I. CASE HISTORY: • Patient demographics: 49 yo African-American male • Chief complaint: Blur @ distance with current bifocal • Ocular History: H/o eye drop prescribed by private provider for “pressure” used 1x/day— pt self discontinued • Family Ocular History: + Glaucoma: Mother, Father & Grandparents • Medical History: Emphysema, H/o Migraine, PTSD, Hyperlipidemia, Sleep Apnea • Medications: Prednisolone 10Mg II. CLINICAL • VA cc 20/40+2 OD, 20/25+2 OS • TA: 36 OU with Goldmann • Confrontational Fields: Restricted OD/OS • Pachymetry: OD: 589, OS: 583 • Gonioscopy: OD: TM 360 w/ 2+ pigment • OS: TM 360 w/ 2+ pigment • HVF 24-2: o OD: Central inferior island of vision remaining o OS: Central inferior island of vision remaining • HVF 10-2: o OD: Superior and Nasal absolute defects. Inferior temporal island of vision remaining: 5H x 6V degree of vision o OS: Superior and Nasal absolute defects. Inferior temporal island of vision remaining: 8H x 7V degree of vision • OCT Macula: o OD: Macula schisis; Deep optic pit and lamina loss evident + ERM o OS: Retinoschisis noted superior to ONH; Deep optic pit and lamina loss evident + ERM

III. PHYSICAL: • Dilated Fundus Exam: 0.9R Extremely thin rim 360 Deep Cup OD; 0.9R thinning 360, small areas of nasal rim intact OS. Macula: Retinoschisis OD, Grade 1 ERM with OU with mild macular traction with flat intact macula OU. IV. DIFFERENTIAL DIAGNOSIS: • PRIMARY: Progression of POAG associated with Acquired pits of the Optic nerve and maculoschisis. • SECONDARY: Congenital optic nerve anomaly with associated maculoschisis contributing to progression of POAG. V. DIAGNOSIS & DISCUSSION: • GENERAL: o Optic Neuropathy associated with glaucoma represents a loss of axons of the retinal ganglion cell fibers at the level of the lamina cribosa. This occurs due to intraocular pressure above physiologic levels and results in the clinical manifestation of cupping. Less commonly, the damaged lamina cribosa can cause focal structural abnormalities resulting in acquired pits of the optic nerve (APON) and papillomacular retinoschisis1. o APON has been reported to be associated with visual field defects in addition to the presence and progression of glaucoma. It has also been suggested that maculoschisis associated with APON can be a result of progression of glaucomatous optic disc damage in cases with untreated glaucoma1. The damage of the disc results in a localized defect of the rim that would permit leakage of fluid into the sub retinal space from the choroid2. o • CASE STUDY: o This patient presented for initial examination with BCVA 20/40 OD & 20/30 OS, severely constricted visual fields in all quadrants of both eyes and pressures of 36 mm Hg OD/OS. DFE examination demonstrates severe rim tissue loss with bilateral asymmetric papillomacular retinoschisis. Spectral domain optic coherence tomography imaged the extreme loss for retinal ganglion cell fibers with the retinoschisis extending from the optic nerve in both eyes. Management included initiation of Latanoprost at bedtime OU and Cosopt BID OU. Surgical intervention to improve the patients visual acuity was NOT indicated due to poor prognosis and lack of remaining retinal ganglion cell fibers.

V. TREATMENT AND MANAGEMENT: o GENERAL: ▪ Lowering the intraocular pressure is the primary mode of treatment in an attempt to delay the progression of glaucoma. It is essential to set a target IOP and monitor at designated intervals with visual field and OCT imaging of the RNFL for progression or stability. ▪ In order to minimize the fluid resulting in maculoschisis many forms of treatment have been utilized. Argon Laser photocoagulation has been proven effective when used along the disc margin in the area of retinal detachment3. Other methods such as Intravitreal gas injections alone or in combination with laser therapy, and a recent proposed technique of surgery of the inner retinal fenestrations have been proposed3. However, the most common mode of treatment has been to perform a pars plans vitrectomy with PVD induction, with or without ILM peeling4. Occasionally this is performed in combination with laser photocoagulation in the peripapillary area. The ILM peeling is performed to further relieve traction, and the laser photocoagulation is not deemed as a necessity due to the lack of data suggesting the fluid transportation from the inner retina3. The end result has been very successful not only in reattaching the detached retina but also improving the visual acuity. Lastly, 25% of maculopathy associated with optic disc pits has been shown to resolve spontaneously however the resulting vision was most often poor3. o CASE STUDY: ▪ The management of this patient’s presentation due to the severely constricted visual field was mainly focused on lowering the IOP by the use of ocular anti-hypertensives. The patient was started on Latanoprost QHS OU and Cosopt (Dorzolamide/Timolol) BID OU. He was then monitored on a weekly basis with routine pressure checks and imaging. The imaging of the retinal nerve fiber layer was not helpful due to the advanced glaucoma. However, the maculoschisis was easily identified on OCT. Conservative management was utilized for the serous detachments in the macula in the form of weekly OCTs of the macula to monitor the condition of the schisis. Surgical intervention was not enforced due to the poor prognosis secondary to the severe loss of retinal ganglion cells resulting in an already significantly reduced visual field. VI. CONCLUSION: • It is crucial to be aware of the possible associated maculopathy when a patient with untreated and progressive glaucoma complains of decreased vision. Optic nerve pits, regardless of acquired or congenital, can contribute to the severe loss of the visual field constituting a very progressive form of glaucoma4. In addition, the maculoschisis can further deter central vision resulting in legally blindness at an accelerated rate. Monitoring these patients at frequent intervals with imaging and consulting retinal specialists is vital in ensuring the prevention of further loss of the retinal ganglion cells. References 1. Yoshitake T, Nakanishi H, Setoguchi Y, Kuroda K, Amemiya K, Taniguchi M, Otani A. Bilateral papillomacular retinoschisis and macular detachment accompanied by focal lamina cribs defect in glaucomatous eyes. Jpn J Opthalmol 2014; 58: 435-442. 2. Rath EZ, Rumelt S. Acute visual loss due to serous retinal detachment from acquired optic pit may be a rare presentation of primary open-angle glaucoma. Can J Ophthalmic 2007; 42(2): 339-340. 3. Georgalas I, Ladas I, Georgopoulos G, Petrou P. Optic disc pit: a review. Graefes Arch Clin Exp Ophthalmol 2011; 249: 1113- 1122. 4. Ohno-Matsui K, Hirakata A, Makoto I, Masahiro A, Tatsuro I. Evaluation of congenital optic disc pits and optic disc colobomas by swept-source optical coherence tomography. Invest Opthalmol Vis Sci. 2013; 54: 7769-7778.