Advertisement

Extrahepatic vitelline vein aneurysm: prenatal diagnosis and follow up

      Abstract

      Umbilical vein varix is a well-described prenatal anomaly in which the prognosis remains unclear. We describe a very rare venous malformation that mimicked an umbilical vein varix consisting of a persistent vitelline vein. From 2003 to 2010, three patients were referred starting at 20 weeks gestation to our prenatal centers for an umbilical vein varix diagnosis. Fetal follow up was unremarkable, with the exception of the dilated vein size (mean: 35 mm at 33 weeks gestation). After birth, the three children presented with thrombosis from the aneurysmal sac to the portal trunk. All the children underwent surgical thrombectomy and resection of the aneurysmal sac after birth. Operative findings showed no umbilical vein but an abnormal dilated and thrombosed vein coming from the umbilicus to the portal vein following the right vitelline vein trajectory. One child was treated with systemic heparin. Median follow up is 5.6 years. Currently, one patient has a normal portal flow. The other two have persistent portal vein thrombosis with portal cavernoma and portal hypertension. This malformation is rare and should be considered in cases of early diagnosed umbilical vein varix whose diameter is greater than 20 mm. We advocate an early surgical thrombectomy with heparinization to prevent portal vein thrombosis.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Benoist G.
        • Gauthier F.
        • Belloy F.
        • et al.
        Antenatal sonographic features of aneurysmal dilatation of a vitelline vein.
        Ultrasound Obstet Gynecol. 2007; 29: 708-711
        • Fasouliotis S.J.
        • Achiron R.
        • Kivilevitch Z.
        • et al.
        The human fetal venous system: normal embryologic, anatomic, and physiologic characteristics and developmental abnormalities.
        J Ultrasound Med. 2002; 21: 1145-1158
        • Mavrides E.
        • Moscoso G.
        • Carvalho J.S.
        • et al.
        The anatomy of the umbilical, portal and hepatic venous systems in the human fetus at 14–19 weeks of gestation.
        Ultrasound Obstet Gynecol. 2001; 18: 598-604
        • Rahman N.
        • Al-Nassar S.
        • Davenport M.
        Congenital mesenterico-portal (Rex) shunt.
        Pediatr Surg Int. 2002; 18: 514-516
        • Kivilevitch Z.
        • Achiron R.
        Fetal extrahepatic vitelline vein aneurysm mimicking an umbilical vein varix.
        J Ultrasound Med. 2010; 29: 1651-1656
        • Moon S.B.
        • Park K.W.
        • Jung S.E.
        Abnormal direct entry of the umbilical vein into the portal vein: report of a case.
        Pediatr Surg Int. 2008; 24: 1243-1245
        • Schild H.
        • Schweden F.
        • Braun B.
        • et al.
        Aneurysm of the superior mesenteric vein.
        Radiology. 1982; 145: 641-642
        • Mahony B.S.
        • McGahan J.P.
        • Nyberg D.A.
        • et al.
        Varix of the fetal intra-abdominal umbilical vein: comparison with normal.
        J Ultrasound Med. 1992; 11: 73-76
        • Sepulveda W.
        • Mackenna A.
        • Sanchez J.
        • et al.
        Fetal prognosis in varix of the intrafetal umbilical vein.
        J Ultrasound Med. 1998; 17: 171-175