Chapter 10: Peds #3

Front 1

Of pediatric cancers what % are solid tumors and what % are leukemias?

Back 1

70% = SOLID (mostly CNS & lymphomas)
30% = leukemias

*2nd leading cause of death in pediatric patients in U.S.

Front 2

What is the most common type of pediatric cancer?
Second most common?

Back 2

1. Leukemia
2. BRAIN tumors (CNS)

Front 3

Pediatric patient with brain tumor that presents with CN palsies, hemiparesis & ataxia, where is the tumor likely located?

Back 3

brain STEM

Front 4

Pediatric patient with brain tumor that presents with SEIZURES & increased intracranial pressure, where is the tumor likely located?

Back 4

Supratentorial region

Front 5

Pediatric patient with brain tumor that presents with ataxia, poor coordination, poor balance & increased intracranial pressure, where is the tumor likely located?

Back 5

Cerebellum or FOURTH ventricle

Front 6

Hodgkins's lymphoma has is more common in which sex?
Usual age of onset?

Back 6

MALES (2:1)
30's

Front 7

What kind of pediatric cancer?
PAINLESS CERVICAL ADENOPATHY

Back 7

HODGKIN's LYMPHOMA

Front 8

Pediatric cancer associated with EBV?

Back 8

BURKITT's LYMPHOMA
- African

Front 9

Burkitt's lymphoma has a predilection for what part of the body?

Back 9

FACIAL BONES - mandible

Front 10

What kind of pediatric cancer?
Primary involvement of ABDOMINAL LYMPH NODES & viscera

Back 10

Burkitt's lymphoma

Front 11

What kind of pediatric cancer?
From cells of the sympathetic ganglia & adrenal medulla

Back 11

NEUROBLASTOMA

Front 12

What kind of pediatric cancer?
3rd most common pediatric neoplasm in children <5 yo

Back 12

NEUROBLASTOMA
*highest incidence at 2 yo

Front 13

What kind of pediatric cancer?
Presentation at abdominal area carries very poor prognosis as it indicates DISTANT METASTASES

Back 13

NEUROBLASTOMA

Front 14

What kind of pediatric cancer?
WILM's tumor

Back 14

NEPHROBLASTOMA

Front 15

What kind of pediatric cancer?
Develops with renal parenchyma

Back 15

NEPHROBLASTOMA

*can be associated with other congenital abnormalities
*bilateral 5% of the time

Front 16

Age group that nephroblastoma most often affects?

Back 16

2-5 yo
- early childhood -young

Front 17

What kind of pediatric cancer?
Most common MALIGNANT soft tissue tumor in children

Back 17

RHABDOMYOSARCOMA

Front 18

What kind of pediatric cancer?
Associated with NEUROFIBROMATOSIS

Back 18

RHABDOMYSOCAROMA

*head & neck

Front 19

Name the 2 most common MALIGNANT bone tumors in children
What age group do these 2 most commonly occur in?

Back 19

1. OSTEOSARCOMA
2. EWING's SARCOMA

Older children >10 yo

Front 20

Where does OSTEOSARCOMA most typically arise from?

Back 20

METAPHYSIS (between shaft and end) of LONG bones
1. DISTAL FEMUR
2. proximal tibia
3. proximal humerus

Front 21

Where does EWING's SARCOMA most typically arise from?

Back 21

DIAPHYSIS (shaft) of FLAT BONES
***PELVIS***

Front 22

What age group does RETINOBLASTOMA usually occur in?
Malignant or benign?

Back 22

<5 yo

MALIGNANT

Front 23

If a retinoblastoma is BILATERAL is it inherited or sporadic?

Back 23

INHERITED = BILATERAL

Front 24

Where do GERM CELL tumors arise from?

Back 24

Although germ cells are located in the gonads, EXTRAgonadal midline sites are involved in 66% of cases (errors during embryonic development leave germ cells outside of the gonads)
- sacrococcygeal area, mediastinum, retroperitoneum, CNS
*can be malignant or benign

Front 25

What is the most common type of LEUKEMIA in the pediatric population?

Back 25

ALL
- Acute lymphoblastic leukemia
- 80% of cases

Front 26

The highest incidence of LEUKEMIA in the pediatric population occurs at what age?

Back 26

2-5 yo -early childhood -young

Front 27

What is the leading cause of death in children >1 yo in the U.S.?
What is the 2nd leading cause?

Back 27

1. TBI
2. Leukemia

Front 28

What are the top 4 mechanisms of pediatric TBI?

