CASE PRESENTATION What lies behind this late-onset wheeze? · loss and general malaise are uncommon...

Case historyA 67-year-old male retired police officer pre-sented to the casualty department in May 2005with worsening dyspnoea on exertion andcough productive of yellowish sputum. He wasbeing treated for hypertension and had beendiagnosed with asthma by his general practi-tioner in March 2005. He had a 15 pack-yearsmoking history, until the age of 35 years. Onexamination, pulse rate was 110 beats per min,temperature 37.5°C and blood pressure130/80 mmHg. A diffuse wheeze across bothlung fields was noted on chest examination.Chest radiography was unremarkable (figure 1).

The patient was diagnosed with an infective

exacerbation of asthma and received: co-amoxi-clav 1.2 g i.v. 8-hourly; hydrocortisone 100 mg i.v.8-hourly; and salbutamol and ipratropium bynebuliser 6-hourly with good effect. He was dis-charged in good condition and given a follow-upappointment at the asthma clinic.

Investigations Lung function tests revealed a forced expiratoryvolume in one second (FEV1) of 33% predictedand a forced vital capacity (FVC) of 72% pred.The FEV1/FVC ratio was 46%. There was 15%reversibility in FEV1 post-bronchodilator therapy.The patient was unable to produce a flow–-volume loop suitable for interpretation.

What lies behind this late-onsetwheeze?

Task 1Interpret the spirometry data.

87Breathe | September 2007 | Volume 4 | No 1

CASE PRESENTATION

Correspondence:Dept of MedicineSt Luke's HospitalGuardamangia HillGuardamangiaMSD 09MaltaE-mail: [email protected]

G. SchembriA. VellaM.V. Balzan

Dept of Medicine, St Luke'sHospital, Guardamangia,Malta

Figure 1.Chest radiograph taken during admission.

case presentationslate-onset asthma.qxd 01/08/2007 12:02

During follow-up visits, repeat pulmonaryfunction tests showed a steady decline in FEV1and FVC, despite increasing asthma treatment.By November 2005, the patient was on flutica-sone inhaler 250 μg 12-hourly, salmeterolinhaler 50 μg 12-hourly and salbutamol inhaleron a pro re nata basis. He was given a tailing-down dose of prednisolone 60 mg in an attemptto control his asthma.

Despite being prescribed oral steroids, thepatient required hospital admission inNovember and December owing to worseningdyspnoea, cough and difficulty in expectoratingthick sputum. During the December admission,inspiratory stridor was suspected on clinicalexamination and an urgent computed tomo-graphy (CT) scan of the neck and chest wasrequested (figures 2 and 3).

Answer 1The FEV1/FVC ratio of 46% is suggestive ofobstructive lung disease. The 15% reversibilitypost-bronchodilator treatment is consistentwith a diagnosis of asthma.

88 Breathe | September 2007 | Volume 4 | No 1

CASE PRESENTATION What lies behind this late-onset wheeze?

Figure 2Spiral thoracic CT scan.

Figure 3Virtual reconstruction of the trachea using the CTimages.

Task 2Interpret the CT scan and virtualreconstruction.

Task 3How would you manage this patient?


A polypoid mass occluding 90% of the tra-chea was noted. Since the patient had respira-tory compromise, he was booked for urgentdebulking of the tumour through a rigid bron-choscope. Rigid bronchoscopy has the advan-tages of superior suctioning capacity of bloodand clots, excellent visualisation and better air-way control when compared with flexible bron-choscopy [1]. After the procedure, there was animmediate improvement in the patient's respira-tory symptoms. All inhalers were stopped andprednisolone was rapidly tailed off.

Following debulking, FVC normalised butFEV1 did not; an important obstructive compo-nent remained, caused by the residual tumour(figure 4). The histological results of the tumour

were reported as mucoepidermoid carcinoma ofthe trachea (figure 5). The patient was subse-quently referred to a specialist centre in the UKfor complete resection of the tumour and tra-cheal reconstruction, to provide a definitive cure.


CASE PRESENTATIONWhat lies behind this late-onset wheeze?

Answer 2A polypoid intrathoracic tracheal mass isobstructing 90% of the trachea.

Answer 3Urgent debulking of the tumour through arigid bronchoscope.

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Figure 4The steady decline in FEV1 (bottomline) and FVC (top line) noted dur-ing the outpatient visits, followedby a sudden improvement afterdebulking.

Figure 5Histology of the trachea showingmalignant proliferation of tubularand epithelial cells compatiblewith mucoepidermoid carcinoma.


DiscussionTracheal tumours are extremely rare, with aprevalence of 0.1–0.2 per 100,000 population[2–4]. Peak incidence occurs in the fifth andsixth decades of life. These tumours are 140times less common than lung tumours. They areslightly more predominant in the lower part ofthe trachea (57%). As in bronchial carcinoma,there is a strong association with smoking. Thetwo main histological types of primary trachealmalignancy are squamous and adenoid cysticcarcinoma. Undifferentiated and small cell carci-nomas are uncommon. Bronchial carcinoid,mucoepidermoid and primary lymphomas arerare. Mucoepidermoid and adenoid cystic carci-nomas are locally invasive but tend to meta-stasise late and are associated with a betterprognosis [3].

