Discussion in 'Spot Diagnosis' started by bb100, Apr 26, 2013.
This syndrome is rare disorder characterised by premature craniosynostoses.
this is Crouzon syndrome
pathology is a combo of 1st and 2 nd branchial arch development and premature closure of mainly the coronal suture.
since the coronal sutures close prematurely the forehead is not will developed and the sockets seem to bulge ( becos they have no roof )
mid face hypoplasia, gives the impression of a protruding chin and the face looks concave ( more appreciable in a profile view )
correct answer :Crouzon syndrome
Clinical spectrum :
abnormal calvarial shape; in severe case can give a "cloverleaf skull"
shallow orbits with exopthalmos :
mid facial hypoplasia :
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