Diffuse Tracheal Diffuse Tracheal NarrowingNarrowing

Lucas Edwards UMMS IVBeth Israel Deaconess HospitalAdvanced Radiology ClerkshipGillian Lieberman, MD

Lucas Edwards, UMMS IVGillian Lieberman, MD

October 2003

Patient PresentationPatient Presentation59-year-old male with a history of chronic illness returning for appraisal of his respiratory statusHe has been followed by a pulmonologist and has received methotrexate and prednisone therapy in the pastReview of systems and physical examination were unremarkableRecent labs: CBC within normal limits

ESR 4BUN 16

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CT ScanCT Scan

High attenuation tracheal wall with thickening and luminal narrowing

BIDMC PACS

Patient

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CT ScanCT Scan

High attenuation tracheal wall with thickening and luminal narrowing

Normal tracheal anatomy at similar level

Patient

BIDMC PACS BIDMC PACS

Comparison

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CT ScanCT Scan

Tracheal narrowing (11 mm) and wall thickening (4 mm)

Right main stem bronchus = 7 mm

Left main stem bronchus = 5 mm

Patient Patient

BIDMC PACS BIDMC PACS

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CT ScanCT Scan

Tracheal narrowing (11 mm) and wall thickening (4 mm)

Normal diameter of intrathoracic trachea

Male: 20 +/- 2 mmFemale: 17 +/- 2 mm

Patient

BIDMC PACS BIDMC PACS

Comparison

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CT ScanCT Scan

Right main stem bronchus 7 mm Left main stem

bronchus 5 mm

Normal diameter of main stem bronchi

Right: 15 mmLeft: 13 mm

Patient

BIDMC PACS BIDMC PACS

Comparison

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FindingsFindings

Diffuse calcification and wall thickening of trachea and main stem bronchi with associated tracheobronchial narrowingTracheal narrowing most prominent below level of aortic archSparing of the subglottic regionUnremarkable lung parenchyma

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Differential DiagnosisDifferential Diagnosis

Let’s consider the differential diagnosis for diffuse tracheal narrowing…

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Differential DiagnosisDifferential Diagnosis

1. Relapsing Polychondritis2. Wegener’s Granulomatosis3. Tracheobronchial Amyloidosis4. Tracheobronchopathia Osteochondroplastica5. Postinfectious stenosis6. Sarcoidosis7. Rhinoscleroma8. Saber-Sheath Trachea

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DiagnosisDiagnosis

This patient presented with a preexisting diagnosis…

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DiagnosisDiagnosis

Relapsing polychondritis

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DiscussionDiscussionA description of relapsing polychondritiswill help us understand the radiological findings in this patient…

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DiscussionDiscussionRelapsing Polychondritis (RP)– Definition

Rare multisystem disorder characterized by recurrent inflammation and destruction of cartilaginous structures

– EpidemiologyMen and women affected equallyMost commonly affects Caucasians although it has been reported in various ethnic groupsRare: incidence approximately 1/250,000Average age at presentation = 47 years

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DiscussionDiscussionRelapsing Polychondritis– Etiology

Unknown!– Pathophysiology

? Autoimmune: pathological finding of T-cells and Ag-Abcomplexes + symptomatic improvement with steroid treatmentAffected tissues

– Cartilage of nose, ears, joints, larynx, trachea, major bronchi

– Proteoglycan-rich sites such as eyes, inner ears, blood vessels, and heart

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DiscussionDiscussionRelapsing Polychondritis– Signs and symptoms

Auricular chondritis (85-91%)Polyarthritis (52-85%)Nasal chondritis (48-72%)

– Saddle nose deformityRespiratory tract chondritis (40-56%)

– Poor prognostic sign– Tracheal tenderness

Cardiovascular disease (24%)– Valvular dysfunction– Aortitis/aortic aneurysm

Audiovestibular damage (46-50%)Ocular inflammation (50-65%)

Lobule sparing

Con

com

itant

www.emedicine.com/derm/topic375.htm

Lucas Edwards, UMMS IVGillian Lieberman, MD

DiscussionDiscussionRelapsing Polychondritis– Associated conditions

Rheumatoid arthritis, systemic vasculitis, various connective tissue diseases

– DiagnosisMcAdam el al criteria (3 of 6 present)

– Bilateral auricular chondritis– Polyarthritis– Nasal chondritis– Ocular inflammation– Respiratory tract chondritis– Audiovestibular damage

Other diagnostic systems are similarly based upon inflammation of multiple cartilages and associated symptoms

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DiscussionDiscussionRelapsing Polychondritis– CXR characteristics

Tracheal stenosisCalcification of cartilaginous structuresEvidence of coexisting vasculitis (i.e. pulmonary parenchymal infiltrates)

JS Prince et al.

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DiscussionDiscussionRelapsing Polychondritis– CT characteristics

Increased airway wall attenuation (from subtle to frank calcification)Increased airway wall thicknessLuminal narrowing of trachea and bronchiCylindric bronchiectasisAir trapping Airway collapse

Expiratory CT

JS Prince et al.

