Ann Thorac Surg 2004;77:1829-1832
© 2004 The Society of Thoracic Surgeons
Case report
Idiopathic tracheal and bronchial stenosis in two pediatric patients
Emma Beddow, FRCS, Enga,
Alexander J. Rice, MDa,
Mary N. Sheppard, FRCPatha,
Peter Goldstraw, FRCS*a
a Department of Thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
Accepted for publication May 30, 2003.
* Address reprint requests to Mr Goldstraw, Department of Thoracic Surgery, Royal Brompton Hospital, Sydney St, London SW3 6NP, UK
e-mail: p.goldstraw{at}rbh.nthames.nhs.uk
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Abstract
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Idiopathic tracheal stenosis, a rare benign chronic inflammatory and fibrotic entity, has previously been reported in the literature in adults, but never in children. Here we report two pediatric cases, one with the rarer condition of idiopathic bronchial stenosis, and we discuss the management and histopathologic findings.
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Introduction
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Idiopathic tracheal stenosis is a rare condition with bronchial stenosis being rarer still. Both are characterized by circumferential chronic inflammation and fibrosis and can only be truly idiopathic when other causes of stenosis have been investigated. Patients present with dyspnea, wheeze, or in more extreme cases, stridor requiring repeated bronchoscopic bougienage to the stenosed segment or surgical resection.
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Case reports
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Patient 1
An 11-year-old boy, wheezy and dyspneic since age 5, had been treated elsewhere with bronchodilators, which were mildly beneficial. There was no previous history of infection, intubation, vasculitis, or trauma. A computed tomographic scan of the chest demonstrated a stenosis in the central trachea, and flow volume loops showed severe inspiratory and expiratory restriction. At surgery, the tracheal rings appeared narrower and more numerous than normal, but the stenosis was not palpable, and bronchoscopy was required to localize the area for resection. On incising the anterior wall of the trachea it was obvious that the stricture was circumferential and involved half the length of the trachea. This was excised and it contained nine thin tracheal rings. An end-to-end tracheal anastomosis was performed. The patient had an uneventful recovery and was discharged home on postoperative day 5. He remains well 10 months later.
Microscopic histologic evaluation of the specimens showed marked erosion of the surface epithelium by vascular granulation tissue with a mixed acute and chronic inflammatory cell infiltrate, including increased numbers of eosinophils. Where it was intact, the bronchial epithelium showed regenerative squamous metaplasia. The underlying lamina propria was expanded by dense fibrovascular connective tissue in which there was patchy perivascular chronic inflammation. Scattered interstitial eosinophils and mast cells were also present, but lymphoid follicles were not seen. Mucus glands were dilated with periglandular edema and acute inflammation secondary to obstruction.
Sputum, blood, urine, and resected tissue were all negative for bacteria, fungi, or vasculitic markers.
Patient 2
An 11-year-old Indian girl who was hepatitis B positive was referred with a 3-month history of severe stridor 7 months after a febrile illness with hemoptysis and dyspnea requiring ventilation and temporary tracheostomy. At rigid bronchoscopy the larynx and upper trachea were normal. A tight stricture was seen 6 cm from the larynx, 2 mm in diameter, extending distally to just proximal to the carina. Both main bronchi appeared normal. There was no evidence of tracheomalacia.
Repeated bougienage gave short-lasting relief of symptoms, but surgery was deemed the most effective long-term treatment. At surgery a transverse cervical incision extending to the chest with a limited median sternotomy was performed. The trachea was mobilized anteriorly from the tracheostomy scar to the carina. Below the scar there were 2 cm of palpably normal trachea above the carina; however, on incising the trachea the stricture was found to be below the site of the previous tracheostomy and consisted of laminated fibrous tissue within normal tracheal rings. This circumferential process appeared to extend up to but not including the carina. The trachea was excised from carina to tracheostomy scar, approximately 2.5 cm in length, along with a small area extending onto the proximal anterolateral right main bronchus. An end-to-end tracheal anastomosis was performed after a suprahyoid release.
Six months later she became dyspneic and was found to have a severe stricture of the middle portion of the right main bronchus. At surgery the stricture was seen in the right main bronchus distal to the previous anastomosis that appeared intact. The right main bronchus was transected and a short segment excised. The lobar bronchi were of normal appearance. The right lung was then reimplanted into the medial wall of the left main bronchus. The patient made a good recovery from surgery, left the hospital alive and well, and returned to India.
Histopathologic evaluation of both specimens showed were very similar as featured in the previous case, although the inflammatory process appeared more active. There was marked ulceration of the surface epithelium with focal squamous metaplasia. Associated with this there was expansion of the lamina propria by florid granulation tissue with underlying cellular fibrovascular tissue (Figs 1, 2).
In addition there were also scattered large lymphoid aggregates with reactive germinal centers, plasma cells, and increased numbers of interstitial mast cells and eosinophils, especially
at the site of maximal stenosis. Both specimens showed circumferential involvement. They did not have the typical fibrotic histologic features normally seen with a posttracheostomy tracheal stenosis, and the resection margins of the tracheal segment excised at the first operation showed normal epithelium. The stricture found at the second operation was in the middle of the right main bronchus. It did not involve the trachea at the site of the previous anastomosis.
