NEUROFIBROMATOSIS IN A PATIENT WITH UNILATERAL PULSATING PROPTOSIS

Jeff Pietrzyk

Abstract

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.

Details

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