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Musculoskeletal Disorders Part 5:
Spinal Column Deformities




 MARIA CARMELA L. DOMOCMAT, RN, MSN
 INSTRUCTOR
 SCHOOL OF NURSING
 NORTHERN LUZON ADVENTIST COLLEGE
 ARTACHO, SISON, PANGASINAN
Overview
                                        2

   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)
   Part 6 : Disorders of foot
   Part 7: Sports Injuries

Maria Carmela L. Domocmat, RN, MSN                    3/5/2012
Spinal column deformities
                                     3

      Scoliosis
      Kyphosis
      Lordosis
      Osteogenesis imperfecta (OI)




Maria Carmela L. Domocmat, RN, MSN          3/5/2012
Scoliosis
Scoliosis
                                         5

   AKA
      Spinal curvature
      Infantile scoliosis
      Juvenile scoliosis




Maria Carmela L. Domocmat, RN, MSN               3/5/2012
Scoliosis
                                         7

   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.”




Maria Carmela L. Domocmat, RN, MSN                  3/5/2012
Causes, incidence, and risk factors

idiopathic scoliosis
  most common type. It is grouped by age.
  In children age 3 and younger--infantile scoliosis.
  In kids age 4 - 10--juvenile scoliosis.
  In older kids age 11 – 18 --adolescent scoliosis.
  most often affects girls.
  Curving generally gets worse during a growth spurt.
Congenital scoliosis
  This type of scoliosis is present at birth. It occurs when the baby’s
  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
                                        12

   Usually there are no symptoms. But symptoms can
   include:
      Backache or low-back pain
      Tired feeling in the spine after sitting or standing for a long
      time
      Uneven hips or shoulders (one shoulder may be higher than
      the other)
      Spine curves more to one side
      Kyphoscoliosis is another type of abnormal curving of the
      spine.



Maria Carmela L. Domocmat, RN, MSN                                3/5/2012
Dx exams
                                        13

    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




Maria Carmela L. Domocmat, RN, MSN                   3/5/2012
Scoliometer screening
                                     14




Maria Carmela L. Domocmat, RN, MSN                 3/5/2012
Treatment
                                         15

   Treatment depends on many things:
      The cause of scoliosis
      Where the curve is in your spine
      How big the curve is
      If body is still growing
   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.

Maria Carmela L. Domocmat, RN, MSN                                  3/5/2012
16

   Scoliosis surgery involves correcting the curve as
   much as possible.
      The spine bones are held in place with one or two metal rods,
      which are held down with hooks and screws until the bone
      heals together.
      Surgery may be done with a cut through the back, belly area, or
      beneath the ribs.
      After surgery, you may need to wear a brace for a little while to
      keep the spine still.
      if the spine curve is severe or getting worse very quickly. The
      surgeon may want to wait until all your bones stop growing,
      but this isn’t always possible.

Maria Carmela L. Domocmat, RN, MSN                               3/5/2012
17

   Scoliosis treatment may also include:
      Emotional support. Some kids, especially teens, may be self-
      conscious when using a back brace
      Physical therapy and other specialists to help explain the
      treatments and make sure the brace fits correctly.




Maria Carmela L. Domocmat, RN, MSN                             3/5/2012
Expectations (prognosis)
                                     18

   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.


Maria Carmela L. Domocmat, RN, MSN                                 3/5/2012
Complications
                                       19

   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



Maria Carmela L. Domocmat, RN, MSN                  3/5/2012
Prevention
                                         20

   Routine scoliosis screening is now done in middle
   and junior high schools.
   Screening has helped detect early scoliosis in many
   kids.




Maria Carmela L. Domocmat, RN, MSN                 3/5/2012
References
                                         26

   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]



Maria Carmela L. Domocmat, RN, MSN                3/5/2012
Kyphosis
                                        27




Maria Carmela L. Domocmat, RN, MSN              3/5/2012
Kyphosis
                                        28

   AKA
      Scheuermann's disease;
      Roundback;
      Hunchback;
      Postural kyphosis




Maria Carmela L. Domocmat, RN, MSN              3/5/2012
Kyphosis
                                        29

   is a curving of the spine that causes a bowing or
   rounding of the back, which leads to a hunchback or
   slouching posture.