Extrathoracic tracheal tumours generallypresent with stridor and hoarseness and aprompt diagnosis is often made. Intrathoracictracheal tumours typically present the most diag-nostic difficulty, with the condition masquerad-ing as adult-onset asthma. WU et al. [2], in aretrospective study of 40 patients with trachealtumours, reported a median delay in diagnosisof 10 months from the initial presentation. In aseries of 67 patients from Taiwan, YANG et al. [3]also reported a delay in diagnosis, in particularfor slow-growing mucoepidermoid and adenoidcystic carcinomas. This delay is usually attributedto the nonspecific presentation of this condition,usually with dyspnoea, cough, hoarseness andwheeze. Recurrent attacks of respiratory obstruc-tion due to secretions and recurrent infectionsare common and are often mistaken for infectiveexacerbations of asthma. Haemoptysis isreported in less than one-third of cases. Weightloss and general malaise are uncommon in theearly stages. There are usually no significantabnormalities on chest radiography. Spirometryis of limited value in distinguishing betweenasthma and a tracheal tumour, as both condi-tions produce an obstructive picture. How, then,can the two conditions be distinguished?

Failure to respond to standard anti-asthmatictreatment and recurrent admissions while onoral steroids should be regarded with suspicionand an alternative diagnosis sought [5]. In the

present patient, there was no objective responseto inhaler therapy despite assurances aboutcompliance and good inhaler technique.

The wheeze heard in large airway obstruc-tion is classically described as monophonic,whereas that heard in asthma is described aspolyphonic. The apparent location of the wheezeis also important. Wheezing secondary to largeairway obstruction is best heard unilaterally, orover the trachea or larynx, whilst that of asthmais heard all over the chest wall. However, eventhe most localised lesions can produce diffusesounds. In the case presented, a monophonicexpiratory wheeze was heard diffusely acrossboth lung fields.

During an infective exacerbation, the patientsometimes spoke about a choking sensationfrom thick sputum, which was difficult to expec-torate. In retrospect, this can be explained bypooling of secretions at the level of the tumour.In practice, however, it is usually attributed tothe bronchitis or pneumonia.

The flow–volume loop can be used to distin-guish extrathoracic or intrathoracic large airwayobstruction from small airway obstruction.Extrathoracic tracheal tumours predominantlyaffect the inspiratory part of the loop, makingstridor a prominent feature. In cases of intra-thoracic tracheal tumour, as presented here,there is markedly diminished expiratory flow butinspiration is largely preserved. During forcedexpiration, the intrathoracic pressure is higherthan the intratracheal pressure, worsening theobstruction and decreasing expiratory flow.During inspiration, airway diameter is improvedand the degree of obstruction reduced.Inspiratory stridor is therefore not a prominentfeature of intrathoracic airway obstruction untilvery late in the disease. Fixed tracheal stenosiswill produce flattening of both the inspiratoryand expiratory flow–volume curves. This is oftensecondary to benign tracheal stenosis, althoughfixed invasive tracheal tumours can sometimesproduce a similar picture.

Care must be exercised when attributingabnormal flow–volume loops to poor technique,and in interpreting flow–volume loops in thepresence of concurrent lung disease. Poor tech-nique does not produce reproducible loops.




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CASE PRESENTATIONWhat lies behind this late-onset wheeze?

Task 4Interpret the flow–volume loops

Figure 6Examples of flow–volume loops.




References1. Johnson JL. Manifestations of hemoptysis: how to manage minor, moderate, and massive bleeding. Postgrad Med 2002; 112:

101–113.2. Wu K-L, Jiang G-L, Fu X-L, Zhou Y-Z, Liu T-F. Primary carcinomas of the trachea: natural history, treatment and results. Asia

Pacific J Clin Oncol 2005; 1: 41–46.3. Yang K-Y, Chen Y-M, Huang M-H, Perng R-P. Revisit of primary malignant neoplasms of the trachea: clinical characteristics and

survival analysis. Jpn J Clin Oncol 1997; 27: 305–309.4. Stewart MG. Primary malignant tracheal tumors. Baylor College of Medicine grand round archive 1992.

www.bcm.edu/oto/grand/1492.html. Date last accessed: September 1, 2006.5. Scottish Intercollegiate Guidelines Network. British Guideline on the Management of Asthma 2005.

www.sign.ac.uk/guidelines/fulltext/63/index.html Date last accessed: September 1, 2006.6. Gelder CM, Hetzel MR. Primary tracheal tumours: a national survey. Thorax 1993; 48: 688–692. 7. Grillo HC, Mathisen DJ. Primary tracheal tumors: treatment and results. Ann Thorac Surg 1990; 49: 69–77.

Further readingMathisen DJ. Surgical management of tracheobronchial disease. Clin Chest Med 1992; 13: 151–171.Soto FJ, Guntupalli KK. All that wheezes is not asthma: diagnosing the mimics. Emerg Med 2001; 33: 40–56.

Surgical resection is potentially curative.Surgical mortality for resection with primaryreconstruction is quoted at 5% in specialist cen-tres, with the highest mortality associated withinfiltration of the carina [3]. Laser therapy andradiotherapy are both performed as palliative

measures and offer no hope for cure. Extensivelocal infiltration and the presence of distantmetastases or multiple primary tumours are bothcontraindications to surgery. Post-surgery, 5-yearsurvival rates have been reported as 25–50%with squamous cell carcinoma, and up to 80%for adenoid cystic carcinoma [6, 7].

In conclusion, tracheal tumours carry a rela-tively good prognosis if diagnosed early. For thisreason, all patients with asthma resistant totreatment or evident monophonic wheezing onphysical examination should be investigatedfully. In such cases, the possibility of intrathoracictracheal obstruction should be kept in mind inorder to offer the patient the best hope for cure.

Answer 4a) Normal.b) Small airway obstruction.c) Extrathoracic tracheal tumour (variableextrathoracic obstruction).d) Intrathoracic tracheal tumour (variableintrathoracic obstruction).

AcknowledgementsThe authors would like to thank A. Betts, D. Mifsud and S. Mifsud.


CASE PRESENTATION What lies behind this late-onset wheeze? · loss and general malaise are uncommon...

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Transcript of CASE PRESENTATION What lies behind this late-onset wheeze? · loss and general malaise are uncommon...