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Anesthesia & Analgesia:
doi: 10.1213/ANE.0000000000000301
Pediatric Anesthesiology: Review Article

Pierre Robin Sequence: A Perioperative Review

Cladis, Franklyn MD, FAAP*; Kumar, Anand MD; Grunwaldt, Lorelei MD; Otteson, Todd MD; Ford, Matthew MS, CCC-SLP; Losee, Joseph E. MD, FAAP

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Continuing Medical Education
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Author Information

From the Departments of *Anesthesiology and Plastic Surgery, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania; and Pediatric Otolaryngology, UH Rainbow Babies and Children’s Hospital CWRU School of Medicine, Cleveland, Ohio.

Accepted for publication March 28, 2014.

Funding: None.

Conflicts of Interest: See Disclosures at the end of the article.

Reprints will not be available from the authors.

Address correspondence to Franklyn Cladis, MD, Department of Anesthesiology, Children’s Hospital of Pittsburgh of UPMC, 4401 Penn Ave., 5th Floor, Pittsburgh, PA 15224. Address e-mail to Cladfp@anes.upmc.edu.

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Abstract

The clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the tongue), and airway obstruction defines the Pierre Robin sequence (PRS). Airway obstruction and respiratory distress are clinical hallmarks. Patients may present with stridor, retractions, and cyanosis. Severe obstruction results in feeding difficulty, reflux, and failure to thrive. Treatment options depend on the severity of airway obstruction and include prone positioning, nasopharyngeal airways, tongue lip adhesion, mandibular distraction osteogenesis, and tracheostomy. The neonate and infant with PRS require care from multiple specialists including anesthesiology, plastic surgery, otolaryngology, speech pathology, gastroenterology, radiology, and neonatology. The anesthesiologist involved in the care of patients with PRS will interface with a multidisciplinary team in a variety of clinical settings. This perioperative review is a collaborative effort from multiple specialties including anesthesiology, plastic surgery, otolaryngology, and speech pathology. We will discuss the background and clinical presentation of patients with PRS, as well as some of the controversies regarding their care.

In 1923, Pierre Robin, a stomatologist from France, coined the term “glossoptosis” to describe the “obstruction of the oral pharynx by the tongue” in the setting of a small mandible. Although others have previously described patients with small mandibles, airway obstruction, and cleft palate, Dr. Robin has been credited with defining the criteria for the Pierre Robin sequence (PRS).1,2 The clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the base of the tongue; Fig. 1), and airway obstruction defines PRS. Some authors have described PRS as micrognathia, glossoptosis, and cleft palate.3,4 Although clefting of the palate is common, it does not occur in all infants with PRS. However, all patients with PRS have airway obstruction,5 and this is a requirement for the clinical diagnosis.

Figure 1
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There is ongoing debate regarding the biologic etiology of PRS. There does not appear to be a clear genetic abnormality, although the etiology of the micrognathia may be different for syndromic and nonsyndromic PRS.6 Some authors have suggested a genetic cause for syndromic PRS and an external mechanism like in utero compression secondary to oligohydramnios for nonsyndromic PRS.6 It is suspected that micrognathia keeps the tongue superiorly positioned between the naturally clefted palatal shelves and prevents normal palatal closure during the first trimester of pregnancy. Although micrognathia appears to be an isolated event for most patients with PRS, there is an association with certain syndromes. The most common syndromes are Stickler, velocardiofacial (22q, 11.2 deletion), fetal alcohol syndrome, and Treacher-Collins syndrome (Table 1).5,7,8

Table 1
Table 1
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The incidence of PRS varies from 1:5000 to 1:85,000.3,6,9,10 The large variation in the incidence of PRS may be dependent on the variability of clinical presentation. The patient with mild symptoms may go unrecognized. Infants with PRS present with respiratory and feeding difficulties. As many as half of these infants will also have associated malformations. Airway obstruction and respiratory distress are the primary respiratory signs. Patients may present with stridor, retractions, and cyanosis. Severe obstruction results in feeding difficulty, reflux, and failure to thrive.

Treatment options depend on the severity of airway obstruction. Obstruction that is mild when supine or relieved completely when prone may be observed without a procedural intervention. Prone positioning may relieve airway obstruction in as many as 70% of these subjects.6 The therapeutic options for more severe obstruction include nasopharyngeal airways (NPA), tongue lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and tracheostomy. Clinical intervention is frequently dependent on the experience of the institution. Centers with larger patient volumes may have a multidisciplinary protocol that defines the clinical course of these patients.

The neonate and infant with PRS require care from multiple specialists including plastic surgery, otolaryngology, speech pathology, gastroenterology, radiology, and neonatology. The anesthesiologist involved in the care of patients with PRS will interface with this multidisciplinary team in a variety of clinical settings. This perioperative review is intended to discuss the background and clinical presentation of patients with PRS and some of the controversies regarding their care.

