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Disorders of phonation in children
Pediatr Clin N Am 50 (2003) 363 – 380
Disorders of phonation in children J. Scott McMurray, MD, FAAP, FACS Division of Otolaryngology, Department of Surgery, Department of Pediatrics, University of Wisconsin Medical School, K4-766 CSC, 600 Highland Avenue, Madison, WI 53792, USA
Phonation is defined as the utterance of vocal sounds. Disorders of phonation, therefore, can be caused by a wide variety of abnormalities along the aerodigestive tract, including laryngeal pathology, velopharyngeal problems, and even cognitive delays that can inhibit a person from making intelligible vocal sounds. This article concentrates on the evaluation and management of common laryngeal abnormalities seen in children with disorders of phonation. Much of this information also has been presented in a previous issue of the Otolaryngologic Clinics of North America [1]. It has been estimated that over one million children suffer from dysphonia nationwide. Dysphonia can be detrimental to children both psychosocially and academically. Fortunately most causes of dysphonia or hoarseness in children are benign and generally are easy to treat. A multidisciplinary approach to the diagnosis and treatment of pediatric voice disorders is essential. The evaluation and treatment of voice problems is important for several reasons. Some voice disorders are potentially life threatening. Dysphonia—such as that seen from recurrent laryngeal papillomata—can be accompanied by lifethreatening airway obstruction. Dysphonic children also may face perceptual and psychosocial problems. In one study [2], men and women majoring in business, education, and economics were asked to listen to the speech of voice-disordered children and normal-speaking children. Without visually seeing the children, listeners’ judged the voice-disordered children as ‘‘dirty, cruel, ugly, unfair, small, weak, slow, clumsy, or sick’’ more often than they did the normal-speaking children [2]. Additionally, children with disorders of phonation often experience prejudice from their peers and teachers. Therefore, it is important that dysphonic children be referred to a team of professionals capable of providing comprehensive evaluation and management.
E-mail address: [email protected] 0031-3955/03/$ – see front matter D 2003, Elsevier Inc. All rights reserved. doi:10.1016/S0031-3955(03)00028-2
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Evaluation An evaluation for pediatric dysphonia may consist of all or part of the following:
History taking Physical examination Indirect functional laryngoscopy Voice evaluation Radiographic evaluation pH probe Direct laryngoscopy, bronchoscopy, and esophagoscopy Diagnosis or decision making Treatment Surgical Medical Speech therapy
History As with any medical disorder, a complete and methodic history is essential in the evaluation of pediatric dysphonia. Fine points recorded during the history may help to narrow the differential diagnosis. The causes of pediatric dysphonia may be classified as infectious, anatomic, congenital, inflammatory, neoplastic, neurologic, and iatrogenic. It is important to determine the time of onset of the voice disorder as well as its duration and chronicity, to help direct the decisionmaking process. The time of onset of dysphonia in relation to birth can help to separate the causes of dysphonia into general categories (Table 1). Many congenital causes of dysphonia are apparent at birth, although some may not present until later. Neurologic causes generally present at birth or at the time of the presentation of the neurologic condition. Iatrogenic causes usually have an antecedent event that may predate the dysphonia by several months, such as subglottic stenosis that develops after endotracheal intubation. Infectious causes (except for laryngeal papillomata) generally have associated systemic signs of illness. Voice strain or Table 1 Differences in the typical onset of dysphonia, by general categories Dysphonia presenting at birth
Dysphonia presenting after birth
Congenital Neurologic
Anatomic Neurologic Neoplastic Inflammatory Infectious Iatrogenic
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aggressive voice use usually is associated with anatomic causes for hoarseness such as vocal nodules. Inflammatory causes for dysphonia, such as reflux laryngitis, may be otherwise silent, presenting primarily with dysphonia. Recently, reflux has been associated with a myriad of laryngeal pathologies. Because the typical symptoms of reflux often are absent and the frequency of reflux episodes required to cause laryngeal problems are relatively few, diagnosis can be difficult. Empiric treatment with proton pump inhibitors may be required as a diagnostic trial. It also is important to distinguish intermittent or recurrent dysphonia from persistent or progressive dysphonia. Intermittent dysphonia may be worse in the morning, perhaps suggesting laryngopharyngeal reflux as a cause; or worse in the evening after voice use, suggesting an anatomic problem such as vocal nodules. Persistent, progressive dysphonia suggests the presence of papillomata or another enlarging neoplasm. Progressive dysphonia also may exhibit fluctuations day to day. The key to the difference between intermittent and fluctuating progressive hoarseness, however, is that the general baseline of the voice disorder also slowly worsens, which may indicate a more serious and potentially life-threatening problem such as a growing neoplasm or papillomata. Associated breathing problems such as stridor also indicate a problem that requires timely evaluation. Stridor is caused by turbulent airflow through the aerodigestive tract. Inspiratory stridor generally indicates an extrathoracic problem such as supraglottic collapse or vocal fold paralysis. An intrathoracic lesion can cause expiratory stridor. A fixed lesion, such as subglottic stenosis, can cause stridor that is biphasic. Although lesions anywhere along the aerodigestive tract can cause a change in the character of the voice, hoarseness generally is caused by abnormalities at the glottis. Dysphagia is not frequently associated with vocal disorders. This association should alert the clinician to laryngeal and hypopharyngeal motor or sensory problems. Dysphagia also may be a sign of a mass lesion, which will inhibit swallowing as well as vocalization. As mentioned previously, significant gastroesophageal (or gastropharyngeal) reflux causing dysphonia otherwise may be silent. Globus sensation and frequent throat clearing may suggest glottic edema caused by reflux. Determining historic clues for reflux in children and infants can be difficult. Gastroesophageal reflux disease (GERD) reflux in infants is very common, and often is exhibited by frequent spitting up or wet burps. The significance and actual incidence of reflux laryngitis in the refluxing infant is not known. Frequent belching or regurgitation also may imply reflux. Incomplete regurgitation with swallowing of the partially regurgitated material may be evident on questioning the child. In adults, even infrequent episodes of GE reflux are sufficient to cause dysphonia [3]. Reflux also is thought to be associated with chronic hoarseness and vocal nodules in children [4]. No criterion for diagnosis exists for significant gastroesophageal reflux inducing laryngeal pathology. Once the other causes for dysphonia have been ruled out, however, an empiric trial of reflux management may be a useful diagnostic tool [5]. Past medical and surgical history also are important to assess. Associated general medical, neurologic, and respiratory conditions may affect the voice.
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Finally, it is important to inquire about other potential iatrogenic causes for the dysphonia, such as previous endotracheal intubation or nasogastric tube insertion.
Physical examination Further information is gained by a complete head and neck examination, with a critical assessment of the character and quality of the voice. The assistance of a speech language pathologist is useful for subjective and objective assessment of the voice. Visualization of the larynx often is the most useful portion of the physical examination (Fig. 1). Indirect mirror laryngoscopy is useful in the cooperative child, but video fiberoptic laryngoscopy is much more informative in most children. The video documentation can be reassessed in real time and again in slow motion after the examination has been completed, which is useful in the evaluation of an uncooperative child. Brief glimpses of the larynx during the examination can be reviewed with slow-motion replay. Transoral visualization using rigid telescopes with angled views is possible in most children 5 to 7 years of age and older, giving the best laryngeal images. Awake fiberoptic endoscopy is possible in nearly all patients, regardless of age. Severe anatomic anomalies and significant retained secretions may obscure the view of the larynx with a flexible fiberoptic approach. Topical decongestants and anesthetics make the examination tolerable. Sedation generally is not used because most children tolerate the fiberscope. This awake fibertopic examination enables a dynamic or functional assessment of the larynx. Laryngeal motion is important to assess during the examination to determine whether there is fixation of the arytenoids or neurologic problems of the larynx. Occasionally, stroboscopy also is used to further dem-
Fig. 1. Normal appearance of the larynx during rigid endoscopy. The anterior commissure can be seen at the apex of the triangle of the glottic inlet. The membranous vocal fold extends from the anterior commissure to the vocal process, which can be seen as a thickening of the posterior one third of the vocal fold. The posterior glottis at the bottom of the figure is an excellent place to look for erythema or edema consistent with reflux laryngitis.
