Airway management in children with cleft palate and/or micrognathia

Cleft lip and/or palate is the commonest congenital craniofacial abnormality affecting approximately 1 in 700 newborns each year. It comprises of a heterogenous group of disorders affecting facial growth and cosmesis, that are associated with increased risk of airway obstruction, sleep disordered breathing (SDB), glue ear and chronic ear disease, feeding difficulties, and failure to thrive (FTT). Cleft palate (CP) can be accompanied by an abnormally undersized jaw, known as micrognathia; although micrognathia can also be found in isolation. The craniofacial abnormalities found in these children can lead to a reduction in airway size due to the tongue falling backwards. The risk of airway obstruction ranges from intermittent airway collapse during sleep (obstructive sleep apnoea, OSA) to potentially life-threatening airway compromise necessitating intubation or a tracheostomy. This paper sets out to describe the pathophysiology of airway compromise in these children, recognising clinical symptoms and appropriate referral strategy, as well as a broad range of management options.     

Radiologic evaluation of adenoids and tonsils in children with obstructive sleep apnea: Plain films and fluoroscopy

Twenty-six children with obstructive sleep apnea were evaluated by lateral neck radiographs during wakefulness, and by polygraphic monitoring and upper airway fluoroscopy during natural sleep. Children with craniofacial abnormalities, palatal surgery, and central nervous system disease were excluded from the study. Moderate or marked enlargement of tonsils and adenoids was noted on lateral neck radiographs of 18 of 26 patients. An objective measure of adenoidal enlargement, the adenoidal-nasopharyngeal ratio, correlated well with subjective judgment of adenoidal size but was not generally more useful than subjective estimation. Upper airway fluoroscopy demonstrated the site and mechanism of obstruction in all patients. Because all children with moderate to marked adenotonsillar enlargement demonstrated obstruction at the adenoidal or tonsillar level on fluoroscopy, we now screen children with suspected sleep apnea with lateral airway radiographs and polysomnography. Fluoroscopy is reserved for children with mild adenotonsillar enlargement, craniofacial dysplasia, prior cleft palate repair, or neuromuscular disorders. These results suggest that the pathogenesis of obstructive sleep apnea in children involves anatomic factors which narrow the upper airway, sleep-related hypotonia of pharyngeal dilator musculature, and compensatory mechanisms to prevent or alleviate asphyxia.     

Using continuous nasal airway pressure in infants with craniofacial malformations

Obstructive sleep apnea (OSA) is common in infants and children with craniofacial malformations. Continuous positive airway pressure (CPAP) represents an effective noninvasive treatment for severe upper airway obstruction in these children, reducing the need of surgery or a tracheostomy. The decision to start CPAP should be discussed by a multidisciplinary team in order to decide the optimal individualized treatment strategy. CPAP initiation depends on patients’ clinical characteristics and local practices, with an increase tendency towards an outpatient program. Follow-up and monitoring strategy varies among centers but benefits from the analysis of built-in software data in order to assess objective adherence and breathing parameters, reducing the need of in-hospital sleep studies. The possibility to wean CPAP should be periodically checked after surgical treatment or when spontaneous resolution is suspected. Finally, these infants with craniofacial malformations should have a long term follow up because of the risk of OSA recurrence over time.     

The pediatric use of bilevel positive airway pressure therapy for obstructive sleep apnea syndrome: a retrospective review with analysis of respiratory parameters

The purpose of this study was objective documentation of clinical benefits of bilevel positive airway pressure in pediatric patients with obstructive sleep apnea. We performed a retrospective chart review and data collection/analysis in a suburban tertiary care children's hospital. The study consisted of 10 pediatric patients (age range: 3 to 18 years); 3 patients had craniofacial abnormalities, 1 patient had neuromuscular disease, and 6 patients were obese. Eleven-channel polysomnography and support with bilevel positive airway pressure were used. We measured obstructive sleep apnea indices, lowest oxygen saturation rate, and average breath lengths before and after bilevel positive airway pressure use. We found that the apnea index decreased from 19.7 +/- 26.46 to 0.82 +/- 1.01, the lowest oxygen saturation increased from 75.60% +/- 14.93% to 89.50% +/- 5.50%, and breath length increased from 3.22 +/- 0.95 to 3.68 +/- 0.82. Bilevel positive airway pressure may be considered as a treatment modality for pediatric patients with obstructive sleep apnea.     

