Idiopathic Stenosis

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Cicatricial stenosis with a lesser inflammatory component, localized in the subglottic larynx and upper trachea, occurs without known cause.23,24 This process is labelled idiopathic laryngotracheal stenosis (Figure 14-9). None of these patients have been intubated for ventilation or have suffered external or internal trauma to the trachea. The lesions are not congenital. There are no findings in this group of patients of associated mediastinal fibrosis or lymph node involvement by any pathologic process. Few patients have histories or findings suggesting esophageal reflux and aspiration. None have had specific or nonspecific tracheal infections, nor did they later develop manifestations of systemic disease such as polychondritis, or amyloid. A few with stenosis due to Wegener's granulomatosis confined to the upper airway were initially misdiagnosed as idiopathic stenosis, prior to routine screening with an anti-neutrophil cytoplasmic antibody (ANCA) test. In a series of 73 patients, 71 were female. Age distribution varied widely from 13 to 74 years, but the disease was seen chiefly in the third, fourth, and fifth decades of life.24 Initial symptoms of dyspnea on effort (in 52%) progressed to dyspnea at rest, with noisy breathing, wheezing, or stridor (in 48%). The duration of symptoms prior to initial presentation varied between 4 months to over 30 years, with the greatest number reporting 1 to 4 years of symptoms. Careful history is necessary to identify the often subtle

Grillo Anastomosis

extends to varying distances into upper trachea. Narrowing usually begins shortly below the vocal cords, but enough space remains below the glottis for laryngotracheal resection and adequate primary anastomosis. C, In this case, the stenosis is more severe immediately below the vocal cords, with the lumen, even at that level, being very narrow and possibly inadequate for anastomosis. Reproduced with permission from Grillo HC et al.23

extends to varying distances into upper trachea. Narrowing usually begins shortly below the vocal cords, but enough space remains below the glottis for laryngotracheal resection and adequate primary anastomosis. C, In this case, the stenosis is more severe immediately below the vocal cords, with the lumen, even at that level, being very narrow and possibly inadequate for anastomosis. Reproduced with permission from Grillo HC et al.23

onset of the earliest symptoms. Patients were frequently misdiagnosed as having asthma. It is essential to obtain ANCA titer on every patient to rule out Wegener's disease.25 Bronchoscopic biopsies are not diagnostic of either disease. Nasoseptal biopsies, on the other hand, may identify Wegener's granulomatosis histologically. Prior to the 1993 report by Grillo and colleagues, only limited or individual case reports were available.23

Koufman, in a detailed study of the laryngeal effects of gastroesophageal reflux, noted that 30% of otolaryngologic patients had pharyngeal reflux, as determined by a double pH probe technique.26 Seventy-eight percent of patients with a laryngeal stenosis from any cause, but principally of postintubation origin, showed pharyngeal reflux. Maronian and colleagues, using three to four port pH probes, with one positioned proximal to the upper esophageal sphincter, recorded a pH of less than 4.0 in 5 of 7 patients with isolated idiopathic subglottic stenosis.27 No control data were presented, however, as has most often been the case in assessing the significance of laryngeal gastroesophageal reflux. The significance of these findings as indicators of possible cause for idiopathic stenosis remains uncertain. Fifteen of our patients had a history or symptoms of gastroesophageal reflux. It must be noted that none of our patients who were operated upon for idiopathic stenosis suffered later progression of stenosis.24 This would have been expected if untreated reflux was an important etiologic factor.

In most cases, radiologic study showed a circumferential lesion of varying length, most often between 1 to 3 cm, centered at the junction between the cricoid cartilage and trachea (Figure 14-10). In 4 cases, the subglottic larynx itself was not involved, but in another 69 cases, subglottic involvement was of varying degrees of severity. The narrowing most often began shortly below the vocal cords and rapidly became a more severe to maximal stenosis at approximately the cricoid level. Although the involvement might be eccentric, it was always circumferential. Vocal cord function appeared normal. Longitudinal roentgenograms brought out the extent of the lesion and its nature most clearly. Flow volume loops, as expected, demonstrated extrathoracic fixed obstruction. Cultures obtained from biopsies and from surgical specimens showed nothing but the usual upper respiratory flora. Skin tests and serologic tests, done

figure 14-10 Images of upper airway of a 50-year-old woman with typical idiopathic laryngotracheal stenosis. A, Tomogram of the larynx and upper trachea. The false and true vocal cords are well outlined at the top. The stenosis involves the subglottic larynx and uppermost trachea. Compare with Figure. 14-9B. B, Computed tomography scan of the neck. The circumferential disposition of the stenosis lies within the ring of cricoid cartilage. Occasionally, the lesion is eccentric, although still circumferential.

