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FIGURE 1-23A,B. Patient with iris (A) and choroidal and optic nerve (B) colobomas in typical inferior location. Note the choroidal skip lesion inferior to the disc.

FIGURE 1-24. Photograph of ectropion uvea caused by peripheral anterior stromal membrane pulling the pupillary margin forward, exposing posterior pigment epithelium. Note there is an associated corectopia and dyscoria. The appearance of the eccentric pupil could be classified as an atypical iris coloboma. This is not a true coloboma because of the location and the presence of an intact iris root.

FIGURE 1-24. Photograph of ectropion uvea caused by peripheral anterior stromal membrane pulling the pupillary margin forward, exposing posterior pigment epithelium. Note there is an associated corectopia and dyscoria. The appearance of the eccentric pupil could be classified as an atypical iris coloboma. This is not a true coloboma because of the location and the presence of an intact iris root.

FIGURE 1-25. Isolated limbal dermoid at inferotemporal limbus, right eye. Hair cilia emanates from the center of the lesion. These limbal dermoids are often associated with large astigmatisms and can cause astigmatic, anisometropic amblyopia.

FIGURE 1-26. Dermolipoma in lateral canthal area, right eye. These are benign; however, if removed, can cause restrictive strabismus and fat adherence syndrome.

Goldenhar's syndrome (oculoauriculovertebral dysplasia) is a clefting anomaly of the first brachial arch and is associated with neural crest cell abnormalities. Goldenhar's syndrome is characterized by the combination of epibulbar dermoids (der-molipomas and limbal dermoids), ocular coloboma (Fig. 1-27), incomplete cryptophthalmos or lid colobomas, preauricular skin tags, vertebral anomalies, and, sporadically, with heart and pulmonary defects.

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