Basilar impression and platybasia in osteogenesis imperfecta tarda.

Abstract

Osteogenesis imperfecta, a rare, genetically transmitted disorder of bone, is known to be associated with the development of basilar impression and platybasia. These deformities of the base of the skull may cause neurosurgical abnormalities secondary to compression of the brainstem and hydrocephalus. The case is presented of a young boy with a family history of osteogenesis imperfecta tarda who suffered respiratory arrest during hospitalization. Cranial nerves and pyramidal tract signs were demonstrated. Roentgenograms showed severe basilar impression and hydrocephalus. Decompression of the brainstem and shunting were performed with improvement in the patient's neurological status. This case represents a rare by significant central nervous system complication of osteogenesis imperfecta. Early recognition and implementation of aggressive treatment are important if irreversible neurological deficits are to be avoided.

• Authors: E Frank, T Berger, J M Tew Jr
• Journal: Surgical neurology (Surg Neurol) Vol. 17 Issue 2 Pg. 116-9 (Feb 1982) ISSN: 0090-3019 [Print] United States
• PMID: 7071726 (Publication Type: Case Reports, Journal Article)

Topics

• Adolescent
• Humans
• Male
• Nervous System Malformations
• Osteogenesis Imperfecta (complications, diagnostic imaging)
• Platybasia (complications, diagnostic imaging)
• Radiography