Goals of this Presentation

1

2

Goals of this Presentation

1. Learn how to prepare for a successful pediatric sleep study

2. Learn what to look for and how to respond during the study

3. Learn about pediatric sleep disorders and their treatments

3

Children:

Not just short adults

4

Pediatric Polysomnography Requires Patience and

Preparation • Polysomnographic procedures may be fear

provoking to children• Children require more time to set up for a

polysomnogram than do adults• Crying and removing electrodes may

extend set up time past the child’s usual bedtime, resulting in an overtired child

5

A Family Centered Care Approach

• Parents are the experts on their child and a constant in their child’s life

• Procedures should be conducted to create the least amount of trauma for the child

• The test environment should be inviting and child-friendly

• Psychological preparation of the child and parent are fundamental to the procedure

• Coping-skill development enhances a child’s sense of mastery and control over a potentially stressful experience

Zaremba et al, JCSM, 2005

6

Important “Mind-Set” Changes by the Polysomnography Staff

FROM TO

needs of the staff needs of the child, parent

“Good Guy – Bad Guy”parent, child and tech on

the same team

a child lying downperforming the

procedure with the child sitting


7

Preparing the Family for a Polysomnogram

• Provide detailed information about the test• Schedule testing for the child’s usual

bedtime• Communications: Confirmation letter sent

with:– Logistics of reaching the center– What to bring (food, transitional objects)– No caffeine, no naps, no hair oils

• Answer questions as they come up

8

What the Parent Should Know

• No acute or very recent medical issues– Parents should call to cancel if child is ill

• Recommend shampoo night before– Avoid scalp oils– Avoid new braids

• Avoid caffeinated beverages• Comfortable, loose two piece pajamas• Bring a favorite book, video• Bring usual medications

9

Creating a Calm Environment

• Take time to establish rapport• Explore the child’s past experiences and

coping strategies• Create a good first impression

– Have books or toys on the bed– Cover set up supplies, equipment if possible

• Use a calm and soothing tone of voice

10

Child and Family Preparation

• On the study night…– Allow the child to explore room and sensors– Define each person’s job– Develop a plan for coping– Maintain patience, flexibility, positive

attitude– Lavish the child with praise

• Focusing on the desired behavior

11

Engaging the Parent

• Make the parent part of the team• Encourage the parent to interact in a

reassuring way with the child• Respond positively to parents

questions and concerns• Provide parents with explanations of

the procedures

12

Optimizing the Environmentfor Sleep and Safety

• Quiet – away from doors, overhead paging• Dark – shades over windows• Can you see, hear, communicate with child?

– Call button, two-way communication for calibrations– Need for infrared lighting

• Safety– Outlet plugs, no sharp corners, bed rails up– Hypoallergenic, latex free supplies, no sharp corners

• Access: emergency equipment, personnel

13

Ground Rules for Bedroom Electronics

• No active phones or pagers in sleep room– Arrange local phone access for parent

• Cell phones must be muted– No calls in the room after lights out

• Plan video or TV to end before lights out– Avoid electronic games immediately before

bed

14

Explanations• Short, objective and concrete explanations

are appropriate for younger children• Children may regress when upset

– May need to aim explanations at a developmental level less than child’s age

• Be honest and careful in your word choice• Sarcasm and teasing may be

misinterpreted and should be avoided

15

Tips for Improving Cooperation

• Younger children may want to sit in their parent’s lap during set-up

• Distractions are often useful (stickers, bubbles, toys, favorite video)

• Medical play may reduce anxiety (put the electrodes on a doll)

• Older children can help by holding electrodes or sensors

16

Positions for Comfort


17Courtesy of Dr. Carol Rosen

Pediatric Polysomnography

Tech Observer Video Camera

SaO2

Leg EMG (2)

Microphone

EKG

Chin EMG (2)

EEG EOG

Nasal EtCO2

Records behaviorDocuments arousals, parasomnias, abnormal sleeping position, and attends to any technical problem

