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Clinical assessment and diagnostic techniques
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reflect OSA but also an elevated central apnoea index. Oximetry is less useful in
obese children because they tend to desaturate more with normal breathing pauses
due to a more restrictive lung function. A child with negative/inconclusive oximetry
undergoing adenotonsillectomy for SDB has a low risk of post-operative respiratory
complications.
An O2 desaturation (≥4%) index of >2 in children with snoring may be a predictor of
an AHI of >1 episode·h−1 in PSG. A more recent study reported that an O2 desaturation
(≥3%) index of 4.3 is a good diagnostic cut-o parameter. A McGill oximetry score of
>2 has been used to define OSA in infants with Pierre Robin sequence and moderateto-severe OSA in patients with Down syndrome. An O2 desaturation (≥4%) index of >4
with a median O2 saturation (S
in infants with mucopolysaccharidosis.
) of <95% has been used to define obstructive SDB
pO
2
In recent years, studies have emerged that have combined oximetry with
electrocardiogram, to derive signals such as the pulse transit time, which may also
allow the detection of arousals. These studies show promising but limited results.
Assessment of daytime sleepiness
The MSLT is a valid and widely used test to measure the tendency to fall asleep and
oers an objective measure of daytime sleepiness. MSLT is performed aer full-night
PSG (≥6 h of sleep). To perform the MSLT, five nap opportunities lasting 20 min each
at 2-h intervals are created. Sleep stages are scored according to standard rules.
Parameters of interest include sleep latency, defined as the time from lights out to
stage N1. Sleep-onset REM periods (SOREMPs) are defined by REM onset within
15 min of sleep onset. The mean sleep latency (MSL) over the five naps is an objective
index of the severity of sleepiness.
Normative values in children are less well defined than in adults, but normal
school-aged children typically remain awake throughout the nap opportunity, or
demonstrate a MSL in the 15–20 min range. Prepubertal children have a longer MSL
while adolescents have the shortest latency. Two studies found a MSL of >20 min in
prepubertal children. Therefore, 30-min naps in children have been proposed. A sleep
latency of 5–10 min indicates moderate daytime sleepiness; ≤5 min indicates severe
sleepiness.
The presence of ≥2 SOREMPS combined with a low MSL is considered diagnostic for
narcolepsy. However, the physician should remain alert for other factors that might
disrupt sleep architecture, including OSA, periodic limb movement disorders or
insucient sleep.
Further limitations of MSLT are the lack of normative data in paediatric patients, the
last-nap eect (increased arousal because of the anticipation of almost going home)
and the influence of patient motivation.
The maintenance of wakefulness test (MWT) is used to assess the ecacy of treatment
in patients. It can be considered as the opposite of the MSLT test and measures the
ability to stay awake for a defined time. It consists of four trials of a 40-min test
protocol, performed at 2-h intervals, with the first trial beginning 1.5–3 h aer the
patient’s usual wakeup time. Its recording montage is identical to MSLT. Normal MWT
values are >30 min; the test is abnormal when it is <12 min. Paediatric normative
values are not available. Motivation to stay awake is very important for reliable
interpretation of this test.
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Further reading
• American Academy of Sleep Medicine (2020). The AASM Manual for the Scoring of Sleep and
Associated Events: Rules, Terminology and Technical Specifications. Westchester, American
Academy of Sleep Medicine.
• American Thoracic Society (1996). Standards and indications for cardiopulmonary sleep
studies in children. Am J Respir Crit Care Med; 153: 866–878.
• Aurora RN, et al. (2011). Practice parameters for the respiratory indications for PSG in children.
Sleep; 34: 379–388.
• Brodsky L (1989). Modern assessment of tonsils and adenoids. Pediatr Clin North Am; 36:
1551–1569.
• Brouillette RT, et al. (2000). Nocturnal pulse oximetry as an abbreviated testing modality for
pediatric obstructive sleep apnea. Pediatrics; 105: 405–412.
• Guilleminault C, et al. (2007). Adenotonsillectomy and obstructive sleep apnea in children:
a prospective survey. Otolaryngol Head Neck Surg; 136: 169–175.
• Hall KL, et al. (2005). Evaluation and management of apparent life-threatening events in
children. Am Fam Physician; 71: 2301–2308.
