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Continuous positive airway pressure (CPAP) for pediatric obstructive sleep apnea

Continuous positive airway pressure (CPAP) for pediatric obstructive sleep apnea
Literature review current through: Jan 2024.
This topic last updated: Mar 09, 2023.

INTRODUCTION — Continuous positive airway pressure (CPAP) therapy is one of several options for treatment of obstructive sleep apnea (OSA) in children. The goals of CPAP therapy are to ensure unobstructed breathing, good sleep quality, improved daytime function, and in some cases, rest from work of breathing. CPAP can be used temporarily between facial surgeries, while the child grows, or chronically in the presence of a craniofacial malformation or other anatomical predisposition for obstruction [1]. When started early in the course of OSA, CPAP therapy should mitigate the associated cardiovascular risk.

The challenges of CPAP titration in children are illustrated by the following vignette: A six-year-old obese male with OSA undergoes CPAP titration. The sleep technologist increases the pressure progressively from 6 to 15 cm H20 pressure with continued signs of paradoxical breathing, snoring, gasping, and increased leak measured on the polysomnogram (waveform 1). The technologist then switches the mode to bilevel ventilation with no change in the respiratory distress. The child arouses frequently and wakes up crying refusing to complete the study. What was the cause of failure in this CPAP titration?

This topic review will discuss a proactive approach to initiating CPAP therapy in children, including desensitization, equipment selection, promoting adherence to therapy, and troubleshooting problems. Other issues related to OSA in children are discussed in the following topic reviews:

(See "Evaluation of suspected obstructive sleep apnea in children".)

(See "Management of obstructive sleep apnea in children".)

(See "Adenotonsillectomy for obstructive sleep apnea in children".)

TREATMENT DECISIONS — In children, adenotonsillar hypertrophy is a common contributing factor to OSA. As a result, adenotonsillectomy is first-line therapy for otherwise healthy children who have moderate or severe OSA and adenotonsillar hypertrophy. Adenotonsillectomy in the absence of substantial hypertrophy may also be initial therapy for children with multifactorial OSA, including contributions from obesity or facial skeletal anatomy, if appreciable adenotonsillar tissue is present. (See "Adenotonsillectomy for obstructive sleep apnea in children".)

Indications — Positive airway pressure therapy (continuous positive airway pressure [CPAP] or bilevel positive airway pressure [BPAP]) is an appropriate option for children with moderate or severe OSA and any of the following:

Minimal adenotonsillar tissue

Persistent OSA despite adenotonsillectomy

Craniofacial abnormalities

Adenotonsillectomy is contraindicated (eg, too risky)

Strong preference for a nonsurgical approach

For such patients, CPAP treats OSA by overcoming the critical closing pressure of the pharynx to maintain a patent pharyngeal airway [2,3]. (See "Management of obstructive sleep apnea in children", section on 'Choice of therapy'.)

Less common clinical uses of CPAP in children are:

Atelectasis – To help expand the lung and recruit alveoli

Cardiogenic pulmonary edema – To improve gas exchange

Obstructive lung disease (eg, severe asthma) – To reduce air-trapping (dynamic hyperinflation) resulting from lower airway obstruction or constriction

Contraindications — CPAP is usually not appropriate for patients with the following conditions:

Recent or active pneumothorax – Because higher airway pressures may worsen the air leak or cause recurrence.

Aspiration risk – Because CPAP may increase the risk of aspiration in patients with copious oral secretions and/or swallowing dysfunction. In children with copious oral secretions, permitting open mouth breathing and oral drainage may reduce the aspiration risk during CPAP use, but full face masks are contraindicated. Children with frequent aspiration of either stomach contents or oral secretions should have these problems addressed before attempting home positive pressure therapy.

Hypoventilation – Children with significant hypoventilation that is primarily due to causes other than OSA may not be treated effectively using CPAP. Hypoventilation is characterized by carbon dioxide (CO2) retention detected on blood gas evaluation (elevated partial pressure of CO2 [PaCO2]) or polysomnography (elevated end-tidal CO2). BPAP and other noninvasive or invasive ventilation strategies can be used for patients with hypoventilation. (See "Congenital central hypoventilation syndrome and other causes of sleep-related hypoventilation in children".)

