Craig R.

Villari

Melissa M. Statham

Multiple infectious and benign conditions can affect

laryngeal biomechanics and detrimentally affect laryn
geal function and vocal performance. A variety of clini
cal presentations is possible ranging from dysphonia or
dysphagia to airway compromise depending on pathol
ogy, the affected laryngeal subsite(s), and premorbid
laryngeal anatomy. Treatment is targeted to the specific
pathology, which is usually diagnosed from a thorough
history, physical examination, and detailed laryngoscopy,
but may also require more specific laboratory or radio
logic examination .

INFECTIONS

OF

THE

LARYNX

Viral Laryngitis
The most common cause of infectious laryngitis isviral
(1). Viral laryngitis is typically self-limited with a normal
dura tion of 5 to 7 days (2). Patients are usually
dysphonic but may also present with odynophagia.
History may include a viral prodrome with upper
respiratory tract symptoms and physical examination
usually demonstrates edema tous, erythematous vocal
folds (Fig. 67.1) with loss of normal vibratory
pliability.Treatment includes supportive care with
hydration and removal of laryngeal phonotory trauma
(phonation and coughing, pollutants). The most common
viral pathogens in the upper respiratory tract include
rhinovirus, influenza A, B, C, and parainfluenza viruses.
Patients with substantive vocal fold edema from viral
laryngitis are at increased risk of repetitive phono trauma
leading to more significant vocal fold injury, such as
midmembranous vocal fold lesions, epithelial and sub
epithelial trauma/ulceration, and scar (3). As such, these
patients should ideally be limited to relative or absolute
voice rest. Evidence suggests that anti-inflammatory
medi cation may decrease subjective discomfort and
decrease odynophagia, but one would not expect such
treatment to decrease duration of illness as it could not
affect the

978

underlying viral etiology (4). Systemic corticosteroids may be

utilized judiciously to treat moderate to severe laryngeal edema
associated with very substantial symptoms, espe cially in patients
with significant vocal demands that can not be mitigated with
behavioral modification. Antibiotics are not indicated in patients
presenting with symptoms typical of viral laryngitis (1). Acute
dysphonia lasting lon ger than 2 weeks is unlikely to result from
viral laryngitis, and other etiologies should be investigated,
including a detailed laryngoscopy.

Bacterial Laryngitis
Although rare, the physician should begin to consider a
bacterial etiology when the supportive measures dis cussed above
fail to decrease symptoms or if symptoms worsen after an initial
plateau of symptoms. Initial clini cal presentation may be similar to
that of viral laryngitis, but supraglottitis and epiglottitis may result.
As with the pediatric population, these conditions require escalated

care, given the potential for airway demise. The causative

bacteria are also similar to those in the pediatric popu
lation and include Haemophilus influenzae, Streptococcus
species, and Staphyloco ccus species. Haemophilus spe cies
remain the most common but methicillin-resistant
Staphylococcus aureus infections have been reported (1,57).
Diagnosis relies on endoscopic examination (Fig.
67.2) of the larynx. Radiologic imaging may be used to
supple ment endoscopic evaluation, and findings can
include the classic "thumb-print" sign of supraglottic
inflamma tion. Treatment depends on the clinical
presentation with attention focused on airway
competence. In a recent study, only 2 of 10 adult patients
with supraglottitis evaluated over a 6-month period
required airway intervention (8). Despite the majority of
patients not needing airway pro tection, increased work
of breathing and/or stridor must

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx

Figure 67.1 Acute laryngitis: note global laryngeal edema and

erythema.

be given proper credence. Medical treatment is targeted

to the pathogen identified by culture. Additional
supportive measures such as hydration and steroids are
indicated (9). Though not common in the United States,
rhinoscle roma can also affect the larynx. Caused by
Klebsiella rhi noscleromatis, patients tend to have
laryngeal involvement (13/22 patients) and may need
emergent tracheotomy to maintain airway patency (3/13
with laryngeal involve ment) (10). In that series, all
tracheotomy patients were decannulated with appropriate
antibiosis. Pathologic examination demonstrates the
gram-negative coccoba cillus of Klebsiella on culture, as
well as Mikulicz cells on
mucosa} biopsy.

