Pediatric and Adolescent Presentations
Paul R. Carney
Sachin Talathi
Edgard Andrade
James D. Geyer
INTRODUCTION
Pediatric and adolescent sleep-related problems are important to study given that they are frequent sources of parental concern. In addition, inadequate or disrupted sleep can have a negative impact on both the physical and mental health of a child. Insufficient sleep can play a role in a child’s control of behavior, attention, and emotions.
Sleep problems occur in 20% to 30% of children (1,2). There is a wide span of sleep disorders that range from common behavioral problems to specific genetically based sleep disorders. A multitude of factors can have a negative influence on sleep, including stress, pain, eating habits, circadian phase, medical problems, and developmental transitions, to name a few. Although disrupted sleep can be caused by neurologic disorders, pulmonary abnormalities, and psychologic difficulties, they can be frequent during all ages, including infancy, toddlerhood, preschool ages, school ages, and adolescence. However, it is important to note that the nature of specific problems is different among each age group.
Although no sleep disorder is confined to the pediatric age range, some disorders occur primarily during childhood. Other disorders may present at all ages but have distinct presentations in children. The disorders that present in childhood may have significantly different causes and require different modes of treatment. Characteristics of some of the sleep disorders that affect infants, children, and adolescents are summarized in Table 11-1.
ROLE OF DEVELOPMENT
It is crucial to recognize that sleep is an active process that involves a cycling of physiologically diverse stages that are modified during development. These active stages of sleep require categorical shifts in awareness and responsiveness throughout the night. The specific neurologic or physiologic functions served by sleep remain unanswered. However, circumstantial evidence suggests a basic neurodevelopmental process linking sleep and the regulation of attention, arousal, affect, and social behavior (3).
The risk of specific sleep disorders appears to be related to an interaction between the biologic maturation of sleep-wake states and psychological development of infants and young children (4). Examples of this can be seen starting at the first years of life, with predominance of rapid eye movement (REM) sleep, associated with arousals, leading to the manifestation of dyssomnias associated with maintaining sleep. In the preschool to school-age period, non-REM (NREM) arousal parasomnias are more likely to occur at transitions between deep sleep (stages 3 and 4) and REM sleep; these are resolved by adolescence. In adolescence, the challenge of increased physiologic need for sleep and heightened academic and social demands compromise the amount of daily sleep obtained and the regularity of sleep-wake schedules, placing them at risk for circadian rhythm dyssomnias (5).
Symptoms and significance can also present differently, depending on the age of the individual. For example, excessive daytime sleepiness in a pathologically sleepy preteen may be evidenced by their unusually long nocturnal sleep cycle, whereas sleepiness in younger children may be evident more by overactivity, inattentiveness, and whining, than by the appearance of sleep itself (6). Infant dyssomnias may reflect insecure attachment issues at young ages, whereas REM parasomnias (nightmares) may reflect disruptions related to stress and trauma at older ages (4).
COMPREHENSIVE EVALUATION
In order to properly assess and treat children who present with sleep problems, a comprehensive evaluation must be done on both sleep and waking behavior. An initial
assessment of a child should include (a) a detailed sleep history, (b) a general medical history, (c) a complete social history, (d) psychologic/developmental screening, and (e) a physical examination (7). It is important to obtain the age of onset, circumstances, degree of debilitation, persistence/worsening versus amelioration, and family history and practices (4). This information must be compared with age-relevant norms. Temporal descriptions of usual sleep/wake habits, napping, bedtimes, nighttime awakenings, and symptoms of daytime sleepiness or irritability are crucial as well. It is important to assess the duration, frequency, and patterns of symptoms, including timing, changes with weekends and vacations, and changes with stressors and special events. Structured sleep diaries and sleep habit questionnaires may be useful. Specific information about snoring, stopped breathing, and sleep-related behaviors, such as walking, talking, and enuresis, should be acquired. Although sleep laboratory studies in children are required less frequently than in the adult, they are critical for the evaluation of certain complaints including excessive sleepiness, unusual sleep-associated motor behavior, suspected sleep-associated respiratory disorders, and unexplained sleepiness (7).
assessment of a child should include (a) a detailed sleep history, (b) a general medical history, (c) a complete social history, (d) psychologic/developmental screening, and (e) a physical examination (7). It is important to obtain the age of onset, circumstances, degree of debilitation, persistence/worsening versus amelioration, and family history and practices (4). This information must be compared with age-relevant norms. Temporal descriptions of usual sleep/wake habits, napping, bedtimes, nighttime awakenings, and symptoms of daytime sleepiness or irritability are crucial as well. It is important to assess the duration, frequency, and patterns of symptoms, including timing, changes with weekends and vacations, and changes with stressors and special events. Structured sleep diaries and sleep habit questionnaires may be useful. Specific information about snoring, stopped breathing, and sleep-related behaviors, such as walking, talking, and enuresis, should be acquired. Although sleep laboratory studies in children are required less frequently than in the adult, they are critical for the evaluation of certain complaints including excessive sleepiness, unusual sleep-associated motor behavior, suspected sleep-associated respiratory disorders, and unexplained sleepiness (7).
