Case report: Mounier-Kuhn syndrome

Abstract

Tracheobronchomegaly or Mounier-Kuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is uncertain and the clinical presentation is variable. The diagnosis is usually made on the basis of the characteristic CT scan findings. We report a case in a 21-year-old man presenting with recurrent lower respiratory tract infections.

Multiple tracheobronchial diverticula in a post‐TB patient: A case report

“…Some literatures have mentioned about the association of tracheal diverticulum with Mounier-Kuhn syndrome, 20,21 but our patient did not demonstrate dynamic airways collapse during respiration, which was further supported by measurements (transverse diameter) of airways in the CT scan. As the diverticula are usually an incidental finding, most remain asymptomatic, 9 but symptomatic patients have varied presentations such as chronic cough, dyspnea, stridor, recurrent tracheobronchitis, 10 recurrent hiccups and burping, 9 dysphonia from recurrent laryngeal nerve compression, 22 paratracheal abscess, 23 pneumomediastinum 24 etc.…”

Mounier-Kuhn syndrome: an unusual underlying cause for chronic coughs and recurrent pneumonias

“…Marfan syndrome, Ehlers-Danlos syndrome, Kenny-Caffey syndrome, ataxia-telangiectasia, connective tissue diseases, Brachmann-de Lange syndrome, Bruton-type agammaglobulinemia, ankylosing spondylitis, cutis laxa, and light chain deposition disease are also associated with secondary tracheobronchomegaly. 5 The diagnosis is missed in most patients with this syndrome; the case we report being one illustrative example. Despite long-term follow-up by physicians, Mounier-Kuhn syndrome had not been diagnosed until CT was performed.…”

Síndrome de Mounier-Kuhn: reporte de caso y revisión de la literatura

“…El SMK es una condición clínico-radiológica bien definida, también denominada traqueobroncomegalia, diverticulosis traqueal, traqueobronquiectasias, traqueocele, traqueomalacia y traqueobronquiopatía malácica 2 , siendo la característica común en todos estos términos la dilatación traqueal, ya sea aislada o asociada a dilatación del árbol bronquial 1 . Estos hallazgos fueron reconocidos por primera vez por Czyhlars en 1897 durante una autopsia 3 , pero la primera descripción la realizó Mounier-Kuhn en 1932 4 .…”