PREPARING THE ANATOMY FOR SUCCESSFUL AIRFLOW — Nasopharyngeal obstruction, typically manifested by mouth breathing, presents an important obstacle to CPAP treatment and must be addressed before proceeding with CPAP titration. In patients with nasal obstruction, increasing CPAP pressure does not improve nasal air flow and, in fact, can cause discomfort or pain as forced air reaches sinus pockets or the Eustachian tubes. Nasopharyngeal obstruction is common among children with OSA, whereas in adults with OSA, the obstruction is typically in the oropharynx or larynx [4].

In children, nasopharyngeal obstruction may be caused by congenital anatomic abnormalities and also by chronic factors that alter nasopharyngeal development. As examples, nasopharyngeal development during infancy and early childhood is affected by bottle feeding rather than breastfeeding or prolonged intubation in infancy [5-7]. Similarly, chronic nasopharyngeal obstruction from adenoid hypertrophy, septal deviation, or turbinate obstruction leads to open-mouth breathing, which, in turn, promotes development of a narrow high-arched palate, crossbite, and slow maxillary development (often referred to as the "adenoid face" (figure 1)) [8-11]. These changes can become permanent if not altered by interventions to improve nasal airflow. Mouth breathing has a high resistance to airflow, causing snoring and obstruction during sleep [12].

Prior to initiating CPAP, the child should be examined to determine whether they can breathe nasally. Any significant nasopharyngeal obstruction should be corrected before attempting CPAP. If the obstruction is caused by soft tissue hypertrophy or swelling, therapy may include medical approaches such as nasal irrigations, topical nasal steroid spray, and oral leukotriene antagonists. Patients with fixed anatomic obstruction may require surgical intervention [13].

To minimize problems with nasal obstruction, we encourage children to perform nasal irrigation prior to CPAP application each night. Kits for nasal washes are available commercially, and videos demonstrating their use are available on the internet. As an example, our dedicated sleep respiratory therapist made an educational video featuring his five-year-old son happily performing his own nasal irrigation. Such videos help to overcome reluctance and concerns among patients and their families (this video is available for public viewing here on YouTube) [14].

FIRST STEPS IN CPAP INTRODUCTION — After determining that CPAP is appropriate anatomically, the next step is to assess whether the child and caregiver are ready and willing to proceed with CPAP therapy.

In our practice, we perform mask fitting and trials of pressure introduction in the office setting, allowing us to assess the child's tolerance to CPAP therapy and model behavioral strategies to overcome resistance. As an example, some children will tolerate the mask placement on the face but not the airflow from the machine, and observing their behavior provides the starting point for training.

Forming a partnership — CPAP adherence in children is variable. Success depends on effective and persistent coaching by the child's parents/caregivers. To be effective in this role, they must understand the importance of CPAP therapy for their child, acquire strategies for overcoming resistance, and have tools and support for troubleshooting problems [15]. Effective training and support of the family is an extended process, often best accomplished by a multidisciplinary team in a pediatric sleep center. Resources may include personnel (respiratory, nursing, clinician, or others), written or video instructions, multiple office visits for follow-up, and phone calls for troubleshooting. Anticipatory guidance about common problems helps families to be more confident and effective.

Desensitization — Psychological preparation for CPAP therapy is useful for all children starting CPAP therapy, but is especially necessary in children with developmental disorders, anxiety, or behavior disorders. A strategy appropriate to the child's age and personality can be developed in consultation with parents/caregivers. If a slow introduction is required, we arrange clinic visits for training. We use these to orient the parent and child to the equipment, provide individual instruction of the child using age-appropriate training methods, and observe and guide the parent's interaction with the child. Older or more tolerant children require less preparation, but may still benefit from a visit to the sleep laboratory and demonstration of the equipment using picture books or training kits. Online videos are available for teaching children and their parents what to expect during a sleep study and CPAP trial. We have found that support groups (both in person or virtual) involving parents and children can be helpful in strengthening the motivation for CPAP adherence, particularly when children demonstrate their application of CPAP for other children.

We utilize the following training techniques for desensitization, guided by principles of behavioral psychology [16,17]:

Positive reinforcement – In which the child is given praise or a small reward for tolerating the application of the mask and/or CPAP machine.

Graduated exposure – In which the child is introduced slowly to the mask, then headgear, then finally the air pressure. When the child is able to tolerate a step without escape behavior, they move on to the next step.

Counter-conditioning – In which the CPAP is applied and used at the same time as an enjoyable and distracting experience, such as a movie, game, or book.

Selective attention – In which the caregiver praises the child for exhibiting cooperative behavior but ignores negative behavior.

