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Computed tomography is the most sensitive and specific of the imaging techniques. The facial bones can be visualized as slices through the skeletal in either the axial, coronal or sagittal planes. Images can be reconstructed into a 3-dimensional view, to give a better sense of the displacement of various fragments. 3D reconstruction, however, can mask smaller fractures owing to volume averaging, scatter artifact and surrounding structures simply blocking the view of underlying areas.
Research has shown that panoramic radiography is similar to computed tomography in its diagnostic accuracy for mandible fractures and both are more accurate than plain film radiograph. The indications to use CT for mandible fracture vary by region, but it does not seem to add to diagnosis or treatment planning except for comminuted or avulsive type fractures, although, there is better clinician agreement on the location and absence of fractures with CT compared to panoramic radiography.
Treatment of condylar resorption is controversial. Orthognathic surgery may be done to reconstruct and stabilize the condyles and disc of the temporomandibular joint. Anti-infammatory medication is also used to slow the resorption process. Orthodontics may be used to treat the occlusion. Arthrocentesis, and arthroscopic surgery are also sometimes used to treat disc displacement and other symptoms.
There are various classification systems of mandibular fractures in use.
Usual diagnosis is via radiograph, patient history, biopsy is rarely needed. Periodic follow ups should included additional radiographs that show minimal growth or regression.
Condylar resorption, also called idiopathic condylar resorption, ICR, and condylysis, is a temporomandibular joint disorder in which one or both of the mandibular condyles are broken down in a bone resorption process. This disorder is nine times more likely to be present in females than males, and is more common among teenagers.
Osteonecrosis of the jaw is classified based on severity, number of lesions, and lesion size. Osteonecrosis of greater severity is given a higher grade, with asymptomatic ONJ designated as grade 1 and severe ONJ as grade 4.
Since elevated PGE2 levels are correlated with PDP, urinary PGE2 can be a useful biomarker for this disease. Additionally, HPGD mutation analyses are relatively cheap and simple and may prove to be useful in early investigation in patients with unexplained clubbing or children presenting PDP-like features. Early positive results can prevent expensive and longtime tests at identifying the pathology.
For the follow-up of PDP disease activity, bone formation markers such as TAP, BAP, BGP, carbodyterminal propeptide of type I procallagen or NTX can play an important role. Other biomarkers that can be considered are IL-6 and receptor activator of NF-κB ligand (RANKL), which are associated with increased bone resorption in some patients. However, further investigation is needed to confirm this use of disease monitoring.
Prostaglandin E2 may also be raised in patients with lung cancer and finger clubbing. This may be related to raised levels of cyclooxygenase-2, an enzyme involved in the metabolism of prostaglandins. A similar association has been noted in cystic fibrosis.
The treatment should be tailored to the cause involved and the severity of the disease process. With oral osteoporosis the emphasis should be on good nutrient absorption and metabolic wastes elimination through a healthy gastro-intestinal function, effective hepatic metabolism of toxicants such as exogenous estrogens, endogenous acetaldehyde and heavy metals, a balanced diet, healthy lifestyle, assessment of factors related to potential coagulopathies, and treatment of periodontal diseases and other oral and dental infections.
In cases of advanced oral ischaemic osteoporosis and/or ONJ that are not bisphosphonates related, clinical evidence has shown that surgically removing the damaged marrow, usually by curettage and decortication, will eliminate the problem (and the pain) in 74% of patients with jaw involvement. Repeat surgeries, usually smaller procedures than the first, may be required. Almost a third of jawbone patients will need surgery in one or more other parts of the jaws because the disease so frequently present multiple lesions, i.e., multiple sites in the same or similar bones, with normal marrow in between. In the hip, at least half of all patients will get the disease in the opposite hip over time; this pattern occurs in the jaws as well. Recently, it has been found that some osteonecrosis patients respond to anticoagulation therapies alone. The earlier the diagnosis the better the prognosis. Research is ongoing on other non-surgical therapeutic modalities that could alone or in combination with surgery further improve the prognosis and reduce the morbidity of ONJ. A greater emphasis on minimizing or correcting known causes is necessary while further research is conducted on chronic ischaemic bone diseases such as oral osteoporosis and ONJ.
