van der Meijs Bram B, Merks Johannes H M, de Haan Timo R, Tabbers Merit M, van Rijn Rick R
Department of Radiology, Onze-Lieve-Vrouwe Gasthuis, Amsterdam, The Netherlands.
Pediatr Radiol. 2009 Mar;39(3):277-81. doi: 10.1007/s00247-008-1073-9. Epub 2008 Dec 16.
We describe a case of rapidly progressive neonatal diffuse hepatic haemangioendotheliomas. The clinical picture was characterized by respiratory insufficiency due to gross abdominal enlargement, coagulopathy, and the development of cardiovascular insufficiency during the course of disease. Pharmacological, radiotherapeutic and endovascular treatment options all proved ineffective. We describe our own experience and the steps taken to treat this child. Unfortunately, the child died as a consequence of the disease. As cases like this are rare and are associated with severe morbidity and high mortality, more knowledge needs to be gained on infantile hepatic haemangioendotheliomas and their optimal treatment. The use of a web-based data registry could be beneficial.
我们描述了一例迅速进展的新生儿弥漫性肝血管内皮瘤病例。临床表现为因腹部明显肿大导致呼吸功能不全、凝血病以及病程中出现心血管功能不全。药物治疗、放射治疗和血管内治疗方案均被证明无效。我们描述了自己的经验以及为治疗该患儿所采取的措施。不幸的是，患儿因该病死亡。由于此类病例罕见且伴有严重的发病率和高死亡率，需要更多地了解婴儿肝血管内皮瘤及其最佳治疗方法。使用基于网络的数据登记系统可能会有所帮助。
