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Manipulative physiotherapy, therapeutic exercises and chiropractic manipulative therapy shows beneficial results for decreasing pain and increasing spinal range of motion. As areas of the spine and tendons can become inflamed NSAIDs such as ibuprofen and Naproxen can be helpful in both relieving pain and inflammation associated with DISH. It is hoped that by minimizing inflammation in these areas, further calcification of tendons and ligaments of the spine leading to bony outgrowths (enthesophytes) will be prevented, although causative factors are still unknown.
The use of orthotic bracing, pioneered by Sydney Haje as of 1977, is finding increasing acceptance as an alternative to surgery in select cases of pectus carinatum. In children, teenagers, and young adults who have pectus carinatum and are motivated to avoid surgery, the use of a customized chest-wall brace that applies direct pressure on the protruding area of the chest produces excellent outcomes. Willingness to wear the brace as required is essential for the success of this treatment approach. The brace works in much the same way as orthodontics (braces that correct the alignment of teeth). The brace consists of front and back compression plates that are anchored to aluminum bars. These bars are bound together by a tightening mechanism which varies from brace to brace. This device is easily hidden under clothing and must be worn from 14 to 24 hours a day. The wearing time varies with each brace manufacturer and the managing physicians protocol, which could be based on the severity of the carinatum malformation (mild moderate severe) and if it is symmetric or asymmetric.
Depending on the manufacturer and/or the patient's preference, the brace may be worn on the skin or it may be worn over a body 'sock' or sleeve called a Bracemate, specifically designed to be worn under braces. A physician or orthotist or brace manufacturer's representative can show how to check to see if the brace is in correct position on the chest.
Bracing is becoming more popular over surgery for pectus carinatum, mostly because it eliminates the risks that accompany surgery. The prescribing of bracing as a treatment for pectus carinatum has 'trickled down' from both paediatric and thoracic surgeons to the family physician and pediatricians again due to its lower risks and well-documented very high success results. The pectus carinatum guideline of 2012 of the American Pediatric Surgical Association has stated: "As reconstructive therapy for the compliant pectus [carinatum] malformation, nonoperative compressive orthotic bracing is usually an appropriate first line of therapy as it does not preclude the operative option. For appropriate candidates, orthotic bracing of chest wall malformations can reasonably be expected to prevent worsening of the malformation and often results in a lasting correction of the malformation. Orthotic bracing is often successful in prepubertal children whose chest wall is compliant. Expert opinion suggests that the noncompliant chest wall malformation or significant asymmetry of the pectus carinatum malformation caused by a concomitant excavatum-type malformation may not respond to orthotic bracing."
Regular supervision during the bracing period is required for optimal results. Adjustments may be needed to the brace as the child grows and the pectus improves.
Around 5 years of age, surgical correction may be necessary to prevent any worsening of the deformity. If the mother has dysplasia, caesarian delivery may be necessary. Craniofacial surgery may be necessary to correct skull defects. Coxa vara is treated by corrective femoral osteotomies. If there is brachial plexus irritation with pain and numbness, excision of the clavicular fragments can be performed to decompress it. In case of open fontanelle, appropriate headgear may be advised by the orthopedist for protection from injury.
For patients with severe pectus carinatum, surgery may be necessary. However bracing could and may still be the first line of treatment. Some severe cases treated with bracing may result in just enough improvement that patient is happy with the outcome and may not want surgery afterwards.
If bracing should fail for whatever reason then surgery would be the next step. The two most common procedures are the Ravitch technique and the Reverse Nuss procedure.
A modified Ravitch technique uses bioabsorbable material and postoperative bracing, and in some cases a diced rib cartilage graft technique.
The Nuss was developed by Donald Nuss at the Children's Hospital of the King's Daughters in Norfolk, Va. The Nuss is primarily used for Pectus Excavatum, but has recently been revised for use in some cases of PC, primarily when the malformation is symmetrical.
Several studies have reported that life expectancy appears to be normal for people with CCD.
In the majority of cases, spinal disc herniation doesn't require surgery, and a study on sciatica, which can be caused by spinal disc herniation, found that "after 12 weeks, 73% of people showed reasonable to major improvement without surgery." The study, however, did not determine the number of individuals in the group that had sciatica caused by disc herniation.
- Initial treatment usually consists of non-steroidal anti-inflammatory pain medication (NSAIDs), but the long-term use of NSAIDs for people with persistent back pain is complicated by their possible cardiovascular and gastrointestinal toxicity.
