Obstructive Sleep Apnea Diagnosis and Treatment - part 7 pot

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Obstructive Sleep Apnea Diagnosis and Treatment - part 7 pot

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268 Pelayo and Li A study from Israel found that children with SDB had lower scores on neuro- cognitive testing compared to controls but the scores improve after treatment (23). This prospective study of 39 children aged five to nine years underwent a battery of neurocognitive tests containing process-oriented intelligence scales. Children with SDB had lower scores compared with healthy children in some Kaufman Assessment Battery for Children (K-ABC) subtests and in the general scale Mental Processing Composite, indicating impaired neurocognitive function. Six to 10 months after adenotonsillectomy, the children with OSAs demonstrated significant improvement in sleep characteristics, as well as in daytime behavior. Their neurocognitive perfor- mance improved considerably, reaching the level of the control group in the sub- tests Gestalt Closure, Triangles, Word Order, and the Matrix analogies, as well as in the K-ABC general scales, Sequential and Simultaneous Processing scales, and the Mental Processing Composite scale. The authors concluded neurocognitive func- tion is impaired in otherwise healthy children with SDB. Most functions improve to the level of the control group, indicating that the impaired neurocognitive functions are mostly reversible, at least 3 to 10 months following adenotonsillectomy (23). An abrupt and persistent deterioration in grades must also raise the question of abnormal sleep and SDB (20,21,50,51). In schools the tiredness and sleepiness may be labeled as “inattentiveness in class,” “daydreaming,” or “not being there” (22,52). Concerns about school perfor- mance were raised in the original description of OSA syndrome in children (3). More recently, the possible association between SDB, learning problems, and atten- tion-deficit disorder has been studied (8,18,19,21,22,52–56). A study by Gozal et al. examined the hypothesis that domains of neurobehavioral function would be selec- tively affected by SDB. They study children with reported symptoms of attention- deficit/hyperactivity disorder (ADHD) and also determined the incidence of snoring and other sleep problems in 5- to 7-year-old children in a public school system. Children with reported symptoms of ADHD and control children were ran- domly selected for an overnight polysomnographic assessment and a battery of neurocognitive tests. Frequent and loud snoring was reported for 673 children (11.7%). Similarly, 418 (7.3%) children were reported to have hyperactivity/ADHD. Children with reported symptoms of ADHD and control children were randomly selected for an overnight polysomnographic assessment and a battery of neurocog- nitive tests. Eighty-three children with parentally reported symptoms of ADHD had sleep studies together with 34 control children. After assessment with the ADHD subscale of the Conners Parent Rating Scale, 44 children were designated as having “significant” symptoms of ADHD, 27 as “mild,” and 39 designated as “none” (con- trols). Overnight polysomnography indicated that OSA was present in 5% of those with significant ADHD symptoms, 26% of those with mild symptoms, and 5% of those with no symptoms. The authors concluded an unusually high prevalence of snoring was identified among a group of children designated as showing mild symptoms of ADHD based on the Conners ADHD subscale. SDB can lead to mild ADHD-like behaviors that can be readily misperceived and potentially delay the diagnosis and appropriate treatment (22). Additional clinical signs of SDB include increased respiratory efforts with nasal flaring, suprasternal or intercostal retractions, abnormal paradoxical inward motion of the chest occurring during inspiration, and sweating during sleep. The sweating may be limited to only the nuchal region particularly in infants; it may be severe enough to necessitate changing clothes during the night. The parents may mention the child feeling warm at night or preferring to sleep without a blanket. Obstructive Sleep Apnea in Children 269 Parents may also observe the child stop breathing, then gasping for breath. It is sur- prising to note how often parents have observed abnormal breathing patterns during sleep but were never questioned about it by pediatricians during regular visits. Information regarding the sleep position is helpful. Typically, the neck is hyper-extended and the mouth is open. Another typical sleeping position is prone with the knee tucked under the chest with head turned to the side and hyperextended. Rarely, the child with SDB prefers to sleep propped up on several pillows (4). Parasomnias may be triggered or exacerbated by SDB. Ohayon (57) has found that individuals identified with SDB have a much higher incidence