Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Pediatric Obstructive Sleep Apnea
"The stupid-lazy child who frequently suffers from headaches at school, breaths through his mouth instead of his nose, snores and is restless at night, and wakes up with a dry mouth in the morning, is well worthy of the solicitous attention of the school medical officer." -- W. Hill, 1889 While the clinical manifestations of obstructive sleep apnea have been recognized for many years, as evidenced from this quote by Dr. Hill in 1889 in the British Medical Journal, and despite numerous historic references to its occurrence, the first report of obstructive sleep apnea the medical literature was in 1966, and this report discussed only cases in adults. The first reports of pediatric obstructive sleep apnea were not until 1976 by Guilleminault, et al at the Stanford University Sleep Disorders Clinic. Pediatric obstructive sleep apnea is commonly encountered by otolaryngologists. It is thought to be different than adult obstructive sleep apnea, and many issues surrounding the diagnosis and management of this potentially lethal disorder are controversial. Obstructive sleep apnea is a disorder of breathing during sleep, characterized by prolonged partial upper airway obstruction and/or intermittent complete obstructions with disruption of normal ventilation during sleep and normal sleep patterns. In the pediatric age group it occurs mostly in toddlers and older children, and is caused by physical obstruction between the nares and the bronchi. Central sleep apnea is caused by central neurologic mechanisms with failure to initiate respiratory effort. Central apnea usually occurs in premature infants and neonates, is associated with an immature nervous system, and may be associated with other life threatening conditions such as infection, hypothermia, hypoxia, and intracranial hemorrhage. Mixed apnea occurs in neonates and young children and most commonly are characterized by central apneic events followed by obstructive events. This summary concentrates on obstructive sleep apnea, since it is most commonly encounter as otolaryngologists.
Recognizing and treating pediatric obstructive sleep apnea is important as symptoms may span the spectrum from no evidence of disease to serious complications including failure to thrive, neurobehavioral changes, cor pulmonale, right heart failure and even death.
It is also important to recognize that many differences exist between adult and pediatric sleep apnea. Some of the differences pointed out by Drs. Carroll and Loughlin at Johns Hopkins include:
The epidemiology of pediatric obstructive sleep apnea is poorly understood due to the lack of extensive epidemiologic studies, and the lack of consensus in the literature regarding criteria used to diagnose obstructive sleep apnea in children. One factor complicating diagnosis of obstructive sleep apnea is the prevalence of snoring. It is estimated that between seven and nine percent of all children snore. In a prospective study of 325 children who presented for their 4 year check-up by Hultcrantz, et al in Sweden, snoring every night was reported in 6.2% of children, and an additional 18% when infected. Factors significantly correlated with snoring included the use of pacifiers, a history of tonsillitis, and the history of parental adenotonsillectomy. The prevalence of pediatric obstructive sleep apnea is estimated to be between 0.5 and 3% in various studies in the literature. By contrast, the prevalence of adult obstructive sleep apnea is estimated to be between 0.4 and 8.5% in the whole population, with elderly males having the highest prevalence between 28% and 67%. One interesting phenomenon noted in the literature is that the incidence of obstructive sleep apnea in children appears to be on the rise. This may be attributed to the reduction of in the number of routine adenotonsillectomies in children, and the higher survival rates of infants with congenital malformations predisposed to obstructive sleep apnea. A trend was noted by Dr. Rosenfeld in 1990 who examined 1,722 tonsillectomies and adenoidectomies performed between 1978 and 1986. During this time period obstructive sleep apnea as an indication for tonsillectomies and adenoidectomies increased from 0% in 1978 to 19% in 1986. As an aside, in 1996, 9/31 or 29% of patients at the Texas Children's Hospital this summer underwent tonsillectomy and adenoidectomy for pediatric obstructive sleep apnea. This data was collected in the initial phase of a study coordinated by Dr. Michael Stewart to develop a health status instrument to allow prospective assessment of changes in health status after tonsillectomy and adenoidectomy. The second phase of this study is currently underway.
With pediatric obstructive sleep apnea it is important to identify predisposing factors, confirm the diagnosis, and identify the site of the lesion. Once all of these are done a treatment plan can be formulated, and efforts can be made to address predisposing factors that may cause or exacerbate obstructive sleep apnea.