Fungal Infection
Fungal laryngitis often occurs in immunocompromised
patients, such as patients with systemic causes for immuno
suppression (HIV, chemotherapy, diabetes, etc.) and indi
viduals who are locally immunocompromised because of

Figure 67.2 Bacterial laryngitis: note posterior glottic purulence.

979

steroid inhaler use (11). Such infections are overwhelmingly

candidal in etiology and will respond to systemic antifun gal
treatment (11,12). Patients will present with laryngo
pharyngeal symptoms, such as dysphagia and dysphonia.
Physical examination demonstrates white plaque-like epi
thelial lesions of the mucosa surfaces, which may be focal
or diffuse (Fig. 67.3). Conservative treatment with a
systemic antifungal and atopical antifungal are appropriate,
but short interval evaluation is necessary to assess resolution
as persis tent lesions merit biopsy and possible culture
oflesions.
Though the majority of fungal infections are Candida
sp., other fungal organisms, such as blastomycosis, coc
cidioidomycosis, and histoplasmosis may also infect the
larynx. These infections target immunocompromised
patients as seen with Candida sp. infections but tend to
occur in endemic geographic regions. Blastomycosis and
histoplasmosis are prevalent in the Mississippi and Ohio
River valleys, and coccidioidomycosis is endemic in the
southwestern United States and Central America.
However, patients may become infected outside of
endemic areas (13). Biopsied lesions require culture with
periodic acid Schiff and histologic examination to
evaluate possible granulomatous conditions such as
tuberculosis (14). Hematoxylin and eosin (H&E) staining
demonstrates pseu doepitheliomatous hyperplasia with
possible ulceration. Microscopic broad-based budding
cells are a pathognomic feature of blastomycosis.
Clinically, patients can present with a range of symptoms
and physical exam findings, spanning from dysphonia to
occult airway compromise (15,16). Treatment involves
long-term systemic antifungal therapy with agents such as
amphotericin or triazole.

Mycobacterial Infection
Laryngeal tubercular infection from M ycobacterium
tubercu losis is classically associated with active pulmonary
disease but can present as isolated laryngitis (17,18).
Laryngeal

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx

Figure 67.3 Fungal laryngitis: note white fungal plaques with

marginal erythema on midmembranous vocal folds.

979

M . tuberculosis infections follow similar natural history

to pulmonary tuberculosis and most commonly present
as lesions in the posterior glottis. Patient factors include
increased prevalence in underdeveloped countries, areas of
over-crowding and communal living, and immunocom
promised populations. While laryngeal infections present
with similar symptoms as pulmonary infections (cough,
hemoptysis, unintentional weight loss, fever, night sweats),
patients may also present with laryngopharyngeal symp
toms such as dysphonia, dysphagia, and odynophagia.
Physical examination can demonstrate exophytic masses
that mimic malignancy (19,20). Pathologic examination
demonstrates caseating granulomas that are pathogno
monic to M . tuberculosis infection. Treatment is targeted
with multidrug regimens with culture guidance, as multi
drug resistant M . tuberculosis strains are on the rise.
Other Infections
Less common infections of the larynx include leprosy
and syphilis. M ycobacterial leprae and M ycobacterium lep
romatosis, the causative infectious agents of leprosy, cause
dramatic systemic and laryngeal epithelial changes. As
with the other laryngeal infections, patients can present
with variable severity in symptoms, with the most severe
being occult aspiration or complete upper airway obstruc
tion requiring tracheotomy (21,22). The World Health
Organization recommends multidrug treatment with com
binations of dapsone and rifampin with possible adjunc
tive clofazimine.
Syphilis is caused by Treponema pallidum infection and
generally presents in stages. The primary stage generally
presents to the otolaryngologist as a painless oropharyn
geal chancre. During the secondary stage, patients can
present with laryngeal manifestations, including leuko
plakia, exophytic mass( es), and very rarely, decreased
vocal fold mobility (23,24). Diagnosis involves serologic
studies (venereal disease research laboratory or rapid
plasma regain) and/or dark-field microscopy to visualize
the pathopneumonic spirochetes sampled from suspect
mucosa} lesions. The mainstay of treatment is penicillin.
For those patients with penicillin sensitivities, definitive
allergy testing and desensitization may be required prior
to treatment.

