scholarly journals ABERNETHY MALFORMATION AS A RARE INDICATION FOR LIVER TRANSPLANTATION: CASE REPORT

2019 ◽  
Vol 141 (5-6) ◽  
pp. 134-137
Keyword(s):  
Liver Transplantation ◽  
Case Report ◽  
Portal Vein ◽  
Liver Tumors ◽  
Splenic Vein ◽  
Vena Cava ◽  
Portal Blood ◽  
Type I ◽  
Abernethy Malformation ◽  
Congenital Cardiac Anomalies

Abernethy malformation or congenital agenesis of the portal vein (CAPV) is a rare malformation of the abdominal splanchnic venous system. This malformation is commonly found in children and is often associated with other malformations such as congenital cardiac anomalies and skeletal system disorders, as well as liver tumors. There are two types of Abernethy malformation. In type I, portal blood bypasses the liver completely, with the superior mesenteric vein and the splenic vein draining into the inferior vena cava separately (type Ia), or together (type Ib). There are no intrahepatic portal vein branches in the liver. Type II is a partial portocaval shunt in which portal blood partially supplies the liver. There is no unified therapeutic approach for all patients with Abernethy malformation, however, liver transplantation is recommended in patients with liver disease (encephalopathy, poor liver function) and those with liver tumors. In this case report we present a case of Abernethy type Ib malformation in a 17-year-old patient with chronic malaise and uper abdominal pain. During diagnostic work-up, an unresectable liver tumor was found and the patient was successfully treated with orthotopic liver transplantation.

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.

scholarly journals Transsplenic portal vein recanalization and direct intrahepatic portosystemic shunt placement to optimize liver transplantation

2020 ◽  
Vol 3 (1) ◽  
Author(s):  
Osman Ahmed ◽  
Abhijit L. Salaskar ◽  
Steven Zangan ◽  
Anjana Pillai ◽  
Talia Baker
Keyword(s):  
Liver Transplantation ◽  
Portal Vein ◽  
Splenic Vein ◽  
Portosystemic Shunt ◽  
Refractory Ascites ◽  
Hepatic Veins ◽  
Sonographic Guidance ◽  
Portosystemic Shunts ◽  
Intrahepatic Portosystemic Shunt ◽  
End To End

Abstract Background Percutaneous trans-splenic portal vein recanalization (PVR) has been reported for facilitation of transjugular intrahepatic portosystemic shunts (TIPS), however has not been applied to patients undergoing direct intrahepatic portosystemic shunt (DIPS). We report the utilization of trans-splenic-PVR with DIPS creation in a patient with chronic portal and hepatic vein occlusions undergoing liver transplantation evaluation. Case presentation A 48-year-old male with decompensated alcoholic cirrhosis complicated by refractory ascites, hepatic encephalopathy, and variceal bleeding underwent CT that demonstrated chronic occlusion of the hepatic veins (HV), extrahepatic portal vein (PV), and superior mesenteric vein (SMV). Due to failed attempts at TIPS at outside institutions, interventional radiology was consulted for portal vein recanalization (PVR) with TIPS to treat the portal hypertension and ascites and also facilitate an end-to-end PV anastomosis at transplantation. After an initial hepatic venogram confirmed chronic HV occlusion, a DIPS with trans-splenic PVR was planned. The splenic vein was accessed under sonographic guidance using a micropuncture set and subsequently upsized to a 6 French sheath over a stiff guidewire. A splenic venogram via this access confirmed occlusion of the PV with drainage of the splenic vein (SV) through gastric varices. The thrombosed PV was then recanalized and angioplastied to restore PV flow via the transsplenic approach. A transjugular liver access kit with a modified 21-gauge needle was advanced into the IVC through the internal jugular vein (IJV) sheath and directed towards the target snare in PV. The needle was used to subsequently puncture the PV through the caudate lobe and facilitate placement of a wire into the SV. The initial portosystemic gradient (PSG) was 20 mmHg. The IJV sheath was advanced through the hepatic parenchymal tract into the main-PV and a stent-graft was placed across the main PV and into the IVC. A portal venogram demonstrated brisk blood flow through the DIPS, resolution of varices and a PSG of 8 mmHg. One month after the procedure, the patient had a significant reduction in ascites and MELD-NA score. Patient is currently listed and awaiting transplantation. Conclusions In the setting of chronically occluded portal and hepatic veins, trans-splenic PVR DIPS may serve as an effective bridge to liver transplantation by facilitating an end to end portal vein anastomosis.

