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PubMed1993

Metaphyseal abnormalities in fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is a rare, congenital disorder characterized by diffuse ossification of extraskeletal connective tissue. The classical features and progression of the disease are described and three cases are presented which fall into the general pattern of FOP clinically and radiologically. A constant feature seen was a slight metaphyseal flaring with spiking at the edges of the metaphyses, compatible with minor alteration in bone morphology during growth. These changes cannot be seen after epiphyseal fusion. The major abnormalities persist into adult life.

Bone and Bones↗

PubMedDate not supplied

Heterotopic ossification.

Heterotopic ossification, the formation of bone in soft tissue, requires inductive signaling pathways, inducible osteoprogenitor cells, and a heterotopic environment conducive to osteogenesis. Little is known about the molecular pathogenesis of this condition. Research into two rare heritable and developmental forms, fibrodysplasia ossificans progressiva and progressive osseous heteroplasia, has provided clinical, pathologic, and genetic insights. In fibrodysplasia ossificans progressiva, overexpression of bone morphogenetic protein 4 and underexpression of multiple antagonists of this protein highlight the potential role of a potent morphogenetic gradient. Research on fibrodysplasia ossificans progressiva also has led to the identification of the genetic cause of progressive osseous heteroplasia: inactivating mutations in the alpha subunit of the gene coding for the stimulatory G protein of adenylyl cyclase. Better understanding of the complex developmental and molecular pathology of these disorders may lead to more effective strategies to prevent and treat other, more common forms of heterotopic ossification.

Adolescent↗

PubMed1981

Cardiopulmonary function in fibrodysplasia ossificans progressiva.

Cardiopulmonary function was evaluated in 21 patients with fibrodysplasia ossificans progressiva. Neither cardiac enlargement nor failure was observed, but six patients had abnormal electrocardiograms. All had marked restrictive spirometry because of chest wall fixation and depended upon diaphragmatic respiration. The severity of chest restriction was independent of sex, age, duration of disease, and extnet of other physical disability. Progression to chronic respiratory failure was not observed. Chest infection in the presence of diminished pulmonary reserve is the major hazard of life in this rare disease and prophylactic measures should be considered.

Adolescent↗

PubMedDate not supplied

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.

Anesthesia, Dental↗

PubMed1978

Fibrodysplasia ossificans progressiva. Biochemical changes in blood serum, urine, skin, bone, and ectopic ossification.

Increased urinary output of total hydroxyproline, hydroxylysine and uronic acid was found in two patients suffering from fibrodysplasia ossificans progressiva. Before treatment of one of the patients, the high molecular weight peptide fraction deriving from newly synthesised collagen was particularly increased. Treatment with disodium etidronate (diphosphonate) reduced the values. The urinary values of sodium, potassium and calcium were also depressed during treatment, and, in both patients, serum phosphate was high, while serum calcium was normal. The hydroxyproline and hydroxylysine contents in skin and bone of FOP patients did not differ from controls, while ectopic ossifications showed a considerable increase in hydroxylysine in relation to normal bone.

Adolescent↗

PubMed2006

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.

Ambulatory Surgical Procedures↗

PubMed1990

Fibrodysplasia ossificans progressiva. An 11-year-old boy treated with a diphosphonate.

Fibrodysplasia ossificans progressiva (FOP) is a severe, rare, autosomal dominant, ectopic ossifying condition, with primary involvement of the skeletal muscles associated with skeletal abnormalities. This report concerns an 11-year-old boy suffering from FOP, who presented significant modification of the musculoskeletal structure of the thorax and problems with articular movements. The patient showed progress after treatment with ethane-1-hydroxy-1,1-diphosphonate (EHDP). In fact, using a scintiscanner we were able to observe a significant improvement in symptoms and a recovery of some of the active sites of ossification.

Child↗

PubMed1982

Extra-articular ankylosis in fibrodysplasia ossificans progressiva.