Sleep apnea

Objective To study clinical presentation of sleep disordered breathing (SDB) in children, their causative factors and response to treatment. Methods A retrospective study of clinical data nd results of overnight polysomnography done at baseline and after therapy were reviewed in 56 patients under 18 years of age. Results Of the 56 patients included in the study 23(41%) cases were positive for SDB. 12 (52.1%) patients had craniofacial abnormalities, 4 (17.3%) had neuromuscular and skeletal disorders, 2 (8.6%) had adenotonsillar hypertrophy, 1 (4.3%) had bilateral vocal cord palsy and 3 (13%) had sleep apnoea associated with multisystemic disorders. Post-operative data showed improvement in all 6 cases of craniofacial abnormalities and both cases of adenotonsillar hypertrophy. Positive airway pressure treatment was useful in cases with obstructive sleep apnea (OSA) due to vocal cord palsy, theracic scoliosis, systemic disorders and central hypoventilation. Conclusion 41% of suspected cases were detected to have SDB. Craniofacial abnormality was the leading cause of OSA in the present study. Surgical correction improved symptoms apnea-hypopnea index (AHI) and desaturation in cases of craniofacial disorders and adenotonsillar hypertrophy. Vocal cord palsy thoracic scoliosis, hypoventilation and systemic disorders associated OSA responded to positive airway pressure ventilation.     

Obstructive sleep apnea syndrome in infants and children]

Obstructive sleep apnea syndrome (OSAS) is characterized by prolonged, generally partial, upper airway obstruction associated with hypoxemia and/or hypercapnia. Main etiological factors include hypertrophy of the tonsils and adenoids, craniofacial abnormalities with reduction in the upper airway caliber, abnormality of neural upper airway control, or a combination of these factors. Symptoms depend on age, but they always include snoring and breathing difficulties during sleep. Diagnosis of OSAS must be established early in order to prevent complications. It is suspected on history, physical examination and investigative confrontation such as nasofibroscopy and imaging. Polysomnography is the gold standard for diagnosis, scoring of the obstruction and distinction between primary snoring and obstructive breathing. Adenotonsillectomy is an effective therapy. For selected patients, craniofacial surgery may be helpful. Some children require continuous positive airway pressure or the nasopharyngeal tube. Tracheotomy is rarely indicated.     

Schnarchen bei Kindern

Background

Snoring in children is a prevalent symptom and may be an indicator of obstructive sleep apnoea. Despite its importance, there is no national guideline on its appropriate management.

Objective

To provide recommendations for the management of snoring in children and adolescents treated in a primary care setting.

Methods

A total of 16 national paediatric sleep experts were included in a Delphi process and formulated recommendations in the form of a step-wise work-up procedure.

Results

The following 8 steps were developed: (1) Identification of true cases of habitual snoring. (2) Identification of high-risk patients who should undergo polysomnography in a sleep laboratory. (3) Identification of mild cases that may be treated with anti-inflammatory medication. (4) Identification of cases that should be referred to an otorhinolaryngologist for potential surgery. (5) Performance of polysomnography in cases that remain unclear despite steps 3 and 4 to rule out obstructive sleep apnoea. (6) Reconsideration of surgery in cases with moderate to severe obstructive sleep apnoea. (7) Identification of severe sleep apnoea cases requiring continuous positive airway pressure therapy. (8) Identification of cases suitable for orthodontic treatment, craniofacial surgery or speech therapy.

Conclusion

This guideline should help to improve the management of snoring children and adolescents in Germany.     