Back 28

1. MVC
2. Fall
3. Sports/rec
4. Assault

Front 29

What kinds of forces cause DAI?

Back 29

- Shearing
- RAPID deceleration
- Rotational

*White matter = myelin = axons

Front 30

Non-accidental traumatic brain injuries in the pediatric population are a result of acceleration-deceleration forces -what is a HALLMARK sign of this type of trauma?

Back 30

RETINAL HEMORRHAGE

*Also look for fxs and multiple injuries

Front 31

Why are young children at greater risk of sustaining SHEARING injuries?

Back 31

Incomplete myelination of the axons in their brains make them more susceptible to DAI

Front 32

Horner's syndrome can be associated with which neonatal brachial plexus injury?

Back 32

KLUMPKE's palsy
- LOWER TRUNK (C8-T1) - affects ciliospinal center which gives input to the superior cervical ganglion

Front 33

The pediatric GCS modifies the verbal category since many pediatric patients do not have the ability to speak yet. How is it modified?

Back 33

5 - coos, babblies (normal)
4 - cries but is consolable
3 - cries but is INconsolable
2 - moans
1 - no response

Front 34

If a child that sustains a TBI is unconscious for 24 hr what level of severity is the injury?

Back 34

MODERATE (15 min - 24 hr)

*>24 hrs unconscious is SEVERE

Front 35

If a child that sustains a TBI has PTA lasting 24 hr what level of severity is the injury?

Back 35

MODERATE (1-24 hrs)

*>24 hrs is SEVERE

Front 36

Basilar skull fxs are frequently associated with injury to what cranial nerve?

Back 36

VIII - vestibulocochlear

Front 37

In terms of prognosis for IQ improvement after a pediatric brain injury, what is the crucial window?

Back 37

first FOUR months postinjury are when the most improvement occurs

Front 38

What % of pediatric TBIs exhibit difficulty communicating?
Dysarthria?

Back 38

communication difficulty = 66%
dysarthria = 33%

Front 39

Precocious puberty following pediatric TBI is more common in which sex?
Will they be taller or shorter than usual?

Back 39

FEMALES
SHORTER - premature epiphyseal closure occurs

*occurs 2-17 mos post TBI

Front 40

Where is the theorized site of injury in Central Autonomic Dysfunction?
What are the signs?

Back 40

HYPOTHALAMLUS or BRAINSTEM
- hyperthermia w/ diaphoresis
- hypertension
- rigidity
- decerebrate posturing
- tachypnea

Front 41

What anti-epileptic medications are as prophylaxis for late seizures in the pediatric population?

Back 41

NONE!
- prophylaxis is NOT recommended -non-efficacious

Front 42

Define posttraumatic epilepsy

Back 42

2 or more late seizures following a TBI

*Late seizure = >1 week after TBI
*Early seizure = <1 week after TBI

Front 43

Several weeks after sustaining a TBI the MRI of a child shows ENLARGED ventricles. What are two possible causes of this?

Back 43

1. Cerebral ATROPHY (hydrocephalus ex-vacuo) -more common

2. true hydrocephalus d/t obstruction of CSF flow

Front 44

In general which type of injury has a better prognosis in the pediatric population, anoxic or traumatic?

Back 44

TRAUMATIC

Front 45

Define CP

Back 45

- lesion to an IMMATURE brain
- NON-progressive
- involves CONTROL of MOVEMENT & POSTURE
- involves COGNITIVE & SENSORY impairment

Front 46

What is the leading cause of childhood disability?

Back 46

CEREBRAL PALSY

Front 47

When do the majority of CP cases occur?
What are the risk factors?

Back 47

PRENATAL PERIOD (in utero)
- intracranial hemorrhage
- placental complications
- toxins/teratogenic agents
- TORCH infections

Front 48

What is the most common PERINATAL (near time of delivery) risk factor for CP?

Back 48

PREMATURITY & LOW BIRTH WEIGHT <2500 g (5.5 lbs)

*premature CEREBRAL VESSELS are much more vulernable to insult

Front 49

What are the 3 MAIN types of CP?

Back 49

1. spastic (pyramidal)
2. dyskinetic (extrapyramidal)
3. mixed

Front 50

What is the mos common type of CP?

Back 50

Spastic DIPLEGIA

LOWERs >> uppers

Front 51

Describe the classic gait pattern of a patient with DIPLEGIC or TRIPLEGIC CP

Back 51

- Scissoring
- Toe walking

*hip flexors, adductors, & gastroc contractures

Front 52

In spastic hemiplegic CP is the arm or the leg usually more involved?

