AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
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INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Obstructive sleep apnea (OSA) syndrome was described more than a century ago. However, OSA in children was first recognized in the 1970s. OSA is a common, but underdiagnosed, condition in children that may lead to substantial morbidity if left untreated. Important recent advances in the understanding of the underlying pathophysiological mechanisms of OSA in children have been coupled with improved approaches to the diagnosis and management of OSA.

Pathophysiology

Snoring and obstructive apnea are symptoms of increased upper airway resistance. The ability to maintain upper airway patency during the normal respiratory cycle is the result of a delicate equilibrium between the forces that promote airway closure and dilation. This "balance of forces" concept was initially proposed by 2 independent groups and reflects the current line of thought regarding the underlying pathophysiological mechanisms that result in the clinical spectrum of obstructive apnea. The 4 major predisposing factors for upper airway obstruction include anatomic narrowing, abnormal mechanical linkage between airway dilating muscles and airway walls, muscle weakness, and abnormal neural regulation.

Anatomic narrowing

At any point in life, a smaller cross-sectional area of the upper airway is associated with decreased ability to maintain upper airway patency. In adults, the upper airway behaves as predicted by the Starling resistor model. According to this model, under conditions of flow limitation, maximal inspiratory flow is determined by the pressure changes upstream (nasal) to a collapsible site of the upper airway, and flow is independent of downstream (tracheal) pressure generated by the diaphragm. Pressures at which the airway collapses have been termed critical closing pressures, or P_crit. In other words, in the presence of a collapsible segment of the upper airway, such as the pharyngeal introitus, the overall resistance to airflow proximal to that segment is the major factor responsible for occlusion of the collapsible segment. This model explains why, for example, snoring and obstructive apnea worsen during a common cold (increased nasal-upstream resistance).

The validity of this model was also confirmed in children, and, interestingly, the collapsibility of the upper airway in children was reduced when compared with that of adults. As predicted by the Starling resistor model, the collapsible segment of the upper airway in children displayed less negative (higher and, therefore, more collapsible) pressures in children with OSA. Components that affect the upstream segment pressures or increase P_crit are of major consequence to the ability to maintain airway patency. For example, a viral cold or allergic rhinitis that induces increased secretion in the nasal passages and mucosal swelling is associated with increased nasal resistance to airflow. Not surprisingly, the magnitude of snoring and the severity of obstructive apnea are increased during periods in which the upstream segment pressure has been adversely affected.

The contribution of the various anatomical nasopharyngeal structures to P_crit and the interactions between these structures that lead to upper airway patency or obstruction during sleep are of obvious importance in increasing the understanding of the pathophysiology of OSA in children.

The static pressure and/or area relationships of the passive pharynx were endoscopically measured in 14 children with OSA and in 13 healthy children under general anesthesia with complete paralysis (Isono, 1998). Children with OSA closed their airways at the level of enlarged adenoids and tonsils at low positive pressures, whereas healthy children required subatmospheric pressures to induce upper airway closure. The cross-sectional area of the narrowest segment was significantly smaller in children with OSA and particularly involved the retropalatal and retroglossal segments. Thus, both congenital and acquired anatomic factors clearly play a significant role in the pathogenesis of pediatric OSA.

Abnormal mechanical linkage between airway dilating muscles and airway walls

Malposition or malinsertion of specific dilating muscles is likely to have major consequences on the mechanical dilating efficiency. Therefore, even if a major weakness is not present, the mechanical disadvantage imposed by muscle shortening or by displacement of the muscle insertion on the pharyngeal wall undoubtedly results in diminished ability to stiffen the airway, thus leading to increased collapsibility or elevation of P_crit.

Control of the upper airway size and stiffness depends on the relative and rhythmic contraction of a host of paired muscles, which include the palatal, pterygoid, tensor palatini, genioglossus, geniohyoid, and sternohyoid muscles. These muscles tend to promote a patent pharyngeal lumen and receive phasic activation in synchrony with phrenic nerve activation. Upon contraction, these muscles promote motion of soft palate, mandible, tongue, and hyoid bone. Although the coordinated action of these muscles during the respiratory cycle has yet to be deciphered, a reasonable generalization is that inspiratory muscle output stiffens the pharynx and related structures and enlarges the lumen.

The optimal activity of these muscles depends on their anatomic arrangement; for example, airway patency is compromised during increased neck flexion by changing the points of attachment of muscles acting on the hyoid bone, such that the resulting vector of their forces may be nullified. The activity of pharyngeal muscles greatly depends on various factors within the CNS and, more particularly, on the brainstem respiratory network. Wakefulness conveys a supervisory function that ensures airway patency, and sedative agents, which compromise genioglossal muscle activity, may result in significant upper airway compromise.

