1

Introduction

Pediatric OSAS and craniofacial malformations present challenges that require innovative approaches and comprehensive treatment strategies. Synchronous airway lesions, craniofacial malformations, obstructive anomalies of the tongue base, nasal vault and choanae are commonly addressed by subspecialists from various clinical and surgical academic traditions who practice variable levels of required communication. This is not a mere social requirement but an important requisite for intelligent and effective airway management. Membership of dedicated airway, aero digestive or craniofacial teams are desirable but not required. I expect this clinical brief to help many brilliant clinicians in their pursuit of perfection.

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Synchronous airway lesions (SALs)

Craniofacial anomalies, syndromic or not, usually present with predictable sites of obstruction apparent from clinical exam and respiratory behavior during the first few days of life. In practice however, up to 15% of children with craniofacial anomalies presenting with respiratory distress have multiple airway problems that need to be addressed in order to achieve satisfactory results . The false belief in the predictability of a ‘single’ problem ‘single site’ is likely showing up in reports of varying success levels addressing ‘single site’ or ‘single’ problems.

In today’s medical environment, many surgical subspecialties are competitively involved with the treatment of neonatal and pediatric airway obstruction (ENT, pediatric ENT, OMFS, pediatric surgeons, plastic surgery etc.) with different skill sets and procedural competencies that should best be complementary to each other. It is important to have a skilled specialist trained in the evaluation and treatment of the entire airway, since a comprehensive, dynamic airway evaluation under general anesthesia is essential before treatment plans are made.

Reports from children’s centers in Pittsburgh and Chicago have been revealing; synchronous airway lesions (SALs) are diagnosed in more than 50% of otherwise normal children scheduled for adenoidectomy to treat upper airway obstruction causing OSA. These children, all under 18 months of age, were diagnosed with a variety of airway anomalies including laryngeal edema, laryngomalacia, tracheal vascular compression, subglottic stenosis, vocal fold lesions, bronchial stenosis, and vocal cord paralysis. Of these, 30% had laryngopharyngeal reflux disease and 13% also had eosinophilic esophagitis diagnosed with esophageal biopsy . Similar findings including tracheal cobblestoning (usually considered to be a sign of aspiration and/or reflux), subglottic stenosis, laryngomalacia, tracheobronchomalacia, external vascular compression, subglottic cysts, laryngeal clefts, vocal cord paresis, subglottic nodule and trapped first tracheal ring were reported in 67% of children up to 3 years of age who were scheduled to undergo adenotonsillectomy for OSA. When tracheal cobblestoning, the most frequent finding, was removed from the analysis, 65% of children in the series still had at least one SAL. Moreover, the severity of OSA as measured by PSG did not predict the presence of an SAL .

In 27% of a heterogeneous group of children with laryngomalacia, airway evaluation helped diagnose epiglottic and vallecular cysts, subglottic stenosis, laryngotracheal cleft, tracheomalacia and vocal cord dysfunction .

When a known syndromic association with an airway anomaly was investigated such as the 22q11 deletion syndrome, subglottic stenosis, glottic web, vocal nodules, laryngomalacia, laryngeal paralysis and bronchial malposition was found in 14% of the series . The most commonly known association of a syndrome with airway compromise is in children with Down syndrome: multiple anomalies of the upper airway including large retro-positioned tongue, small anteroposterior craniofacial distances, short neck, poor muscle tone and, sometimes a cleft palate accompanies a high incidence of SALs including laryngomalacia, tracheal bronchus, subglottic stenosis, tracheomalacia in up to 70% of children evaluated ,

The examples can be extended to include the majority of craniofacial syndromes as well as otherwise healthy children with respiratory compromise. The take home message is to never rely on initial, subjective clinical presentations of OSA or other respiratory problems. The severity of PSG abnormalities even when performed in extremely competent sleep centers, is not predictive of the presence or severity of SALs. Treatment should not be planned without a detailed airway evaluation including flexible laryngeal, nasal and nasopharyngeal endoscopy and rigid bronchoscopy. (Routine esophagoscopy and biopsy during airway evaluation is low yield and should be performed only if eosinophilic esophagitis is suspected). The treatment of airway problems should be coordinated between often competing surgical subspecialties to include a comprehensive airway evaluation.

