A Case of Neurofibromatosis Type1 with Sphenoid Bone Defect in which Orbitoplasty with a Titanium Mesh Plate and DuraGen^® was Effective in resolving Pulsating Exophthalmos

Abstract

  Neurofibromatosis type1 is a rare disease that causes a variety of symptoms including café-au-lait spots, neurofibromas, bone lesions, and ocular lesions. We report here a case of neurofibromatosis type1 in which a sphenoid bone defect caused a frontal lobe herniation into the orbit, and pulsatile protrusion of the eyeball was observed. Surgical reconstruction of cranial and orbital defects at an early stage, without damaging the neural structures, is considered effective but the surgical method should be carefully chosen depending upon the site of the bone defect and the degree of brain herniation. In this study, orbitoplasty using a titanium plate and DuraGen^® (an artificial dural substitute) resulted in rapid elimination of the pulsating exophthalmos. Titanium plates are malleable and suitable for orbital application. Surgical intervention may reduce pulsating exophthalmos and associated ocular motility disorders.