Experiences of two-step ligation and occlusion of congenital portosystemic shunt in children
Objective To summarize the diagnostic and therapeutic experiences of children with congenital portosystemic shunt(CPS)by two-step ligation and occlusion.Methods From January 2020 to July 2022,the relevant clinical data were retrospectively reviewed for 5 CPS children.There were 4 girls and 1 boy.The types of CPS were intrahepatic(n=2)and extrahepatic(n=3).The symptoms were mental retardation(n=2)and cyanosis with mild post-activity dyspnea(n=2).Through catheterization,angiography was performed with balloon occlusion.During operation,it was detected that portal vein pressure was too high,so surgical ligation was changed,and the technique of two-step ligation or partial ligation followed by occlusion was used for treatment.Results Abnormal shunt vessels were partially ligated during operation(n=3)and portal vein blood flow was monitored by B-ultrasound after operation.Abnormal vessels were ligated completely at the bedside at Day 7/10 post-operation.In 2 cases,partial ligation or reduction of shunt vessel diameter was adopted and abnormal shunt vessel re-blocked through vascular intervention technology.Both mental retardation and cyanosis improved markedly post-operation.Pulmonary artery pressure declined obviously.Fasting and 2-hour postprandial blood ammonia concentrations normalized.During a follow-up period of 2 months to 3 years,growth and development were decent.Conclusion CPS may be properly managed through two-step ligation or partial ligation followed by occluding abnormal shunt vessels.
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