Dr.ISHA CHATURVEDI

Dr. KUMUDINI SHARMA

Abstract

Abstract:

A 12-year old male presented with a gradually progressive, painless swelling over right eyelid since the age of 5 years. On examination, there was a soft, massive swelling involving right upper eyelid and orbit, obstructing the visual axis and causing severe proptosis which was pulsatile in nature. His visual acuity was hand movement in right eye and 6/6 in left eye. Both pupils were round and reactive. Slit lamp exam revealed keratoconus in right eye and Lisch nodules in both eyes. Gonioscopy showed normal angle structures. Fundoscopy was normal in both eyes. Radioimaging showed absent right wing of sphenoid with temporal lobe herniation into orbit and a small right temporal encephalocele. Systemic examination revealed café-au-lait spots and axillary freckling. The patient was diagnosed as a case of plexiform neurofibroma and was kept under follow-up to rule out risk of malignant transformation.