Cavernous transformation of the portal vein causing jaundice, presenting in the form of Wilson's disease.
The following is a case review of portal vein cavernous malformation presenting with intermittent cholestasis and jaundice in a 4 year old child. Correct assessment was supported by radiology, later laparoscopy, yet hindered by histopathology representative Wilson's disease and elevated urinary copper excretion. During surgical procedure the stenosis of the common bile duct secondary to extremely dilated portal vein reticulation was solved by Roux-en-Y choledochojejunostomy. After a one-year follow up the child remains asymptomatic.
Dept of Pediatrics, Petz Aladár County Teaching Hospital, Hungary.
V
V
Ruszinkó
M
M
Kovács
L
L
Szönyi
T
T
Verebély
P
P
Willner
D000713:Anastomosis, Roux-en-Y; D018376:Cardiovascular Abnormalities / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D002675:Child, Preschool; D002768:Choledochostomy / Q000379:methods*; D002779:Cholestasis / Q000209:etiology*; D003937:Diagnosis, Differential; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis; D006801:Humans; D007565:Jaundice / Q000209:etiology*; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery