Enteritis cystica profunda with lipoma in the second portion of the duodenum: a case report

Abstract

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: Case report and literature review

“…ECP is a rare benign non-neoplastic condition defined as the displacement of glandular epithelium forming mucin cysts into the submucosa or more profound layers of the gastrointestinal wall [8] . The most common presentation of this disease is colitis cystica profunda, followed by ECP, and finally gastritis cystica profunda.…”

“…However, the most widely reported and best-described proposed pathogenesis of the disease is the association with chronic inflammatory conditions and consequent ulceration of the mucosa, such as ulcerative colitis and Crohn's disease [3] . Other common conditions associated with ECP include Peutz-Jeghers syndrome, prolapse, severe infection, ischemia, and trauma, and rarely occur without an associated condition [8] .…”

“…Regarding the association, with other diseases, such as inflammatory bowel disease (IBD) like Crohn's disease may affect any part of the gastrointestinal tract, often the small intestine [8] . Patients with this condition usually remain asymptomatic or may develop nonspecific signs and symptoms such as abdominal pain, dyspepsia, diarrhea, bleeding, intussusception, and obstruction [10] .…”