AIM:To explore the prevalence of local and genetic thrombophilic disorders as risk factors for portal vein thrombosis (PVT) in our series,the largest ever published in pediatric literature. METHODS:We conducted a case...AIM:To explore the prevalence of local and genetic thrombophilic disorders as risk factors for portal vein thrombosis (PVT) in our series,the largest ever published in pediatric literature. METHODS:We conducted a case-control study enrolling 31 children with PVT and 26 age-matched controls. All were screened for thrombophilia,including genetic disorders,protein C,protein S and homocysteine deficiencies. All coagulation parameters were studied at least 3 mo after the diagnosis of portal vein obstruction.RESULTS:In our study we showed that most pediatric patients with PVT have local prothrombotic risk factors,which are probably the most important factors leading to PVT. However,there is a clear association between the presence of prothrombotic disorders and PVT,suggesting that these increase the risk of thrombosis in patients with local factors such as perinatal umbilical vein catheterization or sepsis. CONCLUSION:Patients with PVT should be screened for inherited prothrombotic disorders regardless of a history of an obvious local risk factor.展开更多
BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisati...BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children.Furthermore, indications are not defined.CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised.The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.展开更多
基金Supported by Grant from Bambino Gesù Children’s Hospital
文摘AIM:To explore the prevalence of local and genetic thrombophilic disorders as risk factors for portal vein thrombosis (PVT) in our series,the largest ever published in pediatric literature. METHODS:We conducted a case-control study enrolling 31 children with PVT and 26 age-matched controls. All were screened for thrombophilia,including genetic disorders,protein C,protein S and homocysteine deficiencies. All coagulation parameters were studied at least 3 mo after the diagnosis of portal vein obstruction.RESULTS:In our study we showed that most pediatric patients with PVT have local prothrombotic risk factors,which are probably the most important factors leading to PVT. However,there is a clear association between the presence of prothrombotic disorders and PVT,suggesting that these increase the risk of thrombosis in patients with local factors such as perinatal umbilical vein catheterization or sepsis. CONCLUSION:Patients with PVT should be screened for inherited prothrombotic disorders regardless of a history of an obvious local risk factor.
文摘BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children.Furthermore, indications are not defined.CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised.The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.