The role of polysomnography in diagnosing and treating obstructive sleep apnea in pediatric patients

Obstructive sleep apnea in children is associated with serious neurocognitive and cardiovascular morbidity, systemic inflammation, and increased health care use, yet remains underdiagnosed. Although the prevalence of obstructive sleep apnea is 1-3% in the pediatric population, the prevalence of primary snoring (PS) is estimated to be 3-12%. The challenge for pediatricians is to differentiate PS from obstructive sleep apnea in a cost-effective, reliable, and accurate manner before recommending invasive or intrusive therapies, such as surgery or continuous positive airway pressure. The validity of polysomnography as the gold standard for diagnosing obstructive sleep apnea has been challenged, primarily related to concerns that abnormalities on polysomnography do not correlate well with adverse outcomes, that those abnormalities have statistical more than clinical significance, and that performing polysomnograms on all children who snore is a practical impossibility. The aim of this article is to review the clinical utility of diagnostic tests other than polysomnography to diagnose obstructive sleep apnea, to highlight the limitations and strengths of polysomnography, to underscore the threshold levels of abnormalities detected on polysomnography that correlate with morbidity, and to discuss what the practical implications are for treatment.     

Obstructive sleep apnea syndrome in children

Partial or complete repetitive obstructions of the upper airway during sleep give rise to clinical symptoms associated with heavy, chronic snoring. The number of obstructive sleep apneas during the night may be less important than the repetitive inspiratory increases in upper airway resistance, even if these are associated only with a partial airway collapse. Oxygen saturation may not be severely affected by partial occlusion during nocturnal recording, although clinical symptoms may occur. Esophageal pressure measurements and breathing frequency during sleep are key features in the polygraphic evaluation of prepubertal children. Tonsillectomy and adenoidectomy may be helpful in treating children with small upper airway during sleep. The marked interaction between upper airway adequacy and craniofacial morphology make it critical to evaluate the impact of partial or complete airway occlusion during sleep on facial prognathism. Nasal continuous positive airway pressure is a safe treatment for persistent, partial or complete upper airway occlusion during sleep, but it does not address the mandibular deficiency often seen in symptomatic children. Orthodontic evaluation and treatment may make maxillomandibular surgery unnecessary during the pubertal years.     

Obstructive sleep apnea syndrome in childhood

Obstructive sleep apnea syndrome (OSAS) is a well-known clinical entity in adults but until now it has been less well studied in children. Several studies have shown that the prevalence of pediatric OSAS is high (between 1% and 3%) and its consequences can be serious. Major risk factors for OSAS in children include adeno-tonsillar hypertrophy, neuromuscular disease and syndromes such as Down's or Pierre-Robin's syndrome. Definitive diagnosis is by nocturnal polysomnography while other methods such as cardiorespiratory records and nocturnal pulse oximetry are undoubtedly useful. Adenotonsillectomy plays a major role in the treatment of OSAS. Nasal continuous positive airway pressure is an alternative in children who show poor response to surgical treatment or in those with craniofacial alterations. In a few cases, nocturnal oxygen administration can be useful.     

Magnetic resonance imaging of obstructive sleep apnea in children

Sleep-disordered breathing has a spectrum of severity that spans from snoring and partial airway collapse with increased upper airway resistance, to complete upper airway obstruction with obstructive sleep apnea during sleeping. While snoring occurs in up to 20% of children, obstructive sleep apnea affects approximately 1–5% of children. The obstruction that occurs in obstructive sleep apnea is the result of the airway collapsing during sleep, which causes arousal and impairs restful sleep. Adenotonsillectomy is the first-line treatment of obstructive sleep apnea and is usually effective in otherwise healthy nonsyndromic children. However, there are subgroups in which this surgery is less effective. These subgroups include children with obesity, severe obstructive sleep apnea preoperatively, Down syndrome, craniofacial anomalies and polycystic ovarian disease. Continuous positive airway pressure (CPAP) is the first-line therapy for persistent obstructive sleep apnea despite previous adenotonsillectomy, but it is often poorly tolerated by children. When CPAP is not tolerated or preferred by the family, surgical options beyond adenotonsillectomy are discussed with the parent and child. Dynamic MRI of the airway provides a means to identify and localize the site or sites of obstruction for these children. In this review the authors address clinical indications for imaging, ideal team members to involve in an effective multidisciplinary program, basic anesthesia requirements, MRI protocol techniques and interpretation of the findings on MRI that help guide surgery.     