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onstrate the vibratory character of the vocal folds. Nodules or lesions of the cover of the vocal fold may decrease the mucosal wave. Intracordal cysts or a lesion of the body of the vocal fold may not affect the presence of the mucosal wave [6]. Vocal fold motion is assessed by performing a functional indirect laryngoscopy. Unilateral and bilateral vocal fold paralysis usually can be distinguished during this examination. Occasionally, it is not possible to fully visualize the glottis during the complete phase of adduction and abduction. This is especially problematic in infants with foreshortened aryepiglottic folds and an omega-shaped epiglottis. Some investigators have used ultrasound to help assess vocal cord motion [7]. Laryngeal electromyography (EMG) has been used on occasion in the assessment of children with vocal and motion abnormalities, but technical difficulties have made this procedure problematic. EMG may be helpful, however, in distinguishing fixation of the vocal fold from vocal cord paralysis, as well as to follow reinnervation after recurrent laryngeal nerve injury [8]. This procedure remains investigational in children, however, because of its technical difficulties. Laryngeal sensory testing has been used widely in the adult population [9,10]; its utility in the pediatric population, however, has yet to be determined. Laryngeal sensory testing may be a helpful adjunct for the assessment of neurosensory disorders that can predispose children to dysphonia or dysphagia [11]. Indirect laryngoscopy also may help to detect inflammatory changes of the larynx. Laryngoscopic findings suggestive of GE reflux have been defined in the literature [12 –14], including posterior glottic erythema and edema, pseudosulcus, interarytenoid edema and corrugated mucosa, laryngeal hypervascularity, and lingual tonsillar hypertrophy. In patients with dysphonia, these findings, which often improve with empiric reflux therapy, should alert the clinician to the possibility of significant gastropharyngeal reflux. Gastropharyngeal reflux with associated laryngitis has been associated with gastroesophageal reflux without typical symptoms of reflux or reflux-induced esophagitis. Normative data are being collected for objective voice quality measures. Normative data regarding fundamental frequency, open quotient, closed quotient, jitter, and shimmer, soon may be available to help in the assessment and management of pediatric voice disorders. Electroglottography measures laryngeal impedance via surface electrodes; this procedure is well tolerated by children and can be obtained in a noisy environment [15]. The best test for the evaluation of vocal quality and detection of change in vocal quality with treatment has yet to be determined for pediatric patients. Subjective assessment of vocal quality may be the best tool, but can lead to research difficulties due to its inherent interobserver and intraobserver variability.
pH probe The usefulness of pH probe testing for the diagnosis of significant gastropharygeal reflux is controversial. The controversy lies in the definition of significant reflux [16] and the known problem of capturing what may be an
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infrequent event during an overnight study. Because the mucosa of the larynx differs from that of the esophagus, less frequent exposure to gastric contents can cause significant changes in the larynx. The most specific test for gastropharyngeal reflux may be laryngeal biopsy [17]. The risks involved with laryngeal biopsy may make it unacceptable for use as a screening tool. Few studies have adequately assessed the role of empiric treatment of gastropharygeal reflux in the management of pediatric dysphonia. Empiric therapy for reflux may be a useful tool, however, in helping to determine the role of gastrolaryngeal reflux in causing dysphonia. Nonacidic reflux also may have a role in laryngeal disease. New tools for detected nonacidic reflux are being developed [18]. These new tools include multichannel impedance devices. This device when combined with a pH probe can detect both acidic and nonacidic reflux. Multichannel impedance measures of esophagel reflux may prove to be important in defining the significance of gastropharyngeal reflux and its role in dysphonia.
Microlaryngoscopy Successful treatment of any disorder requires an accurate diagnosis. Occasionally the correct diagnosis cannot be achieved in the office setting, and thus operative endoscopy is required. With the assistance of a general anesthetic, direct microlaryngoscopy can be performed, allowing the clinician to further visualize and palpate the larynx. Direct microlaryngoscopy is an extension of a physical examination. Biopsy is possible during this procedure as well. Examples when direct microlaryngoscopy is useful may include the differentiation of a vocal nodule and an intracordal cyst. At times it is only during exploration of the vocal fold that the correct differentiation between a vocal fold cyst or vocal fold nodule can be made. Except for the instrumentation, the technique for pediatric laryngoscopy does not differ greatly from that of adult laryngoscopy. The endoscopist must be comfortable with managing the pediatric airway, however. The pediatric patient often has less pulmonary reserve, and because of the small size of the pediatric larynx, there is less tolerance for postinstrumentation edema. Properly sized instruments and the expertise and skill to use them are required. Finally, proper anesthetic assistance and the facilities to manage potential complications must be available before pediatric endoscopy should be attempted.
Differential diagnosis and treatment Infection Edema from localized infections is the most common cause of hoarseness in children and adults. Usually self-limiting, these infections are, in most cases, mild
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in terms of systemic symptoms. Viral prodromes usually precede cough and dysphonia. Although infectious laryngitis is usually of viral origin, it also may be bacterial or, in rare instances, fungal in nature. These infections generally are more aggressive and can cause more significant respiratory symptoms as well. Acute laryngotracheobronchitis is most often caused by parainfluenza viruses I and II, and is known commonly as croup. Low-grade fever, hoarseness, and a severe barky cough with spasmodic stridor follow symptoms of a viral illness. Respiratory syncitial virus and influenza virus types A and B also may cause croup. Medical attention is more often sought due to the associated stridor than to the associated dysphonia. Regardless of the etiologic agents, croup is the clinical diagnosis. Radiographic studies may confirm the diagnosis with the pathognomonic steeple sign found by posteroanterior neck radiograph. Treatment generally is expectant with systemic steroids and observation. A cool mist may be helpful; endotracheal intubation is reserved only for severe cases with lifethreatening respiratory distress [19]. When intubation is required, extreme care must be taken to choose the appropriate-sized endotracheal tube, to avoid iatrogenic stenosis of the subglottis. Croup must be differentiated from more serious laryngotracheal infections, such as membranous laryngotracheobronchitis or epiglottitis. Children with these infections appear toxic, and often have a high fever and a high white blood cell count. Respiratory difficulty often is seen in association with the dysphonia and odynophagia. Membranous laryngotracheobronchitis may present with a prodrome similar to croup, but the child’s symptoms rapidly progress to a more toxic appearance. Neck radiographs demonstrate irregular borders in the trachea that are consistent with thick tenacious secretions. Fiberoptic nasal endoscopy shows thick purulent secretions at the glottis and subglottis, but a normal epiglottis. Occasionally, operative endoscopy is required to clear the trachea of the thick membranous secretions. In these cases, endotracheal intubation will supply a route for continued pulmonary toilet. Rarely, serial operative endoscopy is required to keep the airway clear of secretions. Culture is helpful and usually reveals Staphylococcus aureus or Haemophilus influenzae. Antibiotic therapy should be directed toward these causative organisms unless cultures and sensitivities direct differently. The introduction of the H influenzae type B vaccination has decreased dramatically the incidence of epiglottis in children [20]. Epiglottitis generally does not have a significant or prolonged prodromal period. The child rapidly appears toxic with drooling, posturing, and respiratory difficulty and dysphonia. Muffled voice and arms positioned in a tripod stance often accompany the high fever and tachypnea. The child often is most comfortable sitting upright with the neck extended and the arms forward for support, to maximize the airway. Lateral neck radiographs show a thickened epiglottis (thumb sign). If epiglottitis is suspected, the child should be taken immediately but quietly to a setting in which direct laryngoscopy and airway management is possible. The diagnosis is confirmed by the endoscopy. Once the airway is established, cultures are
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obtained. Initial antibiotic therapy should be directed against H influenzae type B. Other causative agents can include candidiasis or Streptococcus pneumoniae seen in conjunction with immunosuppressed states such as leukemia [21]. Extubation often is possible 48 hours after appropriate medical management, but should be directed by the status of the larynx.
Anatomic causes of dysphonia Cyst Mucus-filled cysts may form beneath the epithelium of the vocal folds, and cause dysphonia by increasing the mass of the body of the vocal fold. Disparity between the mass of each vocal fold will increase the effort required to begin vibration and also may make it more difficult to sustain clear phonatory sounds. Cysts may be difficult to distinguish from nodules in the pediatric larynx, because there is often a contralateral reactive vocal nodule, which gives the typical appearance of screamers nodules with symmetric bilateral thickening of the vocal fold. Subtle differences during indirect endoscopy may suggest the proper diagnosis, but intraoperative exploration of the vocal fold may be required to confirm the diagnosis and treat the intracordal cyst. In adults, the mucosal wave seen during videostroboscopy is not changed in intracordal cysts [6]. Intracordal cysts do not resolve with speech therapy. The voice may improve with speech therapy, however, because the thickening seen in reaction to the cyst may resolve with proper voice use. If significant dysphonia persists or recurs with speech therapy, microlaryngeal techniques for removal of the cyst should be considered. Care must be taken not to disrupt the integrity of the vocal ligament during excision.
Fig. 2. Vocal fold nodules can be seen opposing each other at the anterior to middle one third of the vocal fold. These nodules are discrete and appear in the immediate infraglottic aspect of the vocal fold.
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Vocal fold nodules Nodular thickening of the anterior to middle one third of the vocal folds is the most common cause of hoarseness in children (Fig. 2). Although a child occasionally may present with hoarseness from nodules at the time of their first words, most nodules develop when the child begins group activities. It generally is believed that the nodules occur from voice abuse during vocal play and social interaction. The voice worsens with aggressive vocal attack or loud play noises. Vocal nodules are more common in boys than in girls. Laryngoscopy confirms the diagnosis with thickening seen at the anterior to middle one third of the vocal ligament. The mucosal wave is diminished in vocal nodules, which is believed to be due to the increase in mass of the cover of the vocal fold [6]. Other conditions that increase the potential for vocal abuse range from hearing loss to velopharyngeal insufficiency. If these conditions are present, they should be treated. Voice therapy is the mainstay of treatment. Gastropharyngeal reflux also has been implicated in the cause of nodule formation. It is possible that gastropharyngeal reflux induces inflammation that predisposes a child to nodule formation on the larynx [4]. Nodules also may be maintained between cycles of voice abuse from inflammation caused by gastropharyngeal reflux. If reflux is suspected, empiric therapy should be used as an adjunct to voice therapy. Therapy for nodules generally is directed toward maximizing vocal hygiene and voice use. Speech therapy can help the child to identify ways to use the voice in an optimal manner. If the child’s age and maturity does not allow for mastery of proper voice use, continued observation and periodic retraining is indicated. Often simple alternatives to aggressive voice use or vocal play noises is all that is required to correct the voice abuse and allow for the nodules to resolve. There are rare occasions when a child masters proper voice use but continues to have significant vocal disturbance from nodules. Although this condition is not well studied, these children may benefit from microlaryngeal techniques to remove the nodular thickening. Care must be taken not to disrupt the vocal ligament, whichcan cause permanent scarring or tethering of the vocal epithelium to the vocal ligament and induce lifelong hoarseness. Children who cannot master proper voice use are not, as a rule, candidates for microlaryngeal removal of the nodules.
