International surgical boards

2021-12-16 05:56:49 Treatment modalities in Budd chiari syndrome

For informations regarding the nature of this disease search the topic in the channel.

If the occlusion is predominantly thrombotic, thrombolytic therapy followed by heparin may clear off an occluding thrombus at the hepatic veins.

If the occlusion is due to a short hepatic vein stricture, angioplasty may help, but if there is a large long stricture, a shunt can be forced through it to restore continuity, this is generally done by TIPSS (see fig above).

Surgical portosystemic shunts to bypass the occlusion in the hepatic veins are now rarely done that TIPPS is available.

Some cases of Budd chiari syndrome are caused by inferior vena caval stenosis, so dilatation may be helpful.

Aggressive search for an underlying hematological disorder and treating it if possible is crucial, these can include defeciency of ATIII, protein S, and protein C, or myelofibrosis, polycythemia and paroxysmal nocturnal hemoglobinuria. Open / Comment

2021-12-16 01:50:57 Answer

Q1: Primary sclerosing cholangitis (for details search the topic on channel).

Q2: Patient has occult stool, it is known that ulcerative colitis patients who develop primary cholangitis have very high risk of colon cancer, screening with colonoscopy is needed urgently,, also MRCP reveals a dominant stricture, this is worrying, as it can be a cholangiocarcinoma, endoscopic brushing/biopsy is indicated. Open / Comment

2021-12-15 13:15:23 Question

25 year old male, a known case of ulcerative colitis and is on treatment with oral 5ASA drugs, he started complaining of pruritis that had slowly occured over 2 months, physical examination shows loss of weight and mild icterus, LFT show high elevation of alkaline phosphatase enzyme (8 times the normal value), Stool examination showed occult blood.
Magnetic resonance cholangiopancreatography is done (see above).

Q1: What is the cause of elevated alkaline phosphatase and pruritis in this patient?.

Q2: What would you like to look for next, and by using what investigation?. Open / Comment

2021-12-15 13:06:33 Primary sclerosing cholangitis

This is a chronic fibrosing inflammation affecting the entire biliary tree (both intra and extrahepatic passages).

Often regarded as an autoimmune disease, but unlike primary biliary cirrhosis evidence of autoimmunity is very weak, so the cause is largely unknown.

The condition is more common in young males (opposed to primary biliary cirrhosis which is commoner in middle aged females), and it is intimately and very closely related to inflammatory bowel disease.

Most patients with PSC also have ulcerative colitis, the risk in crohns disease is present but is less than ulcerative colitis.

When PSC develop in patients with UC, the risk of colorectal cancer becomes very high, reason behind this is not known.

Histologically, there is periductal 'onion skin' fibrosis with narrowing of bile duct (see fig above arrows), fibrosis can progress to cause complete obliteration of bile ducts, known as 'Vanshing bile duct syndrome'

MRCP is the main diagnostic test. Open / Comment

2021-12-15 12:27:13 Note

When bile duct system is strictured due to a known cause it is called secondary sclerosing cholangitis, the causes can be divided into iatrogenic and non iatrogenic:

Iatrogenic causes:

1) Bile duct surgery.
2) Infusion of 5 flourodeoxyuridine in treatment of hepatic metastasis.
3) Formalin injection into a hydatid cyst.
4) Injection of alcohol into a hepatic tumour.

Non iatrogenic causes:

1) Bile duct stones leading to cholangitis.
2) Parasitic infestation of biliary system especially clonorchis sinensis infection.
3) Cholangiocarcinoma.
4) AIDS, probably because it promote infection of biliary system with CMV or cryptosporidiosis.
5) IgG4 associated cholangitis (this is a newly recognised disease associated with autoimmune pancreatitis). Open / Comment

2021-12-15 11:34:12 Open / Comment

2021-12-15 11:22:31 AST/ALT = Aspartate transaminase/ Alanine transaminase.
ALP = Alkaline phosphatase.
GGT = gamma glutamyl transpeptidase. Open / Comment

2021-12-15 11:20:56 Open / Comment

2021-12-15 11:15:21 Open / Comment

2021-12-15 11:14:08 Open / Comment