Maria Carmela L. Domocmat, RN, MSN                3/5/2012
Causes, incidence, and risk factors
                                     30

   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)


Maria Carmela L. Domocmat, RN, MSN                                  3/5/2012
31

   Other causes of kyphosis include:
      Certain endocrine diseases
      Connective tissue disorders
      Infection (such as tuberculosis)
      Muscular dystrophy
      Neurofibromatosis
      Paget's disease
      Polio
      Spina bifida
      Tumors


Maria Carmela L. Domocmat, RN, MSN        3/5/2012
congenital kyphosis
                                                  32
   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        3/5/2012
33

   Kyphosis can also be seen with scoliosis. Each cause
   has its own risk factors.




Maria Carmela L. Domocmat, RN, MSN                 3/5/2012
Symptoms
                                        34

   Difficulty breathing (in severe cases)
   Fatigue
   Mild back pain
   Round back appearance
   Tenderness and stiffness in the spine




Maria Carmela L. Domocmat, RN, MSN              3/5/2012
Dx exams
                                        35

   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.




Maria Carmela L. Domocmat, RN, MSN                3/5/2012
Dx exams
                                        36

   Spine x-ray
   Pulmonary function tests (if kyphosis affects
   breathing)
   MRI (if there may be a tumor, infection, or
   neurological symptoms)




Maria Carmela L. Domocmat, RN, MSN                 3/5/2012
Treatment
                                         37

   Treatment depends on the cause of the disorder:
      Congenital kyphosis requires corrective surgery at an early age.
      Scheuermann's disease is treated with a brace and physical
      therapy. Occasionally surgery is needed for large (greater than
      60 degrees), painful curves.




Maria Carmela L. Domocmat, RN, MSN                              3/5/2012
Treatment
                                         38

   Treatment depends on the cause of the disorder:
      Multiple compression fractures from osteoporosis can be left
      alone if there are no nervous system problems or pain.
      However, the osteoporosis needs to be treated to help prevent
      future fractures. For debilitating deformity or pain, surgery is
      an option.
      Kyphosis caused by infection or tumor needs to be treated
      more aggressively, often with surgery and medications.
      Treatment for other types of kyphosis depends on the cause.
      Surgery may be necessary if neurological symptoms or
      persistent pain develop.



Maria Carmela L. Domocmat, RN, MSN                               3/5/2012
Expectations (prognosis)
                                     39

   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.




Maria Carmela L. Domocmat, RN, MSN                   3/5/2012
Complications
                                       40

   Decreased lung capacity
   Disabling back pain
   Neurological symptoms including leg weakness or
   paralysis
   Round back deformity




Maria Carmela L. Domocmat, RN, MSN               3/5/2012
Prevention
                                         41

   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.




Maria Carmela L. Domocmat, RN, MSN                  3/5/2012
References
                                         42

   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                3/5/2012
Lordosis
                                        43




Maria Carmela L. Domocmat, RN, MSN              3/5/2012
44

   Lordosis is an increased curving of the spine.
   AKA: Swayback




Maria Carmela L. Domocmat, RN, MSN                  3/5/2012
45

   The spine has three types of curves:
      Kyphotic curves refer to the outward curve of the thoracic
      spine (at the level of the ribs).
      Lordotic curves refer to the inward curve of the lumbar spine
      (just above the buttocks).
      Scoliotic curving is a sideways curvature of the spine and is
      always abnormal.




Maria Carmela L. Domocmat, RN, MSN                             3/5/2012
46

   A small degree of both kyphotic and lordotic
   curvature is normal.
   Too much kyphotic curving causes round shoulders
   or hunched shoulders (Scheuermann's disease).




