leyla akanli, m.d. f.a.a.p f.c.c.p pediatric pulmonology and sleep medicine pediatric obstructive...
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Leyla Akanli, M.D. F.A.A.P F.C.C.P
Pediatric Pulmonology and Sleep Medicine
PEDIATRIC OBSTRUCTIVE SLEEP APNEA
DEFINITIONSSleep-disordered breathing (SDB) refers to the clinical
spectrum of repetitive episodes of complete or partial obstruction of the airway during sleep.Primary Snoring (PS)
Snoring without obstructive apnea, frequent arousals from sleep,
or gas exchange abnormalities.
Obstructive Hypoventilation Syndrome (OHS)
Persistent partial upper airway obstruction associated with gas
exchange abnormalities, rather than discrete, cyclic apneas.
Upper Airway Resistance Syndrome (UARS)
Increasingly negative intrathoracic pressures during inspiration
that lead to arousals and sleep fragmentation.
Obstructive sleep apnea (OSA)
Disorder of breathing during sleep characterized by prolonged
partial upper airway obstruction and/or intermittent complete obstruction.
PRIMARY SNORING
Snoring is related to upper airway narrowing Can not be diagnosed on clinical basis alone PSG shows No sleep fragmentation No discrete events No desaturation No hypercapnia PSG is essential to differentiate from PS from OSA Clinical consequences of PSD is unknown
UPPER AIRWAY RESISTANCE SYNDROME-UARS
Snoring is due to upper airway narrowing or floppiness Clinical history suggestive sleep fragmentation PSG shows Increased intra-thoracic pressure swings Flow limitation of nasal pressure monitoring Non –REM asynchronous breathing Increased arousals No gas exchange abnormalities PSG is essential to differentiate UARS from OSA
OBSTRUCTIVE HYPOVENTILATION
Prolonged periods of partial airway obstruction More common in children than adults Clinical history similar to OSA PSG demonstrates Asynchronous breathing Absence of discrete events Sleep fragmentation Abnormal gas exchange – maybe present only during
REM sleep –Hypoxia , Hypercarbia PetCO2 > 53 torr
MILESTONES
1837 – Dickens – describes overweight/hypersomnolent boy in the Posthumous papers of the Pickwick Club (term “pickwickian” used by Osler)
1907- Osler 1973-Guilleminault
W. Hill described the obstructive sleep apnea sufferer child as in 1889;
“ The stupid -lazy child who frequently suffers from headaches at school, breathes through his mouth instead of his nose, snores and restless at night and wakes up with a dry mouth in the morning is well worthy of the solicitous attention of the Scholl medical officer.”
ADULT VS PEDIATRIC OSAPediatric OSA
Adult OSA
Age Preschool Elderly
Gender M=F M>F
Etiology Adenoid/Tonsilhypertrophy
Obesity
Weight FTT, normal, or obese
Obese
Behavioral Hyperactive Somnolent
Sleep architecture
Normal Decreased delta and REM sleep
Surgical Rx T&A UPPP
Medical Rx CPAP (rarely) CPAP
EPIDEMIOLOGY Most studies showed 4% to 11% prevalence of parent-
reported apnea. Depending on threshold of AHI to diagnose, the
prevalence of pediatric OSA ranges from 1% to 4% in most studies.
Children with abnormal PSG that go untreated will continue to have abnormal findings.
Snoring and adverse neurocognitive, neurobehavioral outcomes
Overall prevalence of snoring in pediatric patient population 8% and 5% in infants
Always snoring in 1.5%-6%
EPIDEMIOLOGY
Peaks ages two to 8 years As obesity is increasing in pediatrics the age distributed shifted Gender distribution: M>F after puberty, equal pre-puberty Prevalence is higher among African Americans and Asian children Family history Prematurity Other Co-Morbid conditions
PATHOPHYSIOLOGY
Neuromotor tone• Cerebral palsy• Genetic diseases
Structural factors• Adenotonsillar hypertrophy• Craniofacial abnormality• Obesity
Other factors• Genetic• Hormonal• ? Diet, Inflammation, Passive smoking
OSA
Anatomic narrowingRequires increased inspiratory pressures
Abnormal neuromuscular controlReflex activation of dilators in response to airway obstruction often fails
RISK FACTORSAdenotonsillar HypertrophyUpper airway congestion; allergies Upper airway obstruction , choanal stenosis, larnygomalacia, subglottic stenosis GER/LPR Cleft palate Craniofacial dsymorphism : Mid -facial hypoplasia –Down’s syndrome Micrognothia – Pierre-Robin syndrome Cranial base malformation- AchondroplasiaNeuromuscular disorder: Hypotonia-Down’s syndrome, Muscular dystrophy Spasticity –Cerebral Palsy OverweightSickle cell diseaseCystic fibrosisChronic lung disease/ BPDScoliosisBrain and spinal disorders – Spin Bifida, ACM type II
Trisomy 21
Small midface and cranium
Relatively narrow nasopharynx
Macroglossia
Hypotonia
Tendency for obesity
Relatively small larynxIn addition, given their congenital
heart defects, they are already predisposed to cor pulmonale.
