Table 1 Sillence and Glorieux classification of OI
Type I OI (mild) | Fractures; minor deformities |
Almost normal stature | |
Blue sclerae | |
“Dentinogenesis imperfecta” may be present | |
Type II OI (lethal) | Fractures in utero |
Death before birth (respiratory deficiency) | |
Type III OI (severe) | Fractures; kyphoscoliosis; major deformities |
Very small stature | |
Triangular face; variable colour of sclerae | |
“Dentinogenesis imperfecta” is frequent | |
Type IV OI (moderate) | Fractures |
Small stature | |
Variable colour of sclerae | |
“Dentinogenesis imperfecta” may be present | |
Types V, VII and VII have been added to the original classification system (no type I collagen mutation, but abnormal bone on microscopy and similar phenotype) | |
Type V OI | Fractures; hyperplastic callus; interosseous membrane ossification; metaphyseal dense lines |
Normal colour of sclerae | |
No “dentinogenesis imperfecta” | |
Type VI OI | Looser striations mimicking fractures |
No wormian bones | |
Type VII OI | Fractures; coxa vara |
Normal colour of sclerae | |
No “dentinogenesis imperfecta” | |
Rhizomelia | |