ArticleDigestive diseases and sciences2020
Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.
Article in Digestive diseases and sciences, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 46 citations in OpenAlex.
- Early-Onset Gastric Varices After Fontan Completion Associated with a Pre-Existing Gastro-Renal Shunt.Pediatric cardiology · 2026Article
- Congenital portosystemic shunt with extensive collateralization in an adult with recurrent episodes of hyperammonemic encephalopathy.Angiogenesis · 2026Article
- Hemodynamic evolution of umbilical-portal stenosis mimicking portal vein agenesis: description of a prenatal case.Quantitative imaging in medicine and surgery · 2026Article
- Congenital Portosystemic Shunts: A Review.Journal of clinical and translational hepatology · 2026Review
- Endovascular closure of a large intrahepatic portosystemic shunt in a three-month-old infant: a case report and literature review.Frontiers in pediatrics · 2026Article
- Hepatocellular Carcinoma Arising From Well-Differentiated Hepatocellular Neoplasm of Uncertain Malignant Potential in a Patient With Abernethy Malformation.Case reports in surgery · 2026Article
- Congenital extrahepatic portosystemic shunts (Abernethy malformation): A report of two cases.The Journal of international medical research · 2025Article
- Abernethy malformation (Type II) presenting in a 6-day-old boy with Noonan syndrome: a case report.BMC pediatrics · 2025Article
- Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts.Journal of ultrasound · 2025Article
- Fetal Extrahepatic Portosystemic Venous Shunts: Prenatal Diagnosis Management and Therapy - 21 Years of Evolving Insights.Fetal diagnosis and therapy · 2025Article
- Congenital Portosystemic Shunt Presenting As Hyperammonemia Following Fontan Operation.JPGN reports · 2023Article
- Case Report: Early detection and intervention of congenital portosystemic shunts in children.Frontiers in oncology · 2023Article
- Progress of pulmonary hypertension and high manganese levels through congenital portosystemic shunt closure.Clinical case reports · 2022Article
- Review
- Clinical Developmental Cardiology for Understanding Etiology of Congenital Heart Disease.Journal of clinical medicine · 2022Review
- Presentation of Congenital Portosystemic Shunts in Children.Children (Basel, Switzerland) · 2022Review
- Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient.Frontiers in pediatrics · 2022Article
- Abernethy malformation: A comprehensive review.Diagnostic and interventional radiology (Ankara, Turkey) · 2022Review
- Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II.Frontiers in pediatrics · 2021Article
- Down Syndrome Patients with Congenital Portosystemic Shunts: A Case Report and Review.Case reports in gastroenterologyArticle
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundCongenital portosystemic shunt (CPSS) is a rare malformation in which splanchnic venous flow bypasses the liver. CPSS is associated with other congenital anomalies and syndromes and can be associated with life-threatening complications. CPSS and their management remain underreported in the literature. Here, we review the clinical characteristics, management, and outcomes of a cohort of children and young adults with CPSS from two pediatric centers.
methodsCases of CPSS from Cincinnati Children's Hospital Medical Center and C.S. Mott Children's Hospital were reviewed to define CPSS anatomy, associated anomalies, complications, interventions, and outcomes. The imaging features and histopathology of liver lesions were characterized in detail.
resultsA total of 11 cases were identified. Median age was 10 years (range 0-26); 8 (73%) cases were female. Associated anomalies included six patients with heterotaxy (55%), five patients with congenital heart disease (45%), three patients with Turner syndrome (27%), and two patients with omphalocele, exstrophy, imperforate anus, spinal defects (OEIS) complex (18%). Eight (73%) cases had hyperammonemia ± encephalopathy. A 4-month-old presented with hepatopulmonary syndrome, and 12-year-old presented with pulmonary hypertension. Eight patients (73%) had liver lesions including five with premalignant adenomas and three with well-differentiated hepatocellular carcinoma (HCC). Four children underwent successful CPSS occlusion/ligation. Three children underwent liver transplant (2) or resection (1) for HCC without recurrence at extended follow-up.
conclusionsCPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.
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