Osteogenesis imperfecta in childhood: MR imaging of basilar impression.
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
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 standardised 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.


CONCLUSIONS
In eight patients BI could be confirmed by MRI-scan. None of the children had or developed in time neurological symptoms or signs. Follow up of BI by MRI scans was done in seven patients (mean: 5 years; range: 2-6 years). No alteration of intracranial findings were seen at subsequent investigation, although in one child Chamberlain's line increased from 8 (first MRI) to 15 mm (last MRI). BI can be diagnosed by radiographs but in the extreme osteoporotic bone and altered anatomy of the craniocervical junction of children with OI MRI is preferable. As intracranial pathology can be demonstrated by MRI, also a relation can be laid to possible neurological symptoms and signs at clinical examination.


CONCLUSIONS
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 at lateral skull radiographs. The MRI images serve as reference findings to anticipate on possible future symptoms and signs of neurological deficit.
Janus
G J M
GJ
Department of Pediatric Orthopaedics, University Medical Centre, Wilhelmina Childrens Hospital, Utrecht, The Netherlands. janus@knmg.nl
Engelbert
R H H
RH
Beek
E
E
Gooskens
R H J M
RH
Pruijs
J E H
JE
D000293:Adolescent; D001933:Brain Stem / Q000000981:diagnostic imaging / Q000473:pathology; D002552:Cerebral Ventricles / Q000473:pathology; D002648:Child; D002669:Child Welfare; D002675:Child, Preschool; D015331:Cohort Studies; D003937:Diagnosis, Differential; D018450:Disease Progression; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007229:Infant Welfare; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D009426:Netherlands; D010013:Osteogenesis Imperfecta / Q000175:diagnosis*; D010985:Platybasia / Q000175:diagnosis; D011379:Prognosis; D011857:Radiographic Image Interpretation, Computer-Assisted; D015203:Reproducibility of Results; D013223:Statistics as Topic