Common causes of hoarseness in children

INTRODUCTION — 

Common causes of hoarseness in children (table 1) and an overview of their management will be reviewed here. Other related issues, including laryngeal anatomy, physiology of phonation, and evaluation of the child with hoarseness, are discussed separately. (See "Hoarseness in children: Evaluation".)

OVERVIEW — 

"Hoarseness" or "dysphonia" are terms used to describe a change in the quality of the voice. The voice quality can be raspy, breathy, strained, fatigued, rough, tremulous, or weak. There may be a change in pitch, restriction of range, voice breaks, decreased projection, or abnormal resonance. The prevalence of hoarseness in children ranges from 4 to 23 percent [1-3]. Hoarseness can be caused by any process that affects the structure or function of the larynx. (See "Hoarseness in children: Evaluation", section on 'Physiology'.)

Etiologic categories of hoarseness include infection, inflammation, trauma, obstruction or infiltration, and congenital anomalies (table 1) [4]. In children, hoarseness is most often due to a benign and self-limited cause (eg, viral upper respiratory infection or vocal abuse and misuse) and can be managed with education, watchful waiting, and voice therapy. In addition, the growth of the vocal folds and laryngeal apparatus, change in habits, and change in the hormonal milieu that occur during puberty often contribute to improved voice quality. Surgery is reserved for persistent lesions with noted anatomic alterations.

MUCOSAL LESIONS — 

Hoarseness in children is most often caused by benign lesions of the vocal folds (eg, nodules, polyps, hemorrhage, hematoma).

Nodules — Vocal fold nodules are the most common cause of chronic hoarseness in school-aged children [5]. Vocal fold nodules usually are located on the anterior-free edge of the vocal fold at the point of greatest amplitude of vibration (the junction of the anterior one-third and the posterior two-thirds of the vocal fold) (picture 1). They develop from repeated trauma and abuse to the vocal folds (eg, screaming or shouting) that cause an inflammatory reaction with fibrotic healing. Vocal fold nodules are bilateral and can range in size from slightly raised hyperkeratotic lesions to larger broad-based lesions that prevent closure of the vocal folds. Stroboscopy can best examine and define the nature of laryngeal lesions. In the case of vocal fold nodules, the mucosal wave should remain intact. Nodules can be confused with mid-vocal fold fibrotic lesions and congenital lesions with contralateral reactive lesions.

The symptoms of vocal nodules vary depending upon the size. Small lesions may cause mild raspiness and roughness of the voice, whereas large lesions may cause breathiness because of incomplete closure of the vocal folds.

Treatment decisions are guided by the needs assessment of the child. Intervention is warranted if there are social problems such as teasing, withdrawal, or self-image issues because of the vocal quality or if there are problems with others understanding the child's speech, particularly in school. Absent these issues, watchful waiting is acceptable since many of these voice issues resolve with growth.

Voice therapy is the most widely accepted treatment for vocal fold nodules. Resolution or significant improvement typically is seen within three to six months [6-8]. Failure to resolve or progression with therapy suggests possible misdiagnosis and warrants laryngeal stroboscopy. Microlaryngeal surgery may be indicated for nodules that cause persistent and severe vocal dysfunction [7,9]. Children who require surgery also should receive voice therapy to address the underlying vocal abuse or misuse because vocal fold nodules can recur in as little as three days if vocal habits are not changed.

Vocal fold nodules can be accompanied by other lesions of the vocal folds including hemorrhage, vascular varices, and hematoma [10]. The pathogenesis of these lesions is similar to that of vocal fold nodules: vocal abuse leading to inflammatory reaction with fibrotic healing (picture 2). Congenital cystic changes and or ruptured cysts also often present with bilateral mid-vocal fold swelling. A lesion on one side causes trauma on the contralateral side, resulting in bilateral swellings, often offset from each other.

The treatment of these processes mirrors that of vocal fold nodules, with surgery reserved for recalcitrant cases.

