Dr.ANOOP SINGH

Dr. PADMAJA KRISHNAN

Abstract

23 year old boy consulted for cosmetic correction of progressive drooping right upper eyelid since 1 yr. O/E facial dysmorphism and inferonasal eccentric proptosis in RE with restricted dextroelevation. Bilateral upper eyelids showed soft boggy mass in superotemporal orbit (R>L). BCVA RE-6/9 LE-6/6 and Lisch nodules present bilaterally. Multiple Neurofibromas seen on face, chest, trunk and lower limbs also multiple Café au lait spots were noted over the trunk. MRI scan showed plexiform neurofibroma extending from right periorbital region till superior orbital fissure through orbital pre-septal space and superior extraconal compartment compressing and widening partially empty sella and associated right sphenoid wing dysplasia, hamartoma and arachnoid cyst in brain. Patient was put on HRT after hormonal essay post neurologist and endocrinologist opinion for delayed puberty. Cosmetically small Plexiform NF may need integrated approach with regular followups to avert emergencies.