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      Adult-onset portosystemic encephalopathy caused by patent ductus venosus successfully treated with endovascular coil embolization: a rare case report

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          Abstract

          Background

          Patent ductus venosus (PDV) is a congenital shunt between the portal vein (PV) and inferior vena cava (IVC). However, there are few reports on symptomatic adult-onset PDV, and the appropriate management of this condition remains unknown. In particular, there are few reports on the use of endovascular therapy for the treatment of patients with symptomatic adult-onset PDV. However, the strategies, indications, long-term efficacy, and safety of this therapy remain poorly understood. Here we report a rare case of adult-onset PDV successfully treated via endovascular coil embolization using a retrievable IVC filter.

          Case presentation

          A 35-year-old man with a clinical course of progressive general fatigue and ataxia for 3 months was diagnosed with depressive personality disorder in another hospital 2 months ago and then referred to our hospital for detailed examination and further treatment. Blood test results showed hyperammonemia, indicating hepatic encephalopathy. Contrast-enhanced multidetector computed tomography and transarterial portography revealed a portosystemic shunt that connected the left PV to IVC. Endovascular coil embolization was successfully performed after temporary balloon occlusion testing and the placement of a retrievable IVC filter. After the procedure, ammonia levels gradually reduced, and his symptoms improved without any postoperative complications. No clinical symptoms were observed at the 6-year clinical follow up.

          Conclusion

          This report supports the findings of other studies and offers a less invasive therapeutic option, thereby aiding clinicians in making appropriate treatment decisions for these patients.

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          Most cited references29

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          Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.

          Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods. Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications. Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.
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            Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment.

            Hepatic encephalopathy is suspected in non-cirrhotic cases of encephalopathy because the symptoms are accompanied by hyperammonaemia. However, the cause of the large portal-systemic shunt formation observed in these cases is not clear, as cirrhosis and portal hypertension are absent. The frequency of such cases reported in the literature is increasing with progress and spread of abdominal imaging diagnostic techniques. Some cases have been misdiagnosed as psychiatric diseases (dementia, depression and others) and consequently patients have been hospitalized in psychiatric institutions or geriatric facilities. Some paediatric cases have also been misdiagnosed. Therefore, the importance of accurate diagnosis of this disease should be strongly emphasized. Some paediatric cases have also been misdiagnosed. When psychoneurological symptoms are suggestive of hepatic encephalopathy but objective and subjective symptoms or abnormal values of liver function tests are not sufficiently indicative of liver cirrhosis, portal-systemic encephalopathy should be suspected. Abnormal angiograms of the portal vein, superior mesenteric vein or splenic vein are conclusive evidence of portal-systemic encephalopathy. Transrectal portal scintigraphy also provides information useful for detection of shunts and a quantitative estimation of shunt index. We classified the disease into five types based on whether the shunt is formed inside or outside the liver. Type I (intrahepatic type) designates cases in which shunts are located between the portal and systemic veins. Type II designates a type of intra/extrahepatic shunt that originates from the umbilical part of the portal vein and serpentines in the liver, then leaves the liver. Type III (extrahepatic type) occurs most frequently. Type IV (extrahepatic) is accompanied by shunts similar to those in type III, but hepatic pathology presents as idiopathic portal hypertension. Type V (extrahepatic) represents the congenital absence of the portal vein, where the superior mesenteric vein joins the intrahepatic inferior vena cava or the left renal vein. The prevalence of each type in our country was examined by a nationwide investigation. In addition to the conventional diet or drug treatments, obliteration by less invasive interventional radiology using a metallic coil and ethanol has recently been used more frequently than surgical occlusion of shunts. Shunt-preserving disconnection of portal and systemic circulation and partial splenic artery embolization are also performed. International investigation of the disease status and establishment of diagnostic and therapeutic methods for the disease are awaited and investigation of long-term prognosis after therapy is also necessary.
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              The ductus venosus and the mechanism of its closure.

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                Author and article information

                Contributors
                hiromimin3089@hotmail.co.jp
                Journal
                CVIR Endovasc
                CVIR Endovasc
                CVIR Endovascular
                Springer International Publishing (Cham )
                2520-8934
                25 May 2020
                25 May 2020
                December 2020
                : 3
                : 28
                Affiliations
                Department of Cardiovascular Intervention Radiology Center, Taijukai General Hospital Kaisei Hospital, 3-5-28 Muromachi, Sakaide-City, Kagawa 762-0007 Japan
                Article
                118
                10.1186/s42155-020-00118-1
                7246263
                32449102
                788e858d-6b8f-47d8-bb2e-4d08b89af3d2
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                : 19 December 2019
                : 8 April 2020
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2020

                symptomatic adult-onset patent ductus venosus,portosystemic encephalopathy,endovascular coil embolization

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