Block 8 Week 5
Abdominal distension : hepatosplenomegaly
Tutor : Prof DF Wittenberg MD FCP(SA) dwittenb@medic.up.ac.za
Objectives:
To be able to
 list the causes and discuss the investigation of patients presenting with
hepatomegaly and/or hepatosplenomegaly
 list the causes and discuss the principles of investigation and
management of patients suffering from chronic liver disease and portal
hypertension
Illustrative Case Report Case 4: O.N.
ON, a 9 year old girl, has a complaint of gradually progressive abdominal distension. This
has been present for at least 2 years. It is now so bad that she has difficulty lying down on a
flat bed, and becomes short of breath with exertion. She cannot play with her friends
anymore.
She has had a few episodes of epistaxis, and her stools have also been seen to be tinged
with bright blood.
There is no history of any disease. She has not been jaundiced. Her urine and stools are
apparently normal.
On examination, she is a thin girl with gross abdominal distension. She is not jaundiced. She
is fully conscious and cooperative. There are no signs of hepatic decompensation. There is no
lymphadenopathy. She has moderate pedal oedema. Some veins are visible on her
abdomen; these are evident between the upper abdomen and lower chest and the flow
seems to be from abdomen to chest.
Abdominal examination reveals a very large firm liver. This is firm in consistency. The spleen
is not palpable, but there is a large amount of ascites making palpation difficult. Apart from
the discomfort of tense abdominal distension, there is no pain, tenderness or rebound on
abdominal examination. There are no haemorrhoids on rectal examination.
Her heart and lungs are normal on examination. There is no pulsus paradoxus and her blood
pressure is normal.
Blood tests show a normal serum albumen and normal liver function tests. The enzymes are
normal.
Introduction
In order to be able to decide that a patient has hepatomegaly, one needs to
know the range of normal sizes at different ages.
The liver’s position in the abdomen is dependent on its relationships with the
diaphragm and the lung above it.
A paralysed diaphragm on the right hand side means that the liver is not
splinted downwards by the contraction of the diaphragm and therefore moves
higher into the chest. In such a case, the liver may not be well felt even
though it could be significantly enlarged. This can also happen if there is
collapse of a segment of the right lung, again pulling the diaphragm higher up
into the chest than normal.
A lung which is overfilled with air (air trapping) tends to push the diaphragm
down, therefore pushing the liver further into the abdomen than normal. In
such a case the lower margin of the liver may be palpated much further down
than expected, giving the impression of liver enlargement. This happens in
cases of asthma, bronchiolitis, air trapping with mucus plugging or foreign
body.
It follows therefore that the determination of the upper border of the liver is
an essential part of the examination of the liver size. This is done by
percussion of the chest downward from resonant to dull in the midclavicular
line opposite the 9th costal cartilage.
The bottom margin is then similarly identified by palpation or percussion, and
the distance between the two points is the perpendicular width of the liver,
the Liver Span.
This varies by age :
 5 cm at 2 months
 8 cm at 5 years
It is normal to be able to palpate the lower edge of the liver in children. In
the first 6 months of life, the liver may be palpated up to 3,5 cm below the rib
margin, thereafter 2 cm is usual. However, the span is much the better
assessment of true liver size.
Causes of hepatomegaly
It is useful to consider the normal liver architecture, list the causes of
enlargement or hyperplasia for each cell type or structure, and then list
abnormal cells or structures which might be involved:


Hepatocytes : Infiltration or storage with
Fat - Fatty change
Malnutrition, metabolic, toxic diseases
lipid storage
Mucopolysaccharides
Glycogen – Glycogen storage disease
Amyloid protein - Amyloidosis
Mineral - Copper, Iron
Hepatocyte invasion with virus – CMV, EBV, Hep B and A
Abnormal hepatocyte proliferation - cirrhosis
Blood cells :
Congestion and damming up of blood
Congestive cardiac failure
veno-occlusive disease
constrictive pericarditis
Abnormal accumulations
Extramedullary haemopoiesis

Reticulo-endothelial cells :
Generalised RES hyperplasia
HIV, Auto-immunity
Inflammatory cell infiltration
Hepatitis
Fibrosis and cirrhosis
Granulomata
Malignant infiltration
Leukaemia

Abnormal cells/conditions:
Metastases
Abscesses
Cysts
Causes of hepatosplenomegaly
Liver and spleen grossly enlarged
In general, these can be seen in one of the following categories:
1)
The same pathogenetic process happening in liver and spleen at
the same time: Look for a generalised disorder
Reticulo-endothelial hyperplasia
Infiltration with the same type of cells
Leukaemia
Gaucher/Niemann-Pick etc
Inflammatory cells and granulomata eg TB
2)
Disease of the liver with splenomegaly secondary to portal
Hypertension: look for evidence of liver disease/dysfunction
Cirrhosis and chronic liver disease
Hepatic fibrosis and portal hypertension
3)
Portal hypertension without significant liver disease
Hepatic or Portal vein obstruction
Look for evidence of portal hypertension
4)
Disease involving predominantly spleen
Removal of damaged red cells
Malaria
Haemolytic anaemia
Look for evidence of haemolysis
Case analysis :
This girl has the following problems:
Gross liver enlargement
Gross ascites
History of bleeding
The bleeding could be caused by either liver dysfunction with diminished clotting factor
synthesis, or alternatively portal hypertension with bleeding varices. In view of the normal
results on albumen and liver function tests, the second explanation is more likely. A
paracentesis of the abdomen would be expected to yield a transudate without inflammatory
cells and with a low protein content. In that case, the ascites is not due to peritonitis, but is
due to increased hydrostatic oncotic pressure secondary to portal hypertension.
The patient has a very big liver with normal liver function tests and enzyme values. This
rules out hepatitis. A big liver with associated ascites on the basis of increased hydrostatic
pressure is found in obstruction to venous return from the liver (Veno-occlusive disease, Budd
Chiari syndrome of hepatic vein obstruction, inferior vena cava obstruction or constrictive
pericarditis). She does not have the clinical signs of constrictive pericarditis. A clinical
diagnosis of veno-occlusive disease is made.
A radiological contrast study of the inferior vena cava demonstrated complete obstruction of
the hepatic vein. No venous drainage from the liver caused massive congestive hepatomegaly
and portal hypertension.
Task
Study the approach to hepatomegaly, chronic liver disease and portal
hypertension (C & W p 562 - 373