Respiratory Effort

Nasal Oral Airflow

18

During the Night

• Children need more frequent adjustment of sensors during the night than adults

• Nearly all studies of children require that the sensors be replaced at some point during the night

• Technologists should warn the patient and the parent that they will be entering the room during the night

19

Documentation • Due to the prevalence of parasomnias,

children’s studies need frequent documentation

• Children may have significant sleep disorders without dramatic polysomnographic findings

• Recordings may be ambiguous at times (i.e., when breathing sensors have been displaced); technologist observations become crucial to interpretation– For example: “discovered nasal pressure

transducer pushed to side of face – restored to proper position”

20

Describe What You See

• Helpful– Sat up abruptly--staring

and mumbling– Patient breathing quietly– Mom moving, wakes

child– Went into room, snoring

from mother, not patient

• Not Helpful– Possible seizure– Can’t hear patient– Patient moving in

bed– Artifact– Sounds from room

21

The Spectrum of Pediatric Sleep Disorders

Prevalent in Children and

Adults

Prevalent in Children Using

Different Criteria Than

in Adults

More Prevalent in

Children Than Adults

Unique to Children by Definition

Delayed sleep phase syndrome

Periodic limb movement disorder

Obstructive sleep apnea

Restless legs syndrome

Narcolepsy

Sleepwalking, sleep talking

Sleep terrors

Nightmares

Behavioral insomnia of childhood

22

Estimated Prevalence of Sleep Disorders in Children

• Insufficient sleep – 10% (higher in teens – up to 33%)– Behaviorally based - 25%

• Sleep related breathing disorders - 2%• Narcolepsy – 0.05%• Sleep/wake timing (delayed sleep phase) - 7%

teens• Partial arousals (parasomnias)

– Night terrors 2 - 3% – Sleep walking 5%

• Rhythmic movement disorder 3 -15%• Restless legs syndrome – 2%

23

Who Should Have a Polysomnogram?

• All children should be screened for snoring– Habitual snoring with labored breathing– Witnessed apnea– Restless sleep– Evidence of daytime sleepiness

• And be sent for a polysomnogram if they show physical signs of sleep apnea– Growth abnormalities– Signs of upper airway obstruction– Evidence of pulmonary hypertension

American Academy of Pediatrics, 2002

Guidelines for Investigation of Sleep Related Breathing Disorders in Children

24

Prevalence of Sleep Related Breathing Disorders in Children• Habitual snoring – 10%

• Sleep disordered breathing – 2%

• Risk factors– African-American heritage– Family history of OSA– History of prematurity– Chronic conditions - cerebral palsy, trisomy 21,

achondroplasia and other genetic syndromes– Obesity (less risky than in adults)– No gender difference in prepubertal children

Rosen et al 2003

25

Many Pediatric Diagnoses Do Not Require a Polysomnogram

Usually requires polysomnography:

• Obstructive Sleep Apnea, Pediatric• Narcolepsy

Usually diagnosed by tests other than polysomnography (i.e., ICU monitoring)

• Primary Sleep Apnea of Infancy(formerly Primary Sleep Apnea of Newborn)

• Congenital Central Hypoventilation Syndrome

May require polysomnography with extended EEG montage:

• Complicated or atypical parasomnia

Usually does not require polysomnography:

• Behavioral Insomnia of Childhood (Sleep Onset Type)

• Behavioral Insomnia of Childhood (Limit-Setting Type)

• Sleepwalking, Night Terrors• Sleep Enuresis• Restless Legs Syndrome• Sleep Related Rhythmic

Movement Disorder

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Evaluating Breathing during Sleep in Children

• Children experience less desaturation with apnea

• Carbon dioxide monitoring is recommended (< 12 years)

• Monitoring behavior, body position, snoring is important

• Additional measures of effort such as esophageal pressure monitoring may be helpful in special cases

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Scoring Rules

• Apnea is recurrent partial or complete airway obstruction despite continued effort– Adult -- respiratory event is 10 seconds or

longer– Child – “two missed breath” duration

• ETCO2 levels above 50 mm Hg for more than 10% of sleep time may be abnormal

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Types of Sleep Related Breathing Disorders in

