Hepatic manifestations of familial patent ductus venosus in adults

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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Fetal Intra-abdominal Umbilical Vein Varix Complicated with Patent Ductus Venosus and Atrial Septal Defect

Korean Journal of Perinatology ◽

10.14734/kjp.2015.26.4.344 ◽

2015 ◽

Vol 26 (4) ◽

pp. 344

Author(s):

Hye Il Jin ◽

Min Sun Na ◽

Youn Ho Shin ◽

Kyoung Ah Lim

Keyword(s):

Atrial Septal Defect ◽

Septal Defect ◽

Umbilical Vein ◽

Ductus Venosus ◽

Patent Ductus Venosus ◽

Umbilical Vein Varix ◽

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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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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Down Syndrome with Patent Ductus Venosus and Hepato-Biliary-Pancreatic Abnormalities

The Indian Journal of Pediatrics ◽

10.1007/s12098-015-1797-0 ◽

2015 ◽

Vol 83 (1) ◽

pp. 78-80 ◽

Cited By ~ 2

Author(s):

Hiroshi Yamaguchi ◽

Kiyotaka Kosugiyama ◽

Shohei Honda ◽

Okada Tadao ◽

Akinobu Taketomi ◽

...

Keyword(s):

Down Syndrome ◽

Ductus Venosus ◽

Patent Ductus Venosus ◽

Patent Ductus

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Patent ductus venosus in an infant with direct hyperbilirubinemia

Clinical Case Reports ◽

10.1002/ccr3.2266 ◽

2019 ◽

Vol 7 (7) ◽

pp. 1430-1434

Author(s):

Leila Kamali ◽

Maryam Moradi ◽

Shadi Ebrahimian ◽

Mahsa Masjedi Esfahani ◽

Mohamad Saleh Jafarpishe

Keyword(s):

Ductus Venosus ◽

Patent Ductus Venosus ◽

Patent Ductus

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Absent Ductus Venosus Associated with Partial Liver Defect

Case Reports in Obstetrics and Gynecology ◽

10.1155/2018/6591025 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Kenji Horie ◽

Hironori Takahashi ◽

Daisuke Matsubara ◽

Koichi Kataoka ◽

Rieko Furukawa ◽

...

Keyword(s):

Normal Development ◽

Ultrasound Examination ◽

Umbilical Vein ◽

Vascular Anomaly ◽

Male Infant ◽

Ductus Venosus ◽

Morphological Abnormality ◽

Morgagni Hernia ◽

The Right ◽

Absent Ductus Venosus

Absent ductus venosus (ADV) is a rare vascular anomaly. We describe a fetus/neonate with ADV with a partial liver defect. A 41-year-old woman was referred to our institute because of fetal cardiomegaly detected by routine prenatal ultrasound, which revealed absence of ductus venosus with an umbilical vein directly draining into the right atrium, consistent with extrahepatic drainage type of ADV. She vaginally gave birth to a 3,096-gram male infant at 38 weeks of gestation. Detailed ultrasound examination revealed a defect of the hepatic rectangular leaf at half a month postnatally. He showed normal development at 1.5 years of age with the liver abnormality and a Morgagni hernia. Liver morphological abnormality should also be considered as a complication of ADV.

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