2

Synchronous airway lesions (SALs)

Craniofacial anomalies, syndromic or not, usually present with predictable sites of obstruction apparent from clinical exam and respiratory behavior during the first few days of life. In practice however, up to 15% of children with craniofacial anomalies presenting with respiratory distress have multiple airway problems that need to be addressed in order to achieve satisfactory results . The false belief in the predictability of a ‘single’ problem ‘single site’ is likely showing up in reports of varying success levels addressing ‘single site’ or ‘single’ problems.

In today’s medical environment, many surgical subspecialties are competitively involved with the treatment of neonatal and pediatric airway obstruction (ENT, pediatric ENT, OMFS, pediatric surgeons, plastic surgery etc.) with different skill sets and procedural competencies that should best be complementary to each other. It is important to have a skilled specialist trained in the evaluation and treatment of the entire airway, since a comprehensive, dynamic airway evaluation under general anesthesia is essential before treatment plans are made.

Reports from children’s centers in Pittsburgh and Chicago have been revealing; synchronous airway lesions (SALs) are diagnosed in more than 50% of otherwise normal children scheduled for adenoidectomy to treat upper airway obstruction causing OSA. These children, all under 18 months of age, were diagnosed with a variety of airway anomalies including laryngeal edema, laryngomalacia, tracheal vascular compression, subglottic stenosis, vocal fold lesions, bronchial stenosis, and vocal cord paralysis. Of these, 30% had laryngopharyngeal reflux disease and 13% also had eosinophilic esophagitis diagnosed with esophageal biopsy . Similar findings including tracheal cobblestoning (usually considered to be a sign of aspiration and/or reflux), subglottic stenosis, laryngomalacia, tracheobronchomalacia, external vascular compression, subglottic cysts, laryngeal clefts, vocal cord paresis, subglottic nodule and trapped first tracheal ring were reported in 67% of children up to 3 years of age who were scheduled to undergo adenotonsillectomy for OSA. When tracheal cobblestoning, the most frequent finding, was removed from the analysis, 65% of children in the series still had at least one SAL. Moreover, the severity of OSA as measured by PSG did not predict the presence of an SAL .

In 27% of a heterogeneous group of children with laryngomalacia, airway evaluation helped diagnose epiglottic and vallecular cysts, subglottic stenosis, laryngotracheal cleft, tracheomalacia and vocal cord dysfunction .

When a known syndromic association with an airway anomaly was investigated such as the 22q11 deletion syndrome, subglottic stenosis, glottic web, vocal nodules, laryngomalacia, laryngeal paralysis and bronchial malposition was found in 14% of the series . The most commonly known association of a syndrome with airway compromise is in children with Down syndrome: multiple anomalies of the upper airway including large retro-positioned tongue, small anteroposterior craniofacial distances, short neck, poor muscle tone and, sometimes a cleft palate accompanies a high incidence of SALs including laryngomalacia, tracheal bronchus, subglottic stenosis, tracheomalacia in up to 70% of children evaluated ,

The examples can be extended to include the majority of craniofacial syndromes as well as otherwise healthy children with respiratory compromise. The take home message is to never rely on initial, subjective clinical presentations of OSA or other respiratory problems. The severity of PSG abnormalities even when performed in extremely competent sleep centers, is not predictive of the presence or severity of SALs. Treatment should not be planned without a detailed airway evaluation including flexible laryngeal, nasal and nasopharyngeal endoscopy and rigid bronchoscopy. (Routine esophagoscopy and biopsy during airway evaluation is low yield and should be performed only if eosinophilic esophagitis is suspected). The treatment of airway problems should be coordinated between often competing surgical subspecialties to include a comprehensive airway evaluation.

