Sleep-related breathing disorders (SRBD) in children are caused by a diverse group of anatomic and physiologic pathologies. These disorders share a common clinical presentation as stertor or sonorous breathing, occasionally accompanied by apneic events of variable duration. Successful management depends on accurate identification of the site of obstruction and the severity of obstruction. Intervention, both surgical and nonsurgical, is tailored to the disorder. In children with SRBD, such intervention may alter behavior and cognition, improve sleep and feeding, or even save a life.
The pathologies responsible for sleep-related breathing disorders (SRBD) in children are more diverse than those in adults. Although most affected adults and older children experience obstruction at the pharyngeal level, often caused by hyperplasia of the tonsils and adenoid and pharyngeal soft tissues, younger individuals may be affected at a variety of sites in the upper respiratory tract. In small children, the distance between these sites may be quite small, resulting in stertor or stridor or both, and the source of the noisy breathing may be difficult to localize.
Generally speaking, SRBD are characterized by episodic obstruction of airflow through the upper airway during sleep. In children, SRBD may result from decreased caliber of the airway caused by nasal obstruction in an obligate nasal breather, compromised skeletal anatomy, excessively compliant or hyperplastic pharyngeal or laryngeal soft tissues, or neuromuscular compromise, complicated by the diminished muscle tone and neurophysiologic changes that typically accompany sleep. Attempts to overcome the obstruction by increasing respiratory effort often exaggerate collapse of the airway, resulting in a paradoxical increase in resistance to airflow. The physiologic sequelae may include hypoxemia, hypercapnia, and acidosis, which in turn signal central and peripheral chemoreceptors and baroreceptors to initiate the arousals and sudden pharyngeal dilation that characterize SRBD.
Successful management of sleep apnea in children depends on accurate identification of the site of obstruction and the severity of obstruction. Only then can appropriate surgical and nonsurgical remedies be considered.
Diagnosis of sleep-related breathing disorders in children
With the exception of acute life-threatening events usually caused by reflux-induced laryngospasm, the presenting symptom in pediatric SRBD is noisy breathing. Stridor, a term used to describe turbulent airflow through the larynx and lower sites in the airway, is rare because lesions in these areas are not often affected by the dynamic changes that occur during sleep. Conversely, stertor, a term used to describe sonorous breathing in the upper airways, is quite common. In most studies, snoring occurs during sleep in 3% to 12% of children , although some studies suggest prevalence as high as 27% . Only those with hypoventilation, apnea, hypoxemia, or repeated arousals, however, are considered to have SRBD.
In its mildest form, SRBD presents as upper airway resistance syndrome. Affected children demonstrate episodic arousals resulting from partial obstruction of the upper airway, associated with symptoms of heroic snoring, mouth breathing, sleep pauses or breathholding, gasping, perspiration, and enuresis. Daytime manifestations of sleep disturbance include morning headache, dry mouth, halitosis, and most significantly behavioral and neurocognitive disorders . Hypersomnolence may occur in older children and adolescents. Other signs and symptoms include audible breathing with open mouth posture, hyponasal speech, and chronic nasal obstruction with or without rhinorrhea. Approximately 40% of children who snore demonstrate more significant degrees of obstruction characteristic of obstructive hypopnea syndrome or obstructive sleep apnea syndrome as defined later . The most severely affected patients may develop cor pulmonale, right ventricular hypertrophy, congestive heart failure, alveolar hypoventilation, pulmonary hypertension, pulmonary edema, or failure to thrive, and are at risk for permanent neurologic damage and even death.
Physical examination of children with SRBD should include assessment of the patient’s weight and body habitus, a complete examination of the head and neck with attention to potential sites of obstruction, and auscultation of the patient’s heart and lungs. Findings of nasal dyspnea or mouth breathing, hyponasal speech, mandibular hypoplasia, drooling, neuromuscular deficit, and tonsillar hyperplasia all suggest some degree of upper airway obstruction. Fiberoptic assessment of the nasal vault, the adenoid pad, and the distal pharynx and larynx may be useful in selected cases. Ancillary studies including chest radiography and electrocardiography should be performed in severely obstructed children. In many cases of nasal obstruction in infants and young children, CT scanning is desirable to define the bony anatomy and to assess the relationship of nasal masses to the sinonasal tract and the central nervous system. At some institutions, cross-table lateral fluoroscopy may be performed during sleep to aid in localizing the site of obstruction.
