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PANCREATIC DUCTAL ANOMALIESDr. Mathews J Chooracken
23.7.2009
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Pancreatic organogenesis Classification Pancreatic divisum Annular pancreas Anomalous pancreaticobiliary union conclusion
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PANCREATIC ORGANOGENESIS
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NORMAL PANCREATIC DUCT
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PANCREAS DIVISUM
most common congenital pancreatic ductal anatomic variant
dominant dorsal duct syndrome causative lesion is relative stenosis of the minor papilla
rather than pancreas divisum per se
failure of the dorsal and ventral pancreatic anlage to fuse
classic pancreas divisum anatomy small ventral duct which drains through the major
papilla larger dorsal duct which drains through the minor
papilla no communication exists between the dorsal and
ventral pancreatic ducts
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EPIDEMIOLOGY
4–14% of the population autopsy series 3–8% at ERCP 9% at MRCP
Lehman GA, Sherman S Gastrointest Endosc Clin N Am 1998
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INDIAN DATA
4.5% male and 6% female cadavers
Prevalence of 3.7% on ERCP, 9.2% of patients presented with pancreatitis
higher frequency of SPINK1 gene mutation compared with healthy controls
Sahni D, et al. Trop Gastroenterol. 2001 Oct-Dec;22(4):197-201
Dhar A, et al. Indian J Gastroenterol. 1996 Jan;15(1):7-9
Garg PK, et al. J Clin Gastroenterol. 2009 Jul 10
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LAKESHORE DATA ERCP’s -590 since June 2006 Pancreas divisum – 12 Incomplete divisum -1 Sphincterotomy -12 Minor papilla stenting -7 Ductal Stricture -2 Chronic pancreatitis - 4
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TYPES Complete Incomplete
small branch of the ventral duct communicates with the dorsal duct
15 % of cases of pancreas divisum modest to full visualization of the dorsal duct may occur
with vigorous major papillary contrast injection clinical implications are the same as for classic (or
complete) pancreas divisum
"reverse" divisum (inverted) when the accessory duct of Santorini does not connect
with the genu of the main pancreatic duct physiologic significance : overflow ‘valve’ to the main
ductal system is absent gallstone impacted at the major papilla will likely cause
more severe pancreatitis
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OTHER PANCREATOBILIARY ABNORMALITIES
annular pancreas elevated sphincter of Oddi basal pressures partial agenesis of the dorsal pancreas ? increased incidence of cholangiocarcinoma
and ampullary carcinoma
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CLINICAL FEATURES
< 5 % of patients develop pancreatic symptoms.
? Cause of pancreatitis some studies have found that the incidence of pancreas
divisum is the same in patients with and without pancreatitis
symptoms occur infrequently in patients with this anomaly
60 % of patients with pancreas divisum and otherwise unexplained abdominal pain had relief of the pain after surgical sphincteroplasty
In patients with recurrent acute pancreatitis, treatment by either surgical or endoscopic papillotomy of the minor papilla resulted in relief from further attacks of acute pancreatitis by 80 percent
Lehman GA, Sherman S Gastrointest Endosc Clin N Am 1995
Delhaye M, Gastroenterology 1985 Nov;89(5):951-8.
