Atlas of Ophthalmology

Peripapillary Retinoschisis in Presumed Optic Cup Microhole (Fundus Photography, VF, FFA, SD-OCT)

Retina -> Distrofias y Degeneraciones Hereditarias -> Retina Sensorial -> Retinosquísis Degenerativa
Patient: 74 years of age, female, BCVA 0.7 at OD, 0.8 at OS, IOP OD 17 mmHg, OS 15 mmHg, pachymetry OD 560µm, OS 566µm. General Medical History: empty. Ocular Medical History: no visual problems, routine checkup to rule out glaucoma. Main Complaint: no complaints. PURPOSE: To describe a peripapillary and macular retinoschisis, only visible by OCT. Methods: Colour fundus photography, w-w-perimetry (Octopus G1), fluorescein angiography (HRA, Heidelberg Engineering), SD-OCT (Heidelberg Engineering). Colour fundus photography: no glaucomatous optic nerve atrophy, no epiretinal gliosis. W-w-perimetry : peripheral scotoma. Frequency Doubling Technique FDT: paracentral scotoma. OCT: large peripapillary retinoschisis, including the macular area, mimicking a true retinoschisis cavity, although bridging retinal elements were identifiable. Cavity within cup suggests optic pit (arrow). No evidence of choroidal neovascular membrane. Fluorescein Angiography : showing minor leakage on fluorescein angiography within the optic disk (arrow), indicating location of the cavity. No evidence of choroidal neovascular membrane. Discussion: The etiology of the peripapillary and macular retinoschisis is uncertain. There was no history of acute rises in IOP. A congenital optic pit could not be found by ophthalmoscopy. We hypothesized that there might be a communicating bridge connecting the cavity within the optic nerve head and the peripapillary retinoschisis, which was found in the fluorescein angiography . Zumbro et al (1) hypothesized that fluctuations in IOP played a role in vitreous traction and the development of retinoschisis. They supposed that acute elevations in IOP may have led to small changes in axial length or severe optic nerve cupping that allowed vitreous to enter the retina. As there were no severe fluctuations in IOP of our case, but a minor leakage in the optic cup was observed, we believe that the retinoretinoschisis is the result of a structural defect in the optic nerve in the optic disk. Microholes in the thinning and structural defects areas in the optic cup allowed liquid vitreous to enter the retina via the nerve fiber layer. The fluid caused retinal edema, followed by a retinoschisis. Macular and peripapillary retinoschisis can occur in patients with presumed enlarged optic nerve head cups in the absence of obvious congenital anomalies of the disk. We believe the cause is leakage of fluid through a tiny hole in the thin tissue of the cup. This is a similar mechanism to that seen in patients with optic pits Literature: (1) Zumbro DS, Jampol LM, Folk JC, Olivier MM, Anderson-Nelson S. Macular schisis and detachment associated with presumed acquired enlarged optic nerve head cups. Am J Ophthalmol. 2007 Jul;144(1):70-74

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