Ante situm liver resection with inferior vena cava replacement under hypothermic cardiopolmunary bypass for hepatoblastoma: Report of a case and review of the literature

Abstract

Introduction: Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear.

Presentation of case: A 11-months-old boy was referred for a right hepatic lobe mass(90×78mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61×64mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft.

Conclusions: We report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.

Keywords: Ante situm liver resection; Hepatoblastoma; Hypothermic cardiopolmunary bypass; Inferior vena cava tumoral thrombi.

Fig. 1

Computer Tomography imaging at presentation. Computer Tomography imaging at diagnosis showing: A) right hepatic lobe mass with calcifications (90 × 78 mm); B) lung metastasis and tumoral thrombi invading the inferior vena cava and the right atrium trough the right hepatic vein; c) tumour mass in the right extended lobe of the liver.

Fig. 2

Tumoral staging after neoadjuvant chemotherapy and preoperative assessment. Imaging of hepatoblastoma (HBL) after neoadjuvant chemotherapy (SIOPEL 2004 HR protocol): A-B) CT scan showing HBL in the extended-right lobe of liver with tumoral thrombi into the right hepatic vein and the right atrium; C) cavography showing tumoral thrombi infiltrating and compressing the retrohepatic inferior vena cava and retroperitoneal collaterals.

Fig. 3

Technical aspects of ante situm liver resection and inferior vena cava replacement. Intraoperative view of A) hepatic hilum dissection; B) parenchymal transection, on the line of the falciform ligament via anterior approach; C) ante situ hypothermic liver perfusion with Celsior solution (4 °C) through the right portal vein stump and inferior vena cava replacement with donor aortic conduit (note the diaphragmatic ostium resected and reconstructed); D) final view of en-bloc resection of the extended-right hepatic lobe (segments I + IV-VIII), the inferior vena cava with tumural thrombi and the diaphragmatic ostium.

Fig. 4

Computer Tomography imaging after surgery. Computer Tomography scan after 4 months from surgery showing patent left hepatic vein anastomosis (A) and retrohepatic cava replacement with aortic graft from cadaveric donor (B,C).