Forty-four patients with fibrodysplasia ossificans progressiva were identified by a national survey in the United Kingdom. All patients had diagnostic skeletal malformations in addition to ectopic ossification in voluntary muscles and ligaments. Twenty-three of 34 patients (68 per cent) who were traced and examined had limitation of mandibular movement due to ectopic ossification in the muscles of mastication. In three cases such involvement was precipitated by dental therapy and in a further three by other trauma. Operative treatment of the established case was ineffective. Thus management must aim to avoid unnecessary jaw trauma.

Adolescent↗

PubMed2006

Fibrodysplasia ossificans progressiva-like condition in a cat.

Fibrodysplasia ossificans progressiva (FOP)-like condition was diagnosed in a Japanese domestic cat with stiffness, marked atrophy of the muscles, and limited mobility of all joints in both the pelvic limbs. Etretinate, a retinoid, was used for medical management; however, no improvement in the clinical signs was observed. Inheritance of the disorder has not yet been demonstrated. Furthermore, the clinical signs and histopathological findings of feline FOP-like condition in the present case differed from those of the previously reported cases.

Animals↗

PubMed2006

De novo 617G-A nucleotide mutation in the ACVR1 gene in a Taiwanese patient with fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disease with autosomal dominant transmission characterized by the presence of malformations of the big toes and of postnatal progressive heterotopic endochondral osteogenesis. We report the case of 3-year-old girl with dysplasia of the first metatarsal bones and progressive heterotopic ossificans of the right thigh due to previous diphtheria-tetanus-pertussis immunizations and several inappropriate surgical interventions. Direct sequence analysis identified a 617G-A nucleotide mutation in the patient but not in her parents or brother. Pedigree analysis suggests that a de novo mutation in the ACVR1 gene is responsible for the disease in this family. This is the first report of the results of a mutation analysis in a sporadic case of FOP in a Taiwanese patient.

Activin Receptors, Type I↗

PubMed1982

The cervical spine in fibrodysplasia ossificans progressiva.

Radiographs of the cervical spine in 11 patients with fibrodysplasia ossificans progressiva (FOP) have been examined, and abnormalities found in all. In 4, small cervical vertebral bodies with enlarged pedicles were seen in early childhood; later, variable fusion of the vertebrae was apparent in all patients. This fusion was first noted between adjacent neural arches in late childhood but also involved the vertebral bodies in some adult patients. It was only sometimes associated with ossification of the adjacent neck muscles, and appeared to be an important cause of neck stiffness in FOP. The radiological changes may be confused with those of Still's disease or of the Klippel-Feil syndrome.

Adolescent↗

PubMed1995

[Fibrodysplasia ossificans progressiva. Apropos of a case].

PURPOSE OF THE STUDY: Fibro-dysplasia ossificans progressive is a rare inherited disease, presumably transmitted as an autosomal dominant defect. The high level of spontaneous mutations explains the sporadic cases. Pre symptomatic diagnosis could be actually evoked by the association of progressive ossification of soft tissues with congenital anomalies of bones (metacarpal and metatarsal). The purpose of this study is to report a clinical case with a very severe course. CASE REPORT: A 10-year-old boy developed progressive ossification of muscles and soft tissues in multiple sites neck, back, shoulders, elbows, hips and knees. The clinical course was severe due to the ankylosis of all the joints and the decrease of pulmonary reserve with fixation of the chest wall. A malformation of the great toes facilitated the diagnosis. DISCUSSION: This pattern of heterotopic ossification together with the congenital malformations of the great toes defines the developmental phenotype for fibrodysplasia ossificans. A slow course with successive thrusts occurs. Ossification progressively involves tendons, ligaments and the connective tissue of skeletal muscles. Excision of heterotopic bone is futile as the trauma of the operation can lead to the stimulation of new heterotopic ossification at the operative site. Surgery is only useful for biopsy and histological study reveals bone metaplasia. The mature heterotopic bone is histologically indistinguishable from mature skeletal bone. The non skeletic muscles are characteristically spared from ossification. Premature death often results from respiratory failure due to fixation of the thoracic cage. The pathogenesis and the treatment of the disorder are unknown.

Adolescent↗

PubMed2005

Heterotopic ossification: a review.

