7. Congenital disorders of the Larynx.
Laryngomalacia
-Most common congenital anomaly of the larynx
-characterized by partial or complete collapse of the
supraglottic structures on inspiration.
It is the most common cause of congenital stridor, accounting
for 60% of all cases
Males 2x > female
Laryngomalacia
Etiology: Unknown
theories: GERD, Immature neuromuscular control
Immature neuromuscular control may be responsible for the
arytenoid prolapse observed in laryngomalacia, although an
increase in the incidence of laryngomalacia does not occur in
premature infants who have classic hypotonicity.
9. Vocal Fold Paralysis
Epidemiology
Vocal fold paralysis is the second most common congenital
anomaly of the larynx, accounting for 15-20% of all cases.
No gender difference exists in the prevalence of this anomaly.
Up to 45% of patients have other, coexisting airway pathology,
and so a formal microlaryngoscopy and bronchoscopy is essential.
Bilateral TVC Paralysis:Etiology
usually idiopathic. may also occur secondary to central neuromuscular immaturity.
lesions in the central nervous system, including Arnold-Chiari malformation,
hydrocephalus or myelomeningocele,
Birth trauma that causes excessive strain to the cervical spine may cause transient
bilateral vocal fold paralysis lasting 6-9 months.
Clinical presentation
Bilateral vocal fold paralysis manifests as an inspiratory stridor at rest that
worsens upon agitation in children with near-normal phonation and progressive
airway obstruction.
Aspiration is common with bilateral vocal fold paralysis, often resulting in
recurrent chest infections and a failure to thrive.
10. Unilateral TVC Paralysis
Etiology
usually idiopathic.
Lesions in the mediastinum, such as tumors or vascular
malformations, Iatrogenic injury to the left recurrent
laryngeal nerve can occur during surgery for
cardiovascular anomalies
Clinical presentation:
Unilateral TVC paralysis may manifest during the first few
weeks of life, or it may go unnoticed. The most common
symptoms are a hoarse, breathy cry that is aggravated by
agitation. Feeding difficulties and aspiration may also
occur.
14. Congenital Subglottic Stenosis
Epidemiology
is the third most common congenital anomaly of the larynx, accounting
for 15% of all cases.
This condition is the most common laryngeal anomaly that requires
tracheotomy in infants.
Males affected 2x > as females.
Etiology
Incomplete recanalization of the laryngotracheal tube during the third
month of gestation leads to different degrees of congenital subglottic
stenosis, with complete laryngeal atresia being the extreme form
Classification CSS can be classified into 2 types.
1) Membranous CSS is the result of circumferential submucosal
hypertrophy with excess fibrous connective tissue and mucus glands. This
type is the most common and mild form of congenital subglottic stenosis.
15. 2) Cartilaginous CSS results from an abnormal shape of the
cricoid cartilage. The cartilage usually narrows laterally but may
also develop generalized thickening or excessively large anterior or
posterior laminae.
While the lumen at the midportion of the cricoid cartilage is
normally elliptical, In some infants with congenital subglottic
stenosis, an elliptical cricoid is present with a transverse diameter
that is significantly smaller than the anteroposterior diameter,
CSS defined as:
-term infant = lumen diameter is < 4 mm
-preterm infant = lumen diameter < than 3 mm.
The stenosis is then graded:
grade I = less than 50% obstruction;
grade II = 52% to 70% obstruction;
grade III = 71% to 99% obstruction;
grade IV = no detectable lumen.
17. Subglottic Hemangioma
Epidemiology
Subglottic hemangiomas account for 1.5% of all congenital
anomalies of the larynx.
Females are affected 2x > males.
Etiology and pathogenesis: Subglottic hemangiomas develop as a
result of a vascular
malformation derived from the mesenchymal rests of vasoactive
tissue in the subglottis.
Diagnosis
When the diagnosis is unclear, perform biopsy of the lesion with
caution because of the risk of significant hemorrhage.
Plain radiographs of the neck may show an asymmetric narrowing
of the subglottis, which may aid in establishing the diagnosis prior
to endoscopy.
18. Contrast-enhancing airway infantile
hemangioma in posterior subglottis.
Enormous extent infantile hemangiomas can reach.
Arrows indicate large component in the neck that
includes airway infantile hemangiomas.
20. Congenital Laryngeal Webs
Epidemiology
Laryngeal webs are rare congenital anomalies of the larynx.
Etiology and pathogenesis
Incomplete recanalization of the laryngotracheal tube during the third month of gestation
leads to different degrees of laryngeal webs.
