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

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Abstract

Absence of the portal vein with systemic visceral venous return was demonstrated in an 8-year old girl with oculoauriculovertebral dysplasia (Goldenhar syndrome) during preoperative evaluation of a liver mass. Congenital absence of the portal vein is a rare malformation of potential clinical significance.

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References

  1. Hellweg G (1954) Congenital absence of intrahepatic portal venous system simulating Eck fistula. Arch Pathol 57: 425

    CAS  Google Scholar 

  2. Olling S, Olsson R (1974) Congenital absence of portal venous system in a 50-year-old woman. Acta Med Scand 196: 343

    CAS  PubMed  Google Scholar 

  3. Marois D, Van Heerden JA, Carpenter HA, Sheedy PF (1979) Congenital absence of the portal vein. Mayo Clin Proc 54: 55

    CAS  PubMed  Google Scholar 

  4. Gorlin RJ, Jue KL, Jacbosen V, Goldschmidt E (1963) Oculoauriculovertebral dysplasia. J Pediatr 63: 991

    CAS  PubMed  Google Scholar 

  5. Peirpont MEM, Moller JH, Gorlin RJ, Edwards JE (1982) Congenital cardiac, pulmonary and vascular malformations in oculoauriculovertebral dysplasia. Pediatr Cardiol 2: 297

    Google Scholar 

  6. Marks C (1969) Developmental basis of the portal venous system. Am J Surg 117: 671

    Article  CAS  PubMed  Google Scholar 

  7. Starzl TE, Francavilla A, Halgrimson CG, Francavilla FR, Porter KA, Brown TH, Putnam CW (1973) The origin, hormonal nature, and action of hepatrophic substances in portal venous blood. Surg Gynecol Obstet 137: 179

    CAS  PubMed  Google Scholar 

  8. Williams JW, Britt LG, Peters TG, Vera SR, Haggitt RC (1984) Portal vein obstruction in patients requiring hepatic resection or transplantation. Am Surg 50: 465

    CAS  PubMed  Google Scholar 

  9. Atkinson GO, Kudroff M, Sones PJ, Gay BB (1980) Focal nodular hyperplasia of the liver in children: a report of three new cases. Radiology 137: 171

    PubMed  Google Scholar 

  10. Thomson EN, Williams R, Sherlock S (1964) Liver function in extrahepatic portal hypertension. Lancet 2: 1352

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Surgery, Yale University School of Medicine, New Haven, Connecticut, USA

    J. H. Seashore

  2. Department of Diagnostic Imaging, Yale University School of Medicine, 333 Cedar Street, 06510, New Haven, CT, USA

    S. S. Morse, K. J. W. Taylor, Edward B. Strauss & E. Ramirez

Authors
  1. S. S. Morse
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  2. K. J. W. Taylor
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  3. Edward B. Strauss
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  4. E. Ramirez
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  5. J. H. Seashore
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Morse, S.S., Taylor, K.J.W., Strauss, E.B. et al. Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol 16, 437–439 (1986). https://doi.org/10.1007/BF02386831

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  • DOI: https://doi.org/10.1007/BF02386831

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