28. Congenital/Developmental Problems Flashcards Preview

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Flashcards in 28. Congenital/Developmental Problems Deck (46):

Define Developmental Dysplasia of the Hip (DDH)

what problems can it cause?

loosened connection between the femoral head and the acetabulum. congenital or acquired.

Spectrum of possible problems: acetabular dysplasia, hip instability, hip dislocation



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A: normal hip

B: some laxity, acetabulum slightly shallow.

C: progression towards dislocation, some sublexation

D: true dislocation


DDH: epidemiology?

major risk factors?

Fairly common in US: females > males

Risk factors are "packaging problems"!

-Breech birth (puts pressure on hip)

-First born (your job is to stretch out the uterus, good luck)


-Oligohydramnios (not enough fluid in uterus)

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DDH: symptoms?

What are the 2 screening tests?

Asymptomatic. No pain, no change in how leg moves

Screening tests: Ortolani test, Barlow test. Both are physical maneuvers


Describe the Ortolani test

"Innie" test: hip starts dislocated, push hip back IN bu abducting and putting slight posterior pressure on upper trochanter 

(pic is at end of motion you have done)

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Describe the Barlow test


Start with hip abducted, in normal position. push posteriorly and try to dislocate the hip. You will find hips that are prone to popping out of socket

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Ultrasound of neonatal hip to look for DDH

measure alpha angle - how deep/steep is the socket?


On a neonate, should the hips be symmetrical, or ok to have some asymmetry?

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should be symmetrical.

asmmmetry may be sign of sublexed hip (might be the only sign you get)


What do we think?

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Different leg lengths: could be thigh shortening, different tibial lengths. hard to pick up but could also be a sign of hip dysplasia


what do we think?

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asymmetry of gluteal or thigh folds: indicates one hip may be superior

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note small calcification on left side of image. (white dot)

this is a left unilateral dislocation (???)


what are signs of DDH in ambulatory children?

no pain, but may present with Lordosis, leg length discrepancy.

Limp, waddling gait, Trendelenberg sign (pelvis drop due to abductor weakness)

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which side is dislocated?

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Patient's Right side (L side of image) is dislocated

note Shenton's line (#5 on drawing below).

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What are the sequelae of untreated DDH?

Limping, weakness, early OA, pain.


DDH: treatment objectives?

Reduce hip early (get it back where it belongs)

Avoid avascular necrosis

The earlier you get it back to the right place, the better it develops overall


Best treatment for DDH in infants?

What should I watch out for?

Pavlik harness (photo)

dynamic brace/reproduces the Ortolani maneuver. Work 24h/day, adjusted weekly to accomodate growth.

Be careful about femoral nerve palsy (which is noticeable) and avascular necrosis (you will not know for 3-6 months, by which point it is too late)

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When Pavlik fails: what is next line of treatment for DDH?

What if that fails?

Closed reduction and spica cast

have to place case w patient under general anesthesia

Next line: Open reduction (most invasive)

Traction is also an option

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A point about swaddling and DDH -- advice to new parents?

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Babies love it but leave their legs free so they can kick. If you swaddle their legs with their hips extended can contribute to dysplasia


Clubfoot: what are the three components of the deformity?

1. ankle equinus (plantarflection)

2. Heel varus (rotated towards midline)

3. forefront adduction

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Clubfoot: epidemiology?

M:F ratio?



roughly same incidence as DDH (1:1000)


higher incidence if + family history

Otherwise etiology unknown


Clubfoot: what is progression if untreated?

Not painful

But untreated, will lead to deformed, painful feet

will NOT straighten out on its own


Clubfoot: treatment options?

-Ponseti treatment (her fave)

-French Technique

-Surgical reconstruction


Describe the Ponseti technique for Clubfoot treatment

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NON surgical

Weekly manipulation and casting for 5-6 weeks (CAVE cast)

Followed by cutting achilles and further casting

May need to re-cut achilles as it grows back

Bracing full time for months (photo)

Eventually get to point of overcorrection, as foot wants to return

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What does CAVE stand for?

Cavus, Adductus, Varus, Equinus

Type of casting technique used with Ponseti treatment for Clubfoot


Describe the non-Ponseti treatments for clubfoot (French, surgery)

Problems with each?

French: combo of splinting, stretching, taping. (Problem: may need to do Ponseti afterwards....)

Surgery: foot reconstruction. (Problem: results in very stiff and scarred foot)


What other anomalies are associated with clubfoot?


-Tortocollis (fixed rotation of neck due to tight SCM)

-Spinal anomalies (myelomeningocele aka Spina Bifida)


Spina Bifida: define.

What are the 4 possible presentations?

aka Myelomeningocele

Dev defect of neural tube, resulting in an open spinal cord lesion.

1. Anencephaly (no brain/skull/scalp, kiddo looks like a frog)

2. Spina Bifida Occulta (mild)

3. Meningocele (absence of bone covering spinal cord)

4. Myelomeningocele (skin, nerve, bone all extruding out)

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Spina bifida occulta: define?

symptoms? exact location?

–Defect of posterior bony elements of the spine

–Usually asymptomatic

-Location: L5/S1

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Spina Bifida: maternal risk factors?

