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OSTEOGENESIS IMPERFECTA IN CHILDHOOD: MR IMAGING OF BASILAR IMPRESSION -A FOLLOW UP STUDY



Abstract

Objective. To determine on radiographs the presence of Basilar Impression (BI) in children with Osteogenesis Imperfecta (OI). To confirm this sign and altered geometrical relationships of the craniocervical junction in course of time with magnetic resonance imaging (MRI).

Methods and patients: In a cohort study of 130 patients with OI (OI type I:85; OI type III: 21; OI type IV: 24) lateral radiographs of the skull and cervical spine were made in a standardized way. MRI scans were performed when BI was suspected based upon protrusion of the odontoid above Chamberlain’s line. Intracranial abnormalities as well as the basal angle were described. Neurological examination was performed in patients with conclusive BI at MRI scan.

Results and Discussion: In eight patients BI could be confirmed by MRI scan. None of the children had or developed in time neurological symptoms or signs. In follow up no alteration of intracranial findings were seen, although in one child Chamberlain’s line increased from 8 mm (first MRI) to 15 mm (last MRI).

Conclusion: In our cohort study no alteration of the intracranial contents was seen at subsequent MRI scans. Although anatomic deformations exist in BI, no neurological symptoms or signs were present in our study and no operative reconstruction had to be performed. Periodical MRI – scan has not been of influence on the clinical decision making process. At the moment we perform a MRI-scan if BI is suspected on lateral skull radiographs

Local Host: British Society for Children’s Orthopaedic Surgery. Conference Theme: Congenital Deficiencies of the Lower Limb. These abstracts were prepared by A.Catterall.