Ramon syndrome
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(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