Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome)

Morse, Steven S.; Taylor, K. J. W.; Strauss, Edward B.; Ramirez, Enrique; Seashore, John H.

doi:10.1007/bf02386831

Cited by 73 publications

(61 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In addition, the girl had macrostomia and bilateral ear tags and pits, as in OAV defect. We are not aware of other examples of OAV defect with vena cava anomalies, although a case with congenital absence of the portal vein has been reported [Morse et al, 1986]. Our findings support the hypothesis that OAV defect may be part of a malformation complex involving organization of asymmetric structures.…”

Section: Discussionsupporting

confidence: 87%

Anomalous inferior and superior venae cavae with oculoauriculovertebral defect: Review of Goldenhar complex and malformations of left-right asymmetry

et al. 1998

View full text Add to dashboard Cite

We observed a girl with an interrupted, left inferior vena cava with hemiazygous continuation, bilateral superior venae cavae, heart defects, and sacral agenesis. She had macrostomia and bilateral ear tags and pits, as in oculoauriculovertebral defect. Maternal diabetes was present. The combination, which we call OAV-heterotaxia complex, supports the view that some cases of oculoauriculovertebral defect may be part of a midline field defect of blastogenesis.

show abstract

Section: Discussionsupporting

confidence: 87%

Anomalous inferior and superior venae cavae with oculoauriculovertebral defect: Review of Goldenhar complex and malformations of left-right asymmetry

et al. 1998

View full text Add to dashboard Cite

show abstract

“…This anomaly has been reported mostly as a congenital absence of the portal vein in association with liver tumors and other congenital anomalies [1][2][3][4][5][6][7][8][9][10]. In this report, we present the case of a patient with congenital extrahepatic portocaval shunt in association with Dubin-Johnson syndrome who developed hyperplastic nodules in the liver.…”

mentioning

confidence: 87%

Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin–Johnson syndrome

Mori

Kimura

et al. 2000

Abdom Imaging

View full text Add to dashboard Cite

We report a rare case of congenital extrahepatic portocaval shunt diagnosed during evaluation of hyperplastic nodules in the liver. Diagnostic imagings showed hypoplasia of the intrahepatic portal venous system and splanchnic portal venous return to the inferior vena cava through aberrant vessels. Altered hepatic blood flow dynamics due to this shunt may have been implicated in the etiology of the hepatic hyperplastic nodules.

show abstract

“…Morse et al reported a patient with CAPV who underwent liver transplantation for hepatoblastoma that was initially diagnosed FNH 2 years previously (6,7). This suggests that long-term follow-up and monitoring for malignancy should be mandatory, even with an initial diagnosis of benign tumor.…”

Section: Discussionmentioning

confidence: 99%

“…To our knowledge, only 35 cases of CAPV have been reported in the literature (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). The age at diagnosis ranges from 1 day to 64 years.…”

Section: Introductionmentioning

confidence: 99%

Congenital Absence of the Portal Vein Complicating Hepatic Tumors

et al. 2004

View full text Add to dashboard Cite

Congenital absence of the portal vein (CAPV) is a rare malformation that is often accompanied by other anomalies such as cardiac and skeletal malformations and/or hepatic tumors. We describe here a case of CAPV complicating hepatic tumors in a 16-year-old Japanese girl. Abdominal ultrasonography revealed a hyperechoic tumor in the liver and dilatation of the portal vein that appeared to be connected directly with the inferior vena cava. Subsequent abdominal computed tomography (CT) revealed tumors and magnetic resonance angiography confirmed that the portal vein entered directly into the inferior vena cava just above the liver. In addition, there was absence of the right portal vein and the left intrahepatic branch except for the presence of left portal vein only within the porta hepatis. These findings led to a diagnosis of CAPV complicated hepatic tumors. Careful monitoring of these hepatic tumors is ongoing due to the possibility of malignant transformation. (Internal Medicine 43: 194-198, 2004)

show abstract

Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome)

Cited by 73 publications

References 6 publications

Anomalous inferior and superior venae cavae with oculoauriculovertebral defect: Review of Goldenhar complex and malformations of left-right asymmetry

Anomalous inferior and superior venae cavae with oculoauriculovertebral defect: Review of Goldenhar complex and malformations of left-right asymmetry

Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin–Johnson syndrome

Congenital Absence of the Portal Vein Complicating Hepatic Tumors

Contact Info

Product

Resources

About