Mechanoreceptor- and chemoreceptor-mediated genioglossal activity is critical for maintenance of upper airway patency in healthy and micrognathic infants. Changes in genioglossal activity during transitions, from oral to nasal breathing and relative to P_crit, suggest that genioglossus activation is critical for airway patency in micrognathic infants.

Muscle weakness

Little evidence suggests that intrinsic muscle weakness is a major contributor to upper airway dysfunction in conditions other than those associated with neuromuscular disorders. However, in neuromuscular disorders, upper airway obstruction is frequently observed during sleep, further reinforcing the validity of the balance-of-pressures concept.

Abnormal neural regulation

Abnormal respiratory control does not appear to play a significant role in upper airway obstruction during sleep in children with OSA. The ventilatory response to hyperoxic hypercapnic challenge in children and adolescents with OSA was similar to that measured in age- and sex-matched control children (Marcus, 1998). Similarly, no differences were found in the ventilatory response to isocapnic hypoxia. Blunting in central chemosensitivity was reported in some children with OSA undergoing surgery, but, despite such reports, central chemosensitivity during sleep was similar in children with OSA and matched controls. However, arousal to hypercapnia was blunted, suggesting that subtle alterations in the central chemosensitive arousal network may have occurred in these children.

These subtle changes have been further substantiated by examining the ventilatory response to repeated hypercapnia, whereby reciprocal changes in respiratory frequency and tidal volume occur. In addition, children with OSA demonstrate impaired arousal responses to inspiratory loads during rapid eye movement (REM) and non-REM sleep compared with control children. Neural responses to hypoxia and hypercarbia have not been well studied in children with OSA and underlying syndromes.

In addition to the aforementioned considerations, diminished laryngeal reflexes to mechanoreceptor and chemoreceptor stimulation, with reduced afferent inputs into central neural regions underlying inspiratory inputs, can be present. For example, chemoreceptor stimuli, such as increased PaCO₂ or decreased PaO₂, stimulate the airway, dilating muscles in a preferential mode (ie, upper airway musculature is more stimulated than the diaphragm).

This preferential recruitment tends to correct an imbalance of forces acting on the airway and, therefore, maintains airway patency. Similarly, stimuli that result from suction pressures in the nose, pharynx, or larynx rapidly stimulate the activity of upper airway dilators. This effect is also preferential to the upper airway, causing some degree of diaphragmatic inhibition and, thus, compensating for increases in upstream resistance. The function of these upper airway receptors in children with adenotonsillar hypertrophy with and without OSA is not known.

In summary, several potential mechanisms in the maintenance of upper airway patency during sleep and wakefulness have been identified. Each of these mechanisms, or a combination thereof, plays a role in the causation of respiratory compromise in the healthy child or in children with clinical problems that predispose to OSA. A systematic approach to identification and modification of these mechanisms may lead to improvement in therapeutic approaches and avoidance of unnecessary morbidity in these patients.

Frequency

United States

Precise prevalence figures for OSA in children are currently unavailable. Estimates from limited field studies suggest that up to 2% of all children may be affected; however, the prevalence of snoring in the general pediatric population is much higher and has been estimated at 8-27%.

Mortality/Morbidity

Despite the misleadingly benign clinical presentation, the pathological consequences of OSA in children may be severe, and some pathological consequences are still being uncovered. These morbidities can be divided as the corollary of the 4 immediate consequences of upper airway obstruction during sleep, which include sleep fragmentation, increased work of breathing, alveolar hypoventilation, and intermittent hypoxemia.

