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Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management
- Source :
- Seminars in Liver Disease. 32:273-287
- Publication Year :
- 2013
- Publisher :
- Georg Thieme Verlag KG, 2013.
-
Abstract
- Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.
- Subjects :
- medicine.medical_specialty
Vascular Malformations
Hypertension, Pulmonary
medicine.medical_treatment
Prenatal diagnosis
Liver transplantation
Revascularization
Prenatal Diagnosis
medicine
Humans
Neonatal cholestasis
Hepatopulmonary syndrome
Portopulmonary hypertension
Cholestasis
Hepatology
medicine.diagnostic_test
Portal Vein
business.industry
Liver Neoplasms
Infant, Newborn
Infant
Interventional radiology
medicine.disease
Hepatic Encephalopathy
Radiology
business
Ductus venosus
Hepatopulmonary Syndrome
Subjects
Details
- ISSN :
- 10988971 and 02728087
- Volume :
- 32
- Database :
- OpenAIRE
- Journal :
- Seminars in Liver Disease
- Accession number :
- edsair.doi.dedup.....fcdcee782ff734efc6748432766b8d57