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Article

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Published version

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2008-10-01

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Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/48928

Pycnodysostosis. A report of 3 clinical cases

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Abstract

Pycnodysostosis is a rare clinical entity, first described in 1962 by Maroteaux and Lamy. It is a genetic disorder, usually diagnosed at an early age. However, the diagnosis is sometimes late, made as a result of bone fracture, given the severe bone fragility resulting from increased bone density. Oral and maxillofacial manifestations of this disease are very clear. The head is usually large, the nose beaked, the mandibular angle obtuse, and both maxilla and mandible hypoplastic. Dental abnormalities and impaction are observed, as well as alterations in eruption and frequent dental crowding. The differential diagnosis is established with osteopetrosis, cleidocranial dysplasia and idiopathic acro-osteolysis. This article reviews the clinical and radiographic characteristics of pycnodysostosis based on three clinical cases of patients with this disease.

Subject

Malalties dels ossos, Maxil·lars, Malalties hereditàries

Subject (English)

Bone diseases, Jaws, Genetic diseases

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Articles publicats en revistes (Odontoestomatologia)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

Citation

ALVES PEREIRA, Daniela, BERINI AYTÉS, Leonardo and GAY ESCODA, Cosme. Pycnodysostosis. A report of 3 clinical cases. Medicina Oral. Patología Oral y Cirugia Bucal. Vol.  2008, num. 13, pags. 10. ISSN 1698-4447. [consulted: 9 of June of 2026]. Available at: https://hdl.handle.net/2445/48928

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