Optic disc pits represent congenital anomalies in the optic nerve head commonly associated with retinoschisis and serous retinal detachments.1 In contrast, acquired glaucomatous damage to the optic nerve, both localized acquired pits and diffuse Schnabel optic atrophy, has not been linked to retinal detachment. A recent report by Spaide et al2 demonstrated schisis and outer layer detachment, the characteristic features of optic pit maculopathy, in the absence of an optic pit. We describe a patient who developed macular schisis and underlying serous detachment in an eye with a large optic cup following repeated attacks of angle-closure glaucoma.
A 54-year-old man was seen by an ophthalmologist and complained of 4 weeks of intermittent pain and blurring of vision in his right eye. A right afferent pupillary defect was present, and the visual acuity was 20/200 OD and 20/25 OS. At examination, the intraocular pressure in his right eye was 52 mm Hg. Gonioscopy revealed angle closure in his right eye and narrow angles in his left eye. The patient was treated with bilateral peripheral iridotomies and topical glaucoma medications. Fundus examination revealed a serous retinal detachment in the right eye.
During the next 3 months, the patient noted enlargement of a central scotoma in his right eye, and he was referred to the retina service at the University of California, San Francisco. The visual acuity in his left eye had remained stable, yet that in the right eye had dropped to 20/800 OD. Significant asymmetry of the optic nerves was observed, with cup-disc ratios of 0.9 OD and 0.2 OS (Figure 1). The fundus of the left eye was unremarkable, but the right eye had a serous retinal detachment with fluid extending from the disc margin through the macula (Figure 2A), as well as a posterior vitreous detachment. Contact lens examination revealed a small area of dehiscence in the internal limiting membrane just temporal to the right optic disc.
Asymmetrical optic nerve cupping. A, Right optic nerve with severe cupping. B, Normal-appearing left optic nerve. C, Studies of the right optic nerve obtained with the Heidelberg Retina Tomograph (Heidelberg Engineering, Heidelberg, Germany).
A macular schisis detachment of the right eye. A, Fundus photograph of the right eye with fluid extending from the optic disc, a double ring sign consistent with a retinal detachment and overlying retinoschisis, and deep subretinal yellow precipitates in the macula. B, Fluorescein angiogram demonstrates an area of hyperfluorescence in the central and inferotemporal margin of the right optic nerve without subretinal leakage. C, Optical coherence tomography of the right eye with a horizontal scan revealing outer layer retinoschisis connecting to the optic disc. D, A vertical scan through the fovea revealing a neurosensory retinal detachment with overlying retinoschisis.
An area of hyperfluorescence deep in the central and inferotemporal margin of the right optic cup without leakage into the subretinal space was present on fluorescein angiography (Figure 2B). Optical coherence tomography demonstrated an area of retinoschisis continuous with the optic nerve with an associated neurosensory retinal detachment extending through the macula (Figure 2C and D). The patient has deferred any surgical intervention. His intraocular pressure remains well controlled with timolol maleate in the right eye.
The etiology of the schisis and serous retinal detachment in this case remains uncertain. The optical coherence tomographic findings demonstrate retinoschisis and retinal detachment characteristic of the maculopathy associated with congenital optic pits,3 yet a congenital optic pit could not be identified. The history of a central scotoma that followed acute rises in intraocular pressure suggests that the increased pressure and optic nerve cupping may have played a role in the production of the schisis detachment. We cannot exclude the possibility of a congenital optic pit that was obscured by the significant cupping of the nerve head. The patient reportedly had normal fundus examination results in the past, although photographs are not available.
Animal studies of Schnabel optic atrophy have demonstrated that prolonged rises in intraocular pressure may lead to ruptures in the inner limiting membrane and subsequent penetration of vitreous into the retrolaminar space.4 In our patient, the sustained rise in intraocular pressure led to severe optic nerve cupping and may have allowed liquid vitreous to enter the retina via the nerve fiber layer. This is supported by the direct communication of the schisis cavity with the optic nerve, as shown by optical coherence tomography, with the outer layer detachment likely occurring as a secondary event.
We report this case to document the development of macular schisis and retinal detachment in the absence of a congenital optic pit. This case raises the question whether acute rises in intraocular pressure from glaucoma can produce structural defects in the optic nerve head that can lead to a schisis detachment similar to that seen in cases of congenital optic pits.
Correspondence: Alexander R. Irvine, MD, University of California, San Francisco, Department of Ophthalmology, 10 Koret Way, Suite K-301, San Francisco, CA 94143 (email@example.com).
Financial Disclosure: None.
Hollander DA, Barricks ME, Duncan JL, Irvine AR. Macular Schisis Detachment Associated With Angle-closure Glaucoma. Arch Ophthalmol. 2005;123(2):270-272. doi:10.1001/archopht.123.2.270