Children • Upper airway resistance syndrome is common

– Repetitive respiratory effort related arousals without discrete apnea or hypopnea

– No changes in oxygen saturation or ETCO2

• Obstructive hypoventilation is common– Upper airway narrowing with gas exchange

abnormalities, but without clear apnea or hypopnea

• Most prominent in REM

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The Spectrum of Obstructive Sleep Related Breathing Disorders in

ChildrenAPNEAAPNEA

HYPOPNEAHYPOPNEA

RESPIRATORY EFFORT RELATED

AROUSAL

RESPIRATORY EFFORT RELATED

AROUSAL

OBSTRUCTIVE HYPOVENTILATION

OBSTRUCTIVE HYPOVENTILATION

SNORINGSNORING

Degree of Obstruction HIGHLOW

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Normal Breathing – NREM Sleep

Delta activity, K complexe

s, spindles in EEG

Very regular

breathing

No oxygen desaturatio

n or CO2 elevation

8 y/o with daytime sleepiness

Note time scaleNote time scale

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Normal Breathing –REM Sleep

Rapid eye movemen

ts, low voltage fast EEG pattern

Breathing, heart

rate somewh

at irregular

8 y/o with daytime sleepiness

32

RERA

Arousal (alpha activity

at arrow)

Recurrent episodes

of flattened nasal air pressure

and minimal oxygen

desaturation 10 y/o with restless sleep

33

Apnea and Hypopnea

Hypopnea –

between 30 and 70% air

flow

Apnea – less than 30% air

flow

9 y/o with snoring and gasping at night and poor school performance

34

ICSD-2 Diagnostic Criteria: Obstructive Sleep Apnea,

Pediatric• The caregiver reports snoring, and/or labored or

obstructed breathing, during the child’s sleep. • The caregiver reports observing at least one of the

following:i. Paradoxical inward rib-cage motion during inspiration ii. Movement arousals iii. Diaphoresis iv. Neck hyperextension during sleep v. Excessive daytime sleepiness, hyperactivity, or

aggressive behavior vi. A slow rate of growth vii. Morning headaches viii. Secondary enuresis

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ICSD-2 Diagnostic Criteria (cont.)

• Polysomnographic recording demonstrates one or more scoreable obstructive respiratory events per hour (i.e., apnea or hypopnea of at least two respiratory cycles in duration)– Note: Very few normative data are available for

hypopneas, and the data that are available have been obtained using a variety of methodologies. These criteria may be modified in the future once more comprehensive data become available.

Obstructive Sleep Apnea, Pediatric

36

ICSD-2 Diagnostic Criteria (cont.) • Polysomnographic recording demonstrates either i or

ii. i. At least one of the following is observed:

• a. Frequent arousals from sleep associated with increased respiratory effort

• b. Arterial oxygen desaturation in association with the apneic episodes • c. Hypercapnia during sleep • d. Markedly negative esophageal pressure swings

ii. Periods of hypercapnia, desaturation, or hypercapnia and desaturation during sleep associated with snoring, paradoxical inward rib-cage motion during inspiration, and at least one of the following:

• a. Frequent arousals from sleep • b. Markedly negative esophageal pressure swings


37


• Many children have associated cognitive problems and difficulty at school

• Pediatric obstructive sleep apnea is frequently associated with adenotonsillar hypertrophy

• Adenotonsillectomy is effective in most children

• When applied to pediatric recordings, adult polysomnographic measures alone (i.e., AHI) may underestimate the number of patients who would benefit from adenotonsillectomy

38

CPAP Therapy for Children

• Continuous positive airway pressure is an effective second-line treatment in pediatric patients

• A desensitization program is an extremely important part of treatment

• Successful trials reported in 74% of patients, with 86% of those able to use the therapy long-term

39

Primary Sleep Apnea of Infancy

ICSD-2 Diagnostic Criteria• Apnea of Prematurity. Prolonged central respiratory pauses of

20 seconds or more in duration (or shorter-duration events that include obstructive or mixed respiratory patterns and are associated with a significant physiologic compromise, including decrease in heart rate, hypoxemia, clinical symptoms, or need for nursing intervention), are recorded in an infant younger than 37 weeks conceptional age.

