Table Of ContentJ. M. Connor
Soft Tissue
Ossification
Foreword by Victor A. McKusick, MD
With 50 Figures
Springer-Verlag
Berlin Heidelberg GmbH
1983
J.M. Connor, BSc, MB, ChB, MD, MRCP
Consultant in Medical Genetics
Duncan Guthrie Institute of Medical Genetics
Yorkhill, Glasgow G3 8SJ, Scotland
ISBN 978-1-4471-1345-4 ISBN 978-1-4471-1343-0 (eBook)
DOI 10.1007/978-1-4471-1343-0
Library of Congress Cataloging in Publication Data
Connor, J.M. (James Michael), 1951-Soft tissue ossification.
Bibliography: p. lncludes index. 1. Metaplastic ossification. 1. Title. [DNLM: 1.
Ossification, Pathologic. 2. Soft tissue neoplasms. 3. Myositis ossificans. QZ 180 C752s]
RC931.M47C65 1983 611'.018 83-10275
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To Mona and Rachel
Foreword
There is much good clinical medicine and much good biology,
including genetics, in this monograph by Connor. His interest in soft
tissue ossification was an outgrowth of his work on fibrodysplasia
ossificans progressiva (FOP). Although FOP is a rarity, like other
genetic diseases it has many implications for common problems of
medical and surgical practice. The major problem of soft tissue
ossification after total hip replacement (p. 27) is a case in point, as is
also the ossification after severe burns (p. 31) and in paraplegics (p.
75). ,
FOP is a rare mutant state inherited as an autosomal dominant.
Virtually all cases are the consequence of new mutation; the disorder
goes no further in the family line because the affected persons rarely
reproduce. Connor did a model job of ascertaining as completely as
possible cases of FOP in England, and of studying a majority of them
personally. This gave him a chance to estimate incidence and mutation
rate, and to examine the effect of father's age on the occurrence of this
disorder, as well as to provide a valid description of the range in variety
of clinical manifestations and the natural history in an unbiased series.
In connection with all the disorders Connor discusses, not only FOP,
a strong genetical theme is discernible. For example, the author points
out that patients who would get ossification after surgical replacement
of one hip are likely to suffer the same complication on the other side as
well (p. 28). Post-'6urn soft tissue ossification of similar distribution has
been noted in twins, neither of whom was seriously burned (p. 32). A
patient has been reported with ossification in three separate primary
neoplasms (p. 46). Brothers both developed ossification in upper
midline abdominal surgical scars (p. 95). Osteoma cutis, an
exceedingly rare disorder, has been described in mother and son (p.
101). Ossification of the posterior longitudinal ligament of the cervical
spine (p. 91) is strikingly more frequent in Japanese than in other
ethnic groups. Although a commonality in methods of medical and
surgical management and lifestyle, and in other nongenetic 'risk
factors' , may explain in part these observations, they point
nonetheless toward possible innate predisposition.
Here the reader will find fascinating bits of lore. What is the origin of
the expression 'Charley horse'? (See p. 19.) How is traumatic
viii Foreword
ossification in the left deltoid muscle of American sailors related to a
practice adopted from British naval tradition? (See p. 23.) What is the
typical tendon ossification of the super-high divers of Acapulco? (See
p. 27.) And, of course, from an earlier age there are the well-known
'cobbler's bone,' 'rider's bone' ofthe horseman, and 'drill bone' ofthe
infantryman. Did Lot's wife who turned to a pillar of stone and the
ancient Greeks who turned into stone after looking into the face of one
of the Gorgon sisters have FOP? (See p. 54.)
A leading actor in this piece is the inducible osteogenic precursor
cell (lOPC). Its regulation may be awry on a genetic basis in
fibrodysplasia ossificans progressiva, and it probably represents the
common pathogenetic pathway for much soft tissue ossification of an
acquired nature. The biology and pathology of this cell will continue to
be of great interest for some time to come, I suspect.
I can recommend this monograph enthusiastically to orthopedic
surgeons, rheumatologists, radiologists, and sports physicians as well
as to those who specialize in physical medicine and rehabilitation
indeed to all physicians who encounter the problem of soft tissue
calcification and ossification.