Maria Carmela L. Domocmat, RN, MSN              3/5/2012
47

   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                     3/5/2012
Causes
                                       48

   Achondroplasia
   Benign juvenile lordosis (not medically significant)
   Spondylolisthesis




Maria Carmela L. Domocmat, RN, MSN                  3/5/2012
49

   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.

Maria Carmela L. Domocmat, RN, MSN                           3/5/2012
50

   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.




Maria Carmela L. Domocmat, RN, MSN                                        3/5/2012
Home Care
                                         51

   If the back is flexible, lordosis is usually not treated
   and will not progress or cause problems.




Maria Carmela L. Domocmat, RN, MSN                      3/5/2012
52

   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.




Maria Carmela L. Domocmat, RN, MSN                    3/5/2012
53

   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.




Maria Carmela L. Domocmat, RN, MSN                     3/5/2012
54

   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




Maria Carmela L. Domocmat, RN, MSN                              3/5/2012
Treatment
                                                           55

   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
      Paretn’s opinion or preference


http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/orthopaedics/
lordosis.html



Maria Carmela L. Domocmat, RN, MSN                                  3/5/2012
Treatment
                                         56

   Observation
   physical therapy
   bracing.
   most severe cases require surgery




Maria Carmela L. Domocmat, RN, MSN               3/5/2012
Treatment
                                                           57

   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



Maria Carmela L. Domocmat, RN, MSN                                  3/5/2012
58




Maria Carmela L. Domocmat, RN, MSN        3/5/2012
References
                                         59

   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




Maria Carmela L. Domocmat, RN, MSN                 3/5/2012
Osteogenesis imperfecta (OI)
61




                        http://www.oif.org/images/content/pagebuilder/94682.jpg



Maria Carmela L. Domocmat, RN, MSN
Osteogenesis62
                                   imperfecta (OI)
• AKA: Brittle bone disease
• rare genetic disorder in which bone are fragile and
  fracture easily resulting in bone deformity
• an autosomal dominant disease
   •   A person with OI has a 50% chance of passing on the gene and the
       disease to their children.

• involves errors in synthesis of collagen, a connective
    tissue


Maria Carmela L. Domocmat, RN, MSN
Types according to Silence classification system
                                     63

  o has four types based on inheritance as autosomal
    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


Maria Carmela L. Domocmat, RN, MSN
Type 1 osteogenesis
                                     64

    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.



Maria Carmela L. Domocmat, RN, MSN
Type 2 osteogenesis imperfecta
                                     65

    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.




Maria Carmela L. Domocmat, RN, MSN
Type 4 osteogenesis imperfecta
                                     66

   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




Maria Carmela L. Domocmat, RN, MSN
Type 3 osteogenesis imperfecta
                                                                   67

   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-types-
  treatment.html




Maria Carmela L. Domocmat, RN, MSN
s/s
                                     68

   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




Maria Carmela L. Domocmat, RN, MSN
s/s
                                      69

   The classic symptoms include:
      osteoporosis
      history of multiple fractures
      bone deformity
      poor skeletal development
      soft brownish teeth
      hearing loss
      Blue tint to the whites of their eyes (blue sclera)
      Because type I collagen is also found in ligaments, persons
      with OI often have loose joints (hypermobility) and flat feet.



Maria Carmela L. Domocmat, RN, MSN
s/s
                                     70

   Symptoms of more severe forms of OI may include:
      Bowed legs and arms
      Kyphosis
      Scoliosis (S-curve spine)




Maria Carmela L. Domocmat, RN, MSN
bone deformity
                                           71




Maria Carmela L. Domocmat, RN, MSN
blue sclerae
                                                              72




        http://www.thachers.org/images/Osteogenesis_imperfecta_blue_sclera.JPG


                                                          http://cnx.org/content/m15020/latest/Cases_40_41_42-pres1-1.jpg




Maria Carmela L. Domocmat, RN, MSN
soft brownish teeth; bone deformity
                                                               73




http://jorthod.maneyjournals.org/content/vol30/issue4/images/large/Ki
nd.f1c.jpeg




http://www.beltina.org/pics/osteogenesis_imperfecta.j
                                                  pg