Polyps — Vocal fold polyps occur rarely in children. Vocal fold polyps are fluid- and fibrin-filled lesions that occur at the free edge of the vocal fold in the superficial layer of the lamina propria and thus differ from nodules that affect the epithelial layer. These lesions tend to appear unilaterally and usually are located at the junction of the anterior one-third and posterior two-thirds of the vocal folds (picture 3). Trauma, chronic irritation, reflux, smoking, and muscle tension dysphonia may contribute to their formation. Vocal fold hemorrhages can organize into vocal fold polyps. The symptoms of vocal fold polyps include hoarseness, diplophonia (having two different voice tones at the same time), raspiness, and breathiness. Surgical treatment is often necessary, depending on the severity of the voice complaint, although voice therapy is usually attempted.

Muscle tension dysphonia — Children who have a strained, easily tired voice may have some degree of muscle tension dysphonia. Muscle tension dysphonia occurs when the muscles of the larynx attempt to compensate for mucosal abnormality by contracting abnormally. This can lead to a vicious cycle of microtrauma to the vocal cords and resultant dysphonia, which in turn leads to the recruitment of additional counterproductive vocal mechanisms characterized by strain, excess muscle tension, and glottic compression. The end result is a narrowing of the arytenoids, false vocal folds, true vocal folds, or the entire larynx. Muscle tension dysphonia is treated with voice therapy and a change in the vocal habits.

VOCAL FOLD GRANULOMA — 

Vocal fold granulomas are highly vascular lesions of nonspecific granulation tissue that usually appear in the posterior glottis associated with one and, occasionally, both arytenoids. Vocal fold granulomas occur most often secondary to intubation-associated trauma and prolonged intubation [11] but also occur in association with laryngopharyngeal reflux (LPR) disease, laryngeal trauma, habitual throat clearing, and vocal misuse [12,13]. LPR, which differs somewhat from gastroesophageal reflux (GER), is thought to be an important factor in the development and prolongation of vocal fold granuloma. (See 'Gastroesophageal reflux/laryngopharyngeal reflux' below and "Gastroesophageal reflux disease in children and adolescents: Clinical manifestations and diagnosis".)

Children with vocal fold granulomas usually present with hoarseness, diplophonia, breathiness, dysphagia, vocal fatigue, cough, or the feeling of a lump in the throat (globus sensation).

The treatment of vocal fold granulomas depends upon the cause. Voice therapy can help to reverse or prevent poor vocal behaviors. Management of LPR and GER is essential for patients in whom reflux is a contributing factor. Microlaryngeal surgery is reserved for lesions that obstruct the airway and persist despite optimal medical intervention. (See "Gastroesophageal reflux disease in children and adolescents: Management".)

VOCAL FOLD PARALYSIS — 

Vocal fold paralysis is one of the more common laryngeal abnormalities of the larynx and cause of stridor in neonates and infants. Iatrogenic causes are the most common etiology (eg, surgery resulting in injury to the recurrent laryngeal nerve [RLN]). Bilateral vocal fold paralysis occurs with approximately the same frequency as unilateral vocal fold paralysis [14]. Children with vocal fold paralysis have a high rate of spontaneous return of function, particularly if the paralysis is unilateral [14,15].

●Causes – The causes of vocal fold paralysis in children include [14]:

•Iatrogenic, most commonly associated with cardiothoracic surgery, invasive mechanical ventilation, thyroidectomy, and tracheoesophageal fistula repair [16-19]

•Idiopathic, probably the result of viral infection and autoimmune disease

•Neurologic, including Arnold-Chiari malformation, posterior fossa tumor, meningomyelocele, cerebral agenesis, and hydrocephalus (see "Chiari malformations" and "Clinical manifestations and diagnosis of central nervous system tumors in children" and "Myelomeningocele (spina bifida): Anatomy, clinical manifestations, and complications" and "Hydrocephalus in children: Clinical features and diagnosis")

•Injury or compression of the RLN, which may occur because of stretching of the neck during breech delivery or because of intrathoracic lesions (eg, aberrant great vessels, left heart failure, tumors) (see "Vascular rings and slings" and "Neonatal birth injuries", section on 'Laryngeal nerve injury')

●Clinical features – Newborn infants with unilateral vocal fold paralysis may have an absent or weak cry. Signs and symptoms of aspiration may be present (eg, coughing with swallowing and recurrent pneumonia). Children with unilateral vocal fold paralysis typically present with decreased volume and power of voice and hoarseness that has a breathy, raspy quality [17].

