Benign Laryngeal Pathology Congenital Laryngeal Defects

Congenital Wfebs

• most commonly anteriorly based

• Pathophysiology: incomplete recanalization at 8th week of embryological development

• Types: supraglottic (2%), glottic (75%), subglottic (7%)

• SSx: weak cry at birth, aphonia, variable degrees of respiratory obstruction (inspiratory stridor)

• Dx: flexible nasopharyngoscopy, direct laryngoscopy

• Rx: endoscopic lysis if >50% (laser excision), consider keel or open procedure for extensive involvement, may require tracheotomy

Congenital Subglottic Stenosis

• third most common laryngeal anomaly

• Pathophysiology: incomplete recanalization, small diameter cricoid cartilage, or trapped first tracheal ring

1. Membranous: circumferential, thickened mucous glands or fibrous tissue

2. Cartilaginous: abnormal shelf on cricoid or a trapped 1st tracheal ring

3. Mixed

• Grades: I. <70% obstruction, II. 70-90%, III. 91-99%, IV. complete obstruction

• SSx: biphasic stridor in first few months of life, may mimic croup or recurrent URI, failure to thrive

• Dx: endoscopy, chest x-ray, neck plain films, flexible nasopharyngoscopy to assess vocal fold motion

Management

• Secure Airway: may require tracheotomy (decannulation by 2-3 years old)

• Medical Management: reflux regimen, may consider corticosteroids

(controversial)

• Grade I—II: consider endoscopic management (CO2 or KTP laser excision with dilation); contraindications to endoscopic procedures include significant cartilage loss, concurrent laryngotracheal stenosis, posterior commissure involvement, circumferential cicatricial scar or thick scar (>1 cm), and infected tissue (chondritis)

Open Procedures: indicated for Grade III—IV or when endoscopic management is contraindicated

• Anterior Cricoid Split: no graft required, indicated to wean off of a ventilator before a tracheotomy is performed, indicated for a trapped first tracheal ring, requires adequate pulmonary reserve

• Posterior Cricoid Split: indicated for posterior stenosis, may use costal graft, usually requires a stent, may also be used concurrently with an anterior cricoid split

• .Anterior Laryngofissure with Anterior Lumen Augmentation: requires an anterior graft

• Laryngofissure with Division of Posterior Cricoid Lamina: required if posterior glottis involvement, upper tracheal stenosis, or complete glottis stenosis

• Laryngofissure with Division of Posterior Cricoid Lamina with anterior and posterior grafts

• Segmental Resection with End-to-End Anastomosis

• Hyoid Interposition

• Rotary Door Flap

• Epiglottic Reconstruction

Laryngomalacia

• most common laryngeal anomaly

• most common cause of stridor in neonate and chronic pediatric stridor

• approximately 15% associated with secondary airway lesions

• Pathophysiology: immature cartilage, abnormal calcium metabolism

• SSx: intermittent inspiratory stridor that improves in prone position; worse with feeding, crying, or when placed on back; presents within weeks of birth; normal voice; usually self-limiting as cartilage stiffens with growth

• Most Common Laryngeal Findings

1. inward collapse of A—E folds and cuneiform cartilage into laryngeal inlet during inspiration

2. epiglottis collapses into laryngeal inlet

3. short A-E folds

• Dx: clinical history and endoscopy

• Rx: observation (typically resolves with growth), epiglottoplasty (removes excess tissue), correct GERD if present, rarely requires tracheotomy or intubation

Tracheomalacia

• less common than laryngomalacia

• Pathophysiology: immature cartilage, abnormal calcium metabolism

• SSx: expiratory stridor, exacerbated with infection, usually self-limiting as cartilage stiffens with growth

• Dx: clinical history and endoscopy

• Rx: observation (typically resolves with growth), correct GERD if present, rarely requires tracheotomy or intubation

Vascular Rings

1. Double Aortic Arch: most common vascular anomaly to cause stridor, right aortic arch persists, wraps around esophagus and trachea

2. Right Aortic Arch

3. Anomalous Innominate

4. Anomalous Left Common Carotid

5. Pulmonary Artery Sling: left pulmonary artery originates from right pulmonary artery, slings around right main stem bronchus, then between trachea and esophagus, 50% associated with distal bronchial hypoplasia

6. Retro-Esophageal Right Subclavian Artery (Dysphagia Lusoria): most common vascular anomaly to compress aerodigestive tract (see p. 154)

• SSx: biphasic stridor (external compression of trachea), barking cough, dyspnea with feeding

• Dx: MRI, angiography, barium swallow, CT with contrast

• Rx: symptomatic compression requires surgical intervention (pexis of vessel or reimplantation)

Cri du Chat Syndrome

• Pathophysiology: deletion of short-arm of chromosome 5

• SSx: high pitched stridor, mental retardation, microcephaly

• Laryngeal Findings: narrowed endolarynx, diamond shaped, persistent interarytenoid cleft

• Dx: clinical findings

Posterior Laryngeal Clefts and Laryngotracheoesophageal Clefts

• Pathophysiology: posterior cricoid lamina does not fuse or tracheoesophageal septum does not develop