of nightmares, with reports of “drowning,” “being buried alive,” and “choking.” SDB leads to sleep fragmentation or disruption. Any condition that disrupts slow-wave sleep may lead to sleep terrors and sleepwalking in children (58). SDB should be included in the evaluation of any child with parasomnias. A physical finding that may be overlooked in a child with SDB is a narrow and high-arched palate (4). Interestingly, the description of attention-deficit disorder in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) mentions that minor physical anomalies such as high-arched palates may be present (59). Since both conditions may have similar daytime behavior in the same age group, a child with SDB could be misidentified as having attention-deficit disorder. The possibility of a sleep disorder being present should be considered in any child being evaluated for attention-deficit disorder. This is particularly important since treatment of SDB may improve behavior and academic performance (60,61). Diagnostic Criteria The diagnostic criteria used for adults with OSA cannot be used reliably in children (5,49,62,63). The diagnosis of SDB is based on the history, physical findings, and supportive data. Laboratory testing should be, ideally, tailored to the clinical question. For example, if there are concerns about excessive daytime sleepiness, a multiple sleep latency test (MSLT) may be indicated (64). The MSLT is ideally performed in subjects who are at least eight years old. The polysomnogram in a child uses the same technology and the same type of information as recorded in adults. Airflow, respiratory effort, and pulse oximetry comprise the breathing measurements usually monitored. Breathing can be mea- sured with different techniques, ranging from qualitative techniques such as nasal thermocouples which use the temperature difference between inhaled and exhaled air to record individual breaths, to quantitative and invasive measures such as esophageal pressure measurements. The latter technique is less tolerable than others but is particularly helpful to distinguish central from obstructive apneas. End-tidal CO 2 monitoring is another technique that can help detect transient episodes of hypoventilation. Currently, the technique that balances need for quantification with tolerability is measuring airflow using a nasal pressure cannula (65,66). This tech- nique allows for the identification of more subtle breathing episodes but can be harder to interpret than earlier techniques, in particular when a child is mouth breathing. This nasal pressure cannula has facilitated the measurement of an additional abnormal respiratory event, RERA, which is an acronym for respiratory event- related arousal. The term respiratory disturbance index (RDI) may now include the total number of apneas, hypopneas, and RERAs divided by the total sleep time. The RDI should be distinguished from the AHI. However, some sleep study reports may equate the RDI with the AHI if the sleep study did not measure RERAs. 270 Pelayo and Li The clinician needs to be aware that these terms may be used interchangeably, potentially causing confusion. The multitude of available techniques to measure breathing makes it difficult to compare the results from different studies. Along with the absence of controlled studies, another problem with understanding pediatric SDB is that definitions for key terms vary. OSAs are defined as lasting at least 10 seconds in adults. However, since children have faster respiratory rates clinically significant apneas can occur in less time (Fig. 1). Apneas as brief as three or four seconds may have oxygen desatu- rations. There is no universally accepted definition of hypopneas in children. The clinician needs to know how apneas and hypopneas are defined and scored when interpreting a polysomnogram report. The most recent edition of the International Classification of Sleep Disorders (ICSD-2) defines OSA in children as having an AHI ≥ 1 (67). In adults a higher AHI of five is required. Unfortunately it is not uncommon for an adult cutoff value to be used in children (68). There is also contro- versy as to when the adult cutoff value should be applied; the onset of puberty or the age of 18 years is often debated. FIGURE 1 Polysomnogram of a 10-year-old girl depicting several obstructive apneas and hypop- neas during a 60-second epoch of rapid eye movement (REM) sleep, accompanied by esophageal pressure “crescendos,” intermittent snoring (as detected by the Chin EMG and Mic), and oxygen desaturations. Note the rapid respiration rate consistent with that of a child. Abbreviations: C3-A2, C4-A1, O1-A2, Fp1-A2, electroencephalogram electrodes placed centrally (C3, C4), occipitally (O1), and fronto-parietally (Fp1), and referenced to the right (A2) or left (A1) ear; Chin EMG, elec- tromyogram recorded from chin muscles; LOC, left eye electro-oculogram; ROC, right eye