Positioning as a conservative treatment option in infants with micrognathia and/or cleft

Evaluation and management of airway obstruction in prone position were reviewed from studies in infants with micrognathia and/or cleft palate, using polysomnography (PSG) or similar measures, and comparing prone against other positions. Most studies identified were case series from specialist referral centres. Airway obstruction appears more severe on PSG than clinical assessment, but there is no consensus for PSG definitions of mild, moderate or severe airway obstruction. Infants show individual variability in responses to positioning; sleep quality tends to improve when prone, but 22–25% have better respiratory outcomes when supine. Most centres recommend home monitoring if advising that an infant be placed prone to manage their airway obstruction. In conclusion, in case series, success rates for managing infant airway obstruction by prone positioning vary from 12 to 76%. PSG studies comparing prone with other sleep positions can help differentiate which infants show improved airway obstruction and/or sleep quality when positioned prone.     

Pediatric obstructive sleep apnea syndrome and anesthetic management

Sleep-related breathing disorders require special attention in children who spend a considerable time sleeping. Obstructive sleep apnea syndrome is characterized by episodes of upper airway obstruction during sleep. Symptoms include hyperactivity, enuresis, headache, failure to thrive, and increased respiratory effort and total sleep time. The most common cause is adenotonsillar hypertrophy. Coexisting diseases are obesity, neuromuscular and craniofacial anomalies, and Down's syndrome. Early diagnosis is important to minimize neurocognitive, cardiac and developmental complications. Polysomnography is the gold standard for diagnosis. Although the features of pediatric obstructive sleep apnea syndrome are distinctly different from that in adults, it may predispose to the adult type of the syndrome. As therapy concerns several surgical approaches as well as conservative techniques, anesthetic management calls for particular attention. Pre- and postoperative sedation must be performed cautiously and patients must be watched closely with respect to airway obstruction and hypoventilation. Difficult intubation must always be considered.     

Evaluation and management of upper airway obstruction

This review provides an overview of acute and chronic upper airway obstruction. Glottic or subglottic obstruction affects the airway when it is a single channel. This is potentially life-threatening and presents with stridor and/or respiratory distress. Supraglottic airway obstruction generally affects the nasal or nasopharyngeal airway and tends to predominantly affect breathing during sleep. There are a number of different causes for each of these clinical problems.In acute glottic or subglottic obstruction, management consists of a rapid assessment and the establishment of a secure airway when the obstruction is life-threatening. Thereafter the management will be targeted on the underlying condition. In supraglottic airway obstruction it is important to recognize that symptoms may be subtle and a high index of suspicion is needed, particularly when there are co-morbidities which make sleep-disordered breathing more likely. In severe supraglottic obstruction causing significant sleep-disordered breathing adenotonsillectomy is usually the treatment of choice, but other measures including non-invasive continuous positive airway pressure (CPAP) or ventilation may be necessary if this is unhelpful.     

Sleep apnoea in infants

Infancy is characterized by an instability of the control of breathing. Apnoeas of short duration are common, mostly central and more frequent during rapid eye movement sleep. Obstructive apnoeas are rare in healthy infants. Triggering factors, such as respiratory syncytial virus infection, can increase the frequency and duration of apnoeas. Upper airway problems are responsible for obstructive apnoeas as well as for episodes of partial airway obstruction or upper airway resistance syndrome. In some infants, apparent life-threatening events have been related to upper airway anomalies. Congenital central hypoventilation syndrome, a rare respiratory control disorder, may present with apnoeas. Polysomnography is the gold standard for diagnosing sleep-disordered breathing in infants. Nasal continuous positive airway pressure is feasible in infants, and the early diagnosis of abnormal breathing during sleep is of critical importance for neurocognitive development in infants.     

