Table of Contents

HK J Paediatr (New Series)
Vol 24. No. 3, 2019

HK J Paediatr (New Series) 2019;24:155-157

Case Report

Spontaneous Acute Epidural Haematoma in a Paediatric Patient with Congenital Afibrinogenaemia
先天性無纖維蛋白原血症病童出現自發急性硬膜外出血

U Ocak, P Eser Ocak, B Gungorer


Abstract

Congenital afibrinogenaemia, a rare disorder of coagulation, is characterised by the congenital absence of fibrinogen. Uncontrolled umbilical cord stump bleeding is usually the first manifestation of the disease. Although various spontaneous haemorrhages may occur during lifetime, spontaneous epidural haematoma is rare. We report a case of congenital afibrinogenaemia in a paediatric patient who admitted to the emergency department with a complaint of headache. Computerised tomography scan of the head revealed a right parietal acute epidural haematoma. The patient underwent surgical evacuation subsequent to fibrinogen replacement in the emergency room. We decided to present our case since congenital afibrinogenaemia is rare in the emergency care. This report underlines congenital afibrinogenaemia as a potential cause of spontaneous acute epidural haematoma in paediatric patients which may require emergent intervention before the development of irreversible neurological conditions.

先天性無纖維蛋白原血症,一種罕見的凝血障礙,以先天性纖維蛋白原缺乏為特徵。臍帶殘端出血不止通常是該病的第一個表現。雖然一生中可能發生各種自發性出血,但自發性硬膜外出血卻很少見。我們報告一例先天性無纖維蛋白原血症的病童因頭痛到急診科就診。頭部CT掃描顯示右側頂葉急性硬膜外出血。病人在急診室輸注纖維蛋白原後進行了外科血腫抽吸術。我們決定提出我們的病例是因為先天性無纖維蛋白原血症需要急診處理是很罕見的。本報告強調先天性無纖維蛋白原血症是兒童自發性急性硬膜外出血的潛在原因,這可能需要在發展成不可逆的神經損傷之前行緊急干預。

Keyword : Congenital afibrinogenaemia; Paediatric emergency; Spontaneous epidural haematoma

關鍵詞:先天性無纖維蛋白原血症、兒科急診、自發性硬膜外出血

 
 

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