There are many different factors that are believed to predispose a child to obstructive sleep apnea. They can be divided in to soft tissue lesions, skeletal abnormalities, neurologic lesions, and others. Space occupying lesions in the nasal cavity, nasopharynx, oropharynx, or hypopharynx may cause obstructive sleep apnea. Soft Tissue Lesions The most common lesion found in pediatric obstructive sleep apnea is adenotonsillar hypertrophy. Other soft tissue lesions that are less common but may predispose children to developing obstructive sleep apnea include redundant pharyngeal tissue, nasal polyps, septal deviation, laryngomalacia, benign lesions such as recurrent respiratory papillomas, hemangiomas, lymphangiomas, or cysts, malignant neoplasms such as rhabdomyosarcoma or lymphoma, and complications of adenoidectomy and pharyngeal flap surgery. Skeletal lesions Patients with craniofacial abnormalities that cause maxillary or mandibular hypoplasia are also at increased risk of obstructive sleep apnea, due to the relatively smaller size of their airway, and in the case of mandibular hypoplasia, relative macroglossia. Neurologic lesions Neurologic lesions including neonatal anoxia, cerebral palsy, neuromuscular development delay, and progressive degenerative disorders contribute to obstructive sleep apnea since these patients have diminished muscle tone, that is worse during sleep. Adenotonsillectomy may fail to relieve the obstruction in these patients, and they are often the most frustrating to treat because in addition to obstruction, they often have problems with swallowing, leading to drooling and aspiration. Other Factors Other factors that may contribute include inherited disorders that result in multiple problems such as the muccopolysaccharidoses, Down's Syndrome, achondroplasia. Down Syndrome patients have a small pharynx, altered muscular tone, relative macroglossia, often compounded by obesity. Achondoplasia patients have similar pharyngeal anatomy. Muccopolysaccharidoses (Hurler's and Hunter's) have complex obstruction with nasal congestion, redundant pharyngeal tissue, altered pharyngeal support, and may have pharyngeal lesions as well. A familial tendency has also been reported in the literature. Reflux is another factor that may contribute to obstructive sleep apnea.
Signs and symptoms of pediatric obstructive sleep apnea can be divided into night time and day time. Night time symptoms are typically more severe and reported symptoms include snoring, restlessness, enuresis, sleep walking, restlessness, profuse diaphoresis, loud gasping respirations, paradoxical chest movements, and retractions. During the day children may appear happy and otherwise healthy. Daytime symptoms include noisy breathing, mouth breathing, chronic rhinorrhea, morning headache, behavior disturbances, poor school performance, and excessive daytime sleepiness.
Physical findings on routine office examination include adenoid facies, tonsillar hypertrophy, redundant pharyngeal tissue, and other craniofacial abnormalities.
An overnight sleep study or polysomnogram is a useful diagnostic tool to evaluate sleep disorders and is frequently used to document obstructive sleep apnea. At Texas Children's Hospital, a polysomnogram costs about $1000, and involves having the child sleep overnight in the Neurophysiology lab while multiple physiologic functions are monitored. Respiratory variables include:
Non-respiratory variables include:
Apnea is classically defined as cessation of airflow of at least 10 seconds. The definition of hypopnea, which means "little breath", is more confusing. Different centers define it differently. Common definitions include 50% reduction in airflow, 50% reduction in respiratory effort, a reduction in airflow and oxygen saturation, or a reduction in both airflow and effort with a decrease in saturation. The apnea index is defined as the total number of apneic events divided by the total sleep time and multiplied by 60. RDI/AHI apnea/hypopnea index or respiratory disturbance index is defined by the total number of apneas and hypopneas divided by the total sleep time and multiplied by 60.
