1/8. A severely disabling disorder: fibrodysplasia ossificans progressiva.Fibrodysplasia ossificans progressiva (FOP) is an extremely rare hereditary disorder characterised by progressive heterotopic ossification of the soft tissues. The resulting progressive immobilisation of the limbs, jaw and chest wall generally leads to severe disability. We present an 18-year-old girl with advanced FOP. She had three operative interventions to excise her ectopic bones but all resulted in failure. Treatment strategies for this disorder should include the avoidance of exacerbating factors.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
2/8. ankylosis of the jaw in a patient with fibrodysplasia ossificans progressiva.A case of fibrodysplasia ossificans progressiva (FOP) is presented. This uncommon connective tissue disease tends to produce progressing ectopic osteogenesis. Because there are no reported curative procedures for TMJ ankylosis occurring in this condition, a palliative surgical approach is described. Etiology, diagnosis, and prognosis of the disease is reviewed. Recent research in BMP cytokine-induced bone repair may allow new approaches to treating this debilitating disease in the future.- - - - - - - - - - ranking = 4keywords = jaw (Clic here for more details about this article) |
3/8. anesthesia in a child with fibrodysplasia ossificans progressiva.We describe the anesthesia management of a 12-year-old girl, diagnosed with fibrodysplasia ossificans progressiva (FOP), who presented with a submandibular abscess. FOP is a rare, inherited disorder with heterotopic bone formation and progressive musculoskeletal disability. This disability ultimately confines patients to a wheelchair. Minor trauma following dental treatment may lead to ankylosis of the jaw. Subsequent to this disability, which resulted in poor dental hygiene, our patient developed a dental abscess. This spread along the mandibular margin and under the tongue. She presented with an impending airway compromise in an already difficult situation. The options for airway management in a child with limited mouth opening are discussed.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
4/8. Failure of surgery and isotretinoin to relieve jaw immobilization in fibrodysplasia ossificans progressiva: report of two cases.Two patients with fibrodysplasia ossificans progressiva who presented with jaw immobilization due to formation of bone between the maxilla and mandible were treated with surgical resection of their ectopic bone in conjunction with experimental, adjunctive medical therapy using isotretinoin. Both patients had recurrence of their ectopic ossification within 2 months of surgery. Surgery to remove joint-bridging ossifications in FOP is not recommended.- - - - - - - - - - ranking = 5keywords = jaw (Clic here for more details about this article) |
5/8. Fibrodysplasia (myositis) ossificans progressiva in dominica.Fibrodysplasia (myositis) ossificans progressiva is a rare but severely disabling disease in which ossification forms within muscle and leads to progressive restriction of the movements of the jaw, neck, shoulders and hips. Shortening of the big toes is usually present. It is important to recognise this disease as avoidance of intra-muscular injections, surgery and trauma reduces the risk of further ossification.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
6/8. Fibrodysplasia (myositis) ossificans progressiva: clinicopathological features and natural history.patients with fibrodysplasia (myositis) ossificans progressiva (FOP) (n = 28) were studied for up to 24 years. All had characteristic short big toes potentially recognizable at birth; there were radiographic changes in the toes, thumbs, cervical spine and metaphyses of the long bones, including exostoses. Ossification in the large skeletal muscles began from birth to 16 years (mean age 4.6 years) initially in 25 patients in the neck and upper spinal muscles, and later around the hips, major joints and jaw. The rate and extent of disability was unrelated to the time of onset. There was no evidence that any form of treatment produced consistent benefit. Despite the unique combination of skeletal abnormalities and ectopic ossification, the first diagnosis in patients with FOP was often wrong and usually delayed after ectopic ossification began (mean 2.7 years, range 0-14). Except where presentation was unusual, such as progressive stiffness, this delay was mainly due to failure to recognize the significance of the abnormal toes. The most frequent erroneous histological diagnoses were soft tissue sarcoma or fibromatosis. This series emphasizes the usually incorrect initial diagnosis, the misinterpretation of the histology, the unpredictable prognosis and the failure of current treatment. Despite its extreme rarity, there is a need for wider knowledge of this condition both to avoid clinical errors and to stimulate research.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
7/8. Fibrodysplasia ossificans progressiva: report of a case with guidelines for pediatric dental and anesthetic management.Fibrodysplasia ossificans progressiva (FOP) is a rare heritable disorder of progressive heterotopic ossification leading to joint ankylosis throughout the body. Permanent ankylosis of the jaw may be precipitated by minimal soft tissue trauma, a potentially devastating complication following routine dental care during childhood. Assiduous precautions are necessary in administering dental care to children who have FOP as exemplified in this case report. Routine dental prophylaxis is also necessary in order to minimize the need for invasive procedures.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
8/8. myositis ossificans traumatica of the masseter muscle: review of the literature and report of two additional cases.myositis ossificans traumatica of the masseter muscle is uncommon. The condition is benign and results in reactive heterotopic bone formation, usually producing limitation of opening of the jaws. Radiographic and microscopic examination can confirm the diagnosis. Treatment of myositis ossificans traumatica of the masseter muscle is surgical, with other modalities used when occurring in other muscles of the body.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |