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HEPATOSPLENOMEGALY
- Question:
- What is your diagnosis ??

- Answer:
- Most probably Bilharzial Hepatosplenomegaly, functionally compensated, no
complications; BECAUSE:

 Bilharzial: Past history of Bilharziasis.

 HSM: Clinically enlarged liver & spleen.
 Functionally compensated:
- Parenchymatous → No Liver cell failure.
- Vascular → No Portal hypertension.
 No complications:
- No ACD.
- No Bilharzial corpulmonale.

- Question:
- Do you expect to find manifestations of liver cell failure in a Bilharzial liver ??

- Answer:
- No:
 BECAUSE: in Bilharziasis there is only fibrosis ( no degeneration,
no regeneration, no loss of architecture ).
 UNLESS: there is associated viral hepatitis, especially HCV.

- Question:
- Which type of Schistosoma is blamed to cause corpulmonale ??

- Answer:
- S. mansoni: BECAUSE:
They reach the lungs with difficulty only in the presence of portal hypertension &
porto-systemic collaterals which allow the ova to bypass the liver to the lungs,
HOWEVER:
They cause more destructive lesions than S. haematobium.

- Question:
- What is meant by ACD ??

- Answer:
- A mild to moderate Microcytic or Normocytic anemia due to either:
chronic infection, inflammation or malignancy.

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- Question:
- What is the most important diagnostic feature of ACD ??

- Answer:
- A decreased serum iron with Normal or Increased serum Ferritin.

- Question:
- What are the manifestaions of portal hypertension in this patient ??

- Answer:
 Oesophageal varices: proved by upper endoscopy.
 Caput medusae: dilated veins around the umbilicus.
 Venous hum: soft systolic m heard over dilated para-umbilical vein.
 Splenomegaly.
 Ascites.

- Question:
- Does portal hypertension of Bilharziasis cause ascites ??

- Answer:
- Portal hypertension alone rarely causes ascites; it only localizes the transudate
to the peritoneal cavity, HOWEVER:
- In Hepatic Schistosomiasis: Ascites may develop late due to associated
HYPOALBUMINEMIA.

- Question:
- What causes associated HYPOALBUMINEMIA in Hepatic Schistosomiasis??

- Answer:

CAUSES OF ASSOCIATED HYPOALBUMINEMIA

1. Decreased intake: nutritional deficiency due to poverty.
2. Decreased absorption: malabsorption due to intestinal congestion.
3. Decreased production: due to associated cirrhosis in mixed pathology.
4. Increased loss:
 Bleeding: Bleeding oesophageal varices.
 Protein losing enteropathy: Congestive enteropathy & Colonic polyps.

- Question:
- What is the main presentation of Intestinal Bilharziasis ??

- Answer:

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- Bleeding per rectum ( Hematochezia ).

- Question:
- What are the causes of HEMATOCHEZIA ??

- Answer:
 Piles: the most common cause.
 Cancer rectum OR colon: the most serious cause.
 Inflammatory bowel disease: especially UC.
 Intestinal bilharziasis.
 Anal fissure.
 Bacillary dysentery.
 Colonic polyps: familial polyposis.
 Diverticulitis.

 TB of the colon.

- Question:
- How do you test for the presence of ascites ??

- Answer:
- Tense ascites: Transmitted thrill.
- Moderate ascites: Shifting dullness.
- Minimal ascites: Knee-elbow position.

- Question:
- What are the clinical complications of ascites ??

- Answer:
a) Pleural effusion:

 It is probably due to passage of fluid through defects in the diaphragm.

 It is especially common on the right side.

b) Signs of elevated diaphragm:

 Lung bases: diminished breath sounds & dullness.

 Cardiac apex: displaced upwards & outwards.
 Neck veins: congested pulsating neck veins.

- Question:
- What is the most common cause of ascites ??

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- Answer:
- LIVER CELL FAILURE.
- Question:
- What other common important causes of ascites that you know of ??
- Answer:
 Right sided heart failure.
 Portal hypertension ( Bilharzial ) asscociated with Hypoalbuminemia.
 HYPOALBUMINEMIA: Nephritic, Nephrotic, Nutritional.
 Miscellaneous: Meig’s syndrome, Myxoedma.
 EXUDATIVE ascites: TB peritonitis, SBP, Malignant ascites.
Pancreatic ascites.
 CHYLOUS ascites.
 HEMORRHAGIC ascites.

