Presentation, Management, and Outcome of Congenital Portosystemic Shunts in Children: The Boston Children’s Hospital Experience

We describe presentations, complications, associations, and outcomes of CPSS at Boston Children’s Hospital (BCH). Methods: This was a retrospective review of children with CPSS at BCH from 2000 to 2020. Results: Twenty-nine patients had CPSS (17 girls): 14 extrahepatic (EH) and 15 intrahepatic (IH). At diagnosis, 15 were ≤5 days, 7 1 year (range 1–19). Median follow-up duration was 5.2 years (interquartile range [IQR] 1.6–10.9) in EH and 2.2 years (0.2–4.2) in IH CPSS. The most common presentation was antenatal ultrasound 13 (45%) followed by hyperammonemia 10 (34%), whereas 6 (21%) were asymptomatic. Complications were noted in 17 (12/14 EH vs 6/15 IH, P = 0.008). Associated anomalies were present in 25 (14/14 EH vs 11/15 IH, P = 0.10). Spontaneous closure was observed in 8 (28%) patients with IH CPSS, all
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research