ARTERIOHEPATIC DYSPLASIA (ALAGILLES SYNDROME) - UNUSUAL HEPATIC ARCHITECTURE AND FUNCTION

被引：9

作者：

HALVORSEN, RA

GARRITY, S

KUNI, C

DUCRET, RP

LETOURNEAU, JG

BLOOMER, J

机构：

[1] UNIV MINNESOTA,SCH MED,DEPT RADIOL,MINNEAPOLIS,MN 55455

[2] UNIV MINNESOTA,SCH MED,DIV GASTROENTEROL,MINNEAPOLIS,MN 55455

来源：

ABDOMINAL IMAGING | 1995年 / 20卷 / 03期

关键词：

LIVER BILE DUCTS; ARTERIOHEPATIC DYSPLASIA; ALAGILLES SYNDROME; CT; ULTRASOUND; MRI; SCINTIGRAPHY;

D O I：

10.1007/BF00200391

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Background: Alagille's syndrome, also called arteriohepatic dysplasia, is a congenital anomaly consisting of hepatic, ocular, skeletal, and cardiac anomalies. The abdominal imaging findings were reviewed in eight patients with biopsy-proven Alagille's syndrome. One patient also had coexistent hepatocellular carcinoma. Methods: Seven right upper quadrant sonograms, six hepatic CT studies, five hepatobiliary imaging studies, two hepatic MRI examinations, and two sulphur colloid liver spleen radionuclide studies were reviewed. Results: The most striking abnormality was gross distortion of hepatic architecture. Five patients (63%) had marked external hepatic contour abnormalities, usually with either the entire liver or lobe having a predominately spherical shape. The portal vein was displaced by the spherical parenchymal component in four cases. Three other patients demonstrated marked hepatomegaly with no external contour abnormality. Hepatobiliary imaging studies demonstrated markedly prolonged excretion of the radiopharmaceutical in three of four patients examined. Conclusions: A diagnosis of Alagille's syndrome is suggested when a large, deformed and somewhat spherical liver is encountered, especially when hepatobiliary imaging studies demonstrate delayed excretion of radiopharmaceutical.

引用

页码：191 / 196

页数：6

共 15 条

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