• Kaditis A, et al. (2016). Obstructive sleep disordered breathing in 2- to 18-year-old children:
diagnosis and management. Eur Respir J; 47: 69–94.
• Kaditis A, et al. (2017). ERS Statement on obstructive sleep-disordered breathing in 1 to
23-month old children. Eur Respir J; 50: 1700985.
• Littner MR, et al. (2005). Practice parameters for clinical use of the multiple sleep latency test
and the maintenance of wakefulness test. Sleep; 28: 113–121.
• Loughlin GM, et al. (2000). Sleep and Breathing in Children: a Developmental Approach. New
York, Marcel Dekker Inc.
• Simonds AK, et al., eds (2012). ERS Handbook Respiratory Sleep Medicine. Sheeld, European
Respiratory Society.
• Traeger N, et al. (2005). Polysomnographic values in children 2–9 years old: additional data
and review of the literature. Pediatr Pulmonol; 40: 22–30.
• Villa MP, et al. (2015). Diagnosis of pediatric obstructive sleep apnea syndrome in settings with
limited resources. JAMA Otolaryngol Head Neck Surg; 141: 990–996.
• Villa MP, et al. (2016). Use of the sleep clinical record in the follow-up of children with
obstructive sleep apnea (OSA) aer treatment. Sleep Breath; 20: 321–329.
Acknowledgement
This is an update of the ERS Handbook of Respiratory Sleep Medicine first edition chapters ‘Clinical
assessment in children’, by Maria Pia Villa, and ‘Diagnostic techniques in children’, by Stijn
Verhulst and Wilfried De Backer.
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respiratory disorders in
children
Athanasios G. Kaditis, Maria Pia Villa, Anita K. Simonds, Stijn Verhulst and
Brigitte Fauroux
Untreated OSAS and sleep hypoventilation may be related to learning problems,
daytime sleepiness, hyperactivity, delayed somatic growth and adverse eects on the
cardiovascular system, such as pulmonary hypertension (PH). This underlines the
importance of early diagnosis and eective treatment of respiratory disorders during
sleep in order to prevent the development of complications.
Apnoea of prematurity
Apnoea of prematurity and OSA that is unrelated to prematurity are the most common
SDB patterns in infancy. Apnoea of prematurity (a gestational age of <37 weeks) has
been defined as a pause in airflow of ≥20 s in duration or a shorter pause accompanied
by bradycardia or oxygen (O2) desaturation. Apnoeas may be of the central, obstructive
or mixed type and they usually disappear by 36–40 weeks postconceptional age.
It is unclear whether apnoea of prematurity is associated with long-term adverse
eects such as unfavourable neurodevelopmental outcomes, because of the many
other confounding factors in the early preterm period.
Key points
• Adenotonsillectomy is first-line treatment for OSAS in infancy. Other
treatments (orthodontic appliances, CPAP and tracheostomy) may be
indicated in selected patients.
• Adenotonsillar hypertrophy is a frequent cause of OSAS in otherwise healthy
children, but <30% of them achieve a normal AHI (<1 episode·h–1) aer
adenotonsillectomy.
• Oral appliances and functional orthopaedic devices are eective in cases of
maxillary constriction or mandibular retrusion with associated OSAS.
• Long-term home ventilation is required for children with central
hypoventilation.
• NIPPV in neuromuscular disorders should be initiated when nocturnal
hypoventilation develops.
• NIPPV improves quality of life, morbidity and mortality in many stable or
slowly progressive neuromuscular disorders, and may be considered to
palliate symptoms in other more progressive conditions.
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Aer the exclusion of secondary causes of apnoea (i.e. infection, intracranial
haemorrhage, hypoxaemic–ischaemic encephalopathy, seizure disorder, anaemia),
potential therapeutic interventions include prone positioning with the neck elevated by
15°, administration of methylxanthine (theophylline, caeine), O2 supplementation, CPAP
and nasal intermittent positive pressure ventilation. Doxapram, kinesthetic stimulation
and red blood cell transfusion in cases of anaemia are among the interventions that have
been used for the treatment of apnaea of prematurity with unclear benefit.
