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Pediatric Flashcards

1
Q

What possible clinical findings are associated with caudal regression syndrome?

A
  1. Subset of neural tube defect.
  2. Absence of the sacrum + portions of lumbar spine.
  3. Associated with maternal diabetes.
  4. Associated finding: syringomyelia, anorectal stenosis, renal abnormalities, external genital abnormalities, cardiac problems, Motor and sensory abnormalities.

Ref: Alexander and Matthews pg 203.

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2
Q

what percentage of pts with CP have epilepsy?

A

43%.
Risk is increased with structural abnormalities seen on neuroimaging.

Ref: Braddom pg 1260.

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3
Q

What is the Risser staging? At what stages does menses start? At what Tanner stage does peak progression of curve occur?

A

“The Risser staging is a grading system to determine the skeletal maturity of an individual by measuring the ossification of the pelvis (iliac crest). 5 stages: 5 fully skeletally mature 0 no ossification. Lateral to medial.

0: no ossification.
1. < 26% (most rapid skeletal growth)
2: 26-50% - MENSES OCCUR
3: 51-75% (growth slows)
4: 76-99%
5: 100% (iliac apophysis fused to iliac crest; end of growth)
Ref: 2001 AAFP – adolescent idiopathic scoliosis, review and current concepts.

Note: if no apophysis seen on x-ray of pelvis, this signifies either Risser 0 or Risser 5.
However, can clarify by looking at tri-radiate cartilages. If the apophysis are open, then it is Risser 0. And obviously look at the age of the child.

Peak curve progression: Tanner stage 2-3.”

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4
Q

What is the number 1 cause of death in children with CP?

A

Pneumonia (90% of deaths of children with CP related to pneumonia).
Ref: Braddom pg 1260.

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5
Q

What is the greatest risk factor for developing CP?

A
  1. Prematurity (40-150/1000) - up to 100-fold increased risk. < 37 weeks (Alexander/Matthews pg 165), higher risk if < 32 weeks (Braddom pg 1254) - highest risk for periventricular hemorrhage is b/w 23-32 weeks

FACTORS:

  1. immaturity.
  2. fragile brain vasculature.
  3. physical stresses predispose to compromised cerebral blood flow.
  4. vessels next to lateral ventricles vulnerable.
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6
Q

What is the GMFCS classification? What does it stand for? When is it used?

  1. Walk
  2. Walk w/ Limit
  3. Walk w/ Device
  4. Walk w/ Person or Power Device
  5. W/C Transport
A

Gross Motor Functional Classification System: Used for cerebral palsy based on self-initiated movement, with emphasis on sitting, transfers and mobility.

Distinctions based on functional limitations, the need for hand-held mobility devices (such as walkers, crutches, or canes) or wheeled mobility, and to a much lesser extent, quality of movement.

There are actually subgroups based on age 2-4, 2-6, 6-12, 12-18.

  1. WALKS WITHOUT LIMITATIONS:
    (no assistive devices, walks indoors, outdoors, climbs stairs, no limits, can run + jump, decreased speed, balance, coordination).
  2. WALKS WITH LIMITATIONS:
    (limited outdoor activities, difficulty uneven terrain, inclines, crowds walks indoors, outdoors, climbs stairs with railing minimal ability to run + jump).
  3. WALKS WITH HANDHELD MOBILITY DEVICE:
    (walks indoors + outdoors on level surfaces with assistive device, may be able to climb stairs with railing, may propel manual chair, assistance for longer distance or, uneven terrain).
  4. SELF MOBILITY WITH LIMITATIONS, MAY USE POWER W/C:
    (self mobility severely limited even with assistive devices, uses w/c most of time, may propel own power chair).
  5. TRANSPORTED IN WHEELCHAIR:
    (physical impairments that restrict voluntary control of movement, ability to hold neck + head against gravity, impaired in all areas of motor function, cannot sit or stand independently even with assistance, may be able to use power, no independent mobility).

Ref: Gross Motor Function Classification System, Alexander and Matthews, GMFCS-ER.pdf

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7
Q

What is the definition of CP Cerebral Palsy?

A

Cerebral palsy (CP) describes a group of 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. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour, and/or by a seizure disorder.

Ref: Rosenbaum 2005

No explicit upper age limit, practically taken as either 2 or 3 years of age.

3 major criteria (Pediatric Rehab - Principles and Practice)

  1. neuromotor control deficit that alters movement or posture
  2. static brain lesion
  3. acquisition of brain injury either before birth or in first years of life
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8
Q

What is periventricular leukomalacia and what is its significance in CP?

A

Periventricular leukomalacia (affects white matter near lateral ventricles) occurs in up to 56% of all cases of CP.
1. Common outcome of intraventricular hemorrhage in premature infants, Premature (90%) vs Term (20%).
2. Corticospinal tract fibres to lower extremities medial to uppers, so typically have spastic diparesis
3. PVL in 71% diparetics, 35% quadriparetics, 34% hemiparetics.
4. fragile capillaries in watershed zone by lateral ventricles by germinal matrix.
Ref: Alexander and Matthews pg 168; Braddom pg 1253

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9
Q

What are types and common causes/categories of CP?

A

SPASTIC CP:

  1. diplegic (legs > arms) intraventricular hemorrhage (periventricular leukomalacia).
  2. quadriplegic (4 limbs) infection, ischemia (focal, multi-focal, watershed)
  3. hemiplegic – MCA stroke, congenital brain malformation, SDH (trauma).
  4. Double hemiplegic (bilateral, arms > legs) bilateral insult (?stroke).
  5. Triplegic (both legs and 1 arm)
  6. Monoplegic (rare)

DYSKINETIC CP

(basal ganglia, thalamus e.g. Kernicterus, diffuse anoxia)

  1. choreoathetoid.
  2. dystonic.
  3. ballstic
  4. ataxic [some categorize it here]

ATAXIC CP

(cerebellar hypoplasia, granule cell deficiency)

MIXED CP (both spastic and dyskinetic)

HYPOTONIC

Ref: 2007 PMR clinics north america – Green – cerebral palsy; Cucurrullo p765-768; Braddom p1255; Delisa pg 1484

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10
Q

What are the two most common malignant tumours in bone in pediatric age group?

A
  1. Osteogenic sarcoma (osteosarcoma) - adolescence and commonly involve the knee and proximal humerus.
  2. Ewing sarcoma.

Ref: Cuccurullo pg 754.

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11
Q

What are rehabilitation issues of CP in adulthood?

A

In theory, all rehab issues faced in pediatric age could be faced in adult phase of CP.

  1. contractures.
  2. pain.
  3. Issues with ADLS.

Ref: Developmental Medicine & Child Neurology 2001, 43: 76–82.

  1. MSK – back pain, leg pain, overuse injuries, progressive scoliosis, cervical myelopathy in athetoid, hand paresthesias.
  2. GI + GU symptoms: abnormal urodynamics common.
  3. Mobility: declines after 25 y/o, significant decline after 60 y/o.
  4. Independence: less likely to live independently, have intimate relationships, be employed (up to 50%, less likely if: female, lower IQ, dependant for transportation, lower education level).
  5. Fatigue – physical, not mental, predictors: msk pain, deterioration of physical function, limitation in emotional and physical roles, low life satisfaction.
    Ref:?

Note: 87% survival at 30 y/o – epilepsy and MR have negative effect on survival, as does reduced mobility or impaired feeding.

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12
Q

What are associated CNS structural abnormalities/malformations in spina bifida?

A

SPINAL CORD:

  1. tethering.
  2. Diastematomyelia.
  3. diplomyelia.
  4. split cord.

BRAINSTEM:

  1. Arnold type 2 malformation (kinking, inferior displacement of medulla).
  2. syringobulbia.

CEREBELLUM:

  1. Chiari type 2 malformation (elongated vermis, etc).
  2. heterotopia, dysplasia.

VENTRICULAR SYSTEM:

  1. hydrocephalus.
  2. aqueductal stenosis/malformation.

FOREBRAIN:

  1. polymicrogyria.
  2. heterotopia of nuclear structures.
  3. prominent massa intermedia.
  4. thalamic fusion.
  5. agenesis of olfactory bulb/tracts.
  6. corpus callosum dysgenesis.

Ref: Pediatric rehabilitation principles and practice, pg 206.”

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13
Q

What are 3 risk factors for pneumonia in children with CP?

ASP - Clear/Mucus - Spine/Airway

A
  1. Aspiration
  2. decreased mucous clearance
  3. suppuration
  4. kyphoscoliosis
  5. airway obstruction

Ref: Braddom pg 1260.

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14
Q

Treatment of toe walking.

A

Depends upon the cause (idiopathic – observation).
CONSERVATIVE:

  1. stretching gastroc + soleus, hamstrings
  2. night splints
  3. serial bracing or casting
  4. AFO

MEDICAL:

  1. oral antispasticity medications
  2. botulinum toxin (or phenol/alcohol)

SURGICAL:

  1. heel cord lengthening.
  2. hamstring release.