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CLINICAL SETTING
Coincidental finding On routine ERCP May be ignored
Minimal symptoms Can be managed conservatively ? Aggressive therapy to prevent progression
Pancreatitis Aggressive management
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DIAGNOSIS
Gold standard- ERCP short and thin pancreatic ventral duct at the major
papilla (acinarization of the parenychma) filling of the dorsal duct at the minor papilla draining
pancreas from the tail to the anterior part of the head NO connection to the ventral duct
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Suspect pancreas divisum if easy selective cannulation of the bile duct and
inability to enter the pancreatic duct failure of injected contrast in the pancreas to
flow past the head inability to pass a guidewire through the major
papilla into the pancreas
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Minor papilla cannulation is dificult in 1/3rd of cases
Intravenous secretin Spray methylene blue on the surface of minor papilla
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MRCP Dorsal pancreatic duct has a constant caliber crosses the common bile duct anteriorly separated from a smaller ventral duct
equivalent to ERCP Esp. If secretin stimulated MRCP is used
Secretin acts as a hydrographic endogenous contrast agent
Matos C, Metens T, Deviere J, Delhaye M, Gastrointest Endosc 2001;53:728-33
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Endoscopic ultrasound If the ventral duct can be traced from the major
papilla through the body and the tail, PD usually can be excluded
Sahai AV. Gastrointest Endosc 2002
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Assessment of stenosis of minor papillae measurement of the emptying time of the dorsal duct after
pancreatography- not standardised manometric studies - increase in the pancreatic dorsal duct
pressure are of limited usefulness- normal values not defined US- secretin test- poor reproducibility, inability to see MPD in
obesity, due to intestinal gas etc. S-MRCP- persistent dilatation of the main pancreatic duct
greater than 3 mm at 10 minutes after secretin injection abnormal response at S-MRCP did not significantly differ
between patients with or without PD
Matos C, Metens T, Deviere J, Delhaye M, Gastrointest Endosc 2001;53:728-33
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Presence of morphological changes confined to dorsal pancreatic duct- suggestive of pancreas divisum
However, studies have demonstrated changes in ventral duct in patients with PD and chronic pancreatitis
Eisendrath P, et al. Prevalence and clinical evolution of isolated ventral pancreatitis in alcoholic chronic pancreatitis. Gastrointest Endosc 2000; 51:45-50.
Coleman SD, Eisen GM, Troughton AB, CottonPB. Endoscopic treatment in pancreas divisum. Am J Gastroenterol 1994; 89:1152-4
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MANAGEMENT
Attempt to improve the pancreatic outflow through the minor papilla
selection criteria who might benefit from therapy is not clearly defined
However, results are better when the indication is that of recurrent acute pancreatitis as compared to that used for patients with pain alone or chronic pancreatitis
Endotherapy/ surgical options
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Surgical Transduodenal sphincteroplasty of the minor
papilla with cholecystectomy and major papilla sphincteroplasty
Results difficult to compare
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Prospective trial (largest surgical trial) 88 patients Sphincteroplasty Mean follow-up: 29 months 74% of patients with acute recurrent
pancreatitis had good response compared to 34% with pain only
Restenosis rate : 8% Patients with stenotic papilla did better (85%)
suggests a predictive role of secretin testing
Warshaw AL, Et al. Evaluation and treatment of the dominant dorsal duct Syndrome . Am J Surg1990, 159:59–64
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ENDOSCOPIC MANAGEMENT
dilation, sphincterotomy, stenting Balloon dilation reported a high rate of
pancreatitis and is not recommended Sphincterotomy
In 5 series (83 patients) who were studied from 1984 to 1993,
74% of the patients with recurrent acute pancreatitis improved as compared to 26% of patients with pain alone and 46% of patients with chronic pancreatitis
High restenosis rate- upto 20% Hence stenting was advocated
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A prospective, randomized trial compared long-term dorsal duct stenting to continued
conservative therapy 19 patients with pancreas divisum with recurrent
pancreatitis The stents (3 to 7 cm long with multiple side-holes)
were exchanged every three to four months and were left in place for one year.
The stented patients had a much higher rate of improvement (90 versus 11 percent) due to statistically significantly reductions in hospitalizations, emergency department visits, and pancreatitis episodes.
These benefits generally persisted over a mean 24-month follow-up period after stent removal. Ertan A, Gastrointest
Endosc 2000 Jul;52(1):9-14.
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prolonged stent therapy remains largely experimental and is not generally recommended. prolonged pancreatic stenting is associated with
stent occlusion or migration, pancreatitis, pancreatic duct perforation, and pseudocyst formation
induction of ductal and parenchymal changes indicating or simulating chronic pancreatitis
Gastrointest Endosc 1996 Sep;44(3):276-82.
