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Year : 2004  |  Volume : 14  |  Issue : 1  |  Page : 25-27
Images : Fibrodysplasia ossificans progressiva


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Keywords: Fibrodysplasia Ossificans Progressiva

How to cite this article:
Gupta M, Gupta S, Iyer S G, Sharma G L. Images : Fibrodysplasia ossificans progressiva. Indian J Radiol Imaging 2004;14:25-7

How to cite this URL:
Gupta M, Gupta S, Iyer S G, Sharma G L. Images : Fibrodysplasia ossificans progressiva. Indian J Radiol Imaging [serial online] 2004 [cited 2019 Nov 19];14:25-7. Available from: http://www.ijri.org/text.asp?2004/14/1/25/28549
Fibrodysplasia ossificans progressive is an extremely rare genetic disorder of connective tissue characterized by progressive heterotrophic ossification of the soft tissues associated with congenital malformations of the great toe, hands and cervical vertebrae.

Two patients, a seventeen year old girl and a fourteen year old boy presented with complaints of painful swellings since childhood, deformities, stiffness and limitations of joint movements. All these findings were much more severe in the boy, who also had an incapacitating gait disturbance.

Plain radiographs of different parts were obtained, some of which have been illustrated.



Fibrodysplasia ossificans progressiva or Myositis ossificans congenital progressiva was first reported in 1648 by Platin [4]. This extremely rare disorder (incidence 0.1/million) occurs mostly due to spontaneous mutations, but an autosomal dominant pattern of inheritance with variable penetrance has also been found [3]. Over expression of the Bone Morphogenetic Protein 4 has been implicated in its etiology [5].

The disease primarily affects the perimuscular fascia where proliferating fibrous tissue extends into and across muscle bundles in a haphazard fashion, forming bony buttresses which ultimately attach to the bone. Progressive ossification of tendons, ligaments, aponeuroses, muscles and joints lead to severe limitation of movements and gait disturbances. The muscles of the neck, shoulders and trunk are usually the first to be involved.S

Several associated anomalies have been described.

  1. Anomalies of the feet are the most common and are usually bilaterally symmetrical. They usually involve the first metatarsal and the great toe. These include dysplasias and hallux valugus [1],[2],[3]
  2. Anomalies of the hands include dysplasias of the first metacarpal and clinodactyly [2],[3]
  3. Shoulders may show superior subluxation of the glenohumeral joints [2],[3]
  4. The spine may show dysplasias of the cervical vertebrae with enlarged pedicles and fused posterior elements. The dorsolumbar spine may show extensive kyphoscoliosis [2],[3]
  5. Numerous osteochondromas (Exostoses) may also be found [1],[2],[3]


Diagnosis of this disorder rests on the typical radiological findings, combined with the typical history and clinical findings.

 
   References Top

1.Chichareon V, Arporn Maeklong P. Donsakul N. Fibrodysplasia ossificans progressiva and associated osteochondromas of coronoid process in child. Plastic reconstruction surgery 1999 Apr; 103 (4): 1238-43.   Back to cited text no. 1    
2.Smith R, Athanasou N. A, Vipond S.E. Fibrodysplasia ossificans progressiva: clinicopathological and natural history. QJM 1996 Jun; 89 (6); 445-6.   Back to cited text no. 2    
3.Martin H Reed. Soft Tissues. Pediatric skeletal radiology. Williams and Wilkins 1992.   Back to cited text no. 3    
4.Buyse G, Silberstein J, Croemans N, Casaer P. Fibrodysplasia ossificans progressiva: Still turning to wood after 300 years? Eur J Pediatr 1995 Sep; 154(9) 694-9.   Back to cited text no. 4    
5.Lanchoney T.F, Olmstead E. A, Shore E. M. et al . Characterization of bone morphogenetic protein 4 receptor in Fibrodysplasia ossificans progressiva. Clin orthop 1998 Jan; (346) 38- 45.   Back to cited text no. 5    

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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]



 

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