• Sleep fragmentation
  • Experimental fragmentation of the sleep of healthy adults may be achieved with auditory stimuli–inducing arousals. Subjects who were awakened at various intervals during the night demonstrated performance decrements and increased sleepiness on the following day (Bonnet, 1985). This was also true when EEG arousals, rather than behavioral arousals, were induced.
  • The physiological and behavioral effects of partial and total sleep loss due to OSA in adults have been extensively investigated. Daytime tiredness or fatigue is a common symptom, although sleepiness, which is a subjective notion, may not be directly reported. Significant deterioration in functions that require concentration or dexterity, as well as automatic behavior with retrograde amnesia, disorientation, and morning confusion, have all been reported in patients with sleep fragmentation and has led to the term sleep drunkenness. In addition, personality changes and abnormal behavioral outbursts follow sleep fragmentation. Aggressiveness, irritability, anxiety attacks, and depression may occur.
  • Sleep fragmentation in adults affects neuropsychological and cognitive performance. No evidence suggests such impairments are absent in children, and such deleterious effects may be worse, given that the child's brain is undergoing active developmental changes. Reports of decreased intellectual function in children with tonsillar and adenoidal hypertrophy date from 1889 when Hill reported on "some causes of backwardness and stupidity in children." Schooling problems have been repeatedly reported in case studies of children with OSA and, in fact, may underlie more extensive behavioral disturbances, such as restlessness, aggressive behavior, excessive daytime sleepiness, and poor test performances.
  • The neurocognitive and behavioral consequences of disrupted sleep architecture and hypoxemia caused by sleep-disordered breathing in children with OSA have only recently been defined by appropriate scientific methodology in the pediatric population. However, some studies have documented that children with sleep disorders tend to have behavioral problems similar to those observed in children with attention deficit hyperactivity disorder (ADHD). For example, a survey study of 782 children recently documented daytime sleepiness, hyperactivity, and aggressive behavior in children who snore (Gozal, 1998). Inverse correlations between memory and learning performance and the severity of OSA were also found, and other studies have clearly demonstrated significant improvements in school performance after treatment of OSA.
  • In a study of 19 preschool-aged children with OSAÿ, prior to tonsillectomy and adenoidectomy (T&A), cognitive scores were significantly lower in children with OSA versus control subjects (Montgomery-Downs, 2005). Following T&A, the scores of the children with OSA improved compared with preoperative scores and did not differ from those of the matched controls. This underscores the importance of diagnosis and treatment, insofar as the cognitive impairments of children, unlike adults, take place in the developing brain.
  • Sleep deprivation, sleep disruption, and intermittent hypoxia independently may be sufficient to cause daytime effects in vulnerable children. Preliminary evidence suggests that, if left untreated, sleep-disordered breathing may impose long-term decrements in academic performance and the combination of 2 or more of these factors can result in particularly impaired daytime functioning.
  • Although empirical awareness of the deleterious consequences of OSA on neurocognitive function and behavior is well established, the scientific foundation for the causal mechanisms underlying such detrimental effect on intellectual function has yet to be determined. This endeavor is currently a major focus of research programs.
• Increased work of breathing
  • A major cardiovascular consequence of OSA in adults is arterial hypertension. Although the pathophysiological mechanisms of elevation in arterial tension are still under debate, intermittent arousal, hypoxemia, and increases in cardiac afterload during the obstructive apneic event apparently lead to enhanced sympathoadrenal discharge and heightened sympathetic tone, even during waking hours. Significant alterations in autonomic nervous system tone have been documented in children with OSA, and modest diurnal elevations in arterial blood pressure have also been reported. The long-term effects of this process in childhood and the effect on adult health are unknown.
  • A prominent clinical manifestation of increased work of breathing in children with OSA is failure to thrive (FTT). Indeed, reports from the early 1980s found more than a 50% prevalence of FTT in patients with pediatric OSA, and significant catch-up growth patterns have recently been reported after T&A, even in children with obesity and OSA. The causes of poor growth include anorexia and dysphagia due to tonsillar and adenoid hypertrophy, diminished or altered patterns of nocturnal growth hormone secretion, hypoxemia, acidosis, and increased work of breathing during sleep.
  • In a recent study, a substantial reduction in resting energy expenditure was reported after adenotonsillectomy in children with OSA and FTT with concomitant gains in body weight (Marcus, 1994). Another study demonstrated significant recovery in the insulin growth factor 1 axis (Bar, 1999). These findings suggest that an important factor that mediates FTT in pediatric OSA involves the combination of increased energy expenditure caused by increased respiratory effort and disruption of the pathways of the growth hormone somatomedin.
• Alveolar hypoventilation
  • Intermittent hypercapnia frequently occurs among patients with various respiratory disorders, becomes more prominent or sustained during sleep, and is minimal or absent during wakefulness.
  • Children with OSA who snore exhibit classic intermittent alveolar hypoventilation, which is elicited by increased upper airway resistance, concurrent with diminished or insufficient compensatory mechanisms developing during sleep.
  • In adults with OSA, blunted ventilatory drive to hypercapnia during wakefulness develops and may potentially contribute to the pathophysiology of upper airway obstruction.
  • In contrast, waking and sleeping ventilatory responses to hypercapnia in children with OSA are similar to those measured in healthy children. However, arousal responses are attenuated during sleep, suggesting that long-standing interactions between sleep and upper airway resistance in these children primarily affect arousal mechanisms during sleep. Another potential contribution of alveolar hypoventilation and hypercapnia during sleep may relate to exacerbation of the effect of intermittent hypoxia on the vasomotor tone of the pulmonary circulation.
• Intermittent hypoxemia
  • A serious consequence of intermittent hypoxia is elevation of pulmonary artery pressure due to pulmonary vasoconstriction, such that chronic intermittent nocturnal hypoxemia leads to development of pulmonary hypertension and cor pulmonale. In 27 pediatric patients with moderate-to-severe OSA, radionuclide assessment of right ventricular function revealed reduced ejection fraction in 37% of these children and wall motion abnormality in 45% (Tal, 1988). Another potentially serious consequence of intermittent hypoxia may involve its long-term deleterious effects on neuronal and intellectual function. Indeed, in a study on an animal model developed in the coauthor's laboratory, intermittent hypoxia was associated with significant increases in neuronal apoptosis and reduced functionality within brain regions that mediate learning and memory.
  • Because the peak age for OSA coincides with that of a critical period for brain development, delayed diagnosis and treatment of OSA possibly imposes a greater burden on vulnerable brain structures and ultimately hampers the overall neurocognitive potential of children with OSA.
  • Neurobehavioral disturbances and diminished learning capabilities, stunted growth, altered respiratory load response patterns, and pulmonary hypertension are major consequences of OSA in childhood. Early diagnosis and prevention of such morbidities are fundamental aspects of adequate pediatric care in the community.

