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An Alternative Diagnosis for Hemoptysis
The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–3, 2015 Copyright Ó 2015 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter
http://dx.doi.org/10.1016/j.jemermed.2014.12.033
Visual Diagnosis in Emergency Medicine
AN ALTERNATIVE DIAGNOSIS FOR HEMOPTYSIS Francesca Reali, MD,* Umberto Geremia Rossi, MD,† Maurizio Cariati, MD,† and Renato Moreno Dacco`, MD* *Emergency Department, San Carlo Borromeo Hospital, Milan, Italy and †Division of Radiology and Interventional Radiology, Department of Diagnostic Sciences, San Carlo Borromeo Hospital, Milan, Italy Reprint Address: Francesca Reali, MD, Emergency Department, San Carlo Borromeo Hospital, Via Pio II, 3, 20153 Milan, Italy
tory studies were normal. Computed tomography (CT) with 3-dimensional reconstructions confirmed trachea and main bronchi wall irregularity and presence of multiple diverticula (Figures 2–4). Bronchoscopy revealed tracheal dilation, diverticula in the posterior region of the tracheal wall, and enlargement of both main bronchi. On the basis of these findings, the patient was diagnosed with Mounier-Kuhn syndrome (MKS).
CASE REPORT A 34-year-old man presented at our emergency department (ED) with a cough and chest pain. He had been diagnosed earlier with asthma, for which he was being treated with salbutamol. During observation in the ED, he experienced three episodes of hemoptysis. Chest x-ray study demonstrated an irregular outline of trachea (Figure 1). Physical examination and labora-
Figure 1. Chest x-ray study demonstrated an enlarged and irregular outline trachea (arrows).
RECEIVED: 25 June 2014; FINAL SUBMISSION RECEIVED: 8 October 2014; ACCEPTED: 21 December 2014 1
2
F. Reali et al.
Figure 2. Computed tomography axial image of the thorax confirmed an enlarged trachea and main bronchi with wall irregularity and the presence of multiple diverticula (arrowhead).
DISCUSSION MKS is characterized by tracheobronchial dilatation due to atrophy of muscular and elastic fibers and smooth muscle cells. It is more common in men and is typically diagnosed in the third or fourth decade of life. Symptoms of MKS are nonspecific. In the absence of infection, the disease can develop asymptomatically. Bronchiectasis, lower respiratory tract infections, and recurrent pneumonia are clinically prominent. Involvement occurs at different levels, from the trachea down to the fourth bronchial branch. Diagnosis is made by CT scan, revealing abnormally large air passages (1). Bronchoscopy can detect dilatation in the trachea and main bronchi (inspiration), diverticula, and constriction during expiration. The etiology of disease is not exactly known, but it is suggested to be congenital; it is sometimes associated with connective-tissue diseases, ataxia-telangiectasia, ankylosing spondylitis, Ehlers-Danlos syndrome, Marfan
Figure 4. Computed tomography coronal volume rendering technique with air surface algorithm reconstruction that confirmed trachea and main bronchi irregularity with multiple diverticula (arrowhead).
syndrome, Kenny-Caffey syndrome, Brachmann-de Lange syndrome, and cutis laxa. Cases are often sporadic (2,3). Asymptomatic patients require no specific treatment; smoking cessation and minimizing exposure to occupational irritants can be helpful. In symptomatic patients, therapy is supportive, limited to respiratory physiotherapy and to antibiotic use during infectious exacerbations. Tracheal stenting can be helpful in severe cases, but is rarely performed because of the diffuse nature of the disease (4).
Figure 3. Computed tomography coronal and sagittal multiplanar reconstruction of the thorax confirmed the enlarged trachea and main bronchi with wall irregularity (arrow) and the presence of multiple diverticula (arrowhead).
Alternative Diagnosis for Hemoptysis
REFERENCES 1. Ghaneia M, Peymanb M, Aslanic J, Zamel N. Mounier-Kuhn syndrome: a rare cause of severe bronchial dilatation with normal pulmonary function test: a case report. Respir Med 2007;101: 1836–9.
