U2L4: Neuromuscular & Musculoskeletal Disorders

neural tube defects (NTD) patho

failure of embryonal neural tube closure

anencephaly

absence of cerebral hemispheres -> affects top of NT

encephalocele

out pouching of brain between eyes & base of skull -> affects brain & meninges

spina bifida

affects spine & spinal cord

NTD risk factors (4)

- folic acid deficiency

- diabetes

- fever/hyperthermia -> no sauna or hot tub

- maternal drug use

how much folic acid should a pregnant women ingest each day?

400 mcg

normal NT folding

NT starts flat -> starts to fold at 21 days -> folds into tube at 28 days

spina bifida types

- closed (occulta) -> covered by skin

- open -> covered by membrane

occulta spina bifida

minimal symptoms & typically benign but can cause tethered cord

occulta spina bifida presentation

cutaneous clues, hairy patch, dimple, or lipoma on back

types of open spina bifida

meningocele or myelomeningocele

meningocele

sac like protrusion of the meninges & CSF w normal spinal cord -> minor or no deficits

myelomeningocele (spina bifida cystica)

sac like protrusion of meninges, CSF, nerve roots, & portion of the spinal cord

spina bifida prenatal dx (3)

- elevated alpha fetoprotein

- ultrasound

- c section

spina bifida post birth dx (5)

- newborn physical exam

- CT

- MRI

- ultrasound

- myelography

myelomeningocele characteristics (2)

- unprotected neural sac

- lumbar & lumbosacral problems

myelomeningocele associated manifestations (5)

- sensory/motor dys

- bladder/bowel dys

- hydrocephalus

- chiari 2 mal

- seizures

chiari 2 malformation

portion of the cerebellum & brain stem extend into the spinal canal

myelomeningocele repair (2)

- surgical closure asap

- monitoring/aggressive inf tx

options once myelomeningocele is dx (3)

- abortion

- fetal surgery

- surgery post birth within 12-17 hrs

when is myelomeningocele fetal surgery completed?

18-25 weeks & requires strict criteria

myelomeningocele fetal surgery benefits (2)

- stops leakage of spinal fluid -> prevents chiari 2 mal & hydrocephalus

- improved psychomotor development

myelomeningocele fetal surgery risks (3)

- preterm delivery

- placental abruption for this pregnancy & all future ones

- requires 2nd surgery later in life

myelomeningocele surgery positioning & warmth (3)

- radiant warmth

- prone w hips felxed or side lying

- head turned to side to feed

myelomeningocele surgery inf control pre op (4)

- cover sac w moist, sterile, non adherent dressing

- assess for CSF leaks

- sterile gloves & linens

- prophylactic antibiotics

myelomeningocele surgery monitoring (3)

- hydrocephalus risk -> OFC daily

- I&Os -> foley or straight cath

- skin integrity

myelomeningocele infant precautions

treat as if has latex allergy

spina bifida long trm nursing management (5)

- straight cath at home -> clean not sterile

- enema qd

- PT/OT

- shunt for hydrocephalus

- decrease sensation

metatarus adductus

medical adduction of toes & forefoot due to position in utero -> pigeon toes

- most common in 1st child

how does metatarus adductus resolve?

most outgrow by first bday w stretching

- if severe may need cast/splint

congenital clubfoot/talipes equinovarus

bony deformity of foot/ankle -> elevated heel w foot turned inward

- bilateral 50%

congenital clubfoot consideration

inward turned feet is normal within first 14 weeks of life

congenital clubfoot etiology (2)

- family hx

- drug use during pregnancy

positional/postural congenital clubfoot

tightness & shortening of soft tissue -> no bony abnormality

how does positional congenital clubfoot resolve?

spontaneously w possible stretching or casting

syndromic congenital clubfoot

severe w other anomalies & difficult to tx

- high incidence of recurrence

congenital clubfoot - true clubfoot

bony abnormality w shortening of achilles heel

- idiopathic

congenital clubfoot dx (3)

- prenatal ultrasound

- physical exam

- hip dysplasia evaluation

physical exam for congenital clubfoot (2)

- affect foot -> smaller, empty heel pad, midfoot medial crease

- affected limb -> shorter w calf atrophy

congenital clubfoot management (3)

- ponseti method serial casting

- foot orthotic set to abduction

- follow up observation to prevent recurrence

ponseti method serial casting

gradual stretching & casting -> typically lasts 10 weeks

foot orthotics used for congenital clubfoot

ponseti sandals or denis brown bar for 23 hrs/day for 3-6 months

- then naps & bedtime

when is congenital clubfoot tx started?

at birth

congenital clubfoot nursing management (2)

- neurovascular checks & skin integrity

- education

congenital clubfoot management education (3)

- cast care

- change diaper frequently

- check for decreased circulation

osteogenesis imperfecta (OI)

genetic collagen defect that leaves bone easily fractured

genetic defect causing OI

problem w COl1A1 or COL1A2 which codes for pre collagen -> faulty bone mineralization & abnormal bone development