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DiscussionDiscussionRelapsing Polychondritis– Other imaging modalities

MRI– Better distinction between edema, fibrosis, and

inflammation

Bone scintigraphy– Useful in localizing sites for costochondral biopsy when

diagnosis cannot be made clinically

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DiscussionDiscussionWhat other conditions could have explained these findings?Let’s consider some other conditions that are on the differential…

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DiscussionDiscussionWegener’s Granulomatosis– Necrotizing granulomatous vasculitis of upper and lower

respiratory tracts and kidneys– Clinical

M > F but females most commonly affected by tracheal involvement

– Radiologic findingsGranulomatous lung nodules/masses with central necrosis and cavitationTracheobronchial involvement (16% of cases)

– Predominantly affects subglottic region– Circumferential mucosal thickening/luminal narrowing– Ulceration

Sinus and nasal mucosal thickening

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DiscussionDiscussionWegener’s Granulomatosis

JS Prince et al.JS Prince et al.

Circumferential mucosal thickening

Tracheal narrowing from subglottic region to thoracic inlet

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DiscussionDiscussionTracheobronchial Amyloidosis– Focal or diffuse deposition of amyloid in the submucosa of the

trachea and proximal bronchi– Clinical

M > FSymptoms usually begin in middle or late adult lifeUsually “primary” without systemic involvement

– Radiologic findingsCircumferential tracheobronchial thickening/luminal narrowingSubmucosal amyloid = soft tissue densityFocal form: airway polypsDiffuse form: diffuse airway calcification/ossificationPulmonary complications

– Obstructive pneumonia, bronchiectasis

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DiscussionDiscussionTracheobronchial Amyloidosis

JS Prince et al.

Mucosal thickening involving the posterior wall

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DiscussionDiscussionTracheobronchopathia Osteochondroplastica (TBO)– Idiopathic benign disease of trachea and main bronchi characterized

by multiple submucosal osteocartilaginous nodules– Clinical

3:1 male predilectionMost patients asymptomatic

– Radiologic featuresSpares membranous posterior wallAffects lower 2/3 of trachea and proximal bronchiTBO vs. RP

– Diffuse narrowing not typical in TBO – Characteristic clinical stigmata with RP

(i.e. auricular chondritis)

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DiscussionDiscussionTracheobronchopathia Osteochondroplastica (TBO)

Calcified Nodule

Sparing of posterior wall

BIDMC PACS

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DiscussionDiscussionPostinfectious stenosis– Infectious agents

Tuberculosis, histoplasmosis, fungi– Mechanism

Infectious necrosis and ulceration of mucosa leading to granulation tissue and fibrotic stenosis

– Radiologic featuresMultiple areas of focal stenosisCalcification of stenotic regions is rareSegmental or lobar atelectasis is common

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DiscussionDiscussionTuberculosis

Focal stricture of left main bronchus just distal to the carina

JS Prince et al.

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DiscussionDiscussionSaber-Sheath Trachea– Fixed deformity of the intrathoracic trachea – Clinical

Exclusively affects older males with evidence of COPDTracheal deformity a reflection of chronic transmission of increased intrapleural pressure and injury from chronic cough

– Radiologic findingsTracheal coronal diameter < 2/3 the sagittal diameterEmphysematous changes of lung parenchyma

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DiscussionDiscussionKey points– Differentiate focal from diffuse tracheal disease

Focal stenosis may be a complication of endotracheal intubation

– TBO and RPPosterior membranous portion of the trachea usually spared

– Tracheobronchial amyloidosisFocal or diffuse involvement but usually circumferential

– Wegener’s GranulomatosisMost commonly affects subglottic region

– CT findings in combination with clinical assessment are keys to accurate diagnosis

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AcknowledgmentsAcknowledgmentsThanks to Pamela, Dr. Lieberman, Dr. Boiselle, and theresidents for their help throughout the clerkship and in organizing this presentation.

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ReferencesReferences1. JS Prince, DR Duhamel, DL Levin, JH Harrell, PJ Friedman.

Nonneoplastic Lesions of the Tracheobronchial Wall: Radiologic Findings with Bronchoscopic Correlation. Radiographics 2002; 22: S215-S230

2. JV behar, Y Choi, TA Hartman, NB Allen, HP McAdams. Relapsing Polychondritis Affecting the Lower Respiratory Tract. AJR 2002; 178: 173-177

3. NJ Screaton, P Sivasothy, CD Flower, CM Lockwood. Tracheal Involvement in Wegener’s Granulomatosis: Evaluation Using Spiral CT. Clinical Radiology 1998; 53: 809-815

4. C Ozer, MN Duce, A Yildiz, FD Apaydin, H Egilmez, T Arpaci. Primary Diffuse Tracheobronchial Amyloidosis: Case Report. European Journal of Radiology 2002; 44: 37-39

5. K Harp. Relapsing Polychondritis. www.emedicine.com/derm/topic375.htm

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The End

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