figure 14-10 Images of upper airway of a 50-year-old woman with typical idiopathic laryngotracheal stenosis. A, Tomogram of the larynx and upper trachea. The false and true vocal cords are well outlined at the top. The stenosis involves the subglottic larynx and uppermost trachea. Compare with Figure. 14-9B. B, Computed tomography scan of the neck. The circumferential disposition of the stenosis lies within the ring of cricoid cartilage. Occasionally, the lesion is eccentric, although still circumferential.

especially in the earlier patients, were negative for tuberculosis, coccidioidomycosis, histoplasmosis, and blastomycosis. On bronchoscopy, the mucosa over the lesion appeared to be injected, and bled easily. Granulation tissue was uncommon (Figure 14-11). Ulceration was identified in only 1 patient. When these patients presented, the diameter of the aperture ranged from 3 to 10 mm, with most lying between 5 to 7 mm. The trachea distal to the stenosis appeared normal (see Figure 33 [Color Plate 15]).

Since the origin of a laryngotracheal and upper tracheal idiopathic stenosis is not understood, and since the entity had not been previously studied or followed over a long period of time, my initial approach was conservative. Some lesions remained stable over the period of observation, but in others, the obstruction worsened, requiring frequent dilations. Linear extension was not seen, however. Seventy-three patients have been subjected to surgical resection and reconstruction, with 4 cases involving the upper trachea, and in 10 cases, a rim of lower cricoid cartilage as well. In 59 cases with subglottic laryngeal stricture as well, the anterior portion of the subglottic larynx was removed and the posterior portion of the stenosis was managed by laryngotracheoplasty, as described in Chapter 25, "Laryngotracheal Reconstruction" (Figure 14-12). Thirty-six of these 59 patients required posterior laryngeal cricoid resurfacing with membranous tracheal wall. Initially, a protective complementary tracheostomy was performed in patients requiring laryngotracheoplasty. However, this was soon found to be unnecessary as a routine measure. Seven of 73 patients had temporary tracheostomies, but of the last 30 patients, only 1 required this. In 2 patients, in whom the stenosis was extremely severe and extended upward to the undersurface of the vocal cords, laryngofissure, excision of the scar, and resurfacing with buccal mucosa were performed. In 72 patients

figure 14-11 Bronchoscopic findings. A, View through separated vocal cords, demonstrating the funnelled subglottic larynx, with the circumferential ring of a high idiopathic stenosis visible below. Correction required circumferential laryngotracheoplasty as described. B, Closer intralaryngeal view affirms the circular nature of the stenosis. Its short length and the normal tracheal rings below are well seen. No granulation tissue is present but the mucosa is easily abraded. Also, see Figure 33 (Color Plate 15).

figure 14-12 Laryngotracheal resection and reconstruction for idiopathic stenosis, in a 55-year-old woman with a 3-year history of progressive symptoms. A, Preoperative tomogram. The solid arrow indicates the glottis; the open arrow indicates the stenosis. B, Postoperative tomogram. The arrow is at the glottis. Vocal cords are symmetrical. Note the slight indentation at the level of the anastomosis. The patient continued to do well 22 years after the operation.

with proximal idiopathic laryngotracheal stenosis, who underwent operation and had long-term follow-up (median 8 years), 19 (26%) attained excellent results in respect to voice and airway, 47 (64%) had good results, and 5 had only fair results and required occasional dilation (Figure 14-13). One case with poor result required at least annual dilation. In only this patient was there evidence of extension of the inflammatory process beyond that originally seen, suggesting a different process. Of the patients followed conservatively, none demonstrated spontaneous regression or regression in response to other treatments, including systemic or local corticosteroids.

