Surgical intervention for Fraser syndrome improved corneal health in 60% of eyes

Ophthalmology. 2009;116(9):1707-1712.

A systematic periocular surgical approach proved effective in managing structural abnormalities associated with Fraser syndrome, according to a study.

"Although Fraser syndrome is rare, the periocular surgical management of these complex cases may be planned using a systematic approach as described in this study, which is the largest such series to date," the study authors said.

The retrospective study included 13 eyes of seven patients treated for Fraser syndrome-related cryptophthalmos at one center between 1984 and 2007. Investigators reviewed clinical records, patient age at presentation, sex, ethnicity, parental blood typing, systemic factors, follow-up duration and surgical interventions.

Cryptophthalmos was complete in three eyes and abortive in 10 eyes. Surgical intervention for abortive cryptophthalmos was intended to optimize visual potential.

Surgical methods included dissection of corneal adhesions from keratinized cornea, mucous membrane graft, Mustarde eyelid switch flap with division, and further lower lid augmentation as needed.

Surgery was recommended for complete cryptophthalmos if cosmetic improvement was desired and sufficient tissue remained after repair of abortive cryptophthalmos in the fellow eye, the authors said.

Surgical procedures for complete cryptophthalmos included creation of fornices and upper and lower lid reconstruction using local skin or muscle tissue flaps.

Postoperative visual acuity ranged from light perception to 20/200.

"Good outcomes in terms of corneal health were achieved in six of the 10 eyes operated on for incomplete cryptophthalmos," the authors said.