[Limbal dermoid and Goldenhar syndrome. Report of an anatomoclinical study].
Dysembryoplastic neoplasm, the limbal dermoid is a benign congenital tumor involving the outer coat of the eye. This congenital tumor affected the right eye of a 6-year-old girl. The lesion was typically hemispheric, covered with pink skin, located in the inferior and temporal part of the cornea, and devoid of any major consequences except the cosmetic appearance of the eye. There were no other major abnormalities in the clinical examination but a single skin tag could be observed at the homolateral preauricular area without any visible cutaneous fistula next to it. This additional change allowed us to establish the diagnosis of a minor form of Goldenhar syndrome. This malformative syndrome typically includes several changes: dermoid and/or dermolipoma, preauricular tags and/or cutaneous fistulas in the same area, vertebral abnormalities etc. These findings may be limited to dermoid and cutaneous tags, as in our report. Such changes must be looked for in all limbal dermoids.
D'Hermies
F
F
Service d'Ophtalmologie, Hôtel Dieu, 1, place du parvis Notre-Dame, 75181 Paris.
Saragoussi
J J
JJ
Meyer
A
A
Morel
X
X
Fayet
B
B
Dighiero
P
P
Than-Trong
T
T
Halhal
M
M
Vu
T A
TA
Elmaleh
C
C
Renard
G
G
D002648:Child; D003884:Dermoid Cyst / Q000473:pathology*; D005260:Female; D006053:Goldenhar Syndrome / Q000473:pathology*; D006801:Humans