Craniomaxillofacial Fibro-osseous Lesions in Children.

Craniofacial fibro-osseous lesions represent a diverse spectrum of pathologic conditions where fibrous tissue replaces healthy bone, resulting in the formation of irregular, woven bone. They are more commonly diagnosed in young people, with treatment strategies dependent on clinical behavior and skeletal maturity. This article discusses the examples of craniofacial fibro-osseous lesions, based on the latest classifications, along with their diagnostic criteria and management.

Displasia cemento-ósea del maxilar: problemas de diagnóstico diferencial a propósito de un caso / Differential diagnosis of cemento-osseous dysplasia of the maxilla. A case report

La displasia cemento-ósea es una lesión frecuente que afecta a los maxilares y no exige tratamiento intensivo. Su principal interés radica en el riesgo de confusión con entidades de comportamiento más agresivo, como los fibromas osificantes y cementificantes, que exigen tratamiento. No existen unos claros criterios de diagnóstico diferencial histopatológico entre ambas entidades. En este trabajo presentamos el caso de un niño de 13 años al que se realizó una biopsia de una lesión en el maxilar, sin diagnóstico clínico de sospecha. El estudio histológico revela un tejido fibroso poco celular en el seno del cual se observan trabéculas óseas sin ribete de osteoblastos y cúmulos de cemento. Nuestro diagnóstico final fue displasia cemento-ósea. Se muestran los principales rasgos de la lesión y se analizan los criterios que pueden ayudar al difícil diagnóstico diferencial de esta entidad

Cemento-osseous dysplasia is a benign lesion which affects the jaw bones. It is a frequent incidental finding but no aggressive therapy is necessary. However, it may be confused with more aggressive entities, such as ossifying and cementifying fibroma, which do require treatment. There are no clear-cut histopathological criteria to differentiate between these entities. We present a case of a 13-year old boy who underwent a biopsy of a clinically undiagnosed maxillary lesion. Histopathological analysis revealed moderately cellular fibrous tissue with bone trabeculae with no osteoblastic rimming and clusters of intensely basophilic material corresponding to cementum. The final diagnosis was cemento-osseus dysplasia. The main histopathological features important in the distinction of these entities are discussed

Abordagem cirúrgica no tratamento da displasia fibrosa craniofacial- experiência de 14 anos / Surgical approach to the treatment of craniofacial fibrous dysplasia - A 14-year experiencedysplasia - 14 years experience

INTRODUÇÃO: Dentre os tumores benignos do esqueleto craniofacial, a displasia fibrosa caracteriza-se pelo crescimento progressivo e acometimento de jovens, acarretando deformidade funcional e estética. Esse trabalho analisa aspectos clínicos e prognósticos de pacientes com essa patologia, submetidos a tratamento cirúrgico. MÉTODOS: Análise retrospectiva de 19 pacientes com displasia fibrosa craniofacial, acompanhados de janeiro de 1997 a dezembro de 2011, tratados com remodelamento ósseo e ressecção cirúrgica. Revisão da literatura sobre. RESULTADOS: A distribuição etária variou de 8 a 65 anos, com média de 21,75 anos. Dez pacientes (52,7%) eram do sexo feminino. A forma poliostótica predominou, com 15 casos (78,9%). Entre os ossos acometidos destacaram-se esfenóide, etmóide e frontal, nas formas poliostóticas; e maxila e zigoma, nas monostóticas. A principal queixa foi de assimetria da face. Um paciente evoluiu com diminuição de acuidade visual. O tratamento baseou-se na ressecção cirúrgica e reconstrução com enxerto, quando doença localizada, e no desgaste e remodelamento ósseo, em formas poliostóticas. Em apenas um caso (5,2%), com compressão de nervo óptico, foi necessário acesso intracraniano. Reabordagem cirúrgica, por crescimento tumoral, foi necessária em três pacientes. Como complicações, tivemos um caso de lagoftalmo e epicanto no pós-operatório de paciente tratado por cirurgia com acesso infraorbitário. Ausência de outras intercorrências no seguimento a curto e longo prazo. Os resultados de preservação de função e recuperação de contorno facial foram satisfatórios. CONCLUSÃO: Essa experiência, em concordância com a literatura, permite concluir que a cirurgia é eficaz na abordagem de casos selecionados de displasia fibrosa craniofacial.

INTRODUCTION: Fibrous dysplasia is benign tumor of the craniofacial skeleton that primarily affects young patients. It is characterized by the progressive growth of benign fibrous tumors with resulting functional and aesthetic deformities. This study assesses the clinical and prognostic features in patients with fibrous dysplasia who underwent surgical treatment at our institution. METHODS: Retrospective analysis of 19 patients with craniofacial fibrous dysplasia, treated between January 1997 and December 2011 with bone remodeling and surgical resection. We also review the literature regarding fibrous dysplasia. RESULTS: Patients ranged between 8-65 years old, with a mean age of 21.75 years. Ten patients (52.7%) were women. The polyostotic form was predominant and present in15 cases (78.9%). The sphenoid, ethmoid, and frontal bones were most commonly involved in the polyostotic form and the mandibular and zygomatic bones were most commonly involved in the monostotic form. The main complaint was asymmetry of the face. One patient developed decreased visual acuity. Treatment was based on surgical resection and graft reconstruction in the localized form of the disease, and bone abrasion and remodeling in the polyostotic form. Intracranial access was necessary in only one case (5.2%) where the optic nerve was compressed. Repeat surgical treatment due to recurrent tumor growth was necessary in three patients. The only complication occurred in a patient who developed lagophthalmos and epicanthus postoperatively after undergoing surgery using infraorbital access. No other complications occurred during short- and long-term follow-up. Functional preservation and facial contour recovery outcomes were satisfactory. CONCLUSION: Our experience, along with that of other investigators, demonstrates that surgery is effective in treating selected cases of craniofacial fibrous dysplasia.