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2-28-08 Mrs.

Batton

Scoliosis
• Spinal deformity (usually curvature of the spine, ribs are asymmetrical)

• May be in one or more of three spinal planes

• May be congenital or develop in infancy, childhood, or early adolescence


(usually found in early adolescence)

• Don’t know the cause, might be genetic

• Must rule out intra-spinal conditions before diagnosis of scoliosis

• 10 degrees or less of a curve is a postural variation not scoliosis

• If curve is 20 degrees or less and non progressive – usually not treated

Types of Spinal Curvatures

• Kyphosis- increased convexity of the thoracic curve (hunchback)

• Lordosis- (pregnant women) exaggeration of lumbar spinal curve (swayback)

• Scoliosis- lateral curvature of the spine (most common)

• Thoracic- affects breathing

• Lumbar- back pain

• Thoracolumbar- combination of thoracic and lumbar

• Double helix- combination of all three

Diagnosis of Scoliosis

• Screenings with observation of asymmetry of shoulder height, scapular, or


flank shape or hip height

• Standing X-rays

Signs and symptoms

• Uneven shoulders

• Curve of the spine

• Uneven hips
• Slip is always showing on one side, one side of pants hangs down further

Treatment

• Regular clinical and x-ray evaluation

• Minor curve- watch x-rays for progression

• If it progresses- orthotic interventions (braces, exercises, stretching exercise)

• If it still progresses- surgical intervention (Spinal Fusion) major surgery

• Best managed by multidisciplinary team (RT, PT, surgical, etc)

• Treatment depends on magnitude of curve, what kind of curve it is, and


maturity of child and bones, underlying disease processes. The older you
are the less responsive you are to braces.

• Braces and exercises don’t do much good if the curve is greater than 40
degrees (they will have surgical intervention)

Bracing

• Most common types

• Must be worn 23 hours a day to do any good

• If the patient is growing they will have to be regularly checked and refitted,
or if they are gaining/ losing weight

o Boston brace (Wilmington, NY brace)

 Plastic from underarm all the way to the hips

 Chaffing is a problem, encourage to wear a cotton t-shirt


underneath it

o TLSO (thoracolumbosacral orthotic)

 Also used for other back surgeries

 Has a little more room for growing

o Milwaukee brace- only occasionally used in Scoliosis, it is mostly used


for kyphosis.

o Charleston nighttime brace- put on in the bed, very bulky, has to come
off before you get out of bed, because you can’t walk with it on.
2-28-08 Mrs. Batton

Surgical Management

• Only used for correction of severe curves

• Usually for curves of greater than 40 degrees and paralytic or congenital


curves that progress

• Considered for curves resulting in difficulty sitting, balance problems,


respiratory issues

• Surgery-Realignment and straightening with internal fixation and


instrumentation combined with bony fusion (arthrodesis) of the aligned spine

• Goal is to correct the curves and then have a solid back that is pain free and
the person is well balanced with good mobility

• Surgical systems

o Harrington system-rods, hooks, nuts, hooked thru with rods down the
side. Decorticate the back part of the vertebrae and put it across the
vertebrae to form the fusion. ALWAYS LOG ROLE THESE PATIENTS
POST OP- because you have to allow the bone time to harden or the
bone can disalign

o L-Rod Segmental Instrumentation (Luque Rod) L-shaped rods go down


the side, use posterior approach (go in thru the back itself) fuse donor
bone. Client can be up in a few days. No post-op immobilization. But
because of the positioning of the L-rods there is greater risk of nerve
damage

o Multi-hook instrumentation (Cortrel-Dubosset) combines the other two


systems, rods down both sides of the back, very frequently see an
anterior approach (abdominal incision) very complicated patients, a
lot of pain

o Anterior Instrumentation- may put in screws thru vertebral body,


almost always log rolled, will usually have a brace to get up. Provides
better visibility, can correct curve a little better than with posterior
approach.

o New rods have come out that can slide, so that younger people can
have the surgery, because old rods don’t grow with the patient.

o These patients tend to have balance issues until they get used to the
rods

Pre-op
• X-rays

• Pulmonary function studies

• ABG’s

• Routine lab

• Autologous blood donation

• Teaching (mobility, PCA pump, chest tube for anterior approaches, foley, NG
tube, etc.)

