• Title/Summary/Keyword: Fibrodysplasia ossificans progressiva

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Head and neck manifestations of fibrodysplasia ossificans progressiva: Clinical and imaging findings in 2 cases

  • Gyu-Dong Jo ;Ju-Hee Kang ;Jo-Eun Kim ;Won-Jin Yi ;Min-Suk Heo ;Sam-Sun Lee ;Kyung-Hoe Huh
    • Imaging Science in Dentistry
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    • v.53 no.3
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    • pp.257-263
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    • 2023
  • Fibrodysplasia ossificans progressiva is a rare hereditary disorder characterized by progressive heterotopic ossification in muscle and connective tissue, with few reported cases affecting the head and neck region. Although plain radiographic findings and computed tomography features have been well documented, limited reports exist on magnetic resonance findings. This report presents 2 cases of fibrodysplasia ossificans progressiva, one with limited mouth opening due to heterotopic ossification of the lateral pterygoid muscle and the other with restricted neck movement due to heterotopic ossification of the platysma muscle. Clinical findings of restricted mouth opening or limited neck movement, along with radiological findings of associated heterotopic ossification, should prompt consideration of fibrodysplasia ossificans progressiva in the differential diagnosis. Dentists should be particularly vigilant with patients diagnosed with fibrodysplasia ossificans progressiva to avoid exposure to diagnostic biopsy and invasive dental procedures.

Myositis Ossificans Progressiva localized on hands and feet with slow progression - One case report - (수족부에 국한되어 완만한 임상경과를 보이는 진행성 골화성 근염 - 1예 보고 -)

  • Kim, Jae-Hwa;Cho, Duck-Yun;Yoon, Hyung-Ku;Han, Soo-Hong;Kim, Dong-Jun
    • Journal of Korean Foot and Ankle Society
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    • v.6 no.2
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    • pp.247-250
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    • 2002
  • Myositis ossificans progressiva, otherwise called fibrodysplasia ossificans progressiva is a rare hereditary disease of unknown etiology, which is characterized by progressive ectopic ossification in connective tissues of the muscles, tendons, fascia and aponeurosis. We report a case of myositis ossificans progressiva confined to hands and feet, which has an unusual clinical feature with slow progression.

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Fibrodysplasia Ossificans Progressiva - A Case Report - (진행성 골화성 섬유이형성증 - 성인 1례보고 -)

  • Yun, Yeong- Sik
    • The Journal of the Korean bone and joint tumor society
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    • v.10 no.1
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    • pp.50-55
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    • 2004
  • Fibrodysplasia ossificans progressiva is a very rare genetic disorder, but is possible to diagnose with mass on neck or scalp in early neonate or child and accompanying characteristic congenital malformation of great toe. But because inappropriate treatment and complications from misdiagnosis may aggravate the progress of the disease, so the disorder require careful inspection for accurate diagnosis. We describe a case that was misdiagnosed properly and treated inappropriately and the natural history of the disease in adult.

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A CASE REPORT AND DENTAL TREATMENT CONSIDERATIONS OF FIBRODYSPLASIA OSSIFICANS PROGRESSIVA PATIENT (Fibrodysplasia ossificans progressiva 환자의 증례보고 및 치과 치료시 고려 사항)

  • Kweon, Young-Sun;Hyun, Hong-Keun;Kim, Young-Jae;Jang, Ki-Taeg;Lee, Sang-Hoon;Kim, Chong-Chul;Hahn, Se-Hyun;Kim, Jung-Wook
    • Journal of the korean academy of Pediatric Dentistry
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    • v.38 no.1
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    • pp.95-100
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    • 2011
  • Fibrodysplasia ossificans progressiva(FOP) is characterized by episodes of permanent heterotopic ossifications of soft tissues throughout the body. FOP is inherited in an autosomal dominant pattern, but most cases result from new mutations in the ACVR1 gene. Even minimal trauma can cause permanent ossifications of soft tissues and give rise to complications following routine dental care. Dental block anesthesia, severe stretching of the jaw and biopsies are all contraindicated in children with FOP. There is no effective treatment. Since the prevalence of FOP is very low and most patients with FOP are misdiagnosed during childhood, they undergo dangerous and unnecessary treatment that can lead to permanent harm. For patients with FOP, early diagnosis and prevention of complications are most important. This study aims at contemplating the characteristic features and consideration factors for the dental treatment of FOP patients in relation to the case of an eight years-and-one month old boy who was referred to Pediatric Dentistry due to prolonged retention of mandibular right and left deciduous central incisors after being diagnosed with sporadic FOP at Pediatric Orthopaedics, Seoul National University Hospital, and received dental treatment without the exacerbation of the FOP symptoms.

Myositis Ossificans Progressiva - Two Cases Report - (진행성 골화성 근염 - 2예 보고 -)

  • Park, Byeong-Mun;Kim, Dong-Soo;Ko, Young-Kwan;Song, Kyung-Sub;Jeon, Kwang-Pyo;Yoon, Hyung-Ku;Moon, Chan-Sam
    • The Journal of the Korean bone and joint tumor society
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    • v.8 no.2
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    • pp.63-67
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    • 2002
  • Myositis ossificans progressiva, also known as 'fibrodysplasia ossificans progressiva' is a rare disorder, most probably inherited as a mendelian dominant trait with irregular penetrance. It is characterized by congenital malformations of the great toes and progressive edema, calcification and ossification of the fasciae, aponeurosis, ligaments, tendons, and connective tissue in interstitial tissues of skeletal muscle. The basic defect is in the connective tissue, whereas the skeletal muscle remains fundamentally normal. We report two cases of a brother and sister whose the disorder is involved in a same family.

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