The Journal of Bone and Joint Surgery, Vol 76, Issue 10 1442-1450, Copyright © 1994 by Journal of Bone and Joint Surgery, Inc
Spinal deformity in patients who have fibrodysplasia ossificans progressiva
PB Shah, MA Zasloff, D Drummond and FS Kaplan
Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia.
We reviewed roentgenograms and clinical records in order to characterize
the spinal deformity in forty patients who had an established diagnosis of
fibrodysplasia ossificans progressiva. Twenty-six (65 per cent) of the
patients had scoliosis, which, according to the clinical records and the
recollection of the patients, had been present during childhood.
Twenty-three (88 per cent) of the twenty-six curves were unbalanced
c-shaped curves, while the remaining three (12 per cent) were balanced
s-shaped curves. Twenty-one (91 per cent) of the twenty-three c-shaped
curves involved the thoraco-lumbar or lumbar spine. The c-shaped curves
ranged in magnitude from 15 to more than 80 degrees. Curves became rigid by
early adulthood and many resulted in severe pelvic obliquity with impaired
sitting or standing balance. An osseous bridge developed between the
posterolateral aspect of the iliac crest and the posterolateral aspect of
the rib cage in twenty-two (55 per cent) of the forty patients. Nineteen
(86 per cent) of these twenty-two patients had scoliosis; there was a
significant association between the development of scoliosis and the
presence of the osseous bridge (p < 0.005). Ossification of the
paravertebral muscles and fascia during the first decade of life limited
the development of a normal thoracic kyphosis in ten (42 per cent) of
twenty-four patients for whom lateral roentgenograms of the spine were
available. A spinal orthosis was used to treat the scoliosis in two
patients, but this method resulted in breakdown of the skin and failed to
halt progression of the curve.(ABSTRACT TRUNCATED AT 250 WORDS)
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