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FEEDING

Feeding and swallowing are complex biologic functions. In the newborn period, suckling is quite reflexive and likely is controlled at a subcortical level. Nutritive suckling is accomplished by compression of a nipple against the palate. Most infants will have multiple suckles, which fills the vallecular space before swallowing. During deglutition, the tongue expresses and propels the milk/formula posteriorly, while the soft palate rises to separate the mouth from the nose. The bolus travels to the vallecular space before swallowing. The larynx then is pulled upward and mildly forward resulting in epiglottic closure. Simultaneously, respiration ceases momentarily and the vocal folds close. The presence of a cleft palate decreases the infant’s ability to establish negative intraoral air pressure required to suck milk from a normal bottle or breast. This results in decreased organization of bolus formation and transit. The palatal defect creates the infant’s inability to separate the mouth from the nose.11 The prevalence of feeding and swallowing disorders in patients with PRS has been estimated at 25% to 45%.12 These difficulties are linked to their respiratory issues.13–15 However, correcting the airway obstruction will not always improve feeding and swallowing issues.

Positioning an infant prone with PRS may be adequate to manage minor upper airway obstruction by minimizing glossoptosis, but this will not obviate the infants’ feeding challenges. Infants cannot be successfully fed in the prone position, and many cannot safely or adequately swallow once positioned upright for feeding. Some PRS infants need long-term nasogastric or gastrostomy tube feeds. Temporary NPAs have been used for airway management and frequently will manage the airway obstruction but again may not obviate the need for nasogastric or gastrostomy feeds for adequate nutrition and growth.16,17

Surgical interventions have been described to successfully manage glossoptosis and upper airway obstruction.18–20 While this has been associated with improvement in feeding, it is not a guarantee of successful oral feeding. Smith and Senders21 noted a prevalence of nasogastric or gastrostomy tube feedings of 53% in isolated PRS patients, 67% in syndromic patients, and 83% in “unique” (abnormalities that did not satisfy the criteria for a named syndrome) PRS patients. MDO is a technique frequently used for the management of more severe PRS patients. In 2004, Monasterio et al.20 found a trend toward resolution of feeding difficulties in most patients in their cohort with MDO.

Despite correction of the airway obstruction, many infants with PRS will have persistent feeding and swallowing challenges. The causes of these challenges are multifactorial. Baudon et al.22 in 2002 and Baujat et al.23 in 2001 documented, by electromyography and esophageal manometry, an increased prevalence of esophageal motility disorders. Gastroesophageal reflux (GER) is a known comorbidity with PRS and is thought to be due to altered intrathoracic pressures secondary to airway obstruction.24,25 The clinical consequences of GER include aspiration, and pharyngeal and laryngeal edema. Laryngeal edema increases airway obstruction. Because of the incidence and consequences of reflux, most practitioners advocate prophylactic medical treatment.

Infants with PRS require additional feeding support to deal with altered suction abilities13 and formula delivery.26,27 Specialized bottles, nursers, and nipples are typically required for an adequate volume of milk delivery to infants with cleft palate. These feeders allow milk to be actively dispensed into the infant’s mouth during suckling maneuvers. This may include the Mead Johnson Cleft Nurser, Pigeon Cleft Bottle, or Haberman Special Needs Feeder (Fig. 2, A–C).

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Although respiratory distress frequently results in an acute life-threatening event and morbidity in infants with PRS, swallowing and feeding issues can also contribute to infant morbidity and mortality. Untreated aspiration can lead to significant pulmonary morbidity. Inadequate nutrition similarly can result in failure to thrive and associated morbidity. This underscores the critical importance of an in-depth feeding and swallowing evaluation in the PRS infant. Assessment of swallowing safety and aspiration risk is primary for developing an appropriate feeding plan. Some infants can be fed safely with alterations of feeding with specialized feeding equipment, altered formula/milk consistency, or modified feeding schedules. However, many require time to transition from nonoral feeding to oral feeding as their respiratory status improves with interventions and general growth and development. Nasogastric or gastrostomy feeds do not preclude oral feeding attempts and should be considered in any infant with significant nutritional or aspiration risks. Feeding facilitation techniques have also been advocated to improve oral feeding. This includes oral stimulation and tongue massage techniques with a pacifier and gloved fingers.28

Depending on the institution, the PRS infant with feeding difficulties is typically managed by either a speech pathologist and/or an occupational therapist. Given the complex interaction between feeding and airway difficulties in the infant with PRS, early input from both speech pathology and occupational therapy is critical to the success of these patients. Feeding and swallowing evaluations typically take place in the neonatal intensive care unit at the bedside once the infant is medically stable. Oral trials should not be considered until the infant demonstrates baseline stable respiratory status, adequate oropharyngeal secretion control, and adequate level of alertness. Fatigue with oral stimulation should be documented. When aspiration risk is identified, it is critical to obtain further direct swallowing assessment before initiation of ad lib oral feeds.

