J Korean Ophthalmol Soc.  2007 Sep;48(9):1276-1280.

A Case of Neurofibromatosis of the Orbit and Ocular Surface

• Lee KH
• Park JW
• Yoon KC

Abstract

PURPOSE: To report a case of neurofibromatosis of the orbit and ocular surface.
METHODS
A 26-year-old man was admitted complaining of conjunctival mass and exophthalmos. Lisch nodules on the iris were observed in the right eye, and corneal opacity with neovascularization and conjunctival mass were detected in the left eye. Orbital computed tomography revealed diffuse infiltrating facial tumors including the left orbit, suggestive of plexiform neurofibromatosis.
RESULTS
We performed evisceration of the left eye and excision of the left orbital mass. Histopathologic examination revealed neurofibroma which was positive for S-100. No recurrence was detected during a 6-month post-operative period.

Keyword

Lisch Nodule; Orbital Neurofibromatosis

MeSH Terms

Adult
Corneal Opacity
Exophthalmos
Humans
Iris
Neurofibroma
Neurofibromatoses*
Orbit*
Recurrence

Figure

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  Figure 1. The photograph shows a conjunctival mass with proptosis in a 26-year-old male patient.

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  Figure 2. Anterior segment photograph shows a Lisch nodule in the right eye (black arrow).

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  Figure 3. Preoperative CT images demonstrated diffuse infiltrating orbital tumor accompanying with bony defect in the orbit and mild brain hernia (Coronal view with enhancement).

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  Figure 4. Histopathologic findings of conjunctival mass. Tumor cells were wavy, carrot-like spindle-shaped (arrow) and were intimately associated with wire-like strands of collagenous fibers (*100). (A) Hematoxylin and eosin. (B) S-100.

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  Figure 5. Six months after operation, previous lesion markedly improved.

Reference

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