Ruggiero Antonio, Maurizi Palma, Triarico Silvia, Capozza Michele Antonio, Mastrangelo Stefano, Attinà Giorgio
Paediatric Oncology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, Italy.
Drugs Context. 2020 Mar 26;9. doi: 10.7573/dic.2019-11-5. eCollection 2020.
Infantile haemangiomas (IHs) are the most common benign tumours of childhood; despite the benign histology, prognosis depends on severity of visceral involvement, with a mortality rate ranging from 50 to 90%. In this paper, we describe two infants with multifocal infantile haemangiomatosis and hepatic involvement. This condition should receive appropriate management as it can be potentially lethal due to the high risk of systemic complications such as cardiac or fulminant hepatic failure and abdominal compartment syndrome. Both cases presented with liver involvement, but only the infant who had an excellent response to propranolol is still alive. A review of current therapeutic approaches is also presented even though there are, at present, no uniform guidelines for treatment, despite the relative frequency of infantile haemangiomatosis and the potential severe complications.
婴儿血管瘤(IHs)是儿童期最常见的良性肿瘤;尽管组织学表现为良性,但其预后取决于内脏受累的严重程度,死亡率在50%至90%之间。在本文中,我们描述了两名患有多灶性婴儿血管瘤病并伴有肝脏受累的婴儿。由于存在心脏或暴发性肝衰竭以及腹腔间隔室综合征等全身并发症的高风险,这种情况可能具有潜在致命性,因此应给予适当的治疗。两例均有肝脏受累,但只有对普萘洛尔反应良好的婴儿存活下来。尽管目前尚无统一的治疗指南,且婴儿血管瘤病相对常见且可能出现严重并发症,但本文也对当前的治疗方法进行了综述。