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Pediatrics > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Spina Bifida literally means what?

A

“Split spine”

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2
Q

MOST COMMON permanently disabling birth defect in US

A

Spina Bifida

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3
Q

Spina Bifida defined

A

Defective dev. of any part (esp lower segments) of SC

most common permanently disabling birth defect in US**

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4
Q

4 Main types of Spina Bifida

A
  1. Spina Bifida occulta
  2. Closed neural tube defects
  3. Meningocele
  4. Myelomeningocele
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5
Q

MILDEST and MOST COMMON FORM of Spina Bifida

A

Spina Bifida Occulta

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6
Q

1.Spina Bifida Occulta

A
  • mildest/most common
  • one or more vertebrae malformed
  • Occulta=”hidden”
  • layer of skin over defect
  • rarely causes disability or sx’s
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7
Q
  1. Closed Neural Tube Defects
A
  • Diverse group of defects→ spinal cord marked by malformations of fat, bone, or meninges
  • few or no sx’s
  • Malformations can cause incomp. paralysis w/ urinary and B&B dysf.***
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8
Q
  1. Meningocele
A
  • Defect where spinal fluid/meninges protrude thru abnorm vertebral opening
  • SC unaffected, may or may not be covered w/ skin
  • Sx’s vary from NO motor dysf/sx’s all the way to comp paralysis w/ B&B dysf***
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9
Q

MOST SEVERE FORM OF SPINA BIFIDA

A

MYELOMENINGOCELE

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10
Q
  1. Myelomeningocele
A
  • SC/neural elements exposed thru opening in spine
  • MOST SEVERE FORM
  • Results→ partial OR complete paralysis of parts of body below spinal opening
    • like SCI
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11
Q

Spina Bifida Types pics

A

see pics

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12
Q

Spinal dysraphism vs Meningocele vs Myelomeningocele

A

see pics

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13
Q

Etiology of Spina Bifida

A
  • Abnorm growth/dev. of SC or spinal column
  • MM cause unknown
  • Attributed to few factors:
    • genetics
    • teratogens→ alcohol/anticonvulsant exposure in utero
    • Nutrition deficiencies→ dec folic acid
      • daily supp 3mos prior to conception
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14
Q

Incidence/Prevalence

A
  • .4-.9/1000 births
  • Better technology and prenatal screening==> prevents early death from birth trauma to neural sac
  • Perinatal mgmt:
    • option to terminate, better options thru c-section, repair in-utero of neural sac
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15
Q

Spina Bifida: Overview

A
  • M + S loss varies
  • Any lesion== risk for loss of function over time
  • Paralysis may not be observed until later in life:
    • abnorm tissue growth (dysplasia) == pressure on nerves
    • progress. neuro impairment via tethering of SC
      • ==> growth spurts: SC does not lengthen @ same rate
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16
Q

PRIMARY spina bifida clinical impairment:

A

Motor paralysis

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17
Q

Motor paralysis ===

A

PRIMARY spina bifida clinical impairment

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18
Q

Spina Bifida Clinical impairments:

A
  • Primary=> Motor Paralysis***
  • MSK deforms, OP, sensory defs, hydrocephalus, cog/lang delays, latex allergy, upper limb dyscoord, CN palsies, spasticity, progress. neuro involvement, SZs, neurogenic B&B, skin breakdown, obesity
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19
Q

Motor Paralysis→ Primary clinical impairment***

A
  • Paraplegia from SC malform or abnorm
    • main deficit in SB
  • Motor lvl= lowest intact functional segment
  • Lesions mimic 1 of 3 patterns:
    • Comp SC transection
    • Incomp. lesion
    • Skip lesions→ zones of preservation
  • Grading→ International Myelodysplasia Study Group Criteria for Assigning Motor Lvls OR ASIA scale
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20
Q

Motor Paralysis: Common Lvl Characteristics→

know your myotomes!!!

Thoracic Lvl

A
  • Full innervation→ neck, upper limb, shoulder girdle, and trunk
  • NO VOLITIONAL LE MVMTS
  • Higher T/S lesion→ diff sitting upright, transfers, decd respiratory status
    • req aides to assist in all ADLs
  • Lower T/S lesion→ able to sit, IND w/ transfers, attempt amb w/ aides, likely use w/c for primary loco.
  • T/S lesions→ lower cog, req supervision later in life, do not maint. job
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21
Q

Motor Paralysis: Common Lvl Characteristics→

HIGH Lumbar (L1-L2)

A
  • Weak hips, hip flex and ADD contractures
  • SHORT dist amb w/ KAFOs or RGOs and UE support feasible
  • W/C for most mob.
  • 50% achieve IND living, rarely main. job
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22
Q

