Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, Toronto, ON, M5G 2N2, Canada.
Department of Medical Imaging, St. Michael's Hospital, University of Toronto, 30 Bond Street, Toronto, ON, M5B 1W8, Canada.
Abdom Radiol (NY). 2020 Jan;45(1):188-202. doi: 10.1007/s00261-019-02119-8.
To describe liver imaging findings and complications on computed tomography (CT) or magnetic resonance imaging (MRI) in adults with Fontan palliation and investigate whether imaging features show correlations with clinical and physiological parameters.
Our Institutional Review Board approved this retrospective study. Two blinded abdominal radiologists reviewed abdominal CT (n = 21) and MRI (n = 16) images between September 2011 and October 2017 in 37 adults (median age 27 years, interquartile range 21-36 years, 14 males [38%]) with a Fontan palliation (median post-Fontan duration 22 years, interquartile range 19-28 years). Correlation between CT/MRI findings and clinical parameters including laboratory results within 6 months of CT/MRI examinations was assessed by Spearman's rank correlation coefficient.
Lobulated hepatic surface and blunt hepatic edge were seen in 92% (34/37) and 95% (35/37) of patients, respectively. Surface nodularity was noted in 32% (12/37). In 7 patients, there were 11 hepatic nodules which showed arterial-phase hyperenhancement and washout. Among them, 2 were biopsy-proven hepatocellular carcinomas (HCCs), and the remaining 9 were focal nodular hyperplasia (FNH)-like nodules. Suprahepatic inferior vena cava (IVC) diameter showed positive correlations with post-Fontan duration (p < 0.01), serum gamma-glutamyl transferase (p < 0.01), and total bilirubin (p < 0.01).
The livers in post-Fontan adults show a unique morphology of blunt edge and lobulating surface with occasional nodularity. There is a diagnostic challenge in distinguishing HCCs from FNH-like nodules in post-Fontan population due to overlapping imaging findings. Suprahepatic IVC diameter is a potentially useful imaging marker that reflects hepatic dysfunction in Fontan palliation.
描述法乐四联症（Fontan）姑息术后成人患者的计算机断层扫描（CT）或磁共振成像（MRI）的肝脏影像学表现和并发症，并探讨影像学特征是否与临床和生理参数相关。
本回顾性研究经机构审查委员会批准。两位腹部放射科医生于 2011 年 9 月至 2017 年 10 月间对 37 例（男 14 例[38%]，中位年龄 27 岁，四分位间距 21-36 岁）接受 Fontan 姑息术的成人患者的 21 例 CT 和 16 例 MRI 图像进行了盲法阅片。通过 Spearman 秩相关系数评估 CT/MRI 表现与 CT/MRI 检查前 6 个月内的临床参数（包括实验室结果）之间的相关性。
92%（34/37）和 95%（35/37）的患者肝脏表面呈分叶状，95%（35/37）的肝脏边缘呈钝圆状。32%（12/37）的患者肝表面有结节状突起。7 例患者共有 11 个肝结节，呈动脉期强化和廓清。其中，2 个为肝细胞癌（HCC），其余 9 个为局灶性结节性增生（FNH）样结节。肝静脉上腔静脉（IVC）直径与 Fontan 术后时间（p<0.01）、血清γ-谷氨酰转移酶（p<0.01）和总胆红素（p<0.01）呈正相关。
Fontan 姑息术后的成人肝脏呈现出钝圆状和分叶状的独特形态，偶尔伴有结节状突起。由于影像学表现重叠，Fontan 人群中 HCC 与 FNH 样结节的鉴别具有一定的诊断挑战性。肝静脉上腔静脉（IVC）直径是反映 Fontan 姑息术肝功能的一种潜在有用的影像学标志物。
