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Up-to date Review and Case Report

Table I

OI Sillences classification, completed by Glorieux [9].

Type Clinical severity Typical features Typically associated mutations*
I Mild non-deforming osteogenesis imperfecta Normal height or mild short stature; blue sclera; no dentinogenesis imperfecta Premature stop codon in COL1A1
II Perinatal lethal Multiple rib and long-born fractures at birth; pronounced deformities; broad long bones; low density of skull bones on radiographs; dark sclera Glycine substitutions in COL1A1 or COL1A2
III Severely deforming Very short; triangular face; severe scoliosis; greyish sclera; dentinogenesis imperfecta Glycine substitutions in COL1A1 or COL1A2
IV Moderately deforming Moderately short; mild to moderate scoliosis; grey or white sclera; dentinogenesis imperfecta. Glycine substitutions in COL1A1 or COL1A2
V Moderately deforming Mild to moderate short stature; dislocation of radial head; mineralized interosseous membrane; hyperplastic callus; white sclera; no dentinogenesis imperfecta Unknown
VI Moderately to severely deforming Moderately short; scoliosis; accumulation of osteoid in bone tissue, fish-scale pattern of bone lamellation; white sclera; no dentinogenesis imperfecta Unknown
VII Moderately deforming Mild short stature; short humeri and femora; coxa vara; white sclera; no dentinogenesis imperfecta Unknown
*

May or may not be detectable in a given patient.

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