Motor Paralysis: Common Lvl Characteristics→

L3

A
  • STRONG hip flex/ADD, WEAK hip rot and some antigravity knee EXT
  • 3/5 MMT for quads→ KAFOs and UE support for amb.
  • Amb short comm. dist’s w/ WC for long dist.
  • 60% achieve IND living, few maint. job
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23
Q

Motor Paralysis: Common Lvl Characteristics→

L4

*remember everything BELOW→ so L4 still intact

A
  • Antigravity knee flex, grade 4 ankle DF w/ INV
    • medial HS or tib ant. has at least 3/5
  • knee EXT usually strong
  • Functional amb. w/ AFO or crutches→ may do amb train w/ KAFOs and walkers
  • Living/employment similar to L3
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24
Q

Motor Paralysis: Common Lvl Characteristics→

L5

A
  • Lateral HS 3/5, grade 2 glute min/med, grade 3 post tib, grade 4 peroneus tertius
  • Antigravity knee flex+ weak hip EXT, weak ABDs, PFs, and toes, → gluteal lurch during gait
  • Ambs w/out orthotics→ but they help maint alignment defs and sub for lack of push-off
  • AD recommended for comm dist amb
    • goals→ dec gait devs, improve eff, safety and align.
  • 80% IND living, 30% employed full time
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25
Q

Motor Paralysis: Common Lvl Characteristics→

S1

A
  • Mm function present thru S1:
    • Gastroc/soleus= 2/5
    • glute med/max= 2/5
    • improved hip stability, amb w/out aides or AD, mild-mod gluteal lurch w/ mild push off weakness w/ stairs/running
    • MAYBE AFOs→ foot deforms control/align.
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26
Q

Motor Paralysis: Common Lvl Characteristics→

S2, S2-S3, and No-Loss

A
  • PF @ least 3/5, glutes 4/5
  • Decd push off and stride length w/ fast amb or running
  • FOs→ recommended to maint. foot in neutral align.
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27
Q

MSK Deformities in SB

A
  • Spinal+LE deforms, Jt contractures related to:
    • MM imbalances, progress neuro dysf, intrauterine pos., coexist congen malforms,
    • Arthrogryposis (congen. contractures), post natal pos’ing,
    • reduced mvmt in utero 2* paralysis
  • Alignment issues==functional limits (i.e. crouch gait)
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28
Q

MSK Deformities

Common postural findings:

A
  • FHP, rounded shoulders, kyphosis/scoliosis, excess lumbar lordosis w/ APT
  • Rot. deforms of hip or tibia, flexed hips/knees, pronated feet
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29
Q

Crouch Gait (similar to CP)

Occurs 2*:

A
  • insuff. soleus strength to maint. vertical tibia
  • Ortho deformity
  • Hip+knee contractures
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30
Q

MSK Deformities:

Scoliosis, Kyphosis, Lumbar Lordosis

A
  • >> in higher lvl lesions, gen. worsen over time
  • Severe→ hinders lung/chest wall expansion
  • TLSO→ help maint. trunk pos. for function, does not prevent progression of deform*
  • Spinal fusions recommended, ~puberty
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31
Q

MSK Deformities

Hips and Knees

A
  • Hips
    • reduce bone loading=> deforms of femoral head and acetabulum=> inc risk sublux/disloc
      • U/L disloc→ fix, B/L disloc→ leave alone
  • Knees
    • flex/ext contractures
    • varus/valgus deforms.
32
Q

MSK Deformities:

Feet

A
  • Variety of deforms:
    • Club foot, Pes cavus/planus, Claw toe, Ankle PF contractures
  • NOTE: Sx for ankle-feet when cannot be alleviated by orthotics and when child shows desire to stand ~puberty
33
Q

Osteoporosis

A
  • Dec bone mineral density 2* hypOtonia, DEC mm strength, DEC loading→ Fx’s
  • Tx: CAREFUL W/ STRETCHING!!!
    • Incd fx risk
    • Excess/aggressive ROM avoided*
34
Q

Sensory Deficits

A
  • HIGHLY VARIABLE→ sensory lvls do not correlate to motor lvls and skip lvls may be present*
  • Consider:
    • test all derms+multiple sites w/in one derm
    • *skin inspection + pressure relief= critical
    • caution w/ modal’s
    • use of vision to aide in acts requiring incd body aware/learning new skill
      • mirrors, skin inspect
35
Q

Common brain disorder/event w/ SB

A

hydrocephalus

36
Q

Hydrocephalus

*COMMON

A
  • Excess accumulation CSF in ventricles of brain
  • 25% born w/, 60% dev. later, persists t/o lifespan
  • Cerebellar hypoplasia w/ caudal displace of hindbrain thru foramen magnum (Arnold-Chiari II Malformation) assocd w/ hydroceph.
  • usually req’s shunts→ have comps:
    • infection, malfunction→ gradual, subtle sx’s***
37
Q

Hydrocephalus + Chiari Malformations

A

see pics

38
Q

Shunts:

2 Basic Types

A
  1. VP→ Fluid from brain to abdominal cavity
  2. VA→ Ventricle in brain to R. atrium of heart
39
Q

Shunts:

Major precautions

AVOID!

