Ramon syndrome

[MIM 266 270]

(Cherubism-gingival fibromatosis-intellectual retardation syndrome)

Very rare.


Association of:

-        fibrous dysplasia of the jaws (cherubism, see this term)

-          gingival fibromatosis

-         hypertrichosis

-        growth retardation: short stature and weight < percentile 3

-         epilepsy and mental retardation

-         eye abnormalities: retina, Axenfeld anomaly (see this term)

-        sometimes: juvenile arthritis


Anesthetic implications:

difficult mask ventilation and intubation; short stature; epilepsy. In case of juvenile arthritis: check the stability of the atlas-axis joint.


References :

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Updated: November 2020