In patients with bisphosphonates-associated ONJ, the response to surgical treatment is usually poor. Conservative debridement of necrotic bone, pain control, infection management, use of antimicrobial oral rinses, and withdrawal of bisphosphonates are preferable to aggressive surgical measures for treating this form of ONJ. Although an effective treatment for bisphosphonate-associated bone lesions has not yet been established, and this is unlikely to occur until this form of ONJ is better understood, there have been clinical reports of some improvement after 6 months or more of complete cessation of bisphosphonate therapy.
The easiest way to diagnose PDP is when pachydermia, finger clubbing and periostosis of the long bones are present. New bone formation under the periosteum can be detected by radiographs of long bones. In order diagnose PDP, often other diseases must be excluded. For example, to exclude secondary hypertrophic osteoarthropathy, any signs of cardiovascular, pulmonary, hepatic, intestinal and mediastinal diseases must be absent. MRI and ultrasound also have characterictic findings.
Skin biopsy is another way to diagnose PDP. However, it is not a very specific method, because other diseases share the same skin alterations with PDP, such as myxedema and hypothyroidism. In order to exclude these other diseases, hormonal studies are done. For example, thyrotropin and growth hormone levels should be examined to exclude thyroid acropachy and acrome. However, skin biopsy helps to diagnose PDP in patients without skin manifestations.
When clubbing is observed, it is helpful to check whether acroosteolysis of distal phalanges of fingers is present. This is useful to diagnose PDP, because the combination of clubbing and acroosteolysis is only found in PDP and Cheney’s syndrome.
Most temporomandibular disorders (TMDs) are self-limiting and do not get worse. Simple treatment, involving self-care practices, rehabilitation aimed at eliminating muscle spasms, and restoring correct coordination, is all that is required. Nonsteroidal anti inflammatory analgesics (NSAIDs) should be used on a short-term, regular basis and not on an as needed basis. On the other hand, treatment of chronic TMD can be difficult and the condition is best managed by a team approach; the team consists of a primary care physician, a dentist, a physiotherapist, a psychologist, a pharmacologist, and in small number of cases, a surgeon. The different modalities include patient education and self-care practices, medication, physical therapy, splints, psychological counseling, relaxation techniques, biofeedback, hypnotherapy, acupuncture, and arthrocentesis.
As with most dislocated joints, a dislocated jaw can usually be successfully positioned into its normal position by a trained medical professional. Attempts to readjust the jaw without the assistance of a medical professional could result in worsening of the injury. The health care provider may be able to set it back into the correct position by manipulating the area back into its proper position. Numbing medications such as general anesthetics, muscle relaxants, or in some cases sedation, may be needed to relax the strong jaw muscle. In more severe cases, surgery may be needed to reposition the jaw, particularly if repeated jaw dislocations have occurred.
The U.S. Preventive Services Task Force (USPSTF) recommend that all women 65 years of age or older be screened by bone densitometry. Additionally they recommend screening women with increased risk factors that puts them at risk equivalent to a 65‑year‑old. There is insufficient evidence to make recommendations about the intervals for repeated screening and the appropriate age to stop screening. In men the harm versus benefit of screening for osteoporosis is unknown. Prescrire states that the need to test for osteoporosis in those who have not had a previous bone fracture is unclear. The International Society for Clinical Densitometry, however, suggest BMD testing for men 70 or older, or those who are indicated for risk equal to that of a 70‑year‑old. A number of tools exist to help determine who is reasonable to test.