•folic acid deficiency**



•Being exposed to hyperthermia

•Certain medications

•valproic acid



Spina Bifida: what will be the patient's level of functioning? what does it depend on?

Depends on level of myelomeningocele defect.

1. if at thoracic level: functional ambulation is rare.

2. If at L1-L3 level: will need bracing/assistive devices to ambulate. 

3. L4-L5 level: milder, will ambulate later if given aids when learning to walk (braces)

4. Sacral level: children can often learn to walk without aids or braces. Distal weakness may lead to foot deformities --> may benefit from orthotics.


Spina Bifida: why is it impt to recall the system of muscle grading?

bc muscle strength is associated with ambulatory outcome. 

Ex: iliopsoas strength between 0-3 is associated with reliance on wheelchair.

(Strength: 0 = none 1 = twitch, 2 = moves but cannot oppose gravity, 3 moves against gravity, 4 can be overcome, 5 strong)



Define Cerebral Palsy

–A group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain

–Defect is CNS-related: motor neuron problem. The resulting muscle and connective tissue problems you see are the result of this initial motor neuron abnormality


Cerebral Palsy: what are the 4 subtypes? which is most common?

Subtype determined by underlying muscle tone


•Most common

•Velocity-dependent increase in muscle tone



•Dyskinesia (writhing)




•Disturbance of coordinated movement


Both pyramidal and extrapyramidal motor control abnormalities (mix of fine and gross motor problems)


There are a million types of cerebral palsy. for example: monopareisis, hemiparesis, spastic diparesis. What does each term mean?

-Spastic diparesis: both legs involved

-hemoparesis: one leg and ipsilateral arm involved

-Triparesis: both legs + one arm

etc etc


Cerebral Palsy: prevalence compared to DDH, Clubfoot, spina bifida?

What is risk factor for CP?

incidence: 2 or 3 per 1000 live births: more common than DDH, spina bifida, clubfoot (individually). Spina bifida most rare of all these.

The more premature, the higher chance of CP

higher chance in twins, triplets, etc


Cerebral Palsy has a million secondary complications. name a few and name the most common?

(All these are associated with a single motor defect)

–joint/muscle contractures–foot deformity (equinus foot is the most common musculoskeletal deformity in CP)–hip dysplasia (chronic hip subluxation and progressive dislocation); even if the hip joints start normal at birth, it will eventually become dysplastic so watch out for this!!!!–neuromuscular scoliosis–Osteopenia (bc of less walking, esp on affected side) –cognitive impairment (risk increases with greater degree of neuromuscular impairment)–seizure disorder–visual impairment–dysphagia–speech impairment–failure to thrive (high metabolism and it’s hard to keep up with the spastic muscles!) –impaired airway clearance–Chronic pain


Cerebral Palsy: what are early predictors of outcomes?

-persistence of primitive reflexes is bad, since these make it hard to ambulate

-sitting by age 2 is predictive of ambulation

-if not walking by 6, probably will never walk

-adverse factors are mental retardation, epilepsy, certain types of CP

-lack of control of facial muscles doesn't correlate to intelligence - keep working with these kids


CP: treatment?

-coordinated care with ortho, PT, etc etc

-orthoses, adaptive equipment

-spasticity management: GABA agonists, botox inj in calf muscles

-dorsal rhizotomy (neurosurg that breaks problematic nerve roots)


CP: goals of PT?

-voluntary control, tone, balance

-start with head control and work way caudal (if they can't control head, trunk they will never learn to walk)


CP: goals of orthotic treatment?

-prevent deformity

-substitute for, & protect weakened parts


-improve sitting balance

-night splinting to maintain positioning

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CP: gross motor function classification system?

There is basically a page of info on this (EFF HER). The basics: 

Level 1 = normal.

 2= must use handrail.

 3= self-powered

4= short distances only with help,

 5= no good head control WORST

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CP: functional mobility scale?

Opposite from GMFCS in that 1 is worst, 5 is best. 

This scale represents mobility over 3 sets of distances (to represent home, school, community)


CP: types of ortho surgical treatment?

(I condensed about 8 slides into one here.....)

Point of surgery: gait improvement, positioning/posture improvement, hygeine access, pain control.

Types of treatment:

-Tendon lengthening (ex Achilles)

-Long bone realignment

-hip reduction (femoral ostetomy)

-spinal fusion (PSF = Posterior Spinal Fusion: fusing the whole spine, because if you fuse only one section the other parts tend to curve even more)

-hip resection

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CP: what types of orthopedic evaluation should occur periodically?

–Every 6 -12 months

Screening AP pelvis x-ray: Repeat yearly for ambulatory patients, Repeat twice yearly if subluxation

–Screening scoliosis x-ray

–Repeat if needed, based on exam

–Help coordinate all disciplines

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we keep talking about sublexation. wft is that?

From Wiki: partial or complete dislocation of a joint. Significant structural displacement, visible on imaging.

(means slightly different things to ortho, opthomology, dentistry but this seems the most relevant)


Cerebral Palsy: non-surgical treatment of joints?

•Manipulation and casting

–Useful for ankle, knee, elbow, wrist, hand

–Change every few days to weeks

–Sometimes used in conjunction with Botox

–Constraint therapy (immobilize good side to force dev of the bad side)

Pic: example of constraint therapy

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