Ref: first principles (Lam style).

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15
Q

Spina bifida, list 3 urologic surgical procedures to treat neurogenic bladder.

A
  1. sling suspension procedures (improves sphincter incompetence by changing angle).
  2. catheterizable stoma to bladder (mitrofanoff Appendico-vesicostomy).
  3. clam cystoplasty/bladder augmentation (ileum or colon used to enlarge bladder size).

Ref: Pediatr Nephrol (2008) 23:889–896.

  1. urethral implantation.
  2. suprapubic vesicostomy.
  3. artificial sphincter implantation.

Ref: Delisa pg 1498.

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16
Q

Spina bifida risk factors – list 4.

A

ESTABLISHED RISK FACTORS:

  1. Hx previous SB with same partner (30x risk).
  2. folic acid deficiency (2-8x risk).
  3. pre-gestational maternal DM (2-10x risk).
  4. valproic acid/carbamazepine (10-20x risk).

SUSPECTED RISK FACTORS:

  1. Maternal V12 deficiency (3x risk).
  2. maternal obesity (1.5-3.5x risk).
  3. maternal hyperthermia (2x risk).
  4. maternal diarrhea (3-4x risk).
  5. gestational DM.
  6. Fumonisins.
  7. Paternal exposure to agent orange.
  8. drinking water with chlorination disinfection by-products.
  9. EM fields.
  10. hazardous waste sites.
  11. pesticides.

Ref: 2004, lancet – spina bifida.”

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17
Q

Spina Bifida Occulta:
Definition:
How common is it:
What are some clinical findings.

A
  1. Failure of fusion of the posterior elements of the vertebral body (usually L or LS region).
  2. Normal variant in 5-10% of population.

CLINICAL:

  1. pigmented nevus.
  2. angioma.
  3. hirsute patch.
  4. dimple or dermal sinus.
  5. normal neurological exam.
  6. sacral lipoma – rare.
  7. tethered cord – rare.
  8. occasional bowel and bladder symptoms.
  9. no hydrocephalus.
  10. no Chiari malformation.

Ref: Cuccurullo pg 782.

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18
Q

Prophylaxis for mother with 1 child with spina bifida and wants another child?

A 
Canadian guidelines (2007) SOGC (society of obstetrics and gynecologists of Canada):
5 mg/day 3 months prior to conception + 3 months post conception. Afterwards, 0.4 - 1 mg/day folic acid in a multivitamin onwards and postpartum period.

Ref: http://www.sogc.org/media/pdf/advisories/JOGC-dec-07-FOLIC.pdf

AAP (American Academy of Pediatrics):
4000 micrograms (4mg) one month prior to conception to end of 1st trimester.

Ref: Alexander Matthews pg 199-200.”

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19
Q

Predicting CP: POSTER

A

4/6 significant in predicting CP later in childhood:

P = posture and movement patterns
O = oral motor patterns
S = strabismus
T = tone of muscles
E = evolution of postural reactions / developmental milestones
R = reflexes - deep tendon, infantile, and plantar reflexes

Ref: http://pedclerk.bsd.uchicago.edu/page/cerebral-palsy, Levine Criteria - Levine 1980; Arch PMR 61: 385-9 (retrospective chart study of 60 children > 1 year)

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20
Q

Potential Complications of spina bifida – list 15.

Excess Fluid in spine?
Think about cord? Abnormal growth?
Bladder / Bowel / Kidney? Endo? 
MSK? Balance? Coordinate - Eye
Psychology?
A
  1. shunt malfunction (infection, blockage etc).
  2. Arnold-Chiari / Chiari II malformations.
  3. hydrocephalus.
  4. tethered cord.
  5. latex allergy.
  6. neurogenic bladder (hyper or hypotonic, depending on lesion)
  7. neurogenic bowel.
  8. endocrine (precocious puberty, short stature).
  9. MSK (charcot joint, scoliosis, contractures, spasticity)
  10. obesity (sedentary, decreased metabolic rate)
  11. cognition (decreased IQ, cocktail personality)
  12. behavior.
  13. osteoporosis.
  14. benign lumbosacral tumours (lipoma, fibrolipoma).
  15. diastematomyelia.
  16. syringomyelia.
  17. central respiratory dysfunction.
  18. impaired fine motor coordination/ataxia.
  19. pressure ulcers (typical areas, also gibbus deformity).
  20. impaired visual function (strabismus, nystagmus, lateral rectus palsy).
  21. GU (renal dysplasia, lower tract abnormalities).

Ref: Cuccurullo pg 784-786.

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21
Q

Patient with spastic CP:

(a) gait abnormalites at hip, knee, ankle in hemiplegic and diplegic patient?
(b) future problems at hip, knee, ankle

A

HEMIPLEGIC:

  1. head + trunk: lateral flexion towards hemiplegic side.
  2. pelvis: anterior tilt + retraction, increased lumbar lordosis.
  3. hip: adducted, internally rotated.
  4. knee: variable - genu-recurvatum stiff, crouch, jump.
  5. ankle: plantar flexion + inversion + toe flexion.

DIPLEGIC:

  1. pelvis: anterior tilt.
  2. hip: flexed, adducted, internally rotated.
  3. knee: flexed (crouch, stiff knee, jump knee).
  4. ankle: equinus or calcaneous.

COMPLICATIONS:

hip: flexion contracture.
knee: flexion contracture.
ankle: plantar-flexor contracture.

Ref: ? First principles.”

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22
Q

Myelomeningocele

What is it:
What location of spine does it affect mainly?
What are the clinical findings?
Is there a risk of hydrocephalus?

A
  1. bony defect of posterior elements of vertebral body with herniation of meninges and neural elements.
  2. 75% LS, rest thoracic or sacral, rarely cervical.
  3. Majority of spina bifida cystica.
CLINICAL:
1. motor paralysis.
2. sensory deficits.
3. neurogenic bowel and bladder.
4. hydrocephalus (>90%).
5. Chiari II malformation.
Ref: Cuccurullo pg 782.
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23
Q

Myelocele. Definition? Where does myelocele rank in terms of severity in spina bifida cystica?

A
  1. Failure of fusion of posterior elements of spine with cystic cavity in front of the anterior wall of the spinal cord (neural elements herniate out of defect).
  2. Most severe form.

Ref: http://emedicine.medscape.com/article/311113-overview

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24
Q

Meningocele

What is it:
What location of spine does it affect mainly?
How common is it?
Is there a risk of hydrocephalus?”

A

“1. Bony defect of POSTERIOR ELEMENTS of vertebral body with herniation of meninges & CSF (no spinal cord).

  1. 75% LS, rest thoracic or sacral, rarely cervical.
  2. <10% of spina bifida cystica.

CLINICAL:
1. +/- skin covering, incomplete coverage leads to CSF leak.
2. normal neurological exam.
3. no: hydrocephalus or chiari malformation.
4. need to follow.
Ref: Cuccurullo pg 782.”

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25
Q

List 6 surgical modalities are useful in CP.

A

NEUROSURGERY:

  1. selective dorsal rhizotomy.
  2. ITB pump placement.
ORTHOPEDICS:
3. Soft tissue releases.
4. tendon lengthening.
5. tendon transfers.
6. joint fusions.
7. osteotomies (de-rotation or angulation osteotomies). 
Ref: Cuccurullo pg 774-775.
  1. deep brain stimulation for seizures.
  2. ?spinal cord stimulation.
  3. corpus callosotomy.

Ref:?

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26
Q

List 5 etiologies or risk factors for developing cerebral palsy.

What is the number one risk factor/cause of CP?

Prenatal
Social/Economic - Substance - Genetics - Maternal

Perinatal
Premature - BW - Placenta - Infection - ICH

Postnatal
Trauma - Stroke - Anoxia

A

Number 1 risk factor: prematurity (< 32 weeks).

PRENATAL:

  1. intracranial hemorrhage.
  2. placental complications.
  3. toxins (iodine, mercury).
  4. teratogens.
  5. congenital malformations.
  6. infections (Toxo, rubella, CMV, herpes).
  7. maternal causes (seizures, hyperthyroidism, mental retardation).
  8. socioeconomic factors.
  9. reproductive inefficiency.
  10. hypoxic-ischemic events (idiopathic, multiple pregnancies, maternal bleeding, drug use, trauma).

PERINATAL:

  1. prematurity (<32 weeks, low birth weight < 2500 grams, fragile brain structure, vulnerable vessels by germinal matrix).
  2. placenta previa.
  3. placental abruption.
  4. meconium aspiration.
  5. hyperbilirubinemia (Rh incompatibility, G6PD deficiency, ABO incompatibility).
  6. trauma from delivery (SDH).
  7. infection (viral, bacterial).
  8. seizures.
  9. perinatal ICH.

