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

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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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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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

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytab455 ◽

2021 ◽

Author(s):

Marek Kardos ◽

Erwin Kitzmueller ◽

Peter Olejnik ◽

Ina Michel-Behnke

Keyword(s):

Portal Vein ◽

Ductus Venosus ◽

Optimal Timing ◽

Device Closure ◽

Atrial Septal Defect Closure ◽

Patent Ductus Venosus ◽

Occlusion Device ◽

Elevated Liver Enzymes ◽

Shunt Closure ◽

Patent Ductus

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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Hepatic manifestations of familial patent ductus venosus in adults

Gut ◽

10.1136/gut.45.3.442 ◽

1999 ◽

Vol 45 (3) ◽

pp. 442-445 ◽

Cited By ~ 37

Author(s):

S Jacob ◽

G Farr ◽

D De Vun ◽

H Takiff ◽

A Mason

Keyword(s):

Umbilical Vein ◽

Vena Cava ◽

Vascular Anomaly ◽

Fatty Degeneration ◽

Ductus Venosus ◽

Fetal Life ◽

Genetic Trait ◽

Patent Ductus Venosus ◽

Collateral Vein ◽

Patent Ductus

BACKGROUNDThe ductus venosus connects the umbilical vein to the inferior vena cava during fetal life and subsequently closes rapidly after birth. It is known as patent ductus venosus when it remains patent in adulthood.PATIENTSA 43 year old man with a history of panhypopituitarism presented with recurrent bouts of pedal oedema associated with fatigue, hypoalbuminaemia, and elevated prothrombin time. An ultrasound examination of his abdomen with Doppler revealed notable attenuation of the main portal vein with diminished intrahepatic branches; a computed tomography scan with angiography revealed a large collateral vein within the liver consistent with a patent ductus venosus. Sequential liver biopsies showed a considerable reduction in the calibre and number of the portal veins. His younger brother, who was diagnosed with alcohol related cirrhosis, suffered from intermittent bouts of encephalopathy and was found to have the same vascular lesion. A third brother was found to have a patent ductus venosus as well as two large hepatic masses consistent with focal nodular hyperplasia.CONCLUSIONThe syndrome of familial patent ductus venosus has only previously been described in three infant brothers who presented with hepatic encephalopathy and fatty degeneration of the liver. This report documents three brothers with a patent ductus venosus presenting in adulthood with different manifestations of liver disease. The presence of the same vascular anomaly in three brothers is highly suggestive of a recessive genetic trait with an anatomical manifestation of patent ductus venosus.

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