When a history of severe symptoms of sleep disturbance correlates with obvious physical findings of airway obstruction, additional studies to establish a patient’s candidacy for surgical intervention may be superfluous. Studies suggest, however, that in most cases accurate diagnosis of SRBD cannot be established solely on the basis of a history and physical examination . SRBD occurs primarily during REM sleep when children are less likely to be observed by their parents , and in many cases of upper airway resistance syndrome and obstructive hypopnea syndrome parents may misinterpret the symptoms only as snoring in the absence of obstruction. In addition, although hyperplasia of the tonsils and adenoid likely predispose to airway obstruction, airway dynamics during sleep cannot be determined by static examination in the office setting. Furthermore, fiberoptic assessment of the airway is useful in determining anatomic obstruction but offers a distorted wide-angle view of obstructing tissues and does not demonstrate the dynamics of the nasopharynx during sleep. Similarly, radiographic assessment of the adenoid tissue and tongue base may be difficult to interpret . In such cases, polysomnography remains the gold standard for objective correlation of ventilatory abnormalities with sleep-disordered breathing . This test and its interpretation are described in greater detail elsewhere in this issue.
Unfortunately, the expense and scheduling difficulties associated with polysomnography make this a cumbersome method of assessment in many otolaryngology practices. Other techniques of assessment including audiotaping , videotaping , and home polysomnography have demonstrated favorable results, but require further study. Abbreviated polysomnography (ie, overnight oximetry or nap polysomnography) has demonstrated a high positive predictive value and a low negative predictive value, suggesting that patients with negative results may still require additional studies .
Causes of sleep-related breathing disorders in children
Causes of SRBD in children may be grouped on the basis of age, simplifying the differential diagnosis for a given patient ( Box 1 ). Neonates and infants rarely have significant lymphoid hyperplasia, and SRBD in these children are usually related to their immature respiratory physiology or to congenital obstructing lesions. In premature babies, neural pathways that control ventilation, coordination of the larynx and diaphragm, and chemoreceptor responses are not yet fully developed. In such children, hypoventilation, central apnea, and periodic breathing are common, resulting in reflex bradycardia. Hypoxemia and hypercapnia, which are less common because of the short duration of the apneic events, do not reliably evoke compensatory mechanisms. Apnea in infants may also be associated with gastroesophageal reflux, either as a direct result of soiling of the upper airway or because of vagally mediated reflexes that inhibit inspiration. In such cases, management by medical therapy or Nissen fundoplication may be warranted.
Neonates and infants
Nasal aplasia, stenosis, or atresia
Nasal or nasopharyngeal masses
Craniofacial anomalies
Hypoplastic mandible (Pierre Robin, Nager, or Treacher Collins syndromes)
Hypoplastic maxilla (Apert’s syndrome, Crouzon’s disease)
Macroglossia (Beckwith-Wiedemann syndrome)
Vascular malformations of tongue and pharynx
Congenital cysts of the vallecula and tongue
Neuromuscular disorders
Toddlers and older children
Rhinitis, nasal polyposis, septal deviation
Syndromic narrowing of nasopharynx (Hunter’s, Hurler’s, or Down syndromes; achondroplasia)
Adenotonsillar hyperplasia
Obesity
Macroglossia (Down syndrome)
Vascular malformations of tongue and pharynx
Neuromuscular disorders
Iatrogenic
Nasopharyngeal stenosis
Because babies depend primarily on nasal breathing, obstruction of the nose or nasopharynx has more significance than in older children. Common causes include neonatal rhinitis, pyriform aperture stenosis, choanal atresia, dacryocystoceles, and nasal-choanal stenosis related to craniofacial conditions, such as Apert’s syndrome or Crouzon’s disease. Dermoids, teratomas, gliomas, and encephaloceles of the nose and nasopharynx are seen less frequently. Oropharyngeal obstruction in this age group is usually related to micrognathia or macroglossia. Micrognathia may be syndromic, as in children with Treacher Collins or Nager syndromes, or developmental, as in Pierre Robin syndrome. Relative macroglossia is common in Down syndrome and Beckwith-Wiedemann syndrome. Venous and lymphatic malformations of the pharynx and tongue and congenital cysts of the vallecula and tongue may also cause obstruction in this age group ( Fig. 1 ). Laryngeal abnormalities, such as laryngomalacia, more often result in severe stridor while awake rather than in collapse of soft tissues during sleep. Neuromuscular disorders, which may be complicated by impaired pharyngeal tone, impaired excursion of the diaphragm, or effects of medical therapy, begin to cause obstruction in this age group and often progress as the child ages because of adenotonsillar enlargement.
