title: Associating liver partition with portal vein ligation and staged hepatectomy (ALPPS) for the treatment of liver tumors in children.
abstract: Resection is the only curative treatment option for primary and secondary malignant tumors of the liver. Although curative resection is associated with long-term survival rates, it can only be performed in 10% of patients with primary tumors and 25% of patients with liver metastases. Liver insufficiency is one of the most serious postoperative complications of patients undergoing extensive liver resections. When total liver resection is necessary liver transplant is mandatory, with the burden of long-term immunosuppression and its complications. Among several different strategies to increase the resectability of liver tumors, portal vein occlusion (embolization or ligature), bilateral tumor resection in two stages, and resection combined with loco regional therapy are the most popular. A new strategy for patients with marginally resectable liver tumors previously considered to be unresectable was formally reported by Baumgart et al. in 2011, originally developed by Hans Schlitt in 2007. This technique consists of a two-staged hepatectomy with initial portal vein ligation and in situ splitting of the liver parenchyma, and it is known as ALPPS (associating liver partition with portal vein ligation for staged hepatectomy). The aim of this study is to present the first series of pediatric patients with marginally resectable liver tumors previously considered to be unresectable treated with two-stage hepatectomy with initial portal vein ligation and in situ splitting of the liver parenchyma. Two patients were diagnosed with hepatoblastoma, and one each with rhabdomyosarcoma, hepatocellular carcinoma, and nodular focal hyperplasia. ALPPS technique was considered whenever the future liver remnant (FLR) was 40% or less of the total liver volume (TLV) determined by CT or MRI scans. The ratio of FLR to TLV before the first procedure ranged from 0.15 to 0.38, with a mean±sd of 0.253±0.07. In all patients, a rapid growth of the FLR was observed. Estimates of the FRL volume prior to surgical treatment ranged from 110cc to 750cc, with a mean±sd of 361.6±213.75cc. Just before the second procedure, the volume of the remnant liver ranged from 225cc to 910cc, with a mean±sd of 563.6cc±221.7cc. The FRL volume increase had a mean±sd of 72.56%±29.05%, with a median of 83.8%. The second procedure was performed after 7 to 12days with a median of 11days. The only postoperative complication observed in one patient was an asymptomatic right pleural effusion that was aspirated during the second procedure with no further complications. ALPPS was shown to be effective and a safe procedure to treat large tumors in children.
authors.affiliation: Federal University of Paraná, Curitiba Brazil; Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil. Electronic address: julio.wieder@gmail.com.
authors.forename: Julio Cesar
authors.initials: JC
authors.lastname: Wiederkehr
authors.affiliation: Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Sylvio Gilberto
authors.initials: SG
authors.lastname: Avilla
authors.affiliation: Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Elisângela
authors.initials: E
authors.lastname: Mattos
authors.affiliation: Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Izabel Meister
authors.initials: IM
authors.lastname: Coelho
authors.affiliation: Department of Radiology, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Jorge Alberto
authors.initials: JA
authors.lastname: Ledesma
authors.affiliation: Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Alexandra Fernandes
authors.initials: AF
authors.lastname: Conceição
authors.affiliation: Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Henrique Aguiar
authors.initials: HA
authors.lastname: Wiederkehr
authors.affiliation: Division of Liver Transplantation and Surgery, Hospital Pequeno Príncipe, Curitiba Brazil.
authors.forename: Barbara Aguiar
authors.initials: BA
authors.lastname: Wiederkehr
mesh_terms: D006528:Carcinoma, Hepatocellular / Q000601:surgery*; D004621:Embolization, Therapeutic; D005260:Female; D006498:Hepatectomy / Q000379:methods*; D048550:Hepatic Insufficiency / Q000209:etiology; D006801:Humans; D008026:Ligation / Q000379:methods; D008113:Liver Neoplasms / Q000556:secondary / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D011169:Portal Vein / Q000601:surgery*; D011183:Postoperative Complications / Q000601:surgery; D012208:Rhabdomyosarcoma / Q000601:surgery
keywords: Hepatectomy; Liver tumor; Pediatrics; Surgery