In light of our observations in 73 patients of 1) long-term good results from surgical treatment in most, and 2) failure of recurrence or progression of the disease, it is difficult to understand the contradictory results of Dedo and Catten, who reported relentlessly progressive disease and complete failure in their 7 patients treated surgically.28

Pathologically, fibrosis was generally circumferential and of even thickness. Preeminent was dense collagenous fibrosis of keloidal type, which thickened the lamina propria of the trachea (Figure 14-14).23 Fibroblasts were relatively sparse. The surface epithelium usually showed squamous metaplasia. Inflammation was not prominent. Granulation tissue was also seen. Sometimes, this might well have been related to prior dilations, lasering, or other treatment. Cartilaginous rings were intact and essentially normal. There were no histologic characteristics to suggest a relapsing polychondritis, vasculitis, Wegener's granulomato-sis, or amyloid, and calcification or stainable organisms were not seen. The location, configuration, gross appearance, and microscopic appearance of these upper airway stenoses are similar enough to suggest a definable disease entity. The predominance of the disease in females deserves notice.

In idiopathic lesions that involve the subglottic larynx (and these are in the majority), airway narrowing usually begins shortly below the vocal cords. It takes refined and experienced judgment to decide which patients should be operated upon. A reasonably sized "atrium" is needed below the vocal cords to provide an adequate luminal size for successful anastomosis. It should allow a luminal cross-sectional area of the airway, at least 50% of normal. The obliquity of the anastomosis helps to enlarge the new laryngotracheal junc-

Inflammed Trachea From Acid Reflux
and expiratory flows. Reproduced with permission from Grillo HC et al.23

figure 14-14 Pathologic findings in idiopathic stenosis. A, Gross specimen, cross sections oftrachea. The dense fibrosis lies inside of intact cartilaginous rings. B, Photomicrograph reveals keloidal fibrous tissue, which replaces the lamina propria of the tracheal mucosa. Inflammatory changes are found in many near the mucosal surface, and frank granulation tissue in a few (hematoxylin and eosin; x125 original magnification).

Tracheal Mucosal Inflammation

tion. Contraindications to operation are a significant stenosis that begins within 5 mm of the glottis and, less permanently, the presence of florid inflammation and/or granulation tissue in the stenosis. If the stenosis reaches or nearly reaches the undersurface of the vocal cords, then treatment by periodic dilation may be the best option. If florid inflammation and granulation tissue are present, the operation should be deferred. Periodic mechanical dilation (see Chapter 19, "Urgent Treatment of Tracheal Obstruction"), with or without local corticosteroid injection, permits temporizing for as long as is necessary.

Operative failure here would likely be complete and permanent, with scant opportunity for a second repair. Furthermore, the recurrent laryngeal nerves are at risk in all of these patients. Results are also limited in many patients by a permanent slight weakness in the ability to project voice and in a diminished ability to sing, which are common sequelae (42 of 67 patients with good to excellent surgical results) after surgical reconformation of the larynx.

In a much smaller number of patients, a different stenotic process, also idiopathic, was encountered at the supracarinal and carinal levels, which also involved the main bronchi (Figure 14-15). In another small number of patients, a more inflammatory stenosing process would usually (but not always) spread over time, to involve almost the entire trachea and main bronchi (Figure 14-16). In view of the persistently anatomically localized nature of the upper idiopathic tracheal stenoses, it seems likely that these few cases

figure 14-15 Idiopathic stenosis of the distal trachea and carina in a 62-year-old woman with a 10-year history of "asthma," surgical correction of a high tracheal stenosis, with finding of carinal stenosis a year later, the duration of which is unknown. A, Tracheobronchogram showing stenosis of both main bronchi (arrows). Oblique view. B, Bronchoscopic view of the carina in this patient. C, Surgical specimen. Trachea was anastomosed end-to-end to the left main bronchus. The right main bronchial stump was implanted in the right wall of the trachea above the other anastomosis. The result remained good.

figure 14-15 Idiopathic stenosis of the distal trachea and carina in a 62-year-old woman with a 10-year history of "asthma," surgical correction of a high tracheal stenosis, with finding of carinal stenosis a year later, the duration of which is unknown. A, Tracheobronchogram showing stenosis of both main bronchi (arrows). Oblique view. B, Bronchoscopic view of the carina in this patient. C, Surgical specimen. Trachea was anastomosed end-to-end to the left main bronchus. The right main bronchial stump was implanted in the right wall of the trachea above the other anastomosis. The result remained good.

of more diffuse and sometimes progressively longer stenoses are of other etiology or etiologies. These few patients did not show clinical or pathological characteristics of histoplasmosis.

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