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Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes
Frank DiPaola1, Andrew T Trout2,3, Ashley E Walther4
1Division of Pediatric Gastroenterology, Hepatology, and Nutrition, C.S. Mott Children's Hospital, MPB D5200, 1500 East Medical Center Drive, SPC 5718, Ann Arbor, MI, 48109-5718, USA. dipaolaf@med.umich.edu.
Insights
Congenital portosystemic shunt (CPSS) is a rare condition linked to other anomalies. Early diagnosis and management of CPSS, including liver lesions, are crucial for preventing serious complications in children and young adults.
Area of Science:
- Pediatric Surgery
- Hepatology
- Medical Malformations
Background:
- Congenital portosystemic shunt (CPSS) is a rare vascular malformation bypassing the liver.
- It is frequently associated with other congenital anomalies and syndromes.
- CPSS and its management are underreported, necessitating further clinical review.
Purpose of the Study:
- To review clinical characteristics, management strategies, and outcomes of CPSS in pediatric patients.
- To define CPSS anatomy, associated anomalies, and complications.
- To characterize liver lesions and their management in CPSS patients.
Main Methods:
- Retrospective review of 11 CPSS cases from two pediatric centers.
- Detailed characterization of CPSS anatomy, associated anomalies, and clinical presentations.
- Analysis of imaging features and histopathology of liver lesions, interventions, and outcomes.
Main Results:
- The cohort (median age 10 years) showed high prevalence of heterotaxy, congenital heart disease, and Turner syndrome.
- Hyperammonemia/encephalopathy occurred in 73% of cases; hepatopulmonary syndrome and pulmonary hypertension were also observed.
- Eight patients had liver lesions, including premalignant adenomas and hepatocellular carcinoma (HCC), managed with occlusion, ligation, or liver transplantation.
Conclusions:
- CPSS is associated with significant anomalies and syndromes, requiring careful evaluation.
- Liver lesions in CPSS patients carry a risk of premalignant adenomas and HCC.
- Timely management, including endovascular or surgical interventions, can address serious CPSS complications.
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.
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