Toddlers and older children are more affected during sleep by disorders that have had an opportunity to progress. Hyperplasia of the tonsils and adenoid is unquestionably the most common cause of upper airway obstruction in children resulting in sleep-disordered breathing. Severe allergic rhinitis may also develop in children, causing airway obstruction or complicating obstruction caused by other causes. Sinonasal polyposis caused by cystic fibrosis may appear in children in this age group. Similarly, weight gain becomes an issue in older children, and the accumulation of fat in the fascial planes surrounding the pharynx of obese individuals may be a cause of surgical failure following adenotonsillectomy. Some children are affected by syndromes involving progressive reduction of the pharyngeal airway, such as Down syndrome, achondroplasia, and the mucopolysaccharidoses (Hunter’s and Hurler’s syndromes). In the latter group, surgical intervention may actually precipitate deposition of mucopolysaccharide.
In adolescence, lymphoid hyperplasia becomes a less important cause of SRBD as the pharynx increases in size and the tonsils and adenoid recede. As in adults, sleep-disordered breathing is more commonly associated with redundant pharyngeal tissues, obesity, macroglossia, and septal deviation. Progression of neuromuscular disorders may also necessitate surgical intervention in this age group.
Iatrogenic stenosis of the nasopharynx following adenotonsillectomy, uvulopalatopharyngoplasty, or surgery for cleft palate or velopharyngeal insufficiency may result in significant sleep apnea. Corrective surgical intervention for this disorder is often complicated by recurrence.
Causes of sleep-related breathing disorders in children
Causes of SRBD in children may be grouped on the basis of age, simplifying the differential diagnosis for a given patient ( Box 1 ). Neonates and infants rarely have significant lymphoid hyperplasia, and SRBD in these children are usually related to their immature respiratory physiology or to congenital obstructing lesions. In premature babies, neural pathways that control ventilation, coordination of the larynx and diaphragm, and chemoreceptor responses are not yet fully developed. In such children, hypoventilation, central apnea, and periodic breathing are common, resulting in reflex bradycardia. Hypoxemia and hypercapnia, which are less common because of the short duration of the apneic events, do not reliably evoke compensatory mechanisms. Apnea in infants may also be associated with gastroesophageal reflux, either as a direct result of soiling of the upper airway or because of vagally mediated reflexes that inhibit inspiration. In such cases, management by medical therapy or Nissen fundoplication may be warranted.
