A Clinical Classification Scheme for Tracheobronchomegaly (Mounier-Kuhn Syndrome)

Payandeh, Jennifer; McGillivray, Barbara; McCauley, Graeme; Wilcox, Pearce; Swiston, John R.; Lehman, Anna

doi:10.1007/s00408-015-9757-z

Cited by 29 publications

(30 citation statements)

References 33 publications

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“…In recent literature, a novel classification system for Mounier-Kuhn syndrome describes extrapulmonary manifestations as ‘generalised elastolysis’ which is now referred to as Mounier-Kuhn syndrome type 3 5. Considering this histological hallmark of elastin atrophy in Mounier-Kuhn syndrome, we propose that this soft tissue involvement may have a causative role in the pathogenesis of dural ectasia in this patient.…”

Section: Discussionmentioning

confidence: 77%

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: 77%

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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“…During respiration, the tracheobronchial wall can easily collapse resulting in partial or complete obstruction. One would expect extrathoracic tracheal collapse to occur during inspiration and intrathoracic collapse with expiration, and therefore, the nature of the symptoms and signs depends on the site of the abnormality [6]. The condition results from atrophy or the absence of elastic fibers and thinning of muscle.…”

Section: Discussionmentioning

confidence: 99%

Anesthesia for a patient with unexpected giant tracheobronchomegaly

et al. 2017

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Tracheobronchomegaly (also called Mounier–Kuhn syndrome) is a rare disease characterized by flaccid and markedly dilated trachea and main bronchi on inspiration with narrowing or collapse on expiration or cough. It is associated with recurrent lower respiratory tract infection. A 75-year-old man with unexpected giant tracheomegaly had a significant peritubal air leak which impeded an operation. Lumbar epidural anesthesia was performed for a subsequent operation without any sequela. Careful evaluation with chest radiography is basic to exclude a large airway. Chest computed tomography and fiber-optic bronchoscopy provided the diagnosis of a large airway. If a large airway is suspected, these examinations help to evaluate and manage the airway.

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“…Other proposed etiologies for Mounier–Kuhn syndrome 2, 3, including inflammatory and/or elastolysis alterations 6, 17, 18, may be accompanied with progressive airway smooth muscle changes. These observations suggest that tracheobronchomalacia in Mounier–Kuhn syndrome may be, in part, due to localized degeneration/remodeling in airway smooth muscle.…”

Section: Discussionmentioning

confidence: 99%

“…The above histopathological findings in this patient with Mounier-Kuhn syndrome demonstrate that: (i) airway smooth muscle is present, and not congenitally absent; and (ii) regions of airway smooth muscle had undergone degeneration and fibrotic remodeling, as opposed to atrophic loss. Other proposed etiologies for Mounier-Kuhn syndrome [2,3], including inflammatory and/or elastolysis alterations [6,17,18], may be accompanied with progressive airway smooth muscle changes. These observations suggest that tracheobronchomalacia in Mounier-Kuhn syndrome may be, in part, due to localized degeneration/remodeling in airway smooth muscle.…”

Section: Discussionmentioning

confidence: 99%