Year : 2003 | Volume
: 13 | Issue : 4 | Page : 389--391
Images : Fibrodysplasia ossificans progressiva
SV Phatak, PK Kolwadkar, MS Phatak
IGMC and Mayo Hospital, Nagpur, India
S V Phatak
Amarjyoti X-ray and Sonographic Clinic, Wardha Road, Dhantoli Nagpur-440012, MS
|How to cite this article:|
Phatak S V, Kolwadkar P K, Phatak M S. Images : Fibrodysplasia ossificans progressiva.Indian J Radiol Imaging 2003;13:389-391
|How to cite this URL:|
Phatak S V, Kolwadkar P K, Phatak M S. Images : Fibrodysplasia ossificans progressiva. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Jul 4 ];13:389-391
Available from: http://www.ijri.org/text.asp?2003/13/4/389/28714
Fibrodysplasia Ossificans progressiva (FOP) is a disorder of the mesodermal tissue characterized by initial inflammation and subsequently proliferation of fibrous tissue and formation of ectopic bone tissue. Its incidence is one case per two million persons. The prebone lesion of FOP is indistinguishable histologically from the proliferative lesion of aggressive fibromatosis. The ectopic bone tissue formed is located in soft parts mainly in the connective tissue of striated musculature. The lesions start in the paravertebral musculature and progresses into scapula, proximal portions of arms, jaw, pelvic area and skull. The ossification process can intensify in presence of injury . It is an autosomal dominant genetic disease with complete penetrance and variable expression. Over expression of bone morphogenetic protein 4 and its messenger ribonucleic acid (RNA) occurs; this protein has been mapped to chromosome band 14q22-q23.
A boy of 6 yrs (casel) and a girl of 10 yrs (case 2) presented with multiple tender hard swelling in various parts of body associated with stiffness and limitations of movements. Routine blood investigations were normal. Skeletal survey was done in both cases.
The roentgenographic features fall into two categories digital anomalies and ectopic ossification. Digital anomalies may be recognized at birth before ectopic ossification becomes manifest. The most common abnormalities are microdactyly of big toe (90%) and thumbs (50%) , there may be phalange shortening. The microdactyly is due to phalangeal anomalies. The phalanges can be absent. Usually only one large phalanx is present in the big toe or there may be a synostosis between the two phalanges with shortening. Microdactyly of this type is probably unique for this condition. Exostosis can develop, usually replacing the sesamoid bones. Hallux valgus is almost always present. Thumb anomalies are also due to phalangeal shortening. Although the metacarpals may also be short. Synostosis of metacarpals and proximal phalanx may occur but synostosis of phalanges of thumb is not common. All fingers may be shortened. And radial curvature of fifth finger is also reported. Other skeletal anomalies are quite rare but broad femoral necks and large epiphyseal ossification centers. Ectopic ossification in most cases does not appear early in the disease but it has been known to develop during fetal life.In some case there is shortening of middle phalanx of little finger as well. Initially round or linear ossification is seen in soft tissue usually in the neck or shoulder, proximal extremities, the hips and dorsal aspect of trunk are often involved. Eventually columns and plates of bones replace the tendons, fascia and ligaments in these areas. Ossification commonly occurs in planter and palmar fascia and at other area of fibrous attachments i.e. occipital region of skull or anterior portion of calcaneum. Cervical spine shows small vertebral bodies, large pedicles, and progressive fusion of bodies and calcification of nuchal ligaments. The abnormal appearance of the vertebral bodies can be explained on a basis of disturbance of growth due to lack of normal movement stimulation. As the soft tissue fixation starts in the neck its effects are usually maximal in this region. The neck is already fixed so that the development of vertebral body is affected causing a disparity in size between the vertebral bodies and their posterior appendages.
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