The spleen is responsible for the production of humoral antibodies and is a reservoir for blood cells. In case of Splenomegaly, the functions associated with the spleen are hampered. Enlarged spleen or Splenomegaly generally indicates the presence of an underlying medical condition which has to be attended to immediately. Physicians palpate the patient and observe the size variation that is clinically correlated with Splenomegaly. This routine examination is followed by other diagnostic tests which include blood, urine and body fluid analysis to identify the presence of infection.
Symptoms of Splenomegaly
The occurrence of Splenomegaly is usually asymptomatic unless there is an underlying factor such as bacterial, viral or parasitic infection. In most cases, patients complain of upper abdominal discomfort or pain, decreased appetite, weakness because of anemia and susceptibility to infections.
Splenomegaly predominantly occurs because of infections. However other metabolic disorders such as Niemann pick and Gaucher's disease can also serve as diagnostic markers. The susceptibility of Splenomegaly is high among children and elderly persons. Enlarged spleen can be life threatening as it may lead to splenic rupture in the abdominal cavity.
Treatment options for Splenomegaly include treating the underlying infection by administration of antibiotics. Post operative patients must be given antibiotics such as penicillin to avoid relapse of infections. In case of splenic enlargement, contact sports such as football, wrestling, boxing must be avoided. Children must be given vaccinations for Pneumococci, Haemophilus influenza (Bacillus Influenzae) on schedule to avoid infections. Splenectomy is the surgical choice in cases of total spleen malfunction or rupture.
Jacques Caroli, a French physician reported Caroli's disease as a distinct clinical entity - that of an inherited condition – the bile ducts in the liver are widened and there are fibrous changes in the liver and cysts within the kidneys.
Caroli disease is of two types – the most common being the simple or isolated case with widening of the bile ducts, and the second type – a more complex one and the cause is known as Caroli's syndrome. Caroli affects females more than males.
Causes of Caroli's Syndrome
The cause is largely complex genetics in nature. The simple form of Caroli is autosomal dominant trait and the more complex form is an autosomal recessive trait. Congenital hypertension and hepatic fibrosis are linked to this common form. Liver failure and Polycystic kidney disease are also associated with Caroli.
ARPKD and PKHDI are gene linked diseases. While ARPKD is found mutated in patients with Caroli syndrome, PKHDI is found primarily in the kidneys with lower levels in the liver, pancreas and lungs and affects the liver and kidneys. There is a basic difference in the genetic pattern between Caroli disease and syndrome. By far, Caroli is a rare disease and affects about 1 in 1,000,000 people. There are more reported cases of Caroli's syndrome than Caroli's disease.
Symptoms of Caroli's Syndrome
Fever is the first symptom. This is followed by abdominal pain and hepatomegaly. In some, jaundice occurs. Nausea, vomiting, stomach pain, enlarged liver, kidney infection, gallstones are other signs. Whereas autosomal recessive diseases such as polycystic kidney disease, cholangitis, gallstone, biliary abscess, septicemia and liver cirrhosis, renal failure and cholangiocarcinoma - Cancer of the liver ducts, also exhibit Caroli disease. Research proves that those with Caroli are at 100 times more risk for cholangiocarcinoma than the general population. If relevant symptoms of the disease can be checked, Caroli can be diagnosed.
Morbidity is common due to complications and morbid conditions such as sepsis; cholangiocarcinoma should prompt the diagnosis of Caroli. In some, portal hypertension may also be present and this can result in conditions such as Splenomegaly - enlarged spleen and Hematemesis - vomiting of blood. These problems may severely affect the patient's quality of life.
Diagnosis of Caroli's Syndrome
Caroli nowadays is more frequently diagnosed and ultrasonography is the initial investigation of choice. CT is used when insufficient data is found in the USG. CT is an excellent way to demonstrate the extent of the disease. MRI is another tool that is used. However, plain radiography of the abdomen may very rarely reveal small bile duct calcification.
Treatment of Caroli's Syndrome
Treatment methodology for Caroli includes supportive care with antibiotics for cholangitis and Ursodeoxycholic acid for Hepatolithiasis - gallstones in the biliary ducts of the liver. Sometimes surgical resection is used in patients with monolabar disease. Orthotopic liver transplantation is done in patients who cannot be operated radically. If one segment with choledochal cyst is found, it can be surgically treated. In case of bilobar involvement, treatment is palliative with follow up and complications are detected and treated.
The spleen is one of the most important organs in the body. It enables the production of blood cells and also facilitates the removal of unwanted cells or worn out blood cells from the blood stream. The spleen is a significant promoter for erythropoiesis process. Hypersplenism is a complex condition associated with the functionality of the spleen in removing the blood cells. In case of hypersplenism, the activity of the spleen is increased leading to early destruction of the cells which are healthy. In some cases the spleen holds up platelets and healthy RBCs thereby leading to altered hematological functions in the body such as clotting and oxygen supply to various organs. Another possible cause of Hypersplenism is Splenomegaly which refers to enlargement of the spleen.
Clinical manifestations of Hypersplenism
The incidence of hypersplenism is closely associated with underlying conditions such as tuberculosis, malaria, cirrhosis, rheumatoid arthritis and polycythemia. The clinical manifestations of hypersplenism include susceptibility to bacterial infections and viral infections such as infectious mononucleosis. In most cases, hypersplenism is caused because of predisposing factors such as alcoholic liver cirrhosis.
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Bibliography / Reference
Collection of Pages - Last revised Date: March 5, 2024