History

The SLP should take a detailed history of the child’s voice concerns as well as voice use. In addition, the SLP will also take into account a thorough airway history, including previous intubation events, history of intubation difficulty, as well as history of tracheostomy. Understanding the patient’s voice history and the chronic versus intermittent nature of the dysphonia is essential. Given the occurrence of glottic webs in the population of children with 22q11.2 deletion, the SLP should elicit any syndromes or genetic evaluations in the medical history.

Quality of Life Measures

For children with less severe glottic webs, it is essential for the SLP to understand the impact of the voice disorder on the child and the family. Some families may not wish to pursue surgical intervention for less severe dysphonia. For children with congenital glottic webs, their dysphonia may be considered part of “who they are” by family members. Conversations regarding the risks and benefits of surgery to improve pitch or volume should occur as a team approach among the family, SLP, and otolaryngologist.

Laryngeal Visualization

Flexible laryngoscopy is often sufficient to identify the presence of a glottic web in most children. In the newborn, small, less-symptomatic webs may be difficult to identify, and close outpatient follow-up is thus recommended in the first weeks of life. Laryngostroboscopy may be used frequently in children over 8 years of age and in some children as young as 4 years of age who are able to participate. Stroboscopic measurements such as mucosal wave and closure pattern can be noted outside of the affected area of the glottic web in Type I and Type II classifications. Laryngeal hyperfunction can be identified on laryngostroboscopy and serve to direct therapy prior to possible surgical intervention for those children who are candidates for behavioral intervention, based on developmental ability and age.

One case study in the literature describes a 5-year-old female who presented with complaints of a high-pitched voice and chronic dysphonia with previously undiagnosed anterior Type II glottic web. She demonstrated laryngeal hyperfunction and lack of mucosal wave within the web on laryngostroboscopy [8]. Postsurgical perceptual and acoustic measurements indicated improvement in pitch range and normalization of her perceptual habitual speaking pitch [8].

In addition to laryngoscopy to identify possible glottic web, patients who present with hypernasal resonance secondary to 22q11.2 deletion syndrome may also undergo nasopharyngoscopy to evaluate palatal movement, depending on their ability to produce connected speech during that procedure.

Treatment

Patients who present with Type I or Type II glottic web and laryngeal hyperfunction may benefit from a trial of voice therapy using one or more of the voice therapy approaches outlined in this text. When considering surgical intervention, voice therapy to “unload” the hyperfunction may help the child understand their compensatory voice patterns and achieve improved voice outcomes after surgery.

Perioperative Voice Therapy

While there is little documented regarding pediatric postoperative voice outcomes for patients with congenital glottic web, children who present with laryngeal hyperfunction secondary to Type I or Type II glottic web will benefit from continued therapy postoperatively to unload the hyperfunction. Given the high percentage of patients who present with voice symptoms at birth and shortly thereafter, the majority of children who require surgical intervention will undergo a procedure at a young age . These children should return for intermittent postoperative voice evaluations based on their age and ability to participate in assessment. In our experience, children who present with more severe glottic webs and extensive subglottic obstruction will often continue to use supraglottic or mixed glottic and supraglottic phonation postoperatively as their most effective means of communication. Laryngoscopy and voice assessment in conjunction with otolaryngology will determine if the patient is able to produce pure glottic phonation. In these cases, stimulability during assessment will guide therapy recommendations.

History

Congenital glottic webs often present with abnormal cry, biphasic stridor, and respiratory distress in infancy or early childhood. It is estimated that 75% of patients with symptomatic congenital glottic webs will present at birth and nearly all by 1 year of age. The most common presenting symptom is an abnormal cry, which may range from high pitched to deep or even absent. As the infant grows and glottic airflow increases, respiratory manifestations such as stridor and retractions may become apparent. The degree of respiratory distress is grossly correlative to the degree of glottic and subglottic obstruction.

Acquired glottic webs present similarly, but the course is variable in progression and symptoms occur following some airway insult. Both congenital and acquired webs can present with dysphonia, dyspnea, and recurrent respiratory infections secondary to impaired airway clearance. At times, glottic webs may present only after a second concomitant laryngeal insult such as infection or laryngopharyngeal reflux. Webs may also be discovered at the time of endotracheal intubation, where an unanticipated glottic web can lead to difficult airway management.

In the non-emergent setting, obtaining a history should include the perception of the child and parents. Evaluation in a multidisciplinary Aerodigestive Center is optimal for concerted evaluation and management preoperatively and to optimize reconstructive outcomes postoperatively.

Exam

A general head and neck exam is performed. In addition, several aspects of an extended physical exam are included for the patient presenting with congenital anterior glottic web as these may be associated with chromosome 22q11.1 deletion syndromes: velopharyngeal insufficiency, cleft palate, otitis media, dysphagia, cardiovascular malformations, behavioral and developmental disabilities, and renal anomalies. As described by Miyamoto et al., anterior laryngeal webs may be the only indication of velocardiofacial syndrome, and it is reasonable to refer all children with anterior glottic webs for genetic evaluation [3].

Cardiopulmonary exam is essential including auscultation and evaluation for extremity clubbing (in patients presenting outside of infancy). If the child is able, pulmonary function tests may also be conducted to characterize the baseline degree of extrathoracic airway obstruction. There may be a flattening of both the inspiratory and expiratory portions of the flow-volume loop if the glottic web impairs respiratory flow.

