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Displasia Fibrosa en Craneosinostosis
Lauren Middleton1, Pragaash Shanmuganathan1, Peter Anderson1,2
1Cleft and Craniofacial SA, The Women's and Children's Hospital, Adelaide, SA, Australia.
The Journal of craniofacial surgery
|December 30, 2025
Resumen
Esta serie de casos destaca a dos pacientes pediátricos con craneosinostosis que desarrollaron displasia fibrosa. Los hallazgos sugieren una asociación potencial, previamente no reconocida, entre estas dos raras afecciones.
Área de la Ciencia:
- Cirugía craneofacial pediátrica
- Genética médica
- Biología del desarrollo
Sus antecedentes:
- La craneosinostosis es una afección congénita que implica la fusión prematura de las suturas del cráneo.
- La displasia fibrosa es un trastorno óseo raro en el que el hueso normal es reemplazado por tejido fibroso.
- La coexistencia de estas afecciones se informa con poca frecuencia.
Objetivo del estudio:
- Informar sobre dos casos pediátricos de craneosinostosis con desarrollo posterior de displasia fibrosa.
- Detallar la presentación clínica, las estrategias de manejo y los resultados de los pacientes.
- Explorar una asociación potencial, previamente no reconocida, entre la craneosinostosis y la displasia fibrosa.
Principales métodos:
- Revisión retrospectiva de historias clínicas en un centro craneofacial especializado.
- Análisis de historiales médicos, exámenes clínicos e imágenes diagnósticas.
- Evaluación de datos de seguimiento longitudinal para la morfología craneal y el estado neurológico.
Principales resultados:
- Se identificaron dos pacientes pediátricos (un varón, una mujer) con craneosinostosis y displasia fibrosa.
- El paciente varón tuvo sinostosis sagital tratada con remodelación de la bóveda craneal; la paciente mujer con sinostosis metópica fue manejada de forma no quirúrgica.
- Ambos pacientes mostraron morfología craneal estable y sin compromiso neurológico durante el seguimiento.
Conclusiones:
- Los casos presentados sugieren una posible asociación, previamente no documentada, entre la craneosinostosis y la displasia fibrosa.
- Si bien la asociación puede ser coincidente, se justifica una mayor investigación.
- La comprensión de este posible vínculo podría informar futuros enfoques diagnósticos y de manejo.
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