Escape prevention – If the child exhibits escape behavior and attempts to pull off the interface, the caregiver or staff redirect the child's hands while distracting the child. The mask is only removed by the caregiver or staff and only removed during calm cooperation.

Each family handles this training differently, thus the training should be individualized. If parents are unable to direct the child's behavior in an age-appropriate way or if they tend to give in to the child's escape behavior, extra sessions will be necessary to assist them in appropriate limit-setting. The process may require the help of a behavioral psychologist or child life expert.

The PAP nap — In some cases, a daytime nap utilizing CPAP can be helpful. Nap studies are not recommended for the diagnosis of OSA [18] and typically are not adequate for a full CPAP titration, due to the absence of daytime rapid eye movement (REM) sleep, during which higher CPAP pressure is sometimes required. However, nap studies are useful for training especially difficult children, or for demonstrating to the parent or caregiver that the child can tolerate CPAP. Nap studies are also used for pressure estimation and for having extra hands nearby for troubleshooting problems. Insurance reimbursement for positive airway pressure (PAP) naps varies by state, but the respiratory therapist's time may be partially reimbursed using education codes for billing.

THE CPAP PRESCRIPTION — CPAP therapy is indicated for treatment of OSA or respiratory insufficiency and is approved by the US Food and Drug Administration for home use in children.

Insurance approval — Approvals for pediatric use of noninvasive and noncontinuous ventilation at home vary by device, based on weight (generally over 10 kg), age (generally over age one year), or tidal volume (often above 50 mL) or pressure (greater than 4 cm/H2O) [19]. These limitations for insurance approval have been reviewed in a position statement by the American Academy of Sleep Medicine (AASM), which suggests that CPAP might be appropriate for younger, smaller children under the guidance of expert clinicians [20]. The AASM website lists common home CPAP devices used in children in the United States [21]. The availability of certain devices may be dictated by a patient's insurer.

In contrast, in other parts of the world CPAP may be available for home use in infants. The use of CPAP in younger infants is considered off-label in the United States, but health care workers are often faced with the need to prescribe it. CPAP masks, headgear, and interfaces are limited in design and size and frequently do not meet the needs for infants, especially those with craniofacial abnormalities. Therefore, sizing and fitting should be performed in person with multiple interface choices available. Equipment companies sometimes provide samples for initial sizing in the clinic.

Choosing the right equipment — The prescription for positive airway pressure therapy includes:

Patient-device interface – Selection of type and fit of mask (eg, nasal mask or face mask)

Comfort measures – Humidification and warmth of air delivered

Machine and mode – Delivery of CPAP, auto-PAP, or BPAP (continuous, automatically adjusting, or bilevel positive airway pressure, respectively)

These considerations are discussed in the following sections.

Patient-device interface — A properly fitting interface between the patient and device optimizes efficacy by ensuring delivery of the target airway pressure while optimizing patient comfort. The main options for home use of CPAP in children are:

Nasal mask – Covers the nose

Nasal pillows – Seal at the nares only, without covering the nose (picture 1)

Face mask (oronasal interface) – Covers the mouth and nose (picture 2)

For children, we generally use nasal interfaces (nasal masks or pillows). These nasal interfaces allow children to speak, cough, and exhale, which provides a pop-off for excessive pressures. Because of their small size, nasal masks also reduce carbon dioxide (CO2) retention, a cause of discomfort. The masks often include a plastic "comfort" flap that improves the seal when inflated against the face by the applied air pressure. Care should be used to avoid masks that can slip and obstruct the nostrils, and, if possible, the interface should be transparent rather than opaque, to allow a view of the skin and nose.

Keeping the mask in a "floating" position over the face, with minimal tension on the straps, decreases risk of skin damage or maxillary compression. Chronic use of CPAP through a nasal interface in a child may alter growth of the facial skeleton, causing hypoplasia of the mid-face and flaring of the upper incisors [22]. Ideally, the type of interface should be switched periodically (eg, from mask to nasal pillows or different masks) to distribute the pressure on the face over time. Monitoring of maxillary growth and dentition by an orthodontist may be helpful.

