Nemoto Satoshi, Ariizumi Shun-Ichi, Kotera Yoshihito, Omori Akiko, Yamashita Shingo, Kato Taka-Aki, Egawa Hiroto, Yamamoto Masakazu
Department of Gastroenterological Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Surg Case Rep. 2020 May 19;6(1):104. doi: 10.1186/s40792-020-00866-1.
The Fontan procedure has been widely accepted for children with single ventricle physiology and guarantees survival rates of approximately 80% at age 20 years. However, there have been cases of Fontan-associated liver disease (FALD) caused due to congestion, along with recent reports of the development of hepatocellular carcinoma (HCC) in younger patients with FALD. The literature consists of only five previous case reports of patients who underwent hepatectomy for HCC due to poorer cardiac function and liver cirrhosis caused due to congestion.
The patient was a 37-year-old woman who presented with epigastralgia. Computed tomography (CT) revealed a liver tumor, 8 cm in diameter, in the caudate lobe. Liver damage was A, with an indocyanine green retention rate of 6% at 15 min. The levels of alpha-fetoprotein (AFP) and protein induced by vitamin K antagonists-II (PIVKA-II) were elevated to 81,663 ng/ml (normal < 10 ng/ml) and 238 mAU/ml (normal < 40 mAU/ml), respectively. Left ventricular ejection fraction was 56%, and central venous pressure (CVP) was 12 mmHg. Left hepatectomy and caudate lobe resection were successfully performed in the reverse Trendelenburg position which reduced the CVP. The total operation duration was 450 min, with a total blood loss of 3200 ml. The patient's postoperative course was uneventful, and she is still alive 16 months after surgery.
First left hepatectomy with caudate lobectomy during reverse Trendelenburg position which reduced the CVP was performed in a patient with HCC and FALD.
Fontan手术已被广泛应用于单心室生理的儿童，可保证20岁时约80%的生存率。然而，曾有因充血导致Fontan相关肝病（FALD）的病例，近期也有年轻FALD患者发生肝细胞癌（HCC）的报道。文献中仅有5例先前的病例报告，这些患者因心脏功能较差和充血导致肝硬化而接受了HCC肝切除术。
患者为一名37岁女性，表现为上腹部疼痛。计算机断层扫描（CT）显示尾状叶有一个直径8厘米的肝肿瘤。肝脏损伤为A级，15分钟时吲哚菁绿潴留率为6%。甲胎蛋白（AFP）和维生素K拮抗剂-II诱导蛋白（PIVKA-II）水平分别升高至81,663纳克/毫升（正常<10纳克/毫升）和238毫国际单位/毫升（正常<40毫国际单位/毫升）。左心室射血分数为56%，中心静脉压（CVP）为12毫米汞柱。在头低脚高位下成功进行了左肝切除术和尾状叶切除术，该体位降低了CVP。总手术时间为450分钟，总失血量为3200毫升。患者术后恢复顺利，术后16个月仍存活。
对一名患有HCC和FALD的患者在头低脚高位下进行了首次左肝切除术加尾状叶切除术，该体位降低了CVP。