POSTNATAL:

  1. trauma (fall, child abuse, MVC).
  2. toxins (heavy metals, lead, organophosphate).
  3. strokes (sickle cell, AVM rupture, tetralogy of fallot).
  4. infection (bacterial, viral, meningitis).
  5. cancers.
  6. anoxia (near drowning, etc).
  7. ICH.

Ref: Cuccurullo pg 761-62.

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27
Q

List 4 causes of toe walking in 4 year old.

A
  1. cerebral palsy.
  2. autism.
  3. idiopathic.
  4. congenital or post-traumatic limb-length discrepancy.
  5. congenital muscular dystrophy (DMD, BMD).
  6. tethered cord syndrome.
  7. diastematomyelia.
  8. schizophrenia.
  9. global developmental delay.
  10. charcot-marie-tooth disease.
  11. spina bifida.
  12. transient dystonic reaction.
  13. venous malformation of posterior calf muscle.
  14. ankylosing spondylitis.

Ref: 2012 J Am Acad Orthop Surg 2012;20:292-300.
Peds rehab textbook pg 191.

  1. CNS neoplasm.
  2. LGMD2A and 2B.
    Peds rehab textbook pg 396.
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28
Q

List 4 causes of tethered cord in spina bifida

A
  1. scar tissue (post-surgical repair).
  2. di-astemato-myelia.
  3. super-numerary(excessive) fibrous bands.
  4. tight/short/thick filum terminale.
  5. lumbosacral tumour entrapment.
  6. persistent membrane reunions.
  7. dural sinus.
    Ref: Peds rehab principles practice textbook pg 206.
  8. Lipomyelomeningocele (Ref?).
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29
Q

List 2 good prognostic indicators for ambulation in CP.

List 2 poor prognostic factors for ambulation in CP.

A

GOOD PROGNOSTICS:

  1. sitting independently by 2 years.
  2. crawl hands/knees by 1.5-2.5 years.
  3. supine to prone by 18 months (spastic diplegics).

POOR PROGNOSTICS:

  1. persistent primitive reflexes (3 or more) at 18 months.
  2. unable to sit by 4 years.
  3. severe cognitive impairment.
  4. quadriparetic CP.

SUBTYPES:

  1. Hemiplegic and ataxic: 100% ambulatory.
  2. Atonic CP: usually not ambulatory.
  3. Quadriplegic/diplegic/dyskinetic: variable.
  4. diplegic: 80-90% ambulatory.
  5. Quads: 50% ambulatory.
  6. dyskinetic: 75% ambulatory.

Ref: Delisa pg 1484; Braddom pg 1259.
Ref: Prognosis for ambulation in Cerebral Palsy: a Population based study. Wu Y. Et el. November 2004. 14(5): 1264-1271.

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30
Q

Is spina bifida more common in males or females?

A

Female > Male.
Ref: Braddom pg 1276.
About 3:2 ratio. ?Ref.

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31
Q

How do you monitor the hip in spastic quadriplegic CP

A
  1. Risk of subluxation/dislocation of hips increases with GMFCS (90% of grade 5, 0% grade 1).
  2. PHYSICAL EXAM: passive hip abduction <35 degrees or hip flexion contracture >20 degrees at risk.
  3. XR hips (?18 months or at CP diagnosis?).
  4. migration percentage of hip > 30% = hip subluxation.

Ref: Alexander Matthews pg 174.

ETIOLOGY:

  1. persistent anteversion
  2. dysplastic acetabulum
  3. muscle imbalance / overactivity

GMFCS 1: initial 12-24 m, review @ 3, 5 y/o.
GMFCS 2: initial 12-24 m, review q12m until stable, review 4-5, 8-10 y/o.
GMFCS 3, 4, 5: initial 12-24 m, review q6m if not stable, q12m if stable.

Ref: ?

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32
Q

How common is CP?

How does this compare with spina Bifida?

A

1-2.3/1,000 live births for CP.

Ref: Braddom pg 1253.

In spina bifida, varies widely depending on geographical location.
1-2/1,000 births.

Ref: Delisa pg 1496.

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33
Q

GMFCS: gross motor functional classification system

(a) how many levels
(b) 2-key features of stage 1

A

(a) 5 levels:
1. walks without limitations
2. walks with limitations
3. walks with handheld mobility devices
4. self-mobility with limitations, may use powered mobility
5. transport in manual chair

(b)
1. walks indoors, outdoors, and on stairs without limits
2. no assistive devices
3. can run and jump
4. decreased speed, balance and coordination

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34
Q

GMFCS 4, list 3 aids/mobility devices

A

GMFCS 4: mainly wheelchair dependent (preferred mobility). May walk short distances indoors.

  1. Walker with body support (standing frame).
  2. Manual wheelchair.
  3. Power wheelchair.

Ref: GMFCS-ER document.

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35
Q

For cerebral palsy, when does the non-progressive insult to the brain occur?

A

3 years.

“There is no explicit upper age limit specified for the onset of the disturbance of brain development. The
first 2 or 3 years of life, however, are identified as the crucial period for insults resulting in CP.”

Ref: Braddom.

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36
Q

First child had CP, what is risk of 2nd child having CP?

A
  1. Depends upon the cause of the CP (whether there are ongoing risk factors).
  2. Prevalence: 3.6/1000
  3. PRENATAL: maternal mercury exposure, maternal iodine exposure, pre-term birth, multiple gestation, male, pre-natal strokes, placental abnormalities.
  4. PERINATAL: low APGAR score, intrauterine growth restriction, intrauterine infection (TORCH + others), maternal thyroid abnormalities, birth asphyxia, pre-natal stroke, trauma
  5. POSTNATAL: trauma, infection, ICH, coagulopathy, stroke, neoplastic, anoxic injury.

Ref: Cuccurullo pg 761.

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37
Q

Describe potential musculoskeletal abnormalities of the upper extremity in CP.

A
  1. SHOULDER: adducted and internally rotated
  2. ELBOW: flexion contractures (spasticity in biceps, brachoradialis, brachialis); less than 30 degrees rarely have functional significance
  3. WRIST: flexion and ulnar deviation
  4. FINGERS: flexion and swan neck deformities (due to hand intrinsic muscle spasticity)
  5. THUMB: thumb-in-palm deformity with adduction at CMC joint (may be associated with hyperextension of MCP and IP joints)

Ref: Alexander and Matthews p. 175”

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38
Q

Describe potential musculoskeletal abnormalities of the spine in CP.

A
  1. Kyphosis - often seen in combo with significant weakness of spinal extensors and tightness of hamstrings, leading to posterior pelvic tilt
  2. Lordosis - frequently associated with hip flexion contractures
  3. Scoliosis - likelihood increases with severity of CP (overall incidence = 20%, as high as 68% in spastic quad); curves > 40 degrees tend to progress.

Ref: Alexander and Matthews p. 174-5.”

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39
Q

Describe potential musculoskeletal abnormalities at each joint of the lower extremity, in a child with spastic CP:

Hips, 
Knees, 
Leg, 
Feet, 
Ankles.
A

HIPS:

  1. hip dysplasia,
  2. hip dislocation,
  3. hip osteoarthritis,
  4. femoral anteversion,
  5. windswept deformity (imbalanced muscles, adductors, etc).
  6. flexion contractures.

KNEES:

  1. flexion contractures (spasticity in hamstrings and static seating),
  2. patella alta,
  3. genu valgus (associated with excess femoral anteversion)

LEGS: tibial torsion ?reference

FEET: hallux valgus (leading to painful bunion at head of first metatarsal)

ANKLES:
1. equinus (due to tone or contractures in gastrocsoleus) is most common MSK deformity in CP.
2. equinovarus most common in hemiparetic (due to spasticity of post tib and gastrocsoleus - inversion and supination of foot, tight heel cord);
3. equinovalgus more common in older children with spastic diparesis and quadriparesis (due to spasticity of gastrocsoleus and peroneals and weak post tib).
Ref: Alexander and Matthews p. 173-4”

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40
Q

Define myelodysplasia and list 4 different types.

A

Myelodysplasia = group of neural tube deficits caused by congenital malformations of the vertebral column and spinal cord. Essentially neural tube Defects.

Primary neurulation is completed by about 25th day post-conception.
Ref: 2004 Lancet – Spina bifida.

Spina bifida OCCULTA: failure of fusion of posterior elements of vertebrae.

Spina bifida CYSTICA:

  1. Meningocele: protruding sac includes meninges and spinal fluid.
  2. Myelomeningocele: protruding sac includes meninges, spinal fluid, and spinal cord.
  3. Myelocele: cystic cavity in front of anterior wall of spinal cord.

Ref: Cuccurullo pg 781-82.”

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41
Q

CP neuroimaging findings

A
  1. PVL (periventricular leukomalacia): premature, di > quad > hemi
  2. focal ischemia, hemorrhage: MCA strokes, periventricular venous stroke, parasagittal
  3. generalized encephalomalacia: multiple cysts, cortical thinning, white/grey matter loss, microcephaly.
  4. BG/thalamic lesions: dyskinetic, kernicterus.
  5. brain malformations: lissencephaly, polymicrogyria, schizencephaly, holoprosencephaly.

