Rapp Jordan B, Bellah Richard D, Maya Carolina, Pawel Bruce R, Anupindi Sudha A
Department of Radiology, Temple University Hospital, Lewis Katz School of Medicine at Temple University, 3401 N. Broad St., Philadelphia, PA, 19140, USA.
Department of Radiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Pediatr Radiol. 2017 Feb;47(2):197-204. doi: 10.1007/s00247-016-3728-2. Epub 2016 Oct 28.
Children with Alagille syndrome undergo surveillance radiologic examinations as they are at risk for developing cirrhosis and hepatocellular carcinoma. There is limited literature on the imaging of liver masses in Alagille syndrome. We report the ultrasound (US) and magnetic resonance imaging (MRI) appearances of incidental benign giant hepatic regenerative nodules in this population.
To describe the imaging findings of giant regenerative nodules in patients with Alagille syndrome.
A retrospective search of the hospital database was performed to find all cases of hepatic masses in patients with Alagille syndrome during a 10-year period. Imaging, clinical charts, laboratory data and available pathology were reviewed and analyzed and summarized for each patient.
Twenty of 45 patients with confirmed Alagille syndrome had imaging studies. Of those, we identified six with giant focal liver masses. All six patients had large central hepatic masses that were remarkably similar on US and MRI, in addition to having features of cirrhosis. In each case, the mass was located in hepatic segment VIII and imaging showed the mass splaying the main portal venous branches at the hepatic hilum, as well as smaller portal and hepatic venous branches coursing through them. On MRI, signal intensity of the mass was isointense to liver on T1-weighted sequences in four of six patients, but hyperintense on T1 in two of six patients. In all six cases, the mass was hypointense on T2- weighted sequences. The mass post-contrast was isointense to adjacent liver in all phases in five the cases. Five out of six patients had pathological correlation demonstrating preserved ductal architecture confirming the final diagnosis of a regenerative nodule.
Giant hepatic regenerative nodules with characteristic US and MR features can occur in patients with Alagille syndrome with underlying cirrhosis. Recognizing these lesions as benign giant hepatic regenerative nodules should, thereby, mitigate any need for intervention.
阿拉吉耶综合征患儿需接受影像学监测检查，因为他们有发生肝硬化和肝细胞癌的风险。关于阿拉吉耶综合征肝脏肿块影像学表现的文献有限。我们报告了该人群中偶然发现的良性巨大肝再生结节的超声（US）和磁共振成像（MRI）表现。
描述阿拉吉耶综合征患者巨大再生结节的影像学表现。
对医院数据库进行回顾性检索，以查找10年间阿拉吉耶综合征患者肝脏肿块的所有病例。对每位患者的影像学检查、临床病历、实验室数据及可用的病理结果进行回顾、分析和总结。
45例确诊为阿拉吉耶综合征的患者中有20例进行了影像学检查。其中，我们发现6例有巨大局灶性肝肿块。所有6例患者均有巨大的肝中央肿块，超声和MRI表现非常相似，此外还有肝硬化特征。在每例病例中，肿块位于肝VIII段，影像学显示肿块使肝门处的主要门静脉分支分开，以及较小的门静脉和肝静脉分支穿过肿块。在MRI上，6例患者中有4例肿块在T1加权序列上与肝脏信号强度相等，但6例中有2例在T1上呈高信号。在所有6例病例中，肿块在T2加权序列上呈低信号。5例病例中肿块在增强后各期均与相邻肝脏信号强度相等。6例患者中有5例经病理证实导管结构保留，最终确诊为再生结节。
阿拉吉耶综合征合并潜在肝硬化的患者可出现具有特征性超声和磁共振表现的巨大肝再生结节。因此，将这些病变识别为良性巨大肝再生结节应可减少干预的必要性。
