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Archives de pédiatrie
Volume 22, n° 11
pages 1154-1156 (novembre 2015)
Doi : 10.1016/j.arcped.2015.08.005
Received : 4 May 2014 ;  accepted : 11 August 2015
Dysostéosclérose et ostéite chronique de la mandibule
Osteomyelitis of the mandible and dysosteosclerosis

W. Kojmane a, , S. Chaouki a, S. Abourazzak a, M. Hida a, G. Fikri b, M. Boubou b, S. Tizniti b
a Service de pédiatrie, CHU Hassan II, Fès, Maroc 
b Service de radiologie, CHU Hassan II, Fès, Maroc 

Auteur correspondant.

La dysostéosclérose est une affection génétique rare, de pronostic sévère. C’est une ostéochondrodysplasie similaire à l’ostéopétrose mais typiquement caractérisée par une platyspondylie et des métaphyses très élargies. Elle se manifeste habituellement par des atteintes des nerfs crâniens et notamment du nerf optique, des complications hématologiques, des fractures lors de traumatismes minimes, un retard des acquisitions motrices avec une dentition anormale mais aussi par une ostéomyélite du maxillaire inférieur. Nous rapportons un cas de dysostéosclérose compliqué d’une ostéomyélite chronique de la mandibule.

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Dysosteosclerosis is a rare genetic disorder with a poor prognosis. It is an osteochondrodysplasia similar to osteopetrosis but it is typically characterized by platyspondyly and expanded metaphyses. It shows complications such as compression of cranial nerves, especially the optic nerve, hematologic complications, fractures following mild injury, abnormal dentition, neurological and psychological deterioration, in addition to osteomyelitis of the mandible. We report a new case of dysosteosclerosis complicated by osteomyelitis of the lower maxilla.

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