CAROLI DISEASE CASE LÂM SÀNG Bệnh nhân nam 83 tuổi tiền sử khỏe mạnh vào viện vì đau bụng hạ sườn phải âm ỉ 5 ngày Bệnh nhân không sốt, đại tiểu tiện bình thường CAROLI DISEASE Kiều Thị Huyền NT45 Car.
CASE LÂM SÀNG Bệnh nhân nam 83 tuổi tiền sử khỏe mạnh vào viện đau bụng hạ sườn phải âm ỉ ngày Bệnh nhân không sốt, đại tiểu tiện bình thường CAROLI DISEASE Kiều Thị Huyền-NT45 Caroli disease is an autosomal recessive diseass It is associated with polycystic kidney disease, medullary sponge kidney So looking at the kidneys can sometimes help you make this diagnosis The hallmark of Caroli disease is intrahepatic duct dilatation The duct dilatation in Caroli disease is due to a congenital malformation of the ductal plate, which is the precursor of the intrahepatic bile ducts Embryologically each bile duct begins as a single layer of cells that surrounds a portal vein This layer then duplicates Portions of this double layer fuse and resorb leaving unfused portions that become the bile ducts In the normal situation each portal vein is surrounded by interconnecting bile ducts However if the patient has ductal plate malformation, the bile ducts are too numerous and they are ectatic Clinical presentation Caroli disease presents with right upper quadrant pain, recurrent cholelithiasis, and cholangitis with fever and jaundice A very important sign is the central dot sign The central dot corresponds to the portal vein that is surrounded by dilated bile ducts Extrahepatic duct dilatation is present in 53% of cases, secondary to cholangitis and stone or sludge passage These are secondary findings, that are not part of the primary disease When there is extensive fibrosis, these patients can develop cirrhosis over time The cholangiogram is important in the work up of these patients, because obstruction has to be excluded In some of the cases of Caroli disease the imaging findings may simulate a cystic neoplasm Complications Intraductal stones Cholangitis and Abscess Livercirrhosis Cholangiocarcinoma Treatment and prognosis Prognosis is generally poor. If the disease is localized, segmentectomy or lobectomy may be offered In diffuse disease management is generally with conservative measures; liver transplantation may be an option ... Bệnh nhân nam 83 tuổi tiền sử khỏe mạnh vào viện đau bụng hạ sườn phải âm ỉ ngày Bệnh nhân khơng sốt, đại tiểu tiện bình thường CAROLI DISEASE Kiều Thị Huyền-NT45 Caroli disease is... associated with polycystic kidney disease, medullary sponge kidney So looking at the kidneys can sometimes help you make this diagnosis The hallmark of Caroli disease is intrahepatic duct dilatation... diagnosis The hallmark of Caroli disease is intrahepatic duct dilatation The duct dilatation in Caroli disease is due to a congenital malformation of the ductal plate, which is the precursor of the