spontaneous scleral perforation and optic nerve dural ectasia in marfan syndrome

purpose : to report two patients with spontaneous scleral perforation and bleb formation associated with marfan syndrome (mfs) which one of them also showed bilateral optic nerve dural ectasia. case reports : an 11-year-old girl was referred with progressive myopia. past medical history was negative. slit-lamp examination revealed bilateral scleral thinning and perforation with bleb formation, shallow anterior chamber (ac), and microspherophakia. systemic evaluation confirmed the diagnosis of mfs. b-scan ultrasonography and orbital mri showed bilateral optic nerve dural ectasia. a 23-year-old man, known case of mfs, also presented with spontaneous scleral perforation with bleb formation and microspherophakia. in this case, ancillary tests didn't show optic nerve dural ectasia. both patients underwent lensectomy with vitrectomy and iris-fixated intraocular lens implantation in both eyes. conclusion : mfs may be associated with new and rare findings as part of generalized connective tissue disorder.     iranian journal of ophthalmology 201325(2):167-170 © 2013 by the iranian society of ophthalmology