Hwang Sook Min, Jeon Tae Yeon, Yoo So-Young, Kim Ji Hye, Kang Ben, Choe Yon Ho, Cho Haeyon, Kim Jung Sun
Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, 135-710, Korea.
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, 135-710, Korea.
PLoS One. 2016 Feb 22;11(2):e0149681. doi: 10.1371/journal.pone.0149681. eCollection 2016.
To compare preoperative CT findings before liver transplantation between patients with Alagille syndrome (AGS) and those with end-stage biliary atresia (BA).
The institutional review board approved this retrospective study. Eleven children with AGS (median age, 19.0 ± 13.0 months; male to female ratio, 3:8) and 109 children with end-stage BA (median age, 17.9 ± 25.8 months; male to female ratio, 37:72) who underwent abdomen CT as candidates for liver transplant were included. CT images were reviewed focusing on hepatic parenchymal changes, vascular changes, presence of focal lesions, and signs of portal hypertension.
Hepatic parenchymal changes were present in 27% (3/11) of AGS patients and 100% (109/109) of end-stage BA patients (P < .001). The hepatic artery diameter was significantly smaller (1.9 mm versus 3.6 mm, P = 008), whereas portal vein diameter was larger (6.8 mm versus 5.0 mm, P < .001) in patients with AGS compared with patients with end-stage BA. No focal lesion was seen in patients with AGS, whereas 44% (48/109) of patients with end-stage BA had intrahepatic biliary cysts (39%, 43/109) and hepatic tumors (8%, 9/109) (P = .008). Splenomegaly was commonly seen in both groups (P = .082), and ascites (9% [1/11] versus 50% [54/109], P = .010) and gastroesophageal varix (0% [0/11] versus 80% [87/109], P < .001) were less common in patients with AGS than in patients with end-stage BA.
Fibrotic or cirrhotic changes of the liver, presence of focal lesions, and relevant portal hypertension were less common in patients with AGS than in patients with end-stage BA.
比较阿拉吉列综合征（AGS）患者和终末期胆道闭锁（BA）患者肝移植术前的CT表现。
本回顾性研究经机构审查委员会批准。纳入11例AGS患儿（中位年龄19.0±13.0个月；男女比例为3:8）和109例终末期BA患儿（中位年龄17.9±25.8个月；男女比例为37:72），这些患儿均作为肝移植候选者接受了腹部CT检查。对CT图像进行了回顾，重点关注肝实质改变、血管改变、局灶性病变的存在以及门静脉高压的征象。
27%（3/11）的AGS患者存在肝实质改变，而100%（109/109）的终末期BA患者存在肝实质改变（P<.001）。与终末期BA患者相比，AGS患者的肝动脉直径明显更小（1.9mm对3.6mm，P = 0.008），而门静脉直径更大（6.8mm对5.0mm，P<.001）。AGS患者未见局灶性病变，而44%（48/109）的终末期BA患者有肝内胆管囊肿（39%，43/109）和肝肿瘤（8%，9/109）（P = 0.008）。两组均常见脾肿大（P = 0.082），与终末期BA患者相比，AGS患者腹水（9%[1/11]对50%[54/109]，P = 0.010）和胃食管静脉曲张（0%[0/11]对8%[87/109]，P<.001）较少见。
与终末期BA患者相比，AGS患者肝脏的纤维化或肝硬化改变以及局灶性病变的存在和相关门静脉高压较少见。