Post-op

• Wound, VS, circulation assessment

• Neurological function of the extremities

• Log roll

• Pain control

• Skin care

• NGT, usually, for paralytic ileus-assess bowel sounds

• Foley frequently used

• Physical Therapy- usually the day of surgery, get them up pretty quickly

• Multidisciplinary team

• Teaching

• Refer to support groups

Congenital Club foot


• The foot itself is bent, short tendons are lengthened, long tendons are
shortened

• A common deformity in which the foot is twisted out of its normal shape or
position

• Deformities are described according to the position of the ankle and foot

• Treatment starts at birth


2-28-08 Mrs. Batton

• 1 in 700 live births

• Mostly in boys

• Usually both feet are effected

• Can’t walk if it is not corrected

• May have other congenital problems

• Don’t know what causes it

• Seen more in multiple births

• Common variations

o Talipes vargus

 Heel turned in

o Talipes Valgus

 Heel and foot are turned out

o Talipes equines

 Heel is up toes are down

o Talipes calcaneus

 Foot is dorsoflexed

o TEV Talipes EquinoVargus- most common one you will see

• Classifications of Clubfoot

o Seen on ultrasound or at birth

o Positional also called Transitional, Mild or Postural (may be involved


with intrauterine crowding) may resolve itself by doing stretching
exercises, no bone involvement whatsoever (just soft tissue and
tendon are affected)

o Syndromic or teratologic- will have other congenital problems, usually


a significant club foot, almost always requires surgical intervention,
sometimes club foot goes back after corrected

o Congenital Idiopathic (True Clubfoot) bony involvement- bones grow


together and become fixed. Only correction is surgical intervention
and then casting

• Therapeutic Management

o Three Stages

 Correction of deformity

 Maintenance of the correction until normal muscle balance is


regained

 Follow up observation to avert possible recurrence of the


deformity

o Will cast in nursery, parents need to bring them back in a few days for
the first two weeks for a cast check, then every week or two. Usually
done for 2-3 months to see how much it has aligned. Be sure to get
guardians phone number in case they don’t show up to get rechecked

o Denis brown splint- can be taken on and off,

o If no alignment has occurred in the first three months, they will


probably have to do surgery- Between 6 months and 1 year of age

 Will have cast after surgery for 6-12 weeks, then will have PT
and OT to get them walking

• Nursing Care

o Teaching on how to manage the cast, etc.

o Cast care

o Skin care

Developmental Dysplasia of the Hip


(DDH)
• Describes a group of disorders related to abnormal development of the hip
2-28-08 Mrs. Batton

• Reflects a variety of hip abnormalities including shallow acetabulum,


subluxation, or dislocation.

• 10/1000 live births have some type of hip instability

• Involves left hip most of the time and mostly white females

Three Types

• Acetabur dysplasia (preluxation

o Mildest form, no subluxation or dislocation

o Acetabulum is just shallow, but the femoral head stays inside of it

• Subluxation

o Most common form

o Implies an incomplete dislocation of the hip

o Femoral head remains in contact with acetabulum

o Ligaments have been stretched so they don’t hold femur tight in the
acetabulum and causes pressure causing flattening of the acetabulum

• Dislocation

o Femoral head has lost all contact with acetabulum and is displaced
posteriorly, and anteriorly

o One leg will be shorter than the other

Prenatal factors influencing DDH

• Maternal hormone secretion

o Estrogen produces laxity of maternal pelvis at end of pregnancy and


also affects fetal joints

• Mechanical factors of intrauterine posture

o Higher incidence with breech presentations and c-sections

o Twins

o Large infants

Diagnostic Evaluation
• The earlier the diagnosis the better

• Best to diagnose and begin treatment prior to 2 months of age

• As a newborn it appears as a lax hip joint

• Don’t x-ray in babies under 4 months because it doesn’t show up well until
after 4 months

Signs and symptoms

• Asymmetry of gluteal and thigh folds

• Limited hip abduction, as seen in flexion

• Apparent shortening of femur, as indicated by knees in flexion

• Ortolani click- if infant is less than a month old

• Positive Trendelenburg sign or gait if child is weight bearing

o When they lift up leg it drops down

• Waddling gait

• Lordosis- because they are trying to compensate

Treatment

• Will have frequent checks to the doctor for the harness to be adjusted

• If diagnosed early (birth to 6 months) put them in Pavlik harness

o Keeps legs in flexion, pushes femur back in acetabulum and keeps it


there

o Worn continuously, parents shouldn’t adjust it

o Usually worn until 3-6 months of age or until they see improvement

o If still no stability put in hip spika cast

 Goes from waist to toes in order to maintain position needed to


correct problem

• If diagnosed between 6-18 months of age

o Gradual reduction by traction followed by Spica cast immobilization


2-28-08 Mrs. Batton

• If not diagnosed until they are Older child (greater than 4) really hard to treat
if over 6

o Difficult to manage since secondary adaptive changes complicate the


condition

o Open reduction required with casting and rehab

• If the pavlik harness works then they will have the Hewson brace- can be
taken on and off, may wear it for the first year or two