Comprehensive swallowing evaluation should be performed using direct assessments, and these are typically performed as part of PRS treatment protocols in most centers. Assessments may be done before and after surgical or nonsurgical interventions such as MDO, TLA, or placement of an NPA. The “gold standard” for assessing swallow and aspiration risk is the modified barium swallow study, referred to as a cookie swallow study.29 This is completed in the radiology department. Swallowing is assessed under lateral fluoroscopy. The infant is positioned semi-upright in a fluoroscopic infant seating device. Silent aspiration can be identified, which refers to tracheal aspiration in the absence of coughing or choking.

Endoscopic assessment of swallowing has also been described and is frequently used in the PRS population to assess aspiration risk. This type of assessment is referred to as fiberoptic endoscopic evaluation of swallowing (Video 1, Supplemental Digital Content 1, http://links.lww.com/AA/A915).30,31 This can be performed at the bedside. This assessment is typically completed in conjunction with an otolaryngologist and speech pathologist. If milk/formula is seen on the vocal folds or in the larynx proper, the child should be considered an aspiration risk. The other benefit of fiberoptic endoscopic evaluation of swallowing is that it allows direct visualization of multiple components of the upper airway including nose, pharynx, and larynx to the level of the true vocal folds. It does not allow for evaluation of extraction ability (ability to extract milk or formula from a bottle), swallowing organization, bolus control, degree of laryngeal penetration, or direct visualization of tracheal aspiration.

Instrumental evaluation provides information necessary to establish successful oral feeding. Once swallowing safety can be assured, the child can then begin therapeutic feedings at bedside. The speech pathologist, occupational therapist, or critical care nursing team will initiate a program of feeding intervention with the goal of safe and efficient oral nutrition. Multiple techniques may be used during feeding such as chin or cheek support, pacing (altering bottle presentation or pressure and providing breaks), or physical stimulation to facilitate swallowing.

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SURGICAL MANAGEMENT

Otolaryngology

Airway obstruction in the patient with PRS may exist at multiple levels from oropharyngeal obstruction due to glossoptosis to hypopharyngeal obstruction due to collapse of the epiglottis and base of tongue (EBT) (Fig. 3, A–D). Children with PRS are especially prone to this type of hypopharyngeal collapse as a sequelae of the micrognathia. In general, the more severe the micrognathia, the more severe the EBT collapse and symptoms of airway obstruction. Other airway anomalies such as laryngomalacia (LM) or subglottic or tracheal pathology may also be present.

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The airway evaluation begins with a careful history, including details of the delivery, initial Apgar scores, and any apnea or cyanotic episodes in the early neonatal period. Observations regarding the neonate’s ability to feed are important since feeding difficulties and airway abnormalities are inextricably linked, as previously outlined. The physical examination should include a complete head and neck examination documenting any stigmata that would suggest syndromic PRS. The infant should be examined in a variety of positions, including seated, prone, and supine, evaluating for exacerbation of airway symptoms. If the infant is stable, a flexible nasolaryngoscopy is performed in a seated position, on a caregiver’s lap, and then immediately in a supine position to assess differences in the severity of any airway obstruction based on position. The flexible nasolaryngoscopy examination confirms patent choanae, determines the degree of any oropharyngeal obstruction due to glossoptosis, documents any degree of EBT collapse, and evaluates any laryngeal abnormalities, including LM and any sequelae of acid reflux. Additional information gathered from computerized tomography (CT) imaging studies, cine-magnetic resonance imaging (MRI), and polysomnography (PSG) may help determine the severity of the airway obstruction and add information to the anatomic location of the obstruction.

Mild airway obstruction at birth may prompt only conservative measures such as prone positioning or a nasal or oral airway. A lower threshold to perform airway endoscopy should be used in children with a syndrome. More severe airway obstruction that does not improve with conservative measures warrants a diagnostic endoscopy in the operating room consisting of at least a flexible nasolaryngoscopy and a direct rigid laryngo-bronchoscopy (DLB). The exact anatomic location of the airway obstruction may be determined. If the severity of the obstruction warrants it, or if multiple levels of obstruction are present, surgical intervention to improve the safety of the airway is indicated. The DLB also provides an assessment of the difficulty of orotracheal intubation. If additional intervention is required, the patient may be intubated as part of the endoscopic procedure. A DLB in an infant with PRS is more difficult technically because of the retrognathia and the position of the mandible relative to the anteriorly placed larynx.

