Evaluation

Subjective Evaluation

Objective Assessment

Treatment

Voice therapy to improve hoarseness, resonance, and respiratory patterns and decrease hyperfunctional behavior patterns has been shown to be effective in individuals with benign vocal fold lesions [29, 30]. Although RRP does not have the same pathophysiology as vocal cord nodules and polyps, similar voice parameters are negatively affected, and in some cases this allows for a similar therapeutic approach. A systematic review by Desjardin et al. identified indirect and direct treatment approaches that lead to significant improvement in at least one outcome measure (self-assessment, perceptual judgment, acoustic analysis) [31]. While Jani et al. revealed that a combination of direct and indirect voice therapy would be the best intervention for functional dysphonia rather than no therapy, Speyer et al. found that direct voice therapy resulted in improved outcomes compared to indirect voice therapy [32, 33]. A review of the literature on pediatric voice therapy, as well as a discussion of therapy approaches, is included in Chap. 21 (Voice Therapy). Studies of voice therapy in children have not specifically included patients with RRP, likely due to the variable and fluctuating nature of the disease process, especially in children.

Indirect therapy provides parent and child education about current anatomy and physiology of voice, individualized instruction on internal and external vocal hygiene, recognizing vocal danger zones to monitor speaking in loud environments, and emotionally charged situations due to potential use of loud phonation without conscious technique. Recommendations for amplification to reduce vocal effort and loudness required when speaking in noisy environments can also be made to parents. Using stretches or relaxation to decrease compensatory muscle tension in the head and neck and upper torso can also be beneficial if this is present [34].

Direct voice therapy for children with RRP focuses on decreasing hoarseness from two separate potential causes: an increase in mass of vocal folds from papillomas, interfering with appropriate closure and entrained vibration, and reduced pliability and incomplete closure due to scarring from repeated procedures. As a result, children often compensate by speaking louder (with more force), causing increased effort and strain or compensatory extrinsic muscle activation. Decreasing these hyperfunctional behavior patterns is also addressed in voice therapy to decrease tension in the head and neck and respiratory musculature, as well as optimize vibration of the vocal folds, respiratory support, and use of forward resonance.

Non-conversational techniques promote vocal fold tissue healing from behavioral or surgical trauma, efficient vocal fold vibratory patterns, and increased anterior sensory awareness of voice production. The use of semi-occluded tract postures (straw phonation, vocal function exercises, basic training gestures, tongue trills) and resonant voice therapy has been found to be effective to improve perceptual voice quality and overall efficiency of production [35, 36]. Conversational techniques including Lessac-Madsen Resonant Voice Therapy (LMVRT), Casper-Stone Confidential Voice Therapy (CSCVT), Stretch and Flow Phonation, and Accent Method can be helpful in bridging the gap to connected speech [34]. Specific to the treatment of dyspnea, inspiratory muscle strength training was found, in a single subject, to reduce the perception of dyspnea [37].

In providing voice therapy for children with RRP, a particular challenge is the recurrent nature of the disease and the resultant fluctuating voice quality depending on extent of disease and effects of recent or remote surgeries. Given the sometimes rapid progression, we may be dealing with different laryngeal structure and function at each therapy visit. For example, a child with bulky disease may not be able to achieve entrained vibration for phonation and may instead need to focus on compensatory strategies, including amplification, to communicate effectively. Conversely, a child who is several weeks post-surgery may need to focus on improving respiratory/phonatory coordination and forward resonance to achieve their best voice. Another child may have extensive scarring from repeated procedures and may benefit from work on forward resonance and balanced respiratory support to optimize the vibration they can get. Clinicians need to consistently reassess function as well as stimulability for change in each session. While challenging, this should not be given up on, as voice therapy can be extremely helpful in improving a child’s communicative function and quality of life.

