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MARIA CARMELA L. DOMOCMAT, RN, MSN INSTRUCTOR SCHOOL OF NURSING NORTHERN LUZON ADVENTIST COLLEGE ARTACHO, SISON, PANGASINAN
Overview
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Part 1: Degenerative & Metabolic bone disorders: Part 2: Bone infections Part 3: Muscular disorders Part 4: Disorders of the hand Part 5: Spinal column deformities
Scoliosis Kyphosis Lordosis Osteogenesis imperfecta (OI)
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Scoliosis
Scoliosis
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AKA
Spinal curvature Infantile scoliosis Juvenile scoliosis
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Scoliosis
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is an abnormal curving of the spine. But people with scoliosis have a spine that curves too much. The spine might look like the letter C or S.
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Congenital scoliosis
This type of scoliosis is present at birth. It occurs when the babys ribs or spine bones do not form properly.
Neuromuscular scoliosis
This type is caused by a nervous system problem that affects your muscles, such as cerebral palsy, muscular dystrophy, spina bifida, and polio.
Symptoms
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Dx exams
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PE: One shoulder is higher than the other, pelvis is tilted X-rays of the spine Spinal curve measurement (scoliometer screening) MRI of the spine
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Scoliometer screening
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Treatment
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Most people with idiopathic scoliosis do not need treatment. However, should be checked by a doctor about every 6 months. If still growing --back brace.
A back brace prevents further curving. There are many different types of braces. Back braces can be adjusted as you grow. Back braces work best in people over age 10. Braces do not work for those with congenital or neuromuscular scoliosis.
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Expectations (prognosis)
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depends on the type, cause, and severity of the curve. The more severe the curving, the more likely it will get worse after stop growing. People with mild scoliosis do very well with braces. They usually do not have long-term problems. However, scoliosis can make more likely to have back pain when get older. The outlook for those with neuromuscular or congenital scoliosis varies. Patients with neuromuscular scoliosis have another serious disorder (like cerebral palsy or muscular dystrophy) so their goals are much different. Often the goal of surgery is simply to allow a child to be able to sit upright in a wheelchair. Congenital scoliosis is difficult to treat and usually requires many surgeries.
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Complications
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Breathing problems (in severe scoliosis) Low back pain Lower self-esteem Persistent pain if there is wear and tear of the spine bones Spinal infection after surgery Spine or nerve damage from an uncorrected curve or spinal surgery
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Prevention
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Routine scoliosis screening is now done in middle and junior high schools. Screening has helped detect early scoliosis in many kids.
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References
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http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0 002221/ Hedequist DJ. Surgical treatment of congenital scoliosis. Orthop Clin North Am. 2007;38:497-509, vi. [PubMed] Lonner BS. Emerging minimally invasive technologies for the management of scoliosis. Orthop Clin North Am. 2007;38:431-440. [PubMed]
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Kyphosis
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Kyphosis
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AKA
Scheuermann's disease; Roundback; Hunchback; Postural kyphosis
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Kyphosis
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is a curving of the spine that causes a bowing or rounding of the back, which leads to a hunchback or slouching posture.
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Kyphosis can occur at any age, although it is rare at birth. Adolescent kyphosis, also known as Scheuermann's disease, is caused by the wedging together of several bones of the spine (vertebrae) in a row. The cause of Scheuermann's disease is unknown. In adults, kyphosis can be caused by:
Degenerative diseases of the spine (such as arthritis or disk degeneration) Fractures caused by osteoporosis (osteoporotic compression fractures) Injury (trauma) Slipping of one vertebra forward on another (spondylolisthesis)
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congenital kyphosis
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Clinical photograph and MRI scan of a child with an abrupt congenital kyphosis in the thoracic spine. The MRI scan shows a posteriorly located hemivertebra causing impingement on the spinal cord. (Courtesy of Texas Scottish Rite Hospital for Children)
http://orthoinfo.aaos.org/figures/A00423F02.jpg
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Kyphosis can also be seen with scoliosis. Each cause has its own risk factors.
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Symptoms
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Difficulty breathing (in severe cases) Fatigue Mild back pain Round back appearance Tenderness and stiffness in the spine
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Dx exams
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Physical examination by a health care provider confirms the abnormal curve of the spine. The doctor will also look for any nervous system (neurological) changes (weakness, paralysis, or changes in sensation) below the curve.
