types
Last reviewed 01/2018
The four types are as follows:
- I mild disease
- blue sclerae
- mild prepubertal bone fragility
- little or no deformity
- mild short stature
- hearing loss in about 50% of adults
- dentinogenesis imperfecta may be absent (IA) or present (IB)
- bruises easily
- II
perinatal lethal
- blue sclerae
- in utero fractures
- micromelia
- marked long bone deformity
- platyspondyly
- lethal in the perinatal period
- broad but poorly mineralized skull
-
III progressive deforming
- blue then white sclerae
- progressing deforming of the long bones and spine secondary to fractures and gravity
- relative macrocephaly and triangular faces
- extremely short stature
-
IV moderately severe disease
- white sclerae
- moderate fragility and mild to moderate bowing of long bones
- variable short stature
- dentinogenesis imperfecta absent (IVA) or present (IVB)
osteogenesis imperfecta type I - mild disease
osteogenesis imperfecta type II - perinatal lethal
osteogenesis imperfecta type III - progressive deforming