Hort Amy, Karpelowsky Jonathan, Shun Albert, Thomas Gordon
The Department of Surgery, The Children's Hospital Westmead, Part of the Sydney Children's Hospital Network, Westmead, New South Wales, Australia.
Division of Child and Adolescent Health, University of Sydney, Sydney, New South Wales, Australia.
Pediatr Transplant. 2019 Jun;23(4):e13409. doi: 10.1111/petr.13409. Epub 2019 Apr 4.
Complete microscopic tumor resection is critical for successful treatment of hepatoblastoma, and this may include when liver transplantation is required. For tumors involving the IVC or PV, complete resection should include the involved IVC or PV to ensure full tumor clearance. When this is required, the venous reconstruction at transplant or post-excision can be challenging. We present the management of an 18-month-old girl with PRETEXT Stage IV (P, V, F) hepatoblastoma and IVC involvement, where native caval resection and reconstruction was required. The preoperative staging following neoadjuvant chemotherapy was POSTTEXT Stage IV (P, V, F). An orthotopic liver transplantation was performed using a left lateral segment graft from a deceased adult donor. With native hepatectomy, retrohepatic IVC resection from just above the hepatic venous confluence to just above the entry of the right adrenal vein was performed. For caval reconstruction, a venous graft from a deceased donor was used. The graft included the lower IVC with the right common iliac vein and a short stump of the left common iliac vein. The common iliac was a perfect size match for the IVC, and the three natural ostia matched the upper cava, lower cava, and the outflow from the donor left hepatic vein. The patient had an uneventful postoperative course and remains well and disease-free 2 years after transplant with continued patency of the reconstructed cava. When indicated, a donor iliac vein graft with its natural ostia should be considered in caval reconstruction for pediatric liver transplantation.
完整的显微镜下肿瘤切除对于肝母细胞瘤的成功治疗至关重要，这可能包括需要进行肝移植的情况。对于累及下腔静脉（IVC）或门静脉（PV）的肿瘤，完整切除应包括受累的IVC或PV，以确保肿瘤完全清除。当需要这样做时，移植时或切除后的静脉重建可能具有挑战性。我们介绍了一名18个月大患有PRETEXT IV期（P、V、F）肝母细胞瘤且累及IVC的女孩的治疗情况，在此病例中需要进行原位下腔静脉切除和重建。新辅助化疗后的术前分期为POSTTEXT IV期（P、V、F）。使用来自已故成年供体的左外侧叶移植物进行了原位肝移植。在进行原位肝切除时，从肝静脉汇合处上方至右肾上腺静脉入口上方进行了肝后下腔静脉切除。对于下腔静脉重建，使用了来自已故供体的静脉移植物。该移植物包括下腔静脉下部、右髂总静脉和左髂总静脉的一小段残端。髂总静脉与下腔静脉大小完美匹配，三个天然开口分别与上腔静脉、下腔静脉以及供体左肝静脉的流出道相匹配。患者术后过程顺利，移植后2年情况良好且无疾病，重建的下腔静脉持续通畅。在有指征时，小儿肝移植下腔静脉重建应考虑使用带有天然开口的供体髂静脉移植物。
