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Flashcards in Pediatric Deck (133)
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What possible clinical findings are associated with caudal regression syndrome?

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.


what percentage of pts with CP have epilepsy?

Risk is increased with structural abnormalities seen on neuroimaging.

Ref: Braddom pg 1260.


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

"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."


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

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


What is the greatest risk factor for developing CP?

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

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


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

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.

(no assistive devices, walks indoors, outdoors, climbs stairs, no limits, can run + jump, decreased speed, balance, coordination).

(limited outdoor activities, difficulty uneven terrain, inclines, crowds walks indoors, outdoors, climbs stairs with railing minimal ability to run + jump).

(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).

(self mobility severely limited even with assistive devices, uses w/c most of time, may propel own power chair).

(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


What is the definition of CP Cerebral Palsy?

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


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

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


What are types and common causes/categories of 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)


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

5. choreoathetoid.
6. dystonic.
7. ballstic
8. ataxic [some categorize it here]


(cerebellar hypoplasia, granule cell deficiency)

MIXED CP (both spastic and dyskinetic)


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


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

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

Ref: Cuccurullo pg 754.


What are rehabilitation issues of CP in adulthood?

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.

4. MSK -- back pain, leg pain, overuse injuries, progressive scoliosis, cervical myelopathy in athetoid, hand paresthesias.
5. GI + GU symptoms: abnormal urodynamics common.
6. Mobility: declines after 25 y/o, significant decline after 60 y/o.
7. 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).
8. Fatigue -- physical, not mental, predictors: msk pain, deterioration of physical function, limitation in emotional and physical roles, low life satisfaction.

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


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

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

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

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

9. hydrocephalus.
10. aqueductal stenosis/malformation.

11. polymicrogyria.
12. heterotopia of nuclear structures.
13. prominent massa intermedia.
14. thalamic fusion.
15. agenesis of olfactory bulb/tracts.
16. corpus callosum dysgenesis.

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


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

ASP - Clear/Mucus - Spine/Airway

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

Ref: Braddom pg 1260.


Treatment of toe walking.

Depends upon the cause (idiopathic – observation).

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


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


1. heel cord lengthening.
2. hamstring release.

Ref: first principles (Lam style).


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

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.

4. urethral implantation.
5. suprapubic vesicostomy.
6. artificial sphincter implantation.

Ref: Delisa pg 1498.


Spina bifida risk factors – list 4.

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).

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

Ref: 2004, lancet – spina bifida."


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

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.

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.


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

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.


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

Ref: Alexander Matthews pg 199-200."


Predicting CP: POSTER

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:, Levine Criteria - Levine 1980; Arch PMR 61: 385-9 (retrospective chart study of 60 children > 1 year)


Potential Complications of spina bifida – list 15.

Excess Fluid in spine?
Think about cord? Abnormal growth?
Bladder / Bowel / Kidney? Endo?
MSK? Balance? Coordinate - Eye

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.


Patient with spastic CP:

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

(b) future problems at hip, knee, ankle


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.


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


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

Ref: ? First principles."



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

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.

1. motor paralysis.
2. sensory deficits.
3. neurogenic bowel and bladder.
4. hydrocephalus (>90%).
5. Chiari II malformation.
Ref: Cuccurullo pg 782.


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

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.




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

"1. Bony defect of POSTERIOR ELEMENTS of vertebral body with herniation of meninges & CSF (no spinal cord).
2. 75% LS, rest thoracic or sacral, rarely cervical.
3. <10% of spina bifida cystica.

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."


List 6 surgical modalities are useful in CP.

1. selective dorsal rhizotomy.
2. ITB pump placement.

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.

8. deep brain stimulation for seizures.
9. ?spinal cord stimulation.
10. corpus callosotomy.



List 5 etiologies or risk factors for developing cerebral palsy.

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

Social/Economic - Substance - Genetics - Maternal

Premature - BW - Placenta - Infection - ICH

Trauma - Stroke - Anoxia

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

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).

11. prematurity (<32 weeks, low birth weight < 2500 grams, fragile brain structure, vulnerable vessels by germinal matrix).
12. placenta previa.
13. placental abruption.
14. meconium aspiration.
15. hyperbilirubinemia (Rh incompatibility, G6PD deficiency, ABO incompatibility).
16. trauma from delivery (SDH).
17. infection (viral, bacterial).
18. seizures.
19. perinatal ICH.

20. trauma (fall, child abuse, MVC).
21. toxins (heavy metals, lead, organophosphate).
22. strokes (sickle cell, AVM rupture, tetralogy of fallot).
23. infection (bacterial, viral, meningitis).
24. cancers.
25. anoxia (near drowning, etc).
26. ICH.

Ref: Cuccurullo pg 761-62.


List 4 causes of toe walking in 4 year old.

1. cerebral palsy.
5. autism.
14. idiopathic.
13. congenital or post-traumatic limb-length discrepancy.
2. congenital muscular dystrophy (DMD, BMD).

3. tethered cord syndrome.
4. diastematomyelia.
6. schizophrenia.
7. global developmental delay.
8. charcot-marie-tooth disease.
9. spina bifida.
10. transient dystonic reaction.
11. venous malformation of posterior calf muscle.
12. ankylosing spondylitis.

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

15. CNS neoplasm.
16. LGMD2A and 2B.
Peds rehab textbook pg 396.


List 4 causes of tethered cord in spina bifida

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?).


List 2 good prognostic indicators for ambulation in CP.
List 2 poor prognostic factors for ambulation in CP.


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).


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


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.


Is spina bifida more common in males or females?

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