An apnea index of >5 or more events/hour is a commonly used criterion for the diagnosis of obstructive sleep apnea in adults. This was formulated from studies in asymptomatic men and women between the ages of 40 and 60. Unfortunately, several studies have shown that adult criteria for obstructive sleep apnea do not identify children with serious obstruction. Normative data on sleeping respiratory function in children is scant, and there is no universal definition for criteria that define pediatric obstructive sleep apnea. Significant events in children may be shorter but more frequent than episodes of interrupted airflow than adults. In a study of 20 children with suspected obstructive sleep apnea by Rosen at Yale, in 1992, the mean apnea index was 1.9 with a range of 0 to 10.4. Only 3 of the 20 children had apnea indices of 5 or greater. Despite the scarcity of obstructive apnea indices, gas exchange was significantly impaired as evidenced by frequent desaturations. During these studies, children experienced a mean of 24.6 episodes of desaturation >5% per hour with a range 0.7 to 87 desaturation events and 5 children spent more than 15% of the night with an oxygen saturation <90%. Asleep the mean lowest saturation was 66, while the mean awake oxygen saturation was 98%. 16 of the children had desaturations below 80%. Carbon dioxide retention was also common in this group. One interesting finding of this study was that of 194 episodes of severe desaturation (SaO2<15%) only 17 (9%) occurred in association with obstructive sleep apnea events, and none were seen in association with central apnea. This work demonstrates that the majority of children (>80%) with serious upper airway obstruction during sleep are not identified by criteria based on quantitation of obstructive apnea events as in adults. Children do not have repetitive complete obstructive apneas, rather they have a pattern best described as continuous partial obstructive hypoventilation According to the American Thoracic Society's consensus statement regarding the "Standards and Indications for Cardiopulmonary Sleep Studies in Children":
As noted earlier the prevalence of snoring in children is between 7 and 9%, but the prevalence of obstructive sleep apnea in children is thought to be between 0.5-3%. This implies that only a subgroup of children who snore actually have sleep apnea. Several studies have addressed the issue of whether obstructive sleep apnea can be differentiated from primary snoring, which is a benign condition, on clinical grounds alone. In another study retrospective study, this time of 93 children at University of Texas at Dallas, using criteria more commonly used for adults, the authors found that 34 patients had obstructive sleep apnea. Although this study did not contain much statistical analysis, the authors found that the majority of patients in both groups had symptoms associated with disturbed sleep and stated that "obstructive sleep apnea did not appear to be more prevalent with any age, sex, body habitus, or symptom complex." They did find a high association of obstructive sleep apnea with tonsillar hypertrophy, craniofacial abnormalities, neurologic disease, cor pulmonale and failure to thrive. According to these authors: "Polysomnography is probably not cost effective in healthy children with large tonsils and sleep disturbance. Polysomnogrpahy remains a valuable too, however, in those children on whom adenotonsillectomy might not otherwise be performed." In a prospective study of 30 children suspected of having obstructive sleep apnea at NYU, patients were classified into three groups definite obstructive sleep apnea, possible obstructive sleep apnea, and unlikely to have obstructive sleep apnea, based on clinical examination and a audio recording of breathing during sleep. A polysomnogram was performed on all of the children, and using different criteria than the previous two studies, they found that clinical assessment of obstructive sleep apnea in children is sensitive in 92.3% but specific in 29.4% for making the diagnosis as compared to polysomnograms. They recommend obtaining polysomnograms in children with a strong clinical suspicion of apnea, but no other definite indication for tonsillectomy and adenoidectomy. They also point out the significance of a negative or suspicious polysomnogram in a child with a strong clinical history is unknown. In a retrospective study of 83 children, published in 1995, who presented to the John's Hopkins Pediatric Pulmonary Service for evaluation of possible obstructive sleep apnea, they compared the results of polysomnography with data collected in the clinical history. Based on polysomnography, 48 patients had primary snoring and 35 had obstructive sleep apnea using criteria similar to previously described. The found that there was no significant difference between these two groups with respect to age, sex, race, failure to thrive, obesity, history of excessive daytime sleepiness, snoring history, history of cyanosis during sleep, or daytime symptoms except for mouth breathing. Variable that they found significant included daytime mouth breathing, observed apnea, and struggling to breath. None of these significant variables were sufficient to discriminate between primary snoring and obstructive sleep apnea. These authors conclude that primary snoring in children cannot be reliably distinguished from obstructive sleep apnea by clinical history alone.