Caution should be used when prescribing full face masks (oronasal interfaces) for several reasons. First, children with poor mobility may not be able to remove these masks in an emergency such as aspiration. This is a particular concern because CPAP may cause gastric distention and exacerbate gastroesophageal reflux, which may lead to aspiration in children with swallowing dysfunction (eg, those who have had cleft palate repair or certain pharyngeal surgeries). Second, several studies in adults have found that full face masks, in comparison to nasal interfaces, may actually worsen obstructive breathing or decrease effective tidal volumes [2,23,24]. The mechanism for poor performance of the mask in some patients is unclear; it is possible that the mask displaces the tongue into the oropharynx, repositions the chin posteriorly, or causes turbulent airflow by putting positive pressure into the naso- and oropharynx. Despite these limitations, a full face mask interface may be necessary in a few patients, such as those with craniofacial abnormalities and complete nasal obstruction, or when CPAP is used for pulmonary issues in patients without OSA. A full face mask should be tested, when possible, on the patient in a sleep laboratory and shown to be effective before prescribing for home use.

The provider should work closely with the durable medical equipment (DME) company to ensure that the equipment delivered to the home includes the same interface that was found to be successful in the CPAP titration. Unfortunately, the DME company may not provide the same interface, due to cost or availability.

Humidifiers — CPAP causes a sensation of cold air at the nose even when relatively low pressures are used. Many patients prefer warm humidified air when using CPAP, especially in dry climates or with higher CPAP pressures. Other patients prefer the cold pass-over humidifiers, so individual preference should be discussed. Although many machines include a humidifier with adjustable temperature, the prescription should specify the heated option if the patient desires [25]. Heated circuits (tubing) are available for some masks and machines. These are very helpful in high flow/pressure situations in dry climates.

Auto-CPAP — Sleep medicine clinicians frequently use auto-CPAP machines for adult patients. These machines are programmed to adjust CPAP pressure based on detection of apnea, hypopnea, flow limitation, snoring, or free-flowing air. When successful, this avoids the need for CPAP titration in the laboratory. The pressure applied is set within a range determined by the prescriber. Multiple studies and randomized controlled trials have demonstrated that auto-CPAP is generally effective and well tolerated for adult patients with OSA, excluding those with nasal obstruction, hypoventilation, and palatal surgeries [26-28].

In children, we have had variable success with the use of auto-CPAP machines, and few data are available to guide use of this technology in children [29]. This is probably because children tend to move more during sleep compared with adults. Any large position change, swallowing, or coughing can cause the auto-CPAP machine to falsely detect apnea. The machine responds by giving a surge of air, which often fully arouses the child and may prompt CPAP refusal. To avoid this problem when using auto-CPAP in a younger child, we suggest predetermining the likely setting (in clinic, laboratory titration, or home practice) and setting the CPAP to a very small range above and below that chosen pressure. In older children, however, auto-CPAP has been used successfully as an initiation tool [29,30].

Bilevel PAP — Bilevel positive airway pressure (BPAP, also known as bilevel noninvasive ventilation) may be used instead of CPAP for patients with significant hypoventilation (elevated partial pressure of CO2 [PaCO2]) or clinically significant central apneas [31].

BPAP is set so that the inspiratory positive airway pressure (IPAP), which is triggered by time or low pressure, is higher than the exhaled positive airway pressure (EPAP). The pressure difference helps to increase the tidal volume, which treats hypoventilation and sometimes controls central apneas when used with a backup rate.

For children with OSA and without hypoventilation or central apneas, there may not be an advantage of using BPAP over CPAP [32,33]. In fact, if the EPAP is not set high enough for the pharynx to remain open between breaths, the flow or pressure trigger for the inhale breath may not be detected by the machine until the child arouses, thus causing "asynchrony" and defeating the purpose. BPAP machines tend to be more costly than CPAP machines and are therefore less likely to be reimbursed by insurance companies unless there are clear indications for BPAP.

Indications for BPAP in children with OSA include improved tolerability (if demonstrated by adherence), pulmonary issues requiring additional volume, and occasionally a need for higher pressure settings with preserved comfort, as long as the EPAP is still set high enough to overcome the pharyngeal collapse on exhalation. In adults who are nonadherent with CPAP, a switch to BPAP has been shown to improve adherence in a significant number of users [34].

Titration of CPAP in children — Once OSA is diagnosed and the child is properly prepared for CPAP, a titration study in a sleep laboratory allows determination of pressures required to overcome the obstruction and also permits troubleshooting of problems with mask fit, intolerance of airflow, detection of leak, changes required depending on sleep position, and guidance for the parent or caregiver to troubleshoot adherence issues.

