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Mounier-Kuhn With Multiple Diverticula

      A male in his 60s, nonsmoker, with history of gastroesophageal reflux disease, hiatal hernia repair, and recurrent bronchitis presented to an otolaryngology subspecialty clinic with 1 month of mild dyspnea. Office bronchoscopy identified a paratracheal diverticulum and abnormal scar tissue in the mainstem bronchi bilaterally. The trachea demonstrated posterior scar bands with intermittent dilations (Figure 1). Computed tomography (CT) imaging revealed a 44×18–mm cystic, extrapulmonary lesion along the right side of the trachea, as well as tracheobronchomegaly (Figure 2). Given his mild symptoms, ongoing surveillance with annual CT imaging was recommended. At his 1-year follow-up, the patient remained largely asymptomatic, with imaging findings unchanged.
      Figure thumbnail gr1
      Figure 1Bronchoscopy shows scar bands (or complete tracheal rings) with intermittent dilations clear of mucus.
      Figure thumbnail gr2
      Figure 2A, Axial view of tracheal diverticulum (red arrow). B, Coronal view of tracheal diverticulum (red arrow) and tracheobronchomegaly. C, CT three-dimensional reconstruction showing tracheal diverticulum (red arrow) and tracheobronchomegaly.
      Mounier-Kuhn syndrome is a rare disorder of the respiratory tract characterized by tracheobronchial dilation secondary to atrophy of the muscle and connective tissues. Six subtypes have been described according to various clinical features, such as age of onset and imaging findings.
      • Payandeh J.
      • Mcgillivray B.
      • McCauley G.
      • Wilcox P.
      • Swiston J.R.
      • Lehman A.
      A clinical classification scheme for tracheobronchomegaly (Mounier-Kuhn syndrome).
      Adult tracheobronchomegaly with extrapulmonary elastolysis is representative of the third subtype, associated with risk of recalcitrant cough and recurrent respiratory tract infections due to poor clearance of secretions. The acquired tracheal diverticulum identified in this case is a rare sequela of this subtype, developing from impaired structural integrity of the tracheal walls in the face of increased intraluminal pressure from chronic coughing.
      • Soto-Hurtado E.J.
      • Penuela-Ruiz L.
      • Rivera-Sanchez I.
      • Torres-Jimenez J.
      Tracheal diverticulum: a review of the literature.
      As observed here, such diverticula most commonly develop in the right paratracheal region, where there is less cartilaginous support near the adjacent esophageal musculature. In addition, this case highlights examples of other diverticula found in patients with Mounier-Kuhn syndrome, namely, a hiatal hernia.

      Potential Competing Interests

      The authors report no potential competing interests.

      References

        • Payandeh J.
        • Mcgillivray B.
        • McCauley G.
        • Wilcox P.
        • Swiston J.R.
        • Lehman A.
        A clinical classification scheme for tracheobronchomegaly (Mounier-Kuhn syndrome).
        Lung. 2015; 193: 815-822
        • Soto-Hurtado E.J.
        • Penuela-Ruiz L.
        • Rivera-Sanchez I.
        • Torres-Jimenez J.
        Tracheal diverticulum: a review of the literature.
        Lung. 2006; 184: 303-307