Liver dysfunction arising from FALD causes liver fibrosis, cirrhosis and HCC, even in young patients [5]. FALD results from fibrosis of the sinusoidal and portal canals due to excessive liver congestion caused by the high CVP. The stage of FALD depends on the duration after FP and hepatic venous pressure [6]. FALD can develop to liver cirrhosis as early as 11–15 years after FP, and the cumulative incidences of cirrhosis at 20 and 30 years after FP have been reported as 56.6% and 97.9%, respectively [7]. The incidence of HCC in FALD has been estimated as 1.5–5.0% per year [8]. Our patient presented 28 years after FP with no relevant symptoms or blood biochemistry results, strongly suggesting liver fibrosis or cirrhosis.
ICG has commonly been used to evaluate preoperative liver function in Japan, but accurate evaluation is difficult in the presence of factors, such as portosystemic shunt [9]. However, 99mTc-GSA scintigraphy is unaffected by these pathologies [9]. Even though the present case did not require detailed evaluation of liver function, because the small partial hepatectomy was sufficient for cancer treatment, preoperative evaluation of liver function might be difficult for patients with FALD, as previously reported [10]. Since the ICG retention rate at 15 min in the present study indicated poor liver function, 99mTc-GSA scintigraphy was added for further assessment of preoperative liver function. In the results from 99mTc-GSA scintigraphy, LHL 15 indicated favorable liver function, whereas HH 15 indicated poor liver function. This discrepancy between LHL 15 and HH 15 might be explained by the low H 3, representing radioactivity in the cardiac region of interest at 3 min after injection. In the Fontan circulation, systemic–pulmonary artery shunt may result in a low H 3. In fact, the H 3 value of the present case was relatively lower than that of various other patients in our experience (data not shown). However, since detailed reports of FALD and 99mTc-GSA scintigraphy have not been reported previously, further study is required regarding the assessment of liver function using 99mTc-GSA scintigraphy in cases of FALD. Finally, histopathological examination in the present case demonstrated liver cirrhosis.
Most cases of HCC arising from FALD are reportedly treated non-surgically, with poor liver function reducing the tolerance for surgical resection [11]. However, recent improvements in surgical procedures and perioperative management have enabled safe liver resection not only by laparotomy, but also in laparoscopic surgery [12, 13]. The present case appears to represent the third report of laparoscopic liver resection for HCC arising from FALD [12, 13], but the first case in which the origin of a hepatic vein was exposed. Laparoscopic surgery shows some limitations for patients who have undergone FP. Laparoscopic surgery easily leads to low cardiac output due to the elevations in intrathoracic and intraabdominal pressures under positive-pressure ventilation and pneumoperitoneum [12, 13]. Nonetheless, although CVP was elevated after the initiation of pneumoperitoneum in our case, blood pressure was kept stable. Injection of sufficient fluid (about 500 mL/h during anesthesia) might have helped maintain the stable condition in the present case. However, uncontrollable bleeding from hepatic veins may occur readily if a hepatic vein is injured. Although we were able to successfully perform laparoscopic hepatectomy exposing a hepatic vein, we had to prepare options in case of bleeding from a hepatic vein, such as the reverse Trendelenburg position or Pringle maneuver. As noted in the previous study, the Pringle maneuver does not have adverse effects on the Fontan circulation during laparoscopic hepatectomy [13]. On the other hand, IVC clamping can easily lead to low blood pressure in the Fontan circulation [1]. We did not apply the reverse Trendelenburg position during the operation due to concerns regarding unstable blood pressure with decreases in venous return [13], but a recent study suggested the utility of this position to reduce CVP with stable vital signs [1]. However, since no reports have described laparoscopic liver resection applying a reverse Trendelenburg position, further study is required. In addition, although we did not encounter any elevation of the airway pressure to over 20 cmH2O in the present case, strict control of airway pressure may also be required to control bleeding from hepatic veins especially in laparoscopic hepatectomy for patients with FALD [14].
The indications for laparoscopic liver resection among patients with FALD thus have to be determined carefully with close communication between the cardiologist and anesthesiologist. Furthermore, we have to keep in mind the timing of conversion to open surgery when performing laparoscopic surgery, not only because of the risk of intraoperative bleeding, but also because changes in vital signs may easily arise in patients with FALD. Liver resection to treat HCC arising from FALD, therefore, requires stricter criteria than conventional liver resection. Such criteria may include preoperative CVP and wedge pressure of the hepatic veins to predict the likelihood of intraoperative bleeding from hepatic veins. To establish such criteria, we should accumulate data on patients with FALD by creating a large-scale, nationwide database.
In the present case, a hepatic vein had to be exposed to remove the tumor. However, the wall of the hepatic vein showed fibrous thickening rather than fragility, and no bleeding was observed. Liver congestion is known to lead to fibrous thickening of veins, such as the IVC and hepatic veins [15]. The fibrous thickening of the hepatic vein in this case might have been caused by prolonged liver congestion or inflammation due to the tumor, but the precise reasons remain unclear. Further cases need to be accumulated to clarify such anatomical changes.