Back 52

ARM >> leg

*slightly more common on RIGHT side, there are usually also ipsilateral sensory deficits

Front 53

What other impairments are common in CP patients besides movement control & postural deficits?

Back 53

1. Visual deficits
2. Cognitive deficits
3. Seizures

Front 54

In spastic diplegic CP patients what abnormality besides prematurity is usually present?

Back 54

INTRAVENTRICULAR HEMORRHAGE
- usually at 28-32 wks gestation

Front 55

A history of difficult delivery with evidence of perinatal asphyxia will often result in what form of CP?

Back 55

SPASTIC QUADRIPLEGIC

Front 56

What is usually the first indicator that a child has spastic hemiplegic CP?

Back 56

HYPOTONIA on the hemiplegic side
- usually definite hemiplegia is observed by 4-6 mos

*also premature hand dominance (normally occurs at ages 2-4 yo)

Front 57

What type of CP?
Pseudobulbar signs make aspiration and feeding difficulties a concern

Back 57

1. Spastic Quadriplegic CP

2. Dyskinetic CP with ATAXIA

Front 58

What are the 5 different movement patterns of dyskinetic CP?

Back 58

1. Athetoid
2. Choread
3. Choreathetoid (athetosis >> chorea)
4. Ataxic
5. Dystonic

Front 59

What is the most common MIXED type of CP?

Back 59

SPASTIC ATHETOID (athetosis >> spasticity)

Front 60

What is the prognosis for ambulation in a SPASTIC HEMIPLEGIC CP patient?

Back 60

They can usually walk by TWO YEARS of age

Front 61

What type of CP?
Most casese are congenital

Back 61

SPASTIC HEMIPLEGIC

Front 62

What type of CP?
Highest incidence of abnormalities in distribution of MIDDLE CEREBRAL ARTERY.

Back 62

SPASTIC HEMIPLEGIC

Front 63

What type of CP?
Most common type in PREMATURE infants

Back 63

SPASTIC DIPLEGIC

Front 64

What is the prognosis for ambulation in a SPASTIC DIPLEGIC CP patient?

Back 64

Most ambulate - some with assistive devices

Front 65

What type of CP?
Disproportionate involvement of the LEGS

Back 65

SPASTIC DIPLEGIC

Front 66

What type of CP?
Thought to be due to a MAJOR HYPOXIC EVENT

Back 66

SPASTIC QUADRIPLEGIC
*perinatal asphyxia -difficult delivery

Front 67

What is the prognosis for ambulation in a SPASTIC QUADRIPLEGIC CP patient?

Back 67

- 25% independent
- 50% require assistance
– 25% non ambulatory

Front 68

What type of CP?
High incidence of SENSORINEURAL DEAFNESS

Back 68

DYSKINETIC - ATHETOSIS

*due to kernicterus, can also be d/t toxins & TORCH infections

Front 69

What is the prognosis for ambulation in a DYSKINETIC CP patient?

Back 69

- 50% can walk

Front 70

When are the dyskinetic movements of DYSKINETIC CP usually evident by?

Back 70

1-3 years of age

*Uppers usually more involved than lowers

Front 71

What type of CP?
Previously most cases associated with KERNICTERUS

Back 71

DYSKINETIC

*neonatal hyperbilirubinemia causing bilirubin deposition in the BASAL GANGLIA

Front 72

Define the 5 levels of the GROSS MOTOR FUNCTION CLASSIFICATION for CP patients.

Back 72

1. no restrictions
2. some limitations but does not need assist device
3. some limitations & needs assist device
4. limited self-mobility -power chair in community
5. severely limited self-mobility even with assist device/power chair

Front 73

What are two generally good prognostic factors for ambulation with CP patients?

Back 73

Favorable prognosis If by around 2 years old they can..
1. SIT INDEPENDENTLY
2. CRAWL ON HANDS & KNEES

Front 74

What type of CP?
Highest rate of MENTAL RETARDATION

Back 74

SPASTIC QUADRIPLEGIC

*also highest rate of SEIZURES & STRABISMUS

Front 75

What type of CP?
Lowest rate of MENTAL RETARDATION

Back 75

SPASTIC DIPLEGIC & HEMIPLEGIC

Front 76

What type of CP?
Nystagmus is present

Back 76

DYSKINETIC - ATAXIC

Front 77

A CP patient that can do what by age 3 has good intellectual potential?