Race

The current evidence assigns a particularly higher risk for OSA among black children compared with white children. However, the high frequency of OSA among adult Asian populations indicates that the anthropometric characteristics of the craniofacial structures in this racial group also predispose for higher OSA rates in children. The frequency of OSA in Hispanic children is equal to that of white children.

Sex

The prevalence of snoring and OSA among prepubertal children does not differ based on sex. In older adolescents, a male preponderance emerges that essentially reflects the typical male predominance observed in the adult population.

Age

The peak prevalence is in children aged 2-8 years (coinciding with adenotonsillar lymphatic tissue growth). Preterm babies are at risk for more obstructive events while supine but are still at a lower risk of death from sudden infant death syndrome.

CLINICAL

Section 3 of 11  

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History

The clinical presentation of a child with obstructive sleep apnea (OSA) syndrome is nonspecific and requires increased awareness by the primary care physician. Indeed, the medical history is usually normal, unless the pathophysiology of sleep-associated airway obstruction is related to one of the various conditions delineated in Causes.

Clinical findings of tonsillar enlargement or obesity should prompt questioning regarding snoring. Family history of snoring, allergies, and exposure to environmental tobacco smoke are all strongly related to snoring. In the otherwise healthy child, parents principally report snoring during sleep. History of loud snoring 3 or more nights per week should increase suspicion of OSA.

Parents occasionally comment on breathing difficulties during sleep (eg, gasps or heroic snorts), unusual sleeping positions, morning headaches, daytime fatigue, irritability, poor growth and weight gain, and behavioral problems. Nevertheless, even in cases in which a sleep specialist conducts the diagnostic interview, theaccuracy of OSA prediction is poor and does not exceed a sensitivity and specificity of 50-60%, particularly in distinguishing OSA from benign snoring.

Physical

Physical examination findings are generally normal while awake, with the exception of findings related to the predisposing conditions noted (see Causes). Plot a growth chart, including a height-, weight-, and age-adjusted body mass index in order to identify obesity or FTT. Carefully examine the nasal passages for mucosal swelling, cobblestone pattern of the mucosa, polyps, and reduced nasal airflow. Carefully evaluate the size and position of tonsils and uvula, particularly noting hypertrophy or malformation. Unfortunately, although tonsillar hypertrophy may contribute to the severity of OSA, the data available to date have not established a clear relationship between tonsillar size and frequency or severity of apneic events. Furthermore, although more prevalent in patients with OSA, tonsillar hypertrophy is also common in healthy children without OSA, with a prevalence as high as 57%.

Document the width and height of the hard palate, as well as the overall appearance of the soft palate, looking for evidence of cleft or pharyngeal narrowing or compression.

The Mallampati classification may help quantify the degree of oropharyngeal anatomical obstruction. This classification is based on the structures visualized with maximal mouth opening and the tongue extended. The classes are determined by the visible structures. In class I, the soft palate, fauces, uvula, and pillars are visible. In class II, the soft palate, fauces, and a portion of uvula are visible. In class III, the soft palate and base of uvula are visible. In class IV, only the hard palate is visible. The higher the Mallampati classification, the greater the likelihood of oropharyngeal obstruction.

The relative position of the chin with respect to the maxilla is helpful in the identification of mild micrognathia or retrognathia.

Cardiac examination may reveal the presence of a prominent pulmonic second heart sound suggestive of pulmonary hypertension.

No other distinctive findings are usually present unless one of the medical conditions is present (see below).

