Hepatosplenomegaly Flashcards
What is the normal liver span at 2 months?
4cm
What is the normal liver span at 12 years?
9 cm
What is the normal liver span in an adult?
12-15 cm
What does the presentation of jaundice and ascites point to?
Portal hypertension
What are the functions of the liver?
- Bile production and excretion
- Metabolizes fats, proteins and carbohydrates
- Storage of nutrients( vitamins, minerals,glycogen)
- Excretes waste products into bile(bilirubin, cholesterol, drugs, toxins)
- Innate and adaptive immunity
What are the 8 causes of hepatosplenomegaly?
- Inflammation/infection
- Reticuloendothelial hyperplasia
- Storage/metabolic disorder
- Biliary obstruction
- Venous congestion
- Fat accumulation
- Space occupying lesions
- Miscellaneous
What are the inflammatory/infection causes of hepatosplenomegaly?
- TORCHES
- Viral-HIV, Hepatitis B
- Bacterial- TB, Typhoid
- Parasite-bilharzia, malaria
What tests would you do to test causes of inflammation?
- FBC
- ESR
- Mantoux
- LFT
- Blood culture
What are the causes of reticuloendothelial hyperplasia?
- Septicaemia
- Malignancy
- lymphoma, leukemia, neuroblastoma - Granuloma(TB)
What are the causes of storage/metabolic disease?
- Glycogen storage disease
- Lipid storage disease
- Galactosemia
What are the causes of biliary obstruction?
- Biliary atresia
2. choledochal cyst
What are the causes of venous congestion?
- Cardiac failure
- Pericardial effusion
- Constrictive pericarditis
- Budd Chiari syndrome
What special investigations would you do in venous congestion?
- Chest X-ray
- ECG
- Echocardiogram
What are the causes of fat infiltration?
- Uncontrolled DM
- Malnutrition
- Hepatotoxic drugs
- Reye’s syndrome
What tests would you do check for fat infiltrates?
- LFT
2. Serum proteins
What causes the space occupying lesions?
- Neoplasm
2. Absess
What tests would you do in space occupying lesions?
- Alpha feto protein
2. Abdominal ultrasound
What is cholestasis?
Stasis of bile
When should infants with jaundice be evaluated for cholestasis?
After 14 days
What is regarded as abnormal in cholestasis?
The conjugated bilirubin value of more than 1mg/dL If the the total bilirubin is less than 5 OR if the conjugated bilirubin is more than 20% of the total
What are the extra-hepatic causes of cholestasis?
- Biliary atresia
- Choledochal cyst
- Neoplasm
- Gallstones
5 Neonatal sclerosing cholangitis
What are the intra-hepatic causes of cholestasis?
- idiopathic
- Alagille syndrome
- Hepatitis
What is the presentation of cholestasis caused by bile retention or regurgitation?
- Pruritis
- Xanthomata
- Jaundice
What is the presentation of cholestasis caused by portal hypertension?
- Hypersplenism
- Ascites
- Varices
What is the presentation of cholestasis caused by reduced bile delivery to small intestine?
Malabsorption
- Malnutrition
- Steatorrhea
- Rickets caused by vitamin d malabsorption
What lab tests are usually done in these children?
- FBC
- LFT
- Urine and blood culture
- Coagulation studies
What would we see on sonar if biliary atresia?
The inability to visualize the gall bladder and triangular cord sign
What is the most definitive test?
Liver biopsy
What is biliary atresia?
- an inflammatory,idiopathic disease of the bile ducts causing fibro-obliteration.
- leads to bile obstruction and then to liver failure
How do children with biliary atresia present?
They are jaundiced, with acholic stools and dark urine
What do we find on liver function tests of children with biliary atresia?
- Elevated levels of conjugated bilirubin
- Elevated GGT
- Elevated ALP to more than 5 times the normal
If left untreated what is the life expectancy of biliary atresia?
2 years
What do you find on biopsy of the liver?
Biliary cirrhosis
How can we classify biliary atresia?
- Post natal form(presents with isolated biliary atresia)
2. Fetal form(associated with other congenital malformations like meal rotation, asplenia/polysplenia)
What surgical procedure can we do for patients with biliary atresia?
A kasai procedure where the small intestine is connected to the liver directly
What is the success of the kasai procedure based on?
- The age of presentation
- Size of bile duct remnants
- Experience of the centre performing the procedure
What is the ideal age to do a kasai procedure?
45-60 days
What is a complication of doing a kasai procedure?
-ascending cholangitis