Maria Carmela L. Domocmat, RN, MSN
74




      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


Maria Carmela L. Domocmat, RN, MSN
Dx:
                                      75

   serum alkaline phosphatase – increased




Maria Carmela L. Domocmat, RN, MSN
Treatment
                                         76

   Treatment for fracture - generally conservative
      targeting a balance between immobilizing the fracture long enough
      for it to heal and allowing normal muscle function as quickly as
      possible.
   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
                                         77

  o palliative
  o steroids
  o calcium
  o vit C
  o sodium fluoride
  o PT
  o casting
  o bracing
  o telescoping intramedullary rods – to maintain mobility and
    promote ambulation
  o research ongoing – Biphophonates



Maria Carmela L. Domocmat, RN, MSN
Expectations (prognosis)
                                     78

   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.


Maria Carmela L. Domocmat, RN, MSN
Expectations (prognosis)
                                     79

   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).




Maria Carmela L. Domocmat, RN, MSN
Complications
                                          80

   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


Maria Carmela L. Domocmat, RN, MSN
81




           http://jorthod.maneyjournals.org/content/vol30/issue4/images/large/Ki
           nd.f1c.jpeg




Maria Carmela L. Domocmat, RN, MSN
Resources
                                         82

   Ignatavicius and Workman (2006). Medical surgical
   nursing [5th ed]. Singapore: Elsevier.
   http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0
   002540/
   http://www.beltina.org/health-
   dictionary/osteogenesis-imperfecta-types-
   treatment.html
   http://www.oif.org/site/PageServer
   http://www.umm.edu/ency/article/001573.htm


Maria Carmela L. Domocmat, RN, MSN

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Spinal Column Deformities: Scoliosis, Kyphosis, Lordosis & Osteogenesis Imperfecta

