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Answer: Celiac Disease
Celiac disease is a common immunological disorder resulting from inflammatory injury to the mucosa of the small intestine secondary to an inappropriate T-cell–mediated response to the presence of gluten hordeins and secalins in the diet of genetically susceptible individuals, almost all of whom share the major histocompatibility complex class II HLA-DQ2 or HLA-DQ8 haplotype. Presentation in adults is usually nonacute, with symptoms lasting many months to years, and may include nonspecific abdominal pains, diarrhea, vomiting, malnutrition, steatorrhea, and extraintestinal manifestations such as peripheral neuropathy, ataxia, and seizures. Intussusception can present acutely; however, it is usually subacute or chronic owing to the muscular hypotonia of the small intestine. Transient asymptomatic small-bowel intussusception in celiac disease is well reported1and can be seen in up to 20% of patients.2Adults with non-lead-point intussusception should undergo a biopsy of the duodenum to rule out celiac disease, with duodenal biopsy specimens obtained from the distal duodenum to avoid architectural distortion produced by the Brunner glands or peptic duodenitis.
About 20% of cases of celiac disease present in adults older than 60 years.3Sinister complications such as lymphoma, small-bowel adenocarcinoma, and oropharyngeal and esophageal carcinoma are more frequent in these patients. A gluten-free diet may have a protective effect against these malignancies, although there is a suggestion that the risk of lymphoma may persist despite a gluten-free diet.4Intestinal lymphoma in gluten-sensitive enteropathy occurs in 5% to 10% of patients.5Carcinomas of the gastrointestinal tract occur more frequently in patients with celiac disease. The most common site is the esophagus, with a fairly equal incidence in the stomach, large intestine, and small intestine. Carcinomas of the oropharynx, bladder, ovary, lung, and breast have also been reported.5The risk of small-bowel adenocarcinoma is about 80-fold higher in patients with celiac disease than in the general population.6The enteropathy-associated T-cell lymphoma related to celiac disease responds poorly to chemotherapy and is rapidly fatal.
Computed tomographic scanning can be used to evaluate patients with nonspecific abdominal pains. Intestinal fold pattern abnormalities (jejunization), intestinal dilatation, excess of fluid in the bowel, lymphadenopathy in the mesentery, and transient intussusception are some of the features described from computed tomographic scans.7The pathognomic feature of intussusception on the computed tomographic scan is presence of a bowel-within-bowel configuration. Barium studies may reveal the presence of the coiled spring sign. A computed tomographic scan in our patient revealed the presence of multiple intussusceptions as evidenced by the presence of the target sign (Figure 1). Upper endoscopy and duodenal biopsy confirmed the diagnosis of celiac disease. Duodenal biopsy (Figure 2) revealed subtotal villous atrophy with elongation of crypts. There are markedly increased numbers of lymphocytes in the surface epithelium.
Computed tomographic scan of the abdomen shows targetlike thickening of the small-bowel loops due to thick-walled intussuscipiens surrounding the central intussusceptum with mesenteric fat and blood vessels.
Duodenal biopsy showing the luminal aspect of the intestine (hematoxylin-eosin, original magnification ×100).
Indications for surgery in patients with celiac disease include uncertain diagnosis, likelihood of identifying a pathological lesion, malignant lesions, and vascular compromise of the intestine secondary to intussusception. Children and first-degree relatives should be screened by means of IgA antigliadin, IgA antiendomysial, and IgA transglutaminase antibody status and duodenal biopsy. All patients should have 1 normal follow-up biopsy. However, nonresponders may need more biopsies at regular intervals. Histological normalization of biopsy results will occur at 2 years in about 65% of patients, within 5 years in 85% of patients, and in the long term in 90% of patients.8
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Correspondence:Atul Sehgal, MMed, Department of General Surgery, Locked Bag No. 1, Redcliffe Hospital, Queensland 4020, Australia (firstname.lastname@example.org).
Accepted for Publication:March 2, 2007.
Author Contributions:Acquisition of data: Sehgal, Lisle, Duhig, and Stariha. Analysis and interpretation of data: Sehgal, Lisle, Duhig, and Lambrianides. Drafting of the manuscript: Sehgal, Lisle, and Stariha. Critical revision of the manuscript for important intellectual content: Sehgal, Duhig, and Lambrianides. Administrative, technical, and material support: Sehgal, Lisle, and Lambrianides. Study supervision: Sehgal.
Financial Disclosure:None reported.
Image of the Month—Diagnosis. Arch Surg. 2008;143(12):1234. doi:10.1001/archsurg.143.12.1234
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