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Avascular necrosis usually affects people between 30 and 50 years of age; about 10,000 to 20,000 people develop avascular necrosis of the head of the femur in the US each year. When it occurs in children at the femoral head, it is known as Legg-Calvé-Perthes syndrome.
The prognosis after different treatments varies and is based on several factors which include the age of the patient, the affected joint, the stage of the lesion and, most importantly, the state of the growth plate. It follows that the two main forms of osteochondritis dissecans are defined by skeletal maturity. The juvenile form of the disease occurs in open growth plates, usually affecting children between the ages of 5 and 15 years. The adult form commonly occurs between ages 16 to 50, although it is unclear whether these adults developed the disease after skeletal maturity or were undiagnosed as children.
The prognosis is good for stable lesions (stage I and II) in juveniles with open growth plates; treated conservatively—typically without surgery—50% of cases will heal. Recovery in juveniles can be attributed to the bone's ability to repair damaged or dead bone tissue and cartilage in a process called bone remodeling. Open growth plates are characterized by increased numbers of undifferentiated chondrocytes (stem cells) which are precursors to both bone and cartilaginous tissue. As a result, open growth plates allow for more of the stem cells necessary for repair in the affected joint. Unstable, large, full-thickness lesions (stage III and IV) or lesions of any stage found in the skeletally mature are more likely to fail non-operative treatment. These lesions offer a worse prognosis and surgery is required in most cases.
The main risk factors are bone fractures, joint dislocations, alcoholism, and the use of high dose steroids. Other risk factors include radiation therapy, chemotherapy, and organ transplantation. Osteonecrosis is also associated with cancer, lupus, sickle cell disease, HIV infection, Gaucher’s disease, and Caisson disease. The condition may also occur without any clear reason.
Bisphosphonates are associated with osteonecrosis of the mandible. Prolonged, repeated exposure to high pressures (as experienced by commercial and military divers) has been linked to AVN, though the relationship is not well understood.
OCD is a relatively rare disorder, with an estimated incidence of 15 to 30 cases per 100,000 persons per year. Widuchowski W "et al." found OCD to be the cause of articular cartilage defects in 2% of cases in a study of 25,124 knee arthroscopies. Although rare, OCD is noted as an important cause of joint pain in active adolescents. The juvenile form of the disease occurs in children with open growth plates, usually between the ages 5 and 15 years and occurs more commonly in males than females, with a ratio between 2:1 and 3:1. However, OCD has become more common among adolescent females as they become more active in sports. The adult form, which occurs in those who have reached skeletal maturity, is most commonly found in people 16 to 50 years old.
While OCD may affect any joint, the knee—specifically the medial femoral condyle in 75–85% of knee cases—tends to be the most commonly affected, and constitutes 75% of all cases. The elbow (specifically the capitulum of the humerus) is the second most affected joint with 6% of cases; the talar dome of the ankle represents 4% of cases. Less frequent locations include the patella, vertebrae, the femoral head, and the glenoid of the scapula.
The ultimate cause for these conditions is unknown, but the most commonly cited cause factors are rapid growth, heredity, trauma (or overuse), anatomic conformation, and dietary imbalances; however, only anatomic conformation and heredity are well supported by scientific literature. The way that the disease is initiated has been debated. Although failure of chondrocyte differentiation, formation of a fragile cartilage, failure of blood supply to the growth cartilage, and bone necrosis all have been proposed as the starting point in the pathogenesis, recent literature strongly supports failure of blood supply to growth cartilage as most likely.
Dysbaric osteonecrosis is a significant occupational hazard, occurring in 50% of commercial Japanese divers, 65% of Hawaiian fishermen and 16% of commercial and caisson divers in the UK.
Its relationship to compressed air is strong in that it may follow a single exposure to compressed air, may occur with no history of DCS but is usually associated with significant compressed air exposure. The distribution of lesions differs with the type of exposure - the juxta-articular lesions being more common in caisson workers than in divers.
