Primary Intrabulbar Neurofibroma

Purpose: Enucleation of the eyeball for any reason other than malignant tumour is very rare today. Solitary intrabulbar neurofibroma without other signs of neurofibromatosis is a rare benign tumour, and only few cases have been published to date. Material and Methods: Within a ten-year interval from 1 January 2007 to 31 December 2016 we analysed enucleation of the eyeball for reasons other than malignant tumours. Results: Out of a total number of 49 enucleated blind eyes, in every patient visual acuity was without light perception. Of these patients 34 (69.4 %) were indicated for enucleation due to complications following previous post-traumatic surgery, in 14 patients (28.6 %) the reason was secondary glaucoma and other complications following previous intraocular surgery, and in one patient (2 %) a primary isolated intraocular neurofibroma was verified after enucleation. Case Report: A patient with isolated intrabulbar neurofibroma was monitored from childhood age due to an intraocular lesion, which was histologically verified only in adult age. At the time of enucleation the patient was aged 25 years, he had squinted since childhood and since the age of 13 years had been monitored due to hamartoma in the right eye. Due to progression of the intrabulbar lesion, loss of visual acuity (functional condition – eye without light perception) and secondary glaucoma, the right eyeball was enucleated in adult age and subjected to a histopathological examination, which determined intraocular neurofibroma without the presence of neurofibromatosis. Conclusion: It is necessary to subject every enucleated eyeball to a thorough histopathological examination. Isolated intraocular neurofibroma may occur as an isolated orbital or intrabulbar mass, without systemic features.