Infants with bilateral vocal fold paralysis may be asymptomatic for the first six months of life. This is because the vocal folds tend to assume a midline position in bilateral paralysis (picture 4), which protects the airway and permits a normal cry. However, as the child's activity level (and oxygen requirement) increases, symptoms such as dyspnea and stridor become more apparent. Concomitant upper respiratory tract infection or edema of the vocal folds may cause abrupt airway compromise, resulting in respiratory distress and stridor. (See "Assessment of stridor in children".)

●Diagnosis – The diagnosis of vocal fold paralysis can be confirmed by flexible nasolaryngoscopy in the awake infant or child. Direct laryngoscopy in the operating room may be necessary if the child is difficult to examine or if the larynx is poorly visualized or used to rule out other abnormalities of the glottic and subglottic region. In patients with unilateral vocal fold paralysis, the ability of the contralateral vocal fold to compensate and the degree of posterior glottic opening (or chink) should be evaluated. In all patients, the presence of additional anatomic pathology should be noted.

●Subsequent evaluation – Children with vocal fold paralysis should undergo additional evaluation to determine the underlying etiology. A chest radiograph and barium swallow should be performed to rule out abnormalities of the chest and esophagus. Magnetic resonance imaging of the head and neck and computed tomography of the chest may be necessary to rule out associated neurologic and thoracic abnormalities along the course of the RLN. Electromyography, although difficult to perform in children and not universally available, may provide additional diagnostic and prognostic information [20,21]. Additionally, if chronic cough, recurrent pneumonia, or choking episodes are suspected, modified barium swallow or flexible endoscopic evaluation of swallowing should be performed to rule out aspiration. (See "Aspiration due to swallowing dysfunction in children", section on 'Evaluation'.)

●Management of unilateral paralysis – The need for intervention in children with unilateral vocal fold paralysis is based upon the degree of hoarseness and the risk of aspiration. Voice and speech therapy can be used to teach the child to compensate with the nonaffected vocal fold. Most children improve with voice therapy. In cases of mild aspiration, swallow therapy may help to improve compensation.

Children who have gross aspiration and severe phonatory difficulty may require surgical intervention to medialize the paralyzed vocal fold. Medialization permits approximation of the vocal folds to protect the airway and improve voice quality. The medialization procedure varies depending upon the expected duration of paralysis, as follows:

•If short-term paralysis is expected (eg, secondary to nerve trauma during surgery), the vocal fold can be injected with hyaluronic acid, methylcellulose, Gelfoam, fat, or collagen to provide temporary medialization. These procedures are typically done in the operating room with general anesthesia. Teenagers can have these procedures done awake in a procedure room.

•Medialization thyroplasty can be performed in children with long-term vocal fold paralysis. In this procedure, a small window is created in the thyroid cartilage at the level of the vocal folds. Silastic, Gore-Tex, or hydroxyapatite material is then inserted into the window to help medialize the vocal fold.

•RLN reinnervation is another accepted form of treatment for children with vocal fold paralysis due to RLN injury. If the paralysis has lasted longer than one year or the nerve confirmed transected, the ansa cervicalis on the ipsilateral side can be anastomosed to the RLN as it enters the larynx. This does not provide mobility but does provide bulk and tone to the affected vocal fold. A temporary injection is typically done at the time of surgery to provide closure while the nerve regenerates.

●Management of bilateral paralysis – In most children with bilateral vocal fold paralysis, the vocal folds meet in the midline. Chronic respiratory insufficiency and stridor, rather than aspiration, are the major symptoms. The child's voice/cry can be normal. Surgical procedures are necessary to lateralize, or open, the vocal folds. The goal of treatment is to provide an adequate airway while preventing aspiration. Tracheotomy is required in 50 to 60 percent of patients [15,22]. Other surgical techniques that should only be considered in children with permanent vocal fold paralysis include laser arytenoidectomy, cordotomy, and vocal fold lateralization. These procedures are usually performed after tracheotomy in an attempt to achieve a functional voice and an adequate airway.