Congenital causes of dysphonia Saccular cyst Unlike intracordal cysts, saccular cysts are seen in the laryngeal ventricle or aryepiglottic fold. They may encroach on the vocal fold and cause dysphonia. Saccular cysts are ‘‘anterior’’ when confined to the anterior larynx and ventricle or ‘‘lateral’’ when extending into the aryepiglottic fold (Fig. 3). A child’s cry may be muffled from the cyst impressing on the vocal tract and the cyst also may
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Fig. 3. Operative endoscopy demonstrating a lateral saccular cyst. The feeding tube is seen entering the esophageal inlet. The cyst is seen in the aryepiglottic fold on the patient’s right side.
cause respiratory embarrassment. Awake indirect laryngoscopy is important for diagnosis and to help plan management of the airway. Although extremely rare, a supraglottic hemangioma can be mistaken for a saccular cyst. Surgical treatment depends on the size and location of the laryngeal cyst. Small anterior saccular cysts may be treated endoscopically with marsupialization. Although larger lateral saccular cysts may be approached transorally, an external approach may be needed for complete removal of the cyst. This procedure provides the lowest chance for recurrence. The approach requires entry into the periglottic space through the thyrohyoid membrane. Advanced endoscopic and open surgical techniques are used to avoid recurrence or the need for tracheotomy [22,23].
Fig. 4. Congenital laryngeal web seen at rigid endoscopy. The endotracheal tube passes easily through the posterior glottis, whereas the anterior two thirds of the vocal folds are fused together. This anterior glottic web also was associated with thickening of the cricoid cartilage and subglottic stenosis, which required expansion surgery for repair.
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Fig. 5. A congenital anterior glottic web at rigid endoscopy. The presenting symptoms were primarily voice related. The posterior glottis was relatively unaffected and thus respiration was not affected greatly.
Glottic webs Incomplete recannulization of the embryonic larynx leads to anterior glottic web formation. The extent of webbing may vary, from anterior webbing of the glottis to near complete occlusion of the laryngeal inlet (Fig. 4). Glottic webs generally present with a high-pitched and weak cry from birth. Often medical attention is not sought until the age of 2, depending on the severity of the web. Vocal disturbance is the usual presenting symptom. Interestingly, respiratory compromise may not be significant or only a minor component of the presenting symptoms. The anterior glottis is concerned mostly with phonation, whereas the posterior glottis contributes to the airway (Fig. 5). Diagnosis of glottic webs is made by office endoscopy. The treatment plan and surgical approach require rigid endoscopy to determine the extent into the subglottis. There is a temptation to treat webs endoscopically. This technique is useful in the rare thin anterior web, which does not extend into the subglottis. More often, congenital glottic webs are associated with cricoid thickening as well [24 – 26]. These cases require anterior expansion of the cricoid ring with a cartilage graft and lysis of the web at the glottis.
Inflammation Gastropharyngeal reflux Most, if not all, pathologic conditions of the larynx have been attributed to gastropharyngeal reflux at one time or another. The gastropharyngeal reflux disease process remains controversial and difficult to study in adults and children alike. Subglottic stenosis has been shown to be caused by infrequent application
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of gastric contents to the cricoid in an animal model [27]. Reversible dysphonia has been documented to respond to antireflux therapy in adults [3]. Glottic and supraglottic abnormalities attributed to gastropharyngeal reflux have been cataloged [3,12,13,16,28]. The difficulty lies in the definition and confirmation of significant gastric reflux to the level of the larynx. Because even infrequent gastric reflux can cause changes to the larynx, current diagnostic tools may miss significant events. Empiric treatment studies in adults have shown that the treatment of gastropharyngeal reflux with high-dose proton pump inhibitors often improves the quality of the voice and corrects abnormalities seen in the larynx [5]. The controlled studies performed in adults have not been duplicated in children. Studies are underway to elucidate the role of gastropharyngeal reflux in children as a contributing factor in the formation of nodules. Vocal hygiene, vocal behavioral modification, and empiric gastropharyngeal reflux treatment may be most beneficial. Changing the diet to discourage reflux and increasing the time between meals and reclining are important measures to decrease reflux. Working with a gastroenterologist who is aware of the association of reflux and laryngeal disorders often is helpful to maximize the medical management in refractory cases. There are times when antireflux surgery may be required [3].
Neoplastic causes of dysphonia Benign and malignant neoplasms of the pediatric larynx are very rare. When present, however, they may cause dysphonia. Hoarseness in a child should alert the clinician to the possibility of a neoplasm. When evaluating a hoarse child, one should worry about the worst, see what is there, and treat what is seen. Visualization of the larynx to rule out a neoplasm is as important in a child as it is in an adult. Table 2 lists the most common of the rare neoplasms seen in the pediatric larynx. Ninety-eight percent of tumors in the pediatric airway are
Table 2 Benign and malignant neoplasms of the pediatric larynx Site
Malignant neoplasm
Benign neoplasm
Epithelium
Squamous cell carcinoma Mixed tumor Adenocarcinoma Neurofibrosarcoma
Recurrent respiratory papilloma Mixed tumor Adenoma Neurofibroma Granular cell myoblastoma Neurilemoma Hemangioma Lipoma Rhabdomyoma Chondroma Fibroma Lymphangioma
Neurogenic
Connective tissue
Fibrosarcoma Rhabdomyosarcoma Chondrosarcoma Angiosarcoma
Miscellaneous
Metastatic tumors
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benign. If recurrent respiratory papilloma is considered a neoplasm of the larynx, it is then by far the most common neoplasm in the pediatric larynx, with hemangiomas and neurogenic tumors being the next most common neoplasms. Histologic diagnosis is required before treatment plans can be formulated. Voice changes in children with neoplasms are progressive and persistent, and may be associated with stridor. When respiratory compromise is associated with the symptoms of hoarseness, rapid evaluation and treatment are important.
Recurrent respiratory papillomatosis The human papilloma virus (types 6 and 11) has been found to be the causative agent for the recurrent growths seen in the pediatric larynx [29]. The virus has a predilection for the squamociliary junctions of the aerodigestive epithelium [30]. Recurrent respiratory papilloma may present with symptoms of hoarseness, snoring, dysphagia, or stridor with respiratory compromise (Fig. 6). Once the diagnosis has been confirmed by visualization and occasionally biopsy, the gold standard of treatment is periodic mechanical debridement. The carbon dioxide laser and the laryngeal microresector are the instruments most commonly used. Eradication of the viral DNA from normal-appearing tissue is not possible, and recurrence is expected [31]. Laryngeal papillomatosis has been termed juvenile recurrent respiratory papilloma because the recurrent nature of the viral eruptions decreases with increasing age. Factors regulating viral expression and recurrence are not known, but may be hormonal. Flow cytometry may give some indication with regard to the aggressive nature of the papilloma [32,33]. Although rare, respiratory papillomatosis has been associated with malignant transformation [34]. Periodic histologic analysis and viral typing is an important surveillance tool.
Fig. 6. Rigid endoscopy of a pediatric larynx with recurrent respiratory papilloma. The entire laryngeal inlet is filled with papillomata. This child presented with respiratory compromise.
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Treatment is directed at maintaining an adequate airway while preserving the structures of the larynx that are important for phonation. Frequent surgical resection of the viral papilloma is the mainstay of treatment. The carbon dioxide laser has been used to vaporize the viral eruptions, and is an excellent tool, although two disadvantages are its potential for airway fires and laser-induced thermal scarring of the larynx. Increased stiffness of the vocal fold with subsequent hoarseness has been reported when injudicial use of the laser [35]. The use of a microresector system helps to avoid deep thermal injury in extensive lesions [36]. Regardless of the technique used, care must be taken to remove only the superficial layer of the infected laryngeal mucosa so that the deep structures are not disturbed. In addition, the anterior commissure must be protected so that opposing mucosal defects are avoided. This will help to prevent the formation of an anterior web. Adjuvant medical treatments to surgical debridement have been tried, but none have been found to be convincingly effective. Further studies of current antiviral therapies are required before recommendations can be given. Inducing viral remission with limited side effects is desirable.