Ref: Alexander and Matthews pg 168-69.

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42
Q

CP management – list 5 potential treatment options.

  • Think about systems involved

PT x3
OT x3

A
  1. exercises aimed at functional participation
  2. progressive resistance strength training
  3. night splinting
  4. passive stretching
  5. CIMT (Bobath not recommended)
  6. goal based ADL training
  7. weight bearing GMFCS 4+5
  8. delay leg + spine progression
  9. bowel + bladder function
  10. social programs
    Ref: first principles.
  • Think about systems involved
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43
Q

CP associated conditions

Definition + Resp + MSK

A
  1. cognitive impairment - severity proportional to motor + other deficits
  2. psychological disturbance - anxiety, depression, conduct disorder, ADD
  3. seizures - quad > hemi > di, onset 1st 2 years
  4. sleep disturbance
  5. visual impairments / ocular problems
    - strabismus = most common
    - amblyopia, refractory errors, visual field deficits, visual perceptual dysfunction, retinopathy of prematurity, congenital cataracts
  6. hearing loss
  7. sensory loss - stereognosis, 2-point discrimination, proprioception; uni > bi
  8. oromotor dysfunction - dysphagia, drooling, feeding difficulties
  9. language impairment - dysarthria
  10. malnutrition - can cause growth failure
  11. oral + dental health problems
  12. GI - reflux, constipation
  13. GU
    - primary enuresis, detrusor overactivity, small bladder
    - 80% di/hemi, 54% quad continent by 6
  14. pulmonary - bronchodysplasia, aspiration, sleep disordered breathing
  15. bone health
    - GMFCS 4/5 osteopenia by 10
    - risks: severity of neuro deficit, feeding difficulty, anticonvulsants, lower triceps skin-fold z-score
  16. scoliosis
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44
Q

CP – what are risk factors for progression of scoliosis.

Think about
Curve - sitting - young - muscle high low tone - pelvis

A
  1. quadriparesis.
  2. increased spasticity.
  3. larger curve – greater than 40 then to progress no matter the age.
  4. younger age (earlier Risser and Tanner stages).
  5. poor sitting balance.
  6. pelvic obliquity.

Ref: Alexander and Matthews (which references Renshaw et al. Instr Course Lect 1996; 45: 475-490)

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45
Q

Child with spina bifida and achilles lengthening 2 years ago.
Crouched gait - 3 lower limb MSK causes?

A
  1. Achilles lengthening too long
  2. Knee flexion contractures
  3. Weak gastroc-soleus

First principles.

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46
Q

Child with spina bifida and a heel ulcer - treatment?

A
  1. Offload heel (seating issues, wheelchair issues, offloading boots, etc).
  2. Keep eschar intact and dry.
  3. intrinsic factors (nutrition).

Think intrinsic and extrinsic factors from PU note in the SCI section notes

First principles.

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47
Q

A child with CP with hypersalivation and drooling. List 5 management options.

Treat
Stop
Re-Arrange
Adapt

A
  1. oral-motor therapy
  2. biofeedback (behavioural modification)
  3. glycopyrrolate
  4. benztropine
  5. scopolamine transdermal patch
  6. BoNT injection into salivary glands
  7. surgical resection of salivary glands
  8. rerouting of salivary ducts

Ref: Braddom pg 1260.

  1. suction
  2. wads of tissue

(saw patient in neuromuscular clinic with ALS doing this… an example of poor secretion management)”

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48
Q

4 year old boy: toe-walking, scissoring, hypertonic legs > arms, 20 degrees passive hip ABD, equinovarus deformity in gait but passive ankle movement just past neutral
1. diagnosis.
2. List 5 treatments to improve ambulation
List 5 Tx for toe walking / spastic walk.

A

Diagnosis: spastic diparetic cerebral palsy most likely.

  1. CONSERVATIVE: stretching, heat, physiotherapy, r/o interfering factors eg shoes.
  2. BRACING: AFO (solid AFO covering MTP joint).
  3. GAIT AIDS: forearm crutches, walker.
  4. PHARMACOLOGICAL: anti-spastic meds (baclofen, dantrolene, tizanidine etc).
  5. INJECTION: BoNT injections.
  6. SURGICAL: tendon lengthening (post tib), muscle release (adductors etc), Selective dorsal rhizotomy, ITB.
    Ref: first principles.
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49
Q

4 y/o male: spastic diplegic CP, sits independently, can rise from ground, needs wall or table to pull-to-stand, ambulates independently indoors without aid. Outdoors can ambulate on level ground, stairs with railing, but cannot run or jump.

  1. GMFCS level
  2. List 2 predictions about ambulation at 12 y/o
A

GMFCS 2 (ambulates with limitations).

  1. minimal ability to run or jump.
  2. difficulty walking on uneven terrain.

Ref: GMFCS – ER document.

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50
Q

8 y/o male: spastic quad CP seen at the seating clinic. Patient has significant tone, resting position hips and knees >90, scissoring, requires lateral support. Current chair = sling seat + back.
(a) list 4 possible MSK complications

MSK complications in wheelchair.

A
  1. contractures - hip flexion, knee flexion.
  2. scoliosis.
  3. pelvic obliquity.
  4. hip subluxation / dislocation.
  5. increased spasticity.
  6. pressure ulcers.
  7. pain.

Ref: First principles.

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51
Q

List 3 most common vision abnormalities in CP other than strabismus

A
  1. Refractive errors (50%)
  2. amblyopia (15%)
  3. visual field defects (11%)

Ref: British Journal of Ophthalmology 1982, 66, 46-52.

  1. strabismus – exotropia, esotropia (50% in diplegic CP).
  2. homonymous hemianopsia.
  3. impaired accommodation
    .
    Ref: Phys Med Rehabil Clin N Am 18 (2007) 859–882.
  4. CVI (cortical visual impairment).

Ref: Braddom pg 1260.

  1. amblyopia.
  2. nystagmus.
  3. refractory errors.

Ref: Delisa pg 1484.

  1. Congential cataracts.
  2. Retinopathy of prematurity.
    ?Ref:
    Memory aid: cortex to eyes (cortical, perceptual, visual field, retina, lens (cataracts, refractor), amblyopia, nystagmus”
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52
Q

Two brothers ages 8 and 10 with: spasticity, choreaoathetosis, self mutilation. Diagnosis?

A

Lesch-Nyhan syndrome:

  1. rare X-linked recessive (males).
  2. excessive uric acid production.
  3. neurological dysfunction: mental retardation, behavioural problems, movement disorders.
  4. self mutilation (behavioural problems – pts may chew off their own lips and fingertips).

Ref: Emedicine; wikipedia.

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53
Q

List 1 key feature of the following diagnoses:

a. Neiman-Pick Disease.
b. Rett Syndrome.
c. Adrenoleukodystrophy.

A

Neiman-Pick:

  1. AR, fatal metabolic disorder (sphingomyelinase deficiency) - lipid accumulation in organs leading to dysfunction.
  2. various types (A, B, C, D) with varying prognoses (death age 2-3 in type A, live to adulthood in B).
  3. multi-system dysfunction: neurologic, intra-abdominal organ dysfunction.
    * Lipids can accumulate anywhere, and cause neuro dysfunction depending on where

Rett syndrome:

  1. X-linked disorder (only females affected, as fatal in males).
  2. small hands + feet, microcephaly, repetitive hand movements.
  3. neuro: apraxia, seizures, flaccid weakness.
  4. course fluctuates – may progress to teens, then improve.
    * Girl name SAM (seizures, apraxia, microcephaly)

Adrenoleukodystrophy:

  1. rare x-linked disorder of impaired metabolism of VLCFA (very long chain fatty acids).
  2. males affected – females are carriers or mild disease.
  3. leading to progressive brain damage 2nd to adrenal failure (adrenal crisis).
    * multiple presentations, but severe ones have progressive dementia

Ref: Google, various NCBI websites.

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54
Q

Six month old with metatarsus adductus, failed gentle ROM:

(a) next treatment
(b) what associated MSK condition to rule out?

A

DEFINITION:

  1. medial angulation of forefoot relative to hindfoot.
  2. most common cause of intoeing in infants < 1 y/o, up to 1% of births.
  3. most resolve spontaneously (no treatment required).
  4. heel bisector line lateral to 2nd webspace.
    Ref: Cuccurullo pg 726; POSNA article on metatarsus adductus (search on google).
Mild = can easily overcome (observe, no bracing as can easily overstretch).
Moderate = corrects to neutral (passive stretching, can brace but not shown to be beneficial).
Severe = cannot correct to neutral (corrective casting, best if treated before 8 m/o, surgical correction controversial, rarely done before age 5).

