 2 cases of small intestinal adenocarcinomas associated with ECP at the distal ileum, one in a background of active Crohn ileitis (case 1), the other 22 years after pelvic radiation therapy (case 2). Both patients presented with small bowel obstruction and received ileocectomy. Macroscopic examination identified an indurated/strictured area in the distal ileum. Histologically, both cases showed a low-grade tubuloglandular adenocarcinoma arising in a background of chronic ischemic stricture and ECP lined by flat cuboidal cells with mild cytologic atypia resembling pancreatobiliary-type epithelium. There was no conventional dysplasia in the surface or adjacent mucosa. Immunohistochemically, both ECP with metaplasia and invasive carcinomas were diffusely positive for CK7 and CK19, while focally positive for CDX2 or CK20. Both cases showed normal wild-type p53 expression. Case 2 was also mismatch repair protein proficient, with membranous β-catenin staining, and retained nuclear SMAD4 expression. In summary, the 2 cases uniquely exhibits "enteritis-metaplasia-carcinoma" sequence, which has not been reported before. This process appears to bypass conventional dysplasia, be slow and indolent, independent of p53, APC/β-catenin, and SMAD4/TGFβ signaling pathways.

This publication reports enteritis cystica profunda (ECP) in the youngest patient described in the literature, being the fifth case that demonstrates an association between ECP and intestinal intussusception and the seventh case of ECP in children. A 30-day-old boy presented intense abdominal pain associated with digestive bleeding with “red currant jelly” stool. He had a history of bloody stool at 15 days old. Due to family history of atopy and normal physical examination, allergic proctocolitis was suspected and the patient temporarily improved after cow's milk and dairy products were eliminated from his mother’s diet. Abdominal ultrasound confirmed the hypothesis of intestinal intussusception. During the surgical approach, ileoileal invagination was evident together with a polypoid tumor of the intestinal wall. Resection of the lesion and ileoileal anastomosis was performed. In the postoperative period, he presented intestinal adhesions, with the need of reoperation and, subsequently, dehiscence of the aponeurosis and intestinal perforation. The patient underwent operation with good recovery. He was discharged in good health and maintained satisfactory clinical progress. The histopathological study of the resected intestine (terminal ileum) revealed a lesion formed by cystic dilations with invasion to the submucosa and musculature of the intestinal wall, lined by intestinal epithelium cells and without nuclear atypia; these findings are compatible with ECP.

Twenty-one lesions from 19 patients with chronic irradiation enteritis (CIE) were examined to elucidate correlations with the histological findings and either the elapsed time interval or the macroscopic features. The lesions were divided into the early CIE group (E group; the lesions resected within 2 years after irradiation) of 10 lesions and the late CIE group (L group; the lesions resected more than 8 years after irradiation) of 11 lesions. Based on the macroscopic features, the lesions of CIE were divided into three types: ulcerative stricture type (U type; 11 lesions), serosal adhesion type (A type; 6 lesions) and wall sclerosing type (S type; 4 lesions). Only A type lesions were observed in the E group, and U type lesions were significantly more frequently encountered in the L group (9 of 11; 82%) than in the E group (2 of 10; 20%). Moderately to markedly degenerated changes of the vessel wall (8 of 11; 73%), enteritis cystica profunda (8 of 11; 73%), atypical epithelia (7 of 11; 64%), and the occurrence of fistula (2 of 11; 18%) were all significantly more frequently present in the L group than in the E group. No radiation-induced colorectal carcinomas were observed. The authors thus conclude that CIE is a slowly progressive disease. The late CIE showed macroscopically ulcerative stricture type properties with tissue degradation, such as fistulas, perforation, and dysplastic epithelia compared with early CIE; thus, long-standing CIE should be followed for the early identification of further complications. The classification of CIE based on macroscopic features is, therefore, considered to be useful to understand the clinical course of this disease better.

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.

Enteritis cystica profunda and diffuse colitis cystica profunda are exceedingly rare conditions. We report here the first case in which these two entities are associated in one patient. This 48-year-old woman presented initially with a solitary ulcer of the rectum and developed later deep ileal and colonic ulcerations with intervening normal mucosa, polypoid masses, and a dense fibrosis of the bowel wall. Colon and ileum specimens demonstrated submucosal mucous cysts and occasionally herniation of mucosal epithelium into the submucosa, which led to the diagnosis of enteritis and colitis cystica profunda. The exact nature of the underlying ileocolonic disease, which necessitated colectomy and extensive ileal resection, remained undetermined although an aggravating role of iterative surgical procedures is possible. Enteritis cystica profunda and diffuse colitis cystica profunda therefore seem to share a common pathogeny and have to be considered as lesions complicating an underlying disease rather than specific and autonomic diseases.

A 60-year-old male was admitted to the hospital because of abdominal pain and vomiting. Ultrasonography, abdominal CT and barium enema indicated an intussusception at the ileocecal region. On laparotomy ileo-ileo-colic intussusception extending to the ascending colon through the cecum was noticed. After reducing the intussus-ception manually, the ileum including the tumor was excised with end-to-end anastomosis. The resected tumor was the submucosal tumor located about 18cm distant from the ileum end. Histologically it was a hamartomatous polyp. Since dislocated mucosa was found in the submucosal layer and muscle layer, the tumor was diagnosed as enteritis cystica profounda. Our recent experience with intussusception due to enteritis cystica profounda of the small intestine is reported together with a reveiw of the literature.

