European Journal of Cardio-thoracic Surgery 39 (2011) e186—e188 www.elsevier.com/locate/ejcts
Case report
Endoscopic successful management of tracheobronchomalacia with laser: apropos of a Mounier-Kuhn syndrome Herve ´ Dutau a,*, Fabien Maldonado a, David Patrick Breen a, Arlette Colchen b a
Thoracic Oncology, Pleural Disease and Interventional Pulmonology Department, North Hospital, Marseille, France b Thoracic Surgery Department, Ho ˆpital Foch, Suresnes, France Received 28 December 2010; accepted 26 January 2011; Available online 5 March 2011
Abstract Mounier-Kuhn syndrome is a rare condition that combines tracheobronchomegaly (TBM) and severe tracheobronchomalacia. Symptoms can be severe with recurrent bronchopulmonary infections and cough-induced syncope. Therapeutic management is non-specific and limited to chest physiotherapy and antibiotics during infectious exacerbations. We report a case of Mounier-Kuhn syndrome that was successfully managed by treating the posterior collapse of the central airway with yttrium aluminum pevroskyte laser. Endoscopic aspects, respiratory symptoms, and lung function tests all improved and remained stable with a follow-up of 8 years. Laser, at low power settings, could be a new therapeutic option in selected cases of tracheobronchomalacia. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved. Keywords: Tracheomegaly; Tracheobronchomegaly; Tracheobronchomalacia; Excessive dynamic airway collapse; Mounier-Kuhn syndrome; Laser; Bronchoscopy
1. Introduction Tracheobronchomegaly (TBM), or Mounier-Kuhn syndrome, is a rare entity characterized by severe tracheobronchomalacia responsible for disabling respiratory symptoms, recurrent respiratory infections, and intractable cough with occasional cough-induced syncope. Current treatment is limited to the management of these complications via bronchopulmonary hygiene measures and antibiotics when deemed necessary. We report the case of a patient whose tracheobronchomalacia was successfully treated endoscopically with yttrium aluminum pevroskyte (YAP) laser with excellent objective and subjective results.
2. Clinical summary A 68-year-old female patient was referred, 8 years ago, to our tertiary-care center for chronic cough associated with occasional episodes of acute dyspnea. Her past medical history was essentially significant for acute rheumatic fever and gastroesophageal reflux disease. Her clinical course was characterized by recurrent episodes of febrile illness with * Corresponding author. Thoracic Oncology, Pleural Disease and Interventional Pulmonology Department, North Hospital, Chemin des Bourrely, 13015 Marseille, France. Tel.: +33 491 96 59 71; fax: +33 491 96 59 90. E-mail addresses:
[email protected],
[email protected] (H. Dutau).
respiratory infections characterized by abundant purulent sputum production and audible wheezing. Treatment with antibiotics, oral corticosteroids, and bronchodilators was temporarily effective, but a severe episode eventually justified her hospitalization for diagnostic and therapeutic management. Lung auscultation revealed bilateral rales in the bases, predominantly on the left side, with diffuse wheezing. Arterial blood gases in room air were as follows: pH: 7.42, PaO2: 67 mmHg, PaCO2: 34 mmHg, and SaO2: 94%. Lung function data were as follows: forced expiratory volume in 1 s (FEV1) = 1.43 l (76%), forced vital capacity (FVC) = 1.94 l (85%), and FEV1/FVC = 73%. Computed tomography revealed bilateral opacities with evidence of TBM (main stem bronchi diameter was measured at 20 mm and the tracheal one was 26 mm). She was successfully treated with antibiotics, intensive respiratory therapy, corticosteroids, and bronchodilator aerosols. Fiberoptic bronchoscopy was recommended and confirmed the TBM with near-complete collapse of the posterior membrane of the trachea and the left main stem bronchus during expiration (Fig. 1A and B). Rigid bronchoscopy with YAP laser treatment was then suggested as a potential therapeutic option to the patient who agreed to proceed. The posterior membrane was therefore treated with YAP laser in an attempt to devascularize and retract the tissues, thus preventing the excessive dynamic airway collapse responsible for the symptomatology described by the patient. Pressure applied via the bevel of the rigid bronchoscope on the posterior membrane allowed for a
1010-7940/$ — see front matter # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.ejcts.2011.01.074
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H. Dutau et al. / European Journal of Cardio-thoracic Surgery 39 (2011) e186—e188
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3. Discussion
Fig. 1. A: Endoscopic aspects of the trachea: during inspiration; B: during expiration; C: laser application: the bevel of the rigid bronchoscope applies a pressure on the posterior wall to allow the laser beam (black arrow) to penetrate more perpendicularly; and D: posterior membrane after laser devascularization: blanching effect.
