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Revista Española de Cirugía Oral y Maxilofacial

On-line version ISSN 2173-9161Print version ISSN 1130-0558

Abstract

ABURAD DE CARVALHOSA, Artur et al. Osteomyelitis of the maxilla in a patient with Malignant Infantile Osteopetrosis. Rev Esp Cirug Oral y Maxilofac [online]. 2016, vol.38, n.2, pp.96-100. ISSN 2173-9161.  http://dx.doi.org/10.1016/j.maxilo.2014.06.009.

Osteopetrosis is characterized by a considerable increase in bone density resulting in defective remodeling, caused by failure in the normal function of osteoclasts, and varies in severity. It is usually subdivided into three types: benign autosomal dominant osteopetrosis; intermediate autosomal recessive osteopetrosis; and malignant autosomal recessive infantile osteopetrosis, considered the most serious type. The authors describe a case of chronic osteomyelitis in the maxilla of a 6-year-old patient with Malignant Infantile Osteopetrosis. The treatment plan included pre-maxilla sequestrectomy and extraction of erupted upper teeth. No surgical procedure was shown to be the best to prevent the progression of oral infection. Taking into account the patient's general condition, if the patient develops severe symptomatic and refractory osteomyelitis surgery should be considered. The patient and his family are aware of the risks and benefits of surgery and its possible complications.

Keywords : Osteopetrosis; Osteoclerosis fragilis; Marble bone disease; Splenomegaly; Osteomyelitis.

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