There is a definite relationship between length of time exposed to extreme depths and the percentage of divers with bone lesions. Evidence does not suggest that dysbaric osteonecrosis is a significant risk in recreational scuba diving.
Giant osteoclasts can occur in some diseases, including Paget's disease of bone and bisphosphonate toxicity.
Children younger than 6 have the best prognosis, since they have time for the dead bone to revascularize and remodel, with a good chance that the femoral head will recover and remain spherical after resolution of the disease. Children who have been diagnosed with Perthes' disease after the age of 10 are at a very high risk of developing osteoarthritis and coxa magna. When an LCP disease diagnosis occurs after age 8, a better outcome results with surgery rather than nonoperative treatments. Shape of femoral head at the time when Legg-Calve Perthes disease heals is the most important determinant of risk for degenerative arthritis; hence, the shape of femoral head and congruence of hip are most useful outcome measures.
The exact cause of Kienböck's is not known, though there are thought to be a number of factors predisposing a person to Kienböck's.
Recent studies have made a correlation between Kienböck's sufferers and Western European ancestry, but no definitive link can be positively confirmed.
The necrosis of the lunate bone can frequently be traced to a trauma to the wrist, like a compound fracture, which could cause the lunate's blood supply to be interrupted. Blood flows to the lunate through several arteries, each supplying a percentage. When one of these pathways is severed, the likelihood the patient will develop necrosis increases.
Despite a preponderance of evidence, no particular cause has been conclusively verified.
Data exists on the internet that most people suffering from Kienböck's are affected in their dominant hand, though about one-third of sufferers report the condition in their non-dominant hand. In very few cases have there been people that have acquired it in both wrists.
Kienböck's disease is classified as a "rare disorder," meaning that it affects fewer than 200,000 people in the U.S. population.
Many Kienböck's patients are frustrated by the lack of consensus among hand surgeons about optimal treatments for Kienböck's. No matter what the disease's stage of progression, there is no one best treatment, and the decision is often based partially, or even mostly, on incidental factors such as the patient's pain tolerance, the patient's desire to return to active use of the hand (such as in manual occupations), and the surgeon's level of expertise with different treatments.
Though, since each case of Kienböck's is different, the makeup of the wrist and arm bones are important factors which are individualized to each patient. Therefore, one surgery will never be able to solve all the problems associated with the disease. Thus, no consensus can be reached among surgeons.
A sequestrum (plural: sequestra) is a piece of dead bone that has become separated during the process of necrosis from normal or sound bone.
It is a complication (sequela) of osteomyelitis. The pathological process is as follows:
- infection in the bone leads to an increase in intramedullary pressure due to inflammatory exudates
- the periosteum becomes stripped from the osteum, leading to vascular thrombosis
- bone necrosis follows due to lack of blood supply
- sequestra are formed
The sequestra are surrounded by sclerotic bone which is relatively avascular (without a blood supply). Within the bone itself, the haversian canals become blocked with scar tissue, and the bone becomes surrounded by thickened periosteum.
Due to the avascular nature of this bone, antibiotics which travel to sites of infection via the bloodstream poorly penetrate these tissues, hence the difficulty in treating chronic osteomyelitis.
At the same time as this, new bone is forming (known as involucrum). Openings in this involucrum allow debris and exudates (including pus) to pass from the sequestrum via sinus tracts to the skin.
Rarely, a sequestrum may turn out to be an osteoid osteoma, a rare tumor of the bone.
Osteochondrosis is a family of orthopedic diseases of the joint that occur in children and adolescents and in rapidly growing animals, particularly pigs, horses, dogs, and broiler chickens. They are characterized by interruption of the blood supply of a bone, in particular to the epiphysis, followed by localized bony necrosis, and later, regrowth of the bone. This disorder is defined as a focal disturbance of endochondral ossification and is regarded as having a multifactorial cause, so no one thing accounts for all aspects of this disease.
