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Vol. 34. Issue 5.
Pages 268-272 (September - October 2023)
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Vol. 34. Issue 5.
Pages 268-272 (September - October 2023)
Caso clínico
Displasia fibrosa craneofacial y quiste óseo aneurismático en una paciente con síndrome de McCune-Albright. Presentación de un caso y revisión de la literatura
Craniofacial fibrous dysplasia and aneurismal bone cyst in a patient with McCune-Albright syndrome. A case report and review of the literature
Julián Castro Castroa,
Corresponding author
juliancastrocastro@yahoo.es

Autor para correspondencia.
, Juan Manuel Villa Fernándezb, Jesús Patricio Agulleiro Díazc
a Servicio de Neurocirugía, Complexo Hospitalario Universitario de Ourense, Instituto de Investigación Sanitaria Galicia Sur, Ourense, España
b Servicio de Neurocirugía, Complexo Hospitalario Universitario de A Coruña , A Coruña, España
c Servicio de Neurocirugía, Complexo Hospitalario Universitario de Santiago , Santiago de Compostela, A Coruña, España
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Resumen

El síndrome de McCune-Albright (SMA) es un trastorno genético heterogéneo que se caracteriza por la tríada de displasia fibrosa (DF) poliostótica, manchas café con leche y múltiples endocrinopatías hiperfuncionales. En general, se diagnostica clínicamente. De la tríada, 2 de los hallazgos son suficientes para hacer el diagnóstico. La DF craneofacial es un término que se usa para describir la displasia fibrosa, que se localizaba en el esqueleto craneofacial y es común en pacientes con SMA.

El quiste óseo aneurismático (QOA) es una lesión ósea no neoplásica infrecuente que afecta principalmente a los huesos largos y las vértebras, y puede ocurrir muy raramente en los huesos craneofaciales. Los QOA pueden ocurrir como enfermedades óseas secundarias en asociación con varios tumores óseos benignos y malignos y con displasia fibrosa. El QOA secundario que ocurre en la DF craneofacial es excepcional. Presentamos el caso de una paciente de 21 años tratada en nuestro centro de un quiste óseo aneurismático orbitario derecho asociado a SMA y realizamos una revisión de la literatura relevante.

Palabras clave:
Craneotomía
Displasia fibrosa
Quiste óseo aneurismático
Síndrome de McCune-Albright
Abstract

McCune-Albright syndrome (MAS) is a rare heterogeneous genetic disorder that is characterized by a triad of polyostotic fibrous dysplasia (FD), café au lait spots (CAL), and multiple hyperfunctional endocrinopathies. In general, it is diagnosed clinically. From the triads, 2of the findings are enough to make the diagnosis.

Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients.

Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia. Secondary ABC occurring in craniofacial FD is extremely rare. We present the case of a 21-year-old patient treated at our center for a right orbital aneurysmal bone cyst associated with MAS and provide a review of the relevant literature.

Keywords:
Aneurismal bone cyst
Fibrous dysplasia
McCune-Albright syndrome
Craniotomy

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