Nursing Care

• Assessment/ observation

• Teach parents

o Skin care

o Cast care

o How to apply and maintain the device

o Toy selection

o Age/developmentally appropriate activities

o Transportation of the child

Spina Bifida
• Neural tube defect-everything didn’t form like it needed to along the spinal
column

• Occurs when the neural tube (bone and meninges) fails to close thereby
producing defects of varying degrees

• May involve the entire length of the tube or only a small portion

Types of spina bifida

• Spina bifida occulta

o Defect is not visible externally

o Bone is not closed but no protrusion of contents

o Usually between L5-S1


o Might feel a soft, spongy spot

• Spina bifida cystic

o Can see defomity

o Miningocele

 No neural cord is out, just the sac and fluid

o Myelomeningocele

 Sac, fluid, and cord is outside inside of the sac

 Causes compression of the cord

Meningocele

• Midline defect through the osseous spine that encompasses the meninges
and spinal fluid but no neural elements

• Hernia protrusion of a saclike cyst filled with spinal fluid

• Not usually associated with neurological deficit

• Clinically, not usually termed spina bifida

Myelomeningocele

• Protrusion of meninges, spinal fluid, and nerves, through a defect in the


osseous spine- it is visual defect- you can see it

• Hernia protrusion of a saclike cyst containing meninges, spinal fluid and a


portion of the spinal cord with its nerves

• Neuro deficit is present in varying degrees

• Hydrocephalus is frequently present

Diagnosis

• Can determine some major neural tube defects prenatally

• Plan c-section if known in utero

• Ultrasound scanning of uterus

• Elevated alpha-fetoprotein (AFP) in amniotic fluid


2-28-08 Mrs. Batton

• Chorionic villus sampling (CVS)

o Done between 9-11 weeks

• Made on basis of clinical manifestations and examination of the meningeal


sac

• MRI

• CT

• Myelography

• Labs will be checked for organisms known to cause common, major


complications such as meningitis and UTI’s

Signs and symptoms

• Occulta

o Frequently no observable manifestations

o May be associatied with cutaneous manifestations

 Skin depression or dimple

 Port-wine angiomatous nevi

 Dark tufts of hair

 Soft, cutaneous lipomas

o May be neuromuscular disturbances

 Progressive disturbance of gait

• Cystic

o Sensory disturbance usually parallel motor dysfunction

o Below L2

 Flaccid, partial paralysis of lower extremities

 Overflow incontinence with constant dribbling

 Bowel incontinence

o Below S3
 no motor impairment

 maybe saddle anesthesia

o Joint deformities

 Kyphosis

 Club foot

 Hip dislocation

Myelomenigocele management

• Assessment of sac

• Keep pressure off of defect

• Prevent infection, assess for s/s of infection

• Measure head circumference

• Monitor I&O and for urinary retention

• Neuro check

• Avoid rectal temperature

• Family support

• Myelomeningocele should be closed early-usually within first 12-18 hours but


definitely before 72 hours to prevent infection

Post op

• Close observation for cerebrospinal fluid

• Prone position usually for awhile maybe side-lying or partial side-lying

• Limit latex exposure

• Teach family

• Provide referrals

Common Medications

• Ditropan (oxybutynin chloride) smooth muscle relaxant, help with bladder


storage
2-28-08 Mrs. Batton

• Detrol (tolterodine) helps with stress incontinence

• Various antibiotics for UTI

• Stool softeners

Prevention

• Folic acid 0.4mg/day should be given to all women of child bearing age

• For women with history of pregnancy with NTD’s increase intake to 4mg Folic
Acid/day under supervision of physician beginning 1 month before a planned
pregnancy and during the first trimester

Cerebral Palsy
• A nonspecific term applied to disorders characterized by early onset of
impaired movement and posture

• It is non progressive

• Abnormal muscle tone and coordination are primary disturbances

• Most common permanently physical disability of childhood

• Incidence 1.5-3 of every 1000 live births

• Premature delivery is single most important risk factor

Causes

• Brain abnormality?

• Prenatal problems, especially birth hypoxia?

