Tajima T, Honda H, Yanaga K, Kuroiwa T, Yoshimitsu K, Irie H, Kuwabara Y, Taguchi K, Masuda K
Department of Radiology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Pediatr Radiol. 2001 Aug;31(8):584-8. doi: 10.1007/s002470100510.
A boy with Alagille syndrome complicated by hepatic nodular hyperplasia (HNH) is reported. During pre-transplantation evaluation, CT and MRI revealed a large hepatic lesion with multiple small nodular lesions. Angiography demonstrated a large hypervascular lesion and CT arterial portography (CTAP) showed a portal perfusion defect corresponding to the lesion. CTAP also revealed large portal vein branches running through the lesion. Although hepatocellular carcinoma is known to accompany Alagille syndrome, HNH should also be considered when large vessels running through the lesion are demonstrated.
报告了一名患有阿拉吉耶综合征并伴有肝结节性增生（HNH）的男孩。在移植前评估期间，CT和MRI显示一个大的肝脏病变以及多个小结节性病变。血管造影显示一个大的高血运病变，CT动脉门静脉造影（CTAP）显示与该病变对应的门静脉灌注缺损。CTAP还显示大的门静脉分支穿过该病变。虽然已知肝细胞癌可伴有阿拉吉耶综合征，但当显示有大血管穿过病变时，也应考虑肝结节性增生。
