Osteogenesis Imperfecta and Basilar Invagination

Kyriakos Papadimitriou, Ali A. Baaj, Jean Paul Wolinsky

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Osteogenesis imperfecta (OI) was first reported to be linked to basilar impression in 1942. Secondary basilar impression results from skeletal diseases and resultant bone softening, as is frequently seen in OI, Paget disease, Hurler syndrome, hyperparathyroidism, osteomalacia, Hajdu-Cheney syndrome and achondroplasia. The majority of OI patients with basilar invagination (BI) present with severe physical disability and advanced symptomatic BI. Several radiological modalities are utilized to confirm the diagnosis and aid in pre-operative planning. The general goals of nonsurgical management in OI are to decrease the incidence of fractures and increase mobility and independence. If symptomatic BI is present, however, only surgical decompression and stabilization can halt the progression of deficits and potentially reverse them. In this chapter, we outline a review of the diagnosis, management and outcome for patients with OI and BI.

Original languageEnglish (US)
Title of host publicationOsteogenesis Imperfecta: A Translational Approach to Brittle Bone Disease
PublisherElsevier Inc.
Pages343-348
Number of pages6
ISBN (Print)9780123971654
DOIs
StatePublished - Sep 2013

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Keywords

  • Basilar impression
  • Basilar invagination
  • Cranial settling
  • Osteogenesis imperfecta

ASJC Scopus subject areas

  • Medicine(all)
  • Dentistry(all)

Cite this

Papadimitriou, K., Baaj, A. A., & Wolinsky, J. P. (2013). Osteogenesis Imperfecta and Basilar Invagination. In Osteogenesis Imperfecta: A Translational Approach to Brittle Bone Disease (pp. 343-348). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-397165-4.00036-8