Symptoms and Syndromes
11 Hepatomegaly and splenomegaly
Page:
1 Hepatomegaly 210
1.1 Definition 210
1.2 Pathogenesis 210
1.2.1 Replication of cells 210
1.2.2 Enlargement of cellular structures 210 1.2.3 Augmentation of the extracellular space 210
1.2.4 Local processes 211
1.3 Causes 211
1.4 Diagnosis 211
1.4.1 Subjective symptoms 211
1.4.2 Clinical findings 211
1.4.3 Methods of examination 211
2 Splenomegaly 211
2.1 Normal values of the spleen 211
2.2 Definition 212
2.3 Pathogenesis and causes 213
2.4 Functional sequelae 213
2.5 Therapy 213
앫 References (1⫺40) 213
(Figures 11.1 ⫺11.2; tables 11.1⫺11.2)
1 Hepatomegaly
1.1 Definition
Hepatomegaly is present if (1.) palpation locates the lower border of the right lobe of liver to be more than 2 cm (1 ⫺2 finger breadths) below the left costal arch (MCL lateral to rectus abdominis) (caution:
phrenoptosis), (2.) the absolute liver dullness on per- cussion is more than 14 cm, or (3.) the longitudinal diameter of the liver in the MCL is greater than approximately 15 cm in the sonogram.
Hepatomegaly is a cardinal symptom in a number of liver diseases or a concomitant reaction of the liver in various extrahepatic or systemic diseases. When detected, differential diagnostic clarification is al- ways necessary.
Hepatomegaly can affect the entire liver as a diffuse en- largement or only a certain region of the liver as a cir- cumscribed increase in volume. • Under clinical con- ditions, the liver size is ascertained by the combined ap- plication of palpation (to determine the inferior border of the liver) and percussion (to determine the border be- tween the liver and lungs). (s. pp 77, 79) Determination of the liver size by sonography is considerably more pre- cise. • It is also necessary to assess the liver consistency (soft, elastic, firm, compact, hard), the liver surface (smooth, protuberant), the tenderness on pressure, and the sonographically detectable internal structure (homo- geneous, inhomogeneous, formation of foci, enlarged bile ducts or vessels). The density of the normal liver in the CT is 60 ⫾6 HU. (s. p. 171)
1.2 Pathogenesis 1.2.1 Replication of cells
A diffuse enlargement of the liver can be caused by cell replication.
(1.) Replication of hepatocytes in the form of excessive hyperplasia can occur occasionally after extensive parenchymal necrosis or partial liver resection. How- ever, this does not generally cause a clinical discernible form of hepatomegaly.
(2.) In systemic haematological diseases, the liver is usually involved in extramedullary haematopoiesis. This can result in hepatomegaly.
(3.) Diffuse enlargement of the liver can also be brought about by lymphohistiocytic cell infiltrations. This gener-
ally involves inflammatory reactions to viral or bac- terial diseases.
(4.) Diffuse hepatomegaly is also expected to occur as a result of malignant cell growth.
1.2.2 Enlargement of cellular structures
An increase in the volume of sinusoidal cells and hepatocytes due to an enlargement of their cellular structures can be caused actively by proliferation or passively by storage processes.
(1.) Endothelia and Kupffer cells can be stimulated to considerable proliferation, so that in clinical terms hepa- tomegaly occasionally results.
(2.) Proliferation of the smooth endoplasmic reticulum due to the prolonged induction of the biotransform- atory system localized at this site as a result of toxins, noxae or chemicals can bring about clinically and sono- graphically detectable hepatomegaly.
(3.) Hepatocellular storage of abnormal quantities of cholesterol, fat, glycogen, proteins, mucopolysaccha- rides, copper, iron, etc. occasionally leads to pro- nounced hepatomegaly. Hydropic swelling of the hepa- tocytes is also included in this category.
1.2.3 Augmentation of the extracellular space
Diffuse enlargement of the liver can also arise from augmentation of the extracellular space.
