PDF(Pubmed)
Abstract：
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.
METHODS: 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 multi-step 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 endovascular-surgical 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.
CONCLUSIONS: The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.
METHODS: 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 multi-step 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 endovascular-surgical 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.
CONCLUSIONS: The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.
摘要：
背景：先天性肝内动静脉瘘（IAPF）是一种罕见的婴儿血管畸形，如果不治疗，会导致严重的门静脉高压（PH），预后不良。目前,放射学栓塞被认为是简单IAPF的一线治疗;然而，它可能不是解决复杂的肝血管病变。当血管内栓塞不足以完全消除IAPF时，需要手术干预，但它与幼儿的严重发病率和死亡率有关。此外，指示没有定义。
方法：我们介绍了第一例21三体综合征的6个月大女孩患有复杂的先天性IAFP，导致严重的PH，血管内-外科混合手术成功治疗。这项新技术包括多步血管内栓塞，包括输入血管的超选择性动脉栓塞和扩张的门静脉段的直接肝穿栓塞,结合选择性手术结扎供应瘘管的动脉分支，太小了，不能栓塞。复杂的IAPF还与严重的胆汁淤积和肝内/肝外胆管树扩张有关，通过临时胆道引流成功治疗。在24个月的随访中，混合血管内外科手术实现了复杂IAPF的完全闭塞和PH的消退。关于先天性IAPF管理的文献的全面回顾，专注于替代治疗策略，也有报道。
结论：对于复杂的IAPF，放射科-外科联合治疗是一种安全有效的治疗选择，并且避免了大的侵入性手术。
方法：我们介绍了第一例21三体综合征的6个月大女孩患有复杂的先天性IAFP，导致严重的PH，血管内-外科混合手术成功治疗。这项新技术包括多步血管内栓塞，包括输入血管的超选择性动脉栓塞和扩张的门静脉段的直接肝穿栓塞,结合选择性手术结扎供应瘘管的动脉分支，太小了，不能栓塞。复杂的IAPF还与严重的胆汁淤积和肝内/肝外胆管树扩张有关，通过临时胆道引流成功治疗。在24个月的随访中，混合血管内外科手术实现了复杂IAPF的完全闭塞和PH的消退。关于先天性IAPF管理的文献的全面回顾，专注于替代治疗策略，也有报道。
结论：对于复杂的IAPF，放射科-外科联合治疗是一种安全有效的治疗选择，并且避免了大的侵入性手术。
