5 year old girl with abdominal pain and discomfort and one episode of fever spike
- 5 year old girl with abdominal pain and discomfort and one episode of fever spike
- Laboratory reports: Elevated Amylase (518) & Lipase (1840)
- Ultrasound showed bulky head of pancreas and dilated intra and extrahepatic biliary radicles.
FINDINGS- MRCP
- A. Axial and Coronal images of T2_tse_3d images
- B. AXIAL T1W and T2W images
- A. Bilobar intra and extrahepatic biliary radicle dilation with hypointense intraluminal hypointensities (sludge/calculi) within the common bile duct. Pancreatico-biliary maljunction, with union of the common bile duct and pancreatic duct at right angles to each other with relatively long dilated sludge and calculi filled common channel.
- B. Bulky pancreas with dorsal agenesis and Polysplenia
DIAGNOSIS
- Pancreatico-biliary maljunction (Komi classification type Ib), causing moderate intra and extrahepatic biliary radicle dilation with choledocholithiasis.
- Polysplenia and dorsal agenesis of pancreas.
- Acute pancreatitis
DISCUSSION
- Congenital malformation - pancreatic and bile ducts join outside the duodenal wall, usually forming a long common channel
- Prevalence of PBM in Asia is 100 to 1000 times higher than in other parts of the world
- PBM occurs more frequently in women than in men
- Sphincter of Oddi does not regulate the pancreaticobiliary junction (PBJ), and reciprocal reflux of pancreatic juice and bile can occur. Refluxed pancreatic juice injures the biliary epithelium and promotes cancer development
- PBM is divided into that with and without biliary dilatation on the basis of dilatation of the common bile duct
- PBM without biliary dilatation is more difficult to diagnose. Many patients with PBM without biliary dilatation are not diagnosed until adulthood
KOMI classification of Pancreaticobiliary maljunction
Diagnostic Criteria
Complications
- Recurrent Pancreatitis
- Biliary Stones
- Biliary Cancer and Carcinogenesis
Management
- Surgical Treatment
- In patients with PBM with biliary dilatation- resection of the extrahepatic bile duct including the gallbladder
- Patients with PBM without biliary dilatation- prophylactic cholecystectomy
References
Dr VIKHYATH SHETTY
Consultant radiologist
Manipal hospital, Yeshwanthpur, Bengaluru.
Dr. NEHA SATHYANARAYANA
Radiology resident
Manipal hospital, Yeshwanthpur, Bengaluru.
