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CONGENITAL LARYNGEAL WEBS

DURING the sixth week of fetal life the developing laryngeal lumen is obliterated by
proliferating epithelium. The lumen is normally re-established by the tenth week; failure of the
is resorption process leads to a spectrum of conditions characterized by luminal narrowing.
Complete atresia can occur and unless treated immediately at birth, is incompatible with
extrauterine life. Partial atresia in the subglottic region usually produces a cuff of fibrous tissue
within the cricoid cartilage which narrows the lumen circumferentially-this condition known as
congenital subglottic stenosis. At the glottic level the atresia is web-like; the web usually lies
anteriorly with a smooth concave posterior margin while the residual lumen lies posteriorly and
varies with the size of the web. The thickness of the web can vary from a thin membrane to a
thick fibrous bar extending downwards into the subglottic region anteriorly.
Congenital laryngeal webs are extremely uncommon and few otolaryngologists or paediatricians
have more than a few limited experience. Cohen has now reviewed his experience of 51 cases
seen over earlier thirty two years. An earlier report from Benjamin recorded 29 cases seen over
twelve years. There do not seem to be any specific predisposing factors in patient
characteristics, race, sex, or family history, nor a history of difficulties in pregnancy or at birth.
Associated anomalies were recorded in 26 of Cohen's patients and 17 of Benjamin's. Most of
these abnormalities occurred elsewhere in the respiratory tract but other organ systems were
occasionally affected.
Clinical presentation and management depend on the size and nature of the web. The commonest
symptom is a vocal abnormality. The obstructive component of the web reduces the volume of
voice, while restriction of anterior vocal cord movement shortens the vibrating surface, resulting
in increased pitch and limited range. The symptom is present from birth-the cry may be absent
but is usually weak an squeaky. Sometimes the abnormality goes unrecognized until speech
development occurs, and very occasionally the condition remains undiagnosed into adult life.
The second commonest condition feature, and the most important in terms of future
management, is upper airway obstruction with stridor, symptoms vary directly in proportion to
the size of the web. Obstruction may be so severe that emergency intubation or tracheostomy is
necessary and in such cases stridor may be absent because of the very restricted air flow. When
the web is small, stridor may be noted only during periods of stress such as crying or exercise.
Further diminution in the size of the air way occurs during infection which will exacerbate any
air way obstruction and may precipitate emergency intubation or tracheostomy. Recurrent
episodes of croup, especially before the age of six month when infectious croup is uncommon.
Similarly, recurrent attacks of tracheobronchitis or pneumonia may signify and underlying
laryngeal abnormality.
When the air way is severely compromised the immediate therapeutic need is to establish an
adequate airway by means of intubation or tracheostomy. The nature of the abnormality usually
becomes apparent at this time. In most cases, however, the diagnosis is made by elective direct
endoscopic examination when the size and nature of the web can be evaluated. This examination

has been improved greatly by advances in paediatric anaesthesia and by use of the Hopkins rod
telescope with fibreoptic illumination. When the web occludes more than a third of the glottis
aperture some form of treatment is usually required because of actual or potential airway
obstruction. If possible, treatment is best delayed until the child is three or four years of age.
Webs obstructing less than 50% of the laryngeal lumen may be divided endoscopically.
Traditionally division was carried out by microsurgical scissors or forceps followed by
bougienage, but more recently carbon-dioxide techniques have gained popularity although the
long-term results have yet to be evaluated. Repeated endoscopic procedures are usually
required. Intralaryngeal stents have been used to try and prevent restenosis but they usually
provoke a pronounced granulation response and a tracheostomy is then required to maintain the
airway. Larger and thicker webs are more effectively managed by an external laryngo-fissure
approach in which the thyroid cartilage is divided in the midline, the larynx entered, and the web
resected. A keel or stent is then inserted in to the reconstructed lumen to prevent restenosis.
Various patterns of keel have been used. Mc Naught described technique in which he used a keel
of tantalum wire inserted into the lumen through the incision the laryngeal cartilage and fixed
externally, more recently silastic keels have been used. The keel is removed after two months
when epithelialization of the resected area of the anterior larynx has occurred. The tracheostomy
is necessary throughout this period.
Surgical resection either by endoscopic or external routes is usually successful in establishing an
adequate airway. In Cohen's series, of 40 patients who underwent surgery a satisfactory airway
was achieved in all but 2, and the tracheostomy could be removed. Results for improvement in
speech are unfortunately less satisfactory. Cohen claimed improvement in all 40 of his treated
patients, but only 1 had a normal voice restored, and of 17 treated by Benjamin, the voice was
reported as good in 11 some improvement in 3, and no change in 3. The best results are obtained
with the smaller thinner anterior webs.
The therapeutic goal is therefore to provide an adequate airway. Restoration of normal voice has
not proved possible but improvement may be achieved, especially when the web is small enough
to be resected by endoscopic laryngeal surgery.