OSA in the first 2 years of life
OSA that is not related to prematurity may be the result of a variety of pathogenetic
mechanisms: nasal or laryngeal obstruction (choanal atresia, congenital nasal
pyriform aperture stenosis, laryngomalacia, vocal cord paralysis, subglottic stenosis);
adenoidal hypertrophy frequently without tonsillar hypertrophy; congenital
craniofacial deformities (midface or mandibular hypoplasia, Pierre Robin sequence);
neuromuscular or neurological disorders (spinal muscular atrophy (SMA), cerebral
palsy); and syndromic disorders (Trisomy 21, Prader-Willi syndrome).
Indications for treatment
The indications for treatment of OSA in infants and young children are summarised in
the 2017 European Respiratory Society (ERS) statement on obstructive SDB in 1- to
23-month-old children. Treatment is recommended when: 1) clinical manifestations
of upper airway obstruction are obvious, even during wakefulness; 2) there are SDB
symptoms and/or physical examination findings that are indicative of increased upper
airway resistance in combination with an obstructive AHI of ≥1 event·h−1; and 3) the infant
or young child has a complex disorder associated with SDB (craniofacial abnormality,
neuromuscular disorder, achondroplasia, Chiari malformation, mucopolysaccharidosis,
Prader-Willi syndrome, Trisomy 21) and an obstructive AHI of ≥1 event·h−1.
Nevertheless, it should be noted that only a few studies with limited numbers of
participants have reported reference values for PSG parameters in healthy infants.
The severity level of upper airway obstruction in infancy (≤12 months old) that
requires treatment therefore remains controversial. In a systematic review of 71
studies assessing the severity of upper airway obstruction in infants with Pierre Robin
sequence, it was found that a cut-o value of 20 events·h−1 for obstructive AHI was
used as an indication for surgical intervention in most publications.
Treatment approach and treatment options
A stepwise treatment approach is summarised in table 1, moving from less to more
invasive therapeutic interventions according to the cause of airway obstruction.
Endoscopic examination of the upper and lower airway is an important diagnostic tool
to determine the exact site(s) of obstruction.
Adenotonsillectomy for OSA in infants has a higher frequency of postoperative
complications and residual SDB than the same procedure performed in older children.
It is accompanied by appreciable postoperative acceleration in somatic growth. The
procedure has been performed in infants as young as 6 months old, but most ENT
surgeons only operate on children who are >1 year of age.
Indications for CPAP or noninvasive positive pressure ventilation (NIPPV) in the first
2 years of life have been summarised in the 2022 ERS statement on paediatric longterm noninvasive respiratory support. CPAP is generally applied in cases of upper
airway obstruction in order to restore airway patency throughout the entire breathing
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Table 1. A stepwise treatment approach for OSAS in the first 2 years of life
1) OSA and gastro-oesophageal reflux: ecacy of antireflux medication is controversial
2) Adenoidal with/without tonsillar hypertrophy: adenoidectomy with/without
tonsillectomy
3) CPAP or NIPPV if OSA is moderate-to-severe and there is: laryngomalacia; midface or
mandibular hypoplasia; cerebral palsy; SMA; achondroplasia; mucopolysaccharidoses;
or Trisomy 21
4) Supraglottoplasty for laryngomalacia; surgical tongue advancement (i.e. glossopexy
via tongue–lip adhesion) or mandibular distraction osteogenesis for mandibular
hypoplasia; midface advancement for midface hypoplasia
5) Tracheostomy if treatment modalities 1–4 are not adequate or in order to secure the
upper airway while awaiting surgical intervention
Nocturnal PSG, polygraphy or oximetry should be used at each step to move onto the next
treatment step if residual OSA is present.
cycle. NIPPV is the preference in disorders that are associated with upper or lower
airway obstruction, lung disease, decreased capacity of the respiratory muscles to
initiate and sustain breathing or inadequate central control of breathing, resulting in
alveolar hypoventilation.
In cases of severe laryngomalacia, removal of the lateral edges of the epiglottis and
of redundant mucosa covering the arytenoids along with incision of the aryepiglottic
folds can relieve upper airway obstruction eectively (supraglottoplasty). CPAP may be
indicated in cases of persistent OSA aer supraglottoplasty and should be proposed
before a tracheostomy, which remains the last therapeutic option.
