Lithotripsy is a medical procedure wherein shock waves are used to break up kidney stones, ureter or bladder. Extra corporeal shock wave lithotripsy is the most commonly used type. The shock wave is termed extra corporeal as the shock wave is generated outside the body. It is a non-invasive technique. This procedure is used when the stone is too large to pass out on its own or if the stone is stuck in the ureter.
Prior to the treatment the following is followed:
Patient is made to lie down on a comfortable cushion/bed (usually water-filled). A mild sedative, pain killer and antibiotics are administered before the procedure so as to prevent any kind of discomfort, pain or infection. High energy sound waves pass through the body until they hit upon the kidney stone. The machine through which the waves is passed is called as the lithotripter. The kidney stone is broken into several pieces by the wave. The broken stone debris is called gravel. This gravel passes out while urinating. Usually there is no damage to skin or other internal organs as the shock waves are not focused on them. Generally after lithotripsy, people tend to bleed while urinating. This is common and will stop on its own. People who have undergone the procedure should drink plenty of water so as to flush the gravel out. A few patients may report abdominal pain which subsides on its own after a few days. If the symptoms persist, it is suggested that the patient visit the physician.
Lithotripsy should not be performed on people with skeletal deformities, persons with uncontrolled bleeding and pregnant women. Some of the possible side-effects include:
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.
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Bibliography / Reference
Collection of Pages - Last revised Date: January 27, 2023