Neurologic causes of dysphonia Paralysis of the vocal folds may be unilateral or bilateral. The symptoms surrounding each type of paralysis depend on the kind of paralysis present. In general, bilateral vocal fold paralysis is caused by central nervous system anomalies. Unilateral paralysis generally stems from peripheral lesions or iatrogenic causes, although occasionally it may be caused from central lesions as well. Bilateral vocal fold paralysis may present with near-normal or slightly weaker vocalization. Much more prevalent is the associated high-pitched inspiratory stridor. The voice is often stronger than caregivers would expect and thus bilateral vocal fold paralysis often is not included in the differential diagnosis. The vocal folds are in the paramedian position, which is more compatible with vocalization than with breathing. Unilateral vocal fold paralysis often presents with a weak and breathy cry; respiratory distress usually is not seen. Feeding difficulties are noted with unilateral paralysis when the contralateral fold cannot compensate for the restricted movement of the paralyzed vocal fold. There also may be accompanying sensory deficits. Patients with congenital vocal fold paralysis require an extensive workup. Other neurologic deficits may point to a neurologic cause. It is rare, however, that generalized neurologic deficits cause vocal fold paralysis. Some central nervous system disorders that may contribute to vocal fold paralysis include intracerebral hemorrhage, cerebral agenesis, hydrocephalus, and Arnold-Chiari malformations of the brain stem with cerebellar herniation. The entire course of the involved recurrent laryngeal nerve should be studied. Evaluation should include chest radiographs and CT or MRI from the head to the aortic arch. Heart anomalies or surgery to correct them may cause damage to the left recurrent laryngeal nerve in
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particular, because it courses around the aortic arch. Birth trauma also can cause damage to the laryngeal nerves, and malignant neoplasms can cause paralysis of the laryngeal nerves. Occasionally, no cause is identifiable. Iatrogenic injury to the laryngeal nerve from chest or cardiothoracic neck surgery can occur even in the most accomplished hands. Recovery is possible depending on the severity of the injury. Often, no therapy is needed in unilateral paralysis, because the contralateral vocal fold may be able to overcompensate for the paralyzed vocal fold—a phenomenon that is seen much more often in children than in adults. Observation and speech therapy to help with swallowing and speech difficulties are the most common forms of treatment. Occasionally, significant aspiration or dysphonia results from a unilateral vocal fold paralysis, in which case, vocal fold medialization techniques are appropriate. Knowledge of the developing laryngeal framework is required. Use of adult techniques for medialization often results in improper placement of the medialization prosthesis [37]. No long-term series have been reported. There is a theoretic requirement for revision of the framework surgery as the laryngeal cartilages grow. Bilateral vocal fold paralysis may improve if the underlying cause of the paralysis is treated. Bilateral vocal fold paralysis often requires tracheotomy [38]; however, before the decision for tracheotomy is made, close observation and a multidisciplinary approach is required. Some groups report an incidence of tracheotomy as low as 50% in children with bilateral vocal fold paralysis [38]. If tracheotomy is required, there are surgical techniques to expand the glottic inlet in hopes of decannulation. The specific techniques used vary and depend on the anatomy seen. Modification of the laryngeal framework generally is deferred until the age of 2. Expansion of the airway is performed at the expense of glottic closure. This widening of the posterior glottic gap may cause worsening of the voice or an inability to swallow. Endoscopic techniques include partial or complete arytenoidectomy, although posterior cordotomy also may be sufficient. In addition, laryngotomy with arytenoidectomy or arytenoidopexy has had acceptable results [39]. Expansion of the glottic inlet by inserting a cartilage graft into the posterior cricoid framework increases the glottic airway without disturbing the membranous vocal folds [40]. Nerve-muscle pedicles have had disappointing results. Regardless of the technique chosen, care must be taken to balance increasing the airway for breathing with maintaining the ability to vocalize and swallow without aspiration.
Iatrogenic causes of dysphonia Prevention is the best form of treatment for iatrogenic causes of dysphonia. When they occur, however, correct identification of the cause is required before repair can occur. Subglottic stenosis, isolated from the glottis, may cause dysphonia because of changes in airflow and subglottic pressure across the vibrating vocal folds. Repair of the subglottic stenosis can be achieved with expansion of the subglottic unit or resection of the stenosis. Improvement of the
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voice correlates with increased airflow across the glottis. A transglottic stenosis with incorporation of the vocal folds into the scar often leads to poor voice quality even after repair. Quality sound production requires supple vocal fold mucosa to vibrate over the body of the vocal folds themselves. If scarring occurs to the vocal folds, correction of the supple nature of the mucosa is difficult. A usable voice often requires use of other portions of the larynx, most often the supraglottis. Arytenoid dislocation or subluxation is a rare cause of dysphonia, but should be suspected in children with persistent dysphonia after extubation. The diagnosis is suspected with decreased mobility of the vocal fold seen by indirect laryngoscopy, and is confirmed by direct laryngoscopy with palpation of the cricoarytenoid joint. Mobilization is possible after diagnosis during the endoscopy. Although arytenoid subluxation may be confused with vocal fold paralysis, palpation during endoscopy can help to differentiate between the two. Speed, from suspicion to diagnosis and treatment, may be important to avoid fixation of the cricoarytenoid joint.
Summary Vocal disturbances in children are surprisingly common. Typical pathologic conditions differ, however, from those that are seen commonly in adults. Disturbances in the vibratory characteristic of the vocal folds cause dysphonia. The most common causes for dysphonia are infectious, anatomic, congenital, inflammatory, neoplastic, neurologic, or iatrogenic in nature. A child who presents with hoarseness demands a rapid and thorough assessment. Fine points during the history may provide the clinician with clues as to the correct etiologic category. Proper treatment requires an accurate diagnosis. Although some conditions are similar in the adult larynx, treatment of the pediatric larynx often differs. An understanding of the changing and growing pediatric larynx is necessary for treatment and satisfactory results.
References [1] McMurray JS. Medical and surgical treatment of pediatric dysphonia. Otolaryngol Clin North Am 2000;33(5):1111 – 26. [2] Ruscello DM, Lass NJ, Podbesek J. Listeners’ perceptions of normal and voice-disordered children. Folia Phoniatr (Basel) 1988;40(6):290 – 6. [3] Hanson DG, Kamel PL, Kahrilas PJ. Outcomes of antireflux therapy for the treatment of chronic laryngitis. Ann Otol Rhinol Laryngol 1995;104(7):550 – 5. [4] Kalach N, Gumpert L, Contencin P, Dupont C. Dual-probe pH monitoring for the assessment of gastroesophageal reflux in the course of chronic hoarseness in children. Turk J Pediatr 2000; 42(3):186 – 91. [5] Hanson DG, Jiang JJ. Diagnosis and management of chronic laryngitis associated with reflux. Am J Med 2000;108(Suppl 4A):112S – 9S. [6] Shohet JA, Courey MS, Scott MA, Ossoff RH. Value of videostroboscopic parameters in differentiating true vocal fold cysts from polyps. Laryngoscope 1996;106(1 Pt 1):19 – 26.
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[7] Friedman EM. Role of ultrasound in the assessment of vocal cord function in infants and children. Ann Otol Rhinol Laryngol 1997;106(3):199 – 209. [8] Pototschnig C, Thumfart WF. Electromyographic evaluation of vocal cord disorders. Acta Otorhinolaryngol Belg 1997;51(2):99 – 104. [9] Aviv JE, Kim T, Thomson JE, Sunshine S, Kaplan S, Close LG. Fiberoptic endoscopic evaluation of swallowing with sensory testing (FEESST) in healthy controls [see comments]. Dysphagia 1998;13(2):87 – 92. [10] Aviv JE, Sacco RL, Mohr JP, et al. Laryngopharyngeal sensory testing with modified barium swallow as predictors of aspiration pneumonia after stroke. Laryngoscope 1997;107(9):1254 – 60. [11] Link DT, Willging JP, Miller CK, Cotton RT, Rudolph CD. Pediatric laryngopharyngeal sensory testing during flexible endoscopic evaluation of swallowing: feasible and correlative. Ann Otol Rhinol Laryngol 2000;109(10 Pt 1):899 – 905. [12] Belafsky PC, Postma GN, Koufman JA. The association between laryngeal pseudosulcus and laryngopharyngeal reflux. Otolaryngol Head Neck Surg 2002;126(6):649 – 52. [13] Belafsky PC, Postma GN, Koufman JA. The validity and reliability of the reflux finding score (RFS). Laryngoscope 2001;111(8):1313 – 7. [14] Carr MM, Nagy ML, Pizzuto MP, Poje CP, Brodsky LS. Correlation of findings at direct laryngoscopy and bronchoscopy with gastroesophageal reflux disease in children: a prospective study. Arch Otolaryngol Head Neck Surg 2001;127(4):369 – 74. [15] Cheyne HA, Nuss RC, Hillman RE. Electroglottography in the pediatric population. Arch Otolaryngol Head Neck Surg 1999;125(10):1105 – 8. [16] Koufman J, Sataloff RT, Toohill R. Laryngopharyngeal reflux: consensus conference report. J Voice 1996;10(3):215 – 6. [17] McMurray JS, Gerber M, Stern Y, et al. Role of laryngoscopy, dual pH probe monitoring, and laryngeal mucosal biopsy in the diagnosis of pharyngoesophageal reflux. Ann Otol Rhinol Laryngol 2001;110(4):299 – 304. [18] Kahrilas PJ. Will impedence testing rewrite the book on GERD? Gastroenterology 2001; 120(7):1862 – 4. [19] Cressman WR, Myer 3rd CM. Diagnosis and management of croup and epiglottitis. Pediatr Clin North Am 1994;41(2):265 – 76. [20] Keyser JS, Derkay CS. Haemophilus influenzae type B epiglottitis after immunization with HbOC conjugate vaccine. Am J Otolaryngol 1994;15(6):436 – 43. [21] Lee AC, Lam SY. Life threatening acute epiglottitis in acute leukemia. Leuk Lymphoma 2002; 43(3):665 – 7. [22] Donegan JO, Strife JL, Seid AB, Cotton RT, Dunbar JS. Internal laryngocele and saccular cysts in children. Ann Otol Rhinol Laryngol 1980;89(5 Pt 1):409 – 13. [23] Myssiorek D, Persky M. Laser endoscopic treatment of laryngoceles and laryngeal cysts. Otolaryngol Head Neck Surg 1989;100(6):538 – 41. [24] Altman KW, Wetmore RF, Marsh RR. Congenital airway abnormalities requiring tracheotomy: a profile of 56 patients and their diagnoses over a 9 year period. Int J Pediatr Otorhinolaryngol 1997;41(2):199 – 206. [25] Cohen SR. Congenital glottic webs in children. A retrospective review of 51 patients. Ann Otol Rhinol Laryngol Suppl 1985;121:2 – 16. [26] Holinger PH, Brown WT. Congenital webs, cysts, laryngoceles and other anomalies of the larynx. Ann Otol Rhinol Laryngol 1967;76(4):744 – 52. [27] Little FB, Koufman JA, Kohut RI, Marshall RB. Effect of gastric acid on the pathogenesis of subglottic stenosis. Ann Otol Rhinol Laryngol 1985;94(5 Pt 1):516 – 9. [28] Wilson JA, White A, von Haacke NP, et al. Gastroesophageal reflux and posterior laryngitis. Ann Otol Rhinol Laryngol 1989;98(6):405 – 10. [29] Hayjek E. Contribution to the etiology of laryngeal papilloma in children. J Laryngol Otol 1956; 70:166. [30] Kashima H, Mounts P, Leventhal B, Hruban RH. Sites of predilection in recurrent respiratory papillomatosis. Ann Otol Rhinol Laryngol 1993;102(8 Pt 1):580 – 3.