Current treatment options for OSA associated with Pierre Robin sequence include:
1) prone positioning; 2) insertion of a nasopharyngeal tube (commonly used
intervention) or a palatal plate (at centres with experience of this technique); 3) CPAP;
4) surgery, such as tongue advancement (i.e. glossopexy via tongue–lip adhesion)
or mandibular distraction osteogenesis; and 5) tracheostomy. Infants with minor
manifestations can be managed by placement in the prone position, taking advantage
of the eect of gravity on moving the tongue forwards. Use of glossopexy has declined
as it does not always allow complete resolution of airway obstruction symptoms.
Craniofacial procedures for mandibular or midface hypoplasia are discussed in the
following section of this chapter on OSA in children >2 years of age.
OSA in children 2–18 years of age
Indications for treatment
Accumulating evidence demonstrates the beneficial eects of OSA treatment in
childhood. An AHI of >5 events·h−1 in paediatric patients, which reflects moderateto-severe intermittent upper airway obstruction during sleep, decreases aer
adenotonsillectomy in children with adenotonsillar hypertrophy. Although an AHI of
>5 events·h−1 has been clearly related to an increased risk of morbidity, even an AHI
of <1 event·h−1 with no apnoeas or gas exchange abnormalities (primary snoring) has
been associated with EDS and learning problems. When treatment decisions for mild
OSA (AHI of 1–5 events·h−1) are made, it should be taken into consideration that SDB
may persist into adolescence in certain children.
The indications for treatment of paediatric OSA have been summarised in the 2016
ERS statement on the diagnosis and management of obstructive SDB in 2–18-yearold children, as follows:
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• Moderate-to-severe OSA (AHI of >5 events·h−1) irrespective of the presence of
morbidity.
• Mild OSA (AHI of 1–5 events·h−1) with OSA-related morbidity (e.g. enuresis, inadequate
somatic growth, poor academic performance, inattention, hyperactivity, PH, or SBP
or DBP >95th percentile for sex, age and height) or a decrease in quality of life.
• Mild OSA in combination with risk factors for SDB persistence (male sex, obesity,
increasing BMI percentile, tonsillar hypertrophy, narrow mandible).
• OSA treatment is a priority in the presence of major craniofacial abnormalities,
neuromuscular disorders, achondroplasia, Chiari malformation, Trisomy 21,
mucopolysaccharidoses and Prader–Willi syndrome. Patients with these conditions
may be at risk of developing PH and it is unlikely that SDB will resolve spontaneously.
There are no data supporting the treatment of children with primary snoring or upper
airway resistance syndrome.
Treatment of OSA may be followed by: 1) an improvement in quality of life; 2) an
appreciable weight and height gain; 3) resolution of or a decrease in the frequency
of enuresis; 4) a reduction in systemic BP and pulmonary artery pressure, and a
reversal of cor pulmonale; 5) reduced daytime sleepiness, hyperactivity and irritability;
6) improved quality of life; and 7) a reduction in healthcare use.
Treatment approach and treatment options
The available treatment options for OSA in 2–18-year-old children include: antiinflammatory medication and weight control, adenotonsillectomy, orthodontic
devices, CPAP or NIPPV, craniofacial surgery and, as a last resort, tracheostomy.
If a child with OSA is a candidate for treatment, therapeutic interventions should
address all abnormalities predisposing to upper airway obstruction. For example, a
child with adenotonsillar hypertrophy and retrusion of the mandible may benefit from
a combination of adenotonsillectomy and the application of an orthodontic device.
No large, randomised controlled trials have been conducted that define the order of
implementation of available treatment modalities. A stepwise treatment approach
from the less invasive to the more invasive therapeutic interventions was proposed
in the 2016 ERS statement for the diagnosis and management of obstructive SDB in
2–18-year-old children (table 2). Aer completion of one step, the patient should be
re-evaluated and the next treatment option should be applied to treat residual OSA.
Drug-induced sleep endoscopy is a safe tool that can assist in treatment decisions,
especially in children with underlying conditions and/or in children with persistent
disease aer adenotonsillectomy.