The calcific deposits are visible on X-ray as discrete lumps or cloudy areas. The deposits look cloudy on X-ray if they are in the process of reabsorption, and this is also when they cause the most pain. The deposits are crystalline when in their resting phase and like toothpaste in the reabsorptive phase. However, poor correlation exists between the appearance of a calcific deposit on plain X-rays and its consistency on needling. Ultrasound is also useful to depict calcific deposits and closely correlates with the stage of disease.
Distal clavicular osteolysis (DCO) is often associated with problems weightlifters have with their acromioclavicular joints due to high stresses put on the clavicle as it meets with the acromion. This condition is often referred to as "weight lifter's shoulder". Medical ultrasonography readily depicts resorption of the distal clavicle as irregular cortical erosions, whereas the acromion remains intact. Associated findings may include distended joint capsule, soft-tissue swelling, and joint instability.
A common surgery to treat recalcitrant DCO is re-sectioning of the distal clavicle, removing a few millimetres of bone from the very end of the bone.
Diagnosis is confirmed by x-ray imaging. Displaced fractures are readily apparent. A non-displaced fracture can be difficult to identify and a fracture line may not be visible on the X-rays. However, the presence of a joint effusion is highly suggestive of a non-displaced fracture. Bleeding from the fracture expands the joint capsule and is visualized on the lateral view as a darker area anteriorly and posteriorly, and is known as the sail sign. Depending on the child's age, parts of the bone will still be developing and if not yet calcified, will not show up on the X-rays. At times, X-rays of the opposite elbow may be obtained for comparison. There are landmarks on the X-rays that can be used to assess displacement, including the "anterior humeral line", which is a line drawn down along the front of the humerus on the lateral view and it should pass through the middle third of the capitulum of the humerus.
While bone resorption is commonly associated with many diseases or joint problems, the term "osteolysis" generally refers to a problem common to artificial joint replacements such as total hip replacements, total knee replacements and total shoulder replacements. Osteolysis can also be associated with the radiographic changes seen in those with bisphosphonate-related osteonecrosis of the jaw.
There are several biological mechanisms which may lead to osteolysis. In total hip replacement, the generally accepted explanation for osteolysis involves wear particles (worn off the contact surface of the artificial ball and socket joint). As the body attempts to clean up these wear particles (typically consisting of plastic or metal), it triggers an autoimmune reaction which causes resorption of living bone tissue. Osteolysis has been reported to occur as early as 12 months after implantation and is usually progressive. This may require a revision surgery (replacement of the prosthesis).
Although osteolysis itself is clinically asymptomatic, it can lead to implant loosening or bone breakage, which in turn causes serious medical problems.
Jaw dislocation is common for people who are in car, motorcycle or related accidents and also sports related activities. This injury does not pin point specific ages or genders because it could happen to anybody. People who dislocate their jaw do not usually seek emergency medical care. In most cases, jaw dislocations are acute and can be altered by minor manipulations. It was reported from one study that over a seven-year period at an emergency medical site, with 100,000 yearly visits, there were only 37 patients that were seen for a dislocated jaw.
In 1983 Heffez and colleagues published a case report in which they suggested eight criteria for a definitive diagnosis of Gorham's disease:
- Positive biopsy with the presence of angiomatous tissue
- Absence of cellular atypia
- Minimal or no osteoblastic response or dystrophic calcifications
- Evidence of local bone progressive osseous resorption
- Non-expansile, non-ulcerative lesions
- No involvement of viscera
- Osteolytic radiographic pattern
- Negative hereditary, metabolic, neoplastic, immunologic, or infectious etiology.
In the early stages of the disease x-rays reveal changes resembling patchy osteoporosis. As the disease progresses bone deformity occurs with further loss of bone mass and, in the tubular bones (the long bones of the arms and legs), a concentric shrinkage is often seen which has been described as having a "sucked candy" appearance. Once the cortex (the outer shell) of the bone has been disrupted, vascular channels may invade adjacent soft tissues and joints. Eventually, complete or near-complete resorption of the bone occurs and may extend to adjacent bones, though spontaneous arrest of bone loss has been reported on occasion. Throughout this process, as the bone is destroyed it is replaced by angiomatous and/or fibrous tissue.
