Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes

Frank DiPaola, Andrew T. Trout, Ashley E. Walther, Anita Gupta, Rachel Sheridan, Kathleen M. Campbell, Greg Tiao, Jorge A. Bezerra, Kevin E. Bove, Manish Patel, Jaimie D. Nathan

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


Background: Congenital 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. Methods: Cases 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. Results: A 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. Conclusions: CPSS 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.

Original languageEnglish (US)
Pages (from-to)1239-1251
Number of pages13
JournalDigestive Diseases and Sciences
Issue number4
StatePublished - Apr 1 2020
Externally publishedYes


  • Abernethy
  • Hepatocellular adenoma
  • Hepatocellular carcinoma
  • Liver
  • Portosystemic shunt

ASJC Scopus subject areas

  • Physiology
  • Gastroenterology


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