History

A thorough history involving both the parent and the patient should be obtained. Persistent or progressive stridor and dysphonia, with the possible development of respiratory distress, are the most consistent signs and symptoms of RRP in children. Focused questions regarding the time of onset of symptoms, possible airway trauma including a history of previous intubations, and characteristics and quality of the cry or voice changes are important. In most pediatrics series, the time from onset of symptoms to diagnosis of RRP is approximately 1 year [2, 38], although the duration of symptoms before diagnosis varies. The vocal fold is usually the first and predominant site of papillomatous lesions resulting in hoarseness being the principal presenting symptom [39]. Stridor is often the second clinical symptom to develop, initially inspiratory, then becoming biphasic. Less common symptoms include chronic cough, recurrent pneumonia, failure to thrive, dyspnea, dysphagia, or acute respiratory distress. Not uncommonly, a diagnosis of asthma, croup, allergies, vocal nodules, or bronchitis is entertained before a definitive diagnosis is made.

The natural history of RRP is highly variable and unpredictable. The disease may undergo spontaneous remission, persist in a stable state requiring only periodic surgical treatment, or may be aggressive, requiring surgical treatment every few days to weeks and consideration of adjuvant medical therapy.

Exam

Children who present with a history consistent with RRP must undergo a comprehensive physical examination. First, clinical signs of respiratory distress including rapid respiratory rate, degree of distress, signs of fatigue, nasal flaring, and use of accessory muscles must be quickly assessed. If the child appears to be in severe respiratory distress, additional examination should be delayed, and the patient should be transferred immediately to either the operating room (OR), emergency department, or pediatric intensive care unit (PICU) depending on the level of severity. In the stable, well-oxygenated child, additional examination can be carried out. Care should be taken to listen closely to the child’s voice and/or cry as well as begin to characterize the child’s stridor and its relationship to the respiratory cycle. Using a stethoscope to auscultate over the nose, mouth, neck, and chest can localize the location of upper airway obstruction. A child with RRP would not be expected to demonstrate much change in the stridor with position change, in contrast to infants with laryngomalacia, a vascular ring, or a mediastinal mass.

Instrumented Assessment

The diagnosis of RRP is best made with flexible fiberoptic nasopharyngoscopy. Systematic inspection of the pharynx, hypopharynx, and larynx including the subglottis provides critical information necessary for the diagnosis and can assist with preoperative planning. Evaluating airway lumen size and vocal fold mobility helps to determine the urgency of operative intervention. Although dynamic evaluation can be performed when children are spontaneously breathing, endoscopy in the OR under anesthesia is warranted in any child suspected to have RRP who cannot be fully examined in the outpatient setting [40].

Staging systems are helpful in tracking disease progression in individuals and communicating with other professionals. Derkay et al. created a numeric scoring system for evaluating clinical and anatomic disease severity [41, 42]. Clinical symptoms of dysphonia, stridor, intervention urgency, and respiratory distress are scored. Anatomic subsites of the larynx (lingual epiglottis, laryngeal epiglottis, anterior commissure, aryepiglottic folds, false vocal folds, ventricle, true vocal folds, arytenoids, and posterior commissure) are graded on a scale of 0–3, where 0 is none, 1 is surface lesion, 2 is raised lesion, and 3 is bulky lesion. A final numeric score is calculated to determine the extent of disease at each assessment (Figs. 31.1 and 31.2). Airway endoscopy is crucial to determine the full extent of disease and monitor treatment response.

Differential Diagnosis

As stridor and dysphonia are the most common presenting symptoms, differential diagnosis for RRP is broad including benign laryngeal or tracheal tumors, malignant laryngeal or tracheal tumors, foreign body aspiration, gastroesophageal reflux disease, laryngitis, subglottic stenosis, tracheomalacia, vocal fold dysfunction, and vocal fold paralysis.

Management

At present, there is no cure for RRP, and no single treatment has consistently been shown to be effective in eradicating RRP. The current standard of care is surgical intervention with a goal of complete removal of papilloma with preservation of normal structures. In patients with disease burden in sensitive locations, including the anterior or posterior commissure, or highly aggressive disease, the overall goal is removal of sufficient disease to clear the airway while preserving normal structures and avoiding complications of glottic or subglottic stenosis and webbing.

