Angelico Roberta, Lisignoli Veronica, Monti Lidia, Pariante Rosanna, Grimaldi Chiara, Saffioti Maria Cristina, Gagliardi Maria Giulia, Spada Marco
Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Department of Cardiology, Division of Grow Up Congenital Heart, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Int J Surg Case Rep. 2019;59:144-147. doi: 10.1016/j.ijscr.2019.05.029. Epub 2019 May 23.
A well-recognized long-term complication after Fontan procedure (FP), a complex cardiac surgery performed in patients with univentricular hearts, is the development of chronic liver disease and hepatocellular carcinoma (HCC). Due to the risk of cardiac and liver decompensation, liver resection of HCC is challenging and the laparoscopic approach has never been reported.
We present the first case of laparoscopic liver resection (LLR) of HCC in a 33-years-old girl with cardiac-related cirrhosis after FP. Intraoperatively, the pneumoperitoneum was established at 8-10 mmHg and adequate fluid infusion was given to maintain the cardiac preload. After an ultrasound-guided thermoablation along the free-tumor margin of the hepatic lesion, a full laparoscopic non-anatomical resection of the tumor in segment V was performed, without Pringle manouver and blood transfusion requirement. The cardiac function remained stable during the surgery and thereafter, and the post-operative course was uneventful.
HCC in chronic liver disease after FP is associated with high-risk mortality. Due to the complex hemodynamic changes after FP, open surgical resections often aren't feasible and loco-regional percutaneous treatment or combined liver-heart transplantation are the only therapeutic options. This case suggests that LLR in FP patients has low-risk of liver and cardiac decompensation, minimizing the pneumoperitoneum insufflation to ensure low intra-abdominal/intra-thoracic pressures and providing accurate anaesthetic management to maintain proper cardiac preload and output.
LLR for HCC after FP is safe and feasible, and might be considered an alternative treatment of HCC for which the best treatment has not been defined yet.
在单心室心脏患者中施行的复杂心脏手术——Fontan手术（FP）后，一个公认的长期并发症是慢性肝病和肝细胞癌（HCC）的发生。由于存在心脏和肝脏失代偿的风险，HCC的肝切除术具有挑战性，且从未有过腹腔镜手术的报道。
我们报告首例在33岁接受FP术后出现心脏相关性肝硬化的女孩中施行腹腔镜肝切除术（LLR）治疗HCC的病例。术中，气腹压力设定为8 - 10 mmHg，并给予足够的液体输注以维持心脏前负荷。在超声引导下沿肝脏病变的肿瘤边缘进行热消融后，在未行Pringle手法且无需输血的情况下，对Ⅴ段肿瘤进行了全腹腔镜非解剖性切除。手术期间及术后心脏功能保持稳定，术后过程顺利。
FP术后慢性肝病中的HCC与高死亡风险相关。由于FP术后复杂的血流动力学变化，开放性手术切除往往不可行，局部经皮治疗或肝心联合移植是仅有的治疗选择。该病例表明，FP患者行LLR时肝脏和心脏失代偿风险低，尽量减少气腹充气以确保低腹内/胸内压力，并提供精确的麻醉管理以维持适当的心脏前负荷和输出。
FP术后HCC行LLR安全可行，对于尚未确定最佳治疗方案的HCC，可考虑将其作为一种替代治疗方法。