- Epidural corticosteroid injections provide a slight and questionable short-term improvement in those with sciatica but are of no long term benefit. Complications occur in 0 to 17% of cases when performed on the neck and most are minor. In 2014, the US Food and Drug Administration (FDA) suggested that the "injection of corticosteroids into the epidural space of the spine may result in rare but serious adverse events, including loss of vision, stroke, paralysis, and death." and that "The effectiveness and safety of epidural administration of corticosteroids have not been established, and FDA has not approved corticosteroids for this use.".
Non-surgical methods of treatment are usually attempted first, leaving surgery as a last resort. Pain medications are often prescribed as the first attempt to alleviate the acute pain and allow the patient to begin exercising and stretching. There are a variety of other non-surgical methods used in attempts to relieve the condition after it has occurred, often in combination with pain killers. They are either considered indicated, contraindicated, relatively contraindicated, or inconclusive based on the safety profile of their risk-benefit ratio and on whether they may or may not help:
The chest wall is elastic, gradually stiffening over age. Non-surgical treatments have been developed that aim at gradually alleviating the pectus excavatum condition, making use of the elasticity of the chest wall, including the costal cartilages, in particular in young cases.
Physical exercise has an important role in conservative pectus excavatum treatment though is not seen as a means to resolve the condition on its own. It is used in order to halt or slow the progression of mild or moderate excavatum conditions and as supplementary treatment to improve a poor posture, to prevent secondary complications, and to prevent relapse after treatment.
Exercises are aimed at improving posture, strengthening back and chest muscles, and enhancing exercise capacity, ideally also increasing chest expansion. Pectus exercises include deep breathing and breath holding exercises, as well as strength training for the back and chest muscles. Additionally, aerobic exercises to improve cardiopulmonary function are employed.
The first phase of the rehabilitation after surgery includes keeping the foot elevated and iced for the first 2 days after the operation. After those 2 days, using crutches or a wheelchair in which there is no weight applied to the affected foot is recommended to getting around. If no operation was performed, the foot should be submitted to frequent range of motion exercises. The second phase occurs 6 weeks after and consists of keeping the foot elevated and iced while resting and performing exercises in which only slight weight is applied to the affected area for the next two weeks, others recommend six weeks of this phase. In this phase, range of motion exercises should be implemented if surgery was needed for the fracture. The third and final phase of rehabilitation of calcaneal fractures is to allow the full body weight to be used and use crutches or a cane if needed, between 13 weeks to a year the patient is allowed to resume normal activities.
Non-surgical treatment is for extra-articular fractures and Sanders Type I intra-articular fractures, provided that the calcaneal weight-bearing surface and foot function are not compromised. Physicians may choose to perform closed reduction with or without fixation (casting), or fixation alone (without reduction), depending on the individual case. Recommendations include no weight-bearing for a few weeks followed by range-of-motion exercises and progressive weight bearing for a period of 2–3 months.
Displaced intra-articular fractures require surgical intervention within 3 weeks of fracture, before bone consolidation has occurred. Conservative surgery consists of closed reduction with percutaneous fixation. This technique is associated with less wound complications, better soft tissue healing (because of less soft tissue manipulati) and decreased intraoperative time. However, this procedure has increased risk of inadequate calcaneal bone fixation, compared to open procedures. Currently, open reduction with internal fixation (ORIF) is usually the preferred surgical approach when dealing with displaced intra-articular fractures. Newer, more innovative surgical techniques and equipment have decreased the incidence of intra- and post-operative complications.
Treatment usually includes antibiotics, and reducing the mobility of the affected region, either with a back brace or a plaster cast. Without treatment, the patient may form an abscess which may need to be surgically corrected. Due to the poor vascularity of the disc, drugs required for treatment often include potent agents such as Ciprofloxacin along with Vancomycin. Occasionally, oral drugs can be used to treat the infection but it may fail and IV drugs may be required.
If the patient is an adult many surgeons and doctors now recommend moving little and often and within the pain limits of the medication. Discs respond to osmotic pressure therefore movement is beneficial to increase their blood flow and fluid dynamics. This is why disc patients are no longer told to bed rest. In children whether to bed rest or move a little is decided on an individual basis, depending on the site and severity of the discitis.
Presence at birth is extremely rare and associated with other congenital anomalies such as proximal femoral focal deficiency, fibular hemimelia or anomalies in other part of the body such as cleidocranial dyastosis. The femoral deformity is present in the subtrochantric area where the bone is bent. The cortices are thickened and may be associated with overlying skin dimples. External rotation of the femur with valgus deformity of knee may be noted. This condition does not resolve and requires surgical management. Surgical management includes valgus osteotomy to improve hip biomechanics and length and rotational osteotomy to correct retroversion and lengthening.