Idiopathic Ulcerative Laryngitis

Idiopathic ulcerative laryngitis (IUL) was first described
in 2000 and a large clinical series was presented in 2011
(3,25). This condition involves dysphonia and severe
cough following an upper respiratory tract infection and
ulceration( s) of the midmembranous vocal fold. IUL
appears to occur more in females than males (3). An etio
logic agent(s) has not been identified and medical
therapy with antibiotics, antifungal, and proton pump
inhibitors aretypically used but without an obvious
positive response.

These patients should be observed with serial office

endos copy (preferable video recorded) and the urgeto
perform a biopsy should be resisted. Biopsy will most
likely be non diagnostic and result in scar formation at
the ulcer site. Patients should be counseled regarding the
duration of the condition (average 3.3 months with a
range from 2 to 10 months) and the possibility of vocal
fold scar formation following the resolution of the
ulcer(s) (3). Ifprogression of the ulcer(s) occurs during
serial examination a biopsy may be warranted.

INFILTRATION OF THE LARYNX

Multiple inflammatory processes can affect the larynx.
These differ in their underlying presentation and patho
logic and histologic features. As a group, these condi
tions generally cause decreased vocal fold pliability and/
or lesions of the vocal folds, supraglottis, or subglottis.
Symptoms correspond to the severity of vocal fold
infiltra tion and potentially degrees of airway
compromise.

Wegener Granulomatosis
Wegener granulomatosis is associated with necrotiz ing
granulomatous inflammation and vasculitis of small
blood vessels. The disease tends to affect the upper
airway, the lungs, and the kidneys (26). The
otolaryngologist may identify a patient because of
nonhealing ulcers in the nasal cavity or, more rarely,
subglottic airway stenosis. Diagnosis relies on thorough
physical examination, nasal and laryn geal endoscopy,
and testing for classical antineutrophil cytoplasm
antibody (c-ANCA). Interestingly, 10% to 20% of those
with airway symptoms have been reported to have a
negative c-ANCA test (26).
The best treatment for these patients involves a multi
disciplinary approach. Medical management with
systemic corticosteroids and/or cyclophosphamide is used
to obtain remission, and medications such as
methotrexate, trime thoprim methoxazole, or azathioprine
are used for mainte nance therapy (26). Surgical
treatments include subglottic expansion (subglottic
releasing incisions with balloon or rigid dilation) and
intralesional injection of corticoste roids (27). Subglottic
stenting is generally avoided in favor of tracheotomy for
recalcitrant presentations (28). In the setting of inactive
Wegener granulomatosis with resultant subglottic
stenosis, cricotracheal resection is also a viable treatment
option.

Rheumatoid Arthritis
Rheumatoid arthritis affects millions of people
worldwide with a predilection for females. It affects the
larynx in just over one-fourth of cases (29). In
comparison to unaf fected control subjects, patients with
rheumatoid arthritis had more frequent loss of higher

frequency phonation, generalized dysphonia, decreased

vocal fold mobility, and

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx

laryngeal edema (30). These symptoms are modulated by

the active status of the patient's disease. Active rheuma
toid arthritis tends to present with a substantial laryngitis
with erythematous arytenoid mucosa (30-32). Chronic
rheumatoid arthritis also selectively targets the arytenoid
cartilages, but more specifically seems to affect the
cricoar ytenoid joint causing ankylosis and possible joint
fixation (26). Patients may also present with rheumatoid
nodules, also known as bamboo nodes, which are focal
subepithe lial lesions, typically on the superior surface of
the mem branous vocal fold. Treatment of rheumatoid
arthritis relies upon medical management with
immunomodular and anti-inflammatory treatments.
Although outcomes data are sparse, surgical management
may be indicated to man age airway symptoms or to
judiciously remove rheumatoid nodules to improve
phonation (32,33). (See Chapter 68) Alternatively, serial
vocal fold steroid injections are a less invasive treatment
that may improve vocal outcome (34).