(b)

  1. Congenital hip dysplasia (disproven
  2. Torticollis.

Ref: POSNA article on metatarsus adductus.

55
Q

List 3 causes of lateral trunk bending in pediatrics.

Xray of bend patient.

A
  1. Hip issue:
    - Legg-Calve Perthes
    - SCFE
    - Acute transient synovitis
  2. Scoliosis
  3. Pelvic obliquity
  4. Leg length discrepancy

Ref: Pediatric rheumatology 2009, 7:10.
Note: lateral bending may be related to pain in hip, which has much larger differential.

56
Q

Male, hip pain + frog-leg xrays

(a) possible causes of hip pain
(b) type of x-ray view
(c) most likely diagnosis

Common
Don’t Miss
Less Common

A
  1. fracture, stress
  2. dislocation
  3. AVN (Legg Calve Perthes, vasculitis, sickle-cell, steroids, lupus)
  4. SCFE
  5. osteomyelitis
  6. labral tear
  7. osteochondral defect
  8. transient synovitis
  9. neoplasm
  10. septic arthritis.
  11. developmental dysplasia of the hip.
  12. JIA.
  13. lyme disease.

b) X-ray view: Frog leg lateral view or Cassak dancer’s view.
c) SCFE most likely

57
Q

Most likely cause of hip pain in:

(a) 7 y/o child with hip pain
(b) 12 y/o obese boy with hip pain
(c) 2 y/o female, flu 2 weeks ago + normal serology
(d) 5 y/o no fever, normal serology

A

a) AVN - Legg-Calve-Perthes
b) SCFE
c) Transient synovitis
d) JIA

Other differentials:

  • septic arthritis
  • Lyme disease
  • osteomyelitis
  • bone tumor
  • steroids- I think with immuno supression leding to infection
  • developmental dysplasia of the hip
  • fracture +/- dislocation

GL/EK thoughts

58
Q

13 y/o girl hip + knee pain, limping, obese

(a) what is diagnosis
(b) 2 complications

A

SCFE (note: normally seen in males).

  1. AVN with collapse of head
  2. chondrolysis (degradation of cartilage)

Ref: http://orthoinfo.aaos.org/topic.cfm?topic=a00052

  1. decreased ROM – esp. internal rotation
  2. OA
  3. progressive slip
  4. contralateral involvement
  5. rotational deformity
    Ref:?
59
Q

Describe the 4 pathologic stages of Legg-Calve-Perthes disease.

AVNFOR

A
  1. Avascular necrosis.
  2. Fragmentation.
  3. Re-ossification.
  4. Remodeling.

Ref: http://www.childrenshospital.org/az/Site1231/mainpageS1231P1.html

Note: stage 4 has also been classified as ‘residual stage’. Ref: Chapmans orthopedics pg 4424.

  1. Avascular
  2. Subchondral fracture (““crescent sign””)
  3. Resorption
  4. Reossification
  5. Healed

Ref: Cuccurullo p136”

60
Q

List 4 characteristics of SCFE patients.

A
  1. Male > Female (2:1).
  2. adolescent age (M: 12-16, F: 10-14).
  3. Overweight.
  4. African American.
  5. pre-pubertal/delayed puberty.
  6. Associated with hypothyroidism, hypopit, hypogonadism, excessive GH.
    Ref: Ped Rehab textbook pg 285.
61
Q

Picture of girl sitting on heels with extreme hip IR:

  1. name the structural abnormality.
  2. other causes of in toeing.

Think about central causes - joints up to bottom.

A
  1. CP.
  2. W-sitting 2nd to increased femoral anteversion.
  3. developmental dysplasia of the hip.
  4. internal tibial torsion (thigh-foot angle: 0 to 10 normally).
  5. metatarsus adductus.
  6. club foot (plantar flexion + inversion + forefoot adduction + cavus foot).
  7. skew foot (medial forefoot deviation + lateral midfoot translation + valgus hindfoot).

Ref: uptodate.

General differential:

  1. neuromuscular disease
  2. hip disorders
  3. lower leg deformity
  4. foot deformity
62
Q

List 2 reasons for atlanto-axial instability in Down’s syndrome.

A
  1. congenital laxity
    absence of the C1 transverse atlas ligament.
  2. odontoid malformation.
  3. degenerative spondylosis.
  4. C1 hypoplasia (spinal canal stenosis).

Ref: emedicine www.http://emedicine.medscape.com/article/1180354-overview#aw2aab6b3

Atlanto-axial differential:

  1. RA
  2. ankylosing spondylitis
  3. trauma
  4. osteogenesis imperfecta
  5. Down’s
  6. Ehler Danlos
  7. Marfan
  8. URTI with erosion
  9. neurofibromatosis
    Ref?
63
Q

Two y/o girl, holding parents hands while walking, jumps down off ledge, father pulls up by hands. Complains of severe elbow pain, unwilling to move elbow.

  1. diagnosis
  2. treatment
A

Dx: Dislocation/subluxation of the radial head from the annular ligament (Nursemaid’s elbow).

REDUCTION:

  1. thumb on radial head arm extended.
  2. fully supinate.
  3. can additionally try with elbow flexion.
  • Most common elbow injury in children < 5 y/o.
  • Mechanism: elbow extended + forearm pronated + pull on forearm, radial head subluxes from under annular ligament and ligament become interposed between radius and capitellum.
  • XR of elbow usually read as normal.

Ref: Cuccurullo pg 730-731.

64
Q

List 4 primitive reflexes that disappear by 6 months of age

After 6 > Neck and Plantar
Before 6 > 6 Reflexes

A

Note: date is when reflexes disappear.

  1. Moro (4-6m).
  2. Rooting (4m).
  3. Palmar grasp (5-6m).
  4. automatic neonatal walking (3-4m).
  5. tonic labyrinthine (4-6m).
  6. positive supporting (3-5m).

Ref: Cuccurullo pg 779.

  1. Galant (6 m).

REFLEXES THAT DISAPPEAR AFTER 6m:

  1. asymmetric tonic neck reflex (6-7m).
  2. symmetric tonic neck reflex (6-7m).
  3. plantar response (1-2yrs).
65
Q

Describe the moro reflex.

A
  1. pt supine, head supported.
  2. sudden passive neck extension.
  3. infant should abduct shoulders and extend elbows, wrists, and fingers.
  4. Then the shoulders should adduct, with flexion of the elbows and fingers.
  5. Should disappear by 6 months.
66
Q

Describe the galant reflex.

A
  1. newborn held prone in examiner’s hand.
  2. stroke performed just lateral to spine.
  3. NORMAL: spine will laterally flex towards stimulated side.
  4. usually disappears by 6 months.
    Ref: wikipedia.
67
Q

Photo of supine infant, head rotated right, right arm extended, left arm flexed

(a) what primitive reflex
(b) when does it disappear?

A

Asymmetric tonic neck reflex (ATNR).
DATE IT DISAPPEARS:
6-7 m (Cuccurullo pg 779: references Peds rehab, Molnar).

68
Q

Developmental milestones – list ages.

(a) stacks 6 blocks
(b) copy a circle
(c) copy a triangle
(d) scribble spontaneously

A

(a) 2 y/o.
(b) 3 y/o (circle 3, cross/square 4, triangle 5).
(c) 5 y/o.
(d) 14 m/o

(Cuccurullo, pg. 718), 18 months (review notes).

69
Q

Describe the following Primitive reflexes:

  1. automatic neonatal walking
  2. positive supporting
  3. rooting
  4. Moro
  5. tonic labyrinthe
  6. palmar grasp
  7. asymmetric tonic neck reflex
  8. symmetric tonic neck reflex
  9. placing
  10. plantar / Babinski
  11. plantar grasp
A
  1. Automatic neonatal walking: vertical support plantar contact and passive tilting of body forward side to side -> alternating automatic steps with support (3-4 mo)
  2. Positive supporting: light pressure or weight-bearing on plantar surface -> leg extension (3-5 mo)
  3. Rooting: stroke lips or around mouth -> move mouth and head toward stimulus; look for nipple (4 mo)
  4. Moro (Startle): sudden neck extension, unexpected loud noise -> shoulder ABD and shoulder, elbow, finger EXT, then arm FLEX + ADD (4-6 mo)
  5. Tonic labyrinthe: head position in space, strongest at 45 degrees from horizontal (supine or prone) -> predominant extensor tone when supine, flexor tone when prone (4-6 mo)- superman
  6. Palmar grasp: pressure on palm or stretching finger flexors -> flexion of all fingers (5-6 mo)
  7. Asymmetric tonic neck reflex (fencing): turn head to side -> ext of arm + leg on face side, flex arm + leg on other side (6-7 mo)
  8. Symmetric tonic neck reflex: neck flexion -> arms flex + legs extend = commando position; neck extension -> arms extend, legs flex = crawling position (6-7 mo)
  9. Placing: tactile contact on dorsum of foot or hand -> extremity flexion to place hand or foot over obstacle (12 mo)
  10. Plantar: pressure on sole leads to extension of toes (12 mo)
  11. Plantar grasp: pressure on sole distal to metatarsal heads -> flexion of all toes (12-14 mo)

Ref: Cuccurullo pg 779

70
Q

List 4 physiologic postural reflex responses.

