Medical Care
Because the anomaly of pancreatic divisum usually is asymptomatic, treatment is generally offered to symptomatic patients after conducting a complete workup for other causes of pancreatitis and abdominal pain.
Patients with mild symptoms can be managed conservatively. Alternatively, patients with recurrent episodes of pancreatitis or chronic pain may need intervention, which can be performed endoscopically or surgically, to alleviate papillary stenosis. Endoscopic interventions include minor papilla papillotomy (needle-knife sphincterotomy over a stent or pull-type sphincterotomy), endoscopic stenting, and balloon dilation of the minor papilla.
Minor papilla stenosis causing resistance to the flow of pancreatic secretion can lead to increased intraductal pressure. Pathogenesis of pancreatitis and pain in the abdomen is thought to be secondary to increased minor papilla intraductal pressure and, thus, minor papilla ductal decompression is used for management.
Endoscopic minor papilla sphincterotomy can be performed alone or along with stenting.
Sphincterotomy can be performed using a papillotome to make a 4- to 6-mm incision in the 10- to 12-o'clock position or over a stent used as a guide. Patients may need stent exchange, which is usually performed in 3-12 months, and papillary sphincterotomy is repeated. Postendoscopic minor papilla sphincterotomy pancreatitis and hemorrhage are common complications of endotherapy. Papillary restenosis, stent restenosis, and stent migration [21] can occur after endotherapy.
Lu et al found evidence that endoscopic retrograde cholangiopancreatography (ERCP) is a safe and effective treatment for symptomatic pancreas divisum, with no significant differences between underaged (age ≤17 years) and adult (age ≥18 years) groups in procedures, complications, or long-term follow-up results. [22] In this study, investigators divided symptomatic patients with pancreas divisum (N = 82) into underaged and adult groups, reviewed their clinical information, and contacted them by telephone or reviewed their medical records to determine their long-term follow-up outcomes. After a median follow-up of 41 months, the overall response rate was 62.3%. [22]
In a systematic review on endotherapy and surgery for pancreas divisum, in which complete or partial pain relief was considered as “response” to treatment, the pooled response rate of endotherapy for acute recurrent pancreatitis was 79.2%, 69% for chronic pancreatitis, and 54.4% for patients with pain related to pancreas divisum. [13] The pooled overall response rate to endotherapy was 69.4%, whereas the response rate to surgery was 74.9%; the difference between the two was not significant. Thus, endotherapy can be a reasonable first-line treatment option for pain relief in pancreas divisum.
In a Medline search to identify all studies that compared the outcomes of endoscopic or surgical therapy for pancreas divisum, (56 observational studies: 31 endoscopic studies, 25 surgical studies), Hafezi et al found that, compared with endoscopic treatment, surgery had a higher success rate (72% vs 62.3%), lower complication rate (23.8% vs 31.3%), and lower reintervention rate (14.4% vs 28.3%). [23]
As explained above, acute recurrent pancreatitis had a higher response rate than chronic pancreatitis or chronic abdominal pain associated with pancreas divisum. [13, 24] Rustagi et al found a similar result: a 94% response rate for acute recurrent pancreatitis, 57% for chronic pancreatitis, and 54% for chronic abdominal pain. [25]
Surgical Care
All patients selected for surgical treatment should have confirmation of pancreas divisum by endoscopic retrograde cholangiopancreatography (ERCP).
The choice of operation depends on the clinical picture and extent of the disease. [26]
Surgical minor papilla sphincterotomy and sphincteroplasty are discussed as follows:
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Surgical sphincterotomy series and sphincteroplasty series seem to have similar outcomes.
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When patient categorization is detailed, meaning recurrent, acute pancreatitis, chronic pain alone, or chronic pancreatitis is present, patients with recurrent attacks of pancreatitis usually respond better to surgical therapy, mirroring the endoscopic therapy series results.
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In a systematic review, Liao found that the pooled response rate to surgical intervention for acute recurrent pancreatitis, chronic pancreatitis, and chronic abdominal pain was 83.2%, 66.7%, and 51.6%, respectively; the overall response rate to surgery was 74.9%. [13]
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Some studies have found that patients with chronic pancreatitis have better response to surgical procedure than endotherapy. [23]
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Pancreatic divisum. Cholangiopancreatogram showing small ventral duct (duct of Wirsung) and normal biliary tree upon cannulation of the major papilla
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Pancreatic divisum. Pancreatogram showing the dominant dorsal duct (duct of Santorini) upon cannulation of the minor papilla
