Percutaneous treatment of a huge patent ductus venosus and severe portal vein hypoplasia using a Figulla Flex ll ASD-occluder in a two-year-old infant: a case report

Abstract Background Intra- or extrahepatic porto-caval shunts can account for multiorgan dysfunction with pulmonary arterial hypertension and portosystemic encephalopathy as the most serious consequences of bypass of the hepatic circulation. The ductus venosus represents a rare Foetal porto-caval shunt and might be persistently patent in newborns after birth. Treatment strategies include surgical ligation and percutaneous device closure. The degree of portal vein hypoplasia limits therapy making liver transplantation the only option in some of them. Case summary In a newborn female patient a huge persistently patent ductus venosus, known already prenatally, resulted in severe secondary portal vein hypoplasia. She presented with hyperammonemia, elevated liver enzymes and pulmonary hypertension. With only diminutive portal venous branches and exceedingly high portal venous pressures during test-occlusion of the ductus venosus, shunt closure was not possible. At the age of two years more favorable portal venous pressures allowed transcatheter device closure with a nitinol ASD-occlusion device. Pulmonary artery pressures and ammonia levels normalized after the procedure without any signs of portal hypertension. Discussion The case highlights the importance of meticulous imaging using balloon occlusion angiography of porto-caval shunts like the ductus venosus, to search for intrahepatic portal veins. Moreover, portal vein pressure during test-occlusion can identify patients amenable for surgical or endovascular shunt closure. Occlusion devices licensed for other indications like atrial septal defect closure can be used safely in huge porto-caval shunt vessels in a one-step or staged procedure. Optimal timing of the intervention should be tailored to the patient’s needs

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Transjugular Retrograde Cannulation of the Portal Vein via Patent Ductus Venosus: Alternative Access for Endovascular Hepatic Interventions

Journal of Vascular and Interventional Radiology ◽

10.1016/j.jvir.2012.09.027 ◽

2013 ◽

Vol 24 (1) ◽

pp. 81-84 ◽

Cited By ~ 1

Author(s):

Ossama Al Masalmeh ◽

Raja Shaikh ◽

Gulraiz Chaudry ◽

Heung Bae Kim ◽

Steven J. Fishman ◽

...

Keyword(s):

Portal Vein ◽

Ductus Venosus ◽

Patent Ductus Venosus ◽

Patent Ductus

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Portal Vein Hypoplasia and Patent Ductus Venosus

Operative General Surgery in Neonates and Infants ◽

10.1007/978-4-431-55876-7_47 ◽

2016 ◽

pp. 295-298

Author(s):

Yukihiro Inomata ◽

Shintaro Hayashida

Keyword(s):

Portal Vein ◽

Ductus Venosus ◽

Patent Ductus Venosus ◽

Patent Ductus

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The Clinical and Imaging Findings of Patent Ductus Venosus in Children: A Series of Nine Cases

10.21203/rs.3.rs-1152906/v1 ◽

2021 ◽

Author(s):

Yonghua Xiang ◽

Yinghui Peng ◽

Qing Gan ◽

Xiaojun Jiang ◽

Ke Jin

Keyword(s):

Portal Vein ◽

Medical Records ◽

Brain Mri ◽

Vena Cava ◽

Ductus Venosus ◽

Imaging Findings ◽

Laboratory Findings ◽

Patent Ductus Venosus ◽

Clinical Presentations ◽

Patent Ductus

Abstract Purpose: To retrospectively analyze the clinical and imaging findings of patent ductus venosus (PDV) ,so as to improve the diagnostic accuracy of the disease and reduce missed diagnosis and misdiagnosis. Methods Medical records of 9 patients with PDV were retrospectively reviewed. The clinical, and laboratory test data were extracted from the electronic medical records. Two radiologists and one sonographer reviewed all imaging examinations retrospectively. Results 9 patients with PDV were reviewed, including 7 males and 2 females, aged from 16 days to 16.5 years. The initial clinical presentations of PDV were varied, but jaundice and respiratory symptoms were the most common. Laboratory tests showed that 5/9 cases had hypoxemia, 2/9 had hyperammonemia, 7/9 had hyperbilirubinemia, 6/9 had abnormal coagulation function, 4/9 had abnormal myocardial enzymes, 8/9 had hepatic dysfunction, and 3/9 had renal dysfunction. The direct imaging sign of PDV was a vascular structure connecting the left branch of portal vein(LPV) to the inferior vena cava(IVC), running in the depth of the Arantius sulcus. The secondary imaging findings were as follows: All the patients had enlarged liver. 3 patients presented with hypoperfusion in right lobe of liver. The spleen was enlarged in 8 cases but shrank in one. Dilated LPV and atrophic right branch of portal vein (RPV) were observed in all patients. The main portal vein (MPV) was dilated in 8 cases and shrank in one. Dilated right heart and pulmonary artery were observed in all cases. Abnormal renal imaging was observed in 2 patients. The complications and coexistent malformations were as follows: Brain MRI indicated hepatic encephalopathy in 4 cases. 7/9 patients were combined with other malformations, and the most common coexistent malformations were congenital heart disease (CHD) and vascular abnormalities, with 5 and 6 cases respectively. Conclusions PDV can lead to multi-system lesions. The clinical presentations and laboratory findings were diverse. The diagnosis of PDV depends on imaging examinations, and it is important to evaluate the secondary imaging changes. Complications and coexistent malformations were common, and we need to prevent omissions during the imaging evaluations.

