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Nathaniel's Airway

Nathaniel's Airway

I have mentioned Nathaniel's airway difficulties in other blog posts.  With our upcoming trip to Cincinnati Children's Hospital Aerodigestive Center, I want to explain his conditions as we understand them today.

Our local medical team has diagnosed Nathaniel with congenital stage 3 subglottic stenosis with a laryngeal web. The subglottic area of the trachea is directly below the vocal cords. Stenosis means narrowing. Staging is rated 1-4; stage 4 is completely closed.

The bottom right photo in the picture above is a recent view of Nathaniel's airway. The hole is his open airway and is approximately 1.6mm in diameter. While the hole seems big in isolation, it is not. The width is shown in the above photo on the left. The space between the metal forks held above the penny represents Nathaniel's airway. For perspective, the narrowest point of a newborn's trachea is normally between 4mm and 5mm.

The two X's on the photo mark Nathaniel's vocal cords. They are encompassed in tissue - this is a laryngeal web. Normal vocal cords are pictured in the top right photo. The narrowing in Nathaniel's airway starts at his vocal cords and extends down below the vocal cords into the subglottic region of trachea. An individual can have subglottic stenosis without a web in the vocal cords or have a web in the vocal cords with no subglottic stenosis. Nathaniel has both making his anatomy more complicated than either alone.

The trachea becomes a hollow tube by the eighth week of embryonic development; congenital (from birth) webbing and stenosis remain when tissue does not reabsorb at this very early stage in a pregnancy. This combined condition of subglottic stenosis and laryngeal web is very rare,  comprising less than 5% of trachea abnormalities. Only 30% of children with the condition are severe enough to require a trachesotomy. Nathaniel received his trachestomy at birth. Without his trachesotomy tube in place, Nathaniel experiences significant and immediate respiratory distress.  Most children with severe webbing and stenosis like Nathaniel are considered aphonic - or unable to voice. It is unclear if his vocal cords can move.

Severe subglottic stenosis like Nathaniel's usually requires open airway reconstruction which can include rebuilding the trachea with pieces of cartilage taken from the child's rib cage. Prior to surgery, a child's respiratory and digestive systems must be evaluated. Stomach reflux or aspiration complicate the reconstruction surgery. The new airway is delicate and must be protected, so these conditions usually must be resolved prior to being considered a candidate for reconstruction surgery. Nathaniel is being treated for reflux; there is currently no explanation why he aspirates. It is speculated that there is a problem with his recurrent laryngeal nerve which triggers the muscles that control swallowing.

For a more extensive explanation of these conditions, please see the following web resources:

Aphonia
Airway Reconstruction
Laryngeal Web
Subglottic Stenosis

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