more perpendicular angle of laser treatment, allowing for a better penetration in the submucosal tissues (Fig. 1C and D). There was significant clinical improvement reported by the patient in the days following the procedure. This was supported by an objective improvement in the pulmonary function studies obtained post-intervention (FEV1 = 2.3 l (134%), FVC = 2.8 l (133%), and FEV1/FVC = 82%). A bronchoscopic examination 6 weeks after the procedure revealed a marked improvement in the tracheobronchomalacia (Fig. 2). A second treatment was performed 14 months later due to slight recurrence of the posterior membrane collapse, which had remained completely asymptomatic clinically. The patient has been asymptomatic from a respiratory standpoint for the past 8 years, having required a total of four treatments with YAP laser. To date, her lung function data are as follows: FEV1 = 1.950 l (112%), FVC = 2.480 l (116%), and FEV1/FVC = 79%.
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Fig. 2. Endoscopic aspects 6 weeks after laser treatment.
TBM was first described in 1932 by Mounier-Kuhn [1]. It is characterized by recurrent episodes of lower airway infections, chronic bronchitis, and bronchiectasis. Mounier-Kuhn syndrome is defined by a marked dilatation of the trachea and the main bronchi, often responsible for a tracheobronchomalacia that may occasionally be severe [2—5]. Computed tomography typically suggests the diagnosis [6] that can be confirmed by fiberoptic bronchoscopy which typically reveals an expiratory collapse of the intrathoracic airway. Treatment of TBM essentially relies on bronchopulmonary hygiene and antibiotics during infectious exacerbations, occasionally supplemented by flexible bronchoscopy for removal of retained purulent secretions [2—5]. Non-surgical therapeutic options, that is, bronchoscopic, are limited. Commercially available endobronchial stents, whether silicone or selfexpandable metallic stents, do not generally have diameters large enough to prevent migration [7]. However, we have previously reported the successful treatment of a case of tracheomegaly with a 28-mm outer diameter self-expandable metallic stent in a patient with Marfan disease, who experimented an extrinsic compression of the trachea from a thoracic aortic aneurisma [7]. Thus, such stents are not widely available and the appropriate management of these patients remains challenging. The YAP laser is used in the treatment of a variety of disease processes involving the central upper airway. Its wavelength is double that of the yttrium aluminum garnet (YAG) laser, allowing for excellent tissue devascularization and coagulation at low power (15—20 W) in a discontinuous mode [8]. The depth of penetration of the tissues with YAP laser is estimated at 3 mm and may reach the submucosal tissues triggering a retractile fibrotic process that rigidifies the posterior membrane. This effect has been studied in humans in order to reduce palatal flutter as an attempt to reduce snoring [9]. Besides, we have been collecting data on tracheobronchomalacia treated with YAP laser in our division with encouraging preliminary results [10]. We think that this technique could significantly impact the management of patients with tracheobronchomalacia (excessive collapse of the posterior membrane subtype) and warrant further prospective studies.
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[7] Dutau H, Cavailles A, Fernandez-Navamuel I, Breen DP. Tracheal compression in a patient with Marfan’s syndrome-associated tracheomegaly treated by a XXL Stent: the largest diameter airway stent ever placed in a previously undescribed airway condition. Respiration 2009;77:97—101. [8] Dumon MC, Cavaliere S, Vergnon JM. Bronchial laser: techniques, indications, and results. Rev Mal Respir 1999;16:601—8.
[9] Ellis PD. Laser palatoplasty for snoring due to palatal flutter: a further report. Clin Otolaryngol Allied Sci 1994;19:350—1. [10] Dutau H, Michaud G, Milhe F, Dumon JF. Laser therapy for tracheobronchomalacia. In: Proceedings of the 13th world congress for bronchology and 13th world congress for bronchoesophagology. Barcelona: Monduzzi; 2004. p. 21—6.