Often Gorham's disease is not recognized until a fracture occurs, with subsequent improper bone healing. The diagnosis essentially is one of exclusion and must be based on combined clinical, radiological, and histopathological findings. X-rays, CT scans, MRIs, ultrasounds, and nuclear medicine (bone scans) are all important tools in the diagnostic workup and surgical planning, but none have the ability alone to produce a definitive diagnosis. Surgical biopsy with histological identification of the vascular or lymphatic proliferation within a generous section of the affected bone is an essential component in the diagnostic process.
Recognition of the disease requires a high index of suspicion and an extensive workup. Because of its serious morbidity, Gorham's must always be considered in the differential diagnosis of osteolytic lesions.
"Baumann's angle", also known as the humeral-capitellar angle, is measured on an AP radiograph of the elbow between the long axis of the humerus and the growth plate of the lateral condyle.
Reported normal values for Baumann's angle range between 9 and 26° An angle of more than 10° is generally regarded as acceptable. When reducing paediatric supracondylar humerus fractures, a deviation of more than 5° from the contralateral side should not be accepted.
Alteration of Baumann angle: Baumann's angle is created by drawing a line parallel to the longitudinal axis of the humeral shaft and a line along the lateral condylar physis as viewed on the AP image normal is 70-75 degrees, but best judge is a comparison of the contralateral side deviation of more than 5 degrees indicates coronal plane deformity and should not be accepted.
In those with calcific tendinitis of the shoulder high energy extracorporeal shock-wave therapy (which uses sound waves) can be useful. It is not useful in other types of tendinitis. This procedure may be known as .
Outcomes vary depending on the location of the disease, the degree of damage to the joint, and whether surgical repair was necessary. Average healing times vary from 55–97 days depending on location. Up to 1–2 years may be required for complete healing.
Quantitative computed tomography differs from DXA in that it gives separate estimates of BMD for trabecular and cortical bone and reports precise volumetric mineral density in mg/cm rather than BMD's relative Z score. Among QCT's advantages: it can be performed at axial and peripheral sites, can be calculated from existing CT scans without a separate radiation dose, is sensitive to change over time, can analyze a region of any size or shape, excludes irrelevant tissue such as fat, muscle, and air, and does not require knowledge of the patient's subpopulation in order to create a clinical score (e.g. the Z-score of all females of a certain age). Among QCT's disadvantages: it requires a high radiation dose compared to DXA, CT scanners are large and expensive, and because its practice has been less standardized than BMD, its results are more operator-dependent. Peripheral QCT has been introduced to improve upon the limitations of DXA and QCT.
Quantitative ultrasound has many advantages in assessing osteoporosis. The modality is small, no ionizing radiation is involved, measurements can be made quickly and easily, and the cost of the device is low compared with DXA and QCT devices. The calcaneus is the most common skeletal site for quantitative ultrasound assessment because it has a high percentage of trabecular bone that is replaced more often than cortical bone, providing early evidence of metabolic change. Also, the calcaneus is fairly flat and parallel, reducing repositioning errors. The method can be applied to children, neonates, and preterm infants, just as well as to adults. Some ultrasound devices can be used on the tibia.
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.
OFC may be diagnosed using a variety of techniques. Muscles in patients afflicted with OFC can either appear unaffected or "bulked up." If muscular symptoms appear upon the onset of hyperparathyroidism, they are generally sluggish contraction and relaxation of the muscles. Deviation of the trachea (a condition in which the trachea shifts from its position at the midline of the neck), in conjunction with other known symptoms of OFC can point to a diagnosis of parathyroid carcinoma.
