Twenty-seven patients with Type-II congenital kyphosis (failure of
anterior vertebral segmentation) all had progression of the kyphosis which
varied in magnitude. The average rate of progression was 5 degrees per
year. Pain due to compensatory lumbar hyperlordosis (eight patients) and
objectionable deformity (fifteen patients) were the most frequent
complaints. Unlike patients with Type-I kyphosis (failure of vertebral
formation), paraplegia did not occur and associated congenital anomalies
were infrequent (three of the twenty-seven patients). Spontaneous
ossification of the anterior part of seemingly normal intervertebral discs
leading to bar formation and progression of deformity occurred in five
patients. A Milwaukee brace had little effect on this fixed kyphotic
deformity in the six patients in whom the brace was used. Early recognition
and spine fusion are the recommended treatment. Posterior fusion is
sufficient in young children with progressive deformity, whereas combined
two-stage anterior and posterior fusion with osteotomy of the anterior bar
is recommended in children with severe deformity.