Neonates and infants
Nasal aplasia, stenosis, or atresia
Nasal or nasopharyngeal masses
Craniofacial anomalies
Hypoplastic mandible (Pierre Robin, Nager, or Treacher Collins syndromes)
Hypoplastic maxilla (Apert’s syndrome, Crouzon’s disease)
Macroglossia (Beckwith-Wiedemann syndrome)
Vascular malformations of tongue and pharynx
Congenital cysts of the vallecula and tongue
Neuromuscular disorders
Toddlers and older children
Rhinitis, nasal polyposis, septal deviation
Syndromic narrowing of nasopharynx (Hunter’s, Hurler’s, or Down syndromes; achondroplasia)
Adenotonsillar hyperplasia
Obesity
Macroglossia (Down syndrome)
Vascular malformations of tongue and pharynx
Neuromuscular disorders
Iatrogenic
Nasopharyngeal stenosis
Because babies depend primarily on nasal breathing, obstruction of the nose or nasopharynx has more significance than in older children. Common causes include neonatal rhinitis, pyriform aperture stenosis, choanal atresia, dacryocystoceles, and nasal-choanal stenosis related to craniofacial conditions, such as Apert’s syndrome or Crouzon’s disease. Dermoids, teratomas, gliomas, and encephaloceles of the nose and nasopharynx are seen less frequently. Oropharyngeal obstruction in this age group is usually related to micrognathia or macroglossia. Micrognathia may be syndromic, as in children with Treacher Collins or Nager syndromes, or developmental, as in Pierre Robin syndrome. Relative macroglossia is common in Down syndrome and Beckwith-Wiedemann syndrome. Venous and lymphatic malformations of the pharynx and tongue and congenital cysts of the vallecula and tongue may also cause obstruction in this age group ( Fig. 1 ). Laryngeal abnormalities, such as laryngomalacia, more often result in severe stridor while awake rather than in collapse of soft tissues during sleep. Neuromuscular disorders, which may be complicated by impaired pharyngeal tone, impaired excursion of the diaphragm, or effects of medical therapy, begin to cause obstruction in this age group and often progress as the child ages because of adenotonsillar enlargement.
Toddlers and older children are more affected during sleep by disorders that have had an opportunity to progress. Hyperplasia of the tonsils and adenoid is unquestionably the most common cause of upper airway obstruction in children resulting in sleep-disordered breathing. Severe allergic rhinitis may also develop in children, causing airway obstruction or complicating obstruction caused by other causes. Sinonasal polyposis caused by cystic fibrosis may appear in children in this age group. Similarly, weight gain becomes an issue in older children, and the accumulation of fat in the fascial planes surrounding the pharynx of obese individuals may be a cause of surgical failure following adenotonsillectomy. Some children are affected by syndromes involving progressive reduction of the pharyngeal airway, such as Down syndrome, achondroplasia, and the mucopolysaccharidoses (Hunter’s and Hurler’s syndromes). In the latter group, surgical intervention may actually precipitate deposition of mucopolysaccharide.
In adolescence, lymphoid hyperplasia becomes a less important cause of SRBD as the pharynx increases in size and the tonsils and adenoid recede. As in adults, sleep-disordered breathing is more commonly associated with redundant pharyngeal tissues, obesity, macroglossia, and septal deviation. Progression of neuromuscular disorders may also necessitate surgical intervention in this age group.
Iatrogenic stenosis of the nasopharynx following adenotonsillectomy, uvulopalatopharyngoplasty, or surgery for cleft palate or velopharyngeal insufficiency may result in significant sleep apnea. Corrective surgical intervention for this disorder is often complicated by recurrence.
Management of sleep-related breathing disorders in children
Nonsurgical management
Treatment of SRBD in children is tailored to the etiology of the airway obstruction. Medical management, such as thioxanthines and methylphenidate, may be useful in cases of central apnea. Pharmacotherapy may also be considered in less severe cases of obstructive apnea, or when surgical intervention does not address the pathology. Examples of such disorders include neonatal rhinitis, allergic rhinitis, and acute tonsillitis. In cases of chronic upper airway obstruction, mechanical correction by prostheses, positive airway pressure, or weight loss may be worth consideration. In most patients, however, such as those with obesity or neuromuscular disorders in which airway dynamics are affected, surgical management is generally considered before use of positive airway pressure or oral prostheses, because these interventions are rarely tolerated in children and are often ineffective. Such methods of management should be entertained, however, to address residual obstruction following surgery. Rarely, in the most severe or refractory cases, tracheotomy must be considered.
