Cirrhosis & Portal HTN Flashcards
Cirrhosis definition
Many definitions but common one is injury, repair, regeneration and scarring
Primary histological feature
- Marked fibrosis
- Destruction of vascular & biliary system
- Regeneration
- Nodule formation
causes of cirrhosis
Viral hepatitis: the commonest
Alcohol
Biliary obstruction
Veno-occlusive disease
Hemochromatosis
Wilson’s disease
Autoimmune
Drugs and toxins
Metabolic disease
Idiopathic
Classification of Cirrhosis
WHO divided cirrhosis into 3 categories based on morphological character
1. Micro nodular
2. Macro nodular
3. Mixed
Cirrhosis alcohol also know as
Laennec’s cirrhosis
NAFLD / NASH cause of cirrhosis is associated with ?
Obesity, Hyperlipidemia, NIDDM
Diagnosis? ‘
- can be asymptomatic for decades
- Hx and Physical examination
Physical findings;
( Hepatomegaly, jaundice, ascites, spider
angioma, splenomegaly, palmar erythema,
fetor hepaticus, purpura etc.) - Elevated LFTs, thrombocytopenia.
- Definitive diagnosis is by biopsy or gross
inspection of liver. - Noninvasive methods include US, CT scan,
MRI. - Indirect evidence - esophageal varices
seen during endoscopy.
Definitive diagnosis of cirrhosis
Biopsy or gross inspection of liver
Manifestations of Cirrhosis
Hepatorenal syndrome
Hepatic encephalopathy
Portal hypertension
Water retention
Hematologic
Hepatocellular carcinoma
Patho physiology of PH
- Cirrhosis results in scarring (perisinusoidal
deposition of collagen) - Scarring narrows and compresses hepatic
sinusoids (fibrosis) - Progressive increase in resistance to portal
venous blood flow results in PH. - Portal vein thrombosis, or hepatic
venous obstruction also cause PH by
increasing the resistance to portal
blood flow - As pressure increases, blood flow decreases
and the pressure in the portal system is
transmitted to its branches - Results in dilation of venous tributaries
Portal Vein Collaterals
- Coronary vein and short gastric veins -> veins
of the lesser curve of the stomach and the
esophagus, leading to the formation of varices. - Inferior mesenteric vein -> rectal branches which, when distended, form hemorrhoids.
- Umbilical vein ->epigastric venous system
around the umbilicus (caput medusae) - Retroperitoneal collaterals ->gastrointestinal
veins through the bare areas of the liver
Etiology of PH
Causes of PH can be divided into
1.Pre-hepatic
2. Intra-hepatic
3. Post-hepatic
Pre-hepatic PH
Caused by obstruction to blood flow at
the level of portal vein
Examples
- congenital atresia, extrinsic compression,
schistosomiasis, portal, superior mesenteric,
or splenic vein thrombosis
Post-hepatic PH
Caused by obstruction to blood flow at
the level of hepatic vein
Examples
- Budd-Chiari syndrome, chronic heart failure,
constrictive pericarditis, vena cava webs
Budd-Chiari Syndrome
- Caused by hepatic venous obstruction.
- At the level of the inferior vena cava, the
hepatic veins, or the central veins within the
liver itself - result of congenital webs (in Africa and Asia),
acute or chronic thrombosis (in the West), and
malignancy.
Acute symptoms of Budd-Chiari Syndrome
Acute symptoms include
- hepatomegaly, RUQ abdominal pain, nausea,
vomiting, ascites
Chronic form present with the sequelae
of cirrhosis and portal hypertension,
including
- variceal bleeding, ascites, spontaneous bacterial
peritonitis, fatigue, and encephalopathy
Diagnosis of Budd-Chiari Syndrome
- Diagnosis is most often made by US
evaluation of the liver and its
vasculature. - Cross-sectional imaging using contrast-
enhanced CT or MRI.
Gold standard diagnosis of Budd-Chiari Syndrome
Gold standard for the diagnosis has been
angiography
Management has traditionally Budd-Chiari Syndrome
Management has traditionally been surgical
intervention (surgical decompression with a
side-to-side portosystemic shunt)
First line therapy Budd-Chiari Syndrome
Minimally invasive treatment using TIPS may be first-line therapy now
Portal Vein Thrombosis
- Most common cause in children (fewer than
10% of adult pts.) - Normal liver function and not as susceptible
to the development of complications, such
as encephalopathy.