LARYNGEAL INFLAMMATION

Acute laryngitis — Acute laryngitis is defined as an inflammation of the mucosa of the larynx. The two most common causes of laryngitis in children are:

●Viral respiratory tract infections – In addition to dysphonia, a child with a viral illness may have a low-grade fever, malaise, myalgias, rhinorrhea, cough, and mild dysphagia. Mouth breathing secondary to nasal congestion also can cause drying of the vocal fold mucosa and resultant hoarseness [5]. Secondary bacterial infections are rare but should be considered in children with high fever, purulent exudate, or progressive pain.

●Vocal strain – Prolonged or repeated vocal strain can cause submucosal vocal cord hemorrhage and edema with resultant hoarseness. The history usually reveals the inciting event (eg, screaming at sports event, repeated loud singing).

Acute laryngitis is usually a self-limited process and is treated conservatively with hydration, humidification, voice rest, and reassurance. Whispering should be avoided since it can increase the laryngeal trauma. (See "The common cold in children: Management and prevention".)

Acute laryngotracheitis — Acute laryngotracheitis (croup) is a common cause of acute hoarseness and stridor in children <6 years old. Symptoms usually begin with nasal discharge and congestion and progress over 12 to 48 hours to include fever, hoarseness, barking cough, and stridor. The clinical features, diagnosis, and management of croup are discussed in detail separately. (See "Croup: Clinical features, evaluation, and diagnosis", section on 'Clinical presentation' and "Croup: Management".)

Chronic laryngitis — Chronic laryngitis is defined as hoarseness, dysphagia, dysphonia, and vocal fatigue lasting >3 months. Children with chronic laryngitis may present with a chronic cough or chronic throat clearing. Characteristic findings in the larynx include thickened, tenacious mucus; edema of the mucosa of the false and true vocal folds; and thickening of the epithelial lining of the mucosa.

In contrast with acute laryngitis, chronic laryngitis usually has a noninfectious etiology. Chronic laryngitis can be caused by the following factors:

●Exposure to environmental irritants (dry air, solvents, industrial pollution, and some household products)

●Environmental allergy (new products, dust, cockroach feces, and other allergens)

●Chronic sinusitis with postnasal drip

●Medications (eg, inhaled steroids, anticholinergics, and antihistamines)

●Chronic dehydration

●Laryngopharyngeal reflux (LPR) (see 'Gastroesophageal reflux/laryngopharyngeal reflux' below)

●Chronic systemic disease (eg, untreated or undertreated hypothyroidism, sarcoidosis, amyloidosis, granulomatosis with polyangiitis) (see 'Endocrine disorders' below)

●Malignancy (eg, lymphoma, sarcoma) (see 'Malignant tumors' below)

The treatment of chronic laryngitis depends upon the underlying etiology and may involve avoidance of environmental irritants and allergens, treatment of allergic rhinitis, treatment of sinusitis, humidification, increased hydration [23], and correction of any underlying systemic or metabolic disorders. Stroboscopy and possible biopsy of the larynx might be indicated with nonresponders.

GASTROESOPHAGEAL REFLUX/LARYNGOPHARYNGEAL REFLUX — 

Gastroesophageal reflux (GER) and laryngopharyngeal reflux (LPR) can cause hoarseness in children [24]. Children may present with hoarseness, dysphagia, chronic cough, recurrent vomiting, chronic throat clearing, feeling of a lump in the throat (globus sensation), bitter taste in the mouth, and occasional feeling of respiratory obstruction secondary to laryngospasm. (See "Gastroesophageal reflux disease in children and adolescents: Clinical manifestations and diagnosis", section on 'Clinical manifestations' and "Approach to chronic cough in children".)

LPR alone rarely causes voice changes, and laryngoscopy with stroboscopy should be performed to rule out concurrent lesions including vocal fold nodules (picture 1), polyps (picture 3), granuloma, or edema; laryngeal or tracheal stenosis; laryngospasm; subglottic edema; mucosal erythema, hyperemia, hypertrophy, or granuloma; laryngeal edema; and thick mucus [25].

The diagnosis and management of LPR/GER disease are discussed separately. (See "Gastroesophageal reflux disease in children and adolescents: Clinical manifestations and diagnosis", section on 'Diagnostic approach' and "Gastroesophageal reflux disease in children and adolescents: Management", section on 'Treatment approach by presenting symptoms'.)