Tonsillectomy and/or adenoidectomy and other ENT procedures
Surgical excision of the hypertrophic pharyngeal and palatine tonsils (adenoidectomy
and tonsillectomy, respectively) is the standard treatment for OSA in childhood,
reducing upper airway resistance and the tendency of the pharyngeal airway to
collapse. It is unclear whether adenoidectomy alone, tonsillectomy alone, or partial
tonsillectomy are adequate to achieve complete resolution of OSA as compared with
total adenotonsillectomy.
In a randomised, controlled trial on the ecacy of adenotonsillectomy (Childhood
Adenotonsillectomy Trial (CHAT)) a reduction in both the AHI to <2 events·h−1
and the obstructive apnoea index to <1 event·h−1 was more common in the ‘early
adenotonsillectomy’ group than in the ‘watchful waiting with supportive care’ group
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Table 2. A stepwise management approach for 2–18-year-old children with OSAS who have
indications for treatment
1) Weight loss if the child is overweight or obese
2) Nasal corticosteroids for 4–12 weeks and/or montelukast for 16 weeks, if adenoidal
hypertrophy is present and the AHI is 1–10 events·h−1; this may also be used for
residual OSA following adenotonsillectomy
3) Adenotonsillectomy for adenotonsillar hypertrophy
4) Orthodontic devices for craniofacial abnormalities (e.g. oral appliances for
retrognathia or malocclusion and rapid maxillary expansion for maxillary
constriction); orthodontic treatment may be used for residual OSA aer
adenotonsillectomy (AHI of >5 events·h−1)
5) Nasal CPAP if the AHI is >5 events·h−1 and:
There is residual OSA aer adenotonsillectomy
OSA is related to obesity
OSA is related to craniofacial abnormalities
OSA is associated with major craniofacial abnormalities while awaiting a
craniofacial procedure
If OSA is accompanied by nocturnal hypoventilation as in neuromuscular disorders,
NIPPV is preferred
6) Craniofacial surgery if the treatment modalities above are not adequate for OSA
resolution; craniofacial procedures include mandibular distraction osteogenesis for
micrognathia and midface advancement for midface hypoplasia
7) Tracheostomy if treatment modalities 1–6 are not adequate for OSA resolution or to
secure the upper airway while awaiting surgical intervention
Nocturnal PSG, polygraphy or oximetry should be used at each step to move onto the next
treatment step if residual OSA is present. Reproduced and modified from Simonds et al. (2012)
with permission.
(79% versus 46%, respectively). The greatest improvement in AHI is generally seen
in children with an AHI of >5 events·h−1 (moderate-to-severe OSA). Risk factors for
OSA persistence post-adenotonsillectomy include: severe OSA, obesity, retroposition
of the mandible, midface hypoplasia, neuromuscular diseases and complex disorders
like achondroplasia, Prader–Willi syndrome and Trisomy 21.
In addition to relief of upper airway obstruction, adenotonsillectomy may also have
beneficial eects on cognition, attention, behaviour and quality of life, as well as
resolution of nocturnal enuresis and improved somatic growth rate postoperatively. It
should be noted that a subgroup of children manifest rapid weight gain postoperatively
and ultimately, partial recurrence of OSA is seen. This is likely to be due to improved
food intake, reduced nocturnal work of breathing or hormonal changes. The
procedure is not devoid of complications – anaesthetic risks, postoperative pain,
airway compromise, bleeding and in very rare cases even death.
Uvulopalatopharyngoplasty has been applied to patients with Trisomy 21 or cerebral
palsy, and to patients with major craniofacial anomalies. Only case series on the
ecacy of the procedure have been published. Therefore, its usefulness in the
management of paediatric OSA is controversial. Other surgical interventions that may
decrease nasal resistance, such as radiofrequency of the inferior turbinates, have not
been studied systematically.
Intranasal corticosteroids administered for 4–12 weeks in children with mild-tomoderate OSA and adenoidal hypertrophy, or montelukast for 16 weeks, have been
shown to improve symptoms of nasal obstruction and PSG indices. They reduce upper
airway resistance and the severity of obstructive SDB by decreasing the adenoidal
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a) d)c)b)
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tissue volume. Topical corticosteroids and montelukast have also been administered,
showing benefits for children with residual OSA aer adenotonsillectomy.