electro- oculogram; EKG, electrocardiogram; LAT and RAT, electromyogram recorded from the left and right anterior tibialis muscles, respectively; SaO 2 , pulse oximetry; Mic, microphone to detect snoring; Nasal, nasal pressure measured by pressure transducer; Oral, oral airflow measured by thermistor; Chest and Abdomen, impedance bands to measure thoracic and abdominal movement, respectively; P es , esophageal pressure to measure transmitted intrathoracic pressure. Source: Courtesy of Clete A. Kushida, MD, PhD. Obstructive Sleep Apnea in Children 271 Controversy exists over whether a diagnosis of OSA, or the larger spectrum of SDB, should be routinely made without a formal polysomnogram. While some have suggested that this diagnosis can be made in patients using either the history and physical, or the history, physical, and an audio- or videotape, others have found an inability of clinical history alone to distinguish primary snoring from OSA in children (69). The situation is further complicated by the description of UARS in children, which may have been missed in the studies cited above. Therefore, a sleep study is the most definitive test for SDB (70,71). Currently, some otolaryngologists who treat SDB in children may make the surgical recommendation based on clinical findings of airway obstruction, sometimes reviewing an audio- or videotape (72,73). The clinicians must be aware of the potential pitfalls to this practice. Certainly there are individual cases in which a diagnostic sleep studies are not available, but ideally they should be the exception. The challenge we face in sleep medicine is providing easily-accessible and cost-effective care working within a multidisciplinary model. We do not know, for certain, how accurate clinical diagnosis is without objective testing. Until we have a better answer, the diagnostic gold standard should not be disregarded particularly in a tertiary care setting. The American Thoracic Society, American Academy of Sleep Medicine, and AAP all support the use of sleep studies (70,74,75). SDB is not the only sleep disorder a child may have. Clinical impression may have both false negatives and positives resulting in possible misdiagnosis or unnecessary surgery. For example, without confirmatory testing, a child with symptomatic periodic limb movements might be misdiagnosed with SDB and may have unnecessary surgery. Periodic limb movements of sleep and restless legs syndrome may not be rare in children (76). These syndromes can have a vague or difficult history to elicit. Sudden Infant Death Syndrome Sudden infant death syndrome (SIDS) remains one of the most common causes of death among infants throughout the world. In the United States there has been a major decrease in the incidence of SIDS since the AAP released its recommendation in 1992 that infants be placed down for sleep in a nonprone position. A public health initiative was developed using the slogan “back to sleep.” The recommendations also included the need to avoid redundant soft bedding and soft objects in the infant’s sleeping environment. The AAP further refined its position in 2000 and no longer recognized side sleeping as a reasonable alternative to fully supine sleeping. In 2005, the AAP again provided further recommendations to decrease the incidence of SIDS. These included recommending that adults do not share a bed with infants. Instead adults should share the bedroom but sleep on a different surface. The AAP also recommended using pacifiers in the beginning of the night but replacing them in the children’s mouths if they fell out during the night (77). Concerns have been raised that these newer guidelines may have the unintended consequences of disrupting the sleep of families by the infants creating an association with the need for the pacifiers in order to return to sleep during the night. Other experts have also expressed con- cerns that discouraging bed sharing may decrease nursing and bonding (78–81). Sleep-Disordered Breathing in Special Populations SDB may occur more often in special populations (82–86). Any condition or syn- drome associated with craniofacial anomalies may be associated with SDB. Pierre Robin (Fig. 2), Apert’s and Crouzon’s are among these syndromes. Approximately 272 Pelayo and Li half of all children with Down syndrome have SDB. However, symptoms of day- time sleepiness and sleep disruptions at night may be due to non-neurological factors such as maxillofacial abnormalities, large tonsils or adenoids, micrognathia, large tongues, or other abnormalities. Sleep disorders often occur in patients with neuromuscular disease because of associated weakness in respiratory muscles, which is further exacerbated by hypotonia during sleep. In disorders such as Duchenne’s muscular dystrophy, daytime pulmonary function studies do not predict the degree of apneic events during sleep. Rather, these patients can have nocturnal oxygen