  • 1. Musculoskeletal Disorders Part 5: Spinal Column Deformities MARIA CARMELA L. DOMOCMAT, RN, MSN INSTRUCTOR SCHOOL OF NURSING NORTHERN LUZON ADVENTIST COLLEGE ARTACHO, SISON, PANGASINAN
  • 2. Overview 2 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) Part 6 : Disorders of foot Part 7: Sports Injuries Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 3. Spinal column deformities 3 Scoliosis Kyphosis Lordosis Osteogenesis imperfecta (OI) Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 5. Scoliosis 5 AKA Spinal curvature Infantile scoliosis Juvenile scoliosis Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 6.
  • 7. Scoliosis 7 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.” Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 8.
  • 9.
  • 10.
  • 11. Causes, incidence, and risk factors idiopathic scoliosis most common type. It is grouped by age. In children age 3 and younger--infantile scoliosis. In kids age 4 - 10--juvenile scoliosis. In older kids age 11 – 18 --adolescent scoliosis. most often affects girls. Curving generally gets worse during a growth spurt. Congenital scoliosis This type of scoliosis is present at birth. It occurs when the baby’s 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.
  • 12. Symptoms 12 Usually there are no symptoms. But symptoms can include: Backache or low-back pain Tired feeling in the spine after sitting or standing for a long time Uneven hips or shoulders (one shoulder may be higher than the other) Spine curves more to one side Kyphoscoliosis is another type of abnormal curving of the spine. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 13. Dx exams 13 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 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 14. Scoliometer screening 14 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 15. Treatment 15 Treatment depends on many things: The cause of scoliosis Where the curve is in your spine How big the curve is If body is still growing 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 16. 16 Scoliosis surgery involves correcting the curve as much as possible. The spine bones are held in place with one or two metal rods, which are held down with hooks and screws until the bone heals together. Surgery may be done with a cut through the back, belly area, or beneath the ribs. After surgery, you may need to wear a brace for a little while to keep the spine still. if the spine curve is severe or getting worse very quickly. The surgeon may want to wait until all your bones stop growing, but this isn’t always possible. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 17. 17 Scoliosis treatment may also include: Emotional support. Some kids, especially teens, may be self- conscious when using a back brace Physical therapy and other specialists to help explain the treatments and make sure the brace fits correctly. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 18. Expectations (prognosis) 18 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 19. Complications 19 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 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 20. Prevention 20 Routine scoliosis screening is now done in middle and junior high schools. Screening has helped detect early scoliosis in many kids. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 21.
  • 22.
  • 23.
  • 24.
  • 25.
  • 26. References 26 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] Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 27. Kyphosis 27 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 28. Kyphosis 28 AKA Scheuermann's disease; Roundback; Hunchback; Postural kyphosis Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 29. Kyphosis 29 is a curving of the spine that causes a bowing or rounding of the back, which leads to a hunchback or slouching posture. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 30. Causes, incidence, and risk factors 30 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) Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 31. 31 Other causes of kyphosis include: Certain endocrine diseases Connective tissue disorders Infection (such as tuberculosis) Muscular dystrophy Neurofibromatosis Paget's disease Polio Spina bifida Tumors Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 32. congenital kyphosis 32 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 3/5/2012
  • 33. 33 Kyphosis can also be seen with scoliosis. Each cause has its own risk factors. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 34. Symptoms 34 Difficulty breathing (in severe cases) Fatigue Mild back pain Round back appearance Tenderness and stiffness in the spine Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 35. Dx exams 35 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 36. Dx exams 36 Spine x-ray Pulmonary function tests (if kyphosis affects breathing) MRI (if there may be a tumor, infection, or neurological symptoms) Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 37. Treatment 37 Treatment depends on the cause of the disorder: Congenital kyphosis requires corrective surgery at an early age. Scheuermann's disease is treated with a brace and physical therapy. Occasionally surgery is needed for large (greater than 60 degrees), painful curves. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 38. Treatment 38 Treatment depends on the cause of the disorder: Multiple compression fractures from osteoporosis can be left alone if there are no nervous system problems or pain. However, the osteoporosis needs to be treated to help prevent future fractures. For debilitating deformity or pain, surgery is an option. Kyphosis caused by infection or tumor needs to be treated more aggressively, often with surgery and medications. Treatment for other types of kyphosis depends on the cause. Surgery may be necessary if neurological symptoms or persistent pain develop. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 39. Expectations (prognosis) 39 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 40. Complications 40 Decreased lung capacity Disabling back pain Neurological symptoms including leg weakness or paralysis Round back deformity Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 41. Prevention 41 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 42. References 42 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 3/5/2012
