The Journal of Bone and Joint Surgery, Vol 74, Issue 4 598-616, Copyright © 1992 by Journal of Bone and Joint Surgery, Inc
Osteogenesis imperfecta. Radiographic classification, natural history, and treatment of spinal deformities
DA Hanscom, RB Winter, L Lutter, JE Lonstein, BA Bloom and DS Bradford
Gillette Children's Hospital, St. Paul, Minnesota.
The natural history of the radiographic changes associated with
osteogenesis imperfecta was recorded for sixty-four patients. Detailed
clinical data regarding spinal deformities were available for forty-three
patients. The patients could be placed in six well defined groups on the
basis of a cluster of radiographic changes. The radiographic criteria used
to classify patients who had osteogenesis imperfecta included the shape,
dimensions, and appearance of the long bones; the presence of a trefoil
pelvis and protrusio acetabuli; and the shape of the vertebrae. Patients
can be classified more accurately if the dynamic nature of the radiographic
changes is appreciated. Patients who had Type-A disease, a mild form of
osteogenesis imperfecta, maintained the contours of the vertebrae. Most had
straight long bones except for five patients who had mild bowing. Patients
who had Type-B disease had bowed long bones with wide cortices. The pelvis
had a normal contour. The vertebral bodies were biconcave, and kyphosis and
scoliosis developed. Patients who had Type-C disease had thin, bowed long
bones, and protrusio acetabuli developed around the age of ten years.
Patients who had Type-D disease had the same findings as those who had
Type-C disease, with the additional finding of cystic changes around the
knee by the age of five years. The physes closed early, and the cysts
disappeared around the age of fifteen. Patients who had Type-C or D disease
had development of severe spinal deformities. Patients who had Type-E
disease were totally dependent functionally, and spinal deformities
developed at a very early age. The long bones appeared to have no cortex.
Patients who had Type-F disease had complete disruption of the ribs, which
was incompatible with survival. The natural history of scoliosis in the
patients who had Type-B, C, D, or E disease was one of progression of the
curve. Bracing used in the treatment of scoliosis in one patient who had
Type-E disease and five patients who had Type-C disease was unsuccessful.
Arthrodesis of the spine prevented progression of the spinal deformities in
patients who had Type-A disease, but the results of the operation were
variable in the remaining types of the disease.
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