A
  1. head righting.
  2. head and body righting.
  3. protective extension tone/parachute reactions.
  4. equilibrium or tilting reactions.

Ref: Cuccurullo pg 716.

71
Q

Fine Motor Development: list the key findings for the months 0, 4, 7, 10, 14, 18, and years 2, 3, 4, and 5.

A

0m – grasp reflex.
4m – crude palmar, mid-line handplay.
7m – intermediate grasp, transfers hand-to-hand, bangs things.
10m – pincer grasp, bangs things in hands.
14m – piles 2 cubes, scribbles spontaneously, holds crayon full length in palm, uses spoon.
18m – emerging hand dominance, uses cup, holds crayon butt end in palm
2y – 6 cube tower, pencil between thumb + finger, dressing
3y – pencil by finger and wrist action, copies circle, throws overhand, un-buttons
4y – copies cross, draws square, scissors, throws underhand
5y – draws triangle, uses fork, catches with hand
Ref: Cuccurullo pg 717 – 720.

72
Q

2 m/o infant with: poor head control, weak cry, weak limbs, poor feeding, normal facial features
(a) most likely diagnosis

Weak baby

A
  1. SMA 1 (Werdnig-Hoffman Disease).
  2. Congenital myotonic dystrophy.
    Ref: Cuccurullo pg 801, 804.
73
Q

List 4 factors that increase the risk of hip instability after pediatric SCI

A
  1. septic arthritis.
  2. spastic paralysis.
  3. age at time of injury, younger worse (birth to age 10 - 93% will have unstable hip).
  4. autonomic dysreflexia.
  5. underdevelopment of acetabulum and femoral head.

Ref: McCarthy JJ and Betz RR. Hip disorders in children who have spinal cord injury. Orthop Clin N Am 37 (2006) 197 – 202

74
Q

List 4 findings in club foot (talipes equinovarus)

A
  1. small foot.
  2. medial border crease.
  3. hindfoot equinus (plantarflexion).
  4. forefoot & hindfoot varus (inversion).
  5. forefoot (metatarsus) adductus.
    Ref: Braddom pg 45.
  6. midfoot cavus (high medial longitudinal arch).
    Ref: 2012 – Cochrane review, interventions for talipes equinovarus.
75
Q

List 2 management methods for clubfoot (talipes equinovarus).

A
  1. Serial casting (Ponseti method)
  2. Surgical release (multiple methods and approaches)

Ref: Chapmans orthopedics textbook pg 4260

76
Q

Examination of metatarsus adductus

A

Metatarsus adductus is a forefoot deformity, so hindfoot alignment should be normal

  1. Forefoot adducted
  2. Lateral foot border convex
  3. Medial soft-tissue crease (this means more rigid deformity)
  4. Normal hindfoot and subtalar motion
  5. Heel bisector line (midline axis of hindfoot to forefoot; normally through 2nd toe webspace)
  6. Tickle foot for possible active correction

Also look for:

  1. Foot progression angle (foot off line of progression during gait, normally 6-10 ER) - this can come from tibial torsion too
  2. Alignment of patellas (femoral anteversion)
  3. Thigh-foot angle (tibial torsion)

Ref: multiple sources – Cuccurullo pg 726; uptodate; orthobullets; Pediatr. Rev. 2009;30;287-294.

77
Q

When do you worry about metatarsus adductus

A

Rigid metatarsus adductus (cannot be corrected back to neutral) - concern for possible tarsal coalition.

If can stand on toes with hindfoot moving into varus from valgus then windlass mechanism intact and forefoot + midfoot are flexible, therefore no tarsal coalition.
Ref:?

78
Q

Examination for tibial torsion?

A

Thigh foot angle

  • Prone with Knee flexed 90 degrees
  • Look at axis of thigh compared with heel bisector line

Normal: 0 to +10 degrees

Ref: Pediatr. Rev 2004;25;52; Wheeless

79
Q

Examination for femoral anteversion?

A

Craig’s test: Prone, IR and ER hip. Greater than 65° of IR = femoral anteversion.

Ref: Pediatr. Rev.2009;30;287-294.

80
Q

Why are 2-joint muscles more prone to spasticity?

A

More Ia afferent sensory fibers. Just like the H reflex

?Ref: quoted by Dr. Burkholder.

81
Q

Osgood-Schlatter management.

A

WHAT: overuse injury with apophysitis of the tibial tubercle (growth plate) 2nd traction of patella tendon.
WHO: adolescents, 11-13 y/o, 5x more in athletes.
CLINICAL: pain running, jumping, prolonged sitting with flexed knee.
PATHOLOGY: inflammation + microfractures of apophyseal cartilage.
XR: soft tissue swelling, small spicules of HO anterior to insertion.
DDx: infection, neoplasm, patella #, tibial tuberosity avulsion #.

TREATMENT:

  1. Education: Reassurance - self-limited condition, usually resolves once growth complete.
  2. Activity: relative rest (pain is guide to limitation of activity)
  3. Modalities: ice after activity, trial of electrotherapy x 2-3 treatments
  4. Exercise: stretching (tight quads may predispose to condition); strengthening as pain allows
  5. Meds: NSAIDs occasionally
  6. Surgery: only for avulsion #, non-union

Ref: Brukner Khan pg 734-35

?recalcitrant: occasionally immobilize in custom brace, ROM daily

82
Q

Sinding-Larsen disease:

(a) what is it
(b) what causes it

A

(a) apophysitis of the patella tendon insertion into the distal (inferior) pole of the patella.
(b) repetitive traction on the developing ossification center, causing microfracture and inflammation, likely 2nd to repeat extensor mechanism action.
Ref: Brukner Khan pg 736.

83
Q

what are the eponyms for myotonic dystrophy types 1 and 2?

A

DM1: Steinerts disease.
DM2: PROMM (proximal myotonic myopathy).
Ref: 2010 review, Turner et al, The Myotonic dystrophies: diagnosis and management

84
Q

Myotonic dystrophy mode of inheritance

A

DM1
autosomal dominant - 19q13
DMPK gene = myotonin protein kinase
CTG repeats: normal < 27, abnormal >50; more severe: 50-150, max up to 4000) Ref: Amato.
Note: CTG repeats > 37 are unstable and at risk of expanding (Ref: 2010 review, Turner et al).

DM2
autosomal dominant - 3q21
ZNF9 protein = zinc finger 9
CCTG repeats >75, up to 11,000!!!

Ref: 2010 review, Turner et al, The Myotonic dystrophies: diagnosis and management

85
Q

List 3 MSK complications of pediatric SCI

A
  1. osteoporosis / osteopenia
  2. hip subluxation / dislocation / dysplasia
  3. fragility / pathologic fractures
  4. HO
  5. scoliosis
  6. contractures
  7. spasticity
  8. muscle atrophy?
  9. pressure ulcers
  10. AVN
  11. septic arthritis
    Ref: First principles
86
Q

Describe the differences between Type 1 and 2 Chiari Malformations

A

Type 1

  • herniation of cerebellar tonsils through the foramen magnum.
  • compression of cerebellar tonsils + disrupted CSF flow + cervicomedullary compression.
  • most common, least severe.

Type 2 = ‘Arnold-Chiari’

  • herniation of lower brainstem, medulla, pons, 4th ventricle, cerebellar vermis through the foramen magnum.
  • symptomatic in infancy or early childhood, < 1/3 are symptomatic.
  • less common, more severe.
  • symptoms 2nd to brainstem + lower CN dysfunction.
  • invariably associated with myelomeningocele.
87
Q

Congenital limb deficiency with nubbins:

(a) 2 advantages of amputation
(b) 2 disadvantages of amputation

A

Advantages:

  1. better prosthetic fit
  2. improved cosmesis
Disadvantages:
1. neuropathic / phantom pain
2. loss of sensory feedback
3. surgical risks (bleeding, infection, etc).
Ref: First principles.
88
Q

Describe the neonatal management for the following issues in spina bifida:

  1. back defect.
  2. hydrocephalus.
  3. bladder
A
  1. early closure of back defect (<72 hours) to prevent infection & preserve neuro fxn.
  2. hydrocephalus: 15% require immediate VP shunting, 85% monitor – head size, CT scans, symptoms).
  3. bladder: 90% have neurogenic bladder. Baseline renal U/S & voiding cystourethrogram (prophylactic ABX if either hydronephrosis or reflux found); intermittent cath if not voiding; PVR if voiding to check for complete emptying.
    Ref: Braddom pg 1279-80.
89
Q

List 8 possible symptoms of shunt malfunction in VP shunt (spina bifida).