A

AVOID any act. that places heart/stomach above head

  • upside down, wheelbarrow walking, gymnastics
  • 1 way valve mech. may malfunction
  • avoid direct pressure to skull where shunt is
40
Q

Shunt Malfunction Red Flags

*NOTE: PT may be first to ID shunt malf.

A
  • Recurring HA***
  • Irritability***
  • changes in speech, fever, dec school perform, inc strabismus, changes app/wt, worse scoliosis, incd spasticity, decd static grip, diff waking AM, visual changes, incd SZ, dec visuoperceptual coord, “off”
41
Q

Cog/Lang Delays w/ MM and hydrocephalus:

Diffs in discourse

A
  • High freq of irrelevant utterances***
    • “Cocktail personalities”*** talk about same thing (remember Dr. Conklin’s ex of little girl!!!)
  • Delayed ability to learn, diff w/ abstract thought
  • Considerations:
    • environ changes dec stimuli, allow inc processing time, keep cues simple/functional, repetition!!!
42
Q

Latex Allergy

A

18-40% children w/ MM ***

43
Q

Spasticity and MM

A

Highly variable!!

  • HypO, normal, HypERtonia (highly variable)
  • Considerations:
    • Tone scales, observe during functional mobility and Tardieu testing, may fluctuate* (hypO one day, hypER another)
44
Q

Progressive Neuro Dysf.

Typical progression of SB:

A
  • Deterioration of motor function
  • Pain
  • Progression of MSK deformity
  • Changes in B&B functoin
45
Q

SB displays progressive neuro dysf with a typical progression (other card)

Considerations:

A
  • Tethered cord rate is HIGHER for SB pop.
    • Monitor signs→ LBP, bladder incont., diff walking, weak/numb legs or feet
46
Q

Seizures in SB

Etiologies/Considerations

A
  • Etiologies→ hydrocephalus
    • shunt or brain malf.
    • brain infection
  • Considerations:
    • Be aware of sz presentation (ask parents!)
    • Be aware sz meds which may add to cog delays/dec alertness in PT
47
Q

Neurogenic Bowel/Bladder

A

Dysf of system preventing voluntary control of voiding

  • Considerations:
    • Pts not “potty trained” on time, part of team to assist w/ cath program, mbr of team for mobility aspect of B&B program, full bladder or missed BM may impact PT
48
Q

Skin Breakdown w/ SB

A
  • 85-95% all children w/ MM by adulthood
    • pressure, poor fit orthotics, friction/shear transfers or ADLs, poor hygiene/diaper sched.
  • Considerations:
    • orthotics full contact==reduce pressure sores, education press. relieving techs, skin inspection education w/ mirror
49
Q

Obesity

A

Common in children w/ MM!

  • Complicates mobility, ADLs, orthotic wear
  • Considerations:
    • Educate fitness/health lifestyle,
    • Community programs→ swimming, cycling, adapted yoga, refer out if needed (nutritionist)
50
Q

SB Additional Findings:

Upper Limb INcoordination

A
  • Assocd w/ MM+Hydrocephalus
    • poor visuoperceptual skills, fine motor tasks impaired
51
Q

SB Additional Findings:

Cranial Nerve Palsies

Which three?

A

CN VI- Abducens

CN IX- Glossopharyngeal

CN X- Vagus

52
Q

SB Additional Findings

CN Palsies

A
  • VI-Abducens→ Lateral rectus weakness
    • patch, rx lenses, minor sx
  • IX- Glossopharyngeal and X-Vagus→ Swallowing defs
    • typ present @ birth
53
Q

PT Examination: Infancy

Muscle Function

position of choice?

A
  • Side-Lying!!! → gravity-eliminated
54
Q

PT Examination: Infancy

Muscle Function

A
  • Reliable baseline of mm function before/after back closing
    • predicts future function and monitors status/progress
    • S/L== position of choice***
    • test when alert, hungry, crying
    • only observe, not handle
55
Q

PT Examination: Infancy

Sensation

A
  • Sharp test OK
  • Test in quiet state
  • Start @ Distal derms and work way up until noxious stim resp. observed, then stop.
    • Bc everything ABOVE should be intact→ remember like SCI***
56
Q

PT Exam: Infancy

Pain

A

FLACC scale

57
Q

PT Exam: Toddler/Preschool

A
  • ROM/strength thru observation of functional skills
  • Sensation→ Lt. touch typ via tickling→ Age 2***
  • Standardized gross motor assessments approp:
    • PEDI
    • PDMS-2
    • Functional Activities Assess.
    • Wee-FIM (inpatient)
58
Q

PT Examination:

Spina Bifida

*Progressive CNS dysf common @ this age…..

