Abstract

The nasopalpebral lipoma-coloboma syndrome was first described in 1982. It is an autosomal dominant syndrome with complete penetrance, characterized by congenital symmetrical upper lid and nasopalpebral lipomas, bilateral symmetrical upper and lower lid palpebral colobomas, telecanthus, and maxillary hypoplasia. We describe a 2-year-old boy who with this syndrome and discuss the surgical treatment. This is the fifth report in the literature, and the first one in the field of plastic surgery. It is also the first to present pitfalls in the surgical management of this syndrome.

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