A Case of Colitis Cystica Profunda

Abstract

Cystica profunda is a rare non-neoplastic, non-congenital disorder characterized by the presence of multiple benign epithelial-lined mucous cysts that may occur anywhere in the gastrointestinal tract. When it occurs in the colon, this is referred to as colitis cystica profunda (CCP). The condition was first described by Stark in 1766, with Virchow coining the term “colitis cystica profunda” in 1863. The exact etiology of these cysts are unknown, but they appear to arise from the muscularis mucosa. CCP may mimic colonic malignancy radiographically and sometimes endoscopically as well. Additionally, clinical presentation may often be concerning for malignancy, including rectal bleeding and/or mucus in the stool, along with diarrhea or abdominal pain. However, given its benign nature, it is important to distinguish this entity from malignancy. Here, we present a case of CCP. A 49-year-old Caucasian male with no known prior medical history was referred to our center for endoscopic ultrasound (EUS) of lesions noted on colonoscopy. The patient had initially come to medical attention for occasional rectal bleeding, which he had attributed to hemorrhoids. On colonoscopy, there were multiple large submucosal lesions in the splenic flexure and proximal descending colon (Figure 1). EUS of these lesions were notable for anechoic cysts with some hyperechoic areas (Figure 2). For the largest lesion, a small mucosotomy was made using a needle-knife (Figure 3), which demonstrated a small cyst cavity without any fluid or material in the cavity. Biopsies of the external cyst wall were taken that demonstrated benign tissue, consisting of fibrous tissue and focal smooth muscle. Case Highlights: This case illustrates an example of an unusual disease entity, colitis cystica profunda (CCP). Despite its rather impressive endoscopic appearance, its clinical course is benign, and therefore its importance lies in differentiating it from mucinous adenocarcinoma for the purposes of treatment and prognostication.1458_A Figure 1. Endoscopic appearance of colitis cystica profunda. Multiple bulky submucosal lesions noted to be occluding portions of the colonic lumen.1458_B Figure 2. Endoscopic ultrasound appearance of one of the cysts.1458_C Figure 3. Needle-knife mucosotomy being performed into the larges of the cystic lesions.