Diaphragm disease (DD) is an uncommon gastrointestinal abnormality typical of nonsteroidal anti-inflammatory drug (NSAID)-induced injury. DD of the small intestine is well defined in its gross appearance (multiple circumferential stenosing lesions); however, its histologic features have been studied in few cases. To better define and update the histologic features of DD, we describe 10 cases in which patients underwent resection of portions of small intestine for DD. Selected gross features and 12 microscopic features were assessed. The typical gross appearance of DD was associated with focal chronic injury of the mucosa with mild to moderate inflammatory infiltrate. A wide array of additional abnormalities was found: eosinophilic enteritis (3 cases), inflammatory fibroid polyp (1 case), enteritis cystica profunda (1 case), villous atrophy (1 case), and neuromuscular and vascular hamartoma-like changes (9 cases). DD and, therefore, NSAID-related injury, should be considered in the differential diagnosis of several conditions affecting the small intestine.

Human intestinal capillariasis is a rare parasitosis that was first recognized in the Philippines in the 1960 s. Parasitosis is a life threatening disease and has been reported from Thailand, Japan, South of Taiwan (Kaoh-Siung), Korea, Iran, Egypt, Italy and Spain. Its clinical symptoms are characterized by chronic diarrhea, abdominal pain, borborygmus, marked weight loss, protein and electrolyte loss and cachexia. Capillariasis may be fatal if early treatment is not given. We reported 14 cases living in rural areas of Taiwan. Three cases had histories of travelling to Thailand. They might have been infected in Thailand while stayed there. Two cases had the diet of raw freshwater fish before. Three cases received emergency laparotomy due to peritonitis and two cases were found of enteritis cystica profunda. According to the route of transmission, freshwater and brackish-water fish may act as the intermediate host of the parasite. The most simple and convenient method of diagnosing capillariasis is stool examination. Two cases were diagnosed by histology. Mebendazole or albendezole 200 mg orally twice a day for 20-30 d is the treatment of choice. All the patients were cured, and relapses were not observed within 12 mo.

Enteritis cystica profunda (ECP) is a very rare hyperplastic benign lesion of the small intestine. Histologically, it is characterized by mucin-filled cystic spaces frequently lined by benign epithelium within the submucosa and muscularis propria. ECP occurs much less frequently than gastritis cystica profunda (GCP) or colitis cystica profunda (CCP). It is most often seen in adults with Crohn`s disease, Peutz-Jeghers syndrome, hamartomatous polyp, or congenital anomalies. We encountered a case of ECP (90 × 15 mm) accompanied by an elongated lipoma in the duodenal bulb, in the absence of any other disease. An endoscopic polypectomy proved sufficient for diagnosing and treating the ECP. To our knowledge, this is the first reported case of ECP accompanied by a lipoma in the duodenal bulb in the absence of any other intestinal disease.

Although well described in the colon, enteritis cystica profunda is an extremely rare lesion of the small intestine with only a few cases documented in English medical literature. It is a benign condition most often associated with Crohn's disease or Peutz-Jeghers syndrome in the adult. It has been described presenting as ileoileal intussusception in the pediatric population. We report a case of enteritis cystica profunda found incidentally on exploratory laparotomy with evidence of interval development over a 5-month period confirming trauma as the etiology.

Enteritis cystica profunda (ECP) is an exceedingly rare lesion of the small intestine. It has identical histopathologic features as colitis cystica profunda, which is mainly a disease of adults and a rare benign lesion of the distal colon and recturn of uncertain cause. Only 12 cases of ECP with only one pediatric case, have been reported in English-language medical literature. Here the authors present the second pediatric case of ECP with the literature review and discussion of the etiology.

Although there have been only 15 published case reports of enteritis cystica profunda, the condition is frequently seen by pathologists. Its importance lies in the resemblance to bowel adenocarcinoma.

L'enterite kystique profonde, une affection tout a fait inhabituelle, est caracterisee par l'apparition de cavites kystiques non neoplasiques dans la paroi de l'intestin grele. Le troisieme cas d'enterite kystique profonde chez l'enfant est presente avec une mention particuliere pour le diagnostic et le traitement. Cette rare entite peut simuler un abdomen aigu et doit rester a l'esprit dans le diagnostic differentiel. Le traitement chirurgical par excision du segment interesse est souhaitable dans ces cas.

In addition to hamartomatous polyps of the small intestine, a patient with Peutz-Jeghers disease had gross and microscopic lesions of the ileum that were analogous to colitis cystica profunda in the large intestine and rectum. To the authors' knowledge, this is the first such case in an adult to be reported. They suggest the designation, "enteritis cystica profunda." The histology of the lesions and their association with a disease known to produce hamartomatous lesions lend support to the proposition that some cases of colitis cystica are also hamartamatous. It is also believed that some reported cases of small-intestinal adenocarcinoma in patients with Peutz-Jeghers syndrome are in reality examples of the benign process of enteritis cystica profunda.

A patient with a history of abdominal discomfort and rectal bleeding was discovered to have two hamartomatous polyps, one of the jejunum and one of the colon. The former was associated with mucinous cysts at its base. The findings were similar to those described for colitis cystica profunda. This case is a rare example of cystica profunda disease of the small bowel. Uniquely, it was associated with a hamartomatous polyp.

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 symptoms 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.

Enteritis cystica profunda (ECP), an uncommon condition, is characterized by nonneoplastic cystic spaces within the wall of the small bowel. The cases of two patients who had ECP in association with Crohn's disease are reported, and the role of mucosal damage in the pathogenesis of ECP is discussed. Pathologists must recognize this entity and distinguish it from adenocarcinoma, which may arise with increased frequency in the small bowel in patients with Crohn's disease.