Jeff Pietrzyk


BACKGROUND: Background: Neurofibromatosis II is an inherited disorder, of which the defining features include acoustic neuromas (usually bilateral), schwannomas, and meningiomas. Ophthalmic findings may include cataracts, iris Lisch-nodules, optic-nerve sheath menengiomas, and loss of the greater wing of the sphenoid bone.

CASE REPORT(S). Case Report: A 28-year-old white male presents with diplopia and proptosis OS 6 weeks after a left craniotomy and resection of the left lateral wall to remove a meningioma. Patient history is also positive for unilateral decrease in hearing in the left ear (due to a schwannoma). There is family history of neurofibromatosis, appearing in the patient’s mother and sister. Entering best corrected acuities were 20/20 OD, 20/30 OS. IOP measured 15 OD and 22 OS. Confrontation fields were full OU, while motilities showed a decrease in abduction (-4) and slightly limited superior movement (-1). Cover test revealed a 35 pD left ET and 8 right hyper in primary gaze, stemming from a 6th nerve palsy, that increased in left gaze and decreased in right gaze. CN VII testing showed the patient was unable to wrinkle his brow, while CN V testing showed deficiencies in all 3 divisions. A pulsating proptosis was visible OS, with exopthalmometry readings of 17 mm and 25 mm with a base of 105 mm. Pupils were 4 and 5 mm in light, 5.5 and 7.5 mm round in dim, with a 2+ and 3+ reaction to light, respectively, and no APD noted. Slit lamp noted 1+ injection OS, with a PSC noted on the left lens. DFE was unremarkable.

CONCLUSIONS. Conclusion: This case shows a unique presentation of pulsating proptosis secondary to the loss of the greater wing of the sphenoid bone in Neurofibromatosis II. The poster will illustrate ocular photographs and MRI results.


Year: 2001

Program Number: Poster 120

Author Affiliation: Bascom Palmer Eye Institute

Co-Authors: Michelle Caputo

Co-Author Affiliation: Bascom Palmer Eye Institute

Room: Poster 120