[MIM 155 900]

(Orofacial Granulomatosis)

Rare : estimated incidence of 8/10.000. Unknown probably autoimmune origin but cases of autosomal dominant transmission of a mutation of the MROS gene (9p11) have been observed.

The following triad is classically observed:

-        relapsing episodes of unilateral facial palsy

-        recurrent ipsilateral orofacial swelling: firm, non pruritic edema

-        a fissured tongue (lingua plicata) : reddish brown tongue (30 %)

The first episodes begin between the age of 2 and 70 years but more often in the second decade. Attacks of facial paralysis and edema are self-limited but last increasingly longer and leave sequelae: swelling, presence of granulomas.

No effective treatment: corticosteroids, dapsone, immunosuppression.

Histology of granulomas : non-caseating sarcoidal granulomas

Anesthetic implications:

risk of difficult intubation in case of extension to the pharynx or the larynx during an acute phase (stridor, inspiratory dyspnea). Because an allergic origin has been suggested, some advocate the avoidance of histamin-releasing anesthetic agents.

References : 

-        Jayamaha JEL. 
Respiratory obstruction in a patient with Melkersson-Rosenthal syndrome. 
Anesth Analg 1993; 77: 395-7.

-        Tekin M, Kati I. 
Anesthetic management of patients with Melkersson Rosenthal syndrome. 
J Anesth 2008; 22: 294-6.

Updated: January 2019