In adults and older children, CPAP titrations are sometimes performed during a "split-night" study, where one-half of the night is used to establish the diagnosis and the second one-half of the night is used to perform the pressure titration. In younger children, split-night studies are not optimal. First, they may underestimate the degree of sleep-disordered breathing in some children. This is because non-rapid eye movement (NREM) sleep is more prevalent in the first part of the night (used for the diagnostic part of a split-night study) and NREM sleep tends to be associated with continuous partial obstruction, which is clinically significant but not scored as a discreet apnea (waveform 2) [35]. Including the second one-half of the night in the diagnostic evaluation is important because children are particularly likely to have REM-associated events at that time. Second, a split-night study may be overwhelming for a young child. The stimulation of the sleep laboratory and unfamiliar environment can interfere with sleep, and this is exacerbated by attempting to introduce the CPAP mask abruptly in the middle of the night. Therefore, we prefer to perform a full-night study for CPAP titration in which the child is desensitized to both the CPAP mask and pressure while falling asleep with the device in place.

To perform the titration, CPAP pressures should be started at a low level (generally 4 cm of water), and progressively increased to find the level at which the snoring and airflow limitation resolves. The pressure required for resolution of events is often higher if the child is supine, and may be higher in REM sleep when muscle tone is the lowest. Therefore, the final prescription should reflect the pressure required to treat the worst apnea in supine REM sleep. If CPAP is titrated to a pressure that eliminates complete apneas and hypopneas but snoring, flow limitation, tachypnea, or paradoxical respiratory movements persist, partial obstruction may be present. In this situation, higher CPAP pressure may be attempted to overcome the partial obstruction (waveform 3). A trained sleep technologist also may be able to detect and confirm partial obstructive events by listening to the child with a stethoscope.

After successfully titrating CPAP so that the OSA resolves, "treatment-emergent central sleep apnea" (also called "complex sleep apnea") develops in some patients. This is because the sudden improvement in ventilation achieved by successful CPAP lowers the PaCO2 below the threshold for apnea. In addition, altered stimulation of mechanoreceptors in the pharynx may lower the threshold for central apnea. Treatment-emergent central sleep apnea is similar to that observed in Cheyne-Stokes respiration or periodic breathing, in which a delay in detecting changes in PaCO2 causes an overshoot in respiration and a cyclic pattern of breathing [36].

If treatment-emergent central sleep apnea is frequent, it can disrupt sleep; the mechanism is similar to the sleep disruption seen in individuals sleeping at high altitudes, where fluctuating tachypnea alters blood gasses, leading to central apnea. To minimize the problem, the technologist should try to avoid excessive CPAP pressure during the titration. If necessary, switching to a bilevel mode (BPAP) may be tried in cases where arousals become clinically significant (ie, with potential for frequent nighttime disruptions or daytime sleepiness), but BPAP can also exacerbate treatment-emergent central sleep apnea by provoking the hyperventilation that gives rise to the iatrogenic respiratory abnormality.

Mild central apnea secondary to CPAP treatment generally resolves over time. The time-course for resolution of treatment-emergent central sleep apnea varies among patients. The phenomenon is often observed immediately after initiation of CPAP (eg, during the CPAP titration) and tends to resolve with time as the body adjusts to new CO2 levels during sleep. This is because changes in CO2 and oxygen saturation are immediate, whereas changes in acid-base status, 2,3-bisphosphoglycerate (previously called 2,3-diphosphoglycerate or 2,3-DPG), and buffering proteins may continue for a week or two. In many cases, the problem will resolve by the time of the next CPAP titration. When pressure settings cannot be lowered to reduce central apnea, the addition of oxygen to the machine will often improve the symptoms and often obviates the need for switching to BPAP.

MEDICAL FOLLOW-UP OF CHILDREN USING CPAP THERAPY — Sleep medicine clinicians should work closely with the durable medical equipment (DME) company to ensure accurate and prompt delivery of equipment to the patient's home. Prompt delivery of the device allows the child and family to start therapy while the knowledge from their experience in the sleep laboratory is still fresh, and this may improve adherence to treatment.

Follow-up visits and monitoring — Early and close follow-up is important, to reinforce the importance of CPAP use and to solve problems that can lead to nonadherence [37,38]. Nonadherence often leads to insurance denials, so it is critical to establish consistent use of the CPAP device during the first months of therapy.