Perthes' disease is one of the most common hip disorders in young children, occurring in roughly 5.5 of 100,000 children per year. The lifetime risk of a child developing the disease is about one per 1,200 individuals. Boys are affected about three to five times more often than girls. New cases of Perthes' disease rarely occur after age 14 years (if diagnosed after 14 years of age, then it is usually old disease from early in childhood or avascular necrosis from an alternative cause).
White northern Europeans appear to be affected more frequently than other races, though a paucity of reliable epidemiology exists in the Southern Hemisphere. Children of sufferers of the disease themselves may have a very slightly increased risk, though it is unclear if this is because of a genetic predisposition, or a shared environmental factor. It is most commonly seen in persons aged three to 12 years, with a median of six years of age. The UK incidence rates show an intriguing pattern with low incidence rates in London, and a progressive increase in disease in more northerly areas (maximal in Scotland). Some evidence suggests, at least in developed countries, more socioeconomically deprived communities have a greater risk of disease (a similar trend to diseases such as adult heart disease), though the reason for this remains unknown. One possible explanation that has been considered is tobacco smoke exposure, though this is significantly confounded by the strong socioeconomic gradient common to both smoking and Perthes' disease. Dietary factors of the child, and of the mother during pregnancy, are of interest to the research groups.
If the diver has not been exposed to excessive depth and decompression and presents as DON, there may be a predisposition for the condition. Diving should be restricted to shallow depths. Divers who have suffered from DON are at increased risk of future fracture of a juxta-articular lesion during a dive, and may face complications with future joint replacements. Because of the young age of the population normally affected, little data is available regarding joint replacement complications.
There is the potential for worsening of DON for any diving where there might be a need for decompression, experimental or helium diving. Physically stressful diving should probably be restricted, both in sport diving and work diving due to the possibility of unnecessary stress to the joint. Any diving should be less than 40 feet/12 meters. These risks are affected by the degree of disability and by the type of lesion (juxta-articular or shaft).
An osteoclast (from the Greek words for "bone" (ὀστέον), and "broken" (κλαστός)) is a type of bone cell that breaks down bone tissue. This function is critical in the maintenance, repair, and remodelling of bones of the vertebral skeleton. The osteoclast disassembles and digests the composite of hydrated protein and mineral at a molecular level by secreting acid and a collagenase, a process known as "bone resorption". This process also helps regulate the level of blood calcium.
An odontoclast (/odon·to·clast/; o-don´to-klast) is an osteoclast associated with absorption of the roots of deciduous teeth.
In the US, the annual incidence of stress fractures in athletes and military recruits ranges from 5% to 30%, depending on the sport and other risk factors. Women and highly active individuals are also at a higher risk. The incidence probably also increases with age due to age-related reductions in bone mass density (BMD). Children may also be at risk because their bones have yet to reach full density and strength. The female athlete triad also can put women at risk as disordered eating and osteoporosis can cause the bones to be severely weakened.
Currently, the genetic or environmental factors that predispose an individual for chondroblastoma are not well known or understood. Chondroblastoma affects males more often than females at a ratio of 2:1 in most clinical reports. Furthermore, it is most often observed in young patients that are skeletally immature, with most cases diagnosed in the second decade of life. Approximately 92% of patients presenting with chondroblastoma are younger than 30 years. There is no indication of a racial predilection for chondroblastoma.
Perinatal and infantile hypophosphatasia are inherited as autosomal recessive traits with homozygosity or compound heterozygosity for two defective TNSALP alleles. The mode of inheritance for childhood, adult, and odonto forms of hypophosphatasia can be either autosomal dominant or recessive. Autosomal transmission accounts for the fact that the disease affects males and females with equal frequency. Genetic counseling is complicated by the disease’s variable inheritance pattern, and by incomplete penetration of the trait.
