The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

Kim, Eun Sil; Lee, Ki Wuk; Choe, Yon Ho

doi:10.5223/pghn.2019.22.1.80

Cited by 4 publications

(5 citation statements)

References 21 publications

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“…No shunt between the portal vein and the splenic vein was detected, but after entering the splenic hilum, the blood flowed back to the IVC in the form of a thick “spleen kidney” shunt through the collateral circulation, which is different from the usual form of Abernethy syndrome and may be another manifestation of Abernethy malformation type IIC. Several studies[ 12 , 13 ] showed that various congenital malformations are related to Abernethy malformation, and cardiac abnormalities are the most common. This patient presented with no abnormalities on cardiac ultrasound, so cardiac malformations could be excluded.…”

Section: Discussionmentioning

confidence: 99%

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Yao,

Liu,

et al. 2023

World J Radiol

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BACKGROUND Abernethy malformation is a rare congenital vascular malformation with a portosystemic shunt that may clinically manifest as cholestasis, dyspnea, or hepatic encephalopathy, among other conditions. Early diagnosis and classification are very important to further guide treatment. Typically, patients with congenital portosystemic shunts have no characteristics of portal hypertension. Herein, we report an 18-year-old female with prominent portal hypertension that manifested mainly as rupture and bleeding of esophageal varices. Imaging showed a thin main portal vein, no portal vein branches in the liver, and bleeding of the esophageal and gastric varices caused by the collateral circulation upwards from the proximal main portal vein. Patients with Abernethy malformation type I are usually treated with liver transplantation, and patients with type II are treated with shunt occlusion, surgery, or transcatheter coiling. Our patient was treated with endoscopic surgery combined with drug therapy and had no portal hypertension and good hepatic function for 24 mo of follow-up. CASE SUMMARY This case report describes our experience in the diagnosis and treatment of an 18-year-old female with Abernethy malformation type IIC and portal hypertension. This condition was initially diagnosed as cirrhosis combined with portal hypertension. The patient was ultimately diagnosed using liver histology and subsequent imaging, and the treatment was highly effective. To publish this case report, written informed consent was obtained from the patient, including the attached imaging data. CONCLUSION Abernethy malformation type IIC may develop portal hypertension, and traditional nonselective beta-blockers combined with endoscopic treatment can achieve high efficacy.

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Section: Discussionmentioning

confidence: 99%

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Yao,

Liu,

et al. 2023

World J Radiol

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“…Имеющиеся научные публикации преимущественно описывают отдельные клинические наблюдения этой мальформации у детей [2][3][4]. Изза редкости синдрома Абернети и сложности его диагностики проведены лишь единичные многоили одноцентровые исследования [1,5].…”

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“…Диагностика синдрома Абернети основывается исключительно на использовании визуализирующих методов: ультразвукового исследования (УЗИ) с доплерографией, компьютерной томографии (КТ), магнитнорезонансной томографии [1,5,7].…”

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“…При необходимости выполняют трансплантацию печени (в случае гепатоцеллюлярной карциномы), сосудистую реконструкцию, эндоваскулярные и открытые оперативные вмешательства [1,25]. При бессимптомном течении предпочтительна тактика динамического контроля [5].…”

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“…Рос журн гастроэнтерол гепатол колопроктол 2020; 30(5) / Rus J Gastroenterol Hepatol Coloproctol 2020; 30(5) …”

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Abernethy Malformation in Combination with Gilbert’s Syndrome

Губергриц¹,

Бондарь²,

Dyadyk

et al. 2020

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

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Aim. To present a clinical case of the Abernethy syndrome.Key points. Abernethy syndrome is a rare vascular anomaly associated with a congenital absence of the portal vein, as a result of which portal blood from the intestines and spleen drains directly into the systemic circulation bypassing the liver though a complete or partial shunt. In the vast majority of cases, Abernethy syndrome is manifested during the newborn period by jaundice syndrome, hypergalactosemia and encephalopathy. In rare cases, this vascular malformation is diagnosed in older patients during ultrasound screening. A 31 year-old patient sought medical attention with the complaints of sleep disturbance and fatigue. The conducted instrumental observation revealed echo-signs of malformation (agenesia) of the portal vein, which was further confirmed by both X-ray-contrast computed tomography and the pathohistological analysis of liver biopsy slides. The genotype UGT1A1•28 confirmed Gilbert's syndrome. Neutropenia (0.8 × 109/L) with a drop in the level of segmented neutrophils up to 27% was regarded as shunt neutropenia. Number connection test confirmed shunt encephalopathy. Conservative therapy for correcting hepatic encephalopathy was prescribed, followed by a dynamic monitoring of the patient’s condition.Conclusion. Diagnosis of Abernethy malformation is important for choosing the right treatment for the timely correction of complications of the disease and early detection of adenoma or hepatocellular carcinoma.

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Abernethy Malformation and Gastrointestinal Bleeding: A Case Report and Literature Review

Li,

Liu,

Feng

2024

Vasc Endovascular Surg

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Background: Congenital extrahepatic portosystemic shunt (CEPS), also termed Abernethy malformation (AF) is a rare anomaly of the splanchnic venous system. Several approaches, including shunt closures through surgical or radiological interventions and liver transplantations, have been proposed, but clear comparisons among different treatment strategies are still unavailable. Purpose: We report a case in which an unusual portosystemic shunt was present between the dilated inferior mesenteric vein (IMV) to the right ovarian vein. A mini literature review of AF patients presented with gastrointestinal (GI) tract bleeding. Research design: Case report and literature review. Data Collection: An electronic search of PubMed was performed from inception to December 2023. Results: 34 AF patients presented with GI tract bleeding were identified published in the literature. The proportion of type II AF patients presenting with GI bleeding is greater (79%). Conclusions: We regard that both surgical ligation and endovascular closure of the shunt are effective and safe treatments for these patients, but coils embolization alone may not be sufficient to completely close the shunt when the shunt flow is high.

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The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

Cited by 4 publications

References 21 publications

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Abernethy Malformation in Combination with Gilbert’s Syndrome

Abernethy Malformation and Gastrointestinal Bleeding: A Case Report and Literature Review

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