[size=20px]A 14-year-old boy presents to your clinic. Over the last 12 months he had multiple hospitalizations for acute pancreatitis. An extensive GI workup, including genetic analysis, shows no obvious etiology for his pancreatitis. He does not have gallstones on ultrasound. An MRCP revealed that the dorsal pancreatic duct drains into the minor papilla and the ventral duct joins with the bile duct entering the major ampulla. The ducts are not markedly dilated and there are no strictures of the ducts noted in the body of the pancreas. What would be the best next step in the management of this adolescent?

 

A ERCP with sphincterotomy and stent placement

 

B Transduodenal sphincteroplasty

 

C Pancreaticoduodenectomy (Whipple procedure)

 

D Total pancreatectomy with islet cell autotransplantation

 

E Lateral pancreaticojejunostomy

 

 

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A

A

A

A

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Correct answer

A ERCP with sphincterotomy and stent placement

 

Pancreas divisum is a congenital anomaly that occurs in approximately 10% of individuals. It results from a failure of fusion of the dorsal and ventral pancreatic anlages. Due to this failure, the dorsal pancreatic duct drains the majority of the pancreatic gland via the minor papilla. The ventral pancreatic duct then drains through the major papilla.

 

Three subtypes are known:

• Type 1 (classic): no connection at all; occurs in the majority of cases; 70%
  
• Type 2 (absent ventral duct): minor papilla drains all of pancreas while major papilla drains bile duct; 20-25%
  
• Type 3 (functional): filamentous or inadequate connection between dorsal and ventral ducts; 5-6%
  

Divisum can be diagnosed through MRI, MRCP or ERCP. Most patients with divisum are asymptomatic and require no further treatment. A subset of patients can be found to have abdominal pain and recurrent pancreatitis. Many of these patients have an associated genetic variant including SPINK1, PRSS1, or CFTR gene mutations or polymorphisms that are believed to contribute to pancreatitis. The etiology of the pancreatitis is not completely understood but is believed to be due to limited drainage of the dorsal pancreatic duct through a smaller minor papilla.

 

Numerous surgical options have been offered for the treatment of pancreas divisum in a patient who is symptomatic. Transduodenal sphincteroplasty of the minor papilla has been described previously and may be an option in patients who have undergone previous surgical procedures limiting endoscopic access to the duodenum such as a gastric bypass. In patients with dilated dorsal ducts, a lateral pancreaticojejunostomy may be a surgical option similar to that seen in patients with chronic pancreatitis. The rise of endoscopic therapy in the management of biliary and pancreatic disease has made ERCP with minor papilla sphincterotomy the initial procedure of choice. One study showed a therapeutic benefit of ERCP in children especially when there was stone formation in the minor papilla. Due to the risk of stricture formation following sphincterotomy, most will leave a stent at the time of sphincterotomy.