A

GENERAL:

  1. vomiting.
  2. lethargy.
  3. headaches.
  4. seizures.
  5. redness along shunt tract.

INFANTS:

  1. bulging fontanelles.
  2. irritability.
  3. change in appetite.

SCHOOL-AGED:
9. decreased school performance.

Ref: Braddom pg 1280

90
Q

List 5 shunt complications

A
  1. shunt/shunt tract infections.
  2. blockage
  3. peritoneal catheter/peritoneal end malfunction.
  4. extrusion of peritoneal catheter through anus.
  5. ventricular catheter malfunction.
  6. cerebrospinal fluid (CSF) leak from abdominal wound.
  7. shunt system failure.
  8. ventricular end/shunt displacement.
  9. CSF pseudocysts peritoneal cavity.
  10. extrusion of peritoneal catheter from neck, chest, abdominal scar and through umbilicus.
    Ref: Afr J Paediatr Surg. 2012 Jan-Apr;9(1):32-9
  11. drainage of cerebrospinal fluid into the tracheobronchial tree.
    Ref: Neurosurgery: January 1994 - Volume 34 - Issue 1 - p 181-184. Case Report, Thoracic Complications of Ventriculoperitoneal Shunts: Case Report and Review of the Literature.
  12. colon perforation.
    Ref: PEDIATRICS Vol. 54 No. 6 December 1, 1974 pp. 791 -796.
  13. vaginal performation.
    Ref: Journal of Neurosurgery June 1973 / Vol. 38 / No. 6 / Pages 761-762.
91
Q

What is hydromyelia and how do you treat it?

A 
Dilation of the central canal of the spinal cord.
CLINICAL:
1. rapid scoliosis
2. weakness
3. decreased coordination
4. spasticity

Treat hydrocephalus first, the hydromyelia often improves
Ref: Braddom pg 1281.

92
Q

List 5 clinical features of tethered cord.

List 4 causes of tethered cord.

A

CLINICAL:

  1. weakness.
  2. gait abnormalities.
  3. scoliosis.
  4. pain (NOTE: if painless, think syrinx).
  5. orthopedic deformity.
  6. urologic dysfunction.
  7. sensory deficit.
  8. cutaneous findings
  9. increasing spasticity.
  10. contractures.
ETIOLOGY:
1. spina bifida (myelomeningocele).
2. diastematomyelia.
3. lipomyelomeningocele.
4. tight filum terminale.
Ref: Braddom pg 1281; alexander matthews pg 206.
93
Q

How prevalent is latex allergy in spina bifida?

A

20-65% prevalence in spina bifida.
Avoid all latex products in all spina bifida throughout life, as can develop later.
Ref: Braddom pg 1282.

94
Q

What endocrine disorders are associated with spina bifida? (Add on: what neuroendocrine disorders can occur in TBI?

A
  1. central precocious puberty
  2. GH deficiency

In TBI: SIADH, cerebral salt wasting, diabetes insipidus, and precocious puberty.
Ref: Braddom pg 1281.

95
Q

List 4 musculoskeletal complications associated with spina bifida.

A
  1. UMN and/or LMN syndrome (mixed most common).
  2. hips: contracture, subluxation, dislocation.
  3. knees: flexion or extension contractures.
  4. feet: equinus, equinovarus, cavus, rocker bottom deformity, rigid clubfoot.
  5. spine: scoliosis.
  6. pathological fractures: osteopenia, insensate, contractures, immobilization.
    Ref: Braddom pg 1285-86.
96
Q

Scoliosis in spina bifida. Causes? Predictors? What to think about should there be rapid progression?

A

CONGENITAL:

  1. vertebral body abnormality
  2. congenital kyphosis
  3. diastomatomyelia

NEUROMUSCULAR:

  1. weakness
  2. hydromyelia / syringomyelia

PREDICTORS FOR SCOLIOSIS:

  1. ambulatory status.
  2. motor level (90% of thoracic SB have scoliosis).
  3. last intact lamina arch.

RAPID PROGRESSION:
1. tethered cord
2. hydromyelia / syringomyelia
Ref: Braddom pg 1285.

97
Q

How do scoliosis curves in CP compare to idiopathic scoliosis in adolescents?

A
  1. curves occur earlier
  2. larger, stiffer curves
  3. more likely to progress after maturity
  4. more left curves
  5. greater saggital deformity
98
Q

What is the natural history of scoliosis in CP? What are indications for instrumentation?

A
  1. bracing does not alter natural history, but can:
    - improve sitting balance, and
    - delay timing of surgery
  2. indications for instrumentation:
    - curve > 50
    - progression > 10
    - deterioration of function 2nd to scoliosis
  3. course:
    - negative correlation between ambulation and spinal deformity
    - variable progression, can progress into adulthood
    - curves > 50 progress into adulthood
99
Q

How does Myelomeningocele scoliosis progress?

A
  1. the higher the defect the more likely scoliosis (thoracic spina bifida has scoliosis in about 90% of patients – rare in sacral lesions).
  2. progression:
    <20 degrees, slow progression
    >20 degrees, fast progression
    * if rapid progression of otherwise stable curve, think about hydromyelia or tethered cord

Predictors:

  1. ambulatory status
  2. clinical motor level
  3. last intact laminar arch

Ref: Braddom pg 1285.

100
Q

Differences of TBI in children (vs. Adults) – list 5.

A

5 Ss

  1. Shearing
    - incomplete myelination therefore increased shear
    - increased head to trunk mass ratio therefore increased shear injury
  2. Swelling
    - high brain water content (increased risk cerebral edema).
    - diffuse swelling more common 2nd to: increased excitotoxic transmitter diffusion, increased inflammatory response, increased BBB permeability.
  3. Second Impact Syndrome
  4. Skull #
    - growing skull # – linear skull # + dural tear with formation of cyst that erodes bone.
  5. Shaken baby syndrome
  6. Others:
    - increased % of high energy mechanisms, more associated injuries
    - weak neck musculature
    - different biological response, open vs.. closed sutures.
    - different ICP + CPP normals (varies with age).

Ref: first principles, and Peds rehab textbook pg 232-233.

101
Q

What are the most common congenital limb deficiencies?

A 
#1 L/E = longitudinal fibular deficiency (others: partial longitudinal femoral deficiency, total/partial longitudinal tibial)
Ref: Alexander Matthews p347.
#2 U/E = transverse deficiency of upper 1/3rd of forearm
Ref: Alexander Matthews p340.

“Braddom P257 says
 upper limb are more common (58%),
 The most common congenital amputation is at the left short transradial level.

102
Q

What is the typical positioning of the residual limb in proximal focal femoral deficiency (PFFD)?

A
  1. hip flexion.
  2. abduction.
  3. external rotation.
    Ref: Alexander matthews pg 348.
103
Q

List 4 risk factors for congenital limb deficiencies.

A
  1. maternal diabetes
  2. smoking
  3. thalidomide
  4. amniotic band syndrome(starnds of amniotic sac constricting a limb )
  5. maternal vitamin deficiency
    Ref: Alexander Matthews pg 335-336.
104
Q

Describe the ISPO classification of congenital limb deficiencies.

A

TRANSVERSE (all bones after the named segment are absent, partial or total).
1. estimate length of residual bone in thirds (eg. 1/3 distal radius transverse deficiency).
LONGITUDINAL (preservation of bones distal to anomalies).
1. Named bones = absent.
2. Bones not named = present and normal form.
3. Complete absence (bone is not present).
4. Partial absence (components of bone present, long bones in 1/3’s).
Ref: Alexander Matthews pg 336.

105
Q

List two key components of Moebius syndrome.

A
  1. craniofacial abnormalities (eg. Cleft lip).
  2. upper limb deformities (eg. Congenital absence of fingers).
    Ref: Alexander Matthews pg 336.
106
Q

When would you consider fitting a child with a congenital lower limb prosthesis?

A

When the child is ready to pull up to standing position (usually 9-10 months).
Ref: Alexander matthews pg 353.

107
Q

List 4 disorders associated with hereditary limb deficiencies.

A
  1. TAR = thrombocytopenia + absent radius
  2. Fanconi’s = anemia + leukopenia (radial def)
  3. Holt-Oram = congenital heart disease (atrial septal defects, tetralogy of fallot)
  4. Baller-Gerold = premature closure of cranial sutures (horizontal head growth)
  5. VACTERL = multiorgan (vertebral defects, anal atresia, cardiac defects, transesophageal fistula, renal dysplasia, limb deficiency).
    Ref: Cuccurullo pg 722.
  6. Robert’s syndrome = radial deficiency, radial-sided digits.
  7. Cornelia de Lange syndrome (ulnar def)
  8. Ulnar-mammary syndrome
  9. Ulnar fibula dysplasia
    Ref: Alexander matthews p341.
108
Q

What is the most common type of malignancy in children?

