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La displasia fibrosa es una patología ósea benigna causada por una anomalía genética del gen GNSA1. Se caracteriza por el reemplazo de tejido óseo normal por tejido fibroso. Se presenta en dos modalidades; la monostótica que afecta un solo hueso y poliostótica que afecta a múltiples huesos con predilección por los huesos largos y craneofaciales. La presentación y manifestación clínica es variable y depende de la ubicación y la extensión de las lesiones esqueléticas; va desde lesiones solitarias incidentales sin relevancia clínica, hasta enfermedad grave asociada a deformidad, fracturas, dolor y deterioro funcional. La patología ósea podría ocurrir aislada o asociada a manifestaciones endocrinas. El síndrome de McCune-Albright se caracteriza por la tríada de displasia fibrosa poliostótica, pubertad precoz y manchas café con leche. El diagnostico se basa en las características clínicas, radiológicas e histopatológicas y en la mayoría de los casos, el diagnóstico puede ser clínico mediante una adecuada evaluación de las características esqueléticas, endocrinas y dermatológicas. El tratamiento puede ser médico o quirúrgico. Y según la severidad de la presentación clínica y en casos severos se requiere de manejo multidisciplinario. El manejo quirúrgico incluye múltiples modalidades y tiene como objetivo prevenir, manejar las fracturas y minimizar las deformidades.
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