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[31] Bauman NM, Smith RJ. Recurrent respiratory papillomatosis. Pediatr Clin North Am 1996; 43(6):1385 – 401. [32] Stern Y, Mueller K, Willging JP, Myer 3rd CM, Cotton RT. S-phase fraction as a predictor of prognosis in juvenile respiratory papillomatosis. Arch Otolaryngol Head Neck Surg 1998;124(5): 541 – 4. [33] Stern Y, Heffelfinger SC, Walner DL, Cotton RT. Expression of Ki-67, tumor suppressor proteins, growth factor, and growth factor receptor in juvenile respiratory papillomatosis: Ki-67 and p53 as predictors of aggressive disease. Otolaryngol Head Neck Surg 2000;122(3): 378 – 86. [34] Rady PL, Schnadig VJ, Weiss RL, Hughes TK, Tyring SK. Malignant transformation of recurrent respiratory papillomatosis associated with integrated human papillomavirus type 11 DNA and mutation of p53. Laryngoscope 1998;108(5):735 – 40. [35] Ossoff RH, Werkhaven JA, Dere H. Soft-tissue complications of laser surgery for recurrent respiratory papillomatosis. Laryngoscope 1991;101(11):1162 – 6. [36] Myer 3rd CM, Willging JP, McMurray S, Cotton RT. Use of a laryngeal micro resector system. Laryngoscope 1999;109(7 Pt 1):1165 – 6. [37] Link DT, Rutter MJ, Liu JH, Willging JP, Myer CM, Cotton RT. Pediatric type I thyroplasty: an evolving procedure. Ann Otol Rhinol Laryngol 1999;108(12):1105 – 10. [38] Daya H, Hosni A, Bejar-Solar I, Evans JN, Bailey CM. Pediatric vocal fold paralysis: a longterm retrospective study. Arch Otolaryngol Head Neck Surg 2000;126(1):21 – 5. [39] Choi SS, Cotton RT. Surgical management of voice disorders. Pediatr Clin North Am 1989; 36(6):1535 – 49. [40] Gray SD, Kelly SM, Dove H. Arytenoid separation for impaired pediatric vocal fold mobility. Ann Otol Rhinol Laryngol 1994;103(7):510 – 5.
Disorders of phonation in children J. Scott McMurray, MD, FAAP, FACS Division of Otolaryngology, Department of Surgery, Department of Pediatrics, University of Wisconsin Medical School, K4-766 CSC, 600 Highland Avenue, Madison, WI 53792, USA
Phonation is defined as the utterance of vocal sounds. Disorders of phonation, therefore, can be caused by a wide variety of abnormalities along the aerodigestive tract, including laryngeal pathology, velopharyngeal problems, and even cognitive delays that can inhibit a person from making intelligible vocal sounds. This article concentrates on the evaluation and management of common laryngeal abnormalities seen in children with disorders of phonation. Much of this information also has been presented in a previous issue of the Otolaryngologic Clinics of North America [1]. It has been estimated that over one million children suffer from dysphonia nationwide. Dysphonia can be detrimental to children both psychosocially and academically. Fortunately most causes of dysphonia or hoarseness in children are benign and generally are easy to treat. A multidisciplinary approach to the diagnosis and treatment of pediatric voice disorders is essential. The evaluation and treatment of voice problems is important for several reasons. Some voice disorders are potentially life threatening. Dysphonia—such as that seen from recurrent laryngeal papillomata—can be accompanied by lifethreatening airway obstruction. Dysphonic children also may face perceptual and psychosocial problems. In one study [2], men and women majoring in business, education, and economics were asked to listen to the speech of voice-disordered children and normal-speaking children. Without visually seeing the children, listeners’ judged the voice-disordered children as ‘‘dirty, cruel, ugly, unfair, small, weak, slow, clumsy, or sick’’ more often than they did the normal-speaking children [2]. Additionally, children with disorders of phonation often experience prejudice from their peers and teachers. Therefore, it is important that dysphonic children be referred to a team of professionals capable of providing comprehensive evaluation and management.
E-mail address: [email protected] 0031-3955/03/$ – see front matter D 2003, Elsevier Inc. All rights reserved. doi:10.1016/S0031-3955(03)00028-2
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Evaluation An evaluation for pediatric dysphonia may consist of all or part of the following:
History taking Physical examination Indirect functional laryngoscopy Voice evaluation Radiographic evaluation pH probe Direct laryngoscopy, bronchoscopy, and esophagoscopy Diagnosis or decision making Treatment Surgical Medical Speech therapy
History As with any medical disorder, a complete and methodic history is essential in the evaluation of pediatric dysphonia. Fine points recorded during the history may help to narrow the differential diagnosis. The causes of pediatric dysphonia may be classified as infectious, anatomic, congenital, inflammatory, neoplastic, neurologic, and iatrogenic. It is important to determine the time of onset of the voice disorder as well as its duration and chronicity, to help direct the decisionmaking process. The time of onset of dysphonia in relation to birth can help to separate the causes of dysphonia into general categories (Table 1). Many congenital causes of dysphonia are apparent at birth, although some may not present until later. Neurologic causes generally present at birth or at the time of the presentation of the neurologic condition. Iatrogenic causes usually have an antecedent event that may predate the dysphonia by several months, such as subglottic stenosis that develops after endotracheal intubation. Infectious causes (except for laryngeal papillomata) generally have associated systemic signs of illness. Voice strain or Table 1 Differences in the typical onset of dysphonia, by general categories Dysphonia presenting at birth
Dysphonia presenting after birth
Congenital Neurologic
Anatomic Neurologic Neoplastic Inflammatory Infectious Iatrogenic
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aggressive voice use usually is associated with anatomic causes for hoarseness such as vocal nodules. Inflammatory causes for dysphonia, such as reflux laryngitis, may be otherwise silent, presenting primarily with dysphonia. Recently, reflux has been associated with a myriad of laryngeal pathologies. Because the typical symptoms of reflux often are absent and the frequency of reflux episodes required to cause laryngeal problems are relatively few, diagnosis can be difficult. Empiric treatment with proton pump inhibitors may be required as a diagnostic trial. It also is important to distinguish intermittent or recurrent dysphonia from persistent or progressive dysphonia. Intermittent dysphonia may be worse in the morning, perhaps suggesting laryngopharyngeal reflux as a cause; or worse in the evening after voice use, suggesting an anatomic problem such as vocal nodules. Persistent, progressive dysphonia suggests the presence of papillomata or another enlarging neoplasm. Progressive dysphonia also may exhibit fluctuations day to day. The key to the difference between intermittent and fluctuating progressive hoarseness, however, is that the general baseline of the voice disorder also slowly worsens, which may indicate a more serious and potentially life-threatening problem such as a growing neoplasm or papillomata. Associated breathing problems such as stridor also indicate a problem that requires timely evaluation. Stridor is caused by turbulent airflow through the aerodigestive tract. Inspiratory stridor generally indicates an extrathoracic problem such as supraglottic collapse or vocal fold paralysis. An intrathoracic lesion can cause expiratory stridor. A fixed lesion, such as subglottic stenosis, can cause stridor that is biphasic. Although lesions anywhere along the aerodigestive tract can cause a change in the character of the voice, hoarseness generally is caused by abnormalities at the glottis. Dysphagia is not frequently associated with vocal disorders. This association should alert the clinician to laryngeal and hypopharyngeal motor or sensory problems. Dysphagia also may be a sign of a mass lesion, which will inhibit swallowing as well as vocalization. As mentioned previously, significant gastroesophageal (or gastropharyngeal) reflux causing dysphonia otherwise may be silent. Globus sensation and frequent throat clearing may suggest glottic edema caused by reflux. Determining historic clues for reflux in children and infants can be difficult. Gastroesophageal reflux disease (GERD) reflux in infants is very common, and often is exhibited by frequent spitting up or wet burps. The significance and actual incidence of reflux laryngitis in the refluxing infant is not known. Frequent belching or regurgitation also may imply reflux. Incomplete regurgitation with swallowing of the partially regurgitated material may be evident on questioning the child. In adults, even infrequent episodes of GE reflux are sufficient to cause dysphonia [3]. Reflux also is thought to be associated with chronic hoarseness and vocal nodules in children [4]. No criterion for diagnosis exists for significant gastroesophageal reflux inducing laryngeal pathology. Once the other causes for dysphonia have been ruled out, however, an empiric trial of reflux management may be a useful diagnostic tool [5]. Past medical and surgical history also are important to assess. Associated general medical, neurologic, and respiratory conditions may affect the voice.
