Abernethy Malformation – a Rare but Important Diagnosis Prior to Liver-Directed Therapy for Hepatocellular Carcinoma

2019 ◽  
Vol 3 ◽  
pp. 9
Author(s):  
Nainesh Parikh ◽  
Kun Jiang ◽  
Kim Truong
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Portal Vein ◽  
Portosystemic Shunt ◽  
Left Hepatic Lobe ◽  
Abernethy Malformation ◽  
Hepatic Lobe ◽  
Portosystemic Shunts ◽  
Congenital Extrahepatic Portosystemic Shunt ◽  
The Right

A 45-year-old man with incidentally discovered, unresectable HCC were treated with TACE to the left hepatic lobe and TARE to the right hepatic lobe. Upon retrospective review, he was found to have a congenital extrahepatic portosystemic shunt with the absence of the portal vein (Abernethy malformation). This case report discusses variant splanchnic and portal anatomy in the setting of rare, congenital portosystemic shunts and evaluates types of liver-directed therapies for HCC in this setting.

scholarly journals Preoperative Trans-catheter Arterial Chemoembolization and Laparoscopic Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy in Unresectable Hepatocellular Carcinoma – A Case Report

BioMedica ◽  
2021 ◽  
Vol 37 (2) ◽  
pp. 86-92
Author(s):  
Yilin Hu ◽  
Yanbing Shen ◽  
Dan Wang ◽  
Tingjia Cao
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Portal Vein ◽  
Liver Volume ◽  
Postoperative Outcome ◽  
Portal Vein Ligation ◽  
The Right ◽  
Staged Hepatectomy ◽  
Arterial Chemoembolization

<p>Transcatheter arterial chemoembolization (TACE) is considered as a major method to treat hepatocellular carcinoma (HCC). Associated liver partition and portal vein ligation for staged hepatectomy (ALPPS) has been used to treat patients with advanced HCC. This case report documents the safety, efficacy, and feasibility of TACE and laparoscopic ALPPS to treat the unresectable HCC of the right liver with right intra-hepatic metastasis in a male patient. Percutaneous and trans-femoral artery TACE was performed preoperatively, and the ALPPS stage-I at the 4th week and stage-II after 14 days was carried out. Postoperative outcome was assessed after 180 days of follow-up. It is concluded that preoperative TACE and laparoscopic ALPPS offer a fine treatment alternative to the patients with insufficient residual liver volume and relatively-advanced and extensive HCC.</p>

scholarly journals Hepatocellular carcinoma with tumor thrombus extends to the right atrium and portal vein: A case report

2020 ◽  
Vol 12 (11) ◽  
pp. 1128-1135
Author(s):  
Diego Gomez-Puerto ◽  
Oriol Mirallas ◽  
Judit Vidal-González ◽  
Victor Vargas
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Portal Vein ◽  
Right Atrium ◽  
Tumor Thrombus ◽  
The Right

scholarly journals Agenesis of the left hepatic lobe undergoing laparoscopic hepatectomy for hepatocellular carcinoma: a case report

2017 ◽  
Vol 3 (1) ◽  
Author(s):  
Katsunori Matsushita ◽  
Kunihito Gotoh ◽  
Hidetoshi Eguchi ◽  
Yoshihumi Iwagami ◽  
Daisaku Yamada ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Laparoscopic Hepatectomy ◽  
Left Hepatic Lobe ◽  
Hepatic Lobe

Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) With Associated Hepatocellular Carcinoma

2017 ◽  
Vol 20 (4) ◽  
pp. 354-362 ◽  
Author(s):  
Mark Benedict ◽  
Manuel Rodriguez-Davalos ◽  
Sukru Emre ◽  
Zenta Walther ◽  
Raffaella Morotti
Keyword(s):  
Hepatocellular Carcinoma ◽  
Portal Vein ◽  
Vena Cava ◽  
Clinical Manifestations ◽  
Portosystemic Shunt ◽  
Congenital Absence ◽  
Nodular Regenerative Hyperplasia ◽  
Type I ◽  
Small Hepatocellular Carcinoma ◽  
Abernethy Malformation

Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra- and intrahepatic shunts. Two shunts have been defined: Type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava. The clinical manifestations of Abernethy malformation are diverse with a typical presentation consisting of hypoxia and hepto-pulmonary syndrome. Histologically, focal nodular hyperplasia, nodular regenerative hyperplasia, liver adenoma, hepatoblastoma, and hepatocellular carcinoma have all been reported. Herein, we report a case of Abernethy malformation, type Ib, in a 12-month-old male who was found to have a small hepatocellular carcinoma at the time of explant. The immunohistochemical characteristics in relation to the genetic aspects are discussed. To our knowledge, this is the first reported case of hepatocellular carcinoma developing in a patient who is under the age of 5 years with Abernethy malformation.

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