Because of these factors, the incidence of OSA in patients with DS has been estimated to be from 54% to 100%.
REDUCED MUSCLE TONE
REDUCED MUSCLE TONE
Neuromuscular disease Hypothyroidism Cerebral Palsy Moebius, MG Reduced Central Ventilatory Drive
ACM type I/II
Myelomeningocele
Brainstem injury or masses
MEDICAL CONDITIONS
Craniofacial syndromes
Apert
Crouzon
Pierre-robin
Treacher-Collins
Pfeiffer
Miscellaneous
Achondroplasia
Beckwith-Wiedeman
Goldenhar
Marfan
Mucopolysaccoridoses
Prader Willi
Sickle Cell Disease
Prematurity /CLD
CLINICAL FEATURESNocturnal Symptoms
Symptoms vary by age-especially in infants! Snoring-Volume does not correlate with the degree of
obstruction Observed apneic pauses Snorting / gasping / choking Restless sleep Diaphoresis Paradoxical chest wall movement Abnormal sleeping position SweatingMouth BreathingSecondary enuresis
CLINICAL FEATURESDaytime Symptoms-Physical and Behavioral
Morning headachesDifficulty awakening in AMHyponasal SpeechNasal congestion, Chronic RhinorheaMouth breathing, Dry MouthFrequent infectionsDifficulty swallowingPoor appetiteDaytime somnolence-7-10%Mood changes Internalizing behaviorsExternalizing behaviorsADHD Like symptoms, School problems
ASSOCIATED FEATURES
Increase in partial arousal parasomnias Worsening GERD Increase in seizure frequency in predisposed children Other CO-Morbid Sleep problems
RLS,PLMS
Circadian Rhythm Disorders
Bedtime resistance , nightwakings
EVALUATION Medical HistoryDevelopmental and School history
Family History
Behavioral assessment
Physical ExaminationGrowth
HEENT
Cardiac examination
Diagnostic TestsFor the most part are unnecessary
Radiologic StudiesLateral Neck
Laryngoscopy
EKG/ECHO
Cine-MRI
MALLAMPATI CLASSIFICATION
MULLER MANEVEUR
LARNYGOMALACIA
SUBGLOTTIC STENOSIS
GERD
MRI Excellent soft tissue anatomyMultiple planesNo ionizing radiationDisadvantages
CostWeight limitationsNoisyclaustrophobia
HOME OXIMETRY TESTING Readily available and relatively inexpensive Subject to presence of significant artifact Artifact reduction maybe accomplished with
simultaneous –heart rate measurement and Pletsymography waveform
Excellent positive predictive value-97%* Poor negative predictive value-47%* Disorders with predominant sleep disruption and
hypercapnia will be missed.
*Brouillette RT et al. Pediatrics 2000
NAP STUDY
Child may not achieve natural sleep – REM sleep may not be captured
Severity may be underestimated- Events usually worsens as the sleep progress
Excellent positive predictive value-77-100%* Poor negative predictive value-17-49%*
Keens TG, et al.Pediatric Pulmonol 1992, &Chest 2000
POLYSOMNOGRAPHY
PSG IS THE GOLD STANDARD
Meet diagnostic criteria of pediatric OSAS according to ICSD 2 Differentiate OSA from other SDB Define severity of OSAS Screen high risk children Evaluate success of treatment Titrate PAP therapy
POLYSOMNOGRAPHY
It should be performed without sedation and sleep deprivation
In a child- friendly environment By personnel with training in recording and scoring
pediatric PSG’s Should be interpreted by physicians with expertise in
pediatric sleep medicine
Courtesy of Dr. Carol Rosen
PEDIATRIC POLYSOMNOGRPAHY
Tech Observer Video Camera
Sao2
Leg EMG (2)
Microphone
EKG
Chin EMG (2)
EEG EOG
Nasal EtCO2
Record behaviorDocuments arousals, parasomnias, abnormal sleeping position, and attends to any technical problem
Respiratory Effort
Nasal Oral Airflow
PSG PARAMETERS Apnea
Any pause in respiration lasting longer than two breaths.