- Question:
- What is the SPOT DIAGNOSIS of a patient with ascites due to liver cirrhosis ??
- Answer:
- SPIDER MAN: combination of THIN LIMBS + PROTUBERANT ABDOMEN.

- Question:
- What are the signs of chronic increase in intra-abdominal pressure in ascites ??
- Answer:
 Subcostal angle: widening.
 Umbilicus: everted, shifted downwards, ± umbilical hernia.
 Divarication of the recti.

- Question:
- How can you feel the liver & spleen in the presence of ascites ??
- Answer:
- By the dippig method: in this case I will not comment on them; I’ll just say
whether they are felt or not.

- Question:
- What are the important causes of: rapidly developing ascites ??
- Answer:
 Acute fulminant liver cell failure.
 Acute Budd-Chiari syndrome.
Transudative ascites
 Acute portal vein thrombosis.
 Acute nephritic syndrome.

 TB peritonitis.
 SBP. Exudative ascites
 Malignant ascites.
 Pancreatic ascites.
Chylous ascites
 Rupture of Thoracic duct.

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 Hemorrhagic blood diseases or trauma. Hemorrhagic ascites

- Question:
- What are the causes & treatment of Refractory ascites ??

- Answer:
- Resistance to treatment may be due to:
 Lack of salt restriction: treated by adequate salt restriction.
 Severe hypoalbuminemia: treated by IV salt – free albumin.
 Dilutional hyponatremia: treated by fluid restriction & IV mannitol.
 Serious problems: as SBP, TB peritonitis or Malignant ascites:
treat the cause.

- Severe terminal cases may be treated by:

 Le Veen shunt ( peritoneo – venous shunt ):
- Technique: A catheter with one way valve is placed between the peritoneal
cavity and the SVC to drain the ascitic fluid into the circulation.

- Complications: Hypervolemia, pulmonary edema, infection & DIC.

 Ascites ultrafiltration & reinfusion:

- Ultrafiltration: removes the ascitic fluid & concentrates it.
- Reinfusion: returns the fluid to the patient IV.

 Hepatic transplantation.

- Question:
- What are the causes of HUGE SPLENOMEGALY ??

- Answer:
1. Severe portal hypertension: especially Schistosomiasis.

2. Myeloproliferative disorders: CML, PRV, Essential thrombocythemia,

Myelofibrosis.

3. Neoplastic: Lymphomas, Hairy cell leukemia.

4. Hematologic: Thalassemia major.

5. Infections: Chronic malaria, Kala-azar.

6. Inflammation: Sarcoidosis, Felty’s syndrome.

7. Infiltration: Amyloidosis, Gaucher’s disease.

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- Question:
- What are the clinical stages of Hepatic Schistosomiasis ??

- Answer:

CLINICAL STAGES OF HEPATIC SCHISTOSOMIASIS

1. Hepatomegaly.
2. Hepatosplenomegaly.
3. Shrunken liver & splenomegaly.
4. Shrunken liver, splenomegaly & ascites.

- Question:
- What are the investigations that you will ask for ??

- Answer:
1. Investigations for diagnosis of Schistosomiasis:
- Stool & urine analysis: for Schistosoma ova.
- Sigmoidoscopy & biopsy: for Schistosoma ova.
- Serology & skin tests: for Schistosoma antibodies.
- Liver biopsy: shows the typical granuloma & periportal fibrosis.

2. Investigations for diagnosis of Portal Hypertension:

e.g. upper GI endoscopy: for oesophageal varices.

3. Investigations for Liver Function Tests:

- NORMAL in pure Hepatic Schistosomiasis. - AFFECTED in mixed pathology.
- Hypoalbuminemia may be present .

4. Investigations for Liver Imaging: Abdominal ultrasonography & CT.

ALSO:

5. Investigations for Hypersplenism: to decide about splenectomy.

- Question:
- How would you treat such a case ??

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- Answer:
- Refer to the notes.
- DO NOT FORGET: TTT of Hypersplenism if present.