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Spina Bifida Flashcards

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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
Motor Paralysis: **Common Lvl Characteristics→** ## Footnote **S1**
* **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.
26
Motor Paralysis: **Common Lvl Characteristics→** ## Footnote **S2, S2-S3, and No-Loss**
* 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.**
27
MSK Deformities in SB
* 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)
28
MSK Deformities **Common postural findings:**
* FHP, rounded shoulders, kyphosis/scoliosis, excess lumbar lordosis w/ APT * Rot. deforms of hip or tibia, flexed hips/knees, **pronated feet**
29
Crouch Gait (similar to CP) ## Footnote **Occurs 2\*:**
* insuff. **soleus strength** to **maint. vertical tibia** * Ortho deformity * Hip+knee contractures
30
MSK Deformities: ## Footnote **Scoliosis, Kyphosis, Lumbar Lordosis**
* \>\> 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**
31
MSK Deformities ## Footnote **Hips and Knees**
* **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
MSK Deformities: ## Footnote **Feet**
* 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
Osteoporosis
* 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
Sensory Deficits
* 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
Common brain disorder/event w/ SB
hydrocephalus
36
Hydrocephalus ## Footnote **\*COMMON**
* 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
Hydrocephalus + Chiari Malformations
see pics
38
Shunts: ## Footnote **2 Basic Types**
1. **VP→** Fluid from **brain to abdominal cavity** 2. **VA→** Ventricle in **brain to R. atrium of heart**
39
Shunts: ## Footnote **Major precautions** **AVOID!**
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
Shunt Malfunction **Red Flags** ## Footnote **\*NOTE: PT may be first to ID shunt malf.**
* **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
Cog/Lang Delays w/ **MM and hydrocephalus:** ## Footnote **Diffs in _discourse_**
* **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
Latex Allergy
18-40% children w/ MM \*\*\*
43
Spasticity and MM
**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
Progressive Neuro Dysf. ## Footnote **Typical _progression_ of SB:**
* Deterioration of **motor function** * **Pain** * Progression of **MSK deformity** * Changes in **B&B functoin**
45
SB displays **progressive neuro dysf with a typical progression (other card)** ## Footnote **Considerations:**
* **Tethered cord rate is HIGHER for SB pop.** * Monitor signs→ **LBP, bladder incont., diff walking, weak/numb legs or feet**
46
Seizures in SB ## Footnote **Etiologies/Considerations**
* 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
Neurogenic Bowel/Bladder
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
Skin Breakdown w/ SB
* 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
Obesity
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
SB **Additional Findings:** **Upper Limb INcoordination**
* **Assocd w/ _MM+Hydrocephalus_** * poor visuoperceptual skills, fine motor tasks impaired
51
SB **Additional Findings:** ## Footnote **Cranial Nerve Palsies** **Which three?**
CN VI- Abducens CN IX- Glossopharyngeal CN X- Vagus
52
SB **Additional Findings** ## Footnote **CN Palsies**
* **VI-**_A_**bducens→** L**_a_**teral rectus weakness * patch, rx lenses, minor sx * **IX- Glossopharyngeal and X-Vagus→** Swallowing defs * typ present @ birth
53
PT Examination: **Infancy** ## Footnote **Muscle Function** **position of choice?**
* Side-Lying!!! → **gravity-eliminated**
54
PT Examination: **Infancy** ## Footnote **Muscle Function**
* 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
PT Examination: **Infancy** ## Footnote **Sensation**
* 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
PT Exam: **Infancy** ## Footnote **Pain**
FLACC scale
57
PT Exam: **Toddler/Preschool**
* 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
PT Examination: ## Footnote **Spina Bifida** **\*Progressive CNS dysf _common_ @ this age…..**
SCHOOL AGE!!!!
59
PT Examination: **School Age**
* 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
PT Exam: **Adolescence/Transition to Adulthood**
* 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
PT Tx:
* **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
PT Tx Continued:
* 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
Across the Life-Span ## Footnote **Focus on:**
* 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
**Orthotics Gen. Rx Guidelines** **Foot orthotics and SMO's (Supramalleolar Orthosis)**
* S1 and No Loss lvls * **must have adeq. toe clearance and suff. gastroc/soleus strength**
65
Orthotics Gen. Rx Guidelines ## Footnote **AFOs (Ankle-Foot Orthosis)**
* L4-S1 lvls * **weak/absent ankle musculature** * **knee EXTs @ least grade 4**
66
Orthotics Gen. Rx Guidelines ## Footnote **KAFOs (Knee, Ankle, Foot Orthosis)**
* L3-L4 * **weak knee and absent _ankle_ musculature**
67
Orthotics Gen. Rx Guidelines ## Footnote **HKAFOs (Hip, Knee, Ankle, Foot Orthosis)**
* L1-L3 * **contd weakness BUT→ able to control hips** * **\*may be first brace when getting child upright**
68
Orthotics Gen. Rx Guidelines **RGOs (Reciprocating Gait Orthosis)**
* L1 to L3 * **weak hip flexion req'd to operate** **Check YOUTUBE:** they can actually take steps and stops around lower Thoracic \*\*\*
69
Orthotics Gen. Rx Guidelines ## Footnote **THKAFO/Parapodium**
* Thoracic to L2 * **walking usually _non-functional_** Check YOUTUBE: looks more like “waddling”
70
**Name the Orthotic Rx:** * S1 and No Loss lvls * must have **adequate toe clearance and suff. gastroc/soleus strength**
Foot orthotics and SMOs
71
**Name the Orthotic Rx:** * L4-S1 lvls * **weak/absent ankle musculature** * **knee exts @ least grade 4**
AFOs
72
**Name the Orthotic Rx:** * L3-L4 * **weak knee and _absent_ ankle musculature**
KAFOs
73
**Name the Orthotic Rx:** * L1-L3 * **contd weakness BUT able to control _hips_** * **may be first brace when getting child upright**
HKAFOs
74
**Name the Orthotic Rx:** * L1-L3 * **weak hip flex. required to operate**
RGOs
75
**Name the Orthotic Rx:** * **Thoracic** to L2 * **walking usually _non-functional_**
THKAFO/Parapodium
76
PT Tx Ideas for SB?
**Same as SCI → but make it _fun/play_!!!!**
77
Summary of SB:
* 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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