CONGENITAL ANOMALIES — 

Congenital anomalies of the larynx that cause hoarseness include webs (picture 5), clefts, cysts (picture 6), and hemangiomas (picture 7A-B). These are discussed in detail separately. (See "Congenital anomalies of the larynx" and "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications".)

PSYCHOGENIC CAUSES — 

Psychogenic dysphonia refers to a variety of voice disorders related to anxiety, depression, trauma history, personality disorders, and conversion reactions. These disorders can manifest with aphonia, whispering, high-pitched voicing with voice breaks, or a strained voice. Psychogenic dysphonia is usually a defense mechanism or an adaptive behavior. The disorder may manifest after a traumatic event or illness or during the anniversary of a traumatic event (eg, the death of a parent or relative). (See "Somatic symptom disorder: Epidemiology, clinical features, and course of illness" and "Functional neurological symptom disorder (conversion disorder) in adults: Clinical features, assessment, and comorbidity".)

Children who have psychogenic dysphonia typically have a normal cry, laugh, and cough. Psychogenic dysphonia is a diagnosis of exclusion. Patients must have a normal physical examination, including laryngoscopy. The treatment of psychogenic dysphonia centers on the diagnosis and treatment of the underlying psychologic disorder [26].

Paradoxical vocal fold motion — Paradoxical vocal fold motion (PVFM; also referred to as "vocal cord dysfunction," "inducible laryngeal obstruction," "psychogenic stridor," "Munchausen stridor," "factitious asthma," and "pseudo-asthma") typically presents with recurrent acute episodes of dyspnea and stridor. Patients may complain often with throat tightness, choking sensation, dysphonia, and cough. The disorder may begin at any age but most commonly presents during adolescence. Children with this disorder may have a history of prior psychiatric illness, including depression, personality disorder, or posttraumatic stress disorder or they may have a history of childhood sexual abuse. Children and adolescents with PVFM have a normal laugh and cry and resolution of the stridor during sleep or, in some cases, when the patient is unaware of being observed. Patients may present with significant respiratory distress and dramatic inspiratory stridor. Adventitious sounds are loudest above the throat and are less audible through the chest wall, where the sound is attenuated by transmission through the airways and the pulmonary parenchyma. Because PVFM can coexist with true asthma, a thorough evaluation for asthma is essential. Treatment of PVFM is directed at the underlying psychologic etiology and involves psychotherapy coupled with respiratory retraining therapy or laryngeal control therapy, a specialized type of voice therapy. PVFM is discussed in greater detail separately. (See "Inducible laryngeal obstruction (paradoxical vocal fold motion)".)

Puberphonia — Puberphonia, or mutational falsetto, is a disorder that occurs around puberty. It is defined by the failure of the voice to change from the higher pitch of early childhood to the lower pitch of adulthood and may be caused by a maladaptation to the psychologic changes of puberty. Muscular incoordination, hyperfunction of the cricothyroid muscle, and psychologic uncertainty are contributing factors. Children with puberphonia often have associated breathiness and decreased power of projection. Puberphonia is usually treated with voice therapy and problem-directed psychotherapy.

ENDOCRINE DISORDERS — 

Voice changes can occur as a result of endocrine disorders or hormonal changes:

●Hypothyroidism – Patients with hypothyroidism may have a coarse, raspy, breathy, and low-pitched voice resulting from myxedema of the vocal folds that alters vocal fold vibration. Proper hormone replacement and voice therapy can lead to improvement.

●Hyperandrogenism – Increased endogenous androgen levels or exogenoud hormonal therapy with androgen activity occasionally can cause a coarse, low-pitched, raspy, or breathy voice. Monthly voice variation with the onset of menses can sometimes occur. Alteration of hormonal therapy, treatment of the underlying abnormality, and voice therapy can lead to improvement.

TRAUMA — 

Repetitive trauma caused by vocal abuse can result in vocal nodules, polyps, and scarring, as discussed above. (See 'Mucosal lesions' above.)

In addition, the mucosa of the respiratory tract, including the larynx, can be injured through exposure of the respiratory tract to caustic or noxious substance, and the larynx can be injured by blunt or penetrating trauma. (See "Emergency evaluation of acute upper airway obstruction in children", section on 'Trauma'.)