Bisphosphonates are useful in decreasing the risk of future fractures in those who have already sustained a fracture due to osteoporosis. This benefit is present when taken for three to four years. Different bisphosphonates have not been directly compared, therefore it is unknown if one is better than another. Fracture risk reduction is between 25 and 70% depending on the bone involved. There are concerns of atypical femoral fractures and osteonecrosis of the jaw with long-term use, but these risks are low. With evidence of little benefit when used for more than three to five years and in light of the potential adverse events, it may be appropriate to stop treatment after this time. One medical organization recommends that after five years of medications by mouth or three years of intravenous medication among those at low risk, bisphosphonate treatment can be stopped. In those at higher risk they recommend up to ten years of medication by mouth or six years of intravenous treatment.
For those with osteoporosis but who have not had a fracture evidence does not support a reduction in fracture risk with risedronate or etidronate. Alendronate decreases fractures of the spine but does not have any effect on other types of fractures. Half stop their medications within a year. When on treatment with bisphosphonates rechecking bone mineral density is not needed. Another review found tentative evidence of benefit in males with osteoporosis.
Fluoride supplementation does not appear to be effective in postmenopausal osteoporosis, as even though it increases bone density, it does not decrease the risk of fractures.
Teriparatide ( a recombinant parathyroid hormone ) has been shown to be effective in treatment of women with postmenopausal osteoporosis. Some evidence also indicates strontium ranelate is effective in decreasing the risk of vertebral and nonvertebral fractures in postmenopausal women with osteoporosis. Hormone replacement therapy, while effective for osteoporosis, is only recommended in women who also have menopausal symptoms. It is not recommended for osteoporosis by itself. Raloxifene, while effective in decreasing vertebral fractures, does not affect the risk of nonvertebral fracture. And while it reduces the risk of breast cancer, it increases the risk of blood clots and strokes. Denosumab is also effective for preventing osteoporotic fractures but not in males. In hypogonadal men, testosterone has been shown to improve bone quantity and quality, but, as of 2008, no studies evaluated its effect on fracture risk or in men with a normal testosterone levels. Calcitonin while once recommended is no longer due to the associated risk of cancer and questionable effect on fracture risk.
Certain medications like alendronate, etidronate, risedronate, raloxifene and strontium ranelate can be helpful for the preventing of osteoporotic fragility fractures in postmenopausal women with osteoporosis.
Depending upon the treatment required, it is sometimes most appropriate to wait until later in life for a surgical remedy – the childhood growth of the face may highlight or increase the symptoms. When surgery is required, particularly when there is a severe disfiguration of the jaw, it is common to use a rib graft to help correct the shape.
According to literature, HFM patients can be treated with various treatment options such functional therapy with an appliance, distraction osteogenesis, or costochondral graft. The treatment is based on the type of severity for these patients. According to Pruzanksky's classification, if the patient has moderate to severe symptoms, then surgery is preferred. If patient has mild symptoms, then a functional appliance is generally used.
Patients can also benefit from a Bone Anchored Hearing Aid (BAHA).
Because newborns can breathe only through their nose, the main goal of postnatal treatment is to establish a proper airway. Primary surgical treatment of FND can already be performed at the age of 6 months, but most surgeons wait for the children to reach the age of 6 to 8 years. This decision is made because then the neurocranium and orbits have developed to 90% of their eventual form. Furthermore, the dental placement in the jaw has been finalized around this age.
To correct the rather prominent hypertelorism, wide nasal root and midline cleft in FND, a facial bipartition can be performed. This surgery is preferred to periorbital box-osteotomy because deformities are corrected with a better aesthetic result.
During the operation, the orbits are disconnected from the skull and the base of the skull. However, they remain attached to the upper jaw. Part of the forehead in the centre of the face is removed (median faciotomy) in the process. Then, the orbits are rotated internally, to correct the hypertelorism. Often, a new nasal bone will have to be interpositioned, using a bone transplant.
Complications of this procedure are: bleeding, meningitis, cerebrospinal fluid leakage and blindness.
Weight-bearing endurance exercise and/or exercises to strengthen muscles improve bone strength in those with osteoporosis. Aerobics, weight bearing, and resistance exercises all maintain or increase BMD in postmenopausal women. Fall prevention can help prevent osteoporosis complications. There is some evidence for hip protectors specifically among those who are in care homes.
There is no ‘standard treatment’ for people with CFND due to the large variations in phenotypic expression. Each patient needs to be assessed and treated based on their specific presentation in order to restore the aesthetic and functional balance.