The most common site of development of laryngeal webs is at the level of the vocal folds
anteriorly, although they may occur in the posterior interarytenoid or in the subglottic or
supraglottic area.
Clinical presentation
Laryngeal webs may manifest with symptoms ranging from mild dysphonia to significant
airway obstruction, depending on the size of the web.
Stridor is rare except in patients who have a posterior interarytenoid web.
A third of children with laryngeal webs have anomalies of the respiratory tract,
most commonly subglottic stenosis.
Posterior Webs
Diagnosis is made at the time of DL with palpation of the interarytenoid area. Posterior
webs may be associated with subglottic stenosis.
Anterior Webs
Anterior congenital laryngeal webs are also rare anomalies that, if severe, are diagnosed
in the neonatal period after an investigation for the source of aphonia and stridor.
21. Congenital Laryngeal Atresia, Cysts, and Lymphangioma.
Laryngeal atresia is the most rare and most devastating of the congenital anomalies of
the larynx. Only a few studies report documented survivors of such lesions.
Etiology and pathogenesis
Failure of recanalization of the laryngotracheal tube during the third month of gestation
leads to laryngeal atresia.
Clinical presentation
Laryngeal atresia manifests as an acute airway obstruction in the newborn immediately
after clamping the umbilical cord. Examination reveals a neonate with severe respiratory
distress marked by strong respiratory efforts and inability to inhale air or cry
Laryngeal Cysts
Epidemiology
Laryngeal cysts are uncommon congenital anomalies of the larynx. Congenital
saccular cysts represent 25% of all laryngeal cysts.
Etiology and pathogenesis
Obstruction of the laryngeal saccule orifice in the ventricle leads to retention of
mucus, which causes saccular cysts. Ductal cysts arise from blockage of
submucosal mucus glands. These cysts can occur in the vallecula, subglottis, or
vocal cords. They are common in the subglottis after prolonged intubation
because of irritation and blockage of submucosal glands.
23. Laryngeal Lymphangioma
Epidemiology
Laryngeal lymphangiomas are rare congenital anomalies of the
larynx. Half the cases are diagnosed in the neonatal period, and
75% are diagnosed by age 1 year.
Etiology and pathogenesis
Lymphangiomas originate from lymphatic vessel malformations.
Clinical presentation
Individuals with laryngeal lymphangioma may be asymptomatic
or may present with significant airway obstruction when the
lesions attain a large size. Upper respiratory tract infections may
precipitate symptoms by causing a rapid increase in the size of
these lesions.
Diagnosis
Endoscopy is the procedure of choice for aiding in the diagnosis of
laryngeal lymphangiomas.
33. Laryngo-Tracheo-Bronchitis (LTB).
Plain X-ray soft tissue neck, AP view
a. Church steeple or pencil-point sign: squared
appearance of subglottic area replaced by cone shaped
narrowing just below vocal cords
b. Ballooning of hypopharynx
38. Reinke’s edema.
Accumulation of fluid in Reinke’s space
Synonyms: Bilateral diffuse polyposis, Smoker’s polyps, Polypoid corditis,
Polypoid degeneration of vocal cords, Localized hypertrophic laryngitis
10% of benign laryngeal lesions
Reinke’s space
39. Etiology
Irritants: tobacco smoke, dry air, dust, alcohol
Laryngeal allergy
Infection: chronic sinusitis
Idiopathic
Edema limited to superior surface of vocal cord due to
dense fibrous attachment to conus elasticus on under
surface of vocal cord.
40. Angio-neurotic edema.
Recurring attacks of swelling of face, larynx & extremities caused by
edema due to vasodilatation & increased capillary permeability
Types:
Allergic: swelling with itching, laryngeal edema & bronchospasm
Hereditary: Non-pruritic swelling + laryngeal edema + recurrent
abdominal pain with vomiting & diarrhea
Laryngeal Tuberculosis.
Commonly associated with pulmonary TB
Posterior commissure arytenoids, vocal cords, ventricular bands &
epiglottis mainly affected
Method of spread:
Bronchogenic: contact of larynx with sputum containing
tubercular bacilli
Hematogenous
42. Symptoms of LPRD.
Hoarseness
Persistent clearing of throat
Difficulty in swallowing food
Breathing difficulties or choking episodes
Annoying cough after eating
Sticking sensation or lump in throat
Heartburn & indigestion absent
43. Laryngocoele
A laryngocoele refers to a dilatation of the laryngeal ventricular saccule.
