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Pancreas – congenital and traumatic diseases

1. CONGENITAL DISEASES

Agenesis

  • Absence of part of pancreas – usually the dorsal part
  • Due to mutation of PDX1 gene on chromosome 13
    • Severity of this anomaly depends upon the amount of functional pancreatic tissue left
  • Clinical features – abdominal pain, diabetes mellitus, steatorrhoea, weight loss
  • Investigations – abdominal US, CT
  • Treatment – insulin and pancreatic enzymes (creon)
Pancreatic Divisum

Pathogenesis

  • Pancreas is formed from dorsal and ventral buds
    • Dorsal bud gives rise to the tail and body of pancreas
    • Ventral bud gives rise to the uncinate process and inferior head of pancreas
  • The buds also give rise to the two ducts in the pancreas
    • The dorsal duct – duct of Santorini
    • The ventral duct – duct of Wirsung
  • They normally fuse together, the ventral duct forms the main duct of Wirsung and the dorsal duct forms an accessory duct of Santorini
  • Pancreatic divisum results from failure of fusion of dorsal and ventral duct
    • Consequently, the dorsal pancreas drains through the duct of Santorini into the minor papilla
    • And the ventral pancreas drains through the duct of Wirsung into the major papilla of Vater

Clinical features

  • Most people are asymptomatic
  • Chronic abdominal pain
  • Pancreatitis

Investigations

  • MRCP/ERCP – demonstrates the presence of two separately draining ducts in the pancreas
  • CT
  • MRI

Treatment

  • Asymptomatic cases do not need treatment
  • Mild cases – low fat diet, pancreatic enzyme supplementation
  • Minor papillectomy
  • Stenting or balloon dilation of duct
Annular pancreas 
  • Due to failure of complete rotation of ventral bud of the pancreas, results in a ring of pancreatic tissue that completely encircles duodenum – causes obstruction
  • More common in patients with Down syndrome, polyhydraminos, congenital gastrointestinal problems

Types

  • Neonatal – produces symptoms of intestinal obstruction
  • Adult – presents after the age of 20 years; presents with features of peptic ulcer disease and bilious vomiting

Clinical features

  • Fullness after eating
  • Abdominal distension
  • Visible gastric peristalsis
  • Nausea and vomiting

Investigations

  • Abdominal XR – double bubble sign
  • Abdominal US
  • CT
  • MRI/MRCP – to assess pancreatic duct anatomy

Treatment

  • Surgical bypass of blocked portion of duodenum
    • Duodeno-duodenostomy (preferred)
    • Duodeno-jejunostomy
  • Resection of the ring should not be attempted as it can lead to pancreatic fistula

Ectopic pancreas

  • Pancreatic tissue lies outside and separate to pancreatic gland
  • Most common sites – duodenum, jejunum, ileum, Meckel’s diverticulum
  • Clinical features – usually asymptomatic. Incidentally found on endoscopy
  • Complication – carcinoma of ectopic pancreatic tissue
  • Treatment – surgical if symptomatic

2. TRAUMA OF THE PANCREAS

  • Pancreatic traumas are rare, but when they do occur there is a high mortality rate
  • It is rare for pancreatic trauma to occur in isolation
    • Normally accompanies hepatic, gastric, splenic, renal, colonic, vascular injuries
  • Types of injuries
    • Blunt trauma (more common) – motor vehicle accident
    • Penetrating injuries – gunshot wound, stab wound
  • Injury is most common in the body of pancreas – prone to crush injuries from the vertebral column

Classification – AAST Classification

  • Grade 1 – haematoma with minor contusion/laceration; without duct injury
  • Grade 2 – major contusion/laceration; without duct injury
  • Grade 3 – distal laceration or parenchymal injury; with duct injury
  • Grade 4 – proximal laceration or parenchymal injury; with duct/ampulla injury
  • Grade 5 – massive disruption of pancreatic head

Clinical features

  • Epigastric pain
  • Features of shock
  • Features of associated injuries

Investigations

  • Rise in serum amylase – of limited diagnostic value (amylase is an acute phase reactant so increases following any type of inflammation)
  • Abdominal CT – fluid collections (pseudocyst, abscess, haematoma)
  • MRI/MRCP – to evaluate integrity of panc duct
    • However there is a risk of pancreatitis and perforation)

Treatment

  • Typically conservative with fluid management, blood transfusion, pain relief, antibiotics
  • Surgery
    • Major ductal disruption
    • Vascular injury
    • Extensive injury to the head
    • Other organ injury

Complications

  • Pancreatitis
  • Fistula
  • Abscess and sepsis
  • Haemorrhage
  • Pseudocyst
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