Definitive airway management in infants with PRS is challenging and has been the topic of much debate. In general, infants with nonsyndromic PRS improve with conservative measures such as prone positioning or an NPA.32,33 They are also more likely to have symptomatic improvement after surgical interventions such as TLA or MDO. Children with syndromic PRS are more likely to fail TLA or MDO21 and may need tracheostomy tube placement and/or gastrostomy tube placement. Tracheostomy is indicated in children with multilevel airway obstruction and those who fail other surgical interventions to alleviate airway obstruction. Some advocate for tracheostomy in children with airway obstruction and PRS associated with a syndrome and neurologic comorbidities. Tracheostomy may be the required airway intervention if the infant is <2 kg34 because they may be too small to be considered a candidate for MDO.

One of the manifestations of airway obstruction is obstructive sleep apnea (OSA). OSA is defined as a “disorder of breathing during sleep characterized by prolonged partial upper airway obstruction and/or intermittent complete obstruction that disrupts normal ventilation during sleep and normal sleep patterns.”35 The prevalence of OSA in the general pediatric population is approximately from 2% to 3%36 with 3–12% having primary snoring.37 Multiple studies have shown that children with a cleft palate are more likely to have sleep disordered breathing, with prevalence data ranging from 22% to 65%.38 Robison and Otteson38 demonstrated a PSG-confirmed prevalence of OSA of 8.5% in patients with cleft palates, approximately 3 times the healthy pediatric prevalence of 2–3%. In general, younger patients were more prone to OSA, with decreasing prevalence as the population aged. One exception was a subset of patients who experienced an increase in their OSA symptoms after surgical repair of their cleft palate or the performance of a pharyngeal flap for velopharyngeal insufficiency.38 Children with PRS, including age-matched older children with PRS, are at significantly higher risk of OSA, particularly with more severe airway obstruction symptoms early in infancy.

Each child with PRS should be carefully monitored for history or examination findings that may suggest airway obstruction or OSA. There should be a low threshold for performing airway endoscopy and ordering a PSG with any treatment focused on the specific anatomic level of airway obstruction. If there are no surgical options for the treatment of OSA, a trial of bilevel positive airway pressure should be considered.

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Plastic Surgery: Tongue Lip Adhesion and Mandibular Distraction

The optimal surgical management of PRS remains a controversial topic.3,19,39–45 Most practitioners agree on the management of PRS when there is mild airway obstruction. Positioning and nasal airways are not controversial for these patients. Likewise, most agree that the PRS patient with multiple levels of airway obstruction or central apnea will likely benefit from tracheostomy. However, the care of the remaining patients, who are between these 2 clinical extremes, continues to be debated. Current therapy protocols at many major pediatric hospitals base their outcomes on a single arm experience such as NPA, TLA, MDO, or tracheostomy. Often these protocols are based on the availability, technical expertise, and preference of the airway team.3,19 A guideline for the surgical management of PRS is provided in Figure 4.

Figure 4
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The initial evaluation for PRS patients should include a sleep study, CT of the craniofacial skeleton, GER therapy, an airway evaluation with DLB, and a swallowing study. CT is used to evaluate the structure and position of the tongue, mandible, and adjacent soft tissue. Imaging facilitates surgical planning because it indicates the relative position of the inferior alveolar nerve as it enters the mandible, position of tooth buds, minimum airway space, and level of the tongue base. Knowledge of the anatomy assists with execution of the surgical plan.

A complete airway evaluation is performed to detect concurrent critical airway lesions that require therapy or may preclude surgical treatment. Indications for early surgical intervention include patients with medically controlled reflux, moderate to severe obstructive apnea without central apnea, and adequate bone for distractor placement. Patients with central apnea, medically refractory reflux, or with significant LM or tracheomalacia are offered primary tracheostomy followed by the delayed MDO, which facilitates earlier decannulation.

The management of the infant with mild airway obstruction is less controversial and can often be managed with positioning. Placing the patient prone can relieve mild airway obstruction in most PRS patients. Some have estimated that as many as 70% of PRS patients can be managed with positioning alone.6 However, placing a newborn prone conflicts with the current recommendations of the American Academy of Pediatrics “Back to Sleep” program to minimize sudden infant death syndrome. There are no data concerning the effect of prone positioning on sudden infant death syndrome in this population.46 Prone positioning also has implications on feeding.

An NPA may also be used for the patient with mild obstruction. There is a failure rate with NPA, and these patients need to be monitored with an apnea monitor. Abel et al.3 reported successful management of 60% of their patient cohort using NPA alone but noted that 14% of their patients required tracheostomy.