Surgical corrections for the main symptoms;
- Craniosynostosis correction: The preferred age for this procedure is between 6–9 months of age. Performing this surgery at such an early age can limit the further development of facial asymmetry, if the asymmetry is caused by the craniosynostosis, and prevents prolonged elevated intracranial pressure (ICP). However, the data for the exact risk of an elevated intracranial pressure for patients with CFND is lacking in the published literature. The surgery involves a frontal bone advancement in combination with remodellation of the supraorbital rim.
- Orbital hypertelorism: It is preferred to wait with this treatment until the age of 5–8 years old, after permanent dentition. The procedures that can be performed are the facial bipartition and the box osteotomy. Facial bipartition is the more preferable choice as there are less additional corrections needed, as well as providing a more stable long-term result after treatment. After the correction of the orbitas, the medial corners of the eyes are put more into a horizontal line.
- Nasal deformity correction: The correction of the broad nasal base is simultaneously done with the orbital hypertelorism repair. This is for good alignment of the eyes with the nose for the best aesthetic result. A bifid nose tip will only be treated at the age of 18, when the patient's skeleton has fully matured.
T2 weighted MRI in neutral (A) and linear traction (B) of a seven-year-old Doberman with a two-year history of cervical pain treated with NSAIDs and presented acutely tretraplegic: A C6-C7 and C5-C6 traction responsive myelopathy are evident on MRI. The spinal-cord hyperintensity seen at the C5-C6 is suggestive of chronic lesion and most likely responsible for the chronic history of cervical pain, while the C5-C6 lesion was most likely responsible for the acute tetraplegia.
Same dog (A) treated with double implant (B) three days after surgery: The dog became ambulatory three days after surgery. Four weeks after surgery, it had ataxia without conscious proprioceptive deficits, and three months after surgery, the dog was neurologically normal. The owner reported it had been two years since the dog was able to hold its neck in an elevated position.
Congenital vertebral anomalies are a collection of malformations of the spine. Most around 85% are not clinically significant, but they can cause compression of the spinal cord by deforming the vertebral canal or causing instability. This condition occurs in the womb. Congenital vertebral anomalies include alterations of the shape and number of vertebrae.
More common cause: primary defect in endochondral ossification of the medial part of the femoral neck.
Excessive interuterine pressure on the developing fetal hip.
vascular insult.
Faulty maturation of the cartilage and metaphyseal bone of the femoral neck.
Clinical feature: presents after the child has started walking but before six years of age. Usually associated with a painless hip due to mild abductor weakness and mild limb length discrepancy.
If there is a bilateral involvement the child might have a waddling gait or trendelenburg gait with an increased lumbar lordosis. The greater trochanter is usually prominent on palpation and is more proximal. Restricted abduction and internal rotation.
X-ray: decreased neck shaft angle, increased cervicofemoral angle, vertical physis, shortened femoral neck decrease in femoral anteversion. HE angle (hilgenriener epiphyseal angle- angle subtended between a horizontal line connecting the triradiate cartilage and the epiphysisn normal angle is <30 degrees.
Treatment:
HE angle of 45–60 degrees observation and periodic follow up.
Indication for surgery :HE angle more than 60 degrees, progressive deformity, neckshaft angle <90 degrees, development of trendelenburg gait
Surgery: subtrochantric valgus osteotomy with adequate internal rotation of distal fragment to correct anteversion
common complication is recurrence. If HE angle is reduced to 38 degrees less evidence of recurrence
post operative spica cast is used for a period of 6–8 weeks.
Coxa vara is also seen in Niemann–Pick disease.
In 1968, Dr. David Rimoin and colleagues in Baltimore first distinguished between the two major presentations of Jarcho-Levin. Both conditions were characterized as failures of proper vertebral segmentation. However, the condition within the family described in their article appeared to be inherited in an autosomal dominant fashion and had a less severe course than that reported by other investigators. They specified their condition as spondylocostal dysplasia, which has since become known as spondylocostal dysostosis. The subtype of Jarcho-Levin with which they contrasted their reported cases to is now known as spondylothoracic dysplasia.
The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate, in which the notochord (a flexible rod of uniform composition) found in all chordates has been replaced by a segmented series of bones—vertebrae separated by intervertebral discs. The vertebral column houses the spinal canal, a cavity that encloses and protects the spinal cord.
There are about 50,000 species of animals that have a vertebral column. The human vertebral column is one of the most-studied examples.
Diffuse idiopathic skeletal hyperostosis (DISH) is a non-inflammatory spondyloarthropathy which predominantly affects the spine. It is characterized by ankylosis and enthesopathy (ossification of the ligaments and entheses). It most commonly affects the thoracic and thoraco-lumbar spine, but involvement is variable and can include the entire spine.