关键词: bleeding diathesis extradural hematoma osteogenesis imperfecta

来  源:   DOI:10.1055/s-0038-1660826   PDF(Pubmed)

Abstract:
Osteogenesis imperfecta, also named as brittle bone disease, is characterized by fragile bones and short stature caused by mutations in the collagen gene. Subdural and intraparenchymal hematomas are defined and associated with trauma, vascular causes, and systemic bleeding diathesis. Skull fragility may lead to epidural hematoma, which is a life-threatening situation. Vascular fragility and intrinsic platelet defects are the causes of bleeding in patients with osteogenesis imperfecta, which is a major management challenge for neurosurgeons. Here, we reported on a 5-year-old boy with osteogenesis imperfecta with epidural hematoma and skull fracture following a trivial trauma, and made a literature review of 28 cases with extra-/intradural hematoma.
摘要:
成骨不全症,也被称为脆性骨病,的特点是脆弱的骨骼和身材矮小的胶原蛋白基因突变引起的。定义了硬膜下和实质内血肿,并与创伤有关。血管原因,和全身出血素质.颅骨脆性可能导致硬膜外血肿,这是危及生命的情况。血管脆性和内在血小板缺损是成骨不全症患者出血的原因,这对神经外科医生来说是一个重大的管理挑战。这里,我们报道了一个5岁的男孩成骨不全伴硬膜外血肿和颅骨骨折后的轻微创伤,并对28例硬膜外/硬膜内血肿进行文献复习。
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