Patients who fail positioning and/or NPA will require a TLA, MDO, or tracheostomy. The indications for tracheostomy were described above. The TLA surgically attaches the tongue to the lingual surface of the lower lip forcing the tongue anteriorly and inferiorly.42 The TLA is performed with the infant nasotracheally intubated. The tongue flap is a broad, short flap that is based inferiorly on the ventral surface of the tongue, and the lip flap is also a broad, short flap but it is based distally. Sutures are used to sew the genioglossus muscle to the orbicularis oris muscles. It is critical that muscle is sutured together because pure mucosal sutures are not strong enough to hold the repair together. A core stitch placed around the chin point and tongue base functions as an internal support pulling the tongue forward while the adhesion heals. The core stitch is tied over buttons at the chin and tongue base (Fig. 5). The patient remains intubated and is transferred to the intensive care unit for postoperative care. Sedation and paralysis are used for several days to minimize dehiscence of the adhesion. The patient is brought back to the operating room for extubation. A pediatric otolaryngologist is present for extubation in the authors’ institution for assistance with emergency airway evaluation, rigid bronchoscopy, or tracheostomy.

Figure 5
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The purpose of the TLA is to correct the glossoptosis and relieve airway obstruction while the child and mandible are allowed to grow. The TLA is performed during the neonatal period and is left in place for several months. It is typically “taken down” before the first year of life. The reversal of the TLA can be combined with repair of the cleft palate if present. Concerns regarding TLA include dehiscence and subsequent airway obstruction. Patients also require sedation and paralysis during the postoperative period, necessitating meticulous intensive care unit care. Opponents to the TLA argue that it does not correct the underlying pathology (micrognathia).6,46 Reasonable results using TLA have been reported.42,43 Preoperative predictors of TLA success can be determined by clinical features. Rogers et al.42 created the GILLS score to help predict which patients with PRS might fail TLA. The clinical features they evaluated included GER (G), preoperative intubation (I), later operation (>2 weeks of age) (L), low birth weight (<2500 g) (L), and syndromic diagnosis (S). In their experience, patients with more than 2 risk factors had a 43% failure rate.42

The mechanism of improvement after NPA or TLA is not well understood. One explanation is that the TLA relieves the obstruction of the tongue against the posterior wall of the pharynx while the mandible and the airway grow. It has been proposed that the mandible in some patients with PRS demonstrates “catch up” growth. The NPA or TLA reduces airway obstruction until some of this growth can occur. The concept of catch up growth is controversial. There is some evidence that the mandible in PRS patients achieves normal growth by 4 to 6 years.5 However, PRS patients are a disparate and varied group. Some have syndromes, while others do not. Mandibular growth may be quite different for individual PRS patients depending on the etiology of their mandibular hypoplasia. The syndromic PRS mandible may never grow normally. Airway neuromotor development may also explain improvement after NPA and TLA. As infants mature, they may learn to relieve their airway obstruction through a maturation of airway neuromotor control. The NPA or TLA improves airway patency until this development occurs.

MDO has emerged as an effective but controversial treatment for symptomatic PRS.19,42,43 Distraction osteogenesis, a well-described technique for skeletal elongation, has been reported for more than 2 decades in the orthopedic and plastic surgery literature. The modern application of distraction to elongate the diminutive neonatal mandible and directly treat skeletal-related airway obstruction is still evolving. MDO is performed via an internal or external approach based on the craniofacial surgery team’s preference and experience. For the external approach, an incision is placed 1 cm below the horizontal ramus/angle of the mandible. Bilateral mandibular osteotomies are performed, and the distraction device is secured with screws on either side of the osteotomy (Fig. 6, A–D). There are various protocols of latency, distractions, and consolidation. The device is activated after 24-hour latency. Distraction is performed at a rate of 2 mm/d for 5 days, and then 1 mm/d as needed to achieve a 2-mm overjet (upper dental arch relative to lower dental arch). The duration of distraction depends on the distance required to elongate the diminutive mandible.

Figure 6
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After 5 days, the mandible has been distracted approximately 6 to 10 mm, and the infant may be ready for extubation. Clinical features that predict readiness for extubation include a downward displacement of the tongue toward the floor of the mouth and the endoscopic evidence of a patent airway between the tongue base and posterior pharyngeal wall.47 The patient may require intravenous steroids if there is evidence of vocal cord edema. The extubation occurs in the operating room in the presence of the pediatric otolaryngology team in case of an unstable airway. After completion of the distraction protocol, a repeat CT scan is obtained to evaluate the newly expanded airway and to confirm device stability before discharging the patient to their home (Fig. 7, A and B). After the distraction is complete, the distractors remain in place for approximately 12 to 16 weeks to allow consolidation of the new bone. The device is removed in the operating room under general anesthesia after the consolidation phase is complete.