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Dx exams
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Spine x-ray Pulmonary function tests (if kyphosis affects breathing) MRI (if there may be a tumor, infection, or neurological symptoms)
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Treatment
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Treatment
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Expectations (prognosis)
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Adolescents with Scheuermann's disease tend to do well even if they need surgery, and the disease stops once they stop growing. If the kyphosis is due to degenerative joint disease or multiple compression fractures, surgery is needed to correct the defect and improve pain.
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Complications
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Decreased lung capacity Disabling back pain Neurological symptoms including leg weakness or paralysis Round back deformity
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Prevention
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Treating and preventing osteoporosis can prevent many cases of kyphosis in the elderly. Early diagnosis and bracing of Scheuermann's disease can reduce the need for surgery, but there is no way to prevent the disease.
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References
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Spiegel DA, Hosalkar HS, Dormans JP. The spine. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 678. Freeman BL III. Scoliosis and kyphosis. In: Canale ST, Beaty JH, eds. Campbell's Operative Orthopaedics. 11th ed. Philadelphia, Pa: Mosby Elsevier; 2007:chap 38. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0 002220/
Maria Carmela L. Domocmat, RN, MSN
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Lordosis
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A small degree of both kyphotic and lordotic curvature is normal. Too much kyphotic curving causes round shoulders or hunched shoulders (Scheuermann's disease).
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Too much lordotic curving is called swayback (lordosis). Lordosis tends to make the buttocks appear more prominent. Children with significant lordosis will have a significant space beneath their lower back when lying on their back on a hard surface. If the lordotic curve is flexible (when the child bends forward the curve reverses itself), it is generally not a concern. If the curve does not move, medical evaluation and treatment are needed.
Maria Carmela L. Domocmat, RN, MSN
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Causes
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Achondroplasia is a disorder of bone growth that causes the most common type of dwarfism. Achondroplasia is one of a group of disorders called chondrodystrophies or osteochondrodysplasias. Achondroplasia may be inherited as an autosomal dominant trait, which means that if a child gets the defective gene from one parent, the child will have the disorder. If one parent has achondroplasia, the infant has a 50% chance of inheriting the disorder. If both parents have the condition, the infant's chances of being affected increase to 75%. However, most cases appear as spontaneous mutations. This means that two parents without achondroplasia may give birth to a baby with the condition.
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Spondylolisthesis is a condition in which a bone (vertebra) in the lower part of the spine slips out of the proper position onto the bone below it. In children, spondylolisthesis usually occurs between the fifth bone in the lower back (lumbar vertebra) and the first bone in the sacrum (pelvis) area. It is often due to a birth defect in that area of the spine or sudden injury (acute trauma). In adults, the most common cause is degenerative disease (such as arthritis). The slip usually occurs between the fourth and fifth lumbar vertebrae. Other causes of spondylolisthesis include bone diseases, traumatic fractures, and stress fractures (commonly seen in gymnasts). Certain sport activities, such as gymnastics, weight lifting, and football, put a great deal of stress on the bones in the lower back. They also require that the athlete constantly overstretch (hyperextend) the spine. This can lead to a stress fracture on one or both sides of the vertebra. A stress fracture can cause a spinal bone to become weak and shift out of place.
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Home Care
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If the back is flexible, lordosis is usually not treated and will not progress or cause problems.
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Call health care provider if notice that child has an exaggerated posture or a curve in the back. The condition should be evaluated to determine if there is a medical problem.
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physical exam. The child may be asked to bend forward, to the side, and to lie flat on a table so that the spine can be examined in a variety of positions.
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In some cases, particularly if the curve seems "fixed" (not bendable), the following or other diagnostic tests may be recommended:
Lumbosacral spine x-ray Spine x-ray Other tests to rule out suspected disorders causing the condition
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Treatment
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Specific treatment for lordosis will be determined by child's physician based on:
your child's age, overall health, and medical history the extent of the condition child's tolerance for specific medications, procedures, or therapies expectations for the course of the condition Paretns opinion or preference
http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/orthopaedics/ lordosis.html
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Treatment
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Treatment
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The goal of treatment is to stop the progression of the curve and prevent deformity. Management of lordosis will depend upon the cause of the lordosis. Simple exercises may be sufficient if lordosis is associated with poor posture. However, lordosis occurring as a result of a hip problem may be treated as a part of the hip problem.