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ANNULAR PANCREAS
characterized by a ring of pancreatic tissue surrounding the descending portion of the duodenum.
1 in 20,000 Only case reports from India
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PATHOGENESIS
Baldwin’s hypothesis formation of the ring results from hypertrophy or
failure of regression of the left portion of a paired ventral bud
Lecco’s theory adhesion of the free end of a single ventral
pancreas to the duodenal wall
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ventral duct generally courses posteriorly to join the main duct on the left.
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OTHER DISEASE ASSOCIATIONS
intestinal atresias, malrotation, Tracheoesophageal fistula cardiac defects. Down’s syndrome
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CLINICAL FEATURES
two thirds of patients remain asymptomatic for life
one half of patients become symptomatic at birth or during the first year of life with signs of duodenal obstruction
Adults may present with abdominal pain, nausea postprandial fullness, vomiting, upper GI bleeding (from peptic ulceration), acute or chronic pancreatitis and rarely biliary obstruction
Some series have suggested that patients who present with obstructive jaundice have an underlying periampullary malignancy
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DIAGNOSIS
Infants: x-ray abdomen shows double bubble sign
In adults: CT Abdomen ERCP: If CT abdomen is equivocal
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TREATMENT
preferred surgical approach bypass surgery of the annulus,
duodenoduodenostomy, gastrojejunostomy, or a duodenojejunostomy.
Resection of the annulus should be avoided it is associated with complications such as pancreatitis,
pancreatic fistula formation, and incomplete relief of obstruction
In patients presenting with obstructive jaundice, a thorough investigation must be undertaken to evaluate for associated periampullary malignancy.
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PROGNOSIS
40% mortality in infants because of associated congenital anomalies
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ANOMALOUS PANCREATICOBILIARY UNION (APBU)
confluence of the common bile duct and the pancreatic duct is outside the duodenal wall, with a common channel measuring more than 15 mm
1.5 to 3.2% in various series possible cause of choledochal cysts, bile duct
and gallbladder carcinoma, and recurrent pancreatitis
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DIAGNOSIS
ERCP high risk of pancreatitis
in the presence of a common channel, duct opacification often requires repetitive injections of the pancreatic duct
MRCP Detected ABPU in 82% of cases provided that a
common channel 15 mm or longer
Endoscopic ultrasonography detect APBU in 88% of cases if a common channel of
12 mm or longer is observed
Sugiyama M,. Gastrointest Endosc 1997;45:261-7
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TREATMENT
APBU with a congenital choledochal cyst excision of the extrahepatic bile duct and gallbladder
with Roux-en-Y reconstruction of the biliary tree prophylactic cholecystectomy is recommended
because of the higher risk of gallbladder carcinoma development
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CONCLUSIONS
Pancreas divisum is the commonest ductal anomaly
5-10% of prevalence <5% are symptomatic Can be complete, incomplete, reverse
divisum Diagnosis is by ERCP S-MRCP may be equivalent to ERCP
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If the patient has acute recurrent pancreatits, endotherapy and stenting is most useful
Long term stenting is not recommended
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Annular pancreas Ring of pancreatic tissue around D2 Majority are asymptomatic 50% of patients presents in infancy with
duodenal obstruction Diagnosis is by imaging modalities like CT
abdomen Annular bypass is the surgery of choice Infants have higher mortality due to associated
abnormalities
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Anomalous pancreaticobiliary union CBD joins PD outside the duodenum Common channel has 15 mm in length Associated with choledochal cyst,
cholangiocarcinoma gall bladder carcinoma and recurrent pancreatitis
Diagnosis is by ERCP, MRCP, EUS Surgery is indicated if there is associated
choledochal cyst Prophylactic cholecystectomy in patients
undergoing surgery as there is high risk for gall bladder carcinoma