TABLE 11-1 SLEEP DISORDER CHARACTERISTICS | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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New technologies have been developed to augment history taking and nighttime polysomnographic recordings. Instruments particularly well suited for studies of young children who find it uncomfortable to sleep with electrodes in place include home-administered 24-hour ambulatory monitoring, along with alternative home-recording methodologies that do not utilize instrumentation. Body movement detectors in mattresses that are able to track body movements and respiration, and portable, time-lapse, and infrared videosomnography are examples of noninvasive methods (1).
Once assessed, age-dependent responses to treatment must also be taken into consideration. Young children with partial arousals may respond better to schedule adjustment, assurance of sufficient sleep, and parental instruction as opposed to medication. However, adolescents and adults with somnambulism or sleep terrors may respond only to medication and perhaps psychotherapy. The occurrence of enuresis at 4 years of age is difficult to treat and probably should not be. However, an 8-year-old child with a similar problem is relatively easy to treat. In addition, adolescents with enuresis may respond poorly to the usual therapy for younger children. Children are more adaptive than adolescents or adults, and thus it is much easier to treat their sleep-disordered symptoms.
FIRST 3 YEARS OF LIFE
The most frequent sleep-related problem for children between ages 6 months and 3 years is difficulty going to sleep or staying asleep throughout the night (3). Multiple factors have been implicated in the occurrence of repetitive night waking and inability to fall asleep: infant temperament, nutrition, physical discomfort, mild allergy, and parental marital conflict (8,9).
Apnea of Prematurity
Clinical Features
Apnea of prematurity is a disorder of respiratory control, which occurs in 70% to 90% of premature infants who are either <28 weeks of gestation or weigh <1,500 g at birth. The apneas are typically >20 seconds in duration and are associated with bradycardia. A variety of breathing disorders may be present including central apnea, obstructive apnea, mixed apnea, and periodic breathing. These events are more common in active sleep. Apnea of prematurity may predispose the infant to respiratory regulation disorders, cognitive dysfunction, and behavioral disorders.
Causes
The musculature of the pharynx and hypopharynx is somewhat hypotonic in the premature infant. Premature infants also spend a larger amount of time in active sleep, which subsequently enhances the loss of muscle tone in the airway. These factors increase the likelihood of obstructive apnea.
In the premature infant, central ventilatory control has not developed adequately. There is a blunted response to carbon dioxide. Hypoxia does not increase ventilation adequately in the premature infant. In fact, ventilation often decreases in the hypoxic premature infant.
Diagnosis and Treatment
An electroencephalogram should be obtained in these patients to evaluate for possible seizure disorders. Esophageal pH monitoring may be of benefit in patients suspected of gastroesophageal reflux.
Apnea of prematurity typically resolves spontaneously by 48 weeks conceptional age.
Congenital Central Hypoventilation Syndrome
Clinical Features
Congenital central hypoventilation syndrome (CCHS) is characterized by normal respiratory rate and depth during wakefulness that decrease during sleep. There are associated oxygen desaturations and elevations of carbon dioxide during sleep.
Causes
CCHS, an autosomal dominant disorder, is linked to mutations of the Phox2B homeobox gene on chromosome 4p. Haddad syndrome is the combination of CCHS and Hirschsprung’s disease, occurring in approximately 20% of CCHS patients.
Diagnosis and Treatment
There is no definitive treatment for CCHS. Diaphragmatic pacing and home ventilatory management are treatment options. Many patients die during infancy or childhood.
Apparent Life-threatening Event
Clinical Features
An apparent life-threatening event (ALTE) is a frightening episode of apnea, choking, gagging, change in muscle tone, and change in skin color. These events typically occur in the first 6 months of life. They are much more common in premature infants.
Causes
No cause is identified in about half of the cases. Recurrent episodes are often linked to obstructive sleep apnea (OSA), epilepsy, gastroesophageal reflux, cardiac arrhythmias, and CCHS. A wide array of other causes including infections, trauma/abuse, inborn errors of metabolism, and so forth, have been linked to ALTE.
Diagnosis and Treatment
The diagnosis and treatment depends on the clinical history.
