The following entities with which it shares clinicopathological features are unified by this term and should no longer be used 11-12:
- cystic dystrophy of heterotopic pancreas
- pancreatic hamartoma of duodenum
- duodenal wall cyst
- groove pancreatitis
The epidemiology of paraduodenal pancreatitis is similar to that of run-of-the-mill chronic pancreatitis.
This rare entity is predominantly seen in males with a history of ethanol abuse. Peak incidence is roughly 40-50 years old 11-12.
The clinical presentation of paraduodenal pancreatitis is dominated by symptoms resulting from marked duodenal stenosis and impaired motility with patients presenting with nausea and vomiting.
- recurrent episodes of upper abdominal pain and nausea, typically aggravated by fat or protein-rich meals
- weight loss from poor nourishment
Possible signs include:
- slight elevation of serum markers
- pancreatic enzymes (amylase and lipase)
- cholestatic liver enzymes gamma-glutamyltransferase (GGT) and alkaline phosphatase (ALP)
- usually unremarkable level of tumour markers, e.g.
The pathogenesis of paraduodenal pancreatitis remains controversial.
The hallmark of disease is the presence of scar tissue with fibrosis in the pancreaticoduodenal groove or in the groove and the superior portion of the pancreatic head (in the pure and segmental forms of the disease, respectively).
The duodenum is always involved by a chronic inflammatory process, with scar tissue in the wall leading to fibrosis and various levels of stenosis.
Paraduodenal pancreatitis has been suggested to occur via increased viscosity of the pancreatic juice caused by alcohol, thus predisposing to crystal formation and increased production of protein, resulting in stone formation. Impeding the minor papilla either pathoanatomically or functionally this process incapacitates exocrine pancreatic function.
The presence of affected pancreatic elements in the duodenal wall leads to inflammatory injury of the descending (second) part of the duodenum in the pancreatic groove. Changes may be cystic and/or stenotic 11-12.
Histopathological features have been demonstrated to include 11-12:
- dilated pancreatic ducts
- duodenal wall pseudocysts
- granulation tissue containing fused macrophages of foreign-body type
- hyperplasia of Brunner glands
- heterotopic pancreatic acinar tissue interspersed by dense myofibroblastic and neural proliferation
- involvement of the pancreaticoduodenal groove by fibrotic inflammatory changes
Paraduodenal pancreatitis is usually classified into two forms, pure and segmental:
- Pure form: affects exclusively the groove
- Segmental form: extends to the pancreatic head despite a clear predominance in the groove
May depict pathological changes accurately. The following findings have been described in the literature:
- cystic thickening of the duodenal wall with or without duodenal stenosis
- fibrous tissue within the pancreaticoduodenal groove (may show late enhancement after contrast administration)
- common bile duct dilatation
May demonstrate 5,11:
- thickening of the second part of the duodenum
- possibly in continuity to a hypoechoic mass near the pancreatic head
- additional findings reflecting the background pathologic processes
Endoscopic ultrasound (EUS)
May be preferred over transabdominal imaging as it more readily demonstrates the expected pathological changes comprising of:
- circumscript hypoechoic change between the duodenal wall and the pancreatic parenchyma
- thickening of duodenal wall and associated stenosis
- pancreatic calcifications and pseudocysts
- stenotic changes of the common bile duct
- dilatation of the pancreatic duct
EUS may also allow for guided needle tissue samples 11.
The most characteristic finding on MRI is a sheetlike mass between the head of the pancreas and the duodenum 4.
Signal characteristics include:
- T1: hypointense to pancreatic parenchyma
- T2: hypo-, iso-, or slightly hyper-intense
Associated findings include:
- inflammatory changes of the pancreas
- duodenal stenosis
- local cysts
- regular tapering of the pancreatic and common bile ducts
- widening of the space between the distal pancreatic and common bile ducts, and duodenal lumen at MRCP 10
- banana-shaped gallbladder 10
Treatment and prognosis
Conservative treatment options include analgesics, pancreatic rest and abstinence from alcohol. Symptom relief by these measures is frequently not permanent, as they are unable to stop the underlying background pathologic changes.
Organ-sparing surgery with modified Whipple procedure as either pylorus-preserving pancreaticoduodenectomy or Frey procedure have been demonstrated to result in good and lasting pain relief together with improvement in quality of life 14-15.
History and etymology
It was first described by V Becker (German physician) in 1973 3.
The term “groove pancreatitis” appeared later, coined in 1982 by Stolte et al, members of Becker's group. It is the direct translation of the original term "Rinnenpankreatitis".
- “pure groove pancreatitis”: scarring limited to the groove area and
- “segmental groove pancreatitis,” involvement of the pancreatic head
is the achievement of these Becker group members as well 16.
- pancreatic adenocarcinoma of the head of the pancreas is the most relevant differential diagnosis of paraduodenal pancreatitis (particularly in its segmental form). Needle tissue (FNA) samples may be a challenge for the interpreting pathologist
- duodenal adenocarcinoma mainly when they appear as focal thickening of the medial duodenal wall could be hard to differentiate from paraduodenal pancreatitis
- ampullary carcinoma
- acute pancreatitis involving the pancreaticoduodenal groove
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- cystic neoplasm (cystic pancreatic mass differential diagnosis)
- solid neoplasm
- nonepithelial pancreatic neoplasms
pancreatitis (mnemonic for the causes)
- gallstone pancreatitis
- interstitial oedematous pancreatitis
- necrotising pancreatitis
- haemorrhagic pancreatitis
- revised Atlanta classification of acute pancreatitis
- chronic pancreatitis
- Ascaris-induced pancreatitis
- tropical pancreatitis
- autoimmune pancreatitis
- emphysematous pancreatitis
- hereditary pancreatitis
- pancreatitis associated with cystic fibrosis
- segmental pancreatitis
- acute pancreatitis
- pancreatic atrophy
- pancreatic lipomatosis
- pancreatic trauma