Heterotopic pancreas causing intussusception in a child
Authors: Narotam R Jootun, Hock P Cheah, Siddath C Fernando, William S Munro and Martin Veysey
Published online: 3 May 2010
To the Editor: We report the case of a child who presented with intussusception due to heterotopic pancreas.
A 10-year-old girl presented with right iliac fossa pain and nausea. She was febrile (38.4°C) and had right iliac fossa tenderness with guarding. When she started vomiting, she was taken to theatre with a provisional diagnosis of acute appendicitis. During the diagnostic laparoscopy, her appendix did not appear inflamed and she was found to have intussusception due to a 10 mm tumour in the ileum, about 80 mm from the ileocaecal valve. The tumour was resected, and microscopic examination showed the presence of ducts and lobules of exocrine acini, with occasional foci of islet cell tissue extending into the muscularis propria. These features were consistent with heterotopic pancreas.
Heterotopic pancreas is defined as the presence of pancreatic tissue outside its usual location and without anatomic relation either of continuity or of vascularisation with the pancreas.1 Its overall surgical incidence has been estimated as one case in every 500 abdominal explorations at Mayo Clinic.1 During embyrogenesis, the normal pancreas arises from several evaginations originating from the wall of the primitive duodenum. If one or more evaginations remains in the wall of the bowel, it may be carried away from the rest of the gland by the developing gastrointestinal tract and give rise to heterotopic pancreas.2
In adults, the most common locations for heterotopic pancreas, in descending order of frequency, are the stomach, duodenum and jejunum.3 However, in children, the most common location is within a Meckel diverticulum.3
Heterotopic pancreas is usually asymptomatic, and most cases have been found coincidentally at laparotomy performed for other abdominal conditions.4 However, in some people, pathological changes such as inflammation, abnormal hormone secretion and cystic degeneration within the heterotopic pancreas have been reported to cause abdominal pain and discomfort.3,4
Isolated heterotopic pancreas in the ileum is very rare and usually asymptomatic. The lesion within the wall of the ileum may act as a lead point,3,5 and this is thought to be the mechanism of intussusception. It has also been postulated that intussusception arises from local disturbance in the motility of the small intestine caused by the heterotopic pancreas.3
References
- Barbosa J, Dockerty MB, Waugh JM. Pancreatic heterotopic review of literature and report of 41 authenticated surgical cases of which 25 were clinically significant. Surg Gynecol Obstet 1946: 82: 527-542.
- Chandan VS, Wang W. Pancreatic heterotopia in the gastric antrum. Arch Pathol Lab Med 2004; 128: 111-112. 0_i1091882
- Pang LC. Pancreatic heterotopia: a reappraisal and clinicopathologic analysis of 32 cases. South Med J 1988; 81: 1264-1275. 0_i1091884
- Tanaka K, Tsunoda T, Eto T, et al. Diagnosis and management of heterotopic pancreas. Int Surg 1993: 78: 32-35. 0_i1091886
- Hamada Y, Yonekura Y, Tanao A, et al. Isolated heterotopic pancreas causing intussusception. Eur J Pediatr Surg 2000; 10: 197-200. 0_i1091888
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