|
Association of pancreas divisum and recurrent
acute pancreatitis with the IVS8-5T-12TG allele of the CFTR gene
and CFTR dysfunction.Pancreas.
2007 Jul;35(1):90-3.
OBJECTIVES:
Pancreas divisum (PD) occurs in approximately 10% of individuals.
Although a minority of patients with PD develop acute pancreatitis
(AP), PD is found in up to 25% of patients with unexplained AP.
Mild mutations or variants of the cystic fibrosis transmembrane
conductance regulator (CFTR) gene, including the IVS8-5T variant,
are associated with idiopathic pancreatitis, but their
relationship with PD is unknown. We hypothesized for such
association. METHODS: Case of 2 patients with PD, recurrent AP,
and CFTR-related disease are reported. RESULTS: Both patients had
similar clinical patterns (young female adults, nonsevere onsets
of AP, mild upper airway manifestations, no major clinical
criteria for cystic fibrosis). They had 2 mutations or variants of
the CFTR gene (including the IVS8-5T-12TG allele) and mild
abnormalities of the CFTR function (increased sweat chloride
concentrations in one patient, normal basal but low responses to
low-chloride and/or isoproterenol solutions on nasal potential
difference). CONCLUSIONS: These observations suggest that impaired
epithelial ion transport due to mild CFTR genotype (namely,
IVS8-5T-TG12) might be involved as a triggering factor in acute
onsets of pancreatitis in PD, possibly through abnormal pancreatic
fluid secretion. Further studies on CFTR mutations and abnormal
nasal airway ion transport in patients with PD, either with or
without recurrent AP, should be conducted.
Pancreas
divisum--a rare cause of chronic pancreatitis.Chirurgia
(Bucur). 2007 Jan-Feb;102(1):83-7.
Pancreas
divisum (P.D.) is a congenital anatomic variant, characterized by
the nonunion of dorsal and ventral pancreatic ducts. A 20 years
old man followed for 8 years with reccurent abdominal pain and
relapsing acute pancreatitis develope chronic calcific
pancreatitis. He was diagnosed with P.D. on endoscopic retrograde
pancreatography and operative pancreatography. The patient was
treated with longitudinal pancreatico-jejunostomy (PUESTOW-GILLESBY
procedure). His pain resolved following surgical drainage of the
pancreatic duct. Evaluation of the clinical course of this patient
and critical review of other such cases in the literature support
the role of compromised ductal drainage of the pancreas in the
pathogenesis of chronic pancreatitis in P.D.
Pancreas
divisum does not modify the natural course of chronic
pancreatitis.J
Gastroenterol. 2007 Feb;42(2):135-9.
Epub 2007 Mar 12.
BACKGROUND: Pancreas divisum is the most common congenital variant
of the pancreas; however, its clinical significance remains
controversial. The purpose of our study was to determine the role
of pancreas divisum in the development of chronic pancreatitis.
METHODS: We compared the clinical presentation, morphological
findings, and course of disease of 30 patients with chronic
pancreatitis associated with pancreas divisum (there was
coexisting chronic alcohol abuse in 18 cases) to those of 57
patients with chronic pancreatitis and no evidence of pancreas
divisum (15 with nonalcoholic pancreatitis and 42 with alcoholic
pancreatitis). RESULTS: Sex distribution, age at onset of disease,
clinical presentation, course of disease, and frequency of
complications were not affected by the presence of pancreas
divisum. Although the etiology of pancreatitis in patients with
pancreas divisum may be attributed to impaired drainage of the
majority of the gland through the minor papilla, we observed a
relatively low frequency of isolated dorsal duct involvement in
our patients irrespective of alcohol use (25% and 28% in patients
with and without a history of alcohol abuse, respectively).
However, involvement of the ventral duct was commonly observed
(75% and 72%, respectively). CONCLUSIONS: The presence of pancreas
divisum in our study did not modify the natural course of chronic
nonalcoholic or alcoholic pancreatitis. Pancreas divisum is not
likely to play a dominant role in the etiopathogenesis of chronic
pancreatitis.
Management of pancreas divisum.Curr
Treat Options Gastroenterol. 2006
Sep;9(5):391-6.
Pancreas
divisum is the most common congenital anomaly of the pancreas.
