Chapter 94: Fibrodysplasia (Myositis) Ossificans Progressiva

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Fibrodysplasia ossificans Progressiva (FOP)
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Chapter 94: Fibrodysplasia (Myositis) Ossificans Progressiva Frederick S. Kaplan and Eileen M. Shore

From the Primer on the Metabolic Bone Diseases and Disorders of Mineral Metabolism, 7th Edition. www.asbmrprimer.org Figure 1 Figure 1 Fibrodysplasia (myositis) ossificans progressiva. Characteristic features of FOP are seen in early childhood. The presence of short malformed great toes at birth (A, arrows) heralds the later spontaneous appearance of the preosseous soft tissue lesions on the neck and back (B, arrowheads) and should provoke suspicion of FOP even before the transformation to heterotopic bone (arrows). An inspection of the toes (C) and/or genetic DNA sequence analysis of ACVR1 will confirm the diagnosis and may alleviate the need for a lesional biopsy (trauma) that could exacerbate the condition. (Reprinted with permission from the American Society for Bone and Mineral Research from Kaplan FS, Smith RM 1997 Clinical vignette—Fibrodysplasia ossificans progressiva (FOP). J Bone Miner Res 12:855.) Figure 1 Fibrodysplasia (myositis) ossificans progressiva. Characteristic features of FOP are seen in early childhood. The presence of short malformed great toes at birth (A, arrows) heralds the later spontaneous appearance of the preosseous soft tissue lesions on the neck and back (B, arrowheads) and should provoke suspicion of FOP even before the transformation to heterotopic bone (arrows). An inspection of the toes (C) and/or genetic DNA sequence analysis of ACVR1 will confirm the diagnosis and may alleviate the need for a lesional biopsy (trauma) that could exacerbate the condition. (Reprinted with permission from the American Society for Bone and Mineral Research from Kaplan FS, Smith RM 1997 Clinical vignette—Fibrodysplasia ossificans progressiva (FOP). J Bone Miner Res 12:855.) © 2008 American Society for Bone and Mineral Research

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