Amyloidosis
Amyloidosis is an autoimmune condition characterized
by extracellular deposition of fibrillar proteins in affected
tissue. Laryngeal involvement is rare and may not be
asso ciated with primary systemic amyloidosis. However,
laryn geal amyloidosis may be present in conjunction
with other systemic conditions such as multiple myeloma
(35,36). Patients usually present with bulky deposition of
amyloid protein with variable degrees of infiltration of
the vocal fold, paraglottic space, and the supraglottis.
Presenting fea tures include cough, dysphonia,
dysphagia, and possible stridor. Biopsy is required for
diagnosis as amyloid has a pathognomonic apple green
birefringence after staining with Congo red (Fig. 67.4).
Referral is needed to examine for underlying secondary
causes, such as systemic amyloi dosis. There are reports
of complete resolution with radia tion therapy, but this
treatment modality has not gained

981

Figure 67.4 Amyloidosis after Congo red staining: note apple

green birefringence with polarimetric filtered microscopy.

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx

mainstream acceptance (37). Surgical intervention is

usu ally undertaken to address specific symptoms and
can improve vocal deficits. Recurrence is quite common
(38).

Relapsing Polychondritis
Relapsing polychondritis is characterized by intermittent
recurrent episodes and inflammation of cartilaginous
struc tures. While the ears and nose are most commonly
affected, the larynx can also become involved. Early
studies demon strate 14% of patients have laryngeal
involvement at pre sentation but that up to half of
patients eventually develop airway symptoms (39).
Radiographic studies, such as mag netic resonance
imaging (MRI) and computed tomography (CT) can
identify cartilaginous changes. Patients may pres ent to
the otolaryngologist with ear, nasal, and/or airway
complaints such as exertional dyspnea or stridor.
Purulent chondritis of the laryngeal framework has been
described as a sequela of superimposed infection (40).
Medical man agement is paramount as maintenance
includes low dose corticosteroids and/or methotrexate.
Dapsone has also shown to be beneficial (41). Surgical
intervention may be indicated to secure the airway with
tracheotomy. A small case series of patients underwent
airway reconstruction to provide more long-term airway
stability (42).

981

Systemic Lupus Erythematous

Like rheumatoid arthritis, systemic lupus erythematous
(SLE) has a predilection for females. Its effects are not usu
ally limited to the larynx as roughly two-thirds of patients
never experience laryngeal symptoms. Patients can pres ent
with a wide variety of laryngopharyngeal complaints, which
include dysphonia and dyspnea. A study including 12
patients with SLE found that 11had laryngeal abnormal ities
(43). Physical signs ranging from edema or ulceration to
vocal fold paralysis can be seen on examination (44).
However, a direct causal relationship between SLE and the
above laryngeal pathology has yet to be demonstrated.

Pemphigus and Pemphigoid

Pemphigus and pemphigoid are related autoimmune con
ditions differentiated by the target of their autoantibod
ies. While both conditions lead to a robust inflammatory
reaction that can possibly lead to epithelial injury, pem
phigus autoantibodies are directed against intraepithelial
targets while pemphigoid autoantibodies target subepithe
lial antigens. Immunofluorescence of tissue biopsy is
used to identify the characteristic autoantibodies for
definitive diagnosis.
Patients may present with signs of disease within the
nasal cavity or the larynx. The prevalence of laryngeal
involvement seems to differ between the diseases for
unknown reasons. One study demonstrated that 21 of
53 (40%) patients with head and neck manifestations of

Figure 67.6 Sarcoidosis in typical supraglottic location.

Figure 67.5 Laryngeal pemphigus in typical supraglottic location.

pemphigus had laryngeal involvement (45). However, a

separate study of pemphigoid patients demonstrated that
10 of 38 (26%) patients with head and neck symptoms
had laryngeal involvement (46). Other studies have dem
onstrated relatively similar prevalence in pemphigus
(47). Both pemphigus and pemphigoid appear to have a
predi lection for supraglottic mucosa (Fig. 67.5). Highdose cor ticosteroids are utilized to control active disease
and are decreased for maintenance therapy. Other
immunomodu
lators,
such
as
azathioprine,
cydophosphamide, and cydo sporine, have also been
utilized for medical management. Surgical intervention is
limited to diagnostic biopsy and/ or airway intervention,
such as tracheotomy or less invasive airway surgery
(dilation) to provide a stable airway.

