1 Chapter 99: Congenital Disorders of the Larynx ... - Famona Site

Endoscopic findings. A lateral airway radiographic study usually shows the congenital
subglottic stenosis clearly (Fig. 99-21). However, the definitive diagnosis is made at
endoscopy; the diameter of the subglottic region is assessed carefully and gently, especially
if the airway is marginal. The usual finding is an isolated circumferential subglottic stenosis,
the diameter of which is then measured. The outside diameter of a bronchoscope or an
endotracheal tube that passes comfortably through the subglottic region represents the internal
diameter of the cricoid, which is therefore calibrated and recorded for future reference. The
endoscopic findings are less severe than those in patients with acquired subglottic stenosis.
Occasionally diagnostic endoscopy results in minimal but significant mucosal swelling
in the subglottic region, caused by the trauma of excessive manipulation, the passage of too
large a bronchoscope, or repeated manipulation. This is more significant when there is a
preexisting subglottic stenosis. Subglottic edema occurs within the limits of the inexpansible
cricoid cartilage in the immediate postendoscopic period. There may be croupy cough, stridor,
and increasing airway obstruction. Careful technique should reduce the incidence of this
complication to an absolute minimum.
Management
Management is based on the knowledge that children outgrow congenital subglottic
stenosis. Most cases produce few or minimal symptoms, and no definitive treatment is
required unless acute inflammation or trauma from intubation or endoscopy precipitates
serious obstruction. However, when a major degree of congenital subglottic stenosis exists or
when acquired fibrous tissue granulations and edema produce a serious obstruction, a
tracheotomy may be require. Small patients with a stenosis severe enough to require a
tracheotomy may require repeated endoscopic examination over months or years, awaiting
sufficient relative improvement of the subglottic diameter to allow decannulation. This
conservative management prevents the potential complications of external laryngeal surgery
in the infant. On the other hand, Cotton and Evans (1981), among others, have advocated a
more aggressive approach by laryngotracheal surgical reconstruction to obtain a lumen large
enough to permid decannulation. Repeated "dilation treatment" of hard cartilaginous stenosis
is not successful, and ill-judged forced "dilations" inevitably cause further trauma and
scarring. Laser resection has a limited application and must be restricted to treatment of the
soft tissue swelling in selected cases where the stenosis is less than 5 mm thick. There is no
uniform management of subglottic stenosis and no universally successful endoscopic or
external surgical technique. The tracheotomy tube usually should be worn for an extended
period of time to allow the cartilaginous stenosis to grow along with the child's general rate
of growth until the problem is overcome. Decannulation is usually possible even in difficult
cases by the age of 3 to 4 years.
Laryngeal hemangioma
Hemangiomas are congenital malformations that form from mesodermal rests of
vasoformative tissue. In most anatomic sites they can be managed conservatively, but
narrowing of the airway is an exception. Congenital hemangioma in the larynx usually occurs
in the subglottic region, developing in the submucosa and following a typical growth pattern,
with increased size usually causing symptoms within the first 8 weeks or so of life. A review
of autopsy cases (Brodsky et al, 1983) showed that the lesion sometimes extended into the
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