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Finally, it is important to inquire about other potential iatrogenic causes for the dysphonia, such as previous endotracheal intubation or nasogastric tube insertion.
Physical examination Further information is gained by a complete head and neck examination, with a critical assessment of the character and quality of the voice. The assistance of a speech language pathologist is useful for subjective and objective assessment of the voice. Visualization of the larynx often is the most useful portion of the physical examination (Fig. 1). Indirect mirror laryngoscopy is useful in the cooperative child, but video fiberoptic laryngoscopy is much more informative in most children. The video documentation can be reassessed in real time and again in slow motion after the examination has been completed, which is useful in the evaluation of an uncooperative child. Brief glimpses of the larynx during the examination can be reviewed with slow-motion replay. Transoral visualization using rigid telescopes with angled views is possible in most children 5 to 7 years of age and older, giving the best laryngeal images. Awake fiberoptic endoscopy is possible in nearly all patients, regardless of age. Severe anatomic anomalies and significant retained secretions may obscure the view of the larynx with a flexible fiberoptic approach. Topical decongestants and anesthetics make the examination tolerable. Sedation generally is not used because most children tolerate the fiberscope. This awake fibertopic examination enables a dynamic or functional assessment of the larynx. Laryngeal motion is important to assess during the examination to determine whether there is fixation of the arytenoids or neurologic problems of the larynx. Occasionally, stroboscopy also is used to further dem-
Fig. 1. Normal appearance of the larynx during rigid endoscopy. The anterior commissure can be seen at the apex of the triangle of the glottic inlet. The membranous vocal fold extends from the anterior commissure to the vocal process, which can be seen as a thickening of the posterior one third of the vocal fold. The posterior glottis at the bottom of the figure is an excellent place to look for erythema or edema consistent with reflux laryngitis.
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onstrate the vibratory character of the vocal folds. Nodules or lesions of the cover of the vocal fold may decrease the mucosal wave. Intracordal cysts or a lesion of the body of the vocal fold may not affect the presence of the mucosal wave [6]. Vocal fold motion is assessed by performing a functional indirect laryngoscopy. Unilateral and bilateral vocal fold paralysis usually can be distinguished during this examination. Occasionally, it is not possible to fully visualize the glottis during the complete phase of adduction and abduction. This is especially problematic in infants with foreshortened aryepiglottic folds and an omega-shaped epiglottis. Some investigators have used ultrasound to help assess vocal cord motion [7]. Laryngeal electromyography (EMG) has been used on occasion in the assessment of children with vocal and motion abnormalities, but technical difficulties have made this procedure problematic. EMG may be helpful, however, in distinguishing fixation of the vocal fold from vocal cord paralysis, as well as to follow reinnervation after recurrent laryngeal nerve injury [8]. This procedure remains investigational in children, however, because of its technical difficulties. Laryngeal sensory testing has been used widely in the adult population [9,10]; its utility in the pediatric population, however, has yet to be determined. Laryngeal sensory testing may be a helpful adjunct for the assessment of neurosensory disorders that can predispose children to dysphonia or dysphagia [11]. Indirect laryngoscopy also may help to detect inflammatory changes of the larynx. Laryngoscopic findings suggestive of GE reflux have been defined in the literature [12 –14], including posterior glottic erythema and edema, pseudosulcus, interarytenoid edema and corrugated mucosa, laryngeal hypervascularity, and lingual tonsillar hypertrophy. In patients with dysphonia, these findings, which often improve with empiric reflux therapy, should alert the clinician to the possibility of significant gastropharyngeal reflux. Gastropharyngeal reflux with associated laryngitis has been associated with gastroesophageal reflux without typical symptoms of reflux or reflux-induced esophagitis. Normative data are being collected for objective voice quality measures. Normative data regarding fundamental frequency, open quotient, closed quotient, jitter, and shimmer, soon may be available to help in the assessment and management of pediatric voice disorders. Electroglottography measures laryngeal impedance via surface electrodes; this procedure is well tolerated by children and can be obtained in a noisy environment [15]. The best test for the evaluation of vocal quality and detection of change in vocal quality with treatment has yet to be determined for pediatric patients. Subjective assessment of vocal quality may be the best tool, but can lead to research difficulties due to its inherent interobserver and intraobserver variability.
pH probe The usefulness of pH probe testing for the diagnosis of significant gastropharygeal reflux is controversial. The controversy lies in the definition of significant reflux [16] and the known problem of capturing what may be an
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infrequent event during an overnight study. Because the mucosa of the larynx differs from that of the esophagus, less frequent exposure to gastric contents can cause significant changes in the larynx. The most specific test for gastropharyngeal reflux may be laryngeal biopsy [17]. The risks involved with laryngeal biopsy may make it unacceptable for use as a screening tool. Few studies have adequately assessed the role of empiric treatment of gastropharygeal reflux in the management of pediatric dysphonia. Empiric therapy for reflux may be a useful tool, however, in helping to determine the role of gastrolaryngeal reflux in causing dysphonia. Nonacidic reflux also may have a role in laryngeal disease. New tools for detected nonacidic reflux are being developed [18]. These new tools include multichannel impedance devices. This device when combined with a pH probe can detect both acidic and nonacidic reflux. Multichannel impedance measures of esophagel reflux may prove to be important in defining the significance of gastropharyngeal reflux and its role in dysphonia.
Microlaryngoscopy Successful treatment of any disorder requires an accurate diagnosis. Occasionally the correct diagnosis cannot be achieved in the office setting, and thus operative endoscopy is required. With the assistance of a general anesthetic, direct microlaryngoscopy can be performed, allowing the clinician to further visualize and palpate the larynx. Direct microlaryngoscopy is an extension of a physical examination. Biopsy is possible during this procedure as well. Examples when direct microlaryngoscopy is useful may include the differentiation of a vocal nodule and an intracordal cyst. At times it is only during exploration of the vocal fold that the correct differentiation between a vocal fold cyst or vocal fold nodule can be made. Except for the instrumentation, the technique for pediatric laryngoscopy does not differ greatly from that of adult laryngoscopy. The endoscopist must be comfortable with managing the pediatric airway, however. The pediatric patient often has less pulmonary reserve, and because of the small size of the pediatric larynx, there is less tolerance for postinstrumentation edema. Properly sized instruments and the expertise and skill to use them are required. Finally, proper anesthetic assistance and the facilities to manage potential complications must be available before pediatric endoscopy should be attempted.
Differential diagnosis and treatment Infection Edema from localized infections is the most common cause of hoarseness in children and adults. Usually self-limiting, these infections are, in most cases, mild
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in terms of systemic symptoms. Viral prodromes usually precede cough and dysphonia. Although infectious laryngitis is usually of viral origin, it also may be bacterial or, in rare instances, fungal in nature. These infections generally are more aggressive and can cause more significant respiratory symptoms as well. Acute laryngotracheobronchitis is most often caused by parainfluenza viruses I and II, and is known commonly as croup. Low-grade fever, hoarseness, and a severe barky cough with spasmodic stridor follow symptoms of a viral illness. Respiratory syncitial virus and influenza virus types A and B also may cause croup. Medical attention is more often sought due to the associated stridor than to the associated dysphonia. Regardless of the etiologic agents, croup is the clinical diagnosis. Radiographic studies may confirm the diagnosis with the pathognomonic steeple sign found by posteroanterior neck radiograph. Treatment generally is expectant with systemic steroids and observation. A cool mist may be helpful; endotracheal intubation is reserved only for severe cases with lifethreatening respiratory distress [19]. When intubation is required, extreme care must be taken to choose the appropriate-sized endotracheal tube, to avoid iatrogenic stenosis of the subglottis. Croup must be differentiated from more serious laryngotracheal infections, such as membranous laryngotracheobronchitis or epiglottitis. Children with these infections appear toxic, and often have a high fever and a high white blood cell count. Respiratory difficulty often is seen in association with the dysphonia and odynophagia. Membranous laryngotracheobronchitis may present with a prodrome similar to croup, but the child’s symptoms rapidly progress to a more toxic appearance. Neck radiographs demonstrate irregular borders in the trachea that are consistent with thick tenacious secretions. Fiberoptic nasal endoscopy shows thick purulent secretions at the glottis and subglottis, but a normal epiglottis. Occasionally, operative endoscopy is required to clear the trachea of the thick membranous secretions. In these cases, endotracheal intubation will supply a route for continued pulmonary toilet. Rarely, serial operative endoscopy is required to keep the airway clear of secretions. Culture is helpful and usually reveals Staphylococcus aureus or Haemophilus influenzae. Antibiotic therapy should be directed toward these causative organisms unless cultures and sensitivities direct differently. The introduction of the H influenzae type B vaccination has decreased dramatically the incidence of epiglottis in children [20]. Epiglottitis generally does not have a significant or prolonged prodromal period. The child rapidly appears toxic with drooling, posturing, and respiratory difficulty and dysphonia. Muffled voice and arms positioned in a tripod stance often accompany the high fever and tachypnea. The child often is most comfortable sitting upright with the neck extended and the arms forward for support, to maximize the airway. Lateral neck radiographs show a thickened epiglottis (thumb sign). If epiglottitis is suspected, the child should be taken immediately but quietly to a setting in which direct laryngoscopy and airway management is possible. The diagnosis is confirmed by the endoscopy. Once the airway is established, cultures are
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obtained. Initial antibiotic therapy should be directed against H influenzae type B. Other causative agents can include candidiasis or Streptococcus pneumoniae seen in conjunction with immunosuppressed states such as leukemia [21]. Extubation often is possible 48 hours after appropriate medical management, but should be directed by the status of the larynx.