Sarcoidosis
Sarcoidosis is an autoimmune condition defined patho
logically by noncaseating granulomas. Patients most
com monly affected are young adult African American
women. Laryngeal involvement is seen in 3% to 5% of
cases and

usually affects the supraglottis (Fig. 67.6) (48).

Laryngeal complaints from sarcoidosis, such as
nonproductive cough and dyspnea, may be difficult to
differentiate from the pulmonary manifestations of the
disease. Diagnosis of sarcoidosis relies on multiple
modalities as there are usu ally multiple organ systems
involved. The establishment of laryngeal sarcoidosis
relies on laryngoscopic evaluation, with hallmark exam
findings of submucosal infiltration in the infraglottic,
paraglottic space, and the supraglot tis. Involvement of
the epiglottis leads to a distortion and thickening and
has been commonly referred to as a tur ban epiglottis.
Sarcoidosis remains an elusive diagnosis; however,
biopsy of lesions classically reveals noncaseating
granulomas.
Treatment mainly relies on corticosteroids, but other
immunomodulators, such as azathioprine, have also
been administered with good treatment success (49).
Surgical intervention is limited to diagnostic biopsy,
excision of symptomatic lesions, or management of
obstructive airway lesions.

External Beam Radiation

As the role of external beam radiation has increased for the
treatment of head and neck malignancies, many of these
patients later present with laryngopharyngeal complaints,
such as dysphonia, dysphagia, and globus sensation post
treatment. Electron beam radiation induces gradual, dose
dependent fibrotic changes to include muscle atrophy
and fibrosis in the larynx as well as desiccation of mucosa
(Fig. 67.7). Fibrosis within the lamina propria can be
appreciated as decreased mucosa} pliability on strobos copy.
Patients will exhibit atrophy that is disproportionate to
their expected age-related vocal fold volume loss. Vocal fold
hypervascularity is a common finding due to prior vasculitis
incurred during radiation therapy. Improvement in voice is
commonly reported following laryngeal radia tion for early
laryngeal cancer, but voice outcomes associ ated with late
radiation fibrosis of the vocal folds remains

11

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx 1 1 987

do not metasta size, and they generally present as a

smooth, submucosal

Figure 67.7 Radiation effects on the larynx: note global ery

thema, slight atrophy of muscular anatomy, and limited light reflex
indicating decreased secretory function of the mucosa.

uncertain (50,51). A prior report of postradiation vocal

quality suggests that vocal fold stripping or excisional
biopsy rather than limited biopsy for initial diagnosis and
continued tobacco smoking after treatment are signifi
cantly associated with an increased risk of perceived
worse voice quality after treatment (52).
As radiation oncologists develop more sophisticated
techniques to avoid collateral damage to uninvolved
struc tures, the extent of radiation changes may decrease.

BENIGN NEOPLASIA OF THE LARYNX

When one excludes nonneoplastic vocal fold lesions,
such as vocal fold polyps, nodules, and cysts (see
Chapter 68), benign tumors of the larynx are varied and
quite rare. Diagnosis relies on thorough history with
appropriate examination and imaging.

Hamartoma
Hamartomas are rare, benign lesions that can present as
congenital malformations or lesions later in life. They are
generally loosely organized neoplasms with multiple
types of tissue, all of which are native to the affected
subsite of the larynx. Hamartomas can be incidentally
identified or cause significant airway symptoms,
especially in a young child. Presentation and
symptomatology are related to the location of the
neoplasm, and hamartomas have been mostly commonly
identified in the supraglottis and sub glottis (53,54).
Excisional biopsy is both diagnostic and curative if
resection is complete (55).

Chondroma
Chondromas are benign tumors consisting of
cartilaginous cells. They are slow-growing lesions that

12

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx 1 2 987

lesion. Laryngeal chondromas may be difficult to differ

entiate from low-grade chondrosarcomas and clinically
follow a similar course. While the bulk of these tumors
present within the posterior cricoid cartilage, lesions
have been found within other subsites of the larynx as
well as the hyoid bone (56,57). Patients may be
relatively asymp tomatic, but lesions can cause airway
obstruction or exter nal neck masses (56). CT is
generally the preferred imaging modality to define the
extent of the lesion (58). Surgical excision is the
treatment of choice for chondromas. Surgery has been
traditionally performed via open procedures involving
laryngofissure, but, more recently, endoscopic ablation
techniques have been shown to be successful (59).
Comparative efficacy between open and endoscopic
surgical excision is unknown.