3

Tongue-lip adhesion for the treatment of OSAS in craniofacial malformations and mandibular glossopexy (MGP)

Craniofacial malformations including anomalies of the size, position and geometry of the mandible usually are associated with anomalies of the size and position of the tongue as well as primary or associated deficiencies of motor control and muscle tone. The obstruction caused by these anomalies is evident in the first days of life and has to be managed with primacy. Among the techniques used, the tongue–lip adhesion (TLA) procedure has as many proponents as it has opponents. The disagreements over the effectiveness of the procedure between accomplished surgeons can be traced to semantics.

When this procedure is performed as a soft tissue adhesion between the lower lip and the tongue (tip and/or floor of mouth) as the semantics suggest, the results are unsatisfactory. Many surgeons who have reviewed and used such interventions have concluded (sarcastically) that the TLA only works in children who do not need them. More than 80% of institutions have abandoned standard TLA with or without sub periosteal release citing morbidities caused by tethering the tongue. (Exacerbation of dysphagia, prolonged nasogastric tube feeding, gastrostomy tube placement, second surgery for release of the adhesion). The concept and justification of sub periosteal release also is problematic.

The reason for the failure of the simple soft tissue adhesion TLA in relieving the obstruction is its inability to control the tongue base. Soft tissue flaps between the tongue and the lower lip will inevitably stretch and then relax, even if at the time of surgery, relatively adequate forward pull of the tongue base is achieved/observed. When TLA is modified to include a sturdy loop-stitch around the base of tongue that is anchored anteriorly to the mandible, excluding soft tissue flaps between the tongue and lower lip, the technique helps achieve remarkable results. The efficiency of a commercially available kit for the treatment of OSAS in adults (AIRvance ™ formerly known as Repose ® – Medtronic Inc., Minneapolis, Minnesota) is based on the same principles of tongue base repositioning and mandibular anchoring. The least complicated technique involves a 2.0 nylon suture inserted around the tongue base and both ends are threaded under the tongue through the floor of the mouth, (avoiding submandibular ducts and vessels) and looped around or through the mandible briefly surfacing in the gingivolabial sulcus, to be tied together and buried in the FOM or gingival sulcus anteriorly. Threading the suture through the mandible is done with an 18G needle close to mandibular rim (avoiding tooth germs and neurovascular bundles). In the first few weeks of life, going through the mandibular bone with a sharp needle is possible without the use of drills. This is a permanent stitch and will hold the tongue base forward with a mild protrusion of the tongue. The tongue protrusion usually resolves within weeks but the tongue base stays in its anterior position. There are variations of the technique; some surgeons continue to perform the tongue–lip mucosal flaps in addition to the tongue base stitch, but in this author’s opinion, this is not necessary and creates a cosmetic disruption as well as the additional morbidities described previously. Mann et al. have described the tongue base stitch in a weaving pattern . However, in an infant, this proves difficult and in our experience, does not provide better control of the tongue base when compared with a well-placed single (depth 2–3 mm) tongue base suture. Adding a trans-mandibular K-wire to a soft tissue TLA also does not seem to provide adequate tongue base mobilization . Argamaso describes a variation of standard TLA using a circummandibular suture to anchor the anterior tongue to the mandible , which also deviates from the principle of adequate and stable tongue base control. The simple tongue base suspension altogether helps avoid the pitfalls of the standard TLA and should ideally not be referred to as a TLA. We need a better term to describe tongue-base suspension with a soft tissue-to-bone anchor. I propose using the term mandibular glossopexy (MGP). Successful MGP as described, provides immediate and lasting relief from tongue base obstruction, does not exacerbate or cause dysphagia, and is well tolerated by the baby who will need additional positioning and feeding support. If severe neurological compromise is present, or aspiration is a major problem, gastrostomy tubes and tracheotomy should be considered instead of MGP.