According to the American Thoracic Societies Guidelines: Indications:
The American Thoracic Society consensus statement states that "Assessment of sleep and breathing in the home using video and cardiorespiratory recordings with extended oximetry appears promising, but recommendations regarding their use require further clinical trials."
Once the diagnosis of obstructive sleep apnea is made, it is important to recognize the site of obstruction. Burstein, et al in 1995 developed the concept of functional an anatomic airway zones to identify where the lesion is located, and to help focus treatment. The zones are as follows:
Although the most common obstruction is caused by adenotonsillar hypertrophy in zones I and II, it is important to recognize that more than one lesion may be present, and that they may occur at different levels. Tools that are available in the clinic include flexible fiberoptic examination, which provides a dynamic examination of the upper airway. Rigid bronchoscopy and laryngoscopy under general anesthesia with spontaneous respiration, may be necessary in children where the site of obstruction can not be determined by physical examination or radiographic studies. Radiographic studies that have been found to be useful include lateral neck films that can demonstrate adenotonsillar hypertrophy and some other airway lesions. The use of fluoroscopy in awake and sleeping children has also been reported and is a useful, and non-invasive technique for evaluating the airway. It has been reported that on computed tomography examination reduced pharyngeal size correlated with increased sleep disordered breathing rates, and more severe nocturnal desaturations. The drawbacks to computed tomography are radiation exposure, expense, and that it is a non-dynamic study. Crumley, et al in 1987 reported that cine-computed tomography may be a useful dynamic radiologic study for the determining the obstructive site. One drawback to their technique is that images at the time of the report were limited to the axial plane. In 1992, a group in Japan reported the use of ultrafast MRI and found that it has the advantages of being noninvasive with high contrast resolution, allows scanning in multiple planes, allowing the whole airway to be visualized at one time. The utility of these studies in children may be questionable since they may not be cooperative.
Once the diagnosis is made, predisposing factors are identified, and the site of the lesion is identified, a rationale treatment plan can be made to address the cause and the predisposing factors. Treatment of obstructive sleep apnea in children can involve several modalities including surgery, devices to improve the airway and medical therapy. Since the most common cause of obstructive sleep apnea in children is adenotonsillar hypertrophy, the most common treatment is adenotonsillectomy. Other surgical treatments may be indicated depending on the obstructive lesion. Tracheotomy is often required in children with neurologic deficits, and in children in craniofacial abnormalities. The tracheotomy is usually required until there is growth of the craniofacial structures or corrective surgery can be performed. The use of uvulopalatopharyngoplasty in children has not been adequately studied and is rarely indicated. Devices to improve the airway can also be used. The main issue with most of these devices is that compliance is often poor. These devices are generally not indicated in the average patient with adenotonsillar hypertrophy. Nasal airways are probably the cheapest and best tolerated of these devices and can be left in for weeks in small children. Adults and older children may want to use nasal airways only at night. Dental appliances and tongue retaining devices have been reported in the literature and work by advancing the tongue or mandible to hold the pharynx open. These devices are poorly tolerated in adults and children, and rarely result in cure. The use of continuous positive airway pressure (CPAP) and Bilevel positive airway pressure BiPAP has been shown to be safe and well tolerated. Compliance may be an issue due to inconvenience, discomfort, claustrophobia, and expense. CPAP is a good option in children who continue to be symptomatic after adenotonsillectomy. Medical therapy for obstructive sleep apnea in children is usually not curative, but may help with symptoms. Weight loss programs for obese children should be encouraged, supplemental oxygen may be helpful in children who have frequent and severe desaturations, and medications to reduce nasal congestion such as decongestants, antihistamines, and nasal steroids may be beneficial. CNS stimulants are mainly used in neonates with apnea.