Rhabdomyoma
Rhabdomyomas of the larynx are benign tumors
compris ing striated muscle. Laryngeal involvement is
the most common location for rhabdomyomas of the
head and neck (60). These tumors present in variable
locations within the larynx and have been documented to
involve both intrinsic and extrinsic laryngeal
musculature (61,62). Diagnosis with biopsy or magnetic
resonance is indicated, and complete resection is
curative.

Respiratory Papillomatosis

Though primarily seen in the pediatric population, adult

onset recurrent respiratory papillomatosis (RRP) is not an
uncommon presentation. For further information regard
ing juvenile onset RRP, please refer to Chapter 94.
Caused by human papillomavirus (HPV) subtypes 6
and 11, RRP occurs most commonly at the level of
thevocal folds. The virus can be transmitted vertically or
by sexual transmission. RRP can present anywhere within
the upper aerodigestive tract from the nasal vestibule to
the bronchi oles with a predilection for areas of transition
from pseu dostratified columnar to stratified squamous
epithelium.
Lesions can be relatively small, noticeable only
because of resultant dysphonia from decreased vocal fold
muco sal wave propagation, dysphonia related to masseffect that impairs glottal closure, or variable degrees of
airway obstruction (Figs. 67.8 and 67.9). Though benign,
they do have significant morbidity and have the potential
for malignant transformation (63,64). A recent study
includ ing 54 adults demonstrated that dysplasia was
identi fied in 50% of patients, and dysplasia was
diagnosed on biopsy specimens at an average of 16.2
months from initial diagnosis. Of the initial 54 patients, 3
progressed to carci noma in situ while 1patient
progressed to squamous cell carcinoma (64). Use of
inhaled corticosteroids, increasing number of procedures,
distal spread of disease, and pre vious exposure to
cidofovir have been implicated as risk factors for
dysplastic reaction (65). In addition, history of

13

Section IV: Laryngology

Chapter 67: Infection, Infiltration, and Benign Neoplasms of the Larynx 1 3 987

Figure 67.8 Adult RRP occluding anterior glottis, limiting

phonation.

radiation therapy, cigarette smoking, and systemic immu

nosuppression have been implicated in malignant trans
formation (66).
The verrucous papillomatous growth of the lesions are
pathognomonic. Though multiple treatment modalities
are available, conservative removal of disease is the first
line treatment. If cold instrumentation is to be utilized,
careful attention must be dedicated to only removing the
papilloma and leaving the superficial lamina propria undis
turbed. Ablation with CO2 or potassium titanyl phosphate
(KTP) lasers has also been shown to be a successful treat
ment modality for both initial and subsequent treatments
(67). A great benefit of fiber-based laser treatment is that it
can be performed in an awake patient using a channeled
endoscope through which the fiber can be advanced. Awake
procedures decrease use of operative resources and elimi
nate the need and dangers of general anesthetic. Regardless
of the surgical technique utilized, the physician must avoid
deepithelialized surfaces in juxtaposition to avoid anterior
glottic webbing and/or posterior glottic stenosis.

While surgical removal of lesions remains the first-line

treatment for RRP, other adjuvant therapies have been
developed. Cidofovir is an antiviral shown to decrease
dis ease burden in both intralesional injection and inhaled
forms (68,69). Both treatment modalities have been
shown safe, but hepatotoxicity has been identified with
the injected form. Interferon-alpha and indole-3-carbinol
(an extract found in cruciferous vegetables) have both
been used to control disease propagation (70).
The United States Food and Drug Administration
(FDA) approved Gardasil, a vaccination against HPV
subtypes 6, 11, 16, and 18, in June of 2006 as a means of
potentially preventing cervical cancer. While cervical
cancer is mainly caused by HPV subtypes 16 and 18,
thevaccination against subtypes 6 and 11, could
drastically affect the prevalence and presentation of RRP.
Currently, there is no definitive association between
vaccination and disease modulation, thus vaccination is
not presently indicated in patients with prior exposure
(71).