Causes

Medical conditions associated with OSA in children include the following:

• Tonsillar and adenoid hypertrophy
• Achondroplasia, Crouzon syndrome, and Apert syndrome
• Neuromuscular disorders (eg, Duchenne muscular dystrophy, spinal muscular atrophy)
• Myelomeningocele
• Obesity
• Pierre Robin sequence
• Cerebral palsy
• Down syndrome
• Sickle cell diseases
• Choanal stenosis
• Hypothyroidism
• Klippel-Feil syndrome and Hallermann-Streiff syndrome
• Mucopolysaccharidosis
• Osteopetrosis
• Oropharyngeal papillomatosis
• Beckwith-Wiedemann syndrome
• Pfeiffer syndrome, Prader-Willi syndrome, and Treacher-Collins syndrome

DIFFERENTIALS

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Other Problems to be Considered

Primary snoring
Upper airway resistance syndrome
Other sleep disorders

WORKUP

Section 5 of 11  

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Lab Studies

• Parameters measured during an overnight sleep study include the following:
• • Sleep state (>2 EEG leads)
  • Electrooculogram (right and left)
  • Submental electromyelogram (EMG)
  • Airflow at nose and mouth (thermistor, capnography, or mask and pneumotachygraph)
  • Chest and abdominal wall motion (impedance or inductance plethysmography)
  • Electrocardiogram (preferably with R-R interval derivation technology)
  • Pulse oximetry (including a pulse waveform channel)
  • End-tidal carbon dioxide (sidestream or mainstream infrared sensor)
  • Video camera monitor with sound montage (analog or digital)
  • Transcutaneous oxygen and carbon dioxide tensions (in infants and children <8 y)
• The following sleep study parameters are under investigation:
• • Nasal pressure–flow measurements
  • Esophageal manometry
  • Continuous noninvasive blood pressure monitoring
  • Autonomic nervous system tone using finger tonometry
• These studies, continuously monitored by appropriately trained technical personnel, are relatively onerous, and, because of their relative unavailability, they impose long waiting periods between referral and testing times. For these reasons, attempts have recently been made to evaluate the role of ambulatory and domiciliary nighttime studies in defining a more practical algorithmic approach for the diagnosis of obstructive sleep apnea (OSA) in children.
• The results of initial studies indicate that, although home audio tape recordings appear relatively insensitive, oximetry trend analysis with or without additional measures may provide a useful alternative in establishing the definitive cases that require intervention. However, despite high specificity, home oximetry has low sensitivity, and children with negative findings on studies still require complete nocturnal polysomnography.
• Compared with the adult literature, the available normative data for sleep and cardiorespiratory parameters are rather sparse in the pediatric literature, such that most pediatric sleep laboratories use individually established reference ranges rather than referring to an authoritative text. Nevertheless, the general consensus criteria for a normal finding on sleep study are presented in the following table and have been derived from the published literature on this subject and the authors' experience:

  Reference Range Parameters for Sleep Gas Exchange and Gas Exchange in Children

  Criterion	Reference Range Values
  Sleep latency	>10 min
  Total sleep time (TST)	>5.5 hours
  Percent REM sleep	>15% of TST
  Percent stage 3-4 non-REM sleep	>25% of TST
  Respiratory arousal index*	<5
  Periodic leg movements*	<1
  Apnea index*	<1
  Hypopnea index†	<3
  Nadir oxygen saturation	>92%
  Mean oxygen saturation	>95%
  Desaturation index‡	<5
  Highest carbon dioxide	52 mm Hg
  Carbon dioxide >45 mm Hg	<20% of TST

  * Number per hour of TST
  † Nasal/esophageal pressure catheter; number per hour of TST
  ‡ >4% for 5 seconds; number per hour of TST
• The adult criteria usually used around the world for the diagnosis of OSA do not apply to children. In fact, the finding of 10-15 obstructive apneic events per hour of sleep, which represents mild OSA in an adult patient in whom treatment may not even be contemplated, represents a sleep-related respiratory disturbance corresponding to a severely affected child definitely in need of therapeutic intervention. Thus, an apnea-hypopnea index (AHI) of more than 5 events per hour clearly represents an indication for treatment in children. An AHI of fewer than 3 events per hour does not require any intervention, and, in children with an AHI of more than 3 but fewer than 5 events per hour, the cost-benefit ratio of treatment remains to be determined.

Imaging Studies

• Assessment of tonsillar size usually does not require any type of imaging; however, lateral neck radiographs can be used to determine adenoid size. Although MRI can provide very detailed images of soft tissues and bony structures underlying the nasopharynx, such images are not usually required, except in cases of suspected ÿaberrant anatomy.
• Other diagnostic studies may be warranted to evaluate for complications of OSA or to better assess the contribution of an underlying condition. In patients with severe OSA, electrocardiography and echocardiography are particularly important to assess for pulmonary hypertension and cor pulmonale.