Sudden Infant Death Syndrome
Clinical Features
Sudden infant death syndrome (SIDS) is the sudden death of an infant that is unexplained after clinical review, autopsy, and investigation of the death scene. SIDS occurs in approximately 0.8/1,000 live births in the United States.
Causes
The majority of SIDS cases occur prior to 4 months of age. The primary epidemiologic risk factors include prematurity, and sleeping in an overheated environment. Maternal factors include anemia, illicit drug use during pregnancy, and smoking during pregnancy. Sleeping in the prone position appears to be a major contributing risk factor.
Infections and cardiac disorders further enhance the risk of SIDS.
Diagnosis and Treatment
The “Back to Sleep” campaign resulted in a substantial decrease in the incidence of SIDS. In addition to educating parents on the importance of placing the infant on the back to sleep, they should be educated on the importance of avoiding the use of tobacco and drugs during pregnancy and to avoid overheated sleeping environments.
Sleep-onset Association Disorder
Clinical Features
Complaints of sleep problems in the infant and young child usually come from the parents, not the child. Nighttime awakenings sometimes become worrisome to parents. However, most often the problems reflect certain established patterns of interaction between the parent and the child at time of sleep transition. Nighttime arousals are very common in all ages; however, older children and adults are usually unaware of these disruptions.
Causes
A parent may incorrectly conclude that nocturnal awakenings are abnormal, becoming involved in the sleep transition process. The child may become accustomed to parental intervention and become unable to make the transition back to sleep alone, creating a sleep problem or sleep-onset association disorder. The child becomes reliant on the parent to help complete the sleep transition regardless of the time of night.
Diagnosis and Treatment
Diagnosis is usually made with a careful history. Children with this disorder often rapidly respond to simple gradual behavioral interventions, which helps the child learn a new set of sleep-associated habits (10).
Difficulties Learning to Sleep Alone
Clinical Features
Sleeping alone throughout the night without parental intervention is a learned process. All children wake up five to eight times per night, at the end of each sleep cycle, but some children are able to put themselves back to sleep without parental awareness. Most infants are capable of learning this process from about 5 to 7 months of age (3).
Diagnosis and Treatment
The key is to gradually withdraw the amount of parental involvement at sleep onset. The same parental behavioral response is required for middle-of-the-night awakenings. Consistency is also of critical importance if a treatment plan is going to work, especially in conditioning the child to sleep throughout the night. If fear is affecting the progression of this process, it is important to deal with child and/or parental anxiety effectively. Fear can prevent sleep, and fear of safety for one’s child can alter a planned behavioral intervention. In certain cases, it will be important to have parents problem-solve about their child’s fear and how to best accommodate the behavioral treatment plan.
Excessive Nighttime Feedings
Clinical Features
Studies have shown that an increase in nighttime awakenings among infants and toddlers may be related to nighttime feedings. Infants fed large quantities at night (8-32 oz) have been shown to have continued and frequent awakenings, ranging up to eight per night (10,11,12 and 13). Repeated awakenings for ingestion of fluid directly disrupt the functioning of circadian-modulated systems, which may cause further deleterious effects on sleep—wake stabilization (10,14,15).
Diagnosis and Treatment
Diagnosis can be made from a characteristic history: multiple nighttime awakenings, return to sleep only with feeding, significant fluid intake during the night, and
extremely wet diapers. Treatment consists of a gradual decrease in the frequency of feedings during the night (10). Frequent awakenings, three or more per night in a child older than 6 months of age, may cause sleep fragmentation that is harmful to the child. As feedings are decreased and associated habits are eliminated over a couple of weeks, sleep consolidation usually promptly occurs (13).
extremely wet diapers. Treatment consists of a gradual decrease in the frequency of feedings during the night (10). Frequent awakenings, three or more per night in a child older than 6 months of age, may cause sleep fragmentation that is harmful to the child. As feedings are decreased and associated habits are eliminated over a couple of weeks, sleep consolidation usually promptly occurs (13).
Limit Setting
Inability to set limits at bedtime can also lead to sleep deterioration. Typical bedtime struggles may consist of requests for water, stories, use of the bathroom, and adjustment of the lights (10,13). A diagnosis of this sort can be made from history. Through history taking, it may become clear that the parents are unable to enforce nighttime rules with enough consistency to keep the child in bed and quiet so that he or she falls asleep. Parents may have to learn to be firm in their limit setting, enforcing a regular bedtime ritual with an endpoint. The child should also be kept in his or her bedroom with the use of a gate of some sort or closure of the door, if necessary. Positive behavior modification such as a sticker or star chart, as well as other prizes for staying in bed, may elicit a positive response (10).
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