Acute Gallbladder Distension and Recurrent Small Bowel Intussusception in a Child with Celiac Disease

Abstract

We report a 2-year-old boy with celiac disease with acute abdominal pain and distension of the gallbladder that resolved spontaneously in a few hours. Several incidences of intussusception of the small bowel were observed during his hospital stay that were transitory and painless. This type of intussusception is well described in the radiologic literature, but is poorly documented in the pediatric world. These findings may be explained by the alteration of the gallbladder and changes in gastrointestinal motility that have been described in celiac disease. CASE REPORT The patient was a 2-year-old boy who arrived at the emergency room with sudden, acute abdominal pain that had begun 20 minutes before his arrival. He was nauseated, and he appeared pale and sweaty. These was no history of fever, vomiting or previous abdominal pain. His medical history was unremarkable until the age of 1 year when his stools became bulky and malodorous, and he showed a slow weight gain. Gluten had been introduced in his diet when he was 8 months old. Physical examination showed an agitated and pale boy. His weight was 14.1 kg (25th percentile) and height was 98.5 cm (90th percentile). Adipose tissue was scant, and his teeth had a loss of enamel. The abdomen was distended and soft on palpation, although he indicated generalized tenderness on deep palpation with normal peristalsis. No masses, hepatomegaly, or splenomegaly were present. Results of laboratory studies were as follows: white blood cell count 4,800 cells/mm3, hemoglobin 11.8 g/dl, platelet count 276,000 cells/mm3, ferritin 4 ng/ml, aspartate aminotransferase (AST) 56 U/l, alanine aminotransferase (ALT) 64 U/l, total bilirubin 0.3 mg/dl, γ-glutamyl transpeptidase (GGT) 3 U/l, total proteins 5.4 g/dl, and albumin 3 g/dl. Coagulation studies, C-reactive protein, serum amylase, urinalysis, and sweat test results were normal. Stool studies for ova and parasites, and serologic analysis for Epstein-Barr virus, cytomegalovirus, and hepatitis B and C viruses produced negative results. Fecal fat analysis (Van de Kamer method) showed steatorrhea (11.5 g of fat per day). Serum IgG and IgA antigliadin antibodies were increased, and serum IgA antiendomysium antibody was positive. An ultrasound study of the abdomen showed a small bowel with a considerable amount of liquid, with hyperperistalsis but without signs of intussusception. The gallbladder was markedly distended (longitudinal diameter 6.2 cm) in relation to the age of the boy (1), and there was no thickening of the wall or evidence of sludge or stones; the bile duct was normal. The abdominal pain resolved spontaneously in the first 2 hours, and the patient consumed oral feedings uneventfully within 24 hours. The following day, ultrasonography showed a normal-sized gallbladder (longitudinal diameter, 5 cm) without further alteration. A later ultrasound examination showed a normal gallbladder and an image of small bowel intussusception that was painless and that disappeared spontaneously during the procedure. Two days later, a barium contrast study revealed a typical coiled-spring appearance of the small bowel that corresponded to an ileo-ileal intussusception, which was also painless and transient, in the right upper abdominal quadrant. An intestinal biopsy revealed total villous atrophy. DISCUSSION The presence of transient, asymptomatic small bowel intussusception as we have observed in this patient, is considered a specific radiologic sign of celiac disease (2). During the 1960s and 1970s, this sign was described in as much as 7% to 20% of patients of all ages with celiac disease after a barium contrast study (3,4), and more precisely in 18% of children with celiac disease (5). The cause is unknown but is thought to be the hypotony of bowel loops and the intestinal motor abnormalities found in celiac disease (6) that produce transitory intussusception without a lead point. It is notable that such a frequent event in celiac disease is rarely mentioned in the pediatric literature. In the past 10 years, we have seen only two articles that refer to this event (7,8). Riccabona and Rossipal (9) studied 17 children with celiac disease by ultrasound before initiating a gluten-free diet, but they did not observe intussusception or abnormalities in the gallbladder. They described signs suggestive of alterations in motility, such as the presence of hyperperistalsis and a considerable amount of liquid in the small bowel, which we observed in our patient. There is increasing evidence that inflammation and immunologic activity of the intestinal mucosa influences the appearance of gut motility disturbances (10). The enteric nervous system controls the motility of the gastrointestinal tract and is also involved in the regulation of immune and inflammatory processes (11). The enteropathy of celiac disease is probably induced by an abnormal immune response to the ingestion of gluten. Kontakou et al. (12) have suggested that the histologic changes in the intestinal mucosa of patients with celiac disease may be caused by increased expression of cytokines within the mucosa. Cucchiara et al. (13) observed motor abnormalities in children with active celiac disease that suggested a marked derangement of the neural control of gut motility and that reversed after a 6-month gluten-free period. These observations imply that gut motor alterations can arise from intestinal inflammation. Interestingly, Mawe et al. (14) showed that the gallbladder has a similar ganglionic plexus to that of the enteric nervous system, and that the wall of the gallbladder has direct enervation from the neurons arising of the myenteric plexus of the duodenum. Celiac disease is accompanied by gallbladder inertia, with a pronounced diminution of the contractility of the gallbladder wall that causes defective emptying (15). This is the result of reduced endogenous cholecystokinin secretion from the damaged intestinal mucosa (16). Brown et al. (17) have suggested that there is a decreased capacity in the gallbladder wall for response to the cholecystokinin in celiac disease. This alteration is reversible when a gluten-free diet is initiated, and the intestinal mucosa heals (18). Our patient had acute abdominal pain associated with distension of the gallbladder. At first we thought that the patient had hydrops of the gallbladder; but with hydrops, the normalization of the symptoms takes several days-more frequently, weeks (19). The abdominal pain may suggest biliary colic because of it occurs suddenly, quickly becomes severe, disappears in 2 hours, and is accompanied by transient gallbladder distension. The origin could be a transitory obstruction of the cystic duct of unknown cause, along with inefficient emptying of the gallbladder characteristic of celiac disease.