Isolated Heterotopic Pancreas Causing Intussusception

Abstract

Pediatric intussusception is usually idiopathic in origin, and a pathological lesion at the leadpoint is identified in only a small proportion of cases. Meckel's diverticulum is the most common location when such a lesion is found. Heterotopic pancreas has been seen most frequently in the stomach or duodenum; however, ileal lesions are rare. In the ileum, heterotopic pancreas is usually associated with Meckel's diverticulum and may cause intestinal obstruction by intussusception. However, isolated heterotopic pancreas in the ileum is distinctly rare and usually discovered incidentally during surgery for other conditions. In a review of English language literature, there were only a few pediatric cases of isolated ileal heterotopic pancreas causing intussusception. We report three additional pediatric cases and discuss the clinical significance of this unexpected rare entity.

Intussusception Secondary to Isolated Heterotopic Pancreas of the Ileum: Case Report and Review of the Literature

“…4 In a series of 134 children with intussusception, Hamada et al found 8 (5.9%) with intestinal lesions as leading points, 3 with isolated heterotopic pancreas of the ileum. 5 Pang et al reviewed 32 histologically proven cases of heterotopic pancreas, of which 14 (44%) were symptomatic and 18 (56%) were asymptomatic or incidental. Six of the 14 symptomatic cases were in the ileum, and 4 caused intussusception, 2 in Meckel's diverticulum, 1 in a duplication cyst, and 1 as an isolated heterotopic pancreas of the ileum.…”

“…Isolated heterotopic pancreas of the ileum on the other hand is very rare, usually asymptomatic and discovered incidentally during surgery for other conditions, and very rarely as a leading point for intussusception. 5,8,[10][11][12] In the series of 292 consecutive children with intussusception by Puri and Guiney, none of the 27 patients identified with leading points had isolated heterotopic pancreas. 7 In the review of 630 episodes of intussusception by Ong and Beasley, none of the 60 patients with pathological leading had isolated heterotopic pancreas.…”

“…18 In an extensive review of the literature, we found only nine cases (Table 1) of isolated heterotopic pancreas of the ileum as a leading point for intussusception in children. 5,8,[10][11][12]18 Their ages ranged from 2 months to 11 years, and in all except two, the intussusception was ileo-ileal, two of which were recurrent intussusception. Whereas idiopathic intussusception is usually seen in infants between 3 and 12 months of age, intussusceptions secondary to a pathological lesion on the other hand have a different age distribution.…”

“…7 In rare isolated instances, heterotopic pancreas of the ileum acts as a leading point for intussusception. 5,[7][8][9][10][11][12][13] Heterotopic pancreas is defined as pancreatic tissue that has neither anatomic nor vascular continuity with the normally located pancreas. It is not uncommon, and is found in l%-2% of autopsies, and as an incidental finding in about 1 of every 500 upper abdominal operations.…”

“…[1][2][3][4] Of the variety of pathologcial lesions identified as leading points for intussusception, Meckel's diverticulum is the most common, and very rarely, isolated heterotopic pancreas of the ileum. 5 This is a case report of intussusception in an infant caused by an isolated heterotopic pancreas of the ileum. The literature on the subject is also reviewed.…”