Pancreas Divisum

DEFINITION

Congenital abnormality of the pancreatic gland anatomy leading to pancreatitis

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EPIDEMIOLOGY

Common. Estimated at up to 10% of the population.
- but only 5% of these will develop attributable symptoms.
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AETIOLOGY

Pancreatic development
image
Normally the two ducts fuse; dorsal remnant duct often remains as an accessory duct.
Divisum occurs if the ducts do not fuse.


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BIOLOGICAL BEHAVIOUR

Pathophysiology
Relative outflow of obstruction caused by a stenotic accessory orifice.
- failure of full development.
- normal duct flow is up to 2L per day.
No good evidence to support this.

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MANIFESTATIONS

1. Acute recurrent pancreatitis
2. Chronic pancreatitis.
3. Pancreatic-type pain without evidence of pancreatitis.

Mainly presents in 3-4th decades.
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INVESTIGATIONS

Imaging
- CT or MRI; ERCP
ERCP is the reference standard; other methods more commonly used.
- important that minor papilla cannulated if observed during ERCP to demonstrate its anatomy.
Identification of a dominant dorsal duct system important
- but access undependable.
Complexity of patterns / relative duct sizes and arrangements and drainage may affect management but specialist territory
May see a cystic dilation of the distal dorsal pancreatic duct just proximal to the minor papilla.

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MANAGEMENT

Limited evidence

1. Endotherapy
Small accessory papilla orifice is difficult to find, hard to cannulate and anatomically indistinct.
- requires advanced ERCP skills.
Various approaches tried; dilation, stents, combinations.
- high rate of pancreatitis with balloon dilation; avoided.
- perhaps dorsal duct stenting and sphincterotomy.

2. Surgical Therapy
Same idea; enlarge dorsal duct sphincter to improve outflow
Controlled sharp sphincter cutting
May have longer patency but generally reserved for pts failing endotherapy.
Duodenotomy, duct cannulated, both ampullae cut and stitched to lay open / sphincteroplasty.
- closure in 2 layers.

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REFERENCES

Cameron 10th