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Advocates for distraction state that the benefit of correcting the primary defect (micrognathia) results in fewer tracheostomies and improved feeding. Airway dimensions may be increased more significantly with MDO, and dependence on gastrostomy tube feeds may be reduced more with MDO.48 Scott et al.48,49 demonstrated that none of their patients with nonsyndromic PRS after MDO required tracheostomy, and only 7% required gastrostomy tube placement. This compares more favorably than Rogers et al.’s42 data with TLA that showed 7% tracheostomy rate in nonsyndromic PRS and 38% gastrostomy tube placement. Infants with syndromic PRS without neurologic impairment (hypotonia, developmental delay, and seizures) tend to do just as well as their nonsyndromic counterparts. However, syndromic PRS with neurologic deficits (hypotonia, developmental delay, and seizures) may have a higher rate of TLA or MDO failure and complications resulting in higher rates of tracheostomy and gastrostomy tube placement.48,49 In situations where the child is nonsyndromic, with normal pharyngeal tone and with a moderate overjet (<8 mm), TLA and MDO appear to have a similar rate of successful airway management (avoidance of tracheostomy), but MDO appears to be superior to TLA regarding feeding (avoiding gastrostomy tube placement). Mandibular distraction may be preferred in syndromic patients without neurologic impairment and large skeletal imbalance (overjet >8 mm). However, syndromic patients with neurologic impairment have a high rate of tracheostomy regardless of whether TLA or MDO is performed initially.42,49 These patients likely benefit from primary tracheostomy and gastrostomy tube placement with delayed distraction to facilitate early tracheostomy decannulation.

Complications from MDO include injury to the facial nerve, facial scars, distractor displacement, injury to teeth, failure of adequate distraction, and need for redistraction. Injury to the facial nerve has been reported in as many as 15% of patients, but this was usually temporary. Dental injury occurred primarily to the molars and this occurred in 21% of patients. Critics of MDO also express concerns regarding stability of long-term mandibular growth, cost, and the unknown effects on speech, swallowing, dental growth, and reflux.6,46,49

CT is repeated after completion of distraction, initiation of feeding, and then 3 months before removal of the device to reevaluate the airway space and confirm adequate bone generation in the distraction gap. Although modern distraction devices are MRI compatible, CT imaging is preferred due to better bone imaging when compared with MRI.

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Anesthesia Management

Patients with PRS require anesthesia for a variety of procedures including DLB, TLA, MDO, tracheostomy, radiologic procedures, gastrostomy tubes, and Nissen fundoplication. The clinical triad of PRS can present significant challenges for the anesthesia provider including airway obstruction and difficult intubation. This may result in intraoperative and postoperative respiratory complications.

The postoperative concerns occur secondary to airway obstruction. In addition, patients with PRS may be more opioid sensitive from chronic airway obstruction and hypoxia.50,51

Some patients presenting to the operating room with the clinical features of PRS may not have been previously diagnosed with PRS. The preoperative evaluation should identify these neonates and infants and any of their associated syndromes. It is important to recognize the degree of airway obstruction and the presence of OSA because OSA predicts intraoperative and postoperative respiratory complications. Patients who cannot tolerate supine positioning will be more difficult to mask ventilate and may require airway adjuncts such as oral pharyngeal airways, NPA, and laryngeal mask airways (LMAs).

As many as 60% of patients with PRS have an associated syndrome. The most common syndromes are Stickler, velocardiofacial, and Treacher-Collins syndrome. If these syndromes are suspected, a preoperative evaluation may also include echocardiography, especially if a murmur is heard. (Table 1 summarizes the anesthetic concerns related to some of the more common syndromes associated with PRS.)

In addition to micrognathia, the airway examination should identify any abnormalities of the craniofacial skeleton that may be associated with syndromic PRS. The mouth should be evaluated for the presence of a cleft palate. Glossoptosis may be visualized when the mouth is opened (Fig. 1). The physical examination should include room air oxygen saturation in addition to heart rate, respiratory rate, and arterial blood pressure. Patients with moderate to severe airway obstruction will demonstrate stridor, sternal and intercostal retractions, nasal flaring, paradoxical chest and abdominal motion, and oxygen desaturation when placed supine (Video 2, Supplemental Digital Content 2, http://links.lww.com/AA/A916). Mild obstruction is completely relieved with prone positioning or a jaw thrust. Patients with more severe obstruction may already have an NPA or endotracheal tube in place. Finally, if a heart murmur is noted, especially in the presence of a syndrome, a preoperative echocardiogram should be performed.

