Fibrodisplasia ossificante progressiva: relato de caso e achados radiográficos

Júnior, Cyrillo Rodrigues de Araújo; Carvalho, Tarcísio Nunes; Costa, Marlos Augusto Bitencourt; Lobo, Leonardo Valadares; Fonseca, Cristiano Rezio; Teixeira, Kim-Ir-Sen Santos

doi:10.1590/s0100-39842005000100014

Cited by 11 publications

(13 citation statements)

References 15 publications

(11 reference statements)

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“…17,18 In 1918, Rosenstirn conducted an extensive review of the medical literature, describing 115 cases of FOP. 14,17 The disease was first named myositis ossificans progressiva, 19,20 meaning a muscular inflammation that gradually turned into bones. However, this process affects not only muscles, but also soft parts such as articular capsules and ligaments.…”

Section: Epidemiology Of Fopmentioning

confidence: 99%

“…26 Although most reports concern Caucasian patients, the disease affects all ethnic groups, with a predilection for males at a proportion of 4:1. 19,27 Since in most cases there is no family history, some studies have suggested that the disease is due to a sporadic mutation. 19 Some cases of FOP have been reported in the Brazilian medical literature.…”

Section: Epidemiology Of Fopmentioning

confidence: 99%

“…19,27 Since in most cases there is no family history, some studies have suggested that the disease is due to a sporadic mutation. 19 Some cases of FOP have been reported in the Brazilian medical literature. To date, the Brazilian Association for FOP (FOP Brasil, https://pt-br.facebook.com/pages/FOP-BRASIL/252108424811319) has registered 49 cases of the disease.…”

Section: Epidemiology Of Fopmentioning

confidence: 99%

“…Mineralization occurs after 3 to 4 wk, with the final appearance of bone columns that replace soft tissues. 19 As described above, the diagnosis of FOP is made when malformations of the great toes, such as hallux valgus, and pre-osseous tumor-like swellings are present. 29 So far, there is no definitive treatment for FOP and management should be conservative, avoiding any condition that can potentially cause ectopic ossification.…”

Section: Clinical Characteristics Of Fopmentioning

confidence: 99%

“…These focal and evolutionary behaviors explain the normality of the biopsies obtained from patients at an early stage and probably from an unaffected area. 19,38 Normal Bone Formation Bone formation can classically occur by two processes, i.e., endochondral and intramembranous ossification. In the normal endochondral ossification process, bone is formed from a piece of hyaline cartilage, which undergoes changes, degeneration and progressive replacement by bone tissue.…”

Section: Clinical Characteristics Of Fopmentioning

confidence: 99%

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Cellular and morphological aspects of fibrodysplasia ossificans progressiva

Martelli

Santos

2014

Organogenesis

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Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disease that causes bone formation within the muscles, tendons, ligaments and connective tissues. There is no cure for this disorder and only treatment of the symptoms is available. The purpose of this study was to review the literature and describe the clinical, cellular and molecular aspects of FOP. The material used for the study was obtained by reviewing scientific articles published in various literature-indexed databases. In view of its rarity and of the lack of insightful information and the unpredictability of its course, FOP is a challenging disorder for professionals who are confronted by it. However, this rare disease raises a great deal of interest because understanding the mechanism of mature bone formation can encourage research lines related to bone regeneration and the prevention of heterotopic ossification.

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Section: Epidemiology Of Fopmentioning

confidence: 99%

Section: Epidemiology Of Fopmentioning

confidence: 99%

Section: Epidemiology Of Fopmentioning

confidence: 99%

Section: Clinical Characteristics Of Fopmentioning

confidence: 99%

Section: Clinical Characteristics Of Fopmentioning

confidence: 99%

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Cellular and morphological aspects of fibrodysplasia ossificans progressiva

Martelli

Santos

2014

Organogenesis

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Improvement of mouth opening for a patient with fibrodysplasia ossificans progressiva: a case report

Braga¹,

Silva²,

Silva³

et al. 2011

Special Care in Dentistry

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The aim of this article is to describe the care of a patient with fibrodysplasia ossificans progressiva (FOP) and to provide dentists with a guide for how to safely care for patients with FOP. Treatment improved the patient's limited mouth opening. FOP is a rare autosomal dominant disorder characterized by congenital malformation of the fingers and toes by heterotopic ossification progressiva of the connective tissue. This ossification causes a limitation in osteoradicular mobility, mainly affecting the spine, shoulders, hips, and peripheral joints. The disease can manifest from pregnancy until adulthood, with no greater prevalence associated with race or gender. Although rare, the disease can be easily identified by its clinical features, and diagnosis can be confirmed by a radiographic examination. There is no known effective treatment for this disease. All therapeutic treatment must be conservative to avoid any condition that may cause heterotopic ossification. Guidelines to prevent new ossifications are important for patients with FOP. Dental professionals should be cautious in planning treatment, avoiding anesthesia, especially in the mandible, to prevent ankylosis of the temporo-mandibular joints. The prevention of dental caries is essential to avoid the need for more invasive treatment.

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Fibrodysplasia Ossificans Progressiva: Literature Review and Case Report

Кораблева

Berestnev

Kiselyov³

2023

Vopr. sovr. pediatr.

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Background. Fibrodysplasia ossificans progressiva (FOP) is a genetic disease of the heterotopic ossification group associated with the mutation in ACVR1/ALK2 gene. FOP is characterized by progressive heterotopic endochondral ossification of connective tissue that occurs in postnatal period. It leads to formation of qualitatively normal bone in extraskeletal areas. Congenital hallux deformity is typical for this disease. The clinical picture is characterized by aggravations that are usually caused by trauma or viral infections. Formation of Heterotopic ossificate formation can be observed during aggravations. There is no etiological treatment for FOP. Systemic glucocorticosteroids, non-steroidal anti-inflammatory drug (NSAIDs), mast cell stabilisers, antileukotriene drugs and bisphosphonates can be used in these patients. Clinical case description. The child was born with congenital hallux deformity typical for FOP. The disease onset was noted at the age of 2 years 8 months with a tumor-like painful mass on the neck. Oncological (lymphoproliferative) disease was suspected but biopsy from the lesion did not confirm its malignant nature. The child was consulted by pediatric rheumatologist who has diagnosed FOP. Etanercept and zoledronic acid were administrated, though etanercept was later discontinued. For now, the child receives zoledronic acid infusions 2 times per year and daily NSAIDs. Conclusion. The difficulties in FOP diagnosing are associated to its sporadic nature and clinical picture similarity to other diseases. Suspected malignancy leads to biopsy that is highly undesirable in FOP patients due to high risk of iatrogenic complications.

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Fibrodisplasia ossificante progressiva: relato de caso e achados radiográficos

Cited by 11 publications

References 15 publications

Cellular and morphological aspects of fibrodysplasia ossificans progressiva

Cellular and morphological aspects of fibrodysplasia ossificans progressiva

Improvement of mouth opening for a patient with fibrodysplasia ossificans progressiva: a case report

Fibrodysplasia Ossificans Progressiva: Literature Review and Case Report

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