Congenital intrahepatic portocaval shunt associated with trimethylaminuria

Pediatr Surg Int. 1997 Feb;12(2-3):196-7.

Abstract

A case of congenital portal-systemic shunting due to an intrahepatic connection diagnosed by ultrasound scanning and color Doppler in an 8-month-old girl is reported. She began to manifest trimethylaminuria 3 years later. At 7 years of age, she is asymptomatic without therapeutic measures except for diet. This is the seventh reported case and the third in a child to our knowledge.

Publication types

  • Case Reports

MeSH terms

  • Arteriovenous Malformations / diagnostic imaging
  • Arteriovenous Malformations / surgery*
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Liver / blood supply*
  • Metabolism, Inborn Errors / complications
  • Methylamines / urine*
  • Oxidoreductases, N-Demethylating / deficiency
  • Palliative Care
  • Portal Vein / abnormalities*
  • Portal Vein / diagnostic imaging
  • Ultrasonography, Doppler, Color*
  • Vena Cava, Inferior / abnormalities*
  • Vena Cava, Inferior / diagnostic imaging

Substances

  • Methylamines
  • Oxidoreductases, N-Demethylating
  • trimethylamine dehydrogenase
  • trimethylamine