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Optic Disc Pit associated with Maculopathy and Retinoschisis

Einbock W., Schnurrbusch U. E. K., Wiedemann P.,
Universität Leipzig, Klinik und Poliklinik für Augenheilkunde (Leipzig)

Purpose: Optic disc pits represent a rare congenital (1:10000) abnormality attributed to imperfect closure of the upper end of the embryonic fissure and frequently associated with macular detachment. Two third of the patients with optic nerve pit develop between 2.-4. decade of live a maculopathy and a schisislike separation of the inner macular retina of unclear reason.
Method: A 29-year old patient presented with diminished visual acuity (visual acuity R 20/16; L 20/40) of about one year duration relating to a temporal optic pit in the left eye with a central detached retina and a schisislike separation of the retina in the optical coherence tomography. A pars-plana-vitrectomy with surgical posterior vitreous detachment was performed. After ICG-injection a cystic structure corresponding to the central retina detachment was marked probably over the optic nerve pit. After removal of the outer membrane and peeling of an also marked deeper one the central retina seemed reattached and SF6-gasinjection was investigated.
Results: 9 month postoperative the visual acuity in the left eye recovered to 20/25. The central detachment of the retina and a cystic macular edema remained. The OCT showed also a detachment of the retinal pigment epithelium which correlated with the good vision.
Conclusions: The vitreoretinal border seems to play an determining role in the pathogenesis of the maculopathy in cases of optic nerve pits. Neither in the postoperative fundusautofluorescence nor in the electronmicroscopic investigation of the operative removed tissue any evidence for peeled lamina limitans interna could be found. Differentially the nature of the removed membrane could be structurally changed through recidivating resorption or chronic modification. A possible tractional role between vitreous and retina as well as the unclear origin of the subretinal fluid determine the unsatisfying therapeutic options so far.