Diffuse Neonatal Hemangiomatosis Complicated with Cardiac Tamponade: A Case Report

彌漫性新生兒多發性血管瘤引發心包囊填塞

郭冠廷(Kuan-Ting Kuo) ； 蔡呈芳(Tsen-Fang Tsai) ； 蕭正祥(Cheng-Hsiang Hsiao)

《慈濟醫學雜誌》 11卷1期 (1999/03) Pp. 103-108

https://doi.org/10.6440/TZUCMJ.199903.0103

摘要

彌慢性新生兒多發性血管瘤是一種罕見的良性血管意疾病，在嬰兒期具有較嚴重的臨床病程。臨床上的特徵，是在皮膚及內臟可以發現血管瘤。最常被侵犯的器官是腸胃道、肝臟、肺臟、及腦部。約有百分之六十的病人，在出生前幾個月內死亡。死因主要是因為高心搏出量性心臟衰竭，腸胃道出血，及消耗性的凝血病變。我們主要是報告一個患有此疾病的男嬰。他的病灶主要發生在皮膚腸胃道、泌尿道、肺臟、腦部、助膜、腦膜及心包膜，並合併有腸胃道出血、硬腦膜外及硬腦膜下出血，與卡沙巴—莫瑞症候群(Kasabach-Merritt Syndrome)。此外，心包膜外病灶的出血，也造成心包填塞。雖然使用了類固醉及干擾素的治療，病人仍不幸地於出生後第二十一個月死亡。這一個病例指出，在患有彌漫新生兒多發性血管瘤的病患，心包膜外的病灶會造成心包填塞，並加重心臟衰竭的症狀。

關鍵字

血管瘤 ； 心包囊填塞 ； 卡沙巴--莫瑞症候群

並列摘要

Diffuse neonatal hemangiomatosis (DNH) is a rare benign vascular disorder with aggressive clinical behavior in infancy. Clinically, it is characterized by multiple hemangiomas of the skin and visceral organs. The organs most commonly affected are the gastrointestinal tract, liver, lungs, and brain. About 60% of patients die in the first few months of life, due to high-output heart failure, gastrointestinal bleeding and consumption coagulopathy. We report a case of DNH in a male infant. The hemangioma involved skin, gastrointestinal tract, urinary tract, lungs, brain, pleura, meninx and epicardium complicated with persistent gastrointestinal hemorrhage, severe epidural and subdural hemorrhage and Kasabach-Merritt Syndrome. In addition, bleeding from the themangiomas of the epicardum casused cardiac tamponade. The patient died at 21 months of age despite treatment with corticosteroid and α-interferon. This case indicated that hemangioma of epicardium may cause cardiac tamponade and aggravate the symptoms of heart failure in patient with DNH.

並列關鍵字

hemagiomatosis ； cardiac tamponade ； Kasabach-Merritt syndrome