desaturation, significant sleep fragmentation, recurrent hypoven- tilation, and reduced REM sleep. These patients are also at increased risk for aspiration during sleep. Diagnosis and treatment of SDB in these patients can be an important part of comprehensive management. Treatment Not only are the diagnostic criteria different in children than adults but also the treatment options. SDB in adults has four treatments options which may be combined. The most common treatment is continuous positive airway pressure (CPAP) to help splint open the upper airway (see also Chapter 6). When CPAP is used correctly snoring should be absent during sleep. There are several sophisti- cated surgical options with a wide range of success (see also Chapter 11). In adults, oral appliances, which help reposition the mandible, have improved breathing during sleep in selected patients (see also Chapter 12). As a conservative measure, adults with SDB are advised to sleep off their backs, lose weight, and avoid alcohol before sleeping (see also Chapter 13). Unlike adults, in children surgery is the most common treatment for SDB. Adenotonsillectomy is the most common initial treatment for SDB in children (Fig. 3). This procedure can be extremely effective and result in dramatic improvements (and very grateful parents). When surgery is being entertained, as a general rule, the adenoids and tonsils should both be removed during the same surgery. It is tempting in very small children to only remove the adenoids if the tonsils do not appear overly enlarged since this allows for less postoperative pain and lower risk of adverse events such as bleeding. This practice should be discouraged since even though the tonsils do not seem enlarged the surgeon must keep in mind that they are examining a child that is awake and sitting. The relative posterior airway space FIGURE 2 (See color insert.) Infant with Pierre Robin syndrome; micrognathia, spe- cifically mandibular hypoplasia, as depicted is characteristic of this disorder. Obstructive Sleep Apnea in Children 273 may be obstructed when the child is supine, the tongue falling back and the airway narrowing during REM sleep hypotonia. Also in a growing child the tonsils may also grow larger. If only the adenoids are removed there is the risk of having to later return for further surgery to remove the tonsils. Clinicians should be aware that there are several different techniques used to remove tonsils and this may play a role in the efficacy of treatment. The anesthesiologist should be familiar with OSA since postoperative pulmo- nary complications can occur (87). Children with OSA are often thinner than expected. This is may be due to multiple factors including the greater caloric demand of breathing through a narrow airway and possible disruption of growth hormone secretion. Children after OSA surgery may unexpectedly increase their weight (88). Surgery does not always completely cure the child’s SDB. The true cure rate of this surgery for SDB is unknown (23,28,89,90). Most studies that have performed postsurgical sleep studies have used older adult definitions of sleep apnea in the children. Suen et al. designed a prospective study of 69 children aged 1 to 14 years who were referred to an otolaryngologist. Of the 69 children 35 (51%) had a RDI > 5 on polysomnography. Thirty children with a RDI > 5 underwent adenotonsillec- tomy. Of the 30 children 26 had follow-up polysomnography following surgery. All 26 children had a lower RDI after surgery, although four patients still had a RDI > 5. Using a RDI cut off of 5, the cure rate of surgery would be 85%. However, three chil- dren snored with postoperative RDI < 5. If those subjects were considered to have residual SDB then the cure rate of surgery would only be 73%. All patients improved with adenotonsillectomy but the true cure rate is not clear. The possibility of residual SDB should always be considered after surgery if the child is symptomatic. Suen et al. concluded history and physical findings were not useful in predicting outcome (91). Different surgical techniques may improve the success of surgery in these children (92). Some may argue that patients with clear-cut cases of SDB may skip the post- operative sleep study. However, the adult experience teaches us that it is precisely these obviously more severe or “clear-cut” cases that will have residual disease. Adenotonsillectomy will not change the relationship of tongue size and shape to the palate. The parents may report that the child is “100% better” yet still has residual obstruction. If the child still has trouble paying attention in school, a sleep problem may be overlooked and no longer be considered a possibility. The child may end up labeled as having attention-deficit disorder because there was no postoperative sleep test done (36,93). CPAP therapy should be considered if surgery is not a viable option for the child (94–96) (Fig. 4). CPAP uses a small air compressor attached to a mask via a FIGURE 3 (See color insert.) (A) Schematic diagram illustrating oral cavity before (left) and after (right) tonsillectomy. (B) Patient’s oral cavity depicting hypertrophied tonsils. (C) Same patient’s oral cavity following tonsillectomy. 