Double Trouble: Entropion Repair Complicated by a Conjunctival Neurofibroma

Abstract

While ocular involvement is well documented in association with neurofibromatosis type 1 (NF-1), conjunctival neurofibromas are very rare. We describe a challenging NF-1 case of a patient with a conjunctival neurofibroma in association with lower lid involutional entropion and severe conjunctival chemosis. A 65 year-old male presented with age-related left lower lid laxity, retractor dehiscence, inferior conjunctival chemosis and significant ocular discomfort. The patient underwent a left lower lid lateral tarsal strip procedure with retractor plication without much improvement in the chemosis and discomfort. However post-operatively it became evident that an injected mass was present in the left inferior fornix. Surgical debulking of this mass allowed for resolution of the patient’s signs and symptoms as well as proper lower lid alignment. Histopathologic diagnosis with S100 and CD34 positivity confirmed a conjunctival neurofibroma. Although uncommon, it is important to consider the possibility of a conjunctival mass in a patient with persistent chemosis and recurrent lower lid instability, particularly when confronted with a neurocutaneous syndrome.