Anatomic causes of dysphonia Cyst Mucus-filled cysts may form beneath the epithelium of the vocal folds, and cause dysphonia by increasing the mass of the body of the vocal fold. Disparity between the mass of each vocal fold will increase the effort required to begin vibration and also may make it more difficult to sustain clear phonatory sounds. Cysts may be difficult to distinguish from nodules in the pediatric larynx, because there is often a contralateral reactive vocal nodule, which gives the typical appearance of screamers nodules with symmetric bilateral thickening of the vocal fold. Subtle differences during indirect endoscopy may suggest the proper diagnosis, but intraoperative exploration of the vocal fold may be required to confirm the diagnosis and treat the intracordal cyst. In adults, the mucosal wave seen during videostroboscopy is not changed in intracordal cysts [6]. Intracordal cysts do not resolve with speech therapy. The voice may improve with speech therapy, however, because the thickening seen in reaction to the cyst may resolve with proper voice use. If significant dysphonia persists or recurs with speech therapy, microlaryngeal techniques for removal of the cyst should be considered. Care must be taken not to disrupt the integrity of the vocal ligament during excision.
Fig. 2. Vocal fold nodules can be seen opposing each other at the anterior to middle one third of the vocal fold. These nodules are discrete and appear in the immediate infraglottic aspect of the vocal fold.
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Vocal fold nodules Nodular thickening of the anterior to middle one third of the vocal folds is the most common cause of hoarseness in children (Fig. 2). Although a child occasionally may present with hoarseness from nodules at the time of their first words, most nodules develop when the child begins group activities. It generally is believed that the nodules occur from voice abuse during vocal play and social interaction. The voice worsens with aggressive vocal attack or loud play noises. Vocal nodules are more common in boys than in girls. Laryngoscopy confirms the diagnosis with thickening seen at the anterior to middle one third of the vocal ligament. The mucosal wave is diminished in vocal nodules, which is believed to be due to the increase in mass of the cover of the vocal fold [6]. Other conditions that increase the potential for vocal abuse range from hearing loss to velopharyngeal insufficiency. If these conditions are present, they should be treated. Voice therapy is the mainstay of treatment. Gastropharyngeal reflux also has been implicated in the cause of nodule formation. It is possible that gastropharyngeal reflux induces inflammation that predisposes a child to nodule formation on the larynx [4]. Nodules also may be maintained between cycles of voice abuse from inflammation caused by gastropharyngeal reflux. If reflux is suspected, empiric therapy should be used as an adjunct to voice therapy. Therapy for nodules generally is directed toward maximizing vocal hygiene and voice use. Speech therapy can help the child to identify ways to use the voice in an optimal manner. If the child’s age and maturity does not allow for mastery of proper voice use, continued observation and periodic retraining is indicated. Often simple alternatives to aggressive voice use or vocal play noises is all that is required to correct the voice abuse and allow for the nodules to resolve. There are rare occasions when a child masters proper voice use but continues to have significant vocal disturbance from nodules. Although this condition is not well studied, these children may benefit from microlaryngeal techniques to remove the nodular thickening. Care must be taken not to disrupt the vocal ligament, whichcan cause permanent scarring or tethering of the vocal epithelium to the vocal ligament and induce lifelong hoarseness. Children who cannot master proper voice use are not, as a rule, candidates for microlaryngeal removal of the nodules.
Congenital causes of dysphonia Saccular cyst Unlike intracordal cysts, saccular cysts are seen in the laryngeal ventricle or aryepiglottic fold. They may encroach on the vocal fold and cause dysphonia. Saccular cysts are ‘‘anterior’’ when confined to the anterior larynx and ventricle or ‘‘lateral’’ when extending into the aryepiglottic fold (Fig. 3). A child’s cry may be muffled from the cyst impressing on the vocal tract and the cyst also may
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Fig. 3. Operative endoscopy demonstrating a lateral saccular cyst. The feeding tube is seen entering the esophageal inlet. The cyst is seen in the aryepiglottic fold on the patient’s right side.
cause respiratory embarrassment. Awake indirect laryngoscopy is important for diagnosis and to help plan management of the airway. Although extremely rare, a supraglottic hemangioma can be mistaken for a saccular cyst. Surgical treatment depends on the size and location of the laryngeal cyst. Small anterior saccular cysts may be treated endoscopically with marsupialization. Although larger lateral saccular cysts may be approached transorally, an external approach may be needed for complete removal of the cyst. This procedure provides the lowest chance for recurrence. The approach requires entry into the periglottic space through the thyrohyoid membrane. Advanced endoscopic and open surgical techniques are used to avoid recurrence or the need for tracheotomy [22,23].
Fig. 4. Congenital laryngeal web seen at rigid endoscopy. The endotracheal tube passes easily through the posterior glottis, whereas the anterior two thirds of the vocal folds are fused together. This anterior glottic web also was associated with thickening of the cricoid cartilage and subglottic stenosis, which required expansion surgery for repair.
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Fig. 5. A congenital anterior glottic web at rigid endoscopy. The presenting symptoms were primarily voice related. The posterior glottis was relatively unaffected and thus respiration was not affected greatly.
Glottic webs Incomplete recannulization of the embryonic larynx leads to anterior glottic web formation. The extent of webbing may vary, from anterior webbing of the glottis to near complete occlusion of the laryngeal inlet (Fig. 4). Glottic webs generally present with a high-pitched and weak cry from birth. Often medical attention is not sought until the age of 2, depending on the severity of the web. Vocal disturbance is the usual presenting symptom. Interestingly, respiratory compromise may not be significant or only a minor component of the presenting symptoms. The anterior glottis is concerned mostly with phonation, whereas the posterior glottis contributes to the airway (Fig. 5). Diagnosis of glottic webs is made by office endoscopy. The treatment plan and surgical approach require rigid endoscopy to determine the extent into the subglottis. There is a temptation to treat webs endoscopically. This technique is useful in the rare thin anterior web, which does not extend into the subglottis. More often, congenital glottic webs are associated with cricoid thickening as well [24 – 26]. These cases require anterior expansion of the cricoid ring with a cartilage graft and lysis of the web at the glottis.
Inflammation Gastropharyngeal reflux Most, if not all, pathologic conditions of the larynx have been attributed to gastropharyngeal reflux at one time or another. The gastropharyngeal reflux disease process remains controversial and difficult to study in adults and children alike. Subglottic stenosis has been shown to be caused by infrequent application
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of gastric contents to the cricoid in an animal model [27]. Reversible dysphonia has been documented to respond to antireflux therapy in adults [3]. Glottic and supraglottic abnormalities attributed to gastropharyngeal reflux have been cataloged [3,12,13,16,28]. The difficulty lies in the definition and confirmation of significant gastric reflux to the level of the larynx. Because even infrequent gastric reflux can cause changes to the larynx, current diagnostic tools may miss significant events. Empiric treatment studies in adults have shown that the treatment of gastropharyngeal reflux with high-dose proton pump inhibitors often improves the quality of the voice and corrects abnormalities seen in the larynx [5]. The controlled studies performed in adults have not been duplicated in children. Studies are underway to elucidate the role of gastropharyngeal reflux in children as a contributing factor in the formation of nodules. Vocal hygiene, vocal behavioral modification, and empiric gastropharyngeal reflux treatment may be most beneficial. Changing the diet to discourage reflux and increasing the time between meals and reclining are important measures to decrease reflux. Working with a gastroenterologist who is aware of the association of reflux and laryngeal disorders often is helpful to maximize the medical management in refractory cases. There are times when antireflux surgery may be required [3].
