Displasia fibrosa poliostótica - síndrome de McCune-Albright:

Tomás Rienzi; Claudio Silveri; Mariana Risso; Beatriz Mendoza; Gottardo Bianchi

doi:10.29193/RMU.37.1.12

Tomás Rienzi Centro Hospitalario Pereira Rosssell, Clínica Traumatología y Ortopedia, Médico residente
Claudio Silveri Centro Hospitalario Pereira Rosssell, Clínica Traumatología y Ortopedia, Profesor adjunto
Mariana Risso Universidad de la República, Facultad de Medicina, Clínica de Endocrinología y Metabolismo, Médico residente
Beatriz Mendoza Universidad de la República, Facultad de Medicina, Clínica de Endocrinología y Metabolismo, Profesora
Gottardo Bianchi Centro Hospitalario Pereira Rosssell, Clínica Traumatología y Ortopedia, Médico residente

DOI: https://doi.org/10.29193/RMU.37.1.12

Keywords: POLYOSTOTIC FIBROUS DYSPLASIA, MCCUNE-ALBRIGHT SYNDROME, PERIPHERAL PRECOCIOUS PUBERTY

Abstract

Introduction: polyostotic fibrous dysplasia is a low prevalence disease, and for this reason, it often goes undetected. When associated to endocrinopathies and/or hyperpigmented skin lesions we speak about McCune Albright syndrome.
Clinical case: eight-year old school girl who presented pathological fracture of the left femur, which was diagnosed as polyostotic fibrous dysplasia. As it was accompanied by peripheral precocious puberty it constituted an indicative clinical picture of the so-called McCune Albright. Control and treatment were multidisciplinary. The traumatology team performed osteosynthesis with plaques and nails to fix the bone lesion, and evolution consolidated in a regular time frame (3 months). Upon six months follow-up, the girl has no pain and presents no functional repercussion in daily life. From the endocrinological perspective, the girl received precocious puberty treatment with aromatase inhibitors with the purpose of improving her final size and avoid psychological and emotional implications.
The study presents the characteristics of this condition, as well as its vital and functional prognosis.

References

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Polyostotic fibrous dysplasia - McCune-Albright syndrome

A case report

Abstract

References

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