Liver Transplantation in Cases of Portal Vein Thrombosis in the Recipient: A Case Report and Review of the Various Options

2011 ◽  
Vol 43 (9) ◽  
pp. 3490-3492 ◽  
Author(s):  
D. Germanova ◽  
V. Lucidi ◽  
A. Buggenhout ◽  
N. Boon ◽  
N. Bourgeois ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Case Report ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Vein Thrombosis

scholarly journals Abernethy Malformation Type II and Concurrent Nodular Hyperplasia in a Rare Female Case

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Zhen Kang ◽  
Xiangde Min ◽  
Liang Wang
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Type I ◽  
Type Ii ◽  
Malignant Tumours ◽  
Abernethy Malformation ◽  
Vascular Abnormality ◽  
Female Case ◽  
Nodular Hyperplasia ◽  
The Right

Background. Abernethy malformation is a rare splanchnic vascular abnormality characterizing extrahepatic abnormal shunts that is classified into types I and II. Abernethy malformation type I has a female predilection and is associated with a variety of concurrent hepatic benign or malignant tumours while type II with concurrent tumours is very rare in females. Case Report. We report a rare female case of Abernethy malformation type II with concurrent occupying lesion in the right liver, which was successfully transplanted; the occupying lesion was pathologically proven to be nodular hyperplasia. Conclusion. This case might provide further knowledge regarding Abernethy malformation. On imaging, the anatomy of portal vein should be carefully investigated to categorize Abernethy malformation, and a wide variety of differential diagnosis of concurrent occupying lesions should be taken into account.

scholarly journals Correction of extrahepatic portal hypertension in pediatric patient after liver transplantation

2017 ◽  
Vol 19 (1) ◽  
pp. 47-51
Author(s):  
A. R. Monakhov ◽  
B. L. Mironkov ◽  
T. A. Dzhanbekov ◽  
K. O. Semash ◽  
Kh. M. Khizroev ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Blood Flow ◽  
Portal Hypertension ◽  
Portal Vein ◽  
Pediatric Patient ◽  
Portal Blood Flow ◽  
Portal Blood ◽  
Positive Trend ◽  
Portal Vein Stenosis ◽  
Vein Stenosis

Introduction. Liver transplantation is a multi-component and complex type of operative treatment. Patients undergoing such a treatment sometimes are getting various complications. One of these complications is a portal hypertension associated with portal vein stenosis.Materials and methods. In 6 years after the left lateral section transplantation from living donor in a pediatric patient the signs of portal hypertension were observed. Stenosis of the portal vein was revealed. Due to this fact percutaneous transhepatic correction of portal vein stenosis was performed.Results. As a result of the correction of portal blood flow in the patient a positive trend was noted. According to the laboratory and instrumental methods of examination the graft had a normal function, portal blood flow was adequate. In order to control the stent patency Doppler ultrasound and MSCT of the abdominal cavity with intravenous bolus contrasting were performed. Due to these examinations the stent function was good, the rate of blood flow in the portal vein due to Doppler data has reached 80 cm/sec, and a decrease of the spleen size was noted.Conclusion. Diagnosis and timely detection of portal vein stenosis in patients after liver transplantation are very important for the preservation of graft function and for the prevention of portal hypertension. In order to do that, ultrasound Doppler fluorimetry examination needs to be performed to each patient after liver transplantation. In cases of violation of the blood flow in the portal vein CT angiography performance is needed. Percutaneous transhepatic stenting of portal vein is a minimally invasive and highly effective method of correction of portal hypertension. Antiplatelet therapy and platelet aggregation control are the prerequisites for successful stent function.

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