Clinics prescribing CPAP should develop protocols on timing for close initial follow-up. In our practice, this includes an initial phone call and clinic visits, until adherence is demonstrated on the machine compliance monitor, then every six to twelve months (or at shorter intervals for younger children, due to the rapid development of the facial structures in toddlers requiring mask changes). Follow-up visits include:

Equipment verification and condition – Shortly after the device is delivered to the home, it is important to verify that the mask and device match with the prescription generated after fitting performed in the clinic or sleep laboratory. We do this by calling the DME company to verify exactly what equipment was delivered. We ask families to bring the CPAP equipment to follow-up appointments, so that we can check on the condition of the equipment and whether it is being applied correctly. This step is important because we occasionally find that the caregiver is applying the mask upside down, too tight, or has covered the exhale ports with tape, despite the extensive training that we provide.

Review of adherence data – Adherence to CPAP is often overestimated by families [32]. An adherence (compliance) monitor in the CPAP machine provides valuable data and should be included in the CPAP prescription. The compliance monitor should be checked by the DME company or clinic at the time of follow-up and the data used for discussion during the visit. If the patient is not using CPAP as prescribed, the data indicates whether the problem is CPAP initiation, maintaining CPAP use through the night, or both. CPAP machines often have data cards that record this information and can be downloaded at a clinic visit. Machines are also available with modems that transmit such data wirelessly for weekly or even daily remote monitoring as needed.

Review of efficacy data – Data downloaded from the CPAP device data card also reports the frequency of apnea, hypopneas, and mask leaks. If these events occur frequently, the sleep medicine clinician should perform a physical examination looking for weight gain or nasal obstruction. The CPAP requirement in growing children may change over time due to changes in anatomy, increasing obesity, or pubertal development (especially in males because testosterone can worsen OSA). Therefore, repeat CPAP titrations are often needed. Growing toddlers need frequent reassessment and adjustment of the CPAP prescription due to rapid anatomical changes. In this age group we typically monitor downloads every few months, and we also repeat CPAP titrations frequently (eg, one to four times a year). In established CPAP users, titrations are often performed annually prior to and during pubertal development, then every few years thereafter, unless compliance downloads or auto-CPAP demonstrates normal breathing parameters. CPAP downloads are considered helpful additional data but the estimates of the apnea index are not always accurate. Published data from adults suggest that obstructive apneas recorded by the machines are more accurate than hypopneas, whereas limited data in children suggest that apneas may be overestimated by at least one algorithm, especially in children with more severe residual sleep apnea [39]. As a result, CPAP titrations are the gold standard for prescription changes.

Troubleshooting – For all patients, and especially for those with suboptimal adherence, the sleep medicine clinician should ask about problems with comfort, nasal congestion, mouth dryness (often indicating mouth leak if a nasal mask is used), eye irritation (air leak from the upper segment of the mask), or child behavior that might interfere with consistent CPAP use. Behavioral resistance can often be addressed with further training and behavioral modification techniques. The child should be examined for signs of nasopharyngeal obstruction and skin breakdown. (See 'Desensitization' above and 'Barriers to effective CPAP therapy in children' below.)

Myofunctional therapy – Many sleep medicine and dental clinics have teamed up with experts in orofacial myofunctional therapy. This form of therapy aims to improve pharyngeal muscle tone and nasal breathing, and it has been shown to help with CPAP adherence. In cases where CPAP is not successful, myofunctional therapy may improve snoring, oxygen saturation, and obstruction over time. When added as an adjunct or in place of CPAP in mild cases, this therapy may improve success in treatment of sleep-related breathing disorders [40].

When to repeat a CPAP titration — With the advent of CPAP monitors and auto-CPAP, small adjustments for comfort or developing apnea can be done remotely.

Situations in which CPAP titration in the laboratory may be helpful include:

Variable apnea levels are demonstrated on repeat downloads of the machine

Mask interface reliability is questioned (eg, a large leak is detected)

When large pressure changes might be needed (eg, when the child has gained or lost significant amounts of weight)

After surgical corrective procedures that might have changed CPAP needs

In response to physical changes in children, such as onset of puberty, aging, or placement of orthodontic equipment

Barriers to effective CPAP therapy in children — Lack of adherence to CPAP therapy in children is a common problem [15,32,41]. This issue is best addressed by close follow-up and troubleshooting, as discussed above. (See 'Follow-up visits and monitoring' above.)