Other Tests

• Currently, the only available tool for definitive diagnosis of OSA is an overnight polysomnographic evaluation in the sleep laboratory. An overnight polysomnographic study usually includes multiple channels that aim to monitor sleep state, as well as cardiac and respiratory parameters (see Image 2).

Procedures

• Nasopharyngoscopy or direct laryngoscopy and bronchoscopy may be required to determine anatomy prior to contemplated otolaryngologic surgery.

TREATMENT

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Medical Care

• Medical therapy
  • In general, nonsurgical therapy is very limited for the typical patient with obstructive sleep apnea (OSA).
  • For allergic rhinitis or conditions associated with decreased nasal airflow, efforts to improve nasal patency may be of benefit. Thus, the use of antihistaminic preparations and topical nasal therapy may lead to temporary relief.
  • Although systemic steroids do not show any evident beneficial response, studies demonstrate that ÿintranasal steroids and oral leukotriene modifier therapy can eliminate residual OSA following surgery, and these agents may have a role in improving clinical outcomes without surgery. Although such topical therapy may primarily treat OSA, these approaches are currently investigational.
• Positive-pressure ventilation
• • In recent years, positive-pressure ventilation administered via a noninvasive interface (nasal mask) has become a safe, efficient, and viable alternative to further surgery or tracheotomy in children and infants with unresolved OSA after T&A.
  • An important distinction must be made between continuous positive airway pressure (CPAP) and bilevel (or biphasic) positive airway pressure (BiPAP). In CPAP, airway pressure is maintained above atmospheric pressure throughout the respiratory cycle. In BiPAP, pressure is delivered during the inspiratory cycle; exhalation then occurs at either atmospheric pressure or at a preset positive airway pressure, such that differences between inspiratory and expiratory pressures are usually greater than 10 cm H₂O. The BiPAP device may be set to control ventilation entirely (control mode), to deliver breaths only when triggered by a threshold negative pressure or nasal flow generated by the patient (assist mode), or both (assist/control mode). As CPAP does not involve a respiratory phase change in pressure, no control or assist modes are available.
  • Another important aspect of these interventions involves the patient-machine interface. The use of nasal prongs, nasal masks, or facemasks requires individualized case-by-case consideration. However, when a silicone mask is selected, particular care to ensure that the mask fits snugly and is comfortable to the patient is essential for ensuring successful intervention. Pediatric masks are currently available in several sizes and for particular clinical conditions, such as craniofacial syndromes. Custom-made masks can be ordered to fit the facial contours.
  • Inappropriately fit masks inevitably leak, and efforts to seal these leaks frequently result in pressure sores on the bridge of the nose. Bubble-cushioned masks have been developed and sometimes palliate the severity of the air leak while adding to the patient's comfort. In addition, air leaks are more frequently directed upward and may irritate the conjunctiva, leading to increased lacrimation and eye discomfort. Tolerance of CPAP or BiPAP may be greatly increased by devoting a period of time to condition the patient to use the mask during waking hours, particularly in young or developmentally delayed patients.
  • Pay attention to the mask manifold to ensure that no pressure vectors are generated. Multiple techniques may be used to secure the mask and primarily include Velcro, elastic straps, or a tissue cap. Again, the importance of the patient's comfort cannot be overemphasized. Finally, implement adequate parental training and behavioral techniques designed to improve the acceptance and tolerance to these devices in order to increase patient and family compliance. Over the last decade, CPAP has been increasingly used in children as a successful alternative to upper airway surgery or tracheotomy. However, midfacial hypoplasia may develop with long-term use, particularly in children with neuromuscular weakness. In other situations, temporary palliation using supplemental oxygen may be implemented until surgery, provided that sufficient attention is given to the possibility that severe hypercapnia may develop.