Mucosal injury — Exposure injury to the respiratory mucosa through caustic ingestion, inhalation injury, and exposure to noxious chemicals and fumes can cause acute hoarseness, loss of voice, pain, and, in severe cases, stridor and respiratory distress. (See "Assessment of stridor in children".)

Immediate attention to the respiratory status in patients with respiratory exposure is of primary importance. Patients who have stridor, drooling, nasal flaring, retractions, or tachypnea or are using accessory muscles to breathe are at risk for life-threatening airway obstruction and must be managed accordingly [5]. Patients without significant respiratory distress can undergo flexible fiberoptic laryngoscopy in a more controlled setting to evaluate the extent of mucosal injury, which can range from erythema or edema to ulceration, burns, and even perforation.

The management of mucosal injury depends upon the severity and can range from humidification and voice rest to immediate intubation or tracheotomy. Caustic laryngeal injury can lead to scarring, web formation, and glottic and subglottic stenosis, all which can cause chronic dysphonia. Surgical repair is possible in some cases; however, results are poor secondary to decreased ability to heal and chronic scarring.

Intubation injury — During intubation, a child's airway is particularly susceptible to injury from endotracheal manipulation. The relatively small diameter of the larynx and loose approximation of the mucosa to the underlying soft tissue permits early-onset edema and airway compromise. Vocal fold edema and subglottic edema are common in children who have been intubated for more than 24 to 48 hours. It can cause pain, breathiness, raspiness, and a change in pitch after extubation. The vocal folds also can be injured during insertion of a nasogastric tube.

More serious laryngeal injuries resulting from intubation (eg, ulceration, lacerations, granulomas) are rare. However, they can lead to subglottic and glottic stenosis, which can cause respiratory distress, stridor, and hoarseness. Minimizing intubation time, using smaller-sized endotracheal tubes with low pressure and high-volume cuffs, and avoiding cuff pressures >20 cm H₂O should be encouraged when possible. (See "Technique of emergency endotracheal intubation in children", section on 'Cuffed versus uncuffed'.)

Blunt neck trauma — Blunt neck trauma in children is most often because of motor vehicle collisions, bicycle collisions, sports activity, falls, and fights. Blunt neck trauma can injure any of the anatomic structures of the larynx that are necessary for voice production. Children who have sustained blunt neck injury should also be evaluated for associated injuries to the head, cervical spine, and chest [27]. (See "Hoarseness in children: Evaluation", section on 'Anatomy' and "Evaluation and acute management of cervical spine injuries in children and adolescents" and "Thoracic trauma in children: Initial stabilization and evaluation".)

Children who have sustained blunt neck trauma may present with voice change (breathy, raspy), hemoptysis, or frank respiratory distress. Immediate evaluation of the cervical airway is of utmost importance. Children with respiratory distress, crepitus, subcutaneous emphysema, stridor, tracheal deviation, cervical spine tenderness, or asymmetric carotid pulses require immediate establishment of a patent airway, stabilization of the cervical spine, intravenous access, and surgical consultation [27]. Patients without indications of potential life-threatening injury can undergo flexible fiberoptic laryngoscopy in a more controlled setting to evaluate the airway for edema, hematoma, and laceration and the vocal folds for mobility.

The management of children with blunt neck trauma depends upon the severity of injury:

●Children with any of the laryngoscopic findings described above should be further evaluated with computed tomography of the neck with fine cuts through the larynx, which will determine the need for further intervention and possible surgical repair.

●Children with normal vocal fold movement and no evidence of mucosal injury should be monitored closely for the development of respiratory symptoms. Humidification, oxygen saturation monitoring, and serial examination with flexible fiberoptic laryngoscopy will determine the need for further intervention during a period of observation. Injuries to the airway may not be appreciated upon initial physical examination and may progress to cause airway obstruction as late as 48 hours after the injury [27].

Penetrating trauma — Penetrating trauma to the neck requires immediate evaluation, control of the airway, and urgent evaluation by an otolaryngologist. Respiratory distress, vascular compromise, and esophageal injury are potential sequelae of penetrating neck trauma. Complete evaluation may include flexible fiberoptic laryngoscopy, barium esophagram, angiography, and operative endoscopy and exploration.

