Year : 2000 | Volume
: 10 | Issue : 1 | Page : 43--44
Radiological quiz -musculoskeletal
Rajesh Gupta, Smiti Sharma, RK Pal, MK Dwivedi
Dept of Radiodiagnosis, J.L.N. Hospital & Research Centre, Bhilai, India
Room No 1/Dr«SQ»s Hospital, Sector 9, Bhilai
|How to cite this article:|
Gupta R, Sharma S, Pal R K, Dwivedi M K. Radiological quiz -musculoskeletal.Indian J Radiol Imaging 2000;10:43-44
|How to cite this URL:|
Gupta R, Sharma S, Pal R K, Dwivedi M K. Radiological quiz -musculoskeletal. Indian J Radiol Imaging [serial online] 2000 [cited 2019 Nov 21 ];10:43-44
Available from: http://www.ijri.org/text.asp?2000/10/1/43/30635
A nine-year old boy was admitted with multiple, hard, tender swellings in the chest wall and diminished neck movements. Radiographs of the chest, cervical spine and foot were obtained.
FIBRODYSPLASIA OSSIFICANS PROGRESSIVA
The diagnosis of myositis ossificans progressiva is mainly radiological. Specific radiological findings seen in our patient are as follows:
The cervical spine radiograph shows calcification of the ligamentum nuchae and shortening of the antero-posterior diameter of the vertebral bodies [Figure 1]The chest radiograph shows multiple bony projections taking origin from the muscles of the chest wall [Figure 2].The radiograph of the feet shows shortening of the 1st metatarsal bone with deformity of the proximal phalanx [Figure 3].
Fibrodysplasia ossificans progressiva (also called myositis ossificans progressiva) is a disorder that differs clinically and radiographically from myositis ossificans traumatica . It has been suggested that the primary histopathological change involves inflammation in the connective tissues initially and later the skeletal musculature . The etiology of the disease is unknown. Several mechanisms have been postulated of which one suggests excess bone production from the periosteum or periosteal rests. Another suggests an autoimmune reaction or a congenital lack of an inhibitory mechanism to prevent crystal seeding (calcium hydroxyapatite) on collagen fibers which results in spontaneous ossification of the soft tissues .
The clinical features recognized are, conduction deafness due to fusion of the ossicles in the middle ear, diffuse thinning of hair and the appearance of painful masses and nodules in the subcutaneous tissues particularly around the head and neck. There is often stiffness and limitation of movement of the area (e.g. wryneck, stiff shoulder) . Although the predominant site is the head and neck, the dorsal paraspinal muscles, shoulders, elbow and later the pelvic girdle and the lower extremities are involved . Radiographically, the cervical spine shows small vertebral bodies, large pedicles and progressive fusion of vertebral bodies and calcification of the nuchal ligament. The soft tissues show abnormal calcifications in the subcutaneous tissues and muscles. A net of bony masses is noted in the thorax, resulting in restricted chest expansion. In the extremities, the patients may present with adactyly, microdactyly, congenital hallux valgus with symphalangism and fusion of metacarpo-phalangeal joints . Ultimately the entire body is affected with the exception of the diaphragm, hands and viscera. The course of the disease is unaffected by drug therapy.
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