• Apnea of Infancy. Prolonged central respiratory pauses of 20 seconds or more in duration (or shorter-duration events that include obstructive or mixed respiratory patterns and are associated with bradycardia, cyanosis, pallor, or marked hypotonia), are recorded in an infant with a conceptional age of 37 weeks or older.

(formerly Primary Sleep Apnea of Newborn)

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Primary Sleep Apnea of Infancy

• Should be distinguished from Acute Life Threatening Events (ALTE), an ill-defined disorder based on parental complaints and Sudden Infant Death Syndrome (SIDS), a post-mortem diagnosis

• A polysomnogram is the best way to evaluate breathing during sleep

• Prognosis is excellent with infrequent events– Prognosis guarded when frequent resuscitation is

required and events persist over time

41

Congenital Central Alveolar Hypoventilation Syndrome

ICSD-2 Diagnostic Criteria• The patient exhibits shallow breathing, or cyanosis and

apnea, of perinatal onset during sleep. – Note: In severely affected infants, consequences of hypoxia, including

pulmonary hypertension and cor pulmonale, may also be present.

• Hypoventilation is worse during sleep than during wakefulness.

• The rebreathing ventilatory response to hypoxia and hypercapnia is absent or diminished.

• Polysomnographic monitoring during sleep demonstrates severe hypercapnia and hypoxia, predominantly without apnea.

42

Congenital Central Alveolar Hypoventilation Syndrome

• Present from birth• Requires lifelong treatment

– Mechanical ventilation or pacing– Most patients do not need treatment when awake

• Associated with abnormality of the PHOX2B gene

• Associated with Hirschsprung's disease

43

Narcolepsy in Children• Narcolepsy with cataplexy is rare in children

younger than four years old• Daytime sleepiness frequently presents as

reappearance of napping in a child that has stopped napping

• Sleepiness at school may be manifest by symptoms similar to attention deficit disorder

• Diagnosis may be clinical or supported by findings from overnight polysomnography with multiple sleep latency testing. Alternatively, measurement of levels of hypocretin in cerebrospinal fluid may be appropriate for certain patients.

44

Recognizing Sleepiness in Children

• Sleepy children do not always “act sleepy”– Parent may endorse other terms like seems “overtired”

• Children with insufficient or disrupted sleep can show: – Inattention– Hyperactivity– Behavioral disturbances– Poor school performance

• Persistent, overt sleepiness is uncommon in preadolescent children unless the disorder is severe

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Pediatric MSLT• Use standard MSLT protocol from AASM

Practice Parameter– Review procedure with child and parent and

answer any questions– It is recommended that parents leave the

testing room during naps– Ask if child needs to go to the bathroom– Put up side rails if necessary– Remind the child, “I will come back in to the

room when the nap test is over.”

46

SOREMP in a Child

Rapid eye

movement

Alpha activity

Nap #1 00:30

Nap #1 lights out

12 y/o referred for excessive daytime

sleepiness and cataplexy symptoms

Reducedtone

47

Nocturnal Sleep Decreases with Age

Ohayon et al SLEEP 2004;27(7):1255-73.

Min

utes

of

slee

p

48Acebo et al. SLEEP 2005; 28(12): 1568-1577.

Napping is Normal in Very Young Children

Age (months)

49

MSLT Latency

0

2

4

6

8

10

12

14

16

18

20

I II III IV V OlderTeens

Tanner Stage

La

ten

cy

(m

in)

Sleep Latency during MSLT Naps Decreases in Adolescents with

Increasing Tanner Stage

Data from Carskadon MA. The second decade. In Guilleminault C, ed, Sleeping and waking disorders: indications and techniques. Menlo Park: Addison Wesley, 1982: 99-125

NOTE: Mean sleep latency is longer in children compared with adults

50

Sleep Latency Increases with Age after Adolescence

From Arand et al, SLEEP 2005;28(1):123-144.