• associated with TE fistulas, laryngomalacia, congenital heart defects, cleft lip/palate, Down syndrome, others

1. Opitz-Friass (G-syndrome): hypertelorism, cleft lip/palate, hypospadias

2. Pallister-Hall: hypothalamic hamarblastoma, hypopituitary, imperforate anus, postaxial polydactaly

• SSx: inspiratory stridor (like laryngomalacia), pneumonia, aspiration for Type II-IV

• Dx: endoscopy, esophagram

• Classification Types

I. supraglottic arytenoid only II. extends past true vocal fold

III. extends past cervical trachea

IV. extends past thoracic trachea

• Rx: consider tracheotomy, Nissen fundoplication, endoscopic repair for Type I & II, anterior vs. lateral external approaches

Choanal Atresia (CHARGE Association): (see pp. 15-16)

Laryngitis Acute Viral Laryngitis

• Pathogens: rhinovirus (most common), parainfluenza, respiratory syncytial virus, adenovirus, influenza virus, pertussis

• SSx: dysphonia, low-grade fever, hoarseness, cough, rhinitis, postnasal drip

• Dx: clinical history and exam

• Rx: conservative management required (hydration, pyretics, voice rest, decongestants, humidification, smoking cessation), antibiotics not indicated unless suspect secondary bacterial infection

Adult Supraglottitis

• Pathophysiology: typically secondary to purulent rhinosinusitis or tracheobronchitis

• Common Pathogens: H. influenzae (most common), S. pneumoniae, S. aureus, B-hemolytic Streptococcus

• SSx: fever, muffled voice, dysphagia, stridor (inspiratory), obstructive symptoms may progress within hours

• Dx: lateral neck plain films, flexible nasopharyngoscopy, indirect laryngoscopy

1. evaluate airway, severe and progressive symptoms may require fiberoptic intubation versus an urgent surgical airway

2. humidification, hydration, corticosteroids, H2-blocker

3. parenteral antibiotics

Reflux-Induced Laryngitis

• Pathophysiology: inflammatory response of laryngeal mucosa from Laryngopharyngeal Acid Reflux (LPR)

• SSx: hoarseness (worse in the morning), choking spells at night, regurgitation, bitter taste in mouth, globus sensation, cough, chronic throat clearing, postprandial heartburn (seen <50% of the time in

LPR)

• Laryngeal Findings: erythema and edema of the posterior commissure, arytenoids, superior surface of the vocal folds, and laryngeal surface of the epiglottis; diffuse supraglottic edema; laryngeal pachydermia (interarytenoid); granulomas of the vocal process

• for diagnosis and management see pp. 149—150

Acute Spasmodic Laryngitis (False Croup)

• common in toddlers

• Pathophysiology: uncertain noninfectious etiology, may be secondary to GERD or allergy

• Associated: anxiety, allergies

• SSx: nocturnal stridor and respiratory distress (not present during the day), otherwise normal head and neck exam aside from the stridor

• Dx: clinical history and exam

• Rx: supportive care (anti-reflux regimen), address allergies

Bacterial Tracheitis (Membranous Laryngotracheobronchitis—MLTB)

• Pathophysiology: bacterial superinfection

• SSx: thick secretions in airway, fibrinous membrane in trachea, high fever

• Dx: endoscopy with cultures

• Rx: aggressive pulmonary toilet, parenteral antibiotics, may require endotracheal intubation (pulmonary toilet)

Diphtheria

• uncommon since immunization (milder form may present despite immunization)

• Pathogen: Corynebacterium diphtheria

• Risks: nonimmunized children >6 years old

• SSx: sore throat, progressive airway obstruction, thick, gray-green plaques, membranous, friable exudate on tonsils, pharynx, and larynx, low grade fever, acetone breath

• Dx: flexible nasopharyngoscopy, culture and smears

• Complications: nephritis, airway obstruction, death (secondary to neurological toxin)

• Rx: establish airway via tracheotomy, avoid intubation (may dislodge exudative plaques causing an acute airway emergency), diphtheria antitoxins, antibiotics (penicillin or erythromycin), humidity

Croup (Acute Laryngotracheobronchitis, LTB) (see Table 3-3)

• most common cause of stridor in children

• primarily involves the subglottic region

• Pathogen: parainfluenza 1 (most common cause), parainfluenza 3, influenza A, rhinovirus, respiratory syncytial virus

• Risks: children 1-5 years old during fall and winter seasons

• SSx: inspiratory or biphasic stridor, gradual onset (over days), long course (3-7 days), low grade fever, relief in the recumbent position, brassy cough (worse at night), hoarse, non-tender larynx, no dysphagia, no drooling

TABLE 3—3. Contrasting Acute Laryngotracheobronchitis (Croup) and Epiglottitis

Acute Laryngotracheobronchitis

Acute Epiglottitis

Pathogen

Parainfluenza virus 1

Haemophilis influenze B

Age

<5 years old

2—6 years old

Location

subglottic

supraglottic

Onset

gradual (days)

sudden onset (hours)

