A. Nordwig, N. Lorenz, B. Biereder, T. Haufe, M. Kabus (Städtisches Krankenhaus Dresden-Neustadt-Deutschland)  

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15 Years  

Male  

Abdomen-Liver  

inflammatory or infectious  

- 15 year old boy, clinically and neurologically unremarkable
- 03/2005 Diagnosis of rohn's disease, therapy: Azathioprin, Mesalazin
- 11/2005 generalised pruritus, mild scleric icterus
- Labs: Leukocytes 4,1 Gpt/l, Thrombocytes 152 Gpt/l, ASAT 2,28 µmol/s*l, ALAT 3,11 µmol/s*l, Gamma-GT 4,11 µmol/s*l, Bilirubin gesamt 40 µmol/l, Bilirubin direct 17 µmol/l, Bile acides 418 µmol/l , BSG, Alpha1-Glycoprotein, AP, Coagulation, Albumin, Protein electrophoresis, Cholinesterase, Ammonia, Alpha1-Antitrypsin, Iron, Ferritin, Copper, Coeruloplasmin within normal limits.
- Autoantibody screening: pANCA positive, ANA, AMA, SMA, LKM, PBCNA, LC1 negative.
 

PSC is associated in 87% with Ulcerative colitis and in 13% with Crohn's disease and precedes the cIBD (chronic inflammatory bowel disease) symptoms in 50% (Wilschanski M et al, 1995). The pathogenesis of the chronic inflammation of the bile ducts is not clear. Discussed are chronische portal bacterimia, toxic bile metabolites from the intestinal flora, ischemic vascular factors and autoimmun processes which lead to an inflammation and scarring with stenosis of the bile ducts. Affected are initially in most cases small intrahepatic and later on also extrahepatic bile ducts.  

<- view Ultrasound 1

Ultrasound 1: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



<- view Ultrasound 2

Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



<- view Ultrasound 3

Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



<- view Ultrasound 4

Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



<- view MRI 1

MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





<- view X-Ray 1

X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.



 

Total constellation (Consens)  

Drug induced hepatopathy (Azathioprin), autoimmunehepatitis, primary biliary cirrhosis.  

PSC leads often to liver transplantation. Feared is PSC within the transplanted organ. Course and grade of PSC are independent of the activity of cIBD. Therapy of choice is ursodesoxycholic acid with a positive, but only transient effect on the symptoms and lab results (Feldstein AE et al, 2003). There is no proven influence on mortality or liver transplant rate by ursodesoxycholic acid (Breuers U et al, 1992). Very high doses of ursodesoxycholic acid improve possible the log time survival in a PSC patient (40 mg/kg KG). The early endoscopic dilatation of stenotic bile ducts and eventual stent implant is effective in addition to drug therapy (Stiehl A et al, 2002).  

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Primary sclerosing cholangitis, PSC, Crohn, liver disease, Crohn's disease, generalised pruritus, icterus, scleric icterus, child, childhood, pediatric radiology  

A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
URL: www.PedRad.info/?search=20060322175555