• Anoxia secondary to other causative mechanisms?

• 24% of cases have no cause determined

Signs and symptoms

• Spastic

o Have problems with fine and gross motor skills

o The more you try to do something the more spastic you become

• Dyskinetic/athetoid
o Problems with movement and walking

o Almost wormlike movement when walking

o Sometimes have problems with speech and drooling

o May be difficult for them to eat and swallow

o May have jerky movements, that get worse under stress

o Tends to have more of the extra movements during adolescence

• Ataxic

o Have very wide gait, trying to maintain balance

o Have problems typing

• Mixed type/dystonic

o Combination

Diagnosis

• Neruo exam and history are primary modalities for diagnosis, assess motor
involvement, check for brain tumor or other progressive disease, not
noticeable in infants usually

• Observation of any of the classifications of CP previously discussed

• EEG

• Tomography

• Screening for metabolic defects

• Electrolytes

Therapeutic management

• Goal of early recognition

• Multidisciplinary approach of treatment

• Treatment is very individualized

Aims of treatment

• Establish mobile, communication, and self help


2-28-08 Mrs. Batton

• Gain optimal appearance and intergration of motor functions

• To correct associated defects as effectively as possible

• To provide individualized educational opportunities

• To promote socialization experiences with affected and no affected persons

• Physical therapy is one of the most frequently used in conservative


treatments

• Exercise are individualized

• Exercises include

o Stretching

o Passive, active, and resisted movements applied

Therapeutic Devices

• AFOs (ankle-foot orthoses) braces

o Help prevent or reduce deformities

o Increase energy efficiency of gait

o Control alignment

• Other mobilization devices

o Wheeled scooter boards

o Wheeled go-carts

o Special assistive devices

• Technical aids

• Therapeutic toys/games

Surgical Management

• Surgery is only for children who don’t respond to other things

• Surgery used to promote function, not for cosmetic purposes

• Tendon lengthening procedures


• Release of spastic wrist flexor muscles

• Correction of hip and adductor muscle spasticity or contracture formation

Neurosurgical management

• Selective dorsal rhizotomy

o A neurosurgical intervention in which dorsal column sensor rootlets


that have abnormal electrical stimulation are selectively cut

o Requires PT

• Baclofen pump- into abdomen, slow steady amount of balcofen released into
spinal cord, that will prevent side effects of oral administration

o Will have to have pump refilled every 4-6 wks in doctors office

Pharmacology

• Anti-anxiety agents

• Muscle relaxants

• Valium

• Botox- to spastic areas (botulinum toxin)

• Local nerve block

Prognosis

• Moderate disability

o Survival rate about the same until age 20

• Severe disability – not as good due to other disabilities

Muscular Dystrophy
• Largest group of muscle disease in childhood

• All hereditary and all are progressive, some are slow progression and some
are rapid progression

• All will cause a characteristic pattern of weakness and loss of muscle strength
2-28-08 Mrs. Batton

Pathology

• Cause is unknown but appears to be related to a metabolic disturbance


unrelated to the nervous system

• Serum creatinine phosphokinase is consistently elevated and assists in


diagnosis and early detection

Types

• Pseydohypertrophic (Duchenne) Dystrophy

• Facioscapulohumeral Dystrophy

• Limb-girdle dystrophy

Duchenne Muscular Dystrophy

• Most sever and most common

• Males get it

• Onset is usually at about 3 years

• Usually hips, shoulder, quadriceps

• Rapid progression

• Frequent mental retardation

• Elevated serum enzymes (CPK, LDH, SGOT)

• AST levels are extremely elevated 2 years prior to clinical weakness

• Early death due to rapid progression- child not ambulatory by the time they
are 12- late teens on a ventilator

Facioscaulohymeral

• Don’t notice until early adolescence

• Usually shoulder, neck, and face, trouble raising arms over the head, face
won’t work like it should

• Slowly progressive

• Lifespan is usually unaffected

• Occasional mild retardation


Limb-girdle MD

• Shoulder pelvic muscles

• Slow progression

• Onset late childhood, adolescents

Diagnosis

• Serum enzymes

• Muscle biopsy shows degeneration of muscle fibers

• EMG electromyography

Treatment

• No effective treatment

• ROM exercises

• Surgery to release contracture

• Bracing

• Primary goal is to maintain muscle function

• Assistance with ADL’s

• Genetic counseling

• Referrals

Nursing

• Major emphasis is coping

• Provide assistance with ADL’s

• Encourage them to be as independent as possible