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Journal of Indian Association of Pediatric Surgeons, Vol. 12, No. 4, October-December, 2007, pp. 221-223 Case Report Recurrent intussusception, coeliac disease and cholelithiasis: A unique combination Sinha CK, Haider N, Zaw W, Nour S The Children's Hospital, University Hospitals of Leicester Code Number: ip07071 Abstract Authors report an 11-month-old female child, who presented with recurrent episodes of colicky abdominal pain and diarrhea. An abdominal ultrasound revealed small bowel intussusception. She was also noted to have a thick walled gall bladder and a solitary gallstone. Further investigations confirmed the diagnosis of coeliac disease. The combination of small bowel intussusception, coeliac disease and cholelithiasis is unique and has not been reported in the literature. Keywords: Child, cholelithiasis, coeliac disease, intussusception Introduction The association of small bowel intussusception and celiac disease is rare, but known. [1],[2],[3] However, a combination of recurrent small bowel intussusception, coeliac disease and cholelithiasis has never been described in literature. We report an infant with this unique combination.Case History An 11-month-old female child was referred with a six-week history of recurrent abdominal distension and diarrhea. This was associated with excessive cry, which seemed to resolve spontaneously after several hours. Pale, loose stools without mucus or blood were features in most of the episodes. She was admitted to her local hospital and was undergoing investigations for the above symptoms. It was initially thought that she was lactose intolerant and, so, was taken off dairy products. With this intervention, there was a transient improvement, but her symptoms returned after some time. So, a coeliac screen was requested along with other investigations. At her most recent admission, she presented with high fever, lethargy, irritability and clinical signs of shock. On examination, there was tenderness in the right upper quadrant along with moderate hepatomegaly and a palpable gall bladder. Hematological tests were done for white cell count (33.5x10 9 /l), hemoglobin (11.7 g/dl), platelets (438 x10 9 /l) and CRP (229 mg/l). An abdominal X-ray was suggestive of bowel obstruction and an ultrasound showed some free fluid in the right iliac fossa. A possibility of acute appendicitis was raised and she was transferred to the regional pediatric surgical unit for further assessment. After initial resuscitation, she underwent a repeat ultrasound, which showed no features of appendicitis. Her clinical condition improved slowly over the next few days with conservative management. A repeat ultrasound done two days later (for excessive crying and increased abdominal distension) showed a thick walled gall bladder with a solitary gallstone [Figure - 1]. On prolonged ultrasound examination, a spontaneously resolving small bowel intussusception was noticed [Figure - 2]. Simultaneously, her coeliac screen antibody (IgA anti-endomysial) came back positive. She was discharged a week later on gluten free diet with plans to perform an upper gastrointestinal endoscopy and duodenal biopsies at a later date. Ever since the commencement of gluten free diet, she has remained well, with no recurrence of abdominal pain and diarrhea. Astonishingly, there was no stone in the gall bladder on repeat ultrasound! Her parents are not keen for her to have an upper gastrointestinal endoscopy and biopsies in view of her clinical improvement on gluten free diet. One year on, she is thriving well and is asymptomatic. Discussion Small bowel intussusception comprises of less than 10% of all pediatric intussusceptions. [4] Although about 18% of the children with coeliac disease show radiological features of small bowel intussusception, clinical association is rare. [1],[2],[3],[5] Transient small bowel intussusception is considered to be a specific radiological sign of coeliac disease; possibly due to hypotony of bowel loops and/or intestinal motor abnormalities. [6],[7] Cucchiara et al . described motor abnormalities in active coeliac disease that suggested a marked derangement of the neural control of gut motility, which improved after a six-month period of gluten free diet. [8] This is possibly due to increased expression of cytokines within the mucosa. [9] The gallbladder has a ganglionic plexus similar to the enteric nervous system. Nerves arising from the myenteric plexus of the duodenum directly supply the gall bladder wall. [10] Few authors have reported gallbladder inertia in coeliac disease, which causes decreased contractility and defective emptying of the gall bladder. [11],[12],[13] This is due to decreased cholecystokinin secretion from the already damaged mucosa of the small bowel and also a poor response of the gallbladder wall to the endogenous cholecystokinin. [14],[15],[16] The resulting effect is distension of the gall bladder, which predisposes to stone formation (as seen in our case). Although, recurrent small bowel intussusceptions hypothetically appear to be a common physiological event in coeliac disease, the clinical presentation is rarely mentioned in pediatric literature. However, the few case reports that have mentioned the clinical occurrence of intussusception in coeliac disease suggest, that most of them resolve spontaneously with conservative management i.e. the optimum nutritional support. [7],[17] A single instance of gallbladder distension (but not cholelithiasis) in association with small bowel intussusception and coeliac disease has been published so far. [7] Our case report is the first of its kind to present the rare triad of cholelithiasis, small bowel intussusception and coeliac disease. It also emphasizes the fact that coeliac disease may predispose to recurrent intussusception, as well as gallbladder dysmotility and stone formation. References
Copyright 2007 - Journal of Indian Association of Pediatric Surgeons The following images related to this document are available:Photo images[ip07071f2.jpg] [ip07071f1.jpg] |
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