Juvenile nasopharyngeal angiofibroma| aetiology, diagnosis, treatment

Definition:

Congenital web is a medical condition in which the child's trachea (windpipe) is obstructed due to formation of a layer of web-like tissue, which results in breathing difficulties and other symptoms.

Causes: 

Incomplete canalization of the laryngeal lumen.

It is congenital, i.e it presents since birth, but is could be acquired in some cases.

Pathology: 

• Commonest site is glottic, may extend to subglottis.
• May be small, large, or imperforate fibrous ring.

Symptoms:

• Small web: asymptomatic, hoarse cry, mild stridor.
• Large web: weak hoarse cry, evident stridor.
• Imperforate web: Cyanosis with no cry usually fatal.

Signs: 

• Flexible or direct laryngoscopy: symmetrical grayish white membrane may be a semi translucent triangular band, or thick membrane.

Treatment of Congenital web:

A. Mild if no symptoms ………… no treatment.

B. Severe: 

• Tracheostomy if severe obstruction.
• Excision of web by:

      - Microlaryngosurgery (MLS) using laser or cold instrument.

      - Laryngofissure in very thick web

C: Imperforate web: Urgent tracheostomy or rigid bronchoscope to rupture the web.

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