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Revista Médica Sinergia

2022, Number 06

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Revista Médica Sinergia 2022; 7 (06)

Fibrous dysplasia overview

Brenes MMA, Hidalgo SMJ, Sandoval BGA

Language: Spanish
References: 18
Page:
PDF size: 212.69 Kb.


ABSTRACT

Fibrous dysplasia is a benign bone pathology caused by a genetic abnormality of the GNSA1 gene. It is characterized by the replacement of normal bone tissue by fibrous tissue. It presents in two modalities; monostotic affecting a single bone and polyostotic affecting multiple bones with a predilection for long bones and craniofacial bones. The clinical presentation and manifestation is variable and depends on the location and extent of the skeletal lesions; it ranges from incidental solitary lesions with no clinical relevance, to severe disease associated with deformity, fractures, pain and functional impairment. Bone pathology may occur in isolation or in association with endocrine manifestations. McCune-Albright syndrome is characterized by the triad of polyostotic fibrous dysplasia, precocious puberty and café-au-lait spots. Diagnosis is based on clinical, radiological and histopathological features and in most cases, the diagnosis can be clinical by adequate evaluation of skeletal, endocrine and dermatological features. Treatment can be medical or surgical, depending on the severity of the clinical presentation and in severe cases multidisciplinary management is required. Surgical management includes multiple modalities and aims to prevent, manage fractures and minimize deformities.


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