Mounier‐Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases

Akgedik, Recep; Karamanli, Harun; Kızılırmak, Deniz; Kurt, Ali; Öztürk, Hasan; Yıldırım, Berna Botan; Cakir, Lutfullah

doi:10.1111/crj.12600

Cited by 17 publications

(26 citation statements)

References 7 publications

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“…Loss of elastin fibres and thin smooth muscle in the trachea and bronchi results in tracheobronchomegaly 2. This can lead to recurrent respiratory tract infections as our patient had experienced.…”

Section: Discussionmentioning

confidence: 71%

Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis

Sheridan¹,

Godkin

Devitt

2017

BMJ Case Reports

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We present the case of a patient undergoing lumbar spine decompression for stenosis with a history of Mounier-Kuhn syndrome. The patient presented with axial lumbar spine pain over 6 months with progressive radicular pain to the left L3 dermatome. MRI confirmed spinal stenosis at L3/4 level with associated dural ectasia. The patient had an uneventful spinal decompression with resolution of radicular symptoms and axial spine pain. Dural ectasia poses a significant risk when operating on the lumbosacral spine. Larger cerebrospinal fluid volumes and a capacious dural canal can result in anaesthetic and orthopaedic complications such as inadequate spinal anaesthesia, complicated epidural analgesia, intraoperative dural tears and difficult pedicle screw insertion due to narrow pedicles. This is the first case in the literature detailing the association between dural ectasia and Mounier-Kuhn syndrome. We recommend adequate spinal imaging in patients with Mounier-Kuhn syndrome to exclude dural ectasia prior to undergoing lumbosacral spinal procedures.

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Section: Discussionmentioning

confidence: 71%

Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis

Sheridan¹,

Godkin

Devitt

2017

BMJ Case Reports

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“…For example, Down syndrome may be associated with immunodeficiency, incoordinate swallow, GOR and tracheomalacia . Other syndromes which may be complicated by bronchiectasis include Marfan's, Yellow Nail (typical dystrophy, may be a positive family history and lymphoedema), velocardiofacial, ataxia telangiectasia (also multifactorial, immunodeficiency and incoordinate swallow both contributing), Mounier‐Kuhn (tracheobronchomegaly) and Williams Campbell syndrome (congenital bronchomalacia) . Ataxia telangiectasia presents particular diagnostic problems because of the radiosensitivity of these patients, limiting the amount of diagnostic X‐rays which can be performed.…”

Section: Causes Of Bronchiectasismentioning

confidence: 99%

Pathophysiology, causes and genetics of paediatric and adult bronchiectasis

Bush

Floto

2019

Respirology

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Bronchiectasis has historically been considered to be irreversible dilatation of the airways, but with modern imaging techniques it has been proposed that 'irreversible' be dropped from the definition. The upper limit of normal for the ratio of airway to arterial development increases with age, and a developmental perspective is essential. Bronchiectasis (and persistent bacterial bronchitis, PBB) is a descriptive term and not a diagnosis, and should be the start not the end of the patient's diagnostic journey. PBB, characterized by airway infection and neutrophilic inflammation but without significant airway dilatation may be a precursor of bronchiectasis, and there are many commonalities in the microbiology and the pathology, which are reviewed in this article. A high index of suspicion is essential, and a history of chronic wet or productive cough for more than 4-8 weeks should prompt investigation. There are numerous underlying causes of bronchiectasis, although in many cases no cause is found. Causes include post-infectious, especially after tuberculosis, adenoviral or pertussis infection; aspiration syndromes; defects in host defence, which may solely affect the airways (cystic fibrosis, not considered in this review, and primary ciliary dyskinesia); and primary ciliary dyskinesia or be systemic, such as common variable immunodeficiency; genetic syndromes; and anatomical defects such as intraluminal airway obstruction (e.g. foreign body), intramural obstruction (e.g. complete cartilage rings) and external airway compression (e.g. by tuberculous lymph nodes). Identification of the underlying cause is important, because some of these conditions have specific treatments and others genetic implications for the family.

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“…Its clinical presentation can vary from an asymptomatic to chronic dry or moist cough, hemoptysis, progressive dyspnea, repeated respiratory infections, pneumonia with therapeutic failure, ventilatory failure, and even death [4,7]. Physical examination can evidence digital clubbing, rales and/or rhonchi on pulmonary auscultation, tachycardia, tachypnea, fever, and ventilatory failure [4].…”

Section: Introductionmentioning

confidence: 99%

“…It is important to improve bronchial hygiene with postural drainage to counteract the decrease in mucociliary movement, and antibiotics should be used in case of infection. Vaccination against pneumococcus and influenza has been suggested, resulting in the prevention of exacerbations [7]. Bronchial plasty with silicone stent has been described and is associated with improvement compared to medical management alone; however, the availability of an appropriate stent can be a limiting factor for some institutions [4,7].…”

Section: Introductionmentioning

confidence: 99%

“…Vaccination against pneumococcus and influenza has been suggested, resulting in the prevention of exacerbations [7]. Bronchial plasty with silicone stent has been described and is associated with improvement compared to medical management alone; however, the availability of an appropriate stent can be a limiting factor for some institutions [4,7]. In more severe cases, lung transplantation has been considered, but unfavorable results have been reported [7,10,11].…”

Section: Introductionmentioning

confidence: 99%

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Recurrent Pneumonias in a Previously Healthy and Immunocompetent Young Adult: A Case Report Mounier-Kuhn Syndrome

et al. 2020

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syndrome is a rare clinical condition characterized by marked tracheal and bronchial dilatation. It is commonly associated with multiple respiratory infections due to the inability to mobilize secretions, chronic cough, and decline in lung capacity. Although it is a rare disease, the number of cases reported in the literature has increased, and physicians should be aware of its existence to be able to recognize and diagnose it. Suspicion should arise regarding young adults who develop recurrent airway infections when immunosuppression has been ruled out. Case Report: We present the case of a young adult, previously healthy and immunocompetent, who was diagnosed with Mounier-Kuhn syndrome after 5 years of multiple respiratory infections and multiple respiratory failures, who received intensive care management with multiple complications and difficulties in achieving adequate ventilation. Due to the risk of respiratory failure and major complications, its management was challenging. We were able to treat this patient and prevent complications with a multidisciplinary approach with a strong emphasis on constant and intense pulmonary therapy. Conclusions: Mounier-Kuhn syndrome is rare but does occur, and physicians need to have a high index of suspicion to diagnose it. Its management must be done by a multidisciplinary team, and respiratory therapy should be constantly available at bedside.

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Mounier‐Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases

Abstract: In this article, 11 cases with various rarely seen complications are presented and evaluated in the light of current literature. We recommend that if chronic cough, recurrent pulmonary infections, and bronchiectasis seen in a patient, MKS should be kept in mind.

Cited by 17 publications

References 7 publications

Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis

Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis

Pathophysiology, causes and genetics of paediatric and adult bronchiectasis

Recurrent Pneumonias in a Previously Healthy and Immunocompetent Young Adult: A Case Report Mounier-Kuhn Syndrome

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