(1.) An increase in blood both in the sinusoids and in Disse’s spaces culminates in hepatomegaly. This can be witnessed particularly in cases of right heart failure, constrictive pericarditis, veno-occlusive disease and the Budd-Chiari syndrome. Inflammation-related hyper- aemia also occurs in acute viral hepatitis.
(2.) An enhanced formation of lymph or reduced lymph drainage can cause enlargement of the liver. Here fluid- filled cysts can also be regarded as a cause of hepato- megaly.
(3.) A disorder of the bile flow, particularly in infants, leads to extensive hepatomegaly.
(4.) An increase in the extracellular matrix due to col-
lagens, elastin, proteoglycans, glycoproteins, etc. also
produces various degrees of hepatomegaly.
Hepatomegaly and splenomegaly
1.2.4 Local processes
Circumscribed enlargement of the liver is precipitated by local lesions such as cysts, abscesses, parasites (e. g.
echinococcus) and benign or malignant tumours; this also results in diffuse enlargement of the liver in some cases.
1.3 Causes
A number of liver diseases are accompanied by an en- largement of the liver. The liver can also be involved in extrahepatic or systemic pathological diseases, possibly with the simultaneous development of hepatomegaly.
Differential diagnosis is extremely varied, necessitating a broad spectrum of investigations in individual cases.
(1 ⫺3, 6⫺8, 10, 11) (s. tab. 11.1) 1.4 Diagnosis
1.4.1 Subjective symptoms
Hepatomegaly is occasionally accompanied by a feeling of pressure or even tenderness in the right epigastrium.
(5) This tension pain of Glisson’s capsule mainly occurs with a rapid increase in the liver volume (acute viral hepatitis, acute congestive liver, etc.), whereas gradually developing hepatomegaly may be without pain. • The liver parenchyma itself is insensitive to pain. (s. pp 22, 78) Not to be associated with this is pain arising from a specific underlying disease in the liver region, which generally has no direct effect on liver enlargement, e. g.
malignant tumours, cholangitis, perihepatitis.
1.4.2 Clinical findings
Hepatomegaly can easily be ascertained by palpation and percussion; its size can also be well assessed. As- thenic patients may display simulated liver enlargement.
(s. pp 77, 78) • In addition to the typical dextral elevation of the diaphragm in hepatomegaly, there is often a down- ward displacement of intestinal loops as well.
䉴 Sonography is an indispensable investigative method for clarifying the following issues: (1.) differentiation be- tween diffuse and circumscribed hepatomegaly, (2.) as- sessment of the internal structure (homogeneous or in- homogeneous), (3.) determination of foci, cysts, nodes, etc., and (4.) confirmation of hepatomegaly or detection of a simulated enlargement of the liver due to extra- hepatic pathological diseases. (4, 9)
1.4.3 Methods of examination
In the majority of cases, the causality can be established on the basis of the hepatological findings or the prevail- ing disease. Often, however, definite clarification can only be achieved by additional investigative methods. • The indication for computer tomography (9) , and per-
1. Inflammatory liver diseases
⫺ acute viral hepatitis
⫺ acute autoimmune hepatitis
⫺ alcoholic hepatitis
⫺ concomitant hepatitis with infections
⫺ bacterial sepsis
⫺ miliary tuberculosis
⫺ parasitoses (e.g. echinococcus, toxocariasis, capillaria hepatica, schistosomiasis)
⫺ HIV infection
⫺ liver abscess
⫺ bacterial cholangitis, etc.
2. Chronic liver diseases
⫺ chronic hepatitis
⫺ liver cirrhosis
⫺ hepatic granulomatosis, etc.
3. Metabolic / toxic diseases
⫺ fatty liver
⫺ hepatic porphyrias
⫺ amyloidosis of the liver, etc.
4. Biliary diseases
⫺ biliary obstruction
⫺ primary biliary cholangitis
⫺ cholestasis
⫺ Byler’s syndrome, etc.
5. Vascular liver diseases
⫺ congestive liver,
⫺ peliosis hepatis
⫺ constrictive pericarditis
⫺ Budd-Chiari syndrome
⫺ veno-occlusive disease
⫺ arterial diseases, etc.
