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… - JBJS, 1993 - journals.lww.com
RB Cohen, GV Hahn, JA Tabas, J Peeper, CL Levitz, A Sando, N Sando, M Zasloff…
JBJS, 1993journals.lww.com
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 …
Abstract
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.
Lippincott Williams & Wilkins