Abnormal hedgehog protein signaling appears related to the
formation of several pancreatic malformations, including annular
pancreas, pancreatic-biliary malunion, pancreatic rests, and
pancreas divisum. Pancreas divisum by itself should not
necessarily require intervention. A careful evaluation should be
performed to exclude other causes of symptoms. If the patient is
asymptomatic, no further evaluation is necessary. However, a
significant percentage of patients with pancreas divisum and acute
recurrent pancreatitis benefit from intervention. Surgical
sphincteroplasty and endoscopic interventions appear similar in
outcome. Thus, endoscopic intervention with prophylactic temporary
stenting is advised as initial therapy. Surgery should be reserved
for patients with chronic pancreatitis.
Clinical
implications of incomplete pancreas divisum.JOP.
2006 Nov 10;7(6):625-30.
CONTEXT:
Incomplete pancreas divisum is a pancreatic anomaly that results
in an inadequate communication between the ventral and dorsal
pancreatic ducts. Although the relationship between complete
pancreas divisum and pancreatitis has been contentious, clinical
implications of incomplete pancreas divisum have not been noted.
OBJECTIVE: This study was done to investigate the clinical
significance of incomplete pancreas divisum. PATIENTS AND METHODS:
We studied the anatomy of the pancreatic duct system in 3,220
cases using endoscopic retrograde pancreatography; 44 cases had
complete pancreas divisum, and 41 had incomplete pancreas divisum.
MAIN OUTCOME MEASURE: The prevalence of chronic and acute
pancreatitis associated with complete or incomplete pancreas
divisum was compared with that of cases with neither complete nor
incomplete pancreas divisum (controls). RESULTS: All of the
patients with complete or incomplete pancreas divisum who abused
alcohol had chronic or acute pancreatitis. The prevalence of
chronic pancreatitis in patients with complete or incomplete
pancreas divisum was significantly higher than in controls
(P<0.001 and P=0.001, respectively), but acute pancreatitis
occurred more frequently only in patients with complete pancreas
divisum (P=0.010). When we considered pancreatic-type pain as a
pancreatitis-like disease, complete and incomplete pancreas
divisum were suspected as the cause of pancreatitis-like disease
in 39% (15/38: chronic dorsal pancreatitis, n=5; acute
pancreatitis, n=7; pancreatic-type pain, n=3) and 30% (10/33:
chronic dorsal pancreatitis, n=2; acute relapsing pancreatitis,
n=1; pancreatic-type pain, n=7) of patients who did not abuse
alcohol, respectively. CONCLUSIONS: Although the precise
pathophysiology may differ, patients with complete pancreas
divisum and patients with incomplete pancreas divisum may have
similar presentations and a similar prevalence. The clinical
implications of incomplete pancreas divisum may be similar to
those of complete pancreas divisum.
State-art:
diagnosis and management in pancreas divisum.Rev
Gastroenterol Mex. 2005 Jul;70 Suppl
1:133-40.
Pancreas
divisum (PD), the most common congenital variant of pancreatic
duct anatomy, occurs when the ductal systems of the ventral and
dorsal pancreatic ducts fail to fuse during the second month of
gestation. With non-union of the ducts, the major portion of the
pancreatic exocrine secretion enters the duodenum by way of the
dorsal duct and minor papilla. It has been generally accepted that
a relative obstruction to pancreatic exocrine secretory flow
through the minor duct and minor papilla could result in
pancreatitis in small numbers of patients with PD. The debate
whether PD causes pancreatitis continues, although most
authorities agree that PD is a definite cause in a subgroup of
patients. Most patients with PD and well-documented acute
recurrent pancreatitis have responded favorably to surgical
sphincteroplasty of the minor papilla. Endoscopic retrograde
cholangiopancreatography (ERCP) is the most common procedure for
diagnosis PD in patients who have pancreatobiliary symptoms. MRCP
is being increasingly used to establish the diagnosis and secretin
stimulation can improve ductal images greatly. Endoscopic
management of symptomatic patients with PD is evolving. Only a
limited number of series are available, using endoscopic
pancreatic stent placement, minor papilla endoscopic papillotomy,
or both to decompress the dorsal duct in an effort to restore
pancreatic exocrine secretory flow. Even with relatively small
numbers of patients and a near absence of controlled, randomized
trials, it appears that the patients most likely to benefit, as
with surgery, are those with well-documented ARP rather than pain
alone or chronic pancreatitis. Overall we recommend that
pancreatic stenting and pancreatic sphincterotomy should be done
only in large centers with experience in therapeutic ERCP. Further
randomized trials would be of interest.