Blood tests on patients with OFC generally show high levels of calcium (normal levels are considered to range between 8.5 and 10.2 mg/dL, parathyroid hormone (levels generally above 250 pg/mL, as opposed to the "normal" upper-range value of 65 pg/mL), and alkaline phosphatase(normal range is 20 to 140 IU/L).
X-rays may also be used to diagnose the disease. Usually, these X-rays will show extremely thin bones, which are often bowed or fractured. However, such symptoms are also associated with other bone diseases, such as osteopenia or osteoporosis. Generally, the first bones to show symptoms via X-ray are the fingers. Furthermore, brown tumors, especially when manifested on facial bones, can be misdiagnosed as cancerous. Radiographs distinctly show bone resorption and X-rays of the skull may depict an image often described as "ground glass" or "salt and pepper". Dental X-rays may also be abnormal.
Cysts may be lined by osteoclasts and sometimes blood pigments, which lend to the notion of "brown tumors." Such cysts can be identified with nuclear imaging combined with specific tracers, such as sestamibi. Identification of muscular degeneration or lack of reflex can occur through clinical testing of deep tendon reflexes, or via photomotogram (an achilles tendon reflex test).
Fine needle aspiration (FNA) can be used to biopsy bone lesions, once found on an X-ray or other scan. Such tests can be vital in diagnosis and can also prevent unnecessary treatment and invasive surgery. Conversely, FNA biopsy of tumors of the parathyroid gland is not recommended for diagnosing parathyroid carcinoma and may in fact be harmful, as the needle can puncture the tumor, leading to dissemination and the possible spread of cancerous cells.
The brown tumors commonly associated with OFC display many of the same characteristics of osteoclasts. These cells are characteristically benign, feature a dense, granular cytoplasm, and a nucleus that tends to be ovular in shape, enclosing comparatively fine chromatin. Nucleoli also tend to be smaller than average.
Treatment of Gorham's disease is for the most part palliative and limited to symptom management.
Sometimes the bone destruction spontaneously ceases and no treatment is required. But when the disease is progressive, aggressive intervention may be necessary. Duffy and colleagues reported that around 17% of patients with Gorham's disease in the ribs, shoulder, or upper spine experience extension of the disease into the chest, leading to chylothorax with its serious consequences, and that the mortality rate in this group can reach as high as 64% without surgical intervention.
A search of the medical literature reveals multiple case reports of interventions with varying rates of success as follows:
Cardiothoracic (heart & lung):
- Pleurodesis
- Ligation of thoracic duct
- Pleurperitoneal shunt
- Radiation therapy
- Pleurectomy
- Surgical resection
- Thalidomide
- Interferon alpha-2b
- TPN (total parenteral nutrition)
- Thoracentesis
- Diet rich in medium-chain triglycerides and protein
- Chemotherapy
- Sclerotherapy
- Transplantation
Skeletal:
- Interferon alpha-2b
- Bisphosphonate (e.g. pamidronate)
- Surgical resection
- Radiation therapy
- Sclerotherapy
- Percutaneous bone cement
- Bone graft
- Prosthesis
- Surgical stabilization
- Amputation
To date, there are no known interventions that are consistently effective for Gorham's and all reported interventions are considered experimental treatments, though many are routine for other conditions. Some patients may require a combination of these approaches. Unfortunately, some patients will not respond to any intervention.
Even though more studies are necessary for an efficient evaluation of the role played by zinc in senile osteoporosis, doctors recommend a proper supplementation of dietary zinc.
Replacement estrogen has proved to be an efficient way to combat the loss of bone mass in women when such treatment is started in the menopausal stage of their lives. John R. Lee, a Harvard graduate who wrote a book on the subject, came to the conclusion that by adding supplementation with natural progesterone to an existing natural osteoporosis treatment program, bone density was increased every year by 3-5% until it stabilized at the bone density levels expected for a 35-year-old woman, this after studies in 100 women between 38 and 83 with an average of 62 years old.