Surgical management
Preoperative planning is an essential component of the surgical management of patients with SRBD. Postoperative respiratory distress is common after surgery for SRBD because of effects of anesthesia, bleeding, edema, and residual airway compromise. Patients at greatest risk include those with severe obstructive sleep apnea syndrome; diminished neuromuscular tone (ie, cerebral palsy); morbid obesity; skeletal and craniofacial abnormalities, such as hypoplasia of the midface or mandible or nasopharyngeal vault; and very young children (younger than age 2–3 years) . As a result, high-risk individuals who are undergoing even routine procedures, such as adenotonsillectomy, should be admitted to a high-visibility bed in the hospital with continuous cardiac and oxygen saturation monitoring. Intraoperative use of steroids and postoperative placement of nasopharyngeal airways may reduce the risk of airway compromise after surgery. Narcotics and sedatives should be used sparingly in severely obstructed children. Obese patients and those with reduced neuromuscular tone may benefit from airway support with positive airway pressure. In the most extreme cases, overnight endotracheal intubation may be desirable.
Nasal and nasopharyngeal obstruction
SRBD caused by nasal and nasopharyngeal masses is best addressed by removal of the mass. Depending on the pathology, the procedure may be as simple as a transoral, retropalatal approach for adenoidectomy or marsupialization of nasolacrimal duct cysts, or as complex as an anterior craniofacial approach for encephalocele. Nasal and nasopharyngeal neoplasms may require aggressive resection, and preoperative embolization (juvenile nasopharyngeal angiofibroma) or postoperative radiation therapy or chemotherapy (malignancies).
Bilateral choanal atresia and stenosis of the pyriform aperture are causes of obstructive apnea in neonates and require early intervention ( Fig. 2 ). Such infants can be temporized with oropharyngeal airways, but should not leave an intensive care setting until a secure airway is established by tracheotomy or repair of the bony defect. Timing of surgery is controversial; early repair with avoidance of tracheotomy is always desirable; however, children several weeks to several months old better tolerate bleeding and better accommodate instruments used in the nasal cavity.
Although choanal atresia may be approached by either the transpalatal or the transnasal route, improvements in endoscopic and powered instrumentation have made the transnasal approach the first choice for most otolaryngologists . In small children, the procedure is best performed using a small rigid rod-lens telescope and a drill with a protected shaft. Microdebriders designed for intranasal surgery ( Fig. 3 ) are well-suited for this purpose. A 120-degree telescope placed in the mouth with the palate retracted affords the surgeon a view of the nasopharynx so that a urethral sound may be safely passed through the atretic plate. After creation of mucosal flaps with a sickle knife or ablation of the mucosa with the aid of a fiber-delivered laser, the microdebrider can be fitted with a small round bur to initiate bone removal, and subsequently with choanal atresia or Silver Bullet (Medtronic/Xomed, Jacksonville, Florida) burs to expand the opening ( Fig. 4 ). Standard suction blades (2.9 or 3.5 mm) used for sinus surgery facilitate the removal of soft tissue and thin bone. Back-biting forceps are used to remove the posterior portion of the vomer. The opened choanae may be treated with mitomycin C to reduce the risk of restenosis , and stenting for several weeks using endotracheal tubes or Albouker-type stent may be indicated in some cases. In cases of pyriform aperture stenosis, the offending bone may be approached through a sublabial approach and reduced using similar instrumentation .
Nasopharyngeal stenosis, once a common complication of syphilis, may result as a complication of adenotonsillectomy, uvulopalatopharyngoplasty, or surgery for cleft palate or velopharyngeal insufficiency. This disorder often causes obstruction of the upper airway that is even more significant than the disorder the original surgery was intended to correct. Typically, cicatrix forms circumferentially in the nasopharynx as a result of removal of excessive removal of mucosa from opposing surfaces. Simple release of the scarred area results in recurrence, and treatment must include the movement of fresh, well-vascularized tissue to cover the denuded bed. A variety of techniques has been recommended, including Z-plasty , laterally based pharyngeal flaps ( Fig. 5 ) , other advancement and rotation flaps , and radial forearm and jejunal free flaps . Many authors advocate the use of intralesional steroids and topical application of mitomycin C to the surgical site to reduce the risk of recurrence. Postoperative stenting with nasopharyngeal airways or oropharyngeal prostheses is mandatory, although the necessary duration of such stenting is controversial.
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