A very rare case: Caroli syndrome

Team of surgeons performed a five-hour operation

Don Nilo earns his living by working hard in the fields. But in the last few weeks, his health has gone increasingly downhill. Upper abdominal pain, general malaise and jaundice led the father of six to the Diospi Suyana Hospital. Ultrasound and magnetic resonance imaging examinations revealed massively dilated bile ducts in the liver, which were also filled with stones of varying sizes.

Dr. Hilbert de Vries, Dr. Lukas Steffen and Dr. Benjamin Zeier were able to help the 51-year-old a week ago. After removing the gallbladder, they cleared the stone material from the bile ducts. In doing so, they cleaned the ducts in the liver with a very fine endoscope.

The clinical picture corresponds to Caroli syndrome, in which the bile ducts become abnormally congested. The resulting bile stasis promotes stone formation and recurrent inflammation of the bile ducts.
The incidence of Caroli syndrome is 1:100,000. There is a genetic predisposition.

The operation was successful. /KDJ

MRI image. View from below into the patient: The large accumulations (white) are bile duct cysts in the liver tissue. The color black shows accumulations of stones.
In the middle of the picture you can see the endoscope (white), which disappears into the large bile duct at the bottom. Dr. Zeier used this access to remove the stones in the intrahepatic (in the liver) bile ducts.

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