274 Pelayo and Li hose. The mask usually only covers the nose but masks are available that cover the nose and mouth. By forcing positive air pressure in the airway, the negative pressure of inspiration can be countered to avoid airway narrowing or collapse. CPAP is effective but can be cumbersome to use. Over time the CPAP devices have become smaller and quieter. The masks have also improved with many more styles and sizes available. In the recent past in the United States there was no CPAP mask certified for home use in children. Clinicians needed to obtain the mask from out- side of the country or used the smallest available adult mask. This has now changed. CPAP has been approved for home use in children in the United States. A wider range of mask sizes and styles should now become available. Despite these advances CPAP remains a second choice over surgery in most children (70). This is due to the advantage of having a surgical option. The main drawbacks of using CPAP are related to getting a proper-fitting CPAP mask. If the mask is not fitted correctly the air pressure may leak out causing discomfort and sleep disruption. If the mask is too tight it can cause facial abrasions or bruising. In small children the possibility of the CPAP mask interfering with growth of the maxilla should be considered. As the child grows CPAP may require adjustments both in terms of mask size and the amount of pressure delivered to the airway. In addition to a continuous pressure delivery mode, a bilevel mode [bilevel positive airway pressure, (BPAP)] is available. In this mode, the pressure on expiration is lowered from the inspiratory pressure (see also Chapter 7). This may allow the device to be more comfortable and may be preferred in patients with neuromuscu- lar weakness. The most recent advance in positive airway pressure has been the development of machines, which can adjust the pressure required to keep the airway open on a breath-by-breath basis. These so-called “smart CPAP” or auto-positive airway pressure units (see also Chapter 8) are promising but are not part of the mainstream treatment of children at this time (94). The treatment of residual or persistent OSA after surgery is a difficult clinical situation. CPAP has been the recommended option yet CPAP can be cumbersome. FIGURE 4 (See color insert.) (A) Child awake and (B) asleep while wearing a continuous positive airway pressure mask during polysomnographic monitoring in a sleep laboratory. Note wires con- nected to recording electrodes that are placed on the face and on the scalp, which are hidden beneath the head wraps used to prevent dislodgement of electrodes. Obstructive Sleep Apnea in Children 275 If a child has clinically significant SDB after adenotonsillectomy and CPAP is not an option or not tolerated the clinician had been forced to consider more aggres- sive surgery such as a tracheostomy or palliative use of supplemental oxygen. A search for better alternatives is underway. The application of more sophisticated surgical techniques with the possible use of orthodontic treatments is being pur- sued (97–100). In adults with persistent sleep apnea after a uvulopalatoplasty the remaining obstruction is often at the level of the base of the tongue. This may be due to a combination of retrognathia and a narrow hard palate. The most effective surgical correction at this level of obstruction is bilateral maxillo-mandibular advancement. This surgery is not advised for the growing bones of young children. FIGURE 5 (See color insert.) Maxillary osteogenic distraction device placed below the palate of a child’s mouth. Source: Photograph courtesy of Kannan Ramar, MD. FIGURE 6 (See color insert.) Profile of child’s face (A) before and (B) after mandibular distraction osteogenesis. 276 Pelayo and Li The base of tongue obstruction can be minimized in some children with rapid maxillary expansion (99,101). By widening the child’s palate the tongue can fit into its natural position on the hard palate and be less likely to slide back into the hypopharynx (Fig. 5). This procedure is most effective when there is a significant narrow and high-arched palate. Such osteogenic distraction techniques are very promising. These techniques were traditionally reserved in children with cranio- facial anomalies to lengthen bones. These techniques are starting to be adapted for persistent SDB to bring the mandible forward and increase the posterior airway space in the pharynx (Fig. 6). CONCLUSIONS There are important similarities and differences between SRBD in adults and children. 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