Purpose: To report the unusual association of a coloboma of the lens with ipsilateral optic nerve hypoplasia and orbital hemangioma.

Methods: Descriptive case report.

Results: An eight-month-old girl had a gradually enlarging left orbital hemangioma. There was an ipsilateral temporal coloboma of the lens with a remnant of the pupillary membrane attached to the anterior lens capsule in the central region of the coloboma. The left optic nerve head was one half the size of the right one.

Conclusion: The occurrence of intraocular malformations with an ipsilateral orbital vascular malformation may be the result of a developmental ocular and periocular field defect.

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http://dx.doi.org/10.1076/opge.19.4.209.2304DOI Listing

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