Limbal Dermoid Clinical Presentation

Updated: Sep 25, 2018
  • Author: Mark D Sherman, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

Patients present with decreased vision or poor vision, foreign body sensation, cosmetic disfigurement, or an enlarging ocular mass.

Most patients present before age 16 years.

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Physical

Most epibulbar dermoids are located at the inferior temporal limbus.

Rarely, they may only affect the cornea or the bulbar conjunctiva.

Epibulbar dermoids have a dome shape, and the surface may appear keratinized.

Hair follicles and cilia may be visible.

The dermoid appears fleshy and may have fine superficial vascularization.

Associated ocular abnormalities include colobomata of the eyelids, Duane retraction syndrome and other ocular motility disorders, lacrimal anomalies, scleral and corneal staphylomata, aniridia, and microphthalmia.

Associated systemic abnormalities include preauricular appendages and auricular fistulae (in combination with limbal dermoids constituting Goldenhar syndrome). Other abnormalities include hemifacial microsomia, microtia, and vertebral anomalies.

The acronym SCALP syndrome is used to describe individuals with the constellation of clinical findings including nevus sebaceous, central nervous system malformations, aplasia cutis congenital, limbal dermoid, and pigmented nevus. Three cases have been described in the literature. [8]

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Causes

Most cases of limbal dermoids are sporadic and not related to any known toxic exposure or mechanical irritant.

Instances are reported of epibulbar dermoids being related to maternal ingestion of teratogenic agents during the first trimester of development.

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