Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration

Bellah, Richard D.; Hayek, Jorge Elie; Teele, Rita L.

doi:10.1007/bf02010654

Cited by 50 publications

(40 citation statements)

References 5 publications

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“…Goldenhar reported congenital portal vein abnormalities, including cardiac, musculoskeletal and genitourinary abnormality and named it Goldenhar syndrome. Less than 200 cases have been reported in the medical literature so far and only one case of HOCM associated with portal vein anomaly has been reported previously by RD Bellah and colleagues [2]. Our case report is of clinical significance deserves attention and needs to be reported in the medical literature.…”

Section: Introductionmentioning

confidence: 56%

“…In 1989 R.D Bellah and colleagues reported a case of portal vein abnormality with fatal congenital hypertrophic cardiomyopathy [2]. The close relationship between the development of vitelline veins and the heart in embryonic life may be responsible for the association between the cardiovascular malformations and congenital abnormality of portal vein [6].…”

Section: Discussionmentioning

confidence: 99%

“…Congenital abnormality of portal vein associated with cardiac, genitourinary and musculoskeletal abnormality is a rare entity [1,2]. Congenital portal vein anomaly was first reported by Abernathy in 1793 [3].…”

Section: Introductionmentioning

confidence: 99%

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Goldenhar Syndrome

Priti¹,

Mulimani²,

Nivedita³

2015

JCR

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Abstract:A 20 year old male patient presented with ascites since birth, swelling of right scrotum, absence of development of secondary sexual characters, breathlessness on exertion, short limb gait, and hematemesis. Ultrasound imaging and CT angiography of the abdomen revealed absence of portal vein, thrombus at the junction of superior mesenteric vein and splenic vein, cavernous transformation and development of collaterals. Echocardiography revealed hypertrophic obstructive cardiomyopathy (HOCM) and X-ray hip revealed absence of head of femur. Scrotal examination revealed right sided indirect inguinal hernia and hydrocoele. A diagnosis of Goldenhar syndrome was made.Goldenhar syndrome consists plethora of clinical features comprising congenital portal vein absence with associated cardiac defects, skeletal abnormality and urogenital defects. Our case deserves attention and needs to be reported because so far in the medical literature only one case of congenital portal vein absence with HOCM has been reported and also no other case of congenital portal vein absence with thrombosis at the SMV and SV junction associated with HOCM has been reported in the medical literature.

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Section: Introductionmentioning

confidence: 56%

Section: Discussionmentioning

confidence: 99%

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Goldenhar Syndrome

Priti¹,

Mulimani²,

Nivedita³

2015

JCR

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“…Congenital cardiac diseases including atrial septal defect (ASD) [2,21,26,28,37,44,45] or patent foramen ovale [43] , ventricular septal defect (VSD) [16,17,21,26,31,40] , and patent ductus arterious (PDA) [12,23,43,44] are frequently observed along with CAPV. Bellah et al [45] have also detected a fatal congenital hypertrophic cardiomyopathy with ultrasound in an infant. Dextrocardia [1] and Mesocardia [44] that reported congenital cardiac abnormalities are rarely found in CAPV cases.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Dextrocardia [1] and Mesocardia [44] that reported congenital cardiac abnormalities are rarely found in CAPV cases. Congenital stenosis of aortic valve [11] and pulmonary artery valve [45] , found in a few CAPV cases, can cause tricuspid regurgitation [44] . Even stenosis [11] or coarctation [12] has been observed in aorta.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Insight into congenital absence of the portal vein: Is it rare?

Shen

Zhu²

2008

WJG

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Color Doppler sonography in the diagnosis of neonatal intrahepatic portosystemic shunts

Valls

Ceres

Urbaneja

et al. 2000

J. Clin. Ultrasound

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Intrahepatic portosystemic shunts are infrequent in children. We report 3 cases of neonates who presented with jaundice during the first month of life. Color Doppler sonography in the first 2 cases showed direct communication between the right portal and hepatic veins. Both infants were asymptomatic, and the shunts disappeared spontaneously. The third case involved several shunts and an aberrant medial portal vein. This patient developed heart failure and died after surgery. Color Doppler sonography was useful in the diagnosis and follow‐up of the shunts in all 3 cases. © 2000 John Wiley & Sons, Inc. J Clin Ultrasound 28:42–46, 2000.

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Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration

Abstract: The authors report a congenital portosystemic shunt which was detected with ultrasound in an infant with a fatal congenital hypertrophic cardiomyopathy. An aberration in primitive vitelline venous development may have contributed to the formation of this unusual vessel.

Cited by 50 publications

References 5 publications

Goldenhar Syndrome

Goldenhar Syndrome

Insight into congenital absence of the portal vein: Is it rare?

Color Doppler sonography in the diagnosis of neonatal intrahepatic portosystemic shunts

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