Sarma Moinak Sen, Ravindranath Aathira
Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
J Clin Transl Hepatol. 2020 Mar 28;8(1):61-68. doi: 10.14218/JCTH.2019.00041. Epub 2020 Jan 29.
Portal cavernoma cholangiopathy (PCC) is one of the most harrowing complications of extrahepatic portal venous obstruction, as it determines the long-term hepatobiliary outcome. Although symptomatic PCC is rare in children, asymptomatic PCC is as common as that in adults. However, there are major gaps in the literature with regard to the best imaging strategy and management modality in children. Moreover, natural history of PCC and effect of portosystemic shunt surgeries in children are unclear. Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic therapy or bilioenteric anastomosis, both of which are challenging in the presence of extensive collaterals. There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults. In this review, we aimed to collate all existing literature on PCC in childhood and also compare with adult studies. We highlight the difficulties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.
门静脉海绵样变性胆管病（PCC）是肝外门静脉阻塞最严重的并发症之一，因为它决定了长期的肝胆预后。虽然有症状的PCC在儿童中很少见，但无症状的PCC在儿童中与成人一样常见。然而，关于儿童最佳影像学策略和治疗方式的文献存在重大空白。此外，儿童PCC的自然病程以及门体分流手术的效果尚不清楚。被忽视的PCC会导致难治性或顽固性胆管狭窄，需要内镜治疗或胆肠吻合术，而在存在广泛侧支循环的情况下，这两种治疗都具有挑战性。与成人相比，关于门体分流手术对儿童PCC预后影响的研究有限。在本综述中，我们旨在整理所有关于儿童PCC的现有文献，并与成人研究进行比较。我们强调这种疾病的难点，以提供一个全面的平台，专门促进对儿童PCC的进一步研究。
