| Summary: | Introduction: Congenital portosystemic shunts (CPSS) are rare vascular malformations that can lead to severe complications. With advanced imaging techniques, diagnosis is becoming more feasible occurring in fetal life. Different approaches have been adopted to manage these cases, with an increased utilization of interventional therapy recently. This cohort aims to describe the course of children diagnosed with CPSS and the impact of interventional therapy on the outcome. Methods: Retrospective chart review was done for all patients who were diagnosed with CPSS in our institution between January 2006 and December 2015. Results: Six patients were diagnosed with CPSS. During this period, 8,680 mothers carrying 9548 fetuses underwent fetal ultrasound examinations. Three patients were diagnosed antenatally at a median [IQ] gestational age of 33 [26–33] weeks, and three patients were diagnosed postnatally at 0, 2, and 43 months, respectively. At a median follow-up of 87 [74–110] months, 5 patients are alive; 4 of whom had received transcatheter closure for different indications, and one who had spontaneous resolution of her CPSS. One infant died at the age of 6 weeks secondary to sepsis. Conclusion: CPSS can result in significant complications in children. Interventional therapy is feasible at any age group, but long-term follow-up is warranted.
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