Hepatobiliary Pancreat Dis IntVol 8No 2 April 152009 www.hbpdint.

com 215
Author Afliations: Institute of Liver Studies, Kings College School
of Medicine at Denmark Hill, Camberwell, Kings College Hospital,
London, SE5 9RS, UK (Thomas H, Marriott P, Portmann B, Heaton N
and Rela M)
Corresponding Author: Mohamed Rela, MD, Institute of Liver Studies,
Kings College Hospital, Denmark Hill, London SE5 9RS, UK (Tel: +44-
2032993762; Fax: +44-02032993575; Email: mohamed.rela@kcl.ac.uk)
2009, Hepatobiliary Pancreat Dis Int. All rights reserved.
BACKGROUND: Cystic dystrophy in heterotopic pancreas
(CDHP) is a rare benign condition characterized by the
presence of cysts in the wall of the digestive tract associated
with inammation and brosis, intermingled with
heterotopic pancreatic tissue. Treatment options for CDHP
are poorly dened.
METHOD: We report a case of CDHP, and review the
literature focusing on the diagnosis and management.
RESULTS: CDHP is mainly encountered in men in the
fth decade of life in association with chronic pancreatitis
secondary to alcohol ingestion. Alcohol and mechanical
obstruction of heterotopic pancreatic ducts have been
implicated in its pathogenesis. Clinical presentation
is varied and current imaging provides the diagnosis.
Treatment options include somatostatin analogue injections,
endoscopic cyst fenestration and surgical resection
(pancreaticoduodenectomy or gastrointestinal bypass).
CONCLUSION: CDHP is rare and presents a diagnostic
and therapeutic challenge. The long term efcacy and
indications for different treatment options need to be
rened.
(Hepatobiliary Pancreat Dis Int 2009; 8: 215-217)
KEY WORDS: pancreas;
diseases;
duodenum;
cysts;
heterotopia
Introduction
C
ystic dystrophy in heterotopic pancreas
(CDHP) is a rare disorder that was rst
described by Potet and Duclert in 1970 and is
characterized by the presence of cysts in the wall of the
gastrointestinal tract associated with inammation
and brosis, intermingled with heterotopic pancreatic
tissue.
[1-3]
Pancreatic heterotopia is most commonly
seen in the duodenum, and by denition lacks
anatomical and vascular continuity with the pancreas
proper.
[2]
Controversies exist about the optimal
management of CDHP. We describe a case of CDHP
and review the literature focusing on its diagnosis and
management.
Case report
A 43-year-old man presented acutely with epigastric
pain, vomiting and weight loss. Trans-abdominal
ultrasound demonstrated a hypoechoic homogenous
mass in the region of the second part of the duodenum
and head of the pancreas with intra-hepatic biliary
dilation. Contrast enhanced computed tomography
(CT) (Fig.) conrmed these ndings, also revealing
pancreatic duct dilatation with evidence of duodenal
involvement and gastric outlet obstruction. A
Cystic dystrophy in heterotopic pancreas:
a rare indication for pancreaticoduodenectomy
Harun Thomas, Paul Marriott, Bernard Portmann, Nigel Heaton and Mohamed Rela
London, UK
Fig. CT showing mass around duodenum with cystic lesions.
Case Report
216 Hepatobiliary Pancreat Dis IntVol 8No 2 April 152009 www.hbpdint.com
Hepatobiliary & Pancreatic Diseases International
radiological diagnosis of groove pancreatitis was
made. Endoscopic ultrasound revealed a pancreatic
head mass and biopsies showed Brunner gland
hyperplasia with no evidence of malignancy. Because
of the unusual presentation and radiological
ndings, investigations were conducted to exclude
neuroendocrine tumor and autoimmune pancreatitis.
Octreotide scan and autoantibodies were negative,
CA 19-9 was 351 kU/L, and CEA was 4 ng/ml. His
symptoms of gastric outlet obstruction and pain and
the difculty in excluding malignancy, prompted
us to perform an elective pancreaticoduodenectomy,
from which he made an uneventful recovery.
Histological examination revealed a mass lesion
arising from the medial wall of the second part of
the duodenum, measuring 32 mm in thickness and
extending over a distance of 40 mm, with the distal
margin involving the duodenal papillae. Microscopic
examination demonstrated pancreatic heterotopia,
cystic dilation of the pancreatic ducts, marked
myomatosis, sclerosis and inammation. A diagnosis
of duodenal cystic dystrophy of heterotopic pancreas
was made. Associated lymph nodes revealed lymphoid
hyperplasia with no evidence of malignancy.
Discussion
CDHP is an uncommon presentation of complete
pancreatic heterotopia most commonly seen in
proximity to the duodenum. Patients usually present
with visceral pain that characteristically radiates
through to the back, acute pancreatitis, severe weight
loss, upper digestive obstruction or obstructive
jaundice.
[4, 5]
It commonly occurs in men with
alcohol-related problems
[5]
in the fth decade of life
and is associated with chronic pancreatitis in two-
thirds of the patients.
[4]
The pathophysiology of
CDHP is not well understood. It is thought to result
from obstruction of the small ducts of heterotopic
pancreatic exocrine lobules leading to repeated acute
pancreatitis. This mechanism could explain the
occurrence of CDHP in nonalcoholic patients without
chronic pancreatitis. Another theory is that alcohol, or
any other precipitating factor, may have a toxic effect
on the heterotopic pancreatic tissue in the same way
that it affects the pancreas proper, leading to CDHP
associated with chronic pancreatitis.
[6]
CDHP is a distinct entity from chronic
pancreatitis of the pancreas with different pathological
characteristics.
[7]
The location of the lesions within
the duodenal wall with no connection to the pancreas
proper and the presence of an epithelial lining
distinguish CDHP from pancreatic pseudocysts.
[7]

