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表现为十二指肠梗阻和卡萨巴赫-梅里特现象的胰腺卡波西样血管内皮瘤:一名经惠普尔手术治愈的新生儿

Pancreatic kaposiform hemangioendothelioma presenting with duodenal obstruction and kasabach-merritt phenomenon: a neonate cured by whipple operation.

作者信息

Leung Michael, Chao Nicholas Sih Yin, Tang Paula Man Yee, Liu Kelvin, Chung Kenneth Lap Yan

机构信息

Division of Pediatric Surgery, Department of Surgery, Queen Elizabeth Hospital, Kowloon, Hong Kong, Hong Kong.

出版信息

European J Pediatr Surg Rep. 2014 Jun;2(1):7-9. doi: 10.1055/s-0033-1361835. Epub 2014 Feb 10.

Abstract

Aim Kaposiform hemangiondothelioma (KHE) is a rare vascular tumor, commonly associated with Kasaback-Merritt phenomenon characterized by thrombocytopenia and consumptive coagulopathy. We report a case of pancreatic KHE presenting with neonatal duodenal obstruction and Kasaback-Merritt phenomenon. Case Report A full term male baby presented with bile stained vomiting on Day 3 of life. Contrast study and computed tomography scan showed duodenal obstruction by a 5 cm extrinsic hypervascular mass. Platelet count was 23 x 109/L. Laparotomy confirmed a vascular tumor arising from the pancreatic head compressing on the duodenum. Whipple operation was performed. Results Intestinal obstruction and thrombocytopenia resolved after surgery. There was no post-operative complications. Histology confirmed KHE. The boy was tolerating hydrolyzed milk formula and was thriving at 5 months follow up. Conclusion We reported a case of pancreatic KHE presented with neonatal intestinal obstruction and Kasaback-Merritt phenomenon. High index of suspicion is necessary for diagnosis. To our knowledge, this is the youngest patient who underwent Whipple operation.

摘要

目的

卡波西型血管内皮瘤(KHE)是一种罕见的血管肿瘤,通常与以血小板减少和消耗性凝血病为特征的卡萨巴赫-梅里特现象相关。我们报告一例胰腺KHE,表现为新生儿十二指肠梗阻和卡萨巴赫-梅里特现象。病例报告:一名足月男婴在出生第3天出现胆汁样呕吐。造影检查和计算机断层扫描显示十二指肠被一个5厘米的外在高血运肿块梗阻。血小板计数为23×10⁹/L。剖腹探查证实为起源于胰头的血管肿瘤压迫十二指肠。进行了惠普尔手术。结果:术后肠梗阻和血小板减少症得到缓解。无术后并发症。组织学证实为KHE。该男孩能耐受水解奶粉,在随访5个月时发育良好。结论:我们报告了一例胰腺KHE表现为新生儿肠梗阻和卡萨巴赫-梅里特现象。诊断需要高度怀疑。据我们所知,这是接受惠普尔手术的最年轻患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/33ac/4336109/5b4b0a1fe845/10-1055-s-0033-1361835-i130090cr-1.jpg

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