3 2. Krustins E, Kravale Z, Buls A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review. Respir Med 2013; 107:1822–8. 3. Sorenson SM, Moradzadeh M, Bakhda R. Un uomo di 68 anni con frequenti infezioni. Chest Ed Ital 2002;2:62–4. 4. Khasawneh FA, Jou-Tindo AJ. A 30-year-old woman with recurrent lower respiratory tract infections. Chest 2013;143:1500–3.
http://dx.doi.org/10.1016/j.jemermed.2014.12.033
Visual Diagnosis in Emergency Medicine
AN ALTERNATIVE DIAGNOSIS FOR HEMOPTYSIS Francesca Reali, MD,* Umberto Geremia Rossi, MD,† Maurizio Cariati, MD,† and Renato Moreno Dacco`, MD* *Emergency Department, San Carlo Borromeo Hospital, Milan, Italy and †Division of Radiology and Interventional Radiology, Department of Diagnostic Sciences, San Carlo Borromeo Hospital, Milan, Italy Reprint Address: Francesca Reali, MD, Emergency Department, San Carlo Borromeo Hospital, Via Pio II, 3, 20153 Milan, Italy
tory studies were normal. Computed tomography (CT) with 3-dimensional reconstructions confirmed trachea and main bronchi wall irregularity and presence of multiple diverticula (Figures 2–4). Bronchoscopy revealed tracheal dilation, diverticula in the posterior region of the tracheal wall, and enlargement of both main bronchi. On the basis of these findings, the patient was diagnosed with Mounier-Kuhn syndrome (MKS).
CASE REPORT A 34-year-old man presented at our emergency department (ED) with a cough and chest pain. He had been diagnosed earlier with asthma, for which he was being treated with salbutamol. During observation in the ED, he experienced three episodes of hemoptysis. Chest x-ray study demonstrated an irregular outline of trachea (Figure 1). Physical examination and labora-
Figure 1. Chest x-ray study demonstrated an enlarged and irregular outline trachea (arrows).
RECEIVED: 25 June 2014; FINAL SUBMISSION RECEIVED: 8 October 2014; ACCEPTED: 21 December 2014 1
2
F. Reali et al.
Figure 2. Computed tomography axial image of the thorax confirmed an enlarged trachea and main bronchi with wall irregularity and the presence of multiple diverticula (arrowhead).
DISCUSSION MKS is characterized by tracheobronchial dilatation due to atrophy of muscular and elastic fibers and smooth muscle cells. It is more common in men and is typically diagnosed in the third or fourth decade of life. Symptoms of MKS are nonspecific. In the absence of infection, the disease can develop asymptomatically. Bronchiectasis, lower respiratory tract infections, and recurrent pneumonia are clinically prominent. Involvement occurs at different levels, from the trachea down to the fourth bronchial branch. Diagnosis is made by CT scan, revealing abnormally large air passages (1). Bronchoscopy can detect dilatation in the trachea and main bronchi (inspiration), diverticula, and constriction during expiration. The etiology of disease is not exactly known, but it is suggested to be congenital; it is sometimes associated with connective-tissue diseases, ataxia-telangiectasia, ankylosing spondylitis, Ehlers-Danlos syndrome, Marfan
Figure 4. Computed tomography coronal volume rendering technique with air surface algorithm reconstruction that confirmed trachea and main bronchi irregularity with multiple diverticula (arrowhead).
syndrome, Kenny-Caffey syndrome, Brachmann-de Lange syndrome, and cutis laxa. Cases are often sporadic (2,3). Asymptomatic patients require no specific treatment; smoking cessation and minimizing exposure to occupational irritants can be helpful. In symptomatic patients, therapy is supportive, limited to respiratory physiotherapy and to antibiotic use during infectious exacerbations. Tracheal stenting can be helpful in severe cases, but is rarely performed because of the diffuse nature of the disease (4).
Figure 3. Computed tomography coronal and sagittal multiplanar reconstruction of the thorax confirmed the enlarged trachea and main bronchi with wall irregularity (arrow) and the presence of multiple diverticula (arrowhead).
Alternative Diagnosis for Hemoptysis
REFERENCES 1. Ghaneia M, Peymanb M, Aslanic J, Zamel N. Mounier-Kuhn syndrome: a rare cause of severe bronchial dilatation with normal pulmonary function test: a case report. Respir Med 2007;101: 1836–9.
3 2. Krustins E, Kravale Z, Buls A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review. Respir Med 2013; 107:1822–8. 3. Sorenson SM, Moradzadeh M, Bakhda R. Un uomo di 68 anni con frequenti infezioni. Chest Ed Ital 2002;2:62–4. 4. Khasawneh FA, Jou-Tindo AJ. A 30-year-old woman with recurrent lower respiratory tract infections. Chest 2013;143:1500–3.