A rare association of intussusception and celiac disease in a child

ABSTRACT CONTEXT: Intussusception is a common cause of acute intestinal obstruction in the pediatric population and it is normally idiopathic. Rare cases of chronic intussusception require investigation with greater attention. CASE REPORT: We present a clinical case of a three-year-old boy with aqueous diarrhea, abdominal distension, vomiting and weight loss over a two-month period. During the investigation, abdominal ultrasound showed imaging of intussusception. The intraoperative findings showed the intussusception had resolved spontaneously. In further investigation, it was found that the diarrhea was malabsorptive and, after the patient underwent upper gastrointestinal endoscopy, a diagnosis of celiac disease was made. After a gluten-free diet was introduced, the patient showed complete remission of symptoms and regained weight, and normal growth was reestablished. CONCLUSION: If the clinical presentation of intussusception is unusual, etiological investigation should be undertaken. In this case report, celiac disease was the underlying cause.


INTRODUCTION
Intussusception is a common cause of small bowel obstruction in children under five years of age, and its classical symptoms include acute abdominal pain, red currant jelly stools and abdominal mass.However, these classical symptoms are not always present and it sometimes mimics acute viral gastroenteritis, with diarrhea and vomiting.Invagination of the proximal bowel into the distal bowel results in venous congestion and bowel wall edema. 1 If not promptly diagnosed and treated, this condition can lead to arterial obstruction, bowel necrosis and perforation.Almost 90% of the etiology of intussusception in children is ileocolic and idiopathic. 1 The lead point of the invagination is lymphoid hyperplasia of the small bowel, but Meckel's diverticulum, polyps and trauma can also lead to this problem.
Intussusception is diagnosed by means of abdominal ultrasound and can be treated surgically or non-surgically.This case report describes the presence of intussusception in a child with a chronic history of diarrhea, which is an unusual presentation in children that should indicate the possibility of a different diagnosis.After the case description, there is a brief discussion with a systematic search of data in the PubMed, Cochrane and LILACS databases (Table 1).

CASE REPORT
A three-year-old boy with a history of liquid diarrhea for a couple of days, several times a day, with intermittent vomiting and without blood on stools or fever, was first seen by a primary care physician.At physical examination, the child was found to be hydrated, without abdominal pain or distention, and was able to take oral fluids and medication.He was diagnosed as having acute gastroenteritis and was advised to take oral hydrate solution and to return to the primary care center if the symptoms worsened.Fourteen days later, the child returned and the mother complained that he had been suffering from intermittent diarrhea, vomiting and abdominal distention.At examination, he did not show any weight loss, was hydrated and had an overall good clinical condition.At this point, the diagnosis was persistent diarrhea and he was advised to start a lactose-free diet, take albendazole for five days and collect parasitological stool samples.
There was no short-term follow-up on this case by the primary care team because the family requested a second opinion from a different general pediatrician.The second physician made the interpretation that the child possibly had lactose intolerance and ordered a lactose intolerance test with blood analysis.When the patient returned to the original primary care center, two months after the initial onset, he was still presenting aqueous diarrhea three to six times a day, abdominal distension, intermittent vomiting and now weight loss.Parasitological stool samples were negative and blood analysis suggested an iron deficient anemia.The lactose intolerance test was not available at that moment.The diagnosis at this point was chronic diarrhea with probable malabsorption.
The child was referred to a tertiary gastroenterology center for further investigation, with upper gastrointestinal endoscopy, serum xylose tests and stool analysis for fat and pH.
Abdominal ultrasound was performed and identified an onionskin image of part of the small bowel (Figure 1), suggestive of intussusception.On the day of the ultrasound, the patient presented a distended but soft palpable abdomen without abdominal pain and was referred for hospital admission.

DISCUSSION
[3][4] It is the most common cause of intestinal obstruction at pediatric ages and needs prompt intervention, which may be surgical or nonsurgical.
Non-operative methods for treating intussusception include barium enema and hydrostatic or pneumatic reduction.While nonoperative methods are used more commonly than surgical intervention, the latter may still be needed when there are complications (peritonitis, perforation or profound shock) or when non-operative intervention is unsuccessful, or in situations of lack of a trained professional for non-invasive approaches. 1 Most cases of intestinal intussusception at pediatric ages are idiopathic, and the lead point is generally lymphoid hyperplasia of the small bowel.The classical symptoms of intussusception include acute abdominal pain, red currant jelly stools and abdominal mass in a child with normal nutritional status.If the child fails to thrive and has chronic diarrhea, pain or blood stools, further investigation should be conducted after treating the intussusception, as was done in our patient.The differential diagnoses include Meckel's diverticulum, polyps, trauma, celiac disease and enteropathy-associated T-cell lymphoma, among others. 1liac disease is a chronic inflammatory intestinal disease that occurs in about 1 to 2% of the general population.3][4] The proposed cause of intussusception in cases of celiac disease is diffuse inflammation and thickening of the intestinal wall, which lead to hyperperistalsis and increased dilation of the small bowel.This, in turn, could be the lead point for intussusception, which can develop singly or multiply, chronically and with self-resolution or the need for surgical intervention.Less frequently, but with worse prognosis, this lead point could also be associated with a focal lead point in lymphomas. 4e majority of studies on this subject are case descriptions or series of cases, and there is a lack of case-control or randomized clinical studies.In a recent retrospective study on patients undergoing imaging for abdominal pain, intussusception was found more frequently in patients with untreated celiac disease (less than nine months before celiac disease was diagnosed) than in the general population (1.2% versus 0.07% respectively). 2 In a large case-control study evaluating the risk of late celiac disease in patients with intussusception, no significant association was found. 3Nonetheless, using a prospective cohort approach, a post-hoc analysis found that 12 out of 29,060 individuals with celiac disease were given a diagnosis of intussusception after the onset of celiac disease, with a modest but significant increase risk of intussusception after celiac disease had been diagnosed (odds ratio, OR = 1.95; 95% confidence interval, CI = 1.01-

Figure 1 .
Figure 1.Abdominal ultrasound image showing an onionskinlike part of the small bowel suggestive of intussusception.

Table 2 .
Patient's laboratory result values and reference values for normality IgA = immunoglobulin A.