Heterotopic ossification is defined as the presence of lamellar bone at locations where bone normally does not exist. The condition must be distinguished from metastatic calcifications, which mainly occur in hypercalcaemia, and dystrophic calcifications in tumours. It is a frequent complication following central nervous system disorders (brain injuries, tumours, encephalitis, spinal cord lesions), multiple injuries, hip surgery and burns. In addition to this acquired form, hereditary causes also exist, such as fibrodysplasia ossificans progressiva, progressive osseous heteroplasia and Albright's hereditary osteodystrophy. Although these conditions are extremely rare, they can provide useful information on the physiopathology of heterotopic ossification, and thus lead to novel and causal treatment modalities. Heterotopic ossification is no trivial complication. A limitation of the range of joint motion may have serious consequences for the daily functioning of people who are already severely incapacitated because of their original lesion. Increased contractures and spasticity, pressure ulcers and increasing pain further compromise the patient's capabilities. Consequently, we feel that attention should be paid to the pathogenesis and particularly the prevention and treatment of this disorder.

Burns↗

PubMed1989

[Fibrodysplasia ossificans progressiva or Münchmeyer's disease].

Fibrodysplasia ossificans progressiva is a heritable and generalized disorder of connective tissue, characterized by the appearance of bony tissue within the striated muscles, tendons and ligaments; moreover, some skeletal abnormalities may also occur, mainly microdactyly of the big toes. This is a very rare disease, which presents in early life; its course is unavoidably progressive, producing a sort of petrifaction of the patient some years after the first symptoms. It is defined as an autosomal dominant trait, being due in most cases to a new mutation. At present no known treatment is available to stop the course of the disease, but sometimes disodium etidronate may be effective in preventing calcification of heterotopic bony tissue.

Humans↗

PubMed1996

Fibrodysplasia ossificans progressiva in two half-sisters: evidence for maternal mosaicism.

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of connective tissue characterized by congenital malformation of the great toes and by progressive heterotopic ossification of the soft tissues in specific anatomic and temporal patterns. We observed classic findings of FOP in 2 Native American half-sisters with the same unaffected mother and different unaffected fathers. This is the first report of FOP in sibs from different pregnancies with unaffected parents. The findings in this family indicate the possibility of maternal gonadal mosaicism in FOP and provide important new data for genetic counseling in this disease.

Adolescent↗

PubMed2006

Fibrodysplasia ossificans progressiva in a Nigerian: a case report.

A case offibrodysplasia ossificans progressiva in a 3-year and 10-month-old Igala boy is presented. It was characterized by progressive ossification of connective tissue and muscles of the neck and chest. Characteristic digital anomalies particularly microdactyly of the hallux and pollex, hallux valgus, and clinodactyly were also noted. The 2nd to 5th metacarpals had epiphyses at both the proximal and distal ends instead of only at the proximal ends. Although the patient presented in the first year of life, the diagnosis was not made until in his fourth year of life. Early diagnosis of the disease is important in order to avoid all forms of trauma that may worsen the prognosis.

Child, Preschool↗

PubMed1993

Enchondroma in a patient with fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is a rare heritable disorder characterized by progressive heterotopic endochondral bone formation arising in ligament, tendon, and the fibrous connective tissue of skeletal muscle. The disorder is almost invariably associated with skeletal malformations of the endochondral anlage of the feet. A 25-year-old woman with FOP had a histopathologically documented phalangeal enchondroma. This common neoplasm of cartilage and bone seems not to have been previously reported in FOP; it may represent a coincidental occurrence, but in the context of the patient's genetic disorder, an enchondroma may represent an unusual and variable expression of the disease.

Adult↗

PubMed1995

Mild expression of fibrodysplasia ossificans progressiva: a report of 3 cases.

We describe 3 unusually mild cases of fibrodysplasia ossificans progressiva (FOP) in an 80-year-old man, a 44-year-old woman, and a 17-year-old woman. The man, whose daughter had classic features of FOP, lacked malformation of the great toes and experienced unusually slow progression of the disease. Both women displayed late onset heterotopic ossification. The older women displayed an unusually slow progression of the disease. All 3 patients remained ambulatory at the time of examination. Recognition of a mild form of FOP will influence diagnosis, counselling, and research in this rare condition.

Adolescent↗