Craniofacial procedures
Craniofacial procedures are part of the treatment plan for congenital craniofacial
deformities that aect respiration and feeding (mainly midface and/or mandibular
hypoplasia). Mandibular distraction osteogenesis has acquired an important position
in the management protocol of mandibular hypoplasia. With this procedure, the
tongue and the suprahyoid muscles are moved forward and the size of the pharyngeal
airway cross-sectional area increases due to gradual lengthening of the mandible.
In patients with Apert, Crouzon or Pfeier syndromes and moderate-to-severe OSA,
neither tonsillectomy nor adenoidectomy are sucient to improve OSA, and thus
midface advancement appears to be the treatment of choice. Coexistent anatomical
malformations of the nasopharynx are common, causing functional airway obstruction
at this level. Midface advancement usually improves respiration in the short term, but
the long-term ecacy of the procedure, defined as no need for respiratory support
for ≥2 years postoperatively, is not as clear as previously thought. OSA relapse aer
surgery may be due to overcorrection of midface hypoplasia, changing malocclusion
from class III to class II, and a lack of normal growth of the craniofacial bones.
Moreover, collapse of the pharyngeal airway is a functional abnormality that does not
necessarily improve aer the anatomic correction achieved by midface advancement.
Orthodontic procedures
This section will focus on the management of milder craniofacial abnormalities that
predispose to OSA compared with the profound congenital craniofacial deformities
discussed in the previous section.
A narrow upper airway accompanied by maxillary constriction and mandibular
retrusion is a common phenotype relating to paediatric OSA. Mandibular retroposition
predisposes to collapse of the upper airway during sleep and is associated with
posterior displacement of the tongue base, which results in further narrowing of the
upper airway and a high-arched (ogival) palate (figure 1).
Forward shi of the mandible has been achieved through use of oral appliances
and functional orthopaedic devices in children, allowing enlargement of the upper
airway lumen and improving respiration. Rapid maxillary expansion (RME) is a dentofacial orthopaedic procedure commonly used in young patients (>4 years of age) for
the treatment of constricted maxillary arches and maxillary transverse deficiencies.
Figure 1. Examples of common craniofacial phenotypes in children with OSA. a) High-arched
palate and oral breathing; b) narrow maxilla and retrognathia; c) hypotonic lips; d) increased
lower facial height. Reproduced and modified from Simonds et al. (2012) with permission.
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Patients usually display unilateral or bilateral posterior crossbite and anterior dental
crowding. There is oen reduced distance between the lateral walls of the nasal cavity
and the nasal septum, causing increased nasal airway resistance and consequently,
nasal respiratory diculties. RME can relieve nasal obstruction by increasing the
transverse dimensions of the maxilla, which in turn widens the nasal cavity.
The ecacy of RME has been demonstrated in non-obese children suering from nasal
breathing and OSA, but without enlarged tonsils or adenoids. All children had normal
anterior rhinometry with a significant decrease in AHI 4 months aer completion of the
procedure. The eects of RME were also evaluated in a group of non-obese children
with OSA and dental malocclusion i.e. ogival palate associated with deep bite, retrusive
bite or crossbite. RME was applied for 12 months. By the end of treatment, the mean
AHI had decreased significantly compared with baseline, reaching normal values
in most patients. An improvement in clinical symptoms was reported by parents –
reduced snoring, oral breathing, sleep apnoeas, and daytime sleepiness and tiredness.
The beneficial eects of RME persisted 2 years aer its completion.
Orthodontic therapy should therefore be encouraged in selected children with OSA;
early implementation may modify nasal breathing and respiration, thereby preventing
obstruction of the upper airway.
Another important treatment modality is oropharyngeal exercise derived from
speech therapy (myofunctional therapy). Oropharyngeal exercises may be an eective
treatment option for children with OSA, because they adjust physiological breathing
and eliminate oral breathing, both of which are involved in upper airway muscle
function and airway patency.
CPAP and NIPPV
The application of CPAP and NIPPV has been summarised in a 2022 ERS statement
on pediatric long-term noninvasive respiratory support.
Children with OSA and obesity, residual OSA aer adenotonsillectomy or sleep apnoea
with craniofacial abnormalities are candidates for CPAP, which is usually eective in
ameliorating apnoeas and hypopnoeas. During sleep, the child receives continuous
airflow delivered by the CPAP ventilator via various interfaces (nasal mask in most
cases or oronasal mask) to maintain a specific level of PAP.
