卡梅现象患儿死亡病例及文献回顾性分析

Deaths among patients with Kasabach-Merritt phenomenon: a case report and literature review

目的分析伴卡梅现象患儿的死亡原因,并探讨降低此类患儿死亡风险的方法。方法对2例患儿的诊疗过程进行回顾分析,了解死亡相关原因。通过检索Pubmed、中国知网和万方医学网中的文献,对文献中卡梅现象患儿死亡病例进行总结。结果 1例伴卡梅现象的患儿因腹胀、便血入院,输注血小板后进行腹腔探查,术中见腹腔内尤其肠管弥漫性出血,止血困难,患儿术后8 h死亡。1例伴卡梅现象的卡波西型血管内皮瘤患儿行长春新碱输注治疗后出现颅内出血,经激素、血小板输注等治疗,颅内出血仍进一步加重,由于患儿颅内巨大硬膜下血肿致特重型颅脑损伤后,长期处于植物生存状态并发多器官功能衰竭死亡。共检索到17篇文献,32例伴卡梅现象患儿的死亡病例中,24例患儿主要的死亡原因为出血,4例患儿死于肺部感染,2例患儿死于胆管梗阻及肝功能衰竭。结论出血是卡梅现象患儿死亡的主要原因,降低卡梅现象患儿的死亡风险需要更多的医师认识卡梅现象,积极地预防重要脏器出血并采取更加有效的治疗方法。

Objective To investigate the cause of death in infants with Kasabach-Merritt phenomenon(KMP) and to explore ways to reduce the risk of death in such infants. Methods The diagnosis and treatment of 2 infants with Kasabach-Merritt were retrospectively analyzed to understand the causes of their deaths. The cases of deaths in infants with Kame syndrome reported in the literature were summarized. Results One infant associated with KMP presented with a history of abdominal distension and hemafecia. Intraperitoneal exploration was performed after platelet transfusion. Severe diffuse hemorrhage was seen in the abdominal cavity, especially in the intestinal canal. It was difficult to stop bleeding. The infant died of continuous bleeding due to decompensated DIC at 8 hours after operation. The other infant with KMP was treated with oral prednisolone and vincristine for 6 weeks. Unfortunately, the infant developed progressive intracranial hemorrhage and died of multi-organ failure. A total of 17 articles were retrieved. Of 32 reported deaths with KMP, 24 cases listed bleeding as the primary cause of death, four infants died of pulmonary infection, and 2 cases died of biliary obstruction and liver failure. Conclusion Hemorrhage was the main cause of death in patients with KMP. To reduce the risk of death in infants with KMP,more doctors should recognize KMP, actively prevent the bleeding of the major organs, and adopt a more effective treatment strategy.