Back 77

SPEAK in 2-3 WORD SENTENCES

Front 78

What is the Individuals with Disabilities Education Act (IDEA)?

Back 78

FEDERAL LAW
- EARLY INTERVENTION (including PT/OT/SLP) must be provided for children w/ dev delay in 1st 3 yrs of life

Front 79

What is the most widely used exercise technique for treatment of CP?

Back 79

BOBATH aka NDT (neurodevelopmental treatment)
- normalize tone
- INHIBIT ABNORMAL primitive reflexes
- proximal to distal

Front 80

What are two unique features of a TONE REDUCING AFO?

Back 80

1. Foot plate extends PAST the toes -inhibits toe flexion
2. METATARSAL support -blocks reflexogenic stimulation at this area of the foot

Front 81

What is the primary benefit of using KAFOs or HKAFOs in those with CP?

Back 81

Decrease DEFORMITY

*they do little to actually improve gait

Front 82

Phenol and alcohol blocks are usually effective for how long?

Back 82

3-6 months

Front 83

What type of nerve block would you perform to reduce SCISSORING?

Back 83

Obturator - adductors
*anterior & posterior branch

Front 84

What type of nerve block would you perform to reduce CROUCH gait?

Back 84

Sciatic - semimembranosus & semitendinosus

Front 85

What type of nerve block would you perform to reduce spastic genu recurvatum?

Back 85

Femoral - quads

Front 86

What type of nerve block would you perform to reduce plantarflexion tone?

Back 86

Tibial - gastrocs

*allows better fitment/tolerance of AFOs

Front 87

Botox reversibly or irreversibly blocks presynaptic release of ACh?

Back 87

IRREVERSIBLY

*axons fibrils actually form new junctional plates on new areas of muscle to overcome the toxin, usually by ~3 months

Front 88

When does the effect of Botox peak?

Back 88

2 WEEKS post injection

*onset is typically 1-3 days

Front 89

Selective Posterior Rhizotomy is usually performed in CP patients that are older or younger?

Back 89

YOUNGER (3-8 yo)

- laminectomy over the cauda with selective severing of dorsal roots involved in spasticity

Front 90

What type of CP is Selective Posterior Rhizotomy contraindicated in?

Back 90

Dyskinetic ATHETOID
Dyskinetic DYSTONIC

Front 91

What is another name for the INTRATHECAL space?

Back 91

sub-ARACHNOID space

*within the dura but outside of the actual neural tissue of the spinal cord

Front 92

What precaution should you take when prescribing BACLOFEN?
Starting/max dose?

Back 92

Make sure the patient does not have seizure disorder or history of seizures. LOWERS SEIZURE THRESHOLD
- 2.5-5mg BID, max 20mg QID

Front 93

What is the mechanism of action of BACLOFEN?

Back 93

Acts on central GABA-B "B for Baclofen" receptors, which inhibit release of excitatory neurotransmitters

Front 94

What is the mechanism of action of DANTROLENE?

Back 94

Acts peripherally on extra/intrafusal muscles fibers, inhibiting the release of Calcium from the sarcoplasmic reticulum, which prevents muscle contraction

Front 95

What is the drug of choice to reduce spasticity of CEREBRAL origin?

Back 95

DANTROLENE

Front 96

What precaution should you take when prescribing DANTROLENE?
Starting/max dose?

Back 96

- watch for LIVER toxicity
- 0.5mg/kg BID, max 12mg/kg/day

Front 97

What is the mechanism of action of BENZODIAZEPINES?
Starting/max dose?

Back 97

- increase binding of GABA in brain stem & spinal cord, which inhibits excitatory neurotransmitter release
- 1-2mg BID, max 20 mg QID

Front 98

What is the mechanism of action of CLONIDINE?
Starting dose?

Back 98

CENTRAL alpha 2 adrenergic agonist in brain and spinal cord
- 0.1mg patch x7 days

*watch for bradycardia, hypotension

Front 99

What is the drug of choice for reducing spasms & resistance to stretch in central demyelinating conditions like MS?

Back 99

Clonidine

*also good for SCI, stroke

Front 100

What is the mechanism of action of TIZANIDINE?
Starting/max dose?

Back 100

Central alpha 2 adrenergic agonist
2-4mg QHS, max 36mg

Front 101

What precautions should you take when prescribing TIZANIDINE?

Back 101

-wath for orthostatic hypotension
-watch LFTs

Front 102

CP patients with an IQ at what level are considered UNEMPLOYABLE?

Back 102

IQ <50

*Above 80 is considered competitive in the work force