In general, most children with retrognathia have some degree of airway obstruction as part of their anomaly. In managing these children, physicians should attempt to minimize any airway obstruction present while promoting normal neurologic development and ensuring appropriate weight gain. Although positioning may be successful in some patients, alternative measures have been necessary in other children in order to enable retrognathic children to grow and develop normally. The literature is polarized over this issue, and clinical decisions seem to be based as much on emotion as on scientific data. This chapter is intended to help the clinician decide which retrognathic patients warrant operative intervention and those who should be managed conservatively.

Several potential etiologic factors in airway obstruction with retrognathia have been described, including (1) posterior displacement of a normal-size tongue; (2) loss of support of the genioglossus muscle; and (3) development of negative pressure within the upper aerodigestive tract during deglutition and inspiration resulting in glossoptosis. These events cause airway obstruction by means of four different mechanisms: (1) antero/posterior collapse secondary to posterior movement of the tongue against the posterior pharyngeal wall; (2) posterior/superior displacement of the tongue, prompting contact between the tongue, velum, and pharyngeal wall in the superior oropharynx; (3) pharyngeal obstruction secondary to prolapse of the medial walls of the pharynx; and (4) constriction of the pharynx in a circular manner with movement of the tongue as well as both lateral pharyngeal walls. In some children with retrognathia, brain stem immaturity may lead to apnea and hypoxia from vagal overactivity and esophageal motor abnormalities.1–3

The need for intervention in retrognathic children is driven by the potential consequences of unrecognized and inappropriately managed obstruction (i.e., hypoxia, cor pulmonale), failure to thrive, and embarrassment of central nervous system (CNS) activities. It is essential to remember that children with retrognathia may have significant hypoxia without apparent obstructive episodes.⁴ These may occur more frequently with advancing age as the child’s metabolic demands increase associated with increasing physical activity. Similarly, the degree of obstruction and resultant hypoxia may worsen with superimposed upper respiratory infections. Thus, vigilant observation is mandatory in order to prevent the potentially devastating growth and neurologic sequelae of prolonged hypoxia.

Once a patient is recognized as being at risk of airway obstruction with retrognathia, an appropriate evaluation should be undertaken in order to optimize management. In addition to performing continuous pulse oximetry and serial polysomnography (as necessary),⁴ a full endoscopic evaluation of the upper aerodigestive system should be considered, to rule out other possible treatable causes of obstruction.¹ An evaluation for gastroesophageal reflux disease (GERD) may be appropriate, as GERD may worsen known airway compromise. Children with retrognathia are at risk of GERD because of the increased negative intrathoracic pressure often found in this population of children. Both a pH probe and a gastric emptying scan should be obtained to quantify any potential problems.⁴

Although airway obstruction often improves with time as infants develop voluntary tongue control and progressive mandibular growth occurs, this may take months. It is clearly impractical to keep children hospitalized until these events occur, assuming they take place. Thus, as a result of economic issues and the desire to establish family integrity, it is necessary for physicians to develop a rational plan for airway management at home.⁵ Should a child be premature or have associated medical problems that mandate hospitalization, definitive intervention may be delayed until discharge planning begins.

In making a determination about whether a patient needs intervention, one should consider whether the patient has isolated retrognathia or retrognathia associated with a syndrome. A classic example is provided by examining children with Pierre Robin sequence (PRS), who have either isolated Robin sequence (IRS) or Robin sequence with a syndrome (RSS). Associated malformations have been reported in up to 82% of children with PRS. Categorization is important prognostically as one group found that there was a 22.8% mortality in RSS but only a 5.9% mortality in those with IRS.⁶ Another method of categorization places patients into groups with mild obstruction (airway obstruction resolves with posture alone; supine nursing possible), moderate obstruction (utilization of nasopharyngeal airway and prone positioning for feeding after removal of nasopharyngeal airway), and severe obstruction (long-term management with nasopharyngeal airway or tracheotomy).⁷ There are probably more children with mild obstruction than are recognized generally since most studies only involve hospitalized children. PRS patients in the general population may be managed conservatively at home in most circumstances and therefore would not be included in most studies. As a result, the percentage of children with PRS who require surgical intervention is overestimated in the literature. In one study of 125 hospitalized patients with PRS, 45% had mild obstruction, 32% had moderate obstruction, and 23% had major respiratory difficulties.⁶ Several different methods have been described for airway management in children with retrognathia and symptomatic airway obstruction. These include lateral and prone positioning, use of intraoral devices, use of a nasopharyngeal airway, intubation, glossopexy (including tongue–lip adhesion [TLA]), mandibular expansion by traction, and tracheotomy.⁸ Advantages and disadvantages are present in each of these techniques, and their relative merits will be compared and contrasted.

In the retrognathic patient with acute obstruction, prone positioning may be effective if the airway obstruction is secondary to glossoptosis. This allows the effects of gravity to prevent the tongue from falling into the hypopharynx. In addition, this will minimize the effects of negative intrathoracic pressure during swallowing and inspiration, which cause the tongue to prolapse posteriorly due to its lack of voluntary muscular control.⁹

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