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Fig 1. Thickening of mucosa by dense fibro-inflammatory tissue with surface ulceration. (Hematoxylin & eosin; x40 original magnification.)
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Fig 2. Bland fibroblasts in dense collagenous stroma (Hematoxylin & eosin; x200 original magnification.)
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In both patients, the inflammation and fibrosis were confined to the lamina propria and did not involve the cartilage or peri-tracheal connective tissue. The histologic features were not typical of fibromatosis and there was no evidence of polychondritis, vasculitis, granulomas, foreign material, or amyloid. Special stains for organisms were negative. Sputum, blood, and resected tissue were negative for bacteria, fungi, or vasculitic markers.
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Comment
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Causes of tracheal and bronchial stenosis are diverse and include congenital causes, trauma, infection, inhalational burns, and amyloid deposition. Congenital stenoses are usually identified in infancy and other causes are identified by clinical history or histopathologic examination, or both. Thorough investigation into other known causes of tracheal inflammation and stenosis is required to diagnose a patient with truly idiopathic disease.
Reported cases of idiopathic tracheal stenosis are few. Grillo and colleagues [1] reported a series of 49 cases, the largest number reported. Maddaus and colleagues [2] and Corraud and colleagues [3] reported fewer cases along with some single case reports [4, 5]. They all showed the typical features of circumferential fibrotic stenosis with fibrous expansion of the mucosa, inflammation, and in some patients ulceration with granulation tissue formation and squamous metaplasia.
In our first patient, the fibrous tissue was densely collagenous with few fibroblasts and minimal chronic inflammation in the fibrous areas. In contrast, our second patient was characterized by cellular fibrovascular tissue with marked chronic inflammation including lymphoid aggregates and plasma cells, and increased numbers of eosinophils and mast cells. The recurrence of this process in the right main bronchus, distal to an intact anastomosis from the previous operation and after adequate tracheal resection of the original tracheal stenosis, along with these histologic features, differentiates this case from a simple posttracheostomy tracheal stenosis.
Several cases of tracheal stenosis have been reported in association with other idiopathic fibro-inflammatory conditions, including mediastinitis [6] and retro-orbital pseudotumour [7], and the histologic features of these and the changes described in the trachea showed some overlap.
There is a strong female predominance in idiopathic tracheal stenosis with only 3 of the 49 patients in Grillo and colleague's [1] series being male and with the other series [2, 3] showing similar ratios. The cause for this is unknown. Cases of idiopathic bronchial stenosis are even rarer. There is a single case report of idiopathic bronchial stenosis in an 18-year-old woman involving the left mainstem bronchus and right bronchus intermedius. A biopsy of the lesion was taken, but the histologic description given briefly as "hyperplastic changes" of the bronchial wall with no mention of the presence or absence of fibro-inflammatory changes.
The cause of fibro-inflammatory stenosis of the upper airways is not well characterized, but it may represent an immune hypersensitivity reaction. Reported associations of inflammatory upper airway stenosis with autoimmune diseases, including ulcerative colitis and Crohn's disease, also support this link. However, in our two rare pediatric cases there was no evidence of association with any such autoimmune disease.
The management of this rare condition is initially by repeated bronchoscopy and dilatation of the stenotic segment. Our second patient underwent this regime with effective relief of symptoms for 3 months before surgery. Grillo and colleagues [1] advocate that surgical resection should be restricted to those in whom this regime fails to provide adequate relief of symptoms or when a repeated bougienage becomes too frequent and hazardous to continue safely.
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References
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- Grillo H.C., Mark E.J., Mathisen D.J., Wain J.C. Idiopathic laryngotracheal stenosis and its management. Ann Thorac Surg 1993;56:80-87.[Abstract/Free Full Text]
- Maddaus M.A., Toth J.L.R., Gullane P.J., Pearson F.G. Subglottic tracheal resection and synchronous laryngeal reconstruction. J Thorac Cardiovasc Surg 1992;104:1443-1450.[Abstract]
- Corraud L., Jougon J., Velly J.F. Surgical treatment of nontumoral stenoses of the upper airway. Ann Thorac Surg 1995;60:250-260.[Abstract/Free Full Text]
- Havas T., Dodd M., Weldon B., Benjamin B., Pigott P. A case report of subglottic stenosis. Aust N Z J Surg 1984;54:291-294.[Medline]
- Mikaelian D.O. Idiopathic subglottic stenosis in an adult. J Laryngol Otol 1974;88:467-472.[Medline]
- James E.C., Harris S.S., Dillenburg C.J. Tracheal stenosis: an unusual presenting complication of idiopathic fibrosing mediastinitis. J Thorac Cardiovasc Surg 1980;80:410-413.[Abstract]
- Brandenburg J.H. Idiopathic subglottic stenosis. Trans Am Acad Ophthalmol Otolaryngol 1972;76:1402-1406.[Medline]
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Abstract
Introduction
