Surgical correction of incomplete cryptophthalmos in Fraser syndrome

PURPOSE: To demonstrate favorable long-term visual outcome after ocular reconstruction in an infant with Fraser syndrome and with complete left cryptophthalmos.

METHODS: Reconstruction of incomplete right cryptophthalmos in our patient was accomplished in a stepwise manner, beginning in the third week of life, by dissecting the eyelids from the cornea, reconstructing the conjunctival fornices with buccal mucosa, and repairing the upper lid coloboma in a flap reconstruction using the inferior eyelid margin.

RESULTS: At age 3 years, the patient currently has good movement of the right eyelids when blinking, reasonable right tear function, and a visual acuity in the right eye between 20/200 and 20/360 on forced preferential looking.

CONCLUSION: In selected cases of incomplete cryptophthalmos, oculoplastic and corneal surgery may result in useful vision and in good eyelid movement when blinking.