Multiple Round Elevations in the Sigmoid and Descending Colon

Multiple Round Elevations in the Sigmoid and Descending Colon interactiveCase Edgar Jaramillo, MD, PhD, Eduard Jonas, MD, FCS (SA); Lennart Blomqvist, MD, PhD Edgar Jaramillo, MD, PhD, Head of Endoscopic Unit, Division of Gastroenterology, Karolinska Hospital, Stockholm, Sweden.; Series Editor: Edgar Jaramillo, MD, PhD Eduard Jonas, MD, FCS (SA), Specialist Surgeon, Department of Surgery, Danderyd Hospital, Danderyd; Lennart Blomqvist, MD, PhD, Head of the Abdominal Section, Department of Radiology, Karolinska Hospital, Stockholm, Sweden. Medscape Medscape Gastroenterology 05/24/2000 2 1 <H3>Case History</H3>A 46-year-old female with a history of chronic constipation and episodes of minor rectal bleeding underwent colonoscopy. She was diagnosed with an infrarenal abdominal aorta aneurysm for which elective surgery was planned. The patient had undergone graft replacement of the thoracic aorta and aortic valve 6 years earlier because of a dissecting aorta aneurysm and has been on anticoagulation treatment with warfarin since. On inspection of the perianal region, grade III hemorrhoids were observed which began bleeding during insertion of the instrument. A redundant mesocolon and inability to apply external compression (because of the aneurysm) to facilitate advancement of the instrument complicated the procedure, and only a partial colonoscopy could be performed. The examination was terminated in the transverse colon. The sigmoid and descending colon had a nodular appearance due to multiple round, easily compressible elevations of varying size. The overlying mucosa was macroscopically intact (see Figure 1 below). No biopsies were taken. <center><img src="art-mgi7197.jara.fig1.jpg" width="370" height="388" BORDER="1"></center><blockquote>Figure 1. </blockquote> The partial colonoscopy was complemented with a contrast barium enema to exclude pathology in the remaining colon (see Figure 2 below). <center><img src="art-mgi7197.jara.fig2.jpg" width="268" height="318" BORDER="1"></center><blockquote>Figure 2. </blockquote> <h4>1. What is your diagnosis? Is it appropriate or helpful to take biopsies? How would you manage this patient?</h4> <blockquote><a href="ans1.html" target="Answer" onclick="resizeWin('Answer',200,375)">Click here for answer</a></blockquote> <H3>Discussion</H3>PC, a rare condition, is a form of pneumatosis cystoides intestinalis that generally presents late in middle age. It is characterized by the presence of multiple gas-filled cysts within the wall of the colon, occurring most often in the sigmoid colon and rectum. Most cases are believed to be idiopathic but an association with certain psychiatric disorders, chronic obstructive lung disease, and colitis has been reported. A redundant colon is common in these patients. The disease is frequently asymptomatic and often detected as an incidental finding. Associated symptoms may include altered bowel habits (diarrhea and constipation), mucus rectal discharge, rectal bleeding, and passage of abundant flatus. Different pathogenetic mechanisms have been suggested, including dissemination of air via the mesentery in chronic obstructive lung disease, invasion of the bowel wall by gas-producing bacteria, and fermentation of intramural carbohydrates. At histologic examination, multiple noncommunicating thin-walled gas-filled cysts in the submucosa and subserosa with preservation of the overlying epithelium are observed. The characteristic endoscopic findings are suggestive of the diagnosis. Histologic examination of endoscopic biopsies are diagnostic in approximately 50% of cases. Biopsy specimens should include enough submucosa to demonstrate the characteristic cyst wall. Rupture of a cyst at time of biopsy may give a popping sound; puncture and aspiration with an injection needle may deflate the cyst. Abdominal plain films may show radiolucent areas in the bowel wall and are diagnostic of this condition. Contrast enema displays a nodular appearance with radiolucent elevations in the bowel wall. Differential diagnoses include, among others, lymphoma, familiar adenomatous polyposis, and lymphoid hyperplasia. The disease may resolve spontaneously in up to 50% of cases. Inhalation of oxygen at high concentrations or hyperbaric oxygenation can reduce or even obliterate cysts, but a large percentage will recur. Symptomatic treatment with antidiarrheals or laxatives could be of value.