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
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
INDIAN DATA 4.5% male and 6% female cadavers Sahni D, et al. Trop Gastroenterol. 2001 Oct- Dec;22(4):197-201 Prevalence of 3.7% on ERCP, 9.2% of patients presented with pancreatitis Dhar A, et al. Indian J Gastroenterol. 1996 Jan;15(1):7-9 higher frequency of SPINK1 gene mutation compared with healthy controls Garg PK, et al. J Clin Gastroenterol. 2009 Jul 10
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
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
OTHER PANCREATOBILIARY ABNORMALITIES annular pancreas elevated sphincter of Oddi basal pressures partial agenesis of the dorsal pancreas ? increased incidence of cholangiocarcinoma and ampullary carcinoma
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 Delhaye M, Gastroenterology 1985 Nov;89(5):951-8. 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
CLINICAL SETTING Coincidental finding On routine ERCP May be ignored Minimal symptoms Can be managed conservatively ? Aggressive therapy to prevent progression Pancreatitis Aggressive management
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
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
Minor papilla cannulation is dificult in 1/3rd of cases Intravenous secretin Spray methylene blue on the surface of minor papilla
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
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
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
Presence of morphological changes confined to dorsal pancreatic duct- suggestive of pancreas divisum Coleman SD, Eisen GM, Troughton AB, CottonPB. Endoscopic treatment in pancreas divisum. Am J Gastroenterol 1994; 89:1152-4 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.
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
Surgical Transduodenal sphincteroplasty of the minor papilla with cholecystectomy and major papilla sphincteroplasty Results difficult to compare
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
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
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.
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.
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
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
ventral duct generally courses posteriorly to join the mainduct on the left.
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
DIAGNOSIS Infants: x-ray abdomen shows double bubble sign In adults: CT Abdomen ERCP: If CT abdomen is equivocal
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.
PROGNOSIS 40% mortality in infants because of associated congenital anomalies
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
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
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
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
If the patient has acute recurrent pancreatits, endotherapy and stenting is most useful Long term stenting is not recommended
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
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