A

SCHOOL AGE!!!!

59
Q

PT Examination: School Age

A
  • Examine all prior→ sensation, coord, fine motor, gait, mobility, body aware, pain, endurance, functional skills→ all more objective @ this age***
  • ***Progressive CNS dysf common @ this age!!!
    • monitor rapid pds of growth 2* risk motor loss and tethered cord
60
Q

PT Exam: Adolescence/Transition to Adulthood

A
  • Balance/posture→ higher lvl ADLS as body composition changes/has impact
  • Teens means of mobility may change
    • progress. of neuro, alignment defs, post-op comps
  • Skin integrity primary concern→ 2* incd wt = sweat= skin breakdown
  • PT plays lg role in post-ortho sx (scoliosis, LEs)
61
Q

PT Tx:

A
  • Education across lifespan→ positioning, ROM, skin inspect, handling (newborns esp.)
  • Equipment→ DME, orthotics, adaptations for devices (ex. carseats)
    • *allow compensations to inc IND (long handled mirrors and reachers for skin inspect.)
  • Strengthening
  • Encourage independence
    • pre-k/early school age→ huge for asserting IND w/ basic self care/ADLs
  • Mobility training
62
Q

PT Tx Continued:

A
  • Post-ortho sx→ return to PLOF
    • home mods
  • Ongoing adjustments HEP
  • Advocate for pts!!!
    • adapting classes, address part. restrictions and enrollment in comm. activities and wellness programs→ combat obesity and decd social interaction
63
Q

Across the Life-Span

Focus on:

A
  • Monitor s/s shunt malf., changes/progression neuro impairs, jt align/muscle length,
  • Maint structural alignment→ specifically @ spine
  • improve/maint. strength, cog limits w/ respect to motor learning, encourage fitness/health,
  • Refer as needed and work closely w/ team!!!
64
Q

Orthotics Gen. Rx Guidelines

Foot orthotics and SMO’s (Supramalleolar Orthosis)

A
  • S1 and No Loss lvls
  • must have adeq. toe clearance and suff. gastroc/soleus strength
65
Q

Orthotics Gen. Rx Guidelines

AFOs (Ankle-Foot Orthosis)

A
  • L4-S1 lvls
  • weak/absent ankle musculature
  • knee EXTs @ least grade 4
66
Q

Orthotics Gen. Rx Guidelines

KAFOs (Knee, Ankle, Foot Orthosis)

A
  • L3-L4
  • weak knee and absent ankle musculature
67
Q

Orthotics Gen. Rx Guidelines

HKAFOs (Hip, Knee, Ankle, Foot Orthosis)

A
  • L1-L3
  • contd weakness BUT→ able to control hips
  • *may be first brace when getting child upright
68
Q

Orthotics Gen. Rx Guidelines

RGOs (Reciprocating Gait Orthosis)

A
  • L1 to L3
  • weak hip flexion req’d to operate

Check YOUTUBE: they can actually take steps and stops around lower Thoracic ***

69
Q

Orthotics Gen. Rx Guidelines

THKAFO/Parapodium

A
  • Thoracic to L2
  • walking usually non-functional

Check YOUTUBE: looks more like “waddling”

70
Q

Name the Orthotic Rx:

  • S1 and No Loss lvls
  • must have adequate toe clearance and suff. gastroc/soleus strength
A

Foot orthotics and SMOs

71
Q

Name the Orthotic Rx:

  • L4-S1 lvls
  • weak/absent ankle musculature
  • knee exts @ least grade 4
A

AFOs

72
Q

Name the Orthotic Rx:

  • L3-L4
  • weak knee and absent ankle musculature
A

KAFOs

73
Q

Name the Orthotic Rx:

  • L1-L3
  • contd weakness BUT able to control hips
  • may be first brace when getting child upright
A

HKAFOs

74
Q

Name the Orthotic Rx:

  • L1-L3
  • weak hip flex. required to operate
A

RGOs

75
Q

Name the Orthotic Rx:

  • Thoracic to L2
  • walking usually non-functional
A

THKAFO/Parapodium

76
Q

PT Tx Ideas for SB?

A

Same as SCI → but make it fun/play!!!!

77
Q

Summary of SB:

A
  • Children/ado’s w/ SB challenge PTs to test knowledge and be creative w/ tx skills
  • Multi-system presentation==> complex but fun to work with!!!

Pediatrics (27 decks)

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