TUMORS

Recurrent respiratory papillomatosis (RRP) — Recurrent respiratory papillomatosis (RRP) is the most common benign laryngeal tumor in children.

●Cause – RRP is thought to be caused by acquisition of human papillomavirus (HPV) during passage through the birth canal of an infected mother [28]. HPV6 and HPV11 are most commonly involved, although HPV16 has been rarely observed and may be associated with an increased risk of malignant transformation. (See "Human papillomavirus infections: Epidemiology and disease associations", section on 'Recurrent respiratory papillomatosis'.)

●Prevention – HPV vaccination may reduce the burden of RRP. This is discussed separately. (See "Human papillomavirus vaccination", section on 'Other HPV-associated disease'.)

●Risk factors – Risk factors for RRP include being the firstborn child, having undergone vaginal delivery, and having a teenage mother who has genital condylomata [28-30]. In one large retrospective cohort study, the likelihood of RRP was >200 time greater among infants born to mothers with a history of genital warts compared with infants born to mothers without such a history [31]. However, even among infants born to mothers with a history of genital warts, the absolute risk of RRP was very low (<1 percent).

●Presentation – RRP is typically diagnosed between the ages of two and three years, and most children are diagnosed before five years of age [32,33]. Presenting symptoms include hoarseness, breathiness, occasional respiratory distress, intermittent stridor, and aphonia. Airway examination typically reveals multiple verrucous, polypoid growths overlying the true vocal folds, false vocal folds, subglottic region, and trachea.

●Management – The treatment of RRP usually involves microlaryngeal surgery to debulk the lesions [34,35]. The authors of this topic review generally prefer potassium titanyl phosphate laser and/or microdebrider to remove lesions of the laryngotracheal airway. Most children with RRP require multiple surgical treatments before puberty (when RRP often, though not always, regresses), particularly if they were diagnosed before the age of three years [32,33]. In one review of 399 children with juvenile-onset RRP, the mean number of surgical procedures per child was 4.4 per year (range 0 to 19) [33]. The time interval between surgical procedures depends upon disease severity.

Rarely, tracheotomy may be required in patients with severe airway compromise due to aggressive lesions. However, tracheotomy may be associated with an increased risk of recurrence and development of lower airway papillomas, so it is generally avoided in less severe cases [32].

Investigational treatments for RRP include interferon, photodynamic therapy, carbinol, acyclovir, vitamin A, pulsed-dye laser therapy, and others [32,36-38]. Cidofovir, an antiviral agent, has been used to treat RRP by injecting the base of the papillomas after they are resected. Anecdotal evidence supports some benefit, with a decrease in both the frequency and severity of recurrence, although one small randomized trial showed no benefit [39-42]. Bevacizumab, a vascular endothelial growth factor inhibitor, has been used as an adjunct to debulking these tumors [43]. Early evidence shows that its use leads to a decreased rate and severity of recurrence.

Management of RRP should include comprehensive education regarding the condition and information regarding support groups (eg, the RRP Foundation) [37,44].

Other benign tumors — Other benign tumors of the larynx (eg, hemangiomas (picture 7A), cystic hygromas, and neurofibromas) are rare causes of hoarseness in children. Benign laryngeal tumors are usually slow-growing and cause symptoms through interference with vocal fold mobility, compression of surrounding structures, and occlusion of the airway. Symptoms include breathiness, raspiness, voice breaks, altered pitch, stridor, and respiratory compromise. The diagnosis of benign tumors relies upon flexible fiberoptic laryngoscopy, stroboscopy, and possible operative laryngoscopy. Computed tomography scan or magnetic resonance imaging with angiography of the neck with fine cuts through the larynx can help to delineate the extent of the tumor and the possible etiology. Management of benign laryngeal tumors often involves surgical excision. The exception is infantile hemangiomas, which can be treated with propranolol, as discussed separately. (See "Infantile hemangiomas: Management", section on 'First-line therapy'.)

Malignant tumors — Malignant tumors of the larynx are exceedingly rare in childhood. Malignant tumors can be associated with rapid progression of stridor and airway obstruction. (See "Assessment of stridor in children".)

