Amyloidosis

Amyloidosis is a disorder caused by deposition of immunoglobulin-like proteinaceous material into one or more organs of the body. This disease process can be described as either primary or secondary.

Amyloid deposits (arrows) at the carina, where trachea splits into right and left mainstem bronchi, as well as a few centimeters down the left mainstem bronchus (smaller arrow). The deposits appear yellowish and widen or project from the tissues they infiltrate.

Secondary Amyloidosis

In secondary amyloidosis, abnormal B cells (part of the white blood cell family) overproduce these proteins, which are then deposited throughout the body: in heart muscle, tongue, gastrointestinal tract, kidneys, etc. An example of a disease causing secondary amyloidosis is multiple myeloma, a blood disease.

Primary Amyloidosis

An example of primary amyloidosis is when the deposits are found only in a single organ or location. This seems to be the case when amyloidosis affects the larynx.

Amyloidosis in the Larynx

In laryngeal amyloidosis, the deposits seem to be localized either just to the larynx, or to the larynx and pharynx. One sees what looks like yellowish candle wax within the tissues. The amyloid deposits are quite firm, and when biopsied, there is little bleeding.

Treatment for Laryngeal Amyloidosis

Because of their infiltrative nature, amyloid deposits typically cannot all be dissected out of the larynx; instead, then, an operating physician will aim to debulk the deposits in areas where they impair breathing or the voice. That is, when deposits are widespread in the larynx, there does not seem to be any point in removing them except in locations where removal will improve function. Often, repeated procedures are required over many years’ time, though occasionally the condition seems to stop progressing.

Amyloidosis, before and after debulking

Amyloidosis, before debulking (1 of 4)

Panoramic view. Submucosal amyloid deposits in pharyngeal walls and epiglottis, with examples at arrows.

Amyloidosis, before debulking (2 of 4)

View of anterior subglottis shows diffuse infiltration of yellowish, submucosal amyloid.

Amyloidosis, before debulking (3 of 4)

Phonation under strobe light shows deposit at anterior commissure tethering upper surface of left vocal cord, and resultant vibratory asymmetry.

Amyloidosis, after debulking (4 of 4)

Some months later, after laser debulking at short arrow, showing improvement of vibratory closure after tethering reduced. Note increase of nearby supraglottic amyloid deposit does not need to be addressed because it has no functional consequence to the patient, who has other asymptomatic deposits in subglottis, epiglottis, and nasopharynx.

Example 2

Amyloidosis, before debulking (1 of 8)

Panoramic view of a large spherical mass of amyloid material bulging submucosally (contour at dotted line) in the false and aryepiglottic cords. The patient has only a harsh stage whisper, and glottic voice only with inspiratory (inhaling) phonation. The posterior vocal cord and ventricle of the opposite side (left of photo) are visible, but the amyloid mass obscures all but a few millimeters of the posterior cord on its side.

Amyloidosis, before debulking (2 of 8)

Closer view.

Amyloidosis, before debulking (3 of 8)

View yet further down, showing the upper trachea (top-center of photo), the posterior end of one of the vocal cords (left of photo), and the edge of the amyloid mass (bottom-right of photo, again marked by a dotted line). The mass is again obscuring a view of the vocal cord on its side; in fact, it is pressing the cord downward, which affects the voice.

mass overlies most of both vocal cords — Amyloidosis, before debulking (4 of 8)

Phonation, while attempting to see the vocal cords beyond the large overhanging mass. The vocal cords are each marked with an F, one of the posterior ventricles with a V, and the amyloid mass with an A. This mass actually overlies most of both vocal cords and presses them both downward.

Amyloidosis, after debulking (6 of 8)

Looking into the deepest recess of the dissection. The parallel lines indicate the upper border of the thyroid cartilage.