Sub types
Three laryngocoele sub-types are described:
internal - the dilated ventricular saccule is confined to the paralaryngeal space
( 40%),external - the saccule herniates through the thyrohyoid membrane, and
the superficial portion is dilated ( 25%), and mixed - with dilated internal and
external components ( 45%).
Laryngocoele is usually acquired rather than congenital. Risk factors include raised
intralaryngeal pressure secondary to excessive cough or playing blowing
instruments or due to an obstructing lesion such as tumour, and the finding of a
laryngocoele should prompt a search for an underlying laryngeal carcinoma
obstructing the orifice of the laryngeal ventricle
Radiographic features: CT
Typically seen as a well defined, air or fluid filled lesion related to the paraglottic
space, which has continuity with the laryngeal ventricle. The extent will obviously
depend on sub type.
Attenuation characteristics may vary depending on laryngocoele content (e.g. air,
fluid, mucus etc).
49. Polyps are benign lesions of the larynx, occurring mostly in
adult males, that are usually located on the phonating margin
(edge) of the vocal folds and prevent the vocal folds from
meeting in the midline. Polyps can interfere with voice
production and may produce a hoarse, breathy voice that tires
easily. These may respond to conservative medical therapy and
intensive speech therapy. If the lesion fails to respond,
meticulous microsurgery may be indicated. One of the cofactors
in the cause of these lesions may be laryngeal reflux disease.
Nodules are calluses on the vocal folds that occur with
improper voice use or overuse. They are most common in children
and females. They prevent the vocal folds from meeting in the
midline and thus produce an hourglass deformity on closure
resulting in a raspy, breathy voice. Most times these will respond
to appropriate speech therapy.
54. Benign laryngeal tumors include juvenile
papillomas, hemangiomas, fibromas,
chondromas, myxomas, and neurofibromas.
They may appear in any part of the larynx.
Symptoms include hoarseness, breathy voice,
dyspnea, aspiration, dysphagia, pain, otalgia
(pain referred to the ear), and hemoptysis.
Diagnosis is based on direct or indirect
visualization of the larynx, supplemented by CT
Benign laryngeal tumors.
66. Cancer larynx.
About 90-95 % of laryngeal malignancies are squamous
cell carcinoma with various degree of differentiation.
Squamous cell subtypes include keratinizing and non
keratinizing and well differentiated to poorly
differentiated grade.
The rest 5-10% of lesions include verrucous carcinoma,
spindle cell carcinoma, malignant salivary gland and
sarcomas.
Glottic (59% > Supraglottic (40%) > Subglottic (1%).
Smoking contributes to cancer development by causing
mutations or changes in genes, impairing clearance of
carcinogens from the respiratory tract, and decreasing
the body's immune response.
67. Signs and Symptoms of Laryngeal Cancer
Signs and symptoms of laryngeal cancer
include: progressive or persistent hoarseness,
difficulty swallowing, persistent sore throat or
pain with swallowing, difficulty breathing, pain
in the ear, or a lump in the neck. Anyone with
these signs or symptoms should be evaluated by
an Otolaryngologist (Ear, Nose and Throat
Doctor). This is particularly important for people
with risk factors for laryngeal cancer.
90. The malignant lymphomas are divided in Hodgkin
disease and non-Hodgkin lymphomas (NHL). Lymphomas
primary to the larynx are mainly NHL and are predominantly
located in the supraglottic region, as this area of the larynx
contains follicular lymphoid tissue. Among the subtypes of the
NHL, the diffuse large B-cell and the mucosa-associated
lymphoid tissue (MALT)- type marginal zone B-cell lymphomas
are the most commonly found primary laryngeal
hematopoietic neoplasms. Other types of lymphomas, such as
T- or natural killer (NK)-cell lymphomas, are rarely reported.
The presenting symptoms and signs include dysphagia,
dysphonia dyspnea, and cervical lymphadenopathy. The
indirect laryngoscopy usually reveals a polypoid submucosal
supraglottic mass, nonulcerated, without these characteristics
to be specific.
91. Laryngeal lymphoma. Axial CT scan demonstrates moderate uniform enhancement of
a right false vocal cord mass (asterisk) with involvement of both the right aryepiglottic
fold and the right pyriform sinus (black arrow). Note the normal configuration of the
left aryepiglottic fold (white arrow), which is indistinguishable on the right.
92. Large submucosal tumor of the subglottic region. (B) CT scan: axial plane.
Regression of the subglottic tumor after the chemotherapy.
93. Chondrosarcoma of the cricoid cartilage.
Sarcomas of the larynx are rare neoplasms that
constitute less than 1% of laryngeal malignancies.