Background: Type I Neurofibromatosis (NFI) is a dominant autosomal disease with a current incidence of 1 in 3000 births. Plexiform Neurofibromas (PN) are congenital tumours which present aesthetic and functional deficiencies in up to 25% of NFI cases. This study aims to review the clinical findings of orbital PN and therapeutic options available.

Method: This is a descriptive study,which was conducted on five pediatric patients who presented orbital PN at our institution.

Results: Initial treatment was conservative in all cases, performing partial tumour resections,while maintaining a good visual function and acceptable aesthetics. During the follow-up, two patients remained stable, but needing additional conservative procedures. In another patient, the disease progressed to visual loss despite conservative surgery, and the patient was unwilling to undergo further surgical treatments. In the two remaining cases, PN progression had significant functional and aesthetic impact, and an orbital exenteration with reconstruction of the anophthalmic orbit had to be performed.

Conclusions: Management of this entity is challenging and should be individualized according to the involvement of soft tissue, bone structure, visual function and psychosocial factors, based on the age of patients, this requiring a multidisciplinary team. Despite conservative management is our first choice, orbital exenteration for disease control should not be ruled out.

Key words: orbital plexiform neurofibroma; treatment; children