Abstract

SANTOS, Sandra et al. Doença de Camurati-Engelman: A propósito de um caso clínico. Rev. Port. Ortop. Traum. [online]. 2018, vol.26, n.1, pp.75-81. ISSN 1646-2122.

Objective: This clinical case report intends to present the treatment, clinical and radiological outcomes of a traumatic injury (supracondylar femur fracture) in a patient with Camurati-Engelmann disease, bone dysplasia that produces a hyperostotic deformity of the long bone diaphysis. Camurati-Engelmann’s disease is a rare autossomic dominant genetic disease, of unknown prevalence. However, among the various sporadic cases and families described in the literature, 12 patients belong to a Portuguese family, which makes this case report, as well as knowledge of the disease and its pathology, relevant. Likewise, there are no reports on the surgical management of traumatic injuries in this patient population in the current literature, which makes this case particularly interesting. Description: A 46-year old male presented in the Emergency Room with a supracondylar femur fracture, after motorcycle accident. The chosen treatment was surgical, with open reduction and osteossynthesis with an anatomical locked plate. Clinical result at 2 years post-operatively was favorable, with no restrictions on activities of daily living and good range of motion; x-ray studies showed fracture consolidation. Comments: The relevance of the present report is based on the description of the treatment and functional outcome after supracondylar femur fracture in a patient with Camurati-Engelmann disease, a rare presentation which the authors have not found in current literature.

Keywords : Camurati-Engelmann disease; Fracture; Surgical treatment.

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