Complete healing (8 of 8)

Many months after debulking, and with complete healing, the voice can pass for normal, and the laryngeal vestibule is no longer filled with the enormous bulk of amyloid. The nubbin at anterior false cord would only need removal if it became large enough to interfere with voice.

Amyloidosis of the Larynx as Seen Over Time, with Treatment

yellowish proteinaceous material — Primary laryngeal amyloidosis (1 of 7)

An example of primary laryngeal amyloidosis of the larynx. In this case, the yellowish, “candle-wax” proteinaceous material is being deposited just below the margin of the vocal cords (arrows).

Bulky swelling (2 of 7)

When this person produces voice, the bulky swelling just below the margin of the vocal cords creates turbulence, incomplete match, and a rough voice quality. After laser debulking, the patient had a much improved voice for many years.

Amyloidosis (3 of 7)

Eight years later, the patient reappeared. She said voice had been good for many years but had been getting increasingly hoarse for the prior couple of years. Here you see major re-deposition of amyloid material.

Vocal cords cannot close completely (5 of 7)

During voice production under strobe light, the amyloid deposit under the left vocal cord prevents closure.

Amyloids Remain (6 of 7)

A year after laser debulking, the patient continued to have a very good voice, and only reappeared due to an unrelated question. As expected, smally amyloid deposits remain.

Amyloid doesn't affect voice — Voice remains clear (7 of 7)

When she produces voice, match and vibratory ability are very good, explaining her normal voice. It remans to be seen if amyloid will gradually reaccumulate over the next many years and need another debulking.

Proteinaceous deposits of primary laryngeal amyloidosis can occur anywhere and “everywhere” in the larynx

Airway Passage (1 of 4)

This middle-aged woman has experienced gradual deterioration of voice across several years. Note the narrowed airway and protruding lesions.

Submucosal Masses (2 of 4)

In a closer view, one gets the sense of firm submucosal masses (most prominent deposits circled, at arrows). Note the yellowish cast as well. Vocal cord margins are marked with dotted lines.

yellowish amyloid deposits — Amyloid Deposits (3 of 4)

At closer range, the yellowish cast typical of amyloid deposits is seen better.

Diffuse Subglottic Infiltration (4 of 4)

Just below the vocal cords, diffuse subglottic infiltration is also seen, with the most prominent deposit posteriorly at the arrow.

Laryngeal Amyloidosis

This video gives an example of amyloidosis, which is a condition in which abnormal proteins (called amyloids) become deposited in the spaces between cells.

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Amyloidosis

Secondary Amyloidosis

Primary Amyloidosis

Amyloidosis in the Larynx

Treatment for Laryngeal Amyloidosis

Amyloidosis, before and after debulking

Amyloidosis, before debulking (1 of 4)

Amyloidosis, before debulking (2 of 4)

Amyloidosis, before debulking (3 of 4)

Amyloidosis, after debulking (4 of 4)

Example 2

Amyloidosis, before debulking (1 of 8)

Amyloidosis, before debulking (2 of 8)

Amyloidosis, before debulking (3 of 8)

Amyloidosis, before debulking (4 of 8)

Amyloidosis, after debulking (5 of 8)

Amyloidosis, after debulking (6 of 8)

Amyloidosis, after debulking (7 of 8)

Complete healing (8 of 8)

Amyloidosis of the Larynx as Seen Over Time, with Treatment

Primary laryngeal amyloidosis (1 of 7)

Bulky swelling (2 of 7)

Amyloidosis (3 of 7)

Amyloid deposits (4 of 7)

Vocal cords cannot close completely (5 of 7)

Amyloids Remain (6 of 7)

Voice remains clear (7 of 7)

Proteinaceous deposits of primary laryngeal amyloidosis can occur anywhere and “everywhere” in the larynx

Airway Passage (1 of 4)

Submucosal Masses (2 of 4)

Amyloid Deposits (3 of 4)

Diffuse Subglottic Infiltration (4 of 4)

Laryngeal Amyloidosis

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