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Caroli's Syndrome

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.


Cholecystectomy

Cholecystectomy or Gallbladder removal surgery is now commonly carried out by laparoscopic method. This has greatly reduced recovery time over the traditional open surgery. Laparoscopic Cholecystectomy is performed with special tools inserted through small incisions made on the abdomen. A tiny video camera is also inserted to aid the process. Typically Cholecystectomy is resorted in cases where the patient suffers gallstones in the gallbladder or bile duct. Severe Pancreatitis or Cholecystitis (Severe pain coupled with rigidity in the upper right abdomen owing to the infection or inflammation of the gallbladder) are other reasons for resorting to a Cholecystectomy. Some patients and conditions are not suitable for a laparoscopic Cholecystectomy and in these cases, the surgeon opts for the open incision method. Cholecystotomy is a surgical procedure where the gallbladder is opened for removal of the gallstones and the excess bile is drained - but the gallbladder itself is not removed. The actual surgical incision into the gallbladder is called as Cholelithotomy.


Cholecystectomy complications

With laparoscopic Cholecystectomy, a patient can leave the hospital within a couple of days. While this is a relatively simple procedure, the possible complications include blood clots, bile leakage, injury to bile duct and infection. Sometimes, a gallstone might still remain within the bile duct and might get pushed into the central bile duct. In case of injury to bile duct, complex corrective surgery has to be done. For gallstones still remaining in the bile duct, an endoscopic retrograde cholangiopancreatography (ERCP) is conducted. After laparoscopic cholecystectomy, patients might experience bloating and diarrhea. Some patients notice indigestion and abdominal pain. Jaundice might be noticed in a few.


Diet after gallbladder removal

After gallbladder removal surgery, many a patient suffers diarrhea for a few months till the bile flow is regulated. Remember that there is no gallbladder to store the bile and release it when necessary. Now, in the absence of a gallbladder, the bile flows directly into the small intestine. Here it is used for the digestion of fats. But some of it might flow into the large intestine causing irritation and resulting in diarrhea. Unregulated bile supply might lead to improper digestion of fat. It is not surprising to note that the fat content of the stool is higher in those who have had gallbladder surgery. A low fat diet must be followed after gall bladder removal. Fluids and high fiber diet help in preventing constipation or diarrhea post gall bladder surgery. The patient may experience burping and feeling of fullness coupled with gas.


  • Eating smaller meals at more frequent intervals helps.
  • Add new foods slowly and watch out for any symptoms.
  • Pack in lean protein, poultry, fish and low-fat dairy into your meals.
  • Caffeine tends to worsen abdominal gas and diarrhea in some patients after Cholecystectomy.
  • Limit your intake of sweets, cake, cookies and chocolate bars. They might result in intestinal distress.

Opt for whole grains and generous servings of fruits and vegetables.Ensure that low fat dairy products are used post gall bladder surgery. Avoid convenience foods and fast foods, as they tend to pack in hidden fats. Limit the use of oil to about 2-3 tsp. a day.



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Collection of Pages - Last revised Date: November 11, 2019