Compliance with CPAP can be improved dramatically by adequate therapeutic
education of the patient and caregivers. Gradual acclimatisation and introduction of
the mask as the child sleeps, as well as continued gentle and intensive family support,
may improve adherence.
The pressure level that is necessary to ‘stent’ the pharyngeal airway so as to prevent
the collapse of its walls is determined in the sleep laboratory prior to prescribing the
CPAP ventilator. The recommended starting pressure level during titration is 4 cmH2O,
with a mean therapeutic pressure of 8 cmH2O. The goal of CPAP is to lower AHI to
<5 events·h−1.
In patients with OSA and concomitant alveolar hypoventilation due to neuromuscular
disorder or chronic lung disease, noninvasive bilevel positive pressure ventilation
should be used instead of CPAP.
Lifestyle management
A balanced diet and regular physical exercise should be implemented in all children
with OSA, particularly those who are obese. Limited evidence indicates that weight
loss by as much as 35% in children with OSA and severe obesity can be accompanied
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by an improvement in the severity of intermittent upper airway obstruction during
sleep. However, it should be noted that such a degree of weight reduction is dicult
to achieve and sustain. Avoidance of exposure to cigarette smoke and indoor allergens
are additional measures that could reduce upper airway inflammation, resistance to
airflow and the tendency for pharyngeal airway collapse.
Sleep hypoventilation syndromes: indications for treatment
Congenital central hypoventilation syndrome
Ventilatory support is life support for patients with congenital central hypoventilation
syndrome (CCHS) or central hypoventilation that is secondary to dierent causes.
Hypoventilation is most severe during sleep but may also occur during daytime in
some severely aected patients.
Patients with CCHS have decreased tidal volume and respiratory rate during sleep and
in some cases even during wakefulness. The main objective of ventilatory support in
central hypoventilation is to provide optimal ventilation all day, every day, beginning
immediately aer diagnosis in order to preserve optimal neurocognitive outcomes.
In patients with CCHS, use of positive pressure ventilation via tracheostomy is
recommended in the first several years of life, allowing early discharge home. Aer
6–8 years of age, most children with CCHS who require ventilatory support during
sleep can only be successfully transitioned from positive pressure ventilation via
tracheostomy to NIPPV. Direct comparisons between NIPPV and invasive ventilation
via tracheostomy in terms of outcome have not been carried out in CCHS.
NIPPV has been reported as initial treatment performed in some infants with CCHS.
However, there is no data on the long-term outcome of patients receiving NIPPV as
the initial and sole mode of ventilation. It must be kept in mind that O2 administration
without ventilatory support is inadequate in patients with central hypoventilation as
it increases O2 saturation (S
in pulmonary PH or coma.
), but worsens hypoventilation, subsequently resulting
pO
2
Respiratory pacing may be recommended for active children with central
hypoventilation who require ventilatory support day and night. These children may
combine dierent modes of ventilatory support, receiving positive pressure ventilation
during the night-time and using respiratory pacing in the daytime, so that they are
totally free of the ventilator during their diurnal activities. Regardless of the mode of
ventilation, patients with central hypoventilation require continuous monitoring with
pulse oximetry and end-tidal carbon dioxide tension (P
) during sleep.
ETCO
2
Neuromuscular disorders
The probability of developing nocturnal hypoventilation in neuromuscular diseases
depends on the diagnosis, age and extent of respiratory muscle weakness. For
example, in type I SMA, respiratory insuciency is likely to occur in the first few
months of life, whereas in Duchenne muscular dystrophy, SDB is not usually present
until the late teenage years. Sleep studies should be part of regular follow-up, and
for most authorities, the indication to start NIPPV is nocturnal hypoventilation on an
overnight recording of gas exchange, even if the patient is asymptomatic and daytime
P
is near normal. Successful NIPPV treatment is associated with resolution of
aCO
2
symptoms such as poor sleep, headaches or concentration/cognitive diculties, and
importantly it results in decreased morbidity and increased survival. In children with
severe progressive respiratory failure (e.g. due to type I SMA), NIPPV may be used to
improve symptoms and facilitate discharge home.
403ERS Handbook: Respiratory Sleep Medicine
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