Versus at least 10 s in adults. Hypopnea
Reduction of airflow by 50% for two respiratory cycles accompanied by reduction of saturation by 3% or arousal from sleep.
AHI
Sum of Apneas and Hypopneas per hour of sleep. RDI
Sum of Apneas, Hypopneas, and respiratory event-related arousals per hour of sleep.
No universally accepted PSG normal reference values AHI >1.5 or AI >1 per hour is most often used to identify
children- up to 12 years with OSA. Oxygen saturation<91% Change in nadir 02 from baseline>9% Maximal ETCO2>54
PEDIATRIC POLYSOMNOGRAPHY
In contrast to adults, children have:
Obstructive hypoventilation
Fewer obstructive apneas
Desaturation with shorter events–Higher respiratory rate–Lower functional residual capacity–Smaller oxygen store
PEDIATRIC OSA -SEVERITY OSA SEVERITY LEVEL
AHI SpO2 NADIR %
PEAK ETCO2TORR
PEAK ETCO2 > 5O T0rr%TST
MILD 1-4 86-91 >53 10-24
MODERATE 5-10 76-85 >60 25-49
SEVERE >10 <75 >65 >50
PARADOXICAL RIB-CAGE MOTION
HYPERCAPNIA
OBSTRUCTIVE APNEA
This tracing depicts cyclic obstructive apneas
MANAGEMENTAny child with AHI> 5 intervention is necessary.Less of a consensus regarding AHI 1-5.
Surgical–Adenotonsillectomy – First Line of therapy–Turbinate reduction–Craniofacial surgery- Mandibular advancement Lefort osteotomies and maxillary distraction.–Uvulopalatopharyngoplasty- Not a good idea !–Tracheostomy
Medical–Weight loss–Continuous positive airway pressure –Intranasal steroids (modest effect)-Mild patients–Leukotriene antagonist- Mild patients–Oral appliances–Positional therapy–Snore aids
ADENOTONSILLECTOMY
First-line of treatment
Presence of additional risk factors not a contraindication to adenotonsillectomy
25 % residual OSA Re-assessment of high risk groups with post-operative polysomnography is recommended CHAT study –RCT 5-9 years old
HIGH RISK PATIENTSRisk Factors for Postoperative Respiratory Complications in Children with OSAS undergoing Adenotonsillectomy
–Age Younger than 3 years–Severe OSAS on PSG, AHI>10–Pulmonary hypertension–Congenital heart disease–FTT–Prematurity, CLD.–Recent URI– Morbid Obesity–Trisomy 21–Craniofacial abnormalities–Neuromuscular disorders, CP–Asthma
SEVERE OSA
Children with severe OSA show a significant improvement in RDI and quality of life.
OSA does not resolve in the majority of these patients.
Postoperative PSG is recommended for all children with severe OSA.
To identify those who may
require further therapy.
SEVERE OSA
CPAP Almost always an alternative to surgery Surgical failure; Morbid Obesity Complex OSA Non-Surgical candidates Local and systemic anti-inflammatory effect Act as a pneumatic splint Stimulates ventilation Reduces activity of inspiratory, upper airway muscles
and diaphragm Restores sleep, promotes weight loss Improves cardiac function, Suppresses GERD Decrease AHR FDA approved for children > 30 kg
CPAP
CPAP-AIRWAY
Management Algorithm
COMPLICATIONS OF OSA
Effects on growth
Neurocognitive morbidity
Cardiovascular consequences
Metabolic
IMPROVEMENT IN WEIGHT
Marcus et al. J Pediatr 1994
Girls Boys
NEUROCOGNITIVE MORBIDITY
Hyperactivity, inattention, aggression
Impaired school performance
Daytime sleepiness
Depression
CARDIOOVASCULAR MORBIDITY
Pulmonary Hypertension
Cor Pulmonale
Systemic Hypertension
HYPERTENSION
HYPERTENSION
Marcus et al. Am J Respir Crit Care Med 1998
AAP GUIDELINES Screening of all children for snoring Specialty referral of complex high-risk patients Urgent evaluation of cardio-respiratory failure PSG as Gold Standard for diagnosis Adenotonsillectomy as first-line treatment Inpatient monitoring of high-risk patients Post-operative reevaluation to determine if additional
treatment is required