Congenital orbital fibrosis: a distinct clinical entity.

PURPOSE: To describe congenital orbital fibrosis as a distinct clinical entity, and highlight its constellation of features.

MATERIAL AND

METHODS

Retrospective, noncomparative, interventional case series of 4 patients with congenital orbital fibrosis. One patient underwent exploration of the orbit, release and biopsy of scar tissue. Two patients underwent strabismus surgery. One of these also underwent upper eyelid lengthening. Blepharoptosis, eyelid retraction, enophthalmos, proptosis, presence of a diffusely infiltrating orbital mass with secondary involvement of extraocular muscles, and dysplasia of the affected bony orbit were identified.

RESULTS

One patient presented with eyelid retraction, 1 with ptosis, and the remaining 2 had normal eyelid height. Furthermore, one patient demonstrated true enophthalmos. Of the remaining 3, 1 had symmetrical exophthalmometry, and 2 presented with proptosis. The later 2 patients presented with ipsilateral facial hypoplasia, accompanying orbital wall dysplasia and decreased orbital volume, confirmed on computed tomography. In addition, all patients demonstrated an irregular, retrobulbar mass located medially within the orbit, incorporating the medial rectus muscle.

CONCLUSION

Congenital orbital fibrosis is a nonfamilial, nonprogressive, unilateral, distinct clinical entity, characterized by the presence of a diffusely infiltrating orbital mass with secondary involvement of extraocular muscles resulting in variable symptomatology due to the cicatricial process.