Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts

Rajeswaran, Shankar; Johnston, Andrew D.; Green, Jared; Riaz, Ahsun; Thornburg, Bartley; Mouli, S.; Lautz, Timothy B.; Lemoine, Caroline; Superina, Riccardo; Donaldson, James S.

doi:10.1016/j.jvir.2019.08.007

Cited by 24 publications

(27 citation statements)

References 16 publications

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“…Treatment with LT has been proposed, but is unnecessary in the vast majority of children because of modern endovascular shunt closure techniques. ( 324,325 ) A significant proportion of these patients may have severe congenital cardiac, genitourinary, and musculoskeletal defects. ( 323 ) Acquired portopulmonary hypertension (15% of cases) and hepatopulmonary syndrome (3%) require careful management ( 323 ) of pulmonary hypertension preoperatively and postoperatively.…”

Section: Congenital Portosystemic Shuntsmentioning

confidence: 99%

Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases

et al. 2021

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Section: Congenital Portosystemic Shuntsmentioning

confidence: 99%

Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases

et al. 2021

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“…This association has been hypothesized to be related to the lack of portal vein flow, which can lead to compensatory arterial hyperperfusion and the formation of regenerative nodules. Although rare, these neoplasms may degenerate to hepatocellular carcinoma [14][15][16][17][18]. Imaging characteristics of these hepatic neoplasms can be challenging due to the absence of intrahepatic portal systems and their associ-…”

Section: A B C D E Fmentioning

confidence: 99%

“…ated portal venous or delayed imaging patterns. Apparently, a biopsy of these lesions is critical in cases showing an interval growth or a change in enhancement pattern [14][15][16][17][18].…”

mentioning

confidence: 99%

Living donor liver transplantation in a pediatric patient with congenital absence of the portal vein

Namgoong

Hwang

Park

et al. 2021

Ann Hepatobiliary Pancreat Surg

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Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV combined with focal nodular hyperplasia (FNH) and hepatocellular adenoma. A 9-year-old girl who had been diagnosed with multiple FNH had CAPV. Her blood ammonia level was raised to 137 µg/dL. However, she did not complain of any symptoms. To treat CAPV and FNH, we decided to perform LDLT. The graft was a left liver graft from 39-year-old mother of the patient. Recipient hepatectomy was performed according to standard procedures of pediatric LDLT. Portal vein reconstruction was performed using interposition of an iliac vein homograft conduit to the superior mesenteric vein-splenic vein confluence. The CAPV-associated congenital splenorenal shunt was securely ligated. The pathology report of the explant liver showed a 2 cm-sized hepatocellular adenoma and multiple FNH lesions measuring up to 7.1 cm. The patient recovered uneventfully from the LDLT operation. The reconstructed portal vein was maintained well without any hemodynamic abnormalities. In conclusion, as CAPV patients can have various vascular anomalies, combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.

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“…No significant difference was observed in the decrease of serum ammonia levels between patients treated surgically or endovascularly. For the patients treated with observation, no comparison was made due to the rarity of patients [16]. The experience in treating this patient also demonstrates that simple conservative treatment with regular follow-up visits can also be suitable for those patients who do not have severe complications, this depends on a comprehensive and adequate assessment of patients' condition.…”

Section: Resultsmentioning

confidence: 99%

A Case Report: Type II Abernethy Malformation Complicated with Congenital Polydactyly and Enlargement of all Cardiac Chambers

Zhu¹,

Wang²,

Qian³

2020

MEDJ

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Background: Abernethy malformation is a kind of congenital malformation of the portal vein system caused by abnormal portacaval shunts. It can be in combination with many other congenital malformations. There has been a limited number of patients since the first patient was reported, leading to limited knowledge of this kind of disease. Methods: In August 2018, we treated a patient diagnosed with type II Abernethy malformation complicated with both congenital polydactyly and enlargement of all cardiac chambers, which is extremely rare and can be supplementary to the existing cases. According to a comprehensive and adequate assessment of patients' condition, we treated him with oral silybin (70 mg every time and 3 times a day) for 3 months, and advised him to make follow-up visits. Results: At the latest follow-up, we knew the health condition of this patient was generally satisfactory, whether in terms of laboratory test results or his daily life experience. Conclusion: Although the major therapy for Abernethy malformation is surgery, this case suggests that simple conservative treatment with regular follow-up visits can be suitable for certain patients.

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Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts

Cited by 24 publications

References 16 publications

Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases

Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases

Living donor liver transplantation in a pediatric patient with congenital absence of the portal vein

A Case Report: Type II Abernethy Malformation Complicated with Congenital Polydactyly and Enlargement of all Cardiac Chambers

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