Limbal dermoid
 

A dermoid in the inferotemporal aspect of limbus. Pre-auricular skin tag in a patient with  limbal dermoid.

. There is a round, raised, whitish lesion in the limbus of this patient's R/L eye. The appearance is that of a limbal dermoid.  Further examination: look at the patient's glasses, there is usually significant astigmatism due to distortion of the cornea the condition is associated with Goldenhar's syndrome; look for: preauricular skin tag, Duane's syndrome and facial asymmetry (with mandibular under-development which may be ipsilateral or contralateral, the mouth is small).

Questions:

What other mandibulofacial dystoses do you know of?

Answer
Treacher-Collins' syndrome:
this is an autosomal dominant condition with malar hypoplasia, absent zygomatic arch, anti-mongoloid slant of the palpebral fissure and coloboma of the outer third of the lower lids. In the lower face, there is hypoplasia of the lower jaw with macrostomia and abnormal dentition. The ears are usually malformed.

Return to the main page