Isolated orbital fibrous dysplasia associated with ipsilateral keratoconus.
Title | Isolated orbital fibrous dysplasia associated with ipsilateral keratoconus. |
Publication Type | Journal Article |
Year of Publication | 2010 |
Authors | Liarakos, V. S., Ilari L., Chalvatzis N., Papaparaskeva K., & Mavrikakis I. |
Journal | Orbit |
Volume | 29 |
Issue | 3 |
Pagination | 154-7 |
Date Published | 2010 Jun |
ISSN | 1744-5108 |
Keywords | Biopsy, Needle, Ethmoid Sinus, Exophthalmos, Fibrous Dysplasia of Bone, Follow-Up Studies, Humans, Immunohistochemistry, Keratoconus, Lacrimal Apparatus Diseases, Male, Orbit, Rare Diseases, Risk Assessment, Tomography, X-Ray Computed, Treatment Outcome, Vision Disorders, Young Adult |
Abstract | INTRODUCTION: Fibrous dysplasia is a primary orbital bone tumor, described as a benign disorder in which proliferation of fibrous tissue and osteoid replaces and distorts the bone from which it derives. Unilateral keratoconus is a rare entity. Herein, we report a case of an extensive ethmoidal fibrous dysplasia associated with ipsilateral keratoconus, and review the literature on the subject.MATERIALS AND METHODS: A 22-year-old man presented with left painless proptosis evolving over 10 years. There was associated ipsilateral epiphora and gradual visual loss. On examination his visual acuity was 20/20 OD and CF OS. His left globe was displaced lateraly 12 mm, with 9 mm of proptosis. The extraocular movements were normal. Left nasolacrimal duct obstruction was noted. Clinical signs of keratoconus were present only in the left cornea. High resolution corneal topography confirmed unilateral keratoconus and a CT scan showed an ovoid mass with a well defined sclerotic margin arising from the left ethmoid sinus and invading the orbit.RESULTS: The patient underwent resection of the lesion via a modified Lynch incision. Complete regression of proptosis was observed immediately after surgery. Histopathological examination revealed irregular trabeculae of woven bone in different levels of maturation in a moderately cellular fibrous matrix without nuclear atypia. Trabeculae were without osteoblastic rimming or osteoclastic resorption. There has been no recurrence over a 2-year follow-up period.CONCLUSION: To our knowledge, the coexistence of unilateral isolated craniofacial fibrous dysplasia with ipsilateral keratoconus has not been reported so far. |
DOI | 10.3109/01676830903421226 |
Alternate Journal | Orbit |
PubMed ID | 20497083 |