PURPOSE: Cryptophthalmos was first described in 1872 as a rare syndrome consisting of congenital upper eyelid and eyebrow coloboma and a rudimentary ocular bulb. Later, Fraser described it as being part of a complex syndrome with multiple anomalies. Many cases have been described since then, but very few surgical techniques have been proposed for its correction. In this paper, the authors present a surgical procedure for correction of the upper eyelid and ocular bulb anomalies in cryptophthalmos. METHODS: Five patients with Fraser syndrome were seen from 1993 to 2008. They were in the age group of 18 months to 24 years. Of these, 4 patients underwent reconstruction of the upper eyelid. RESULTS: The large upper eyelid coloboma and the cutaneous pterygium were treated with a 2-stage procedure, with good results obtained with the adoption of Mustardé's technique. CONCLUSIONS: The complexity of the surgical treatment increases when there is no possibility of rudimentary eye bulb preservation. In upper eyelid reconstruction, the authors adopted Mustardé's technique with fixation of the lower eyelid transposed flap to the levator muscle, leading this way to relatively good upper eyelid motility. The authors consider imperative that the upper fornix be reconstructed with adequate depth to allow perfect positioning of an aesthetic prosthesis, in this way improving the final aesthetic and functional results.
PURPOSE: Cryptophthalmos was first described in 1872 as a rare syndrome consisting of congenital upper eyelid and eyebrow coloboma and a rudimentary ocular bulb. Later, Fraser described it as being part of a complex syndrome with multiple anomalies. Many cases have been described since then, but very few surgical techniques have been proposed for its correction. In this paper, the authors present a surgical procedure for correction of the upper eyelid and ocular bulb anomalies in cryptophthalmos. METHODS: Five patients with Fraser syndrome were seen from 1993 to 2008. They were in the age group of 18 months to 24 years. Of these, 4 patients underwent reconstruction of the upper eyelid. RESULTS: The large upper eyelid coloboma and the cutaneous pterygium were treated with a 2-stage procedure, with good results obtained with the adoption of Mustardé's technique. CONCLUSIONS: The complexity of the surgical treatment increases when there is no possibility of rudimentary eye bulb preservation. In upper eyelid reconstruction, the authors adopted Mustardé's technique with fixation of the lower eyelid transposed flap to the levator muscle, leading this way to relatively good upper eyelid motility. The authors consider imperative that the upper fornix be reconstructed with adequate depth to allow perfect positioning of an aesthetic prosthesis, in this way improving the final aesthetic and functional results.