Rhabdomyosarcoma is the most common childhood malignant tumor to affect the larynx, followed by other members of the sarcoma group. Other tumors that occur in the pediatric larynx include squamous cell carcinoma, chondrosarcoma, lymphoma, plasmacytoma, mucoepidermoid carcinoma, metastatic carcinoma, and neuroectodermal tumors. (See "Rhabdomyosarcoma in childhood and adolescence: Epidemiology, pathology, and molecular pathogenesis".)

The diagnosis is based upon laryngoscopic examination and biopsy. Radiographic evaluation including computed tomography, magnetic resonance imaging, and barium swallow are commonly required. The treatment is based upon tumor histology.

INFORMATION FOR PATIENTS — 

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword[s] of interest.)

●Basics topic (see "Patient education: Laryngitis (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Most common causes – In children, hoarseness is most often due to a benign and self-limited cause (eg, viral upper respiratory infection or vocal abuse/misuse) and can be managed with education, watchful waiting, and voice therapy. (See 'Overview' above.)

•Benign vocal cord lesions – Benign lesions of the vocal folds (eg, nodules (picture 1), polyps (picture 3), hemorrhage, hematoma) develop from repeated trauma and abuse to the vocal folds (eg, screaming or shouting). Vocal fold granulomas may be related to vocal misuse, habitual throat clearing, laryngeal trauma, previous endotracheal intubation, or laryngopharyngeal reflux. (See 'Mucosal lesions' above and 'Vocal fold granuloma' above.)

•Laryngitis – Viral upper respiratory infections (URIs) often cause inflammation of the mucosa of the larynx (laryngitis), which may manifest as hoarseness in association with other URI symptoms (eg, rhinorrhea, cough, sore throat). (See 'Acute laryngitis' above.)

●Other causes – While acute laryngitis and vocal strain are the most common causes of hoarseness in childhood, there are a wide range of conditions that can affect the structure or function of the larynx, resulting in hoarseness (table 1):

•Congenital anomalies – Congenital anomalies of the larynx that cause hoarseness include webs (picture 5), clefts, cysts (picture 6), and hemangiomas (picture 7A-B). These typically present in infancy. (See "Congenital anomalies of the larynx".)

•Vocal fold paralysis – Vocal fold paralysis may be unilateral or bilateral (picture 4). Iatrogenic causes are the most common etiology (eg, surgery resulting in injury to the recurrent laryngeal nerve). There is a high rate of spontaneous return of function, particularly if the paralysis is unilateral. (See 'Vocal fold paralysis' above.)

•Gastroesophageal reflux and laryngopharyngeal reflux – Children with hoarseness due to reflux may present with hoarseness, dysphagia, chronic cough, recurrent vomiting, chronic throat clearing, feeling of a lump in the throat (globus sensation), or a bitter taste in the mouth. (See 'Gastroesophageal reflux/laryngopharyngeal reflux' above and "Gastroesophageal reflux disease in children and adolescents: Clinical manifestations and diagnosis", section on 'Clinical manifestations'.)

•Psychogenic causes – Psychogenic dysphonia refers to a variety of voice disorders related to anxiety, depression, trauma history, personality disorders, and conversion reactions. It is a diagnosis of exclusion. (See 'Psychogenic causes' above.)

•Hormonal causes – Hormonal causes of hoarseness include hypothyroidism and hyperandrogenism. (See 'Endocrine disorders' above.)

•Trauma – Mucosal injury (eg, caustic ingestion, inhalation injury), intubation injury, and blunt or penetrating neck trauma may result in acute or chronic hoarseness in children. (See 'Trauma' above.)

•Tumors

-Recurrent respiratory papillomatosis (RRP) is the most common benign laryngeal tumor in children. It is thought to be caused by acquisition of human papillomavirus (HPV) during passage through the birth canal of an infected mother. (See 'Recurrent respiratory papillomatosis (RRP)' above.)

-Other benign tumors of the larynx that may cause hoarseness include hemangiomas (picture 7A), cystic hygromas, and neurofibromas. (See 'Other benign tumors' above.)

-Malignant tumors of the larynx are exceedingly rare in childhood. Rhabdomyosarcoma is the most common childhood malignant tumor to affect the larynx. (See 'Tumors' above.)