A prospective study from University of Cincinnati by Gerber, et al in 1996, attempted to evaluate the ability of a set of cost-effective criteria to identify pediatric patients in whom perioperative respiratory compromise is most like to develop after adenotonsillectomy. They examined 292 tonsillectomies and their was no documentation on the indications for tonsillectomy in the results. They found that the risks were significantly increased in children < 3 years of age, neuromuscular disorders, chromosomal abnormalities, difficulty breathing during sleep, restless sleep, snoring and an upper respiratory tract infection. Respiratory compromise did not occur in any patients who did not snore. Despite the lack of objective data presented, and the fact that they rarely obtain polysomnograms, the authors state that obstructive sleep apnea is an absolute contraindication to outpatient surgery. A retrospective review of 37 children with documented obstructive sleep apnea at University of Minnesota by Rosen, et al, in 1994 revealed that 10 of these patients had significant postoperative respiratory compromise secondary to obstructive sleep apnea. Risk factors that they identified as significantly increasing the risk of postoperative obstruction included age < 2 years, craniofacial anomalies, failure to thrive, hypotonia, morbid obesity, previous upper airway trauma, cor pulmonale, and having a uvulopalatopharyngoplasty. Also patients who had an apnea-hypopnea index of > 40, based on adult criteria, or an O2 saturation nadir of less than 70% were at high risk, and recommend that the children should be monitored overnight for at least the first postoperative night in a monitored unit with pulse oximetry and an apnea monitor. Nasal CPAP or BiPAP may be needed in cases were obstruction occurs. Another prospective study of 15 patients also published in 1996 from Johns Hopkins by Helfaer et al looked at the issue of postoperative respiratory compromise in mild pediatric obstructive sleep apnea after adenotonsillectomy. This group performed preoperative and postoperative polysomnograms on a group of children between 1 and 18 years of age without underlying medical problems or craniofacial abnormalities, with mild obstructive sleep apnea (defined as 1-15 events on the preoperative polysomnogram). The postoperative polysomnograms were performed in the intensive care unit on the first postoperative night. All of these patients had improvement on the postoperative polysomnograms and no patient had a dangerous desaturation. Based on this study the authors recommend that this patient population can receive immediate postoperative care after adenotonsillectomy in a nonintensive setting.
Several considerations must be taken into account in children who fail adenotonsillectomy. These patients need to be reassessed. The polysomnogram should be repeated to determine if the patient continues to have obstructive episodes. Also, thorough examination including endoscopic and radiologic evaluation should be undertaken to rule out other lesions that may be contributing to the problem. In the acute postoperative period, a nasal airway may be effective in relieve obstruction from edema, and or CPAP may be indicated. Multiple medical problems often complicate the care of neurologically impaired children, and adenotonsillectomy often does not completely relieve their obstructive sleep apnea. In these patients, CPAP will usually play an integral role in their therapy. In patients who can not tolerated CPAP, whose symptoms are not relieved by CPAP, or who have problems with aspiration in addition to their symptoms, often require tracheotomy as their therapy. Several authors have advocated more aggressive surgeries in patients with severe refractory obstructive sleep apnea in order to avoid tracheostomy, but they have found that many of the patients with neurologic deficits fail these surgeries as well. Case Presentation A four-year-old boy presented to the Otolaryngology Clinic for evaluation of obstructive sleep apnea after evaluation by several outside physicians. The patients symptoms included noisy breathing, gasping for air during sleep, loud snoring, restless sleep, frequent arousals, excessive diaphoresis and persistent purulent rhinorrhea. He slept for approximately twelve hours a day, but was fatigued when awake. His medications included a beclomethasone dipropionate (Vancenase) nasal inhaler and ephedrine. His past medical history was significant for bilateral myringotomy with placement of ventilating tubes and KTP-YAG laser adenoidectomy, at age 18 months, for recurrent otitis media with effusion. His otitis media with effusion has been persistent since his ventilating tubes have extruded, and the sleep apnea symptoms also developed after this surgery. The parents stated that he had no breathing or feeding problems prior to surgery. He had no history of sleep apnea or stridor. The work-up performed prior to presentation, included: a soft tissue lateral film of the neck that revealed minimal adenoid hypertrophy, and a two-hour home sleep study that was interpreted as having multiple apnea events; including 21 apneas and 42 hypopneas. His oxygen saturations dropped as low as 85% with an average of 95%. Significant findings on head and neck examination included excessive mucopurulent secretions, and tonsillar hypertrophy. Flexible fiberoptic nasal endoscopy revealed obstruction of the right posterior choana with only a pin-sized opening, and complete obstruction of left. Computed tomography confirmed the endoscopic findings. 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