Surgical Care

• Tonsillectomy and adenoidectomy
  • Although OSA has multiple etiologies in children, once the diagnosis of OSA has been established and its severity assessed, T&A is usually the first line of treatment. Nevertheless, tonsillotomy, rather than tonsillectomy, has been recently advocated as equally effective with less postoperative morbidity. Most of these surgical procedures can be performed safely on an outpatient basis.
  • Notwithstanding the surgery being planned, carefully consider the existence of risk factors for perioperative morbidity and adverse outcomes in the surgical planning. Children with severe OSA, children younger than 2 years, and children with craniofacial syndromes or other conditions that contribute to the pathophysiology of OSA are at a higher risk for surgical complications. For example, in children with severe OSA, the existence of pulmonary hypertension and right ventricular dysfunction has been linked to the onset of cardiac arrhythmias during the process of anesthesia induction. Thus, preoperative echocardiographic assessment is indicated in these patients.
  • Similarly, for all of the risk categories mentioned above, an obtunded patient in whom the anesthetic effects on upper airway tone and reflexes are still compromised has a high risk of postsurgical upper airway obstruction. Finally, the development of idiopathic pulmonary edema following the relief of upper airway obstruction has also been noted. Therefore, in this high-risk group of patients, pursue overnight cardiorespiratory monitoring in the intensive care unit.
• Other surgical procedures: Additional surgical options may include uvulopharyngopalatoplasty, epiglottoplasty, and mandibular advancement procedures. However, most facilities lack extensive experience with these procedures in children, and the overall outcomes from these interventions have not been appropriately documented in the pediatric population. Thus, seriously consider nonsurgical alternatives before recommending additional surgery.
• Tracheotomy: In the past, when surgery did not relieve the degree of sleep-associated respiratory disturbance, a tracheotomy was frequently performed. Currently, this alternative is rarely, if ever, pursued because of the development of noninvasive approaches to maintain upper airway patency during sleep.

Consultations

For the otherwise healthy child with enlarged tonsils and adenoids, consultation with a pediatric sleep specialist and referral to a pediatric sleep laboratory for diagnosis are usually sufficient. When findings support the existence of OSA, refer the patient to a pediatric otolaryngologist for T&A and take appropriate perioperative and postoperative precautions in higher-risk groups. When obesity is present, refer the patient to a nutritional interventional program. Similarly, pursue echocardiography and input from a pediatric cardiologist when pulmonary hypertension is clinically suspected.

When craniofacial syndromes or neuromuscular disorders are the major cause of OSA, a multidisciplinary approach is mandatory for improved outcomes.

Diet

Introduce an appropriate diet in patients who are obese to facilitate weight reduction. Weight reduction is most successful with the aid of a nutritionist or an established weight reduction program.

MEDICATION

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Preliminary studies suggest that intranasal steroids and an oral leukotriene modifier therapy may reduce the severity of obstructive sleep apnea (OSA); however, this intervention is currently considered investigational.

FOLLOW-UP

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Further Outpatient Care

• In most otherwise healthy children with obstructive sleep apnea (OSA), T&A results in complete resolution of the problem, and a postsurgical evaluation in the sleep laboratory is usually not recommended. ÿHowever, residual mild sleep-disordered breathing is found in more than one third of these patients after T&A, particularly those included in the high-risk category. Thus, T&A alone may not suffice, and polysomnographic evaluation 6-8 weeks after T&A may confirm the need for additional treatment, including the use of intranasal steroids and oral leukotriene modifier therapy or CPAP/BiPAP.
• Patients treated with noninvasive ventilation require close follow-up by a pediatric pulmonologist and may periodically require a repeat polysomnographic evaluation. Treat patients who are found to have significant hypoxemia during polysomnography as soon as possible with overnight supplemental oxygen until T&A can be performed. Carefully assess the patient when using oxygen because of the rare possibility that significant hypercapnia may develop during the night.

Deterrence/Prevention

• Although no specific prevention has been reported, a high index of suspicion in patients with predisposing conditions or suggestive history is necessary for early detection. The need for increased awareness of and screening for OSA among primary care providers is significant. History obtained during preventive health visits should include questions regarding snoring (frequency, quality), obvious nocturnal airway obstruction or apnea, restless sleep, mouth breathing, daytime inattention, hyperactivity or hypersomnolence, and family history of OSA. Loud snoring 3 or more nights per week warrants further investigation.
• Obesity is increasing in children; 16-33% of children and adolescents are obese. Primary care providers should provide basic weight loss information and support and readily refer patients to a pediatric weight loss program. A pediatric sleep disorders clinic should work closely with a weight loss program and can be a portal of entry for a patient into such care systems.

Complications

• Complications of OSA are discussed in detail in Mortality/Morbidity. Complications include deficits in neurocognitive function, FTT, alterations in autonomic tone, modest elevations in arterial blood pressure, pulmonary hypertension, and cor pulmonale. Children with obstructive sleep apnea have an increased prevalence of night terrors and other parasomnias.

Prognosis

• In children with enlarged tonsils and adenoids that lead to OSA, a T&A usually results in complete cure, although no definitive studies have clearly demonstrated this issue. The outcome of patients who require extensive surgical management obviously depends on the severity of the condition that leads to upper airway compromise. With the emergence of noninvasive ventilation as an alternative option for these children, upper airway obstruction during sleep can be conservatively and successfully managed in most children. In children with FTT, treatment of OSA leads to resolution of the somatic growth disturbance. Similarly, pulmonary hypertension resolves. Although major improvements in neurobehavioral outcomes are expected, data are currently insufficient to support a complete recovery in some of the cognitive abilities affected by OSA.