51

Interpreting Pediatric MSLT Results

• Two or more sleep onset REM periods are necessary to support a diagnosis of narcolepsy

• Age has a complicated and profound impact on MSLT mean sleep latency

• Limited normative data is available• Mean sleep latencies that might be considered

normal for adults are often abnormal for children

• The ICSD-2 states, “The MSLT has not been validated as a diagnostic test in children younger than eight years of age.”

52

Parasomnias

• Children are often referred to the sleep center because of unusual behaviors during the night– Sleepwalking– Sleep terrors– Nightmares– Seizures

53

Abnormal Breathing and EEG Activity in Sleep

9 y/o with known epilepsy and snoring

54

Sleepwalking and Sleep Terrors: Partial Arousal

ParasomniasPartial arousal parasomnias

– Occur during first half of night– Arise from slow wave sleep– Child is not awake

• Sleepwalking– Child moves around room or house– May be quiet or agitated– May engage in purposeful activities, like unlocking

door

• Sleep terrors– Child abruptly sits up screaming– Appears frightened and agitated

55

Night Terrors

• Deep NREM sleep• First third of night• Child confused or

agitated• Difficult to reassure• Intense arousal lasting

2-10 min• Abrupt return to sleep• No recall in the

morning

Nightmares

• REM sleep• Last half of night• Child alert; describes

dream content• Comforted by parent• Difficulty going back to

sleep• Recall the following day

56

Technologist Response to Unusual Behaviors

• Parasomnias can lead to injury– Be sure patient is safe

• Parasomnias sometimes resemble seizures– Seizures (especially frontal lobe) can resemble

parasomnias

• During study describe what you see• Note event on record when it is happening

– Sitting up yelling– Patient mumbling – can’t understand words– Patient’s left arm and leg twitching– Mother trying to comfort, patient keeps yelling “mommy”– Patient trying to get out of bed

57

Confusional Arousal

5 y/o with witnessed apnea and restlessness

58

Restless Legs Syndrome

• The patient reports an urge to move the legs, usually accompanied or caused by uncomfortable and unpleasant sensations in the legs.

• The urge to move or the unpleasant sensations – begin or worsen during periods of rest or inactivity (lying or

sitting)– are partially or totally relieved by movement, such as

walking or stretching, at least as long as the activity continues

– are worse, or only occur, in the evening or night

ICSD-2 Diagnosis in Adult Patients

59

Restless Legs Syndrome ICSD-2 Diagnostic Criteria• The child meets all four essential adult criteria for RLS listed above

and relates a description, in his or her own words, that is consistent with leg discomfort.

OR• The child meets all four essential adult criteria for RLS listed above

but does not relate a description in his or her own words that is consistent with leg discomfort.

AND• The child has at least two of the following three findings:

i. A sleep disturbance for age ii. A biological parent or sibling with definite RLS iii. A polysomnographically documented periodic limb movement

index of five or more movements per hour of sleep Note: Criteria for probable and possible childhood RLS have been

developed for research purposes and are included in a National Institutes of Health diagnostic workshop report.

60

Restless Legs Syndrome (RLS) & Periodic Limb Movement Disorder

(PLMD)• Prevalence in children 0.5-2%, familial

link– RLS - “growing pains”– PLMD – leg jerks - what’s normal

• Relationship with hyperactivity?

• Can be associated with:– Iron deficiency/low ferritin– Chronic renal disease

• Diagnostic controversies in adults– Scant data in children– May present as insomnia or sleepiness

61

Criteria for Sleep Related Rhythmic Movement Disorder

ICSD-2 Diagnostic Criteria• The patient exhibits repetitive, stereotyped, and

rhythmic motor behaviors.• The movements involve large muscle groups.• The movements are predominantly sleep related,

occurring near nap or bedtime, or when the individual appears drowsy or asleep.

