Posters

Limbal dermoid as part of the Goldenhar syndrome: about two cases

Poster Details

First Author: K.Madbouhi MOROCCO

Co Author(s):    A. Redallah   B. Mrini   Y. Boubnane   F. Boudguigue   O. Charkaoui        

Abstract Details

Purpose:

The limbus dermoid is the most common form of all limbal-localized corneal conjunctival tumors. It can be associated with other anomalies to define a syndrome of the first arch : the Goldenhar syndrome, which represents the models of malformation syndromes related to the dysembryoplasies of the gill arches.The limbus dermoid is a benign evolution lesion whose only possible consequences would be astigmatism in visual development. On the other hand, a complete and attentive examination of any child carrying such a malformation is necessary, because it can detect a syndrome malformation sometimes more complex: the Goldenhar syndrom.

Setting:

Department of Ophthalmology A, the specialty hospital of Rabat

Methods:

A 19-year-old woman with a conjunctival limbic lesion of 6mm by 7mm since her birth. Examination of the appendages revealed upper and lower palpebral dermolipomas. General examination revealed abnormalities in the shape of the right ear, and two cutaneous diverticula on the right and left ears. The second case is about a 2 years old chid, addressed to our training as part of the general assessment of a polymalformative syndrome associating psychomotor retardation, hypoplasia of the left ear flag associated with auricular cutaneous appendages with retrognathism. On ophthalmological examination in the left eye, we found a limbal dermoid without astigmatism.

Results:

The first case was treated, under local anesthesia, by a simple resection of the raised portion of the limbal dermoid occured without any incident with a good clinical evolution. The pathology examination confirmed diagnosis of the limbal dermoid. Therapeutic absention was engaged for the second case.

Conclusions:

The dermoide of the limbus is a lesion with simple diagnosis and benign evolution. However, in front of any dermoids of the limb, we have to examine the aspect of the massive face, to look carefully for the peri-auricular, vertebral and costal lesions which can be associated with a goldenhar syndrom.

Financial Disclosure:

None