Neoplastic causes of dysphonia Benign and malignant neoplasms of the pediatric larynx are very rare. When present, however, they may cause dysphonia. Hoarseness in a child should alert the clinician to the possibility of a neoplasm. When evaluating a hoarse child, one should worry about the worst, see what is there, and treat what is seen. Visualization of the larynx to rule out a neoplasm is as important in a child as it is in an adult. Table 2 lists the most common of the rare neoplasms seen in the pediatric larynx. Ninety-eight percent of tumors in the pediatric airway are
Table 2 Benign and malignant neoplasms of the pediatric larynx Site
Malignant neoplasm
Benign neoplasm
Epithelium
Squamous cell carcinoma Mixed tumor Adenocarcinoma Neurofibrosarcoma
Recurrent respiratory papilloma Mixed tumor Adenoma Neurofibroma Granular cell myoblastoma Neurilemoma Hemangioma Lipoma Rhabdomyoma Chondroma Fibroma Lymphangioma
Neurogenic
Connective tissue
Fibrosarcoma Rhabdomyosarcoma Chondrosarcoma Angiosarcoma
Miscellaneous
Metastatic tumors
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benign. If recurrent respiratory papilloma is considered a neoplasm of the larynx, it is then by far the most common neoplasm in the pediatric larynx, with hemangiomas and neurogenic tumors being the next most common neoplasms. Histologic diagnosis is required before treatment plans can be formulated. Voice changes in children with neoplasms are progressive and persistent, and may be associated with stridor. When respiratory compromise is associated with the symptoms of hoarseness, rapid evaluation and treatment are important.
Recurrent respiratory papillomatosis The human papilloma virus (types 6 and 11) has been found to be the causative agent for the recurrent growths seen in the pediatric larynx [29]. The virus has a predilection for the squamociliary junctions of the aerodigestive epithelium [30]. Recurrent respiratory papilloma may present with symptoms of hoarseness, snoring, dysphagia, or stridor with respiratory compromise (Fig. 6). Once the diagnosis has been confirmed by visualization and occasionally biopsy, the gold standard of treatment is periodic mechanical debridement. The carbon dioxide laser and the laryngeal microresector are the instruments most commonly used. Eradication of the viral DNA from normal-appearing tissue is not possible, and recurrence is expected [31]. Laryngeal papillomatosis has been termed juvenile recurrent respiratory papilloma because the recurrent nature of the viral eruptions decreases with increasing age. Factors regulating viral expression and recurrence are not known, but may be hormonal. Flow cytometry may give some indication with regard to the aggressive nature of the papilloma [32,33]. Although rare, respiratory papillomatosis has been associated with malignant transformation [34]. Periodic histologic analysis and viral typing is an important surveillance tool.
Fig. 6. Rigid endoscopy of a pediatric larynx with recurrent respiratory papilloma. The entire laryngeal inlet is filled with papillomata. This child presented with respiratory compromise.
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Treatment is directed at maintaining an adequate airway while preserving the structures of the larynx that are important for phonation. Frequent surgical resection of the viral papilloma is the mainstay of treatment. The carbon dioxide laser has been used to vaporize the viral eruptions, and is an excellent tool, although two disadvantages are its potential for airway fires and laser-induced thermal scarring of the larynx. Increased stiffness of the vocal fold with subsequent hoarseness has been reported when injudicial use of the laser [35]. The use of a microresector system helps to avoid deep thermal injury in extensive lesions [36]. Regardless of the technique used, care must be taken to remove only the superficial layer of the infected laryngeal mucosa so that the deep structures are not disturbed. In addition, the anterior commissure must be protected so that opposing mucosal defects are avoided. This will help to prevent the formation of an anterior web. Adjuvant medical treatments to surgical debridement have been tried, but none have been found to be convincingly effective. Further studies of current antiviral therapies are required before recommendations can be given. Inducing viral remission with limited side effects is desirable.
Neurologic causes of dysphonia Paralysis of the vocal folds may be unilateral or bilateral. The symptoms surrounding each type of paralysis depend on the kind of paralysis present. In general, bilateral vocal fold paralysis is caused by central nervous system anomalies. Unilateral paralysis generally stems from peripheral lesions or iatrogenic causes, although occasionally it may be caused from central lesions as well. Bilateral vocal fold paralysis may present with near-normal or slightly weaker vocalization. Much more prevalent is the associated high-pitched inspiratory stridor. The voice is often stronger than caregivers would expect and thus bilateral vocal fold paralysis often is not included in the differential diagnosis. The vocal folds are in the paramedian position, which is more compatible with vocalization than with breathing. Unilateral vocal fold paralysis often presents with a weak and breathy cry; respiratory distress usually is not seen. Feeding difficulties are noted with unilateral paralysis when the contralateral fold cannot compensate for the restricted movement of the paralyzed vocal fold. There also may be accompanying sensory deficits. Patients with congenital vocal fold paralysis require an extensive workup. Other neurologic deficits may point to a neurologic cause. It is rare, however, that generalized neurologic deficits cause vocal fold paralysis. Some central nervous system disorders that may contribute to vocal fold paralysis include intracerebral hemorrhage, cerebral agenesis, hydrocephalus, and Arnold-Chiari malformations of the brain stem with cerebellar herniation. The entire course of the involved recurrent laryngeal nerve should be studied. Evaluation should include chest radiographs and CT or MRI from the head to the aortic arch. Heart anomalies or surgery to correct them may cause damage to the left recurrent laryngeal nerve in
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particular, because it courses around the aortic arch. Birth trauma also can cause damage to the laryngeal nerves, and malignant neoplasms can cause paralysis of the laryngeal nerves. Occasionally, no cause is identifiable. Iatrogenic injury to the laryngeal nerve from chest or cardiothoracic neck surgery can occur even in the most accomplished hands. Recovery is possible depending on the severity of the injury. Often, no therapy is needed in unilateral paralysis, because the contralateral vocal fold may be able to overcompensate for the paralyzed vocal fold—a phenomenon that is seen much more often in children than in adults. Observation and speech therapy to help with swallowing and speech difficulties are the most common forms of treatment. Occasionally, significant aspiration or dysphonia results from a unilateral vocal fold paralysis, in which case, vocal fold medialization techniques are appropriate. Knowledge of the developing laryngeal framework is required. Use of adult techniques for medialization often results in improper placement of the medialization prosthesis [37]. No long-term series have been reported. There is a theoretic requirement for revision of the framework surgery as the laryngeal cartilages grow. Bilateral vocal fold paralysis may improve if the underlying cause of the paralysis is treated. Bilateral vocal fold paralysis often requires tracheotomy [38]; however, before the decision for tracheotomy is made, close observation and a multidisciplinary approach is required. Some groups report an incidence of tracheotomy as low as 50% in children with bilateral vocal fold paralysis [38]. If tracheotomy is required, there are surgical techniques to expand the glottic inlet in hopes of decannulation. The specific techniques used vary and depend on the anatomy seen. Modification of the laryngeal framework generally is deferred until the age of 2. Expansion of the airway is performed at the expense of glottic closure. This widening of the posterior glottic gap may cause worsening of the voice or an inability to swallow. Endoscopic techniques include partial or complete arytenoidectomy, although posterior cordotomy also may be sufficient. In addition, laryngotomy with arytenoidectomy or arytenoidopexy has had acceptable results [39]. Expansion of the glottic inlet by inserting a cartilage graft into the posterior cricoid framework increases the glottic airway without disturbing the membranous vocal folds [40]. Nerve-muscle pedicles have had disappointing results. Regardless of the technique chosen, care must be taken to balance increasing the airway for breathing with maintaining the ability to vocalize and swallow without aspiration.
Iatrogenic causes of dysphonia Prevention is the best form of treatment for iatrogenic causes of dysphonia. When they occur, however, correct identification of the cause is required before repair can occur. Subglottic stenosis, isolated from the glottis, may cause dysphonia because of changes in airflow and subglottic pressure across the vibrating vocal folds. Repair of the subglottic stenosis can be achieved with expansion of the subglottic unit or resection of the stenosis. Improvement of the
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voice correlates with increased airflow across the glottis. A transglottic stenosis with incorporation of the vocal folds into the scar often leads to poor voice quality even after repair. Quality sound production requires supple vocal fold mucosa to vibrate over the body of the vocal folds themselves. If scarring occurs to the vocal folds, correction of the supple nature of the mucosa is difficult. A usable voice often requires use of other portions of the larynx, most often the supraglottis. Arytenoid dislocation or subluxation is a rare cause of dysphonia, but should be suspected in children with persistent dysphonia after extubation. The diagnosis is suspected with decreased mobility of the vocal fold seen by indirect laryngoscopy, and is confirmed by direct laryngoscopy with palpation of the cricoarytenoid joint. Mobilization is possible after diagnosis during the endoscopy. Although arytenoid subluxation may be confused with vocal fold paralysis, palpation during endoscopy can help to differentiate between the two. Speed, from suspicion to diagnosis and treatment, may be important to avoid fixation of the cricoarytenoid joint.
Summary Vocal disturbances in children are surprisingly common. Typical pathologic conditions differ, however, from those that are seen commonly in adults. Disturbances in the vibratory characteristic of the vocal folds cause dysphonia. The most common causes for dysphonia are infectious, anatomic, congenital, inflammatory, neoplastic, neurologic, or iatrogenic in nature. A child who presents with hoarseness demands a rapid and thorough assessment. Fine points during the history may provide the clinician with clues as to the correct etiologic category. Proper treatment requires an accurate diagnosis. Although some conditions are similar in the adult larynx, treatment of the pediatric larynx often differs. An understanding of the changing and growing pediatric larynx is necessary for treatment and satisfactory results.
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