ABSTRACT
A healthy 4-year-old male presented a fundus examination with a unilateral contractile peripapillary staphyloma surrounded by redundant retina and retinal pigment epithelium atrophy. Five years later, best-corrected visual acuity decreased to hand motion due to a retinal detachment with macular hole. One month after first vitrectomy, scleral buckle and intraocular gas, retina re-detached. Second surgery was performed with silicon oil tamponade and lensectomy without intraocular lens (IOL). Subretinal silicon oil was detected at the third month of follow-up when vitrectomy, inferior retinectomy, and laser photocoagulation of temporal border of staphyloma with silicon oil tamponade were performed. The retina remained attached and best-corrected visual acuity was 20/600 with intraocular silicon oil. A fourth surgery was performed for emulsified silicon oil extraction replaced with intraocular gas. At 6 months of follow-up, the retina re-detached again. This is a challenging vitreoretinal surgery in which re-detachments were due to retinal folds around the contractile staphyloma that raised macular hole. This is the first report of the combined presentation of contractile peripapillary staphyloma, retinal detachment and macular hole with a long-time follow-up period of years.