Hemangioma
Laryngeal hemangiomas are more prevalent in the
pediatric population, and this is presented in Chapter 104.
In con trast to pediatric hemangiomas, which usually
present in the subglottis, adult laryngeal hemangiomas
present in the supraglottis (Fig. 67.10) (72).
Hemangiomas are usually asymptomatic but can cause
airway obstructive symptoms (73). As these are
incredibly rare tumors, no standardized treatment exists.
A conservative approach emphasizing medical
management with either corticosteroids or radia tion
therapy was historically advocated. Though success ful in
the pediatric population, laser ablation was initially
avoided in adults for concern that the vasculature would
exceed CO2 laser coagulation capacity. However, a report
of four laryngeal hemangiomas ablated without incident
calls that initial theory into question (74). Propanolol has
also been successful in treating laryngeal hemangiomas
in children, but there are no reports of its use in the adult
population (75).

Benign Salivary Neoplasia

Pleomorphic adenoma, a benign salivary neoplasm,
may arise within the larynx. Scattered case reports iden
tify fewer than 20 instances within the literature. More
than half of the cases appeared in the supraglottis, and
patients most commonly present with dysphonia. Surgical
resection was reported as curative, though follow-up was
limited (76).

Fibroma
Figure 67.9 Adult RRP nearly occluding entire glottis.

Laryngeal fibromas are exceedingly rare with fewer than

a dozen cases reported. Histologic examination demon
strates abundant extracellular matrix with interspersed

Figure 67.10 Adult supraglottic hemangioma. Cobblestone-appearing lingual tonsils are visible
at the inferior aspect of this image.The epiglottis is completely obscured by this hemangioma.

paucicellular areas, and the extracellular matrix tends to

be composed of "cytologically bland spindle cells." The
reported cases all appear to be isolated lesions that pre
sented with dysphonia and cough (77).Radiographic
imag ing (er and MRI) can delineate the full extent of
the lesion
in planning for surgical resection. Excision with margins is
advocated to minimize chance of recurrence (78).

Schwannoma
Schwannomas arise from nerve sheath fibers and account
for less than 1% of all laryngeal tumors. The endoscopic
appearance may be mistaken for a laryngocele and com
monly appear as smooth submucosal mass within the
pyri form sinus or aryepiglotticspace (79). Patients may
present
with globus sensation, dysphagia, dysphonia, and if large,
airway obstruction (80). Imaging with er and/or MRI
help
to plan surgical resection. Histopathologic examination
demonstrates the classic Antoni A and Antoni B areas
seen with other schwannomas. The associated nerve was
not identified in the available case reports. Some patients
have postoperative dysphonia and vocal fold paresis,
possibly implying recurrent laryngeal involvement (79).

Granular Cell Tumor

Granular cell tumors can occur anywhere within the
body but are often seen within the head and neck (81).
The larynx, however, is a rare location for these neo
plasms. They are neural in derivation and, within the

larynx, tend to grow slowly and isolate within the

vocal folds themselves. Presenting symptoms include
hoarse ness, strider, dysphagia, and cough. Biopsy
must be com pleted to evaluate for malignant neoplasm
as there is an association with pseudo-epitheliomatous
hyperplasia, which can mimic squamous cell
carcinoma. Serologic staining of biopsy specimens will
yield positive results for S-100, neuron-specific
enolase, vimentin, and CD 68 (81). Complete resection
with microlaryngeal phono surgical instruments and
principles can yield cure with good vocal outcome.

LARYNGOCELES AND SACCULAR CYSTS

While laryngoceles and saccular cysts are not neoplasms,
they present as benign appearing masses in the larynx.
The laryngeal saccule is a mucous gland containing
appendage that lies between the false vocal fold and the
thyroid carti lage. It is an out pouching of the normal
laryngeal ventricle and extends as a blind-ended sac
posterolateral to the edge of the laryngeal surface of the
epiglottis. The function of the saccule is unknown
although ithas been theorized that it may represent a
vestigial air sac. Both laryngoceles and saccular cysts
involve expansion of the saccule to form a mass.
Laryngoceles by definition must have air contained
within their lumen, while saccular cysts are strictly fluid
filled masses.
Laryngoceles contain air due to patent communication
with the laryngeal lumen. Further classification of laryn
goceles depends on their location. They can be defined as

