Giant osteoma of the ethmoid sinus with orbital extension: craniofacial approach and orbital reconstruction.

Osteomas are the most common fibro-osseous lesions in the paranasal sinus. They are benign tumours characterized by slow growth and are often asymptomatic. Treatment is indicated in sphenoid osteomas that threaten the optic canal or orbital apex and in symptomatic cases. The choice of surgical management depends on the location, size and experience of the surgeon. An open approach allows tumour removal with direct visual control and remains the best option in large tumours, but the continued progression in endoscopic approaches is responsible for new indications in closed techniques. Immediate reconstruction allows aesthetic and functional restoration of neighbouring structures, which should one of the goals in the treatment of this benign entity. We report a case of a giant ethmoid osteoma with orbital invasion treated by a combined open craniofacial approach with reconstruction of the anterior cranial base and orbital walls. The literature is reviewed and aetiopathogenic theories, diagnostic procedures and surgical approaches are discussed.

[Cherubism: diagnosis and treatment in the pediatric age]. / Querubismo: diagnóstico y tratamiento en la edad pediátrica.

Cherubism is a benign bone dysplasia of childhood, exclusively involving maxillary bones and spontaneous resolving after puberty in different grades. Approximately, 280 cases have been reviewed in the literature. It is an autosomal dominant disorder in which the normal bone is replaced by cellular fibrous and immature bone, resulting in painless symmetrical enlargement of the jaws. Diagnosis is based in clinical and radiological findings, confirmed by histology. Treatment is a controversial issue, and it is recommended surgical management as conservative as possible during the rapid growth phases. An aggressive case of cherubism is reported, diagnosed and followed since early childhood until puberty, with progressive involvement of facial bones developing in a disruption of facial contours and occlusion. The patient is treated by several surgical interventions oriented to minimize the aesthetic impact of the disease being as conservative as possible. The highlights of this case are the great proportion of the lesions, the functional and emotional disturbances brought out by these lesions and the difficulty to choose the most appropriate age and form of treatment.

Querubismo: diagnóstico y tratamiento en la edad pediátrica / Cherubism: diagnosis and treatment in the pediatric age

El querubismo es una displasia ósea benigna de la infancia, que afecta exclusivamente a los huesos maxilares y se resuelve en gradovariable de forma espontánea durante la pubertad. Se han descrito aproximadamente 280 casos en la literatura, la mayoría en varones. Es una enfermedad hereditaria autosómica dominante en la que el hueso normal es sustituido por hueso fibroso e inmaduro, dando lugar a una expansión indolora simétrica de los maxilares. El diagnóstico es clínico y radiológico, confirmado por la histología. El manejo es controvertido, recomendándose una actitud quirúrgica lo más conservadora posible durante la fases de crecimiento rápido. Se presenta un caso agresivo de querubismo diagnosticado y seguido desde la infancia precoz hasta la pubertad, con afectación progresiva amplia de los huesos faciales, provocando una disrupción de los contornos de la cara y la oclusión. El paciente es tratado mediante una serie de intervenciones encaminadas aminimizar el impacto estético de la enfermedad de la forma más conservadora posible. El interés del caso se centra en la amplia extensión dela enfermedad, los trastornos funcionales y emocionales que provoca y la dificultad para elegir el momento adecuado y el tipo de intervención (AU)

Cherubism is a benign bone dysplasia of childhood, exclusively involving maxillary bones and spontaneous resolving after puberty indifferent grades. Approximately, 280 cases have been reviewed in the literature. It is an autosomal dominant disorder in which the normal bone is replaced by cellular fibrous and immature bone, resulting in painless symmetrical enlargement of the jaws. Diagnosis is based inclinical and radiological findings, confirmed by histology. Treatment is a controversial issue, and it is recommended surgical management as conservative as possible during the rapid growth phases. An aggressive case of cherubism is reported, diagnosed and followed since early childhood until puberty, with progressive involvement of facial bones developing in a disruption of facial contours and occlusion. The patient is treated by several surgical interventions oriented to minimize the aesthetic impact of the disease being as conservative as possible. The highlights of this case are the great proportion of the lesions, the functional and emotional disturbances brought out by these lesions and the difficulty to choose the most appropriate age and form of treatment (AU)

Queratoquiste odontogénico mandibular. Presentación como trismus de larga evolución / Mandibular odontogenic keratocyst. Presentation as gradually developing trismus

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