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The Journal of Bone and Joint Surgery, Vol 75, Issue 2 215-219, Copyright © 1993 by Journal of Bone and Joint Surgery, Inc
The natural history of heterotopic ossification in patients who have fibrodysplasia ossificans progressiva. A study of forty-four patients
RB Cohen, GV Hahn, JA Tabas, J Peeper, CL Levitz, A Sando, N Sando, M Zasloff and FS Kaplan
Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania, Philadelphia 19104.
Forty-four patients who had fibrodysplasia ossificans progressiva responded
by mail to a questionnaire regarding the age at the onset of heterotopic
ossification at fifteen commonly involved anatomical sites. The average age
of the patients when they responded to the questionnaire was twenty-seven
years (range, three to sixty-nine years). The average age at the onset of
ossification was five years (range, birth to twenty-five years). The most
common sites of early heterotopic ossification were the neck, spine, and
shoulder girdle. Thirty-five (80 per cent) of the patients had had some
restrictive heterotopic ossification by the age of seven years. By the age
of fifteen years, forty-two (more than 95 per cent) of the patients had
severely restricted mobility of the upper limbs. In these patients,
heterotopic ossification proceeded in a direction that was axial to
appendicular, cranial to caudad, and proximal to distal; this pattern
appeared typical for fibrodysplasia ossificans progressiva.
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