"Pathologically, Caroli disease and Caroli syndrome are ciliopathies, belonging to the spectrum of fibropolycystic liver disease which results from in utero malformation of the ductal plate 4. There is a high association with fibrocystic anomalies of the kidneys which share the same genetic defect (PKHD1 gene, chromosome region 6p12.3–p12.2) 15."
"T1: hypointense dilatation of IHBD"
"T2: hyperintense"
"T1 C+ (Gd): enhancement of the central portal radicles within the dilated IHBD 1"
"MRCP: demonstrates continuity with the biliary tree"
"Localised Caroli disease"
Expected headings
"Associations"
"Localised Caroli disease"
"Complications"
"Pathologically, Caroli disease and Caroli syndrome are ciliopathies, belonging to the spectrum of fibropolycystic liver disease which results from in utero malformation of the ductal plate 4. There is a high association with fibrocystic anomalies of the kidneys which share the same genetic defect (PKHD1 gene, chromosome region 6p12.3–p12.2) 15."
"The prognosis is generally poor. If the disease is localised, segmentectomy or lobectomy may be offered. In diffuse disease management is generally with conservative measures; liver transplantation may be an option 1."
"The prognosis is generally poor. If the disease is localised, segmentectomy or lobectomy may be offered. In diffuse disease management is generally with conservative measures; liver transplantation may be an option 1."