Cough

barky

normal

Posture

supine

upright

Drooling

no

yes

Fever

low grade

high fevers

Radiographs

steeple sign

thumbprinting

Treatment

supportive

airway management

and antibiotics and antibiotics

• Dx: clinical history and exam, plain neck films ("steeple" or "wine bottle" sign, narrowed subglottis), flexible nasopharyngoscopy

• Complications: pulmonary edema, pneumonia, membranous laryngotracheobronchitis

Management

• Assess Airway: intubation or tracheotomy rarely required unless there is a coexisting laryngeal abnormality (subglottic stenosis)

• Medical Management: humidified oxygen, parenteral fluids, nebulized racemic epinephrine, corticosteroids (controversial), antibiotics not required unless suspect bacterial superinfection

• Endoscopy: indicated if no resolution with conservative management or if intubation or tracheotomy required

Epiglottitis (see Table 3-3)

• Pathogen: Haemophilis influenzae B

• Risks: children >1 year old to adult (most common between 2-6 years of age)

• SSx: sudden onset (hours) and short course, high fever, dysphagia, drooling, dyspnea, "sniffing position" (neck flexed and head extended), no cough, normal voice, tender larynx

• Dx: clinical history and exam, plain neck films ("thumbprint sign"), serum HIB capsule antigen, cultures

• Complications: septicemia, acute airway obstruction (death)

Management

• avoid aggravating patient (do not examine airway with a tongue blade, draw blood, perform rectal temperatures, etc)

• Establish Emergent Airway: intubation performed in the operating room with preparation for a tracheotomy

• Endoscopy: examine and culture epiglottis

• Postoperative Care: monitored bed, parenteral antibiotics and corticosteroids for 7-10 days (consider ampicillin [20% resistance] with chloramphenicol or cefuroxime), consider extubation after 2-3 days

Chronic Laryngitis

• Common Etiologies: smoking, pollution, vocal abuse, sinusitis, rhinitis, laryngopharyngeal acid reflux

• SSx: hoarseness, pain, edema, dysphagia, respiratory compromise

• Dx: flexible nasopharyngoscopy, videostroboscopy, endoscopy (thick erythematous vocal folds) with biopsy to rule out malignancy

• Rx: address etiology (stop smoking, voice rehabilitation, treat rhinosinusitis, reflux regimen), humidification, mucolytics, consider short course of corticosteroids

TB Laryngitis

• typically secondary to pulmonary TB

• Histopathology: cellular inflammation, granuloma in subepithelium, perichondritis

• Lesion: granulation and ulcerative tissue in posterior glottis (posterior interarytenoids most common, laryngeal surface of epiglottis, vocal folds)

• Rx: Isoniazid, Rifampin, voice rest, narcotics for pain

Syphilitic Laryngitis

• rare manifestation of oropharyngeal syphilis

• Secondary Stage SSx: temporary mild edema, painless

• Tertiary Stage SSx: gummas may break down cartilage

• Rx: penicillin, tetracycline, erythromycin

Scleroma of the Larynx/Rhinoscleroma

• Pathogen: Klebsiella rhinoscleromatis

• Histopathology: pseudoepitheliomatous hyperplasia of the larynx (similar to blastomycosis)

• Rx: long-term antibiotics as dictated by culture and sensitivity

Leprosy (Hansen's Disease)

• Pathogen: Mycobacterium leprae

• Lesion: ulcerative lesions in the supraglottis

• Dx: biopsy (foamy leprous cells containing the bacillus), nasal smear

• Rx: Dapsone (diaminodiphenylsulfone), corticosteroids

Perichondritis of the Larynx/Polychondritis

• Causes: infection (TB, syphilis, septic laryngitis), trauma, tracheotomy, radiation effect, malignancy

• most commonly involves the thyroid cartilage, rarely involves epiglottis since fibroelastic cartilage (adherent perichondrium) protects from infection

• SSx: insidious onset, fever, odynophagia, tenderness, hoarseness, cough, dyspnea

• Dx: endoscopy reveals pale mucosal edema, CT of neck

• Complication: subperichondrial abscess, stenosis (respiratory compromise)

• Rx: establish airway, aggressive antibiotic regimen, consider incision and drainage of abscess, consider surgical debridement of necrotic or exposed cartilage

• NOTE: Relapsing Polychondritis has 50% laryngeal involvement (see also p. 317)

Fungal Laryngitis

• Risks: immunocompromised (uncontrolled diabetes, AIDS, chronic corticosteroids, etc), radiation, poor nutrition status, debilitating illnesses, long-term antibiotics

• SSx: odynophagia, mucositis, dysphonia, cough, dyspnea, aspiration

• Dx: endoscopy and biopsy (may be confused with malignancy)

• Rx: establish airway, antifungal regimen

Pathogens

• Candidiasis (Moniliasis): adherent, friable, cheesy, white plaques; spread from oral cavity (see also p. 174)

• Aspergillosis: allergic, noninvasive, or invasive forms (see also p. 42)

• Blastomycosis: red laryngeal ulcers or miliary nodules on vocal fold (see also p. 211)

• Histoplasmosis: ulcerative lesions in larynx (anterior larynx and epiglottis) (see also pp. 210-211)

• Coccidiomycosis: nodular laryngeal mass (see also p. 211)

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