Case Report
Copyright ©The Author(s) 2020.
World J Gastroenterol. Oct 7, 2020; 26(37): 5731-5744
Published online Oct 7, 2020. doi: 10.3748/wjg.v26.i37.5731
Table 1 Patients' clinical features, management and outcome
Case/genderType of shuntAge at diagnosisAssociated congenital anomaliesMain clinical features at presentationAdditional clinical features and development of complicationsManagementOutcome
1/MIntrahepatic8 yrCongenital vascular malformation of the left shoulderDiffuse abdominal pain and gastrointestinal haemorrhageSigns of increased blood flow through pulmonary circulation, prominent gastric submucosal vasculaturePercutaneous closure of the shunt with Amplatzer plugAge 13 yr: No shunt present, no signs and symptoms
2/MIntrahepatic< 1 moDuodenal membraneDirect hyperbilirubinemia, increased liver enzymesNoneConservative treatment with close follow-upAge 8 mo: Spontaneous shunt regression, no signs or symptoms
3/MExtrahepatic (1A)22 moNonePulmonary arterial hypertension, hyperammonemiaWorsening of pulmonary hypertension, basal ganglia hyperintensity on brain MRILiver transplantation at five years of ageAge 14 yr: Persistent pulmonary arterial hypertension
4/MExtrahepatic (1A)< 1 moKlippel–Trénaunay syndromeHyperbilirubinemia, hyperammonemia, hypovitaminosisOsteoporosis, hypoglycemic episodes, regenerative liver nodulesConservative treatment with close follow-upAge 9 yr: Shunt present, no further complications
5/FExtrahepatic (1B)14 yrASD type secundumBone fractures from severe osteoporosisFocal nodular hyperplasia, basal ganglia hyperintensity on brain MRIConservative treatment with close follow-upAge 18 yr: Shunt present, no further complications, stable hepatic lesions