Letter to Editor: Congenital extrahepatic portosystemic shunts (Abernethy malformation): A new variant

Abernethy malformations are a rare collection of congenital hepatic portosystemic shunts. Our 19-year-old patient is with a type 2 Abernethy malformation elected permanent shunt closure following worsening dyspnea. This report details a single stage endovascular technique wherein shunt closure was achieved immediately by placement of an aortic endograft. At 5-month follow-up, the patient reported decreased shortness of breath. Furthermore, ultrasound investigation demonstrated a patent portal vein and right heart catheterization 6 months after procedure revealed decreased pulmonary hypertension relative to preprocedure values. This one step method may serve as an alternative treatment strategy to multistage endovascular closure techniques of type 2 Abernethy malformations.

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

American Journal of Interventional Radiology ◽

10.25259/ajir_25_2019 ◽

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.

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Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Hepatology ◽

10.1002/hep.30817 ◽

2019 ◽

Vol 71 (2) ◽

pp. 658-669 ◽

Cited By ~ 10

Author(s):

Anna Baiges ◽

Fanny Turon ◽

Macarena Simón‐Talero ◽

Stephanie Tasayco ◽

Javier Bueno ◽

...

Keyword(s):

Observational Study ◽

Abernethy Malformation ◽

Portosystemic Shunts

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A case of two shunts in the endovascular treatment of type II Abernethy syndrome

CVIR Endovascular ◽

10.1186/s42155-021-00279-7 ◽

2022 ◽

Vol 5 (1) ◽

Author(s):

Brenden Bombardier ◽

Adam Alli ◽

Aaron Rohr ◽

Zachary Collins ◽

Kavi Raval

Keyword(s):

Blood Flow ◽

Portal Vein ◽

Endovascular Treatment ◽

Rare Condition ◽

Portal System ◽

Type Ii ◽

Abernethy Malformation ◽

Complete Occlusion ◽

Post Procedure ◽

Portosystemic Shunts

Abstract Background Abernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population. Case presentation We report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7 mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT. Conclusions This report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.

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Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.784739 ◽

2022 ◽

Vol 8 ◽

Author(s):

Xue-qin Lin ◽

Jing-yi Rao ◽

Yi-fei Xiang ◽

Li-wei Zhang ◽

Xiao-ling Cai ◽

...

Keyword(s):

Signs And Symptoms ◽

Term Treatment ◽

Hospital Treatment ◽

Abernethy Malformation ◽

Case Presentation ◽

Portosystemic Shunts ◽

Long Term Treatment ◽

History Of ◽

Portal Veins

Background: Abernethy malformation is an extremely rare anomaly of the splanchnic venous system, and only 2 cases that manifested as syncope had been reported previously.Case Presentation: A 24-year-old male had a 15-year history of jaundice and was in long-term use of hepatoprotective drugs. He was admitted for complaint of syncope. He underwent a series of examinations and cardiac ultrasound showed that his pulmonary artery pressure was elevated. Further imaging revealed the absence of intrahepatic portal veins. His blood ammonia was significantly increased. All signs and symptoms pointed to an Abernethy diagnosis. He was finally diagnosed as having Abernethy type II. He was discharged after 17 days of in-hospital treatment with sildenafil (50 mg/day) and ornithine aspartate (20 g/day).Conclusion: We now report this rare case of syncope that is caused by Abernethy malformation. As a typically pediatric disease, it was not identified in this patient until adulthood due to long-term treatment for jaundice and liver cirrhosis. Furthermore, we present a review of portosystemic shunts previously reported in the literature.

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Abernethy Malformation in Children with Focal Liver Lesions

Journal of radiology and nuclear medicine ◽

10.20862/0042-4676-2021-102-3-166-171 ◽

2021 ◽

Vol 102 (3) ◽

pp. 166-171

Author(s):

D. E. Arutyunyants ◽

V. V. Khovrin ◽

T. N. Galyan ◽

A. S. Kulichkin ◽

A. V. Filin ◽

...

Keyword(s):

Portal Vein ◽

Focal Liver Lesions ◽

Liver Lesions ◽

Congenital Disease ◽

Abernethy Malformation ◽

Vascular Abnormality ◽

Portosystemic Shunts ◽

Clinical Cases

Abernethy malformation is a congenital disease characterized by complete or partial underdevelopment of the portal vein, leading to the occurrence of congenital extrahepatic portosystemic shunts. The anomaly with the absence of the portal vein is detected in 80% of cases in childhood. Patients with this pathology are very frequently examined when detecting space-occupying liver lesions and assuming the presence of a vascular abnormality. The paper describes clinical cases of benign focal liver lesions and Abernethy malformation in children.

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Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation)

The American Journal of Surgical Pathology ◽

10.1097/pas.0b013e3182230ce4 ◽

2011 ◽

Vol 35 (9) ◽

pp. 1381-1390 ◽

Cited By ~ 47

Author(s):

Mikhail Lisovsky ◽

Angelos A. Konstas ◽

Joseph Misdraji

Keyword(s):

Abernethy Malformation ◽

Portosystemic Shunts

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Abernethy malformation and hepatocellular carcinoma: a serious consequence of a rare disease

BMJ Case Reports ◽

10.1136/bcr-2019-231843 ◽

2020 ◽

Vol 13 (1) ◽

pp. e231843 ◽

Cited By ~ 1

Author(s):

Manuel Jaklitsch ◽

Mafalda Sobral ◽

Ana Maria Carvalho ◽

Hugo Pinto Marques

Keyword(s):

Hepatocellular Carcinoma ◽

Treatment Strategies ◽

Nodular Regenerative Hyperplasia ◽

Portal Flow ◽

Abernethy Malformation ◽

Liver Tumours ◽

Portosystemic Shunts ◽

Nodular Hyperplasia ◽

Patient Series ◽

Malignant Liver

Congenital portosystemic shunts (CPSS) are a rare vascular consequence of embryogenetic vascular alterations or the persistence of the fetal circulation elements, first described by John Abernethy in 1793 and classified by Morgan and Superina, into complete and partial portosystemic shunts. Its prevalence to this day has not been defined. We present a patient series of a 44-year-old and 47-year-old man and woman, with this rare congenital malformation and underlining hepatocellular carcinoma (HCC) treatment strategies. Over half of the individuals with CPSS have benign or malignant liver tumours, ranging from nodular regenerative hyperplasia, focal nodular hyperplasia, adenomas, HCC and hepatoblastomas. Additionally, it is known that half of individuals with Abernethy malformation type Ib will develop one or multiple types of tumours. There seems to be a direct association with tumorigenesis and CPSS, which is the primary consequence of absent portal flow. Surgery is the treatment of choice, either as a curative resection or orthotopic liver transplantation if recommended as per the criteria, in which replacing the hepatic parenchyma in the setting of an Abernathy malformation will correct the underlining hyper-arterialisation.

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