• The behaviors result in a significant complaint as manifest by at least one of the following:

i. Interference with normal sleepii. Significant impairment in daytime functioniii. Self-inflicted bodily injury that requires medical

treatment (or would result in injury if preventable measures were not used)

62

Sleep Related Rhythmic Movements

• Repetitive movements– Head banging or head rolling– Body rocking

• Before sleep, light sleep, or even awake• Prevalence of rhythmic movements decreases

with age– At nine months = 59%– At eighteen months = 33%– At five years = 5%

• No gender difference• Polysomnogram or treatment rarely indicated

63

Sleep Enuresis

Primary• The patient is older than

five years of age• The patient exhibits

recurrent involuntary voiding during sleep, occurring at least twice a week.

• The patient has never been consistently dry during sleep.

Secondary • The patient is older than five

years of age• The patient exhibits

recurrent involuntary voiding during sleep, occurring at least twice a week.

• The patient has previously been consistently dry during sleep for at least six months.

ICSD-2 Diagnostic Criteria

64

Prevalence of Enuresis

0.0

10.0

20.0

30.0

40.0

4 5 6 7 8 10 18

Age (years)

Ch

ildre

n (

%)

65

Developmental Overview of Common Non-respiratory Sleep

Problems

Newborn/ Young Infant

Older Infant and

ToddlerPre-

schooler School Age TeenagerUsually normalDevelopmentalSelf limited

Night wakingsDifficulty settlingNight terrors

Night wakingsBedtime resistanceNight terrorsSleep walking

Insufficient sleepBedtime resistanceSleep walking

Insufficient sleepDelayed sleep phaseNarcolepsy

Rhythmic movementsBedtime fears

Rhythmic movementsBedtime fearsNightmares

EnuresisBruxism

66

Behavioral or Life Style Sleep Problems

• Sleep onset association disorder• Limit setting disorder• Poor “sleep hygiene”

• Caffeine• Irregular schedule• TV/computer/cell phone/electronics in bedroom

• Overlap with delayed sleep phase– Perpetuated by weekend sleep-in and late day

naps

• Management – change behaviors

67

Behavioral Insomnia of Childhood (Sleep-onset Type)

ICSD-2 Diagnostic Criteria • Falling asleep is an extended process that requires

special conditions• Sleep-onset associations are highly problematic or

demanding• In the absence of the associated conditions, sleep

onset is significantly delayed or sleep is otherwise disrupted

• Awakenings require caregiver intervention for the child to return to sleep.

68

Sleep Onset TypeTypical Presentations

• Child falls asleep during rocking or patting, needs to be rocked or patted after night waking

• Child falls asleep feeding, needs to be fed to fall asleep

• Child falls asleep with parent singing, reading or lying next to child, but cannot fall sleep alone

• Child falls asleep in car seat, needs to be driven around to fall asleep

69

Behavioral Insomnia of Childhood (Limit-setting Type)

ICSD-2 Diagnostic Criteria• The child has difficulty initiating or maintaining

sleep• The child stalls or refuses to go to bed at an

appropriate time or refuses to return to bed following a nighttime awakening

• The caregiver demonstrates insufficient or inappropriate limit setting to establish appropriate sleeping behavior in the child

70

Limit-setting TypeTypical Presentations

• Child is two years or older• “Stalling” behaviors at bedtime

– Needs a drink or food– Multiple stories– Crying, clinging– Gets out of bed (“curtain calls”)

• Parent’s behavior contributes to problem– Irregular or inappropriate schedules– Inconsistent application of rules– Secondary gain for child

71

Contributing Factors

• Circadian rhythms develop over the first few months of life – infants have frequent awakenings and irregular schedules at birth

• Homeostatic drive to sleep is blunted by frequent napping

• Environmental factors such as warmth, soothing sounds and vestibular stimulation promote sleepiness

• Learned associations serve as triggers for sleep onset

72

Behavioral Insomnia of Childhood:

Treatment Options• Extinction • Graduated extinction (“Ferberizing”)• Positive routines• Faded bedtime with response cost• Scheduled awakenings• Parent education• Medications (efficacy unproven in children)

– Prescription– Over-the-counter