Other common problems include:

Mask fit and air leak – A common complication of CPAP is leak from the interface or circuit. Leak can occur at the mask level due to improper fit or excessive tightening of the mask. This second statement is counterintuitive, but excessive tightening can cause kinks in the comfort flap (mask-to-skin interface), leading to increased leaks. Leak causes insufficient pressure and breakthrough apnea. Most CPAP machines compensate for small leaks by increasing the flow to ensure a set pressure. Higher flows due to large leaks can wake the child. In machines with alarms, an air leak causes false alarms that cause frustration and contribute to nonadherence. Air leak can be lessened by use of a mask that fits on the philtrum of the mouth and bridge of the nose without significant play around the nose; care must be taken to ensure the comfort flap or bottom of the mask is not in danger of obstructing the nares. The headgear is important for discouraging mask slip. Unfortunately, many devices do not come with a choice in headgear size, thus requiring a different choice in interface for some children, or adaptation of the headgear by families if they have sewing skills. (See 'Patient-device interface' above.)

Devices should be inspected at clinic follow-up since circuits and masks can develop holes. DME companies are required to replace circuits, mask, and filters at prescribed intervals, and the family should be aware of this schedule so that they can verify that the DME company is performing the service.

Skin and eyes – Irritation of the eyes or skin breakdown are possible complications. Air leak from the top of the mask can cause eye irritation. Parents and caregivers should be instructed to ensure the mask "floats" above the nose when the machine is turned on but is snug enough above the nose to allow the leak (if any) to exit above the mouth. If the mask interface is tightened too much, the comfort flap at the skin can wrinkle or kink and cause skin breakdown. Skin breakdown is worsened in cases of excess secretions and many hours of CPAP use without checking the skin. To minimize the risk of skin breakdown, the mask should be placed only as tight as gravity, followed by adjustment of the headgear to seal the mask or flap without kinks.

Nasal congestion – High flows, cold air, or mouth leaks can cause rhinorrhea and nasal congestion, which can be minimized by the use of a heated humidifier. If use of the humidifier causes excessive condensation ("rain out") in the circuit (tubing) of the machine, this can be addressed by the use of a warming sleeve on the circuit or internal circuit heating wires. Nasal steroids and nasal irrigation with buffered saline decrease nasal irritation and congestion, as discussed above (see 'Preparing the anatomy for successful airflow' above). If nose bleeds develop, decreasing the steroids and/or directing the steroid spray to the side of the nare instead of the septum can help. Increasing humidity in the room, in addition to the CPAP humidifier and nasal wash, may help in dry climates.

Open-mouth breathing – In many children, open-mouth breathing is due to nasal obstruction or lax facial muscle tone, and is a barrier to effective CPAP. In this case, the mouth-breathing may resolve physiologically if nasal airflow is established by relieving congestion. If mouth breathing continues despite open nasal passages, chin straps may be used to keep the mouth closed. Full face masks also may be used for patients with mouth-breathing, but must be used with caution, as discussed above (see 'Patient-device interface' above). In other children, oral breathing is not a problem; the CPAP is effective and the open mouth is used as a pressure "pop off," and can even be helpful by allowing secretions to exit the mouth. Thus, there is no need to address open-mouth breathing if the CPAP titration shows normal gas exchange and resolution of OSA.

CPAP NONRESPONDERS — Children who continue to demonstrate significant OSA despite CPAP therapy should be evaluated for sites of obstruction other than the tonsils or adenoids. Some children, especially those with Down syndrome, suffer from clinically significant lingual tonsil tissue that encroaches on the epiglottis and narrows the airway leading to continued obstruction on the CPAP downloads [42]. Strategies to evaluate and treat these other sites of airway obstruction are discussed in a separate topic review. (See "Adenotonsillectomy for obstructive sleep apnea in children", section on 'Management of patients with residual OSA after adenotonsillectomy'.)

Intermittent nasal obstruction from infection or allergy may also demonstrate worsening on CPAP therapy. High-arched palates and abnormal dentition may result in the need for maxillary expanders or orthodontic devices [43]. Oral dental appliances available to pediatric dentists have been designed to fit the smallest of children even before permanent teeth have erupted. These devices improve dental malalignments and nasal breathing and might prevent subsequent need for dental braces. Although long-term evidence for apnea treatment is weak, we have successfully used oral appliances in children as young as two years of age, with improvement of maxillary compression, OSA, and nasal breathing. Use of devices for OSA in children and supporting evidence are reviewed separately. (See "Management of obstructive sleep apnea in children", section on 'Orthodontics'.)

