Full text of DOI:10.1007/PL00008355

Eur J Pediatr 82000) 159: 861±862
Ó Springer-Verlag 2000
ORIGINAL PAPER
Stefan Scholz á Ste€an Lo€ á Hartmut Wirth
Double ileoileal intussusception caused by a giant
polypoid mass of heterotopic pancreas in a child
Received: 14 December 1999 / Accepted: 16 May 2000
Abstract Heterotopic pancreatic tissue has been found in several abdominal and intra-
thoracic locations. In the ileum, it is a rare, usually asymptomatic, incidental ®nding.
Conclusion A unique case of a recurrent ileoileal intussusception in an 11-year-old girl
is presented caused by a giant polypoid mass composed of ectopic pancreatic tissue that
remained undetected during several diagnostic tests during two previous admissions and
laparoscopic abdominal exploration.
Key words Heterotopic pancreas á Intussusception á Ileal polyp á Paediatric
pable tender mass in the right lower quadrant of the abdomen and
ultrasound demonstrated a characteristic `target sign' suggesting
intussusception. All laboratory investigations were normal. At la-
parotomy, the suspected intussusception was located and delivered
through a midline incision. A double ileoileal intussusception was
discovered about 40 cm before the ileocaecal valve and easily re-
duced. As the intussusceptum, a walnut-sized thickening at the top
Introduction
Normally, heterotopic pancreatic tissue remains
asymptomatic. However, depending on location, size,
physiologic activity and in¯ammatory or neoplastic
complications clinicians may be confronted with diver- of a 6 cm long stalked polyp, thick as an index ®nger, could be
palpated through the ileal wall 8Fig. 1). The mesenterium in that
si®ed clinical manifestations.
region was considerably indurated. Consecutively, the small seg-
ment of the ileum containing the polyp was resected and an end-to-
end anastomosis was performed. The girl remains asymptomatic 1
Case report
year later.
Microscopic examination showed an approximately 2.5 cm
nodule of heterotopic pancreatic tissue located within the splintered
muscular layer of the ileal wall at the base and inside the pedun-
culated polypoid mass. The nodule was composed of regular pan-
creatic tissue including islets of Langerhans. The prominent ileal
mucosa showed extensive ulceration with bleeding and rough
®brosis of the ectopic pancreatic tissue, presumably as a conse-
quence of the chronic mechanical alteration caused by peristaltic
waves and recurrent intussusception.
An 11-year-old girl was referred to our clinic with a 1-month his-
tory of recurrent `crampy' abdominal pain. On admission, she
presented with a tender mass at McBurney's point which was so-
nographically suspicious for an abscess. However, all laboratory
studies were normal and the mass disappeared after an enema. The
moderate abdominal pain did not improve with conservative
measures. Exploratory laparoscopy revealed caecal adhesions to
the lateral abdominal wall and a suspected in¯amed appendix
which was removed, otherwise no speci®c ®ndings. Histology
showed a scarred and obliterated appendix. Two weeks later she
was admitted again with abdominal pain. At examination, her
abdomen was soft with, again, a slightly tender mass in her lower
abdomen, which was interpreted as hard stool. Her presumable
faecal impaction resolved with food rest and enema treatments.
Four months later she was referred by her gynaecologist with
another mild episode of `crampy' abdominal pain. On questioning,
she admitted that her abdominal pain had recurred regularly after
her previous hospital stay. Physical examination revealed a pal-
Discussion
In a comprehensive review of 471 cases, the most fre-
quent sites of pancreatic heterotopia were the stomach
825.5%), the duodenum 822.7%), the jejunum 815%) and
Meckel diverticulum 85.3%) [1]. To explain the origin of
S. Scholz 8&) á S. Lo€ á H. Wirth
Kinderchirurgische Universitatsklinik Mannheim,
Fakultat fur Klinische Medizin der Universitat Heidelberg,
Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany
e-mail: stefan.scholz@kch.ma.uni-heidelberg.de
Fax: +49-621-3833823

862
reduced the intussusception, mimicking constipation as
the cause of abdominal pain. Macroscopically, the stool
was never suspicious for blood, however, testing for
occult blood was not done. The heterotopic pancreatic
polyp functioning as a leading point for the intussus-
ception was not discovered at exploratory laparoscopy
although the pathology was within the ileal part which
should be explored for Meckel diverticulum 8up to 2 ft
from the ileocaecal valve). The reliance in evaluating the
small bowel by laparoscopic means may be generally
overrated as this procedure is highly dependent on the
experience of the paediatric surgeon. Here, the clinical
symptoms primarily imitated more regular causes of
moderate recurrent abdominal pain such as constipation
and chronic appendicitis leading to the delayed correct
diagnosis and treatment. Moen and Mack [8] even
needed two laparotomies to discover the cause of an
intussusception in an adult.
Depending on its location, local excision 8e.g. in the
stomach) or segmental resection with restoration of the
continuity by an anastomosis 8e.g. in the alimentary
tract) has been shown to be a safe and adequate treat-
ment [6, 9].
Fig. 1 Intra-operative ®ndings after reduction of the double ileoileal
intussusception. The paediatric surgeon's hands demonstrate the
contour of the polyp through the ileal wall. Note the oedematous,
previously invaginated, ileum
this developmental anomaly, Skandalakis et al. [10]
encouraged the hypothesis of metaplasia of pluripoten-
tial endodermal cells of the embryonic foregut which
might explain occasional reports on unusual sites such as
the fallopian tube [7]. However, no universally accept-
able theory has yet been found. The histological
appearance of ectopic pancreatic tissue is usually similar
to that of normally situated pancreas and it might be
a€ected by the same pathological conditions. Its clinical
signi®cance depends on its location, size, physiological
activity and complications. Patients with presumed
pancreatic heterotopia may present with various clinical
features as abdominal pain, anaemia, melaena, weight
loss, haematemesis or gastric outlet syndrome [3].
Predominantly, the ectopic tissue becomes clinically
symptomatic and radiologically detectable when it is
located in the stomach or duodenum and its size exceeds
1 cm [4, 6]. When becoming symptomatic in the small
intestine, pancreatic tissue frequently causes intussus-
ception with consecutive bowel obstruction [2, 5].
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In the presented case, the ileal intussusception was
intermittent and recurrent which was supported by the
histology and the periodic target sign on ultrasound.
The initial sonographic and physical ®ndings 8tender
mass) resolved after an enema which presumably