A case of proximal jejunal ectopic pancreas causing sporadic vomiting.

Abstract

Aberrant rests of pancreatic tissue can be found throughout the gastrointestinal system and are known as pancreatic heterotopia or ectopic pancreas (EP). Authors report a 12-year-old girl with jejunal EP with a long-lasting history of sporadic bilious vomiting. Upper gastrointestinal (GI) study showed delayed passage beyond duodeno-jejunal junction. During laparotomy a 2x2 cm mass was encountered on the mesenteric border of the jejunum, 3 cm distal to the ligament of Treitz. Histopathologic examination revealed pancreatic tissue. The mass was excised and end-to-end anastomosis was performed. Postoperative course of the patient was uneventful and she is doing well after 10 months. Intestinal obstruction due to EP has been reported to occur only if it causes intussusception. Intestinal obstruction without intussusception due to jejunal EP has not been reported. In our case, the EP tissue was located just beneath the mucosa and involved the muscular layer. The foreign body effect of the EP tissue involving the muscular layer may cause dysmotility and/or local spasm, which we think were responsible for the long-lasting sporadic bilious vomiting in our patient.

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The Turkish Journal of Pediatrics 2003; 45: 161-164 Case
A case of proximal jejunal ectopic pancreas causing
sporadic vomiting
Mustafa Olguner1, Tunç Özdemir1, Oðuz Ateþ1, Feza M. Akgür1
Tanju Aktuð1, Erdener Özer2
Departments of 1Pediatric Surgery, and 2Pathology, Dokuz Eylül University Faculty of Medicine, Ýzmir, Turkey
SUMMARY: Olguner M, Özdemir T, Ateþ O, Akgür FM, Aktuð T, Özer E. A
case of proximal jejunal ectopic pancreas causing sporadic vomiting. Turk J
Pediatr 2003; 45: 161-164.
Aberrant rests of pancreatic tissue can be found throughout the gastrointestinal
system and are known as pancreatic heterotopia or ectopic pancreas (EP).
Authors report a 12-year-old girl with jejunal EP with a long-lasting history of
sporadic bilious vomiting. Upper gastrointestinal (GI) study showed delayed
passage beyond duodeno-jejunal junction. During laparotomy a 2x2 cm mass
was encountered on the mesenteric border of the jejunum, 3 cm distal to the
ligament of Treitz. Histopathologic examination revealed pancreatic tissue. The
mass was excised and end-to-end anastomosis was performed. Postoperative
course of the patient was uneventful and she is doing well after 10 months.
Intestinal obstruction due to EP has been reported to occur only if it causes
intussusception. Intestinal obstruction without intussusception due to jejunal
EP has not been reported. In our case, the EP tissue was located just beneath
the mucosa and involved the muscular layer. The foreign body effect of the
EP tissue involving the muscular layer may cause dysmotility and/or local
spasm, which we think were responsible for the long-lasting sporadic bilious
vomiting in our patient.
Key words: ectopic pancreas, pancreatic heterotopia, heterotopic pancreas, intestinal
obstruction.
Aberrant rests of pancreatic tissue can be found
throughout the gastrointestinal (GI) system
and are known as pancreatic heterotopia or
ectopic pancreas (EP). Despite many cases
having been reported, EP causes diagnostic
difficulties for clinicians. We herein report a
12-year-old girl with jejunal EP that caused
sporadic vomiting.
Case Report
A 12-year-old girl with a long-lasting history of
sporadic bilious vomiting was transferred from
another hospital. There she had been treated
conservatively with nasogastric decompression
and had tolerated oral feedings for about a week
but unfortunately her symptoms reappeared.
Afterwards she was transferred on her parents’
demand. Her weight and height were within
appropriate limits for her age when she arrived.
Plain abdominal radiograph showed a huge
stomach extending to pelvic bones, and no air-
fluid levels were detected. Upper GI study showed
delayed passage beyond duodeno-jejunal
junction (Fig. 1). During laparotomy a 2x2
cm mass was encountered on the mesenteric
border of the jejunum, 3 cm distal to the
ligament of Treitz. When the jejunal lumen
was opened through a longitudinal incision on
the antimesenteric side, the mass was seen
to be lying just beneath the mucosa but was
not obstructing the lumen. Macroscopically
misinterpreting the mass as a lymph node
and not being able to obtain frozen section
examination due to the operation being
performed late in the evening, the jejunal
incision was closed transversely and incision
biopsy was done. Histopathologic examination
revealed pancreatic tissue. After the operation
nasogastric drainage of the patient did not
diminish so she was reoperated. During the
second laparotomy the mass and surrounding
bowel were inflamed. The jejunal segment
with the mass was excised and end-to-end