6. Tumours
⫺ benign liver tumours
⫺ malignant liver tumours
⫺ metastatic liver 7. Cystic liver
8. Congenital diseases
⫺ hereditary liver storage diseases (haemochromatosis,Wilson’s disease, amyloidosis, glycogenoses, lipopathies, tyrosinaemia, Zellweger’s cerebrohepatorenal syndrome, fructose intolerance,
α
1-antitrypsin deficiency, Burka’s syndrome, galactosaemia, Mauriac syndrome, etc.)
⫺ malformations of the liver or the bile ducts 9. Systemic haematological diseases
10. Systemic immunological diseases
Tab. 11.1: Causes of hepatomegaly
haps also angio-CT, should be checked first. As non- invasive procedures, these should be given priority over bioptic methods. (s. tab. 8.2) • Angiography may be indi- cated in cases where enhanced vascularity is suspected.
(s. tab. 8.3)
If biopsy proves necessary, thought must be given to whether a fine-needle biopsy or a liver biopsy is appro- priate. (s. tab. 7.3)
It should not be overlooked, however, that some indis- tinct liver findings can be diagnosed reliably and at little risk by laparoscopy, with or without biopsy. (s. tab. 7.11)
2 Splenomegaly
2.1 Normal values of the spleen
A normal spleen is 11 (10 ⫺14) cm in length, 7 (6⫺8) cm in width and 4 (3 ⫺4) cm in depth. The weight of the spleen varies considerably (< 100 g to > 250 g); a mean value of 150 ⫺170 (⫺180) g can be accepted. • The nor- mal diameter of the splenic artery is 4 ⫺5 mm, while that of the splenic vein is 8 ⫺14 mm with a normal mean value of about 10 mm. With a flow rate of 500 ⫺700 ml per minute, the blood flow through the spleen exceeds the arterial blood supply of the liver by a factor of almost 3. • The longitudinal axis of the spleen runs par- allel to ribs 9 ⫺11 from the upper dorsal to the lower ventral.
The size and volume of the spleen can be determined quite reliably by present-day methods of investi- gation, so that even slight increases in the size of the organ can be detected. However, in view of the wide spectrum of variation in the size of the “normal”
spleen, thought must be given to the following ques- tions: (1.) at what point should “splenomegaly” be diagnosed, and (2.) once a spleen has been classed as enlarged, should it be regarded as pathological.
Determination of the size of the spleen by palpation and percussion is most unreliable. (19) (s. p. 79) • X-ray exam- ination only permits determination of the longitudinal axis in 50 ⫺90% of cases, whereas measurement of the thickness is totally inadequate, and measurement of the width is not even possible.
䉴 The values of a normal or enlarged spleen can be recorded quite reliably in a sonogram. (16, 18, 23, 26) As- sessment is sometimes made more difficult by overlying ribs or as a result of air in the stomach or intestines.
Due to the uncertain sonographic identification of the upper pole of the spleen, it seems adequate to determine the maximum width and thickness in the transverse sec- tion. Normal values are 4 ⫻ 7 ⫻ 11 cm (whereby the transverse diameter is 4 ⫺5 cm and the longitudinal diameter is < 11 cm). (s. p. 135)
䉴 The size of the spleen can be determined with an accuracy of ⫾5% by computer tomography. The max- imum width and thickness can be measured directly from the transverse section. The length is calculated
from the difference between the upper and lower splenic poles.
Normal values are as follows: longitudinal extension 11 ⫺15 cm, longitudinal axis of the ellipsoid cross- section 7 ⫺10 cm, transverse axis 4⫺5 cm. The product of the normal mean values gives the CT spleen index, which correlates quite well with anatomical reality:
11 ⫻ 7 ⫻ 4 cm ⫽ 308 (160⫺440). In computer tomo- graphy, the normal density of the spleen is 46 ⫾ 12 HU or 50 ⫾ 8 HU.
䉴 Selective spleen scintigraphy by chemically or heat- denatured erythrocytes and
197Hg-BMHP-,
51Cr- or
99m