Mounier-Kuhn, syndrome

[MIM 275 300]

(tracheobronchomegaly)

Very rare. Autosomal recessive transmissin. Dilatation of trachea and bronchi due to the absence or atrophy of the elastic fibers in the tracheal wall. May be associated with a form of Ehlers-Danlos syndrome or cutis laxa (se thse terms). There are sometimes diverticula on the posterior wall of the trachea. The trachea and the bronchi are fragile and can collapse entirely or partly during expiration or cough. This pathology is revealed in general around 30-40 year of age but earlier cases have been reported.

Varied symptomatology: simple breathing difficulty or repeated respiratory infections due to accumulation of mucus and ineffective cough, or even respiratory failure.


Anesthetic implications:

pre- and post-operative respiratory physiotherapy; difficulties in ventilation and risk of  inhalation of pharyngeal or gastric contents  if there are leaks around the cuff of the endotracheal tube. It is useful to measure the width of the trachea (CT scan) preoperatively to determine the best external diameter of the endotracheal tube and its optimal intratracheal position. Some suggest placing a balloon catheter in the esophagus to prevent inhalation of gastric content.


References : 

-         Messahel FM. 
Tracheal dilatation followed by stenosis in Mounier-Kuhn syndrome. 
Anaesthesia 1989; 44: 227-9.

-         Randak CO, Weinberger M. 
A child with progressive multiple tracheal diverticulae: a variation of the Mounier-Kuhn syndrome.
Pediatr Pulmonol DOI: 10.1002/ppul.22663

-         Cataldo R, Galli B, Proscia P, Carassiti M. 
Management of a patient with Mounier-Kuhn syndrome undergoing repeated general anesthetics (letter). 
Can J Anesth 2013; 60-602-3.

-         Ushakumani DS, Grewal N, Green M. 
Mounier-Kuhn syndrome: anesthetic experience. 
Case reports in Anesthesiology 2012 ID 674918 (2 pages)

-         Subramani S, Freeman B, Rajagopal S. 
Anesthetic considerations for bilateral lung transplantation in Mounier-Kuhn syndrome. 
J Cardiothor Vasc Anesth 2015; 29: 727-730 

-        Liang C, Zhang L, Yu J, Miao C.
Complicated airway management because of multiple tracheal diverticula in a patient with MounierKuhn syndrome.
Anesthesiology 2024 ; 140 : 291-2.


Updated: January 2024