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anastomosis was performed. Histopathologic
examination of the mass and adjacent
intestine showed pancreatic acini with lobular
configuration in the jejunal submucosa, which
was invading muscularis propria, and muscle
fibers were dispersed within the pancreatic
Fig. 1. Huge distended stomach with the delayed
passage in the upper gastrointestinal system series.
Fig. 2. Pancreatic acini with lobular configuration;
muscle fibers (arrow) are dispersed within the
pancreatic lobules (H & E x100).
Fig. 3. Pancreatic acini are seen adjacent to muscular layer in the jejunal submucosa (H & E x40).
acinar lobules (Figs. 2, 3). Postoperative course
of the patient was uneventful and she is doing
well after 10 months.
Discussion
Ectopic pancreas is a relatively rare entity,
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usually of no clinical importance and found
incidentally during laparotomy for another
condition or during autopsy1,2. The most
common locations for EP are stomach,
duodenum and jejunum3. Most patients with
EP are symptom-free. If present, symptoms
are nonspecific. Most commonly reported
nonspecific symptoms are abdominal pain,
epigastric discomfort, nausea, vomiting and
gastrointestinal bleeding4,5. Specific symptoms
are usually due to EP tissue itself leading to
intussusception and pyloric obstruction4.
Additionally, pancreatic cyst, inflammation and
carcinoma may develop in the EP tissue4.
The mechanism of the nonspecific symptoms
of EP has been attributed to the local effects
of the pancreatic tissue. According to this
proposal, foreign body effect and secretions of
the pancreatic tissue may cause local spasm
and chemical irritation in the surrounding
tissue2. However, Dolan et al.3 disagree with
this mechanism in that they believe the mass
of the EP tissue is usually too small to cause
foreign body effect and also that it is incapable
of producing a sufficient amount of enzymes.
They have also questioned why a congenital
anomaly would not have caused symptoms
from the beginning. Actually, the significance
of the nonspecific symptoms in EP has been
found to be related to the size and mucosal
relation of the EP tissue6. The EP greater
than 1.5 cm and close to the mucosa is more
likely to produce symptoms6. Additionally, EP
tissues just beneath the mucosa have more
tendency to involve the muscular layer7. This
muscular involvement seems to be related to
the significance of the symptoms in EP close
to the mucosa. Adult and infant autopsy series
have shown that the EP tissue may enlarge
with age2. It seems highly probable that the
EP tissue reaches sufficient size with age to
produce symptoms.
Specific symptoms are directly related to the EP
tissue itself. Pancreatic cyst, inflammation, and
carcinoma may develop in the EP tissue and
may be the source of ectopic hormone secretion
that may cause hypoglycemia, Zollinger-Ellison
syndrome or acromegaly8-13. The obstructive
symptoms of the EP are mainly related to the
localization of the EP tissue. While prepyloric
EP may cause pyloric obstruction, EP near the
sphincter of Oddi may lead to obstructive
jaundice14-16. Prepyloric EP may cause pyloric
obstruction by occluding the pyloric lumen
or by inducing pylorospasm5,14,16. Obstructive
symptoms may easily occur by these two
mechanisms in an anatomically narrow area
like the pylorus. However, obstruction resulting
from luminal occlusion by the EP tissue seems
unlikely in intestines where the lumen is wider
and more flexible than the pylorus.
Actually, intestinal obstruction due to EP
has been reported to occur only if it causes
intussusception15,17. Intestinal obstruction
without intussusception due to jejunal EP
has not been reported. In our case, the EP
tissue was located just beneath the mucosa and
invaded the muscular layer. The foreign body
effect of the EP tissue, disrupting the muscular
continuity, may cause dysmotility and/or local
spasm, which we think were responsible for
the long-lasting sporadic bilious vomiting in
our patient.
Ectopic pancreas may cause symptoms with one
or more mechanisms, as described above. The
radiological features are sometimes characteristic:
the lesions appear well defined, with a dome-
shaped filling defect with a central umbili-
cation2,18. Similar findings can be observed
during the endoscopic examination18. The best
treatment for EP is the complete excision of the
tissue with wedge resection19. Partial excision
or biopsy can cause insistence of the symptoms
by the ongoing foreign body effect.
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