Pancreatic
tumor associated with pancreas divisum.:
J
Gastroenterol Hepatol. 2005 Jun;20(6):915-8.
BACKGROUND: Although there has been considerable controversy
regarding pancreas divisum and pancreatitis, little discussion of
this has taken place. The purpose of the present paper was to
investigate the relationship between these two conditions.
METHODS: A retrospective investigation was undertaken of
pancreatic tumors associated with pancreas divisum, in 650 cases
of pancreatic carcinoma, 80 cases of intraductal papillary
mucinous tumor of the pancreas and 32 cases of pancreas divisum.
RESULTS: Of these 32 cases, four (12.5%) were associated with
pancreatic tumor: pancreatic carcinoma (n = 3) and intraductal
papillary mucinous tumor (n = 1). All tumors developed from the
dorsal pancreas of pancreas divisum. Periductal and interlobular
fibrosis detected in the non-carcinomatous pancreas of the margin
of distal pancreatectomy implied that chronic dorsal pancreatitis
associated with pancreas divisum preceded carcinoma. CONCLUSIONS:
Pancreatic tumors were detected in 12.5% of cases of pancreas
divisum. In pancreas divisum, longstanding pancreatic duct
obstruction caused by relative stenosis of the minor duodenal
papilla might be a factor promoting oncogenesis.
Pancreas
divisum and the dominant dorsal duct syndrome.:
Ann Chir. 2005
Jan;130(1):5-14.
Pancreas
divisum, the most frequent congenital malformation of the
pancreas, results from the absence of embryologic fusion of the
dorsal and ventral pancreatic ducts which keep an autonomy of
drainage. The dorsal pancreatic duct is dominant and drains the
major part of the pancreatic fluid through a non adapted accessory
papilla. The high prevalence of pancreas divisum in patients
presenting recurrent acute pancreatitis, the presence of
obstructive pancreatitis electively located on the dorsal
pancreatic duct and the results of the treatments targeted on the
accessory papilla are the arguments pleading for the pathogenic
character of the pancreas divisum. Currently, the diagnosis of
pancreas divisum is based on magnetic resonance imaging. For
symptomatic patients (after exclusion of patients with intestinal
functional disorders), results of endoscopic sphincterotomy or
surgical sphincteroplasty are favourable in 75% of patients with
recurrent acute pancreatitis. They are worse in patients with
chronic pain. Surgical sphincteroplasty must be discussed in the
same manner as the endoscopic treatment for sometimes avoiding
multiplication of the procedures.
Discrepancy between
pancreatographic and histopathological findings in the ventral
pancreas of pancreas divisum. :
JOP. 2004 Nov
10;5(6):480-3.
CONTEXT:
Pancreas divisum is a congenital anomaly in which the ventral and
dorsal pancreatic ducts do not communicate. CASE REPORT: Autopsy
case of pancreas divisum with a history of heavy consumption of
alcohol was presented. Pancreatography via the major duodenal
papilla showed a short ventral pancreatic duct of 1 cm in length,
and hypoplasia of the ventral pancreas was suspected. As large
cysts were confined to the dorsal pancreas, isolated dorsal
pancreatitis was also suspected. At autopsy, extensive fibrosis
was detected in both the ventral and dorsal pancreas.
Immunohistochemical examination revealed that PP-rich islets were
scattered in the fibrotic area between the ventral pancreatic
parenchyma and the neck of the pancreas, suggesting that this
fibrotic area originated from the ventral pancreas. These facts
suggest that the short ventral pancreatic duct was not induced by
hypoplasia of the ventral pancreas but was shortened secondarily
by the alcohol-induced fibrosis. CONCLUSIONS: In pancreas divisum,
a short ventral pancreatic duct resulting from secondary factors
may be confused with that originating from hypoplasia of the
ventral pancreas.
|
