Osteogenesis imperfecta (OI) is an inherited disease characterised by abnormal collagen
formation leading to increased risk of fracture.
1
Depending on the sub-type, modes of inheritance largely occur via an autosomal dominant
fashion and clinical manifestations vary from very mild disease to lethal forms, which
can involve intrauterine death.
1
,
2
In addition to concerns regarding suitable bone stock, increased risk of intraoperative
fracture, and soft tissue considerations, the operative surgeon should also be aware
of hemorrhagic diathesis found in OI patients. Patients with OI often have an inherent
coagulation defect due to abnormal collagen within endothelial capillary beds.
3
,
4
,
5
,
6
Postoperatively, hypertrophic callus or heterotopic ossification (HO) can also be
expected in OI patients, and can be utilised to provide additional stability and a
potential bony template for future fixation if refracture were to occur.
7
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Article info
Publication history
Accepted:
May 9,
2011
Identification
Copyright
© 2011 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.