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An adult case of primary biliary cholangitis accompanied by a patent ductus venosus showing type-I congenital absence of the portal vein

Kanzo ◽

10.2957/kanzo.59.217 ◽

2018 ◽

Vol 59 (4) ◽

pp. 217-223

Author(s):

Soichiro Kiyono ◽

Hitoshi Maruyama ◽

Kazufumi Kobayashi ◽

Taro Shimada ◽

Masanori Takahashi ◽

...

Keyword(s):

Portal Vein ◽

Congenital Absence ◽

Ductus Venosus ◽

Primary Biliary Cholangitis ◽

Type I ◽

Adult Case ◽

Patent Ductus Venosus ◽

Patent Ductus

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The Clinical and Imaging Findings of Patent Ductus Venosus in Children: A Retrospective Analysis

10.21203/rs.3.rs-154362/v1 ◽

2021 ◽

Author(s):

Yonghua Xiang ◽

Yinghui Peng ◽

Siping He ◽

Qing Gan ◽

Ke Jin

Keyword(s):

Portal Vein ◽

Medical Records ◽

Laboratory Tests ◽

Brain Mri ◽

Vena Cava ◽

Ductus Venosus ◽

Imaging Findings ◽

Patent Ductus Venosus ◽

Clinical Presentations ◽

Patent Ductus

Abstract Purpose Patent ductus venosus (PDV) was a rare congenital portosystemic shunt, and few studies have been reported to date. The purpose of this study was to investigate the clinical and imaging findings to improve the recognition of PDV. Materials and Methods Medical records of 9 patients with PDV in Hunan Children’s Hospital from May 2013 to December 2020 were retrospectively reviewed. The clinical, and laboratory tests data were extracted from the electronic medical records. Two pediatric radiologists evaluated all imaging examinations of the patients.Results 9 patients with PDV were reviewed, including 7 males and 2 females, aged from 16 days to 16.5 years, median age was 1.6 years. PDV diameter ranged from 4.0mm to 17.5mm. The initial clinical presentations of PDV were varied, but jaundice and respiratory symptoms were the most common. Laboratory tests showed that 5/9 cases had hypoxemia, 2/9 had hyperammonemia, 7/9 had hyperbilirubinemia, 6/9 had abnormal coagulation function, 4/9 had abnormal myocardial enzymes, 8/9 had hepatic dysfunction, and 3/9 had renal dysfunction. The direct imaging sign of PDV was a vascular structure connecting the left branch of portal vein(LPV) to the inferior vena cava(IVC), running in the depth of the Arantius sulcus. The secondary imaging findings were as follows: All the patients had enlarged liver, especially the left lobe, and one of the patients presented with diffuse nodules in the liver. 3 patients presented with hypoperfusion in right lobe of liver. The spleen was enlarged in 8 cases but shrank in one. Dilated LPV and atrophic right branch of portal vein (RPV) were observed in all patients. The main portal vein (MPV) was dilated in 8 cases and shrank in one. Dilated right heart and pulmonary artery were observed in all cases. Abnormal renal imaging was observed in 2 patients. The complications and coexistent malformations were as follows: Brain MRI indicated hepatic encephalopathy in 4 cases. 7/9 patients were combined with other malformations, and the most common coexistent malformations were congenital heart disease (CHD) and vascular abnormalities, with 5 and 6 cases respectively. Conclusions PDV was a rare vascular malformation that can lead to multi-system lesions. The clinical presentations and laboratory findings were diverse. The diagnosis of PDV mainly depends on imaging examinations, and it is important to evaluate the secondary imaging changes. Complications and coexistent malformations were common, and we need to prevent omissions during the imaging evaluations.

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