  • 43. Lordosis 43 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 44. 44 Lordosis is an increased curving of the spine. AKA: Swayback Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 45. 45 The spine has three types of curves: Kyphotic curves refer to the outward curve of the thoracic spine (at the level of the ribs). Lordotic curves refer to the inward curve of the lumbar spine (just above the buttocks). Scoliotic curving is a sideways curvature of the spine and is always abnormal. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 46. 46 A small degree of both kyphotic and lordotic curvature is normal. Too much kyphotic curving causes round shoulders or hunched shoulders (Scheuermann's disease). Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 47. 47 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 3/5/2012
  • 48. Causes 48 Achondroplasia Benign juvenile lordosis (not medically significant) Spondylolisthesis Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 49. 49 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 50. 50 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 51. Home Care 51 If the back is flexible, lordosis is usually not treated and will not progress or cause problems. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 52. 52 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 53. 53 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. Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 54. 54 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 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 55. Treatment 55 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 Paretn’s opinion or preference http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/orthopaedics/ lordosis.html Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 56. Treatment 56 Observation physical therapy bracing. most severe cases require surgery Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 57. Treatment 57 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 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 58. 58 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 59. References 59 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 Maria Carmela L. Domocmat, RN, MSN 3/5/2012
  • 61. 61 http://www.oif.org/images/content/pagebuilder/94682.jpg Maria Carmela L. Domocmat, RN, MSN
  • 62. Osteogenesis62 imperfecta (OI) • AKA: Brittle bone disease • rare genetic disorder in which bone are fragile and fracture easily resulting in bone deformity • an autosomal dominant disease • A person with OI has a 50% chance of passing on the gene and the disease to their children. • involves errors in synthesis of collagen, a connective tissue Maria Carmela L. Domocmat, RN, MSN
  • 63. Types according to Silence classification system 63 o has four types based on inheritance as autosomal 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 Maria Carmela L. Domocmat, RN, MSN
  • 64. Type 1 osteogenesis 64 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. Maria Carmela L. Domocmat, RN, MSN
  • 65. Type 2 osteogenesis imperfecta 65 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. Maria Carmela L. Domocmat, RN, MSN
  • 66. Type 4 osteogenesis imperfecta 66 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 Maria Carmela L. Domocmat, RN, MSN
  • 67. Type 3 osteogenesis imperfecta 67 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-types- treatment.html Maria Carmela L. Domocmat, RN, MSN
  • 68. s/s 68 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 Maria Carmela L. Domocmat, RN, MSN
  • 69. s/s 69 The classic symptoms include: osteoporosis history of multiple fractures bone deformity poor skeletal development soft brownish teeth hearing loss Blue tint to the whites of their eyes (blue sclera) Because type I collagen is also found in ligaments, persons with OI often have loose joints (hypermobility) and flat feet. Maria Carmela L. Domocmat, RN, MSN
  • 70. s/s 70 Symptoms of more severe forms of OI may include: Bowed legs and arms Kyphosis Scoliosis (S-curve spine) Maria Carmela L. Domocmat, RN, MSN
  • 71. bone deformity 71 Maria Carmela L. Domocmat, RN, MSN
  • 72. blue sclerae 72 http://www.thachers.org/images/Osteogenesis_imperfecta_blue_sclera.JPG http://cnx.org/content/m15020/latest/Cases_40_41_42-pres1-1.jpg Maria Carmela L. Domocmat, RN, MSN
  • 73. soft brownish teeth; bone deformity 73 http://jorthod.maneyjournals.org/content/vol30/issue4/images/large/Ki nd.f1c.jpeg http://www.beltina.org/pics/osteogenesis_imperfecta.j pg Maria Carmela L. Domocmat, RN, MSN
  • 74. 74 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 Maria Carmela L. Domocmat, RN, MSN
  • 75. Dx: 75 serum alkaline phosphatase – increased Maria Carmela L. Domocmat, RN, MSN
  • 76. Treatment 76 Treatment for fracture - generally conservative targeting a balance between immobilizing the fracture long enough for it to heal and allowing normal muscle function as quickly as possible. 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
  • 77. Treatment 77 o palliative o steroids o calcium o vit C o sodium fluoride o PT o casting o bracing o telescoping intramedullary rods – to maintain mobility and promote ambulation o research ongoing – Biphophonates Maria Carmela L. Domocmat, RN, MSN
  • 78. Expectations (prognosis) 78 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. Maria Carmela L. Domocmat, RN, MSN
  • 79. Expectations (prognosis) 79 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). Maria Carmela L. Domocmat, RN, MSN
  • 80. Complications 80 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 Maria Carmela L. Domocmat, RN, MSN
  • 81. 81 http://jorthod.maneyjournals.org/content/vol30/issue4/images/large/Ki nd.f1c.jpeg Maria Carmela L. Domocmat, RN, MSN
  • 82. Resources 82 Ignatavicius and Workman (2006). Medical surgical nursing [5th ed]. Singapore: Elsevier. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0 002540/ http://www.beltina.org/health- dictionary/osteogenesis-imperfecta-types- treatment.html http://www.oif.org/site/PageServer http://www.umm.edu/ency/article/001573.htm Maria Carmela L. Domocmat, RN, MSN