Patient Education

• Compliance issues are of particular importance in patients treated with noninvasive ventilation. Weight loss through an appropriate program of diet and exercise is clearly beneficial for patients with OSA who are obese.
• For excellent patient education resources, visit eMedicine's Sleep Disorders Center. Also, see eMedicine's patient education article Disorders That Disrupt Sleep (Parasomnias).

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Refer patients in whom obstructive sleep apnea (OSA) is diagnosed following polysomnography for appropriate therapy. Failure to make an appropriate referral could result in legal action if a patient were to develop complications that could be attributed to OSA, such as cor pulmonale.

Special Concerns

• Please see Sleep Apnea for additional reading.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Medical complications associated with obstructive sleep apnea in children.
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Media file 2:  Compressed overnight polysomnography tracing of a 6-year-old boy who snores, showing multiple events of obstructive apnea (green-shaded areas) associated with oxyhemoglobin desaturation (yellow-shaded areas) and EEG arousals (red-shaded areas).
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Media file 3:  Parameters monitored during an overnight pediatric sleep study.
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Media file 4:  Normal parameters for sleep gas exchange and gas exchange in children.
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Media type:  Image

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• ATS. Cardiorespiratory sleep studies in children. Establishment of normative data and polysomnographic predictors of morbidity. Am J Respir Crit Care Med. Oct 1999;160(4):1381-7. [Medline]. [Full Text].
• ATS. Standards and indications for cardiopulmonary sleep studies in children. Am J Respir Crit Care Med. Feb 1996;153(2):866-78. [Medline].
• Bar A, Tarasiuk A, Segev Y, et al. The effect of adenotonsillectomy on serum insulin-like growth factor-I and growth in children with obstructive sleep apnea syndrome. J Pediatr. Jul 1999;135(1):76-80. [Medline].
• Bonnet MH. Effect of sleep disruption on sleep, performance, and mood. Sleep. 1985;8(1):11-9. [Medline].
• Gozal D. Sleep-disordered breathing and school performance in children. Pediatrics. Sep 1998;102(3 Pt 1):616-20. [Medline]. [Full Text].
• Isono S, Shimada A, Utsugi M, et al. Comparison of static mechanical properties of the passive pharynx between normal children and children with sleep-disordered breathing. Am J Respir Crit Care Med. Apr 1998;157(4 Pt 1):1204-12. [Medline]. [Full Text].
• Kheirandish L, Goldbart AD, Gozal D. Intranasal steroids and oral leukotriene modifier therapy in residual sleep-disordered breathing after tonsillectomy and adenoidectomy in children. Pediatrics. Jan 2006;117(1):e61-6. [Medline].
• Loughlin GM, Carrol JL, Marcus CL, eds. Sleep and Breathing in Children: a Developmental Approach. In: Lung Biology in Health and Disease. New York, NY:. Marcel Dekker, Inc;2000.
• Marcus CL. Pathophysiology of childhood obstructive sleep apnea: current concepts. Respir Physiol. Feb 2000;119(2-3):143-54. [Medline]. [Full Text].
• Marcus CL, Ward SL, Mallory GB, et al. Use of nasal continuous positive airway pressure as treatment of childhood obstructive sleep apnea. J Pediatr. Jul 1995;127(1):88-94. [Medline]. [Full Text].
• Marcus CL, Carroll JL, Koerner CB, et al. Determinants of growth in children with the obstructive sleep apnea syndrome. J Pediatr. Oct 1994;125(4):556-62. [Medline].
• Marcus CL, Lutz J, Carroll JL, Bamford O. Arousal and ventilatory responses during sleep in children with obstructive sleep apnea. J Appl Physiol. Jun 1998;84(6):1926-36. [Medline]. [Full Text].
• Montgomery-Downs HE, Crabtree VM, Gozal D. Cognition, sleep and respiration in at-risk children treated for obstructive sleep apnoea. European Respiratory Journal. 2005;25(2):336-42. [Medline]. [Full Text].
• Richards W, Ferdman RM. Prolonged morbidity due to delays in the diagnosis and treatment of obstructive sleep apnea in children. Clin Pediatr (Phila). Feb 2000;39(2):103-8. [Medline].
• Rosen CL. Clinical features of obstructive sleep apnea hypoventilation syndrome in otherwise healthy children. Pediatr Pulmonol. Jun 1999;27(6):403-9. [Medline]. [Full Text].
• Tal A, Leiberman A, Margulis G, Sofer S. Ventricular dysfunction in children with obstructive sleep apnea: radionuclide assessment. Pediatr Pulmonol. 1988;4(3):139-43. [Medline].

Article Last Updated: Feb 26, 2007