http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/orthopaedics/ lordosis.html
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References
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Spiegel DA, Dormans JP. The spine.In: Kliegman RM,Behrman RE, Jenson HB, Stanton BF, eds.Nelson Textbook of Pediatrics.19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 671. http://www.lpch.org/DiseaseHealthInfo/HealthLibr ary/orthopaedics/lordosis.html
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http://www.oif.org/images/content/pagebuilder/94682.jpg
A person with OI has a 50% chance of passing on the gene and the disease to their children.
tissue
dominant or autosomal recessive and clinical findings of each type o Types I and IV more mild manifestations of disease; can persist into adulthood o Types II and III more severe and can result in fracture in utero, during the birthing process, or in early childhood; associated with high mortality rate
Type 1 osteogenesis
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most common. People who have type 1 disease generally reach normal height and have few obvious skeletal deformities. typically causes more fractures during childhood than in adulthood. Hearing loss is pronounced and begins early in childhood.
most rare and the most severe. produces numerous deformities of the skeleton often is fatal in infancy. abnormal collagen formation also profoundly affects the lungs, causing significant breathing problems.
more severe than type 1 but less severe than type 3. Fractures are most common before puberty. Hearing loss begins in early childhood and is often profound
produces obvious skeletal deformities. Fractures before birth are common; UTZ can detect them in the fetus. also affects the lungs and muscles. Hearing loss begins in early childhood and often becomes complete by adolescence.
http://www.beltina.org/health-dictionary/osteogenesis-imperfecta-typestreatment.html
s/s
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All people with OI have weak bones, which makes them susceptible to fractures. Persons with OI are usually below average height (short stature). However, the severity of the disease varies greatly
s/s
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s/s
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bone deformity
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blue sclerae
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http://www.thachers.org/images/Osteogenesis_imperfecta_blue_sclera.JPG
http://cnx.org/content/m15020/latest/Cases_40_41_42-pres1-1.jpg
http://jorthod.maneyjournals.org/content/vol30/issue4/images/large/Ki nd.f1c.jpeg
http://www.beltina.org/pics/osteogenesis_imperfecta.j pg
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This baby's extremities are positioned oddly because there have been multiple fractures due to osteogenesis imperfecta (OI). This disease leads to multiple fractures. The basic problem is a defect in the formation of type 1 collagen that forms bone matrix. There are several types of OI with different inheritance patterns. http://medgen.genetics.utah.edu/photographs/diseases/high/peri021.jpg
Dx:
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Treatment
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Physical activity
helps strengthen muscles and bone, which in turn minimizes fractures.
The most numerous fractures occur during childhood when the bones are growing and thus have lower mineral content. The risk for fracture is lifelong, however, and may increase in women after menopause when bone density naturally declines.
Maria Carmela L. Domocmat, RN, MSN
Treatment
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palliative steroids calcium vit C sodium fluoride PT casting bracing telescoping intramedullary rods to maintain mobility and promote ambulation o research ongoing Biphophonates
o o o o o o o o o
Expectations (prognosis)
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How well a person does depends on the type of OI they have. Type I, or mild OI, is the most common form. Persons with this type can live a normal lifespan. Type II is a severe form that is usually leads to death in the first year of life.
Type III is also called severe OI. Persons with this type have many fractures starting very early in life and can have severe bone deformities. Many become wheelchair bound and usually have a somewhat shortened life expectancy.
Expectations (prognosis)
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Type IV, or moderately severe OI, is similar to type I, although persons with type IV often need braces or crutches to walk. Life expectancy is normal or near normal. There are other types of OI, but they occur very infrequently and most are considered subtypes of the moderately severe form (type IV).
Complications
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Complications are largely based on the type of OI present. They are often directly related to the problems with weak bones and multiple fractures.
Hearing loss (common in type I and type III) Heart failure (type II) Respiratory problems and pneumonias due to chest wall deformities Spinal cord or brain stem problems Permanent deformity
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http://jorthod.maneyjournals.org/content/vol30/issue4/images/large/Ki nd.f1c.jpeg
Resources
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Ignatavicius and Workman (2006). Medical surgical nursing [5th ed]. Singapore: Elsevier. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0 002540/ http://www.beltina.org/healthdictionary/osteogenesis-imperfecta-typestreatment.html http://www.oif.org/site/PageServer http://www.umm.edu/ency/article/001573.htm