Hepatomegaly

Question 1

What are the causes of isolated hepatomegaly?

Answer 1

Massive hepatomegaly
1. Malignancy - hepatocellular carcinoma
(Or more commonly secondary metastasis)
2. Alcoholic liver disease
3. Polycystic liver disease

Mild to moderate
(All causes of massive included)
4. Cirrhosis - NASH, alcoholic cirrhosis
5. Autoimmune - PBC, PSC, AIH
6. Hepatic congestion - CCF, constrictive pericarditis, restrictive cardiomyopathy
7. Infectious - hepatitis (Hep B, Hep C), toxoplasmosis, hydatid, liver abscess
8. Infiltration - amyloidosis, sarcoidosis, glycogen storage disease
9. Vascular - Budd-Chiari, sickle cell
10. Tertiary syphilis
11. Obstetric liver disease

Question 2

Which tumours commonly spread to the liver?

Answer 2

1. Commonest - colorectal ca
2. Oesophageal
3. Lung
4. Breast
5. Renal
6. Endometrial
7. Bone tumours
8. Sarcomas

Question 3

What are the infective causes of acute hepatitis?

Answer 3

Hepatitis A
Hepatitis B
Hepatitis C
Hepatitis E
Epstein-barr virus (EBV)
Cytomegalovirus (CMV)
Toxoplasmosis
Herpes simplex virus

Question 4

Approach to hepatomegaly

Answer 4

1. Nutritional status
   - Cachetic - cirrhosis, malignancy
   - Normal - infectious, alcohol without cirrhosis, early malignancy, congestion, vascular

1A. Anaemia - chronic liver disease, sickle cell anaemia, malignancy
1B. Jaundice

2. Examination of hepatomegaly
   - Normal liver span: 10.5cm in men, 7cm in women
   (upper border via percussion in right midclavicular line, lower border via palpation)
   - Tenderness: acute hepatic enlargement
   - Surface and edge: smooth, regular or irregular/nodular
   - Consistency: soft, firm or hard
   - Pulsatility and bruit - alcoholic, HCC, portal hypertension venous hum

2A. Sister Mary Joseph (SMJ) nodule over the umbilicus
- Gastric or colon adenocarcinoma, HCC, lymphoma deposit

3. Signs of liver cirrhosis, portal hypertension, ascites
4. Abdominal scars - paracentesis, liver biopsy
5. Lymphadenopathy - infection, malignancy
6. Examine JVP if cirrhosis, ascites present
7. Ask to examine other systems - thyroid, breast, pelvis, lungs

Question 5

What are the benign liver tumours?

Answer 5

1. Cavernous haemangioma - women of childbearing age
2. Hepatic adenoma
3. Focal nodular hyperplasia - women of childbearing age

Question 6

What are the hepatic manifestation of sickle cell anaemia?

Answer 6

1. Gallstone disease - chronic haemolysis causing pigment stones
2. Sickle hepatic crisis - sickle thrombosis causing sinusoidal obstruction
   > RUQ pain, jaundice, tender hepatomegaly
3. Intrahepatic cholestasis - sickle thrombosis in sinusoids causing swelling, intrahepatic obstruction
4. Iron overload - blood transfusion
5. Risk of viral hepatitis from blood transfusion

Question 7

What is Budd Chiari Syndrome?
What are the causes?
What are the clinical manifestations?
How to confirm diagnosis and treatment?

Answer 7

Obstruction to hepatic venous outflow
Can occur at any level: hepatic venules, veins or inferior vena cava
Commonly due to thrombosis - causing stasis, congestion and damage to hepatic parenchymal cells

Causes:
1. Myeloproliferative disease
2. Thrombophilias - protein C/S deficiency, ATIII, FV leiden mutation, prothrombin gene mutation
3. APLS
4. Paroxysmal nocturnal haemoglobinuria

Clinical manifestations:
1. Jaundice
2. Abdominal pain and hepatomegaly
3. Hepatic encephalopathy
4. May not have ascites - development of hepatic venous collateral circulation

Diagnosis:
1. US Doppler hepatic vein

Treatment:
1. Anticoagulation
2. Medical management of ascites, encephalopathy, liver failure
3. Emergency - thrombolysis, angioplasty
4. Fulminant liver failure -> liver transplant

Question 8

Pathophysiology of hepatomegaly

Answer 8

1. Inflammation (hepatitis)
   - Hepatocellular swelling, oedema of interstitial space, cholestasis
   - Viral hepatitis, alcohol hepatitis, autoimmune, DILI, bacterial infections
2. Vascular congestion
   - Impeded outflow with blood backed up causing hepatic sinusoids and central veins dilatation, potentially fibrotic
   - Right heart failure, constrictive pericarditis, Budd-Chiari syndrome, veno-occlusive disease
3. Infiltrative
   - Substances or cells accumulation displaces normal architecture
   - Steatohepatitis (NASH), glycogen storage disease, Wilson’s disease, haemochromatosis, amyloidosis
4. Malignancy
   - HCC, cholangioca, metastasis, lymphoma, leukaemia
5. Extramedullary haematopoiesis
   - Myelofibrosis, thalassaemia
6. Biliary obstruction (cholestasis)
   - Bile flow blockage causes hepatocyte swelling, ductular proliferation, inflammation
   - CBD stone, pancreatic tumour, cholangioca, PBC, PSC, biliary atresia
7. Cellular regeneration/hyperplasia
   - Reactive hyperplasia and regeneration in response to injury
   - Early stages of liver cirrhosis