Intubation for patients with PRS may be performed using a variety of techniques. These techniques may be performed with no sedation, sedation, or general anesthesia. The hallmark for managing the known pediatric difficult airway under sedation and general anesthesia is to maintain spontaneous ventilation. Several airway techniques have been described to assist in the difficult airway for the patient with PRS. These include LMA, fiberoptic scope, retrograde wire, Glidescope,52 Shikani scope,53 Airtraq,54 and Air-Q scope. Some patients may present with such significant respiratory distress and fatigue that an LMA can be placed without anesthesia. Markakis et al.55 were the first to describe this in 1992. Asai et al.,56 in 2008, described a case series of 5 neonates (2.8–3.5 kg) with PRS requiring anesthesia. In all 5 patients, the LMA was placed without sedation or topical local anesthetic. All the patients became calm after the LMA was placed when the airway obstruction was relieved. Stricker et al.57 describe placing an awake LMA in PRS patients before inducing anesthesia. A fiberoptic scope was subsequently placed to facilitate endotracheal tube placement. General anesthesia may also be induced before securing the airway; however, maintaining spontaneous ventilation is essential and may be very difficult or impossible without airway adjuncts. The options to relieve the obstruction include obtaining help, 2-handed jaw thrust, oral pharyngeal airway, NPA, and LMA. If the airway remains obstructed despite these maneuvers, laryngoscopy should be performed for intubation. If intubation is not possible, an emergent bronchoscopy with a rigid bronchoscope by an otolaryngologist may secure the airway. Emergent tracheostomy is a final, although not ideal, rescue maneuver in the pediatric “cannot intubate, cannot ventilate” scenario. Extracorporeal membrane oxygenation may be an alternative rescue option if it is immediately available and if the patient weighs >2 kg and is older than 34 weeks gestational age.

Proper laryngoscopy skills are essential when intubating patients with micrognathia. Using a paraglossal approach may be more effective than standard laryngoscopy. This technique was described by Henderson58 in 1997 and then applied by Semjen et al.59 in 2008 in 6 patients with PRS. They successfully intubated 5 of the 6 PRS patients. The laryngoscope blade is placed in the right corner of the mouth and passed along the groove between the tongue and the right tonsil, using leftward and anterior pressure. The tongue is displaced to the left, and there should be no tongue hanging over the blade at any time. This technique reduces the distance to the glottic opening (Fig. 8). The space along the right side of the mouth is also reduced, and this makes passing an endotracheal tube more challenging. The authors used a gum elastic bougie to facilitate endotracheal intubation.

Figure 8
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If a fiberoptic scope is used, it can be placed orally through an LMA or nasally to visualize the glottic opening. When used nasally, an NPA can be placed in the opposite nares and used as a conduit to introduce volatile anesthetic and oxygen into the posterior pharynx of the spontaneously breathing patient. In smaller patients, the fiberoptic scope may be too large to allow the endotracheal tube to be loaded onto it. In these patients, the scope can be placed nasally to visualize the glottis while an orally placed endotracheal tube is passed into the glottic opening under direct fiberoptic visualization.60

There are other techniques that have been described to facilitate intubation in pediatric patients and they may prove to be useful in the patient with PRS. These techniques include video laryngoscopes (Storz videolaryngoscope, Glidescope) and ultrasound. There is 1 case report of an intubation with the Storz video laryngoscope in a 9-kg infant with PRS.61 The Glidescope Cobalt (Verathon), while it has not been described in the airway management of PRS, can be used in infants and neonates. Fiadjoe et al.62 recently compared the Glidescope with direct laryngoscopy in infants and neonates. Although none of these patients had craniofacial anomalies, the Glidescope was effective in patients younger than 1 year. Time to obtain best laryngoscopic view was faster with the Glidescope compared with direct laryngoscopy with a Miller 1 blade. However, the time to pass an endotracheal tube was slower with the Glidescope. The Glidescope has different disposable adapters and per the manufacturer may be used in neonates as small as 1.5 kg.

Patients presenting to the operating room after MDO may be significantly easier to mask ventilate and intubate. Frawley et al.63 described their experience with 51 PRS patients before and after MDO. Before distraction, the incidence of difficult intubation was 71%, and this decreased to 8.3% after distraction.

A variety of techniques can be used for anesthesia maintenance for patients with PRS. There is little evidence to support one technique over another. Volatile anesthetic is ubiquitous and safe and is likely the primary anesthetic used in most centers. Sevoflurane is one of the most commonly used anesthetic drugs used for pediatric patients but isoflurane could be used as well. Desflurane may be advantageous regarding emergence but it has some limitations that may preclude its use in pediatric PRS patients. Desflurane increases airway reactivity and should not be used in pediatric patients with bronchial hyperreactivity (asthma, upper respiratory tract infection, or bronchopulmonary dysplasia).64 In addition, the package insert for desflurane has changed and now indicates that it should not be used with an LMA in 2- to 6-year-old pediatric patients. There is an increased risk of laryngospasm, coughing, and secretions (suprane [desflurane] package insert). There is no information on patients younger than 2 years, but this population may also be at increased risk of airway complications if desflurane is used with an LMA.

