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.
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。