Hypophosphatasia is a rare disease that has been reported worldwide and appears to affect individuals of all ethnicities. The prevalence of severe hypophosphatasia is estimated to be 1:100,000 in a population of largely Anglo-Saxon origin. The frequency of mild hypophosphatasia is more challenging to assess because the symptoms may escape notice or be misdiagnosed. The highest incidence of hypophosphatasia has been reported in the Mennonite population in Manitoba, Canada where one in every 25 individuals are considered carriers and one in every 2,500 newborns exhibits severe disease. Hypophosphatasia is considered particularly rare in people of African ancestry in the U.S.
All clinical sub-types of hypophosphatasia have been traced to genetic mutations in the gene encoding TNSALP, which is localized on chromosome 1p36.1-34 in humans (ALPL; OMIM#171760). Approximately 204 distinct mutations have been described in the TNSALP gene. An up-to-date list of mutations is available online at The Tissue Nonspecific Alkaline Phosphatase Gene Mutations Database. About 80% of the mutations are missense mutations. The number and diversity of mutations results in highly variable phenotypic expression, and there appears to be a correlation between genotype and phenotype in hypophosphatasia”. Mutation analysis is possible and available in 3 laboratories.
Kienböck's disease is a disorder of the wrist. It is named for Dr. Robert Kienböck, a radiologist in Vienna, Austria who described osteomalacia of the lunate in 1910.
It is breakdown of the lunate bone, a carpal bone in the wrist that articulates with the radius in the forearm. Specifically, Kienböck's disease is another name for avascular necrosis (death and fracture of bone tissue due to interruption of blood supply) with fragmentation and collapse of the lunate. This has classically been attributed to arterial disruption, but may also occur after events that produce venous congestion with elevated interosseous pressure.
Freiberg disease, also known as a Freiberg infraction, is a form of avascular necrosis in the metatarsal bone of the foot. It generally develops in the second metatarsal, but can occur in any metatarsal. Physical stress causes multiple tiny fractures where the middle of the metatarsal meets the growth plate. These fractures impair blood flow to the end of the metatarsal resulting in the death of bone cells (osteonecrosis). It is an uncommon condition, occurring most often in young women, athletes, and those with abnormally long metatarsals. Approximately 80% of those diagnosed are women.
Initial treatment is generally 4–6 weeks of limited activity, often with crutches or orthotics. In rare cases, surgery is necessary to reduce the bone mass of the metatarsal.
The condition was first described by Dr. Alfred H. Freiberg in 1914. He initially thought the condition was caused by acute physical trauma, which is why it was initially called an infraction.
Peripheral arthritis has been reported in 92% of cases of SAPHO as well.
In children, the SAPHO syndrome is most likely to affect the metaphysis of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.
Tooth ankylosis refers to a fusion (ankylosis) of teeth to bone. The condition is diagnosed with radiographs (X-rays), which show loss of the periodontal ligament space and blending of the root with the bone. Clinically the tooth sounds solid when percussed (tapped) compared to the dull, cushioned sound from normal teeth. Ankylosis of teeth is uncommon, more so in deciduous teeth than permanent teeth.
A related condition, bisphosphonate-associated osteonecrosis of the jaw (BON), has been described as a side-effect of amino-bisphosphonates, a class of phosphorus-based drugs that inhibit bone resorption and are used widely for treating osteoporosis, bone disease in cancer and some other conditions.
BON, sometimes called "bis-phossy jaw",
is primarily associated with the use of intravenous bisphosphonates in the treatment of cancer. The percentage incidence of BON from this use is approximately 1000 times higher than the incidence of BON caused by the use of oral bisphosphonates.
Repair with cementum or dentin occurs after partial root resorption, fusing the tooth with the bone. It may occur following dental trauma, especially occlusal trauma, or after periapical periodontitis caused by pulp necrosis. Ankylosis itself is not a reason to remove a permanent tooth, however teeth which must be removed for other reasons are made significantly more difficult to remove if they are ankylosed.
Schmorl's nodes or Schmorl's nodules are protrusions of the nucleus pulposus of the intervertebral disc through the vertebral body endplate and into the adjacent vertebra.