J Thorac Cardiovasc Surg 2006;132:737-738
© 2006 The American Association for Thoracic Surgery
Lung transplantation in a patient with Mounier-Kuhn syndrome
Omar A. Minai, MD, FCCPa,
Atul C. Mehta, MBBSa,
Gosta Pettersson, MDb,
Karnak Demet, MBBSa
a Departments of Pulmonary, Allergy, and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, Ohio
b Department of Cardiothoracic Surgery, Cleveland Clinic Foundation, Cleveland, Ohio
To the Editor:
We previously reported the first case of successful lung transplantation in a patient with Mounier-Kuhn syndrome.1
The patient was a 59-year-old woman with a history of bronchiectasis of unknown etiology since childhood. The patient developed very severe obstructive lung disease and multiple episodes of pneumonia due to Pseudomonas aeruginosa that necessitated hospitalization. Computed chest tomography and bronchoscopy were diagnostic of Mounier-Kuhn syndrome. The patient underwent bilateral lung transplantation, and the degree of bronchomegaly was not as pronounced as the tracheomegaly and posed little difficulty during transplantation. The patient had a difficult postoperative course, with multiple failed extubations, P aeruginosa pneumonia, and prolonged respiratory failure necessitating tracheostomy. The patient was discharged home after making a successful recovery, followed by inpatient rehabilitation therapy. Subsequent hospitalization for surgical closure of the tracheostomy was followed by pneumonia, the need for mechanical ventilatory support, and redo tracheostomy. The prolonged hospitalization included recurrent pneumonias, malnutrition, and skin breakdown. Sepsis eventually developed, and supportive therapy was withdrawn after discussion with the patient's family.
Another recent article2 also described double-lung transplantation for chronic obstructive pulmonary disease and bronchiectasis in a patient with Mounier-Kuhn syndrome. The patient developed dynamic airway collapse and required stent placement in both the main bronchi. These cases highlight the potential dangers of lung transplantation in patients with Mounier-Kuhn syndrome. Typically, patients with bronchiectasis undergo bilateral lung transplantation because of the risk of recurrent infections. Mounier-Kuhn syndrome is a very rare cause of bronchiectasis; however, in immunocompromised patients, it may pose challenges beyond those posed strictly as a result of recurrent suppurative infections of the pulmonary parenchyma. Because of a lack of longitudinal muscle fibers and thinning of the muscular layer, their central airways are floppy and widely dilated, with out-pouchings, allowing for pooling of secretions and persistent colonization with resistant infections.1,3 This makes recurrent infections very likely despite the removal of both lungs. These mechanical abnormalities of the central airways can also compromise secretion clearance as a result of an ineffective cough.1 In addition, the large difference in diameter between the central and peripheral airways and the collapsibility of central airways on exhalation make effective mechanical ventilation a challenging proposition because of poor laminar flow.4 Our patient also exhibited a lack of spontaneous closure of the tracheostomy stoma, thus necessitating surgical closure, which was soon followed by pneumonia. Although this was not exhibited by our patient to a significant degree, the other potential problem in these patients can conceivably be a large disproportion between the bronchial diameter of the donor and the recipient, thus making surgery complicated and compromising subsequent healing of the anastomosis. Because lung transplantation has become the standard of care for managing various advanced lung diseases, it is important to recognize this unusual cause of bronchiectasis so as to better inform our patients of the risk of potential complications.
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References
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- Shah S, Karnak D, Mason D, Murthy S, Pettersson G, Budev M, et al. Pulmonary transplantation in Mounier-Kuhn syndrome. a case report. J Thorac Cardiovasc Surg 2006;131:757-758.[Free Full Text]
- Drain AJ, Perrin F, Tasker A, Stewart S, Wells F, Tsui S, et al. Double lung transplantation in a patient with tracheobronchomegaly (Mounier-Kuhn syndrome). J Heart Lung Transplant 2006;25:134-136.[Medline]
- Lazzarini-de-Oliveira LC, Costa de Barros Franco CA, Gomes de Salles CL, de Oliveira Jr AC. A 38-year-old man with tracheomegaly, tracheal diverticulosis, and bronchiectasis. Chest. 2001;120:1018-1020.[Free Full Text]
- Giannoni S, Benassai C, Allori O, Valeri E, Ferri L, Dragotto A. Tracheomalacia associated with Mounier-Kuhn syndrome in the intensive care unit: treatment with Freitag stent. A case report. Minerva Anestesiol 2004;70:651-659.[Medline]
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