Validated radiological criteria exist for the
diagnosis of CDHP. Its characteristics on contrast
enhanced CT include multiple cysts located in a
thickened duodenal wall and lesion enhancement
after contrast injection because of associated
inammatory changes.
[8]
Endoscopic ultrasonography
reveals the presence of duodenal wall thickening,
cysts, and a network of channels around the cysts. It
allows for differentiation of other diseases including
duodenal duplication, necrotic or cystic tumors, and
pancreatic pseudocysts.
[7, 9, 10]
Magnetic resonance
pancreatography may also be useful as, in addition to
demonstrating cysts within the thickened duodenal
wall, it is highly sensitive at delineating the pancreatic
and biliary ducts.
[5]
CDHP is often misdiagnosed
as a tumor mass, particularly when associated with
marked inammatory changes
[5]
as in this case.
CDHP is one of many benign conditions that
affect the duodenal wall in the region of the minor
pancreatic papilla including pancreatic hamartoma,
para-duodenal wall cyst, myoadenomatosis, and
groove pancreatitis, which have been collectively
termed "paraduodenal pancreatitis".
[1]
They share
similar clinicopathologic ndings, including
thickened duodenal wall containing dilated ducts and
pseudocystic changes, Brunner's gland hyperplasia,
dense myxoid stromal proliferation with intervening
rounded lobules of pancreatic acinar tissue, spillover
of brosis into the adjacent pancreas and marked
scarring with ill-dened borders mimicking
pancreatic tumors. Whether they represent distinct
disease entities or a spectrum of the same disease
process is not known.
Treatment of CDHP is controversial, and options
include somatostatin analogue injections, endoscopic
cyst fenestration, and surgical resection: usually
pancreaticoduodenectomy or gastrointestinal bypass.
[4]

Denitive management is required in 40% of patients,
with less than one-third requiring surgical treatment.
[4]

Symptomatic improvement can be obtained with
fasting, gastric aspiration, analgesics, and abstinence
from alcohol. Octreotide has a variable effect on
pain, reduction of cyst size, and weight gain.
[11, 12]

Endoscopic fenestration may be benecial when the
cysts are few, large and supercial.
[13]
However, the
cysts in CDHP are usually small and numerous,
resulting in incomplete cyst fenestration and only
temporary symptom relief. Patients with severe
symptoms treated medically often relapse and need
surgical management.
[14]
Indications for surgery
include persistent severe symptoms, relapse after
Hepatobiliary Pancreat Dis IntVol 8No 2 April 152009 www.hbpdint.com 217
Cystic dystrophy in heterotopic pancreas
medical treatment, and diagnostic uncertainty
with a suspicion of malignancy. Gastric and biliary
diversions
[7]
have been proposed for obstructive
lesions in the absence of pain, where malignancy can
be condently excluded. Surgical resection may be
localized when the lesion is located in the second part
of the duodenum,
[15]
but usually requires pancreatico-
duodenectomy.
CDHP is an uncommon complication of pancreatic
heterotopia whose natural history is not known.
Symptoms may be severe and warrant surgery, as in
the case presented, however the long-term efcacy and
exact indications of the different treatment options
have not been clearly dened.
Funding: None.
Ethical approval: Not needed.
Contributors: TH wrote the rst draft of this report. All authors
contributed to the intellectual context and approved the nal
version. RM is the guarantor.
Competing interest: No benets in any form have been received
or will be received from a commercial party related directly or
indirectly to the subject of this article.
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Received July 17, 2008
Accepted after revision March 6, 2009