A
  1. Leukemia (30%).
  2. CNS tumours (19% - second most common).
    Ref: Cuccurullo pg 753.
109
Q

What is the most common presentation for Hodgkins lymphoma in children?

A

painless cervical adenopathy.

Ref: Cuccurullo pg 753.

110
Q

Where is Burkitt’s lyphoma seen? What locations does it usually affect?

A

Africa (?due to EBV)

  1. facial bones and mandible
  2. abdominal nodes and viscera
  3. massive proliferation of primitive lymphoid cells

Ref: Cuccurullo pg 754.

111
Q

What tumour in children arises from the sympathetic ganglia and adrenal medulla? What age group does it usually affect?

A

neuroblastoma.
2 years old has highest incidence. Usually affects children < 5 years old.
Ref: Cuccurullo pg 754.

112
Q

What organ does Wilms tumour affect? What age group is usually affected?

A

Renal parenchyma (ie. Nephroblastoma).
2-5 years.
Ref: Cuccurullo pg 754.

113
Q

What are the 3 most common causes of abdominal masses in children?

A
  1. wilm’s tumour (nephroblastoma)
  2. neuroblastoma
  3. hydronephrosis

Ref: Cuccurullo p754

EK thoughts: depends on age (neonatal, infants); renal masses are most common, but Wilm’s is pretty rare overall compared to hydronephrosis, polycystic kidney, etc.

114
Q

What is the most common malignant soft tissue tumour in children?

A

rhabdo-myo-sarcoma.
Diagnosis is based on biopsy.
Associated with neurofibromatosis.
Ref: Cuccurullo pg 754.

115
Q

Of the two most common malignant bone tumours in children, which one has a predilection for the metaphysis, and which one has a predilection for the diaphysis?

A

Osteosarcoma – metaphysis (distal femur > proximal tibia > proximal humerus)
Ewings sarcoma – diaphysis and flat bones (eg pelvis).

Memory aid, eWING… the wings on the ischial bones…??

Ref: Cuccurullo pg 754.

116
Q

What is the leading cause of death in children > 1 year of age?

A

TBI.
10/100,000 die per year in US from TBI.
Second most common is leukemia.
Ref: Cuccurullo pg 755.

117
Q

What are 4 signs of nonaccidental trauma?

A
  1. retinal hemorrhages
  2. Multiple fractures (at various stages of healing).
  3. Multiple injuries
    Ref: Cuccurullo pg 756.
  4. transverse metaphyseal fracture near growth plate (femur, tibia, humerus).
  5. unusual locations of fractures (posterior rib, sternum scapula).
  6. subdural hematomas (esp multiple).
  7. spiral fracture of long bones.
    Ref: Secrets, pg 660.
  8. Delay in seeking medical attention
  9. History incompatible with severity of injuries
    Ref: Alexander and Matthews p233

*Clinical Triad: Subdural hemorrhage, Retinal hemorrhage, Encephalopathy (Ref: Alexander and Matthews p233)

118
Q

How does the treatment of HO in children differ than adults?

A

Should not use etidronate in children, as associated with reversible rachitic syndrome in growing children.
Ref: Cuccurullo pg 760.
Arch Phys Med Rehabil. 1994 Jan;75(1):118-20.

119
Q

What is the acetabular index? What is it used for?

A 
Used in pediatric hip x-rays to assess developmental hip dislocation.  It is the angle formed by:
1. a line through the acetabular roof
2. Hilgenreiner's line
Normal is <25 degrees.
Ref: Alexander matthews pg 388.
120
Q

What is the only primitive reflex that is not present at birth and that is also absent at 1 year of age?

A

Symmetric tonic neck reflex (STNR).

Ref: PMR Secrets pg 657.

121
Q

what is codman triangle? What pediatric conditions is it seen in?

A

A triangular area of new subperiosteal bone formation when a lesion (eg tumour) raises periosteum away from bone.

  1. osteosarcoma
  2. ewing’s sarcoma

Ref: Wikipedia

122
Q

What is the definition of diastematomyelia?

A

Diastematomyelia is a post-neurulation defect that results in a SAGGITAL CLEAVAGE of the spinal cord, most commonly affecting the lumbar and thoracolumbar levels.
Ref: Alexander and Matthews pg 206.

Diastematomyelia is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra.

Diastematomyelia is a rare congenital anomaly that results in the ““splitting”” of the spinal cord in a longitudinal (sagittal) direction.
Ref: Wikipedia.

123
Q

How common is duchenne muscular dystrophy DMD?

A

1 in 3600-6000.

Ref: Lancet Neurol2010; 9: 177–89.

124
Q

At what age are children with Duchenne muscular dystrophy usually diagnosed?

At what age do children with duchenne DMD usually lose independent ambulation by?

A
  1. 5 years of age, when physical ability diverges from peers markedly.
  2. earlier diagnosis if clear family history.
  3. usually lose ability to ambulate independently by 13 years old.
    NOTE: age of loss of ambulation is without steroids – with steroids, boundaries are less distinct.
    Ref: Lancet Neurol2010; 9: 177–89
125
Q

What is the only disease modifying treatment for duchenne muscular dystrophy?

A

Steroids (Deflazacort and prednisone)

  • Deflazacort 0.9 mg/kg/day, OR
  • Prednisone 0.75 mg/kg/day

Shown to:

  1. Increase strength, timed muscle function, and pulmonary function
  2. Slows decline in muscle strength and function

Ref: 2006 – Continuum – muscular dystrophies article; Lancet Neurology 2010

126
Q

Hyperlordosis in Duchenne’s:

(a) cause
(b) what is compensation

A

Myopathic Gait

CAUSE:
- weakness of back and hip extensors, causing anterior pelvic tilt (trunk is thus positioned anteriorly to hip)

COMPENSATION:

  • compensation by lumbar hyperlordosis moves COG posterior to hip and stabilizes hip in extension
  • weak hip extensors also cause knee instability, so compensate by decreasing knee flexion and toe-walking

Ref: Cuccurullo p790

127
Q

What is the natural history of scoliosis in Duchenne Muscular Dystrophy? When to suggest surgery?

A
  1. occur in 95%
  2. natural history = progression to > 100 degrees at 10/y after into wheelchair
  3. surgery before:
    - FVC declines at 4% per year with progression
    - ideally surgery before FVC < 40% predicted
  4. bracing - slows progression but does not halt,offer better seating balance for better function
  5. surgical:
    - fusion at 20-30 degrees
    - extend into upper thoracic area to avoid cephalad progression
    - extent to sacrum if pelvic obliquity > 10 or curve > 40 degrees

Ref: ?

Lancet Neurology 2010:

  • Curves <15-20 degrees -> AP film annually
  • Curves >20 degrees -> AP film q6mo
  • Spinal fusion to: straighten spine, prevent further worsening, eliminate pain due to vert #, slow rate of resp decline
  • Posterior spinal fusion in non-ambulatory with >20 spinal curvature, not on steroids, not yet mature
  • If pelvic obliquity >15, fuse upper-T to sacrum
  • If no pelvic obliquity, can fuse to 5th lumbar
128
Q

Why do you get pseudohypertrophy in the calves of kids with Duchenne muscular dystrophy?

A
  1. Unstable sarcolemma membrane from absent dystrophin results in breakdown of muscle fiber.
  2. As disease progresses dead muscle fibers are cleared away by macrophages and replaced by fatty and connective tissue elements, conveying a deceptively healthy appearance to the muscle (pseudohypertrophy), especially calves and forearms.
    Ref: Alexander matthews pg 279.
129
Q

9yo boy with DMD (duchenne), independent ambulator. What are 3 lower extremity contractures that he is at risk for developing?

A 
LOWER LIMB
1. ankle Plantar flexion.
2. knee flexion.
3. hip flexion.
4. iliotibial band tightness.
UPPER LIMB:
5. elbow flexion.
6. wrist flexion.
Ref: Alexander matthews pg 290.
130
Q

List 6 management issues/categories in duchenne muscular dystrophy.

A
  1. orthopedic management: scoliosis, contractures, etc.
  2. cardiac management: heart failure.
  3. pulmonary management: restrictive lung disease.
  4. GI/Speech/swallowing/nutrition management.
  5. disease modifying management: corticosteroids.
  6. rehabilitation management: help with ADL/IADLs – ROM, stretch, orthoses, wheelchair, etc.
    Ref: Lancet Neurol2010; 9: 77–93.
131
Q

Photo of infant lying supine with head rotated to the right; right arm extended, left arm flexed at the elbow. What is this primitive reflex?

A

ATNR: asymmetric tonic neck reflex.

Fencing reflex.

132
Q

When is a Pavlik harness suitable for congenital DDH (developmental dysplasia of the hip)?

A

DDH < 6 months of age and a hip that is reducible.

Ref: orthobullets – DDH.

133
Q

What causes hindfoot valgus?

A

Gastrocnemius muscles-jenn Q bank

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