PEDBASE.org - The Pediatric Database - Detailed information of LARYNGEAL WEB

LARYNGEAL WEB

DEFINITION:

A congenital disorder of the larynx characterized by congenital stridor in infancy and hoarseness in childhood.

EPIDEMIOLOGY:

incidence: 5% of congenital laryngeal abnormalities
age of onset:
- newborn (stridor) -> childhood (hoarseness)
risk factors:
- sporadic
- M = F

PATHOGENESIS:

1. Background

differentiation of the larynx occurs between 4-10 weeks of gestation

2. Etiology

considered to be a developmental abnormality occurring between the 4-10th week of gestation where the epitheilial fusion between two sides of the larynx fails to dissolve resulting in incomplete recannulization of the primitive larynx
75% of congenital laryngeal webs are present at birth

3. Types

based on the degree of occlusion of the lumen

1. Type 1

35% covering the anterior glottis

2. Type 2

35-50% occlusion of the lumen with the vocal cords visible

3. Type 3

50-70% occlusion of the lumen with the vocal cords posssibly visualized
most common type

4. Type 4

75-90% occlusion with vocal cords not visualized

CLINICAL FEATURES:

1. Infancy

inspiratory and expiratory stridor +/- upper airway obstruction with respiratory distress
paroxysmal dyspnea +/- cyanosis
aphonia, weak or feeble cry
poor feeding +/- failure to thrive

2. Childhood

hoarseness, weak high-pitched voice, easy tiring of voice
recurrent respiratory tract infections
chronic cough
dyspnea on exertion

3. Associated Findings (in 10-15% of cases)

respiratory tract anomalies
cardiac anomalies - ToF, VSD

INVESTIGATIONS:

1. Direct Laryngoscopy/Bronchoscopy

diagnostic
98% are anterior and 2% posterior
location
- 75% - glottic
- 12.5% - supraglottic
- 12.5% - subglottic

MANAGEMENT:

1. Supportive

respiratory support with severe or life-threatening upper airway obstruction, i.e, intubation, tracheostomy

2. Surgery

1. Resection

scissors, CO2 laser, radiofrequency current
- may need repeated dilation with bougienge to prevent reformation of the web by scarring
external laryngofissure +/- intralaryngeal shunts or keels to prevent restenosis
no resection may be necessary for small webs

3. Prognosis

normal life span
may have a prolonged hoarseness of voice

Pediatric Database - LARYNGEAL WEB

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