When RRP presents with severe respiratory distress caused by papilloma obstructing the airway, tracheostomy may need to be performed. It has been suggested that tracheostomy may activate or contribute to the spread of disease lower in the respiratory tract [43]. As a result, most otolaryngologists agree that a tracheostomy is a procedure to be avoided unless absolutely necessary, and, when tracheostomy is unavoidable, decannulation should be considered as soon as the disease has been managed effectively.

The carbon dioxide (CO₂) laser is now preferred by some over previously used cold instrumentation for removal of RRP involving the upper airway [44]. The use of the CO₂ laser with an operating microscope has gained popularity due to its excellent precision with minimal bleeding. Multiple procedures performed over time are recommended to avoid tracheostomy and allow for optimal phonation with preservation of normal laryngeal anatomy.

In addition to the CO₂ laser , the potassium titanyl phosphate (KTP) laser can also be used [45]. The 532-nm wavelength selectively targets hemoglobin and coagulates the vascular supply to the papilloma. This allows for spontaneous involution of the lesions postoperatively and decreases the probability of scar or web formation that may occur with complete lesion removal [45]. A recent study demonstrated that HPV DNA was not present on the laser fiber after the procedure, decreasing some concern for potential transfer of disease from patient to surgeon [46].

While there are many advantages to the laser in patients with RRP, the drawbacks relate primarily to safety. Although uncommon, the laser beam may reflect off nearby metal resulting in potential injury to the surgeon or areas of the patient that are not protected by a wet towel. Additionally, the laser smoke or “plume” has been found to contain active viral DNA, a potential source of infection [47–49]. The most dreaded safety concern is that the laser beam generates heat that, if the beam inadvertently strikes the endotracheal tube in the oxygen-rich environment, could lead to an explosion or fire in the airway. Employing standard laser safety precautions can help mitigate these potential risks. First, all team members in the room should wear eye protection, and the patient’s face and shoulders should be covered with wet towels. Clear closed loop communication should be performed between the surgeon and assistant who is operating the laser, specifying “laser on” and “laser on standby” when warranted. Oxygen should be kept at 30%, and a laser-safe endotracheal tube should be used.

An alternative technology which can be incorporated in the therapeutic regimen for pediatric RRP is the microdebrider. Initially adapted from the sinus microdebrider, the laryngeal microdebrider is effective in removing bulky, exophytic papillomatosis disease with reduced postoperative pain scores, improved voice quality, shorter procedure time, and decreased procedure cost compared to the CO₂ laser [50]. Although uncommon, significant complications including major vocal fold scar, airway compromise, severe hemorrhage, and unintentional tissue loss have occurred [51].

Indications

In pediatric RRP, the normal airway lumen is inherently small and thus maintaining adequate airway patency is of the utmost importance . For this reason, parent education regarding early signs of airway obstruction as well as compliance with regular clinical and endoscopic monitoring is crucial. A key management principle in RRP is to focus attention and effort on preventing the need for a tracheostomy. Indications for surgical intervention include any voice, swallowing, or airway symptoms.

Key Aspects of the Consent Process

Risks associated with microdirect laryngoscopy should be discussed, including injury to the lips, gums, tongue, and teeth, as well as potential for transient or longer-lasting dysgeusia. Voice or swallow function could worsen or simply fail to improve. It may not be possible or safe to remove all disease, which may predispose to earlier recurrence. Operating on the airway carries an inherent risk of airway edema which may require overnight observation, temporary placement of an endotracheal tube, or, in rare cases, tracheotomy. Particularly relevant for papilloma is the risk of inducing a web, which is avoided by not treating the medial surface of both vocal folds at the same time.

Equipment

An operating laryngoscope with its associated suspension system is utilized for optimal exposure. Both rigid 0- and 70-degree Hopkins rod telescopes are used for intraoperative exam and photodocumentation. An operating microscope is used for the microsurgical portion. A full set of microlaryngeal instruments should be available. An ENT Microdebrider with laryngeal blade attachment is needed.

Steps