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J Pediatr Genet 2018; 07(04): 185-190
DOI: 10.1055/s-0038-1660826
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Osteogenesis Imperfecta and Extra-/Intradural Hematomas: A Case Report and Review of the Literature

Fatih Yakar
1   Department of Neurosurgery, Kars Harakani Hospital, Kars, Turkey
2   Department of Neurosurgery, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, Turkey
,
Emrah Celtikci
1   Department of Neurosurgery, Kars Harakani Hospital, Kars, Turkey
3   Department of Neurosurgery, School of Medicine, Gazi University, Ankara, Turkey
,
Onur Ozgural
2   Department of Neurosurgery, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, Turkey
,
Umit Eroglu
2   Department of Neurosurgery, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, Turkey
,
Yusuf Sukru Caglar
2   Department of Neurosurgery, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, Turkey
› Author Affiliations
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Publication History

25 January 2018

11 May 2018

Publication Date:
14 June 2018 (online)

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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.

Keywords

osteogenesis imperfecta - extradural hematoma - bleeding diathesis
 

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