CAROLI DISEASE chẩn đoán hình ảnh bệnh caroli

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 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

 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 ductsHowever if the patient has ductal plate

malformation, the bile ducts are too

numerous and they are ectatic

 Caroli disease presents with right upper

quadrant pain, recurrent cholelithiasis, and cholangitis with fever and jaundice

Clinical presentation

 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

 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 

Treatment and prognosis