In addition to a volatile drug, anesthesia maintenance can be supplemented with an opioid and an α-2 agonist such as dexmedetomidine. If intubation is continued into the postoperative period (TLA, MDO), a longer-acting opioid may be preferred over remifentanil. If extubation is planned (cleft lip, cleft palate), an α-2 agonist provides analgesia and may reduce opioid requirements. Likewise, some practitioners may prefer an ultra-short-acting opioid like remifentanil in this setting. PRS patients should be extubated awake, and an NPA can be placed before extubation to minimize postoperative airway obstruction. Figure 9 outlines an algorithm for the anesthesia management of PRS patients for TLA, MDO, or tracheostomy.

Figure 9
Figure 9
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Postoperative Concerns

The primary concern in the postoperative management of the patient with PRS is airway obstruction. Acute postoperative airway obstruction may result in hypoxia, negative pressure pulmonary edema, and death. Patients with PRS are particularly prone to postoperative respiratory complications from airway obstruction for several reasons including previous airway obstruction, opioid sensitivity, and surgically induced airway edema.

Chronic hypoxia from airway obstruction (OSA) increases opioid sensitivity if the obstruction is severe. Brown et al.50 have demonstrated that pediatric patients with severe OSA (oxygen saturation nadir <80%) are at increased risk of postoperative respiratory complications, and they require significantly less opioid after tonsillectomy. The mechanism may involve an upregulation of opioid receptors in the brainstem. Many patients with PRS have moderate to severe OSA preoperatively and may be at risk of opioid sensitivity and postoperative respiratory complications including airway obstruction, oxygen desaturation, and an escalation in postoperative respiratory care. Nonopioid analgesics should be maximized including local anesthetic (regional blocks or local infiltration), acetaminophen, and ketorolac. Opioid doses should be reduced, and patients should be observed in a monitored setting with pulse oximetry.

Surgery for the patient with PRS often involves the airway. These procedures include MDO, TLA, direct laryngoscopy, and cleft palate repair. In one study, Dell’Oste et al.65 described significant edema of the palate, tongue, and pharynx after cleft palate repair. This may have been related to the duration of the mouth retractor. There are several case reports of significant tongue edema after cleft palate surgery resulting in postoperative airway obstruction. This may result in difficult postoperative ventilation and intubation. Periodically (every 1 to 2 hours), the mouth retractor should be released to minimize the risk of postoperative tongue edema.

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CONCLUSIONS

The perioperative care of the neonate and infant with PRS involves input from many specialists. The primary concern is upper airway obstruction with subsequent respiratory distress and feeding difficulty. The later may manifest as reflux and failure to thrive. PRS comprises a heterogeneous group of patients. Some may present with an isolated mandibular abnormality, while other patients with PRS have associated anomalies or syndromes. The patient with mild airway obstruction can be managed conservatively with NPAs, prone positioning, and mechanical feeders. Moderate or severe obstruction requires more invasive interventions such as gastrostomy tube placement, TLA, MDO, and tracheostomy. Selecting the most appropriate surgical intervention continues to be dependent on the culture and expertise of the medical center. However, emerging data is helping to clarify appropriate treatment options.

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DISCLOSURES

Name: Franklyn Cladis, MD, FAAP.

Contribution: This author helped write the manuscript.

Attestation: Franklyn Cladis approved the final manuscript.

Conflicts of Interest: This author received research funding from Hospira.

Name: Anand Kumar, MD.

Contribution: This author helped write the manuscript.

Attestation: Anand Kumar approved the final manuscript.

Conflicts of Interest: The author has no conflicts of interest to declare.

Name: Lorelei Grunwaldt, MD.

Contribution: This author helped write the manuscript.

Attestation: Lorelei Grunwaldt approved the final manuscript.

Conflicts of Interest: The author has no conflicts of interest to declare.

Name: Todd Otteson, MD.

Contribution: This author helped write the manuscript.

Attestation: Todd Otteson approved the final manuscript.

Conflicts of Interest: The author has no conflicts of interest to declare.

Name: Matthew Ford, MS, CCC-SLP.

Contribution: This author helped write the manuscript.

Attestation: Matthew Ford approved the final manuscript.

Conflicts of Interest: The author has no conflicts of interest to declare.

Name: Joseph E. Losee, MD, FAAP.

Contribution: This author helped write the manuscript.

Attestation: Joseph E. Losee approved the final manuscript.

Conflicts of Interest: The author has no conflicts of interest to declare.

This manuscript was handled by: Peter J. Davis, MD.

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