Occasionally, glottic webs are diagnosed at the time of difficult intubation as an emergent consult to the otolaryngologist. In these instances, it is reasonable to maintain spontaneous ventilation while performing microdirect laryngoscopy and bronchoscopy. The otolaryngologist should have rigid and fiber-optic intubation equipment and smaller-than-anticipated endotracheal tubes on hand. Consideration of a laryngeal mask airway as a rescue device can also be considered, depending on the nature of the glottic web. Lastly, tracheostomy may be necessary to secure the airway if the glottic and subglottic airway will not permit passage of an endotracheal tube.

Instrumented Assessment

In the office setting, videolaryngostroboscopy, perceptual measures, acoustic measures, aerodynamic measures, and quality of life questionnaires, such as the Pediatric Voice Handicap Index, should be completed. Videostroboscopic examination should be carried out for voice and anatomic evaluation. Rigid laryngoscopy may be completed with a 70-degree laryngoscope if patient age and cooperation allow. The standard stroboscopic protocol, including vocal tasks such as habitual pitch production, pitch glide, and respiratory tasks, is completed and recorded. Essentially, a glottic web will shorten the vibratory margin of the true vocal folds which can lead to higher pitch. Laryngeal hyperfunction and posterior glottic flaring may also be evident [8]. The mucosal wave and amplitude may be difficult to delineate, especially in thick glottic webs. Because many children will not cooperate with rigid videostroboscopy, endoscopic flexible stroboscopy is an acceptable alternative. During flexible transnasal laryngoscopy (with or without stroboscopy), attention is also paid to any evidence of velopharyngeal insufficiency (VPI) via nasopharyngoscopy. VPI has been reported to be concurrent with glottic web in patients with 22q11.2 deletion syndromes. VPI may result in hypernasal resonance despite adequate treatment of the glottic web.

Operative endoscopy with microdirect laryngoscopy and bronchoscopy is essential to diagnose and fully characterize the glottic web and is ideally done with both zero-degree and angled rigid telescopes as a staging procedure prior to deciding upon further treatment. This affords evaluation of web thickness, involvement of the conus elasticus and subglottis, and Cohen classification grade. A right-angled probe is used to evaluate for concurrent laryngeal cleft and can also be used to palpate behind the anterior aspect of the web to assess the cartilaginous component within the subglottic lumen. The arytenoids are palpated to ensure normal joint mobility, with the caveat that advanced glottic webs may limit arytenoid movement. Nonetheless, normal posterior glottis has been proposed as a favorable factor for endoscopic management of anterior glottic webs [6]. A comprehensive airway evaluation is essential so as to identify concurrent pathologies such as subglottic stenosis, tracheoesophageal fistula, tracheomalacia, and laryngeal cleft. Airway sizing should also be performed in this setting, and information regarding recommended airway management should be given to the parents and documented in the medical record. Photodocumentation is strongly encouraged at each operative evaluation and intervention. Of course, if the child is experiencing respiratory failure or severe distress, securing the airway takes precedent over staging the glottic web which can be conducted at a later time.

Differential Diagnosis

The differential diagnosis for glottic webs most commonly includes subglottic stenosis in those patients presenting with stridor or respiratory insufficiency. Subglottic stenosis may also be present as part of the inferior extent of thick anterior glottic webs. More severe forms of laryngeal atresia are also included in the differential diagnosis if suggested by the clinical presentation. Patients with cri-du-chat syndrome may have similar vocal presentations to children with an anterior glottic web.

Management

Proper management of glottic webs rests on making an accurate diagnosis and fully characterizing the lesion. An estimated 60% of congenital glottis webs require operative intervention. From an operative standpoint, both open and endoscopic management techniques have been described. Historically, the gold standard for operative management included an anterior thyrotomy and tracheostomy. However, in 1991, Lichtenberger developed an endo-extralaryngeal needle holder which afforded the placement of a silicone keel into the anterior glottis after lysis of the web while obviating the need for tracheostomy [9]. Currently, the decision for open versus endoscopic techniques rests largely on the characteristics of the web and surgeon preference. The extent of reconstruction is largely determined by the subglottic characteristic of the web. Current literature suggests that the web thickness, specifically with subglottic extent, may be the most important consideration in choosing operative technique. Grossly, thinner webs need less invasive surgical technique. Specifically, thin glottic webs may respond well to simple endoscopic lysis with or without adjunctive dilation or microsurgical mucosal flap procedures [10, 11], whereas uniformly thick webs with subglottic extension will often require more advanced techniques such as laryngotracheal reconstruction with stenting or laryngofissure with keel placement (Fig. 32.4) [12, 13]. Such webs are most often seen in Cohen Types III (Fig. 32.5) and IV (Fig. 32.6); however, glottic extension is not always correlative with subglottic extension. In those webs with a thick subglottic “sail,” the laryngeal surface of the anterior cricoid cartilage is the extent of web incision, and therefore, an anterior or anterior/posterior graft is often needed to address the respiratory symptoms and decrease relapse. Treatment of large glottic webs with significant subglottic extension by laryngotracheal reconstruction in the neonatal period is controversial. While some authors advocate early intervention, others maintain that definitive surgical correction should be undertaken at a later age [5, 14–16]. Considerations including patient comorbidities, swallowing dysfunction, and pulmonary status must always be considered. For that reason, it is the opinion of the authors that patient-specific characteristics are the most important consideration when determining the age of repair.

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