OI classification

over 20 types classifies by clinical features & inheritance pattern

type 1 OI

mildest & most common

type 2 OI

most severe & lethal in infnacy

OI clinical manifestations (6)

- bone fragility

- joint hypermobility

- short stature

- blue sclera

- hearing loss in 20s

- dentinogenesis imperfecta

dentinogenesis impertfecta

fragile, discolored teeth

OI complications (5)

- fractures

- hearing loss

- acute & chronic pain

- scoliosis

- resp problems

OI meds

biphosphonates -> increases bone density & decrease calcium

- more beneficial for vertebral bones than long bones

OI supportive care (4)

- braces & splints

- PT

- surgery

- positioning & activity support

OI management goals (3)

prevent:

- positional contractures & deformities

- weakness & osteoporosis

- malalignment of lower extremity joints

OI management do's (3)

- support fully when lifting

- provide supported positioning

- handle limb minimally if fracture is suspected

OI management don'ts (2)

- hold by ankle when changing diaper

- lift from under armpits

OI education & screenings (3)

- teach prevention & suitable activities

- refer to genetic counseling

- screen for OI in chidlren w fractures

OI pt consideration

high pain tolerance

developmental dysplasia of the hip (DDH)

abnormal development of the hip -> head of femur slips in & out of acetabulum

when is the acetabulum fully developed?

at 5 yrs of age

DDH cause

in utero or infancy femur loses contact w acetabulum -> acetabulum gets shallow

dysplasia DDH

little displacement

subluxation DDH

ball of femur is pulling taught but still has soem contact

dislocation DDH

ball of femur has no contact w acetabulum

who is most likely to get DDH? (2)

- female

- 1st born child

what infants must be checked for DDH?

female in breech position

DDH etiology (5)

- breech or multiple fetus

- constant swaddling w legs straight

- joint laxity

- large infant

- oligohydraminos

DDH dx (3)

- positive barlow & ortalani maneuver

- ultrasound -> not accurate before 6 wks

- x ray -> not accurate till 4-6 months

DDH manifestations (5)

- asymmetric gluteal or thigh folds

- limited hip abduction

- leg length discrepancy

- limping

- toe walking

DDH tx for newborn-6 months (2)

- pavlik harness

- closed reduction surgery & hip spica cast for 12 wks if harness fails

pavlik harness for DDH

aligns femur w acetabulum & worn 23 hrs/day

DDH tx for 6-24 months (4)

- hip abduction orthosis for mild

- bryant skin traction

- surgical closed reduction

- hip scipa cast for 12 wks

bryant skin traction

used for adduction contractions before surgery -> lifts buttocks

DDH tx for older children (3)

- traction

- surgical pelvic & femoral osteotomy

- hip scipa cast for 12 wks

*more difficult to tx

pavlik harness nursing management (4)

- only off for bathing & don't adjust straps

- gentle massage daily under straps

- diaper & undershirt under straps

- no lotions or powder

hip scipa cast nursing management (2)

- cast care

- double diaper

torticollis

tightness of the sternocleidomastoid causing wryneck -> head tilted to one side

torticollis management (2)

- PT -> gentle stretching several times/day

- TOT collar

stretching done for torticollis

immobilize affected shoulder -> gently turn head toward unaffected side -> hold for 10-30 secs

torticollis cause

in utero positioning or difficult birth

torticollis complication

plagiocephaly

blount's disease (tibia vera)

progressive bowing of legs due to pressure on outer growth plate of tibia

blount's disease risks (4)

- walkign before 12 months

- obesity

- family hx

- female black children

types of blount's disease (3)

- infantile -> 1-3 y/o (most common)

- juvenile -> 4-10 y/o

- adolescent -> >=11 y/o

blount's disease management (3)

- bracing 23 hrs/day for months or yrs

- osteotomy for severe or older children

- tx <4 leads to better outcome

cerebral palsy (CP)

chronic non-progressive motor dysfunction caused by insult to the motor portion of the brain

biggest sign of CP

missing milestones

CP clinical manifestations (5)

- delayed gross motor development

- abnormal motor fxn -> feeding difficulties

- altered muscle tone

- abnormal posturing

- reflex abnormalities -> clonus

associated deficits w CP (7)

- seizures

- vision, hearing, or speech problems

- learning disabilities

- behavior problems

- resp problems

- bowel/bladder problems

- bone abnormalities (scoliosis)

spastic CP

poor control of motion, balance, & posture -> hemiparesis, quadriparesis, diplegia

diplegia

only affects lower body -> normal intellectually

dyskinetic CP

abnormal involuntary movement -> athetoid (worm like) & dystonic

ataxic CP

lack of coordination w purposeful movement -> wide based gait

*caused by cerebellum damage

mixed CP

combo of spastic & dyskinetic

CP dx (4)

- hx

- metabolic & genetic testing

- MRI

- neuro exam

CP management (5)

- physical, occupational, speech therapy

- meds

- nutrition support -> asp risk

- surgery

- modify environment

meds for CP

relieve spasticity & seizure control

surgery for CP (2)

- selective dorsal rhizotomy -> cut nerves in back to relieve spasticity

- orthopedic for joint deformities