Interventions in the paediatric sleep laboratory: the use and titration of respiratory support therapies

During sleep changes in central and peripheral neurological pathways and muscle tone result in unique vulnerabilities in the respiratory system. Abnormalities of the respiratory system that are not apparent in wakefulness can become evident during particular sleep states, making overnight polysomnography (PSG) a valuable diagnostic indicator of the source as well as the severity of the abnormality. In this review these respiratory disorders are grouped according to whether they are attributable to upper airway collapse, poor gas diffusion or inadequate ventilation (respiratory effort). Inadequate ventilation may be secondary to abnormal respiratory drive (control) or to inadequate pulmonary muscle function. As a diagnostic tool, overnight PSG can help distinguish whether the origin of the disorder is central or peripheral on the basis of which sleep state is associated with greatest abnormality. The most common treatment interventions include supplemental oxygen, continuous positive airway pressure and non-invasive ventilation. Ventilation may be with either pressure or volume cycle devices. Overnight PSG is used for the titration and monitoring of these treatments since all these forms of respiratory support require regular adjustment to match patient requirements. The methods for titration and goals of optimal therapy in the paediatric sleep laboratory are discussed.     

Nasal continuous positive airway pressure (CPAP) therapy for obstructive sleep apnea in Hallermann-Streiff syndrome

An 8-year-old girl with Hallermann-Streiff syndrome (oculomandibulofacial syndrome) was examined. She had a history of severe snoring, reported nocturnal apnea, excessive daytime hypersomnolence, nocturnal enuresis, and failure to thrive. Overnight polysomnography confirmed severe obstructive sleep apnea. Long-term nasal continuous positive airway pressure (CPAP) therapy completely relieved the obstructive sleep apnea and was associated with improved weight gain and growth.     

Evaluation of epiglottoplasty as treatment for severe laryngomalacia

Six patients with severe laryngomalacia underwent epiglottoplasty. Four of these patients had life-threatening episodes of airway obstruction before surgery; of these, two had required tracheal intubation and one had required cardiopulmonary resuscitation. Two patients had failure to thrive and two had cor pulmonale. Patients had required a mean of two hospitalizations related to upper airway obstruction. We performed polysomnography during a daytime nap, both before and after epiglottoplasty, in all patients. Respiratory effort, arterial oxygen saturation, and end-tidal carbon dioxide pressure were monitored with continuous electrocardiograms and electrooculograms. All patients had abnormal polysomnograms preoperatively. Six patients had obstructive apnea, four had hypoxemia (arterial oxygen saturation less than 90% while breathing room air), and four had hypoventilation (end-tidal carbon dioxide pressure greater than 45 mm Hg) before epiglottoplasty. Mean age (+/- SEM) at epiglottoplasty was 10.3 +/- 5.3 months. No patients had surgical complications. An endotracheal tube was in place for 25 +/- 7 hours postoperatively, and patients were discharged 4 +/- 1 days postoperatively. Polysomnography performed 2.8 +/- 1.0 months after surgery showed that all patients had improved. Two patients had residual, mild episodes of obstructive apnea, and one patient had mild hypoventilation and desaturation. No patient had further life-threatening events or required further hospitalizations after epiglottoplasty. We conclude that epiglottoplasty is an effective and safe treatment for a selected group of patients with severe laryngomalacia.     

Sleep physiology and sleep-disordered breathing: the essentials

Sleep is essential, but poses a risk to breathing in some children. We have outlined the developmental changes in sleep patterns and physiology, and the evidence of deleterious effects of sleep deprivation and of sleep-related breathing disorder (SRBD). Some factors increase the risk of adenotonsillar surgery and should be excluded before contemplating surgery in a secondary care setting. Screening for SRBD is indicated in some conditions, which are discussed. Although simple studies may suffice for many patients, there are a few who need more detailed assessment with polysomnography. A managed clinical network would be the most appropriate model to ensure appropriate organisation and utilisation of scarce resources in this area.