Objective:To explore the clinical,imaging and pathological characteristics of congenital hepatic fibrosis(CHF) in children in order to improve radiologists' capability to recognize and diagnose the disease.Methods:Clinical,pathology and imaging data of 15 children with CHF diagnoses by pathology were retrospectively collected.All 15 cases underwent ab-dominal CT scan with contrast-enhancement,while 5 cases additionally underwent MRCP and MRI scan,and 2 cases under-went MRI scan with contrast-enhancement.Results:Among the 15 cases of CHF were 4 males and 11 females,and a medi-an age of 6 years (1.6～14 years).One case was the biliary type,and 14 cases were portal hypertension.One case of biliary type revealed simply a gallbladder stone.Fourteen cases were classified as portal hypertension.Six cases had imbalanced left and right lobe volumes.Six cases showed high signal intensity in the T2WI of the Glisson system.Fourteen cases of associated symptoms of portal hypertension and four cases of portal vein cavernous degeneration.Thirteen cases showed mild dilation of intrahepatic bile ducts,including 5 cases suggestive of Caroli syndrome.Ascites occurred in 7 cases.Twelve cases had an en-larged spleen.Nine cases had abnormality in the kidney.Six cases had polycystic kidneys,and 2 cases had medullary sponge kidneys;1 case only showed multiple small cysts in the right kidney.Conclusion:CHF in children frequently appears as portal hypertension.Imaging findings include an imbalance proportion of the left and right lobes of the liver,which is fre-quently accompanied by mild dilatation of the bile duct and portal hypertension.If abnormalities in the kidneys are found during the examination,CHF should be considered seriously,and pathology is required for diagnosis.
万方数据
维普