Figure 67.11 Combined laryngocele. Axial CT showing air-filled

dilation of the saccule extending through the thyrohyoid mem
brane into the neck.

internal, external, or combined. Internal laryngoceles are

strictly confined within the thyroid cartilage, external laryn
goceles lie exclusively outside the cartilaginous laryngeal
framework, and combined laryngoceles spanboth the inside
and outside of the thyroid cartilage (82,83) (Fig. 67.11).
Saccular cysts are also classified according to their
loca tion; anterior and lateral. Anterior saccular cysts
appear as rounded fluid-filled masses emanating from
the ante rior portion of the ventricle and extend medially
into the lumen of the larynx (Fig. 67.12). They lie
superior to the glottal level at or near the anterior
commissure, and inter fere with phonation or airway
depending on their size. Lateral saccular cysts expand
within the paraglottic space and appear similar to internal
laryngoceles as a submuco sal fullness in the ventricular
fold.
Although the etiology of saccular masses is unclear,
they result from abnormal dilation of the saccule. It has
been suggested that those who routinely develop high
trans glottic pressures (glass blowers, trumpet players)
are at a higher risk of developing laryngoceles. It is
thought that saccular cysts arise secondary to obstruction
of the saccular orifice as they have been found in patients
with laryngeal carcinoma or following an upper
respiratory tract infection (84). Congenital saccular cysts
can occur in infants and present as a weak cry, stridor, or
cyanosis (83).
Patients with laryngoceles and saccular cysts report
symptoms consistent with a laryngeal mass: dysphonia,
stridor, chronic cough, a neck mass, and occasionally
dys phagia. Severity of symptoms depends on the size
and location of the lesion. Small or nonobstructing
lesions may be asymptomatic. The diagnosis is most
commonly made by physical examination including
transnasal or transoral laryngeal imaging and neck exam.
In the case of

Figure 67.12 Anterior saccular cyst. Fluid-filled mass arising

from the saccule and protruding into the laryngeal lumen.

anterior saccular cysts, a mass can be seen emanating from

the vestibule to the laryngeal lumen while lateral saccular
cysts and laryngoceles present as a submucosal mass in the
false vocal fold. External and combined laryngoceles can
present as a neck mass that enlarges with valsalva. Both
laryngoceles and saccular cysts can become acutely infected
to form a laryngopyocele or an infected saccular cyst.
Super-infection can lead to rapid expansion and acute pre
sentation with worsening symptoms, fever, and occasion
ally, airway obstruction.
Fine-cut CT is a useful adjunctive tool diagnostically.
The presence of air within the lesion differentiates laryn
goceles from saccular cysts. The location and extent of
the lesion can be accurately assessed with a fine-cut CT
scan. Endoscopic excision of these lesions is the mainstay
of treatment and the recurrence rate is very low with
long term follow up (84).

SUMMARY
The larynx can be subject to infectious agents, inflamma
tory conditions, and neoplasia. The initial management
of the patient must be to ensure a stable, secure airway.
Once the airway is ensured, a thorough history and physi
cal examination, followed by detailed laryngeal
endoscopy

and directed biopsy, can usually narrow the differential

diagnosis and guide the physician to appropriate diagnos
tic testing. Treatment should address the patient's symp
toms and ideally ensure both airway stability and future
vocal performance.

Multiple infections, inflammatory, and benign

processes can affect the larynx. Each has its own
unique presentation and treatment considerations.
Infectious laryngitis is most commonly viral in eti
ology, and should be initially treated with voice
rest and supportive measures in most cases.
Bacterial, fungal, and mycobacterial infection is
considerably more rare.
Inflammatory and infiltrative processes of the lar
ynx can occur from Wegener granulomatosis (typi
cally
subglottic
involvement),
sarcoidosis
(typically supraglottic involvement), amyloidosis,
and auto immune processes (such as rheumatoid
arthritis, SLE, and pemphigus/pemphigoid)
The most common benign neoplasm of the larynx
is
laryngeal
papillomatosis.
Laryngeal
chondromas,
hamartomas,
schwannomas,
fibromas, pleomor phic adenomas, and granular
cell tumors are far more rare.

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