Other forms of noninvasive ventilation are being tested in children with some evidence for efficacy. High-flow humidified nasal cannula air has been used in hospitals for years but is now available for home use as a humidified air or oxygen delivery system. The use of these devices has been tested in children with OSA and may prove to be an alternative to CPAP in some children intolerant to CPAP [44,45].

Children with ongoing hypoxemia despite CPAP therapy should be evaluated for pulmonary disease or lower airway obstruction. The differential diagnosis of children who continue to demonstrate excessive daytime sleepiness, hyperactive behavior, or other daytime behavior seen in children with untreated OSA also includes periodic limb movement disorder, narcolepsy, or central nervous system abnormalities. In these cases, referral to a sleep clinician with expertise in pediatric OSA and neurology is warranted.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Sleep-related breathing disorders including obstructive sleep apnea in children".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topics (see "Patient education: Sleep apnea in children (The Basics)")

SUMMARY AND RECOMMENDATIONS

Goals of treatment – Continuous positive airway pressure (CPAP) therapy is one of several options for treatment of obstructive sleep apnea (OSA) in children. The goals of CPAP therapy are to ensure unobstructed breathing, good sleep quality, improved daytime function, and, in some cases, rest from work of breathing. (See 'Introduction' above.)

In children, the most common indication for CPAP is moderate or severe OSA in a child with minimal adenotonsillar tissue or no such tissue after adenotonsillectomy. (See 'Indications' above.)

Anatomy assessment – Nasopharyngeal obstruction, typically manifested by mouth breathing, presents an important obstacle to CPAP treatment, especially in children, and should be addressed if possible before proceeding with CPAP titration. It may be caused by congenital anatomic abnormalities and also by chronic factors that alter nasopharyngeal development, resulting in the characteristic "adenoid face" (figure 1). (See 'Preparing the anatomy for successful airflow' above.)

Partnership and desensitization – CPAP adherence in children is variable. Success depends upon preparation of the child and training and support of the family, often coupled with behavioral management strategies. (See 'First steps in CPAP introduction' above.)

The CPAP prescription – The prescription for positive airway pressure therapy includes the patient-device interface, comfort measures including humidification, and the machine and mode of delivery. (See 'The CPAP prescription' above.)

Interface – For children, we generally use nasal interfaces (nasal masks or pillows). These nasal interfaces allow children to speak or cough when necessary. Caution should be used when prescribing full face masks for children due to risks of aspiration. (See 'Patient-device interface' above.)

Humidification – Although many machines include a humidifier with adjustable temperature, the prescription should specify the heated option if the patient desires it. Many patients prefer warm humidified air when using CPAP, especially in dry climates or with higher CPAP pressures. (See 'Humidifiers' above.)

Device – A variety of home CPAP devices are available, and use of certain devices may be dictated by the patient's insurer. Few data are available for use of auto-CPAP in children, and we have had variable success with these machines. Bilevel positive airway pressure (BPAP) may be tried instead of CPAP for patients with significant hypoventilation or clinically significant central apneas. (See 'Insurance approval' above and 'Auto-CPAP' above and 'Bilevel PAP' above.)

CPAP titration – We suggest using a full-night study for CPAP titration in children rather than a split-night study. During the titration, CPAP pressures should be started at a low pressure (4 cm of water) and progressively increased to find the level at which apneas, hypopneas, snoring, and airflow limitation resolve. The final prescription should reflect the pressure required to treat the worst apnea in supine rapid eye movement (REM) sleep. (See 'Titration of CPAP in children' above.)

Follow up and monitoring – Early and close follow-up is critical to reinforce the importance of CPAP use and to solve problems that can lead to nonadherence. Follow-up visits include verification and inspection of the equipment, review of data on adherence and efficacy downloaded from the CPAP machine and troubleshooting problems with comfort, convenience, or child behavior that may interfere with adherence. (See 'Follow-up visits and monitoring' above.)

Repeat CPAP titrations are often needed because the CPAP requirement in growing children may change over time due to changes in anatomy, increasing obesity, or pubertal development. Alternating interface types (nasal masks and nasal pillows) helps to reduce the chances of maxillary bone compression. (See 'When to repeat a CPAP titration' above and 'Barriers to effective CPAP therapy in children' above.)

CPAP nonresponse – Lack of response to CPAP therapy may be caused by sites of obstruction other than the tonsils or adenoids, intermittent nasal obstruction from infection or allergy, or lower airway disease. (See 'CPAP nonresponders' above.)

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References

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