Diagnosis Portal Vein Thrombosis
Diagnosis by sonography, CT and MRI
Initial manifestations of portal vein thrombosis
variceal bleeding in a noncirrhotic patient
with normal liver function
Portal Vein Thrombosis - Causes
- Umbilical vein infection (the most common cause
in children). - Coagulopathies (protein C and antithrombin III
deficiency). - Hepatic malignancy, myeloproliferative disorders.
- Inflammatory bowel disease.
- pancreatitis.
- trauma.
- Most cases in adults are idiopathic.
Portal Vein Thrombosis - Therapeutic options
- Therapeutic options are esophageal variceal ligation and sclerotherapy.
- Distal splenorenal shunt.
- Rex shunt in patients whose intrahepatic
portal vein is patent (most commonly
children).
Splenic Vein Thrombosis - cause
Most often caused by disorders of the
pancreas
- acute and chronic pancreatitis, trauma, pancreatic
malignancy, and pseudocysts
Related to the location of the splenic vein
The common variceal in Splenic Vein Thrombosis + management
- Gastric varices are present in 80% of patients
- Occurs in the setting of normal liver function
- Readily cured with splenectomy (variceal
hemorrhage), although observation for
asymptomatic patients is acceptable.
Complications of PH
- GI bleeding due to gastric and
- esophageal varices - Ascites
- Hepatic encephalopathy
Varices - Most life threatening complication is
- bleeding from esophageal varices
- Distal 5 cm of esophagus
- Usually the portal vein-hepatic vein
pressure gradient >12 mm Hg - Bleeding occurs in 25-35% of pts. With
varices and risk is highest in 1st yr
Prevention of Varices
- Primary prophylaxis: prevent 1st
episode of bleeding. - Secondary prophylaxis: prevent recurrent episodes of bleeding
Include control of underlying cause of
cirrhosis and pharmacological and surgical
interventions to lower portal pressure.
Beta blockade: Beta blockade (Nadolol,
Propranolol)
Nitrates:Organic nitrates
Endoscopy: Sclerotherapy (no longer used) and
variceal ligation
Surgery: No longer performed
Treatment of Varices
Initial Management:
Airway control
2. Hemodynamic monitoring
3. Placement of large bore IV lines
4. Full lab investigation (Hct, Coags, LFTs,)
5. Administration of blood products
6. ICU monitoring
Pharmacologic Treatment of Varices
Endoscopic Therapy for Varices
Balloon Tamponade
TIPS
Surgical Intervention
Surgical Shunts
Pharmacologic Treatment of Varices
- Decreases the rate of bleeding.
- Enhances the endoscopic ability to visualize
the site of bleeding. - Vasopressin - potent splanchnic
vasoconstrictor; decreases portal venous
blood flow and pressure. - Somatostatin: decrease splanchnic
blood flow indirectly; fewer side
effects. - Octreotide: Initial drug of choice for
acute variceal bleeding.
Endoscopic Therapy for Varices
- Endoscopic Sclerotherapy: complications
occur in 10-30% and include fever,
retrosternal chest pain, dysphagia,
perforation. - Endoscopic variceal ligation: becoming the initial intervention of
choice; success rates range from 80-100%
Balloon Tamponade
- Sengstaken-Blakemore tube
- Minnesota tube
- Alternative therapy for pts. who fail
pharmacologic or endoscopic therapy
TIPS
Transjugular inrahepatic portasystemic shunt
1st line treatment for bleeding esophageal
varices when earlier-mentioned methods fail
Success rates 90-100%
Significant complication is hepatic
encephalopathy
Surgical Intervention
- Liver transplantation
only definitive procedure for PH caused by cirrhosis - Shunts
- Totally diverting (end-side portacaval)
- Partially diverting (side-side portacaval)
- Selective (distal splenorenal shunt
Devascularization
 Surgical Shunts - Non Selective Shunts
1) End-to-side Portocaval Shunt.
2)-Side-to-side Portocaval Shunt.
3)-Large diameter Interposition Shunt (e.g.
Mesocaval Shunt).
4)-Central Splenorenal Shunt.
Surgical Shunts - Selective Shunts
1)-Distal Splenorenal Shunt ( Warren
Shunt)
2)-Small diameter Portocaval H-graft
Shunt.
Portosystemic Shunt:
Whipple introduced portocaval shunt in
1945 —- Reduce the variceal bleeding
& ascites
In non selective PS-shunt, post shunt
encephalopathy is common
Encephalopathy is higher
if (1)-Pt. Age >50Y
2)-Poor liver function(G-C)
3)-Previous history of encephalopathy