Baltimore, Maryland Victor A. McKusick, MD
March 1983
Preface
Bone is normally confined to the skeleton, where it has important
mechanical and metabolic functions. In several diseases bone may also
form in the soft tissues and result in physical disability. These diseases,
although individually uncommon, collectively pose important clinical
problems with regard to differential diagnosis and management.
Although such diseases are usually considered an orthopedic
problem, patients not infrequently present to other specialists, notably
rheumatologists, sports physicians and those concerned with
rehabilitation medicine. In the subsequent differential diagnosis,
collaboration between clinician, radiologist and pathologist is essential
as diagnosis on the basis of clinical, radiological or pathological
findings alone can be misleading. In this group of diseases correct
diagnosis is crucial, since they differ widely in prognosis and therapy
which is appropriate for one may be frankly harmful in another.
Soft tissue ossification receives consideration in all major textbooks
of orthopedics, radiology and pathology. However, these accounts
often fail to encompass current understanding of this group of
diseases. Moreover, there appears to be a need for a monograph which
combines these different standpoints for, as emphasized earlier, a
collaborative approach to diagnosis is desirable.
This monograph was written with this approach in mind and is
directed to the specialists who are involved in the day-to-day care of
patients with ossifying lesions. Thus, its order follows the logical
clinical approach. Chapter 1 deals with the differential diagnosis and
Chapters 2 to 6 can then be consulted for a self-contained account of
each disease which includes both medical and surgical aspects of
management. In addition to outlining established facts, these chapters
also indicate important areas of controversy.
The last three chapters serve both as an outline of the current status
of research into the regulation of osteogenesis and to indicate the
clinical implications of recent advances in this area. In a book of this
size it is not possible to describe in detail all of the diseases in which
amorphous calcification, as opposed to ossification, of the soft tissues
can occur. Further, visceral calcification and ossification is given only
brief mention.
Many people have influenced the preparation of this manuscript and
x Preface
I am particularly grateful to: Prof. V.A. McKusick and Prof. E.A.
Murphy (Departments of Medicine and Medical Genetics, The Johns
Hopkins Hospital); Prof. D .A. Price Evans (Department of Medicine,
University of Liverpool); Prof. Peter Beighton (Department of
Human Genetics, University of Cape Town); Dr. Roger Smith, and
Dr. Jim Triffitt (Nuffield Orthopaedic Centre, Oxford); and the
specialists on both sides of the Atlantic who have allowed me to study
their patients with soft tissue ossification.
I would also like to thank the University of Liverpool and the
National Institute of Health (grant number T32 GM07471-05) for
funding during my research on this group of diseases and during the
preparation of this manuscript.
Baltimore, 1983 J.M. Connor
Contents
1 Differential Diagnosis or Soft Tissue Calcification ................ 1
History ........................................................................ 3
Physical Examination ..................................................... , 6
LaboratoryTests ............................................................ 9
Radiology .................................................................... 9
Biopsy ......................................................................... 14
Summary ..................................................................... 14
2 Ossification After Major Trauma .................................... 17
Ossification After Repeated Minor Trauma .......................... 23
Hematoma Ossification ................................................... 24
Post-traumatic Ossification of Ligaments and Tendons ............ 25
Ossification After Hip Replacement.................................... 27
Ossification After Burns................................................... 31
Ossification After Tetanus ................................................ 33
3 Tumor Ossification ..................................................... 38
Parosteal Osteosarcoma........... ........................................ 38
Soft Tissue Osteosarcoma................................................. . 40
Other Ossifying Connective Tissue Tumors........................... 42
Ossifying Skin Tumors..................................................... 43
Visceral Tumor Ossification .............................................. 44
Pseudomalignant Myositis Ossificans .................................. 46
4 Fibrodysplasia Ossificans Progressiva .............................. 54
Prevalence ................................................................... 55
Clinical Features ............................................................ 55
Radiology .................................................................... 61
Laboratory Studies ......................................................... 65
