Dome-Shaped Macula in a Patient with Posterior Staphyloma: An Atypical Case Presentation Janice Hui, OD and Sherrol Reynolds OD, FAAO

Abstract A 46-year-old African-American female is found to have a posterior staphyloma OS. SD- OCT reveals a dome-shaped macula and extrafoveal schisis. Although common in pathological , she had minimal myopia and no history of refractive surgeries.

I. Case History • 46 year old African-American female presented with complaint of decreased vision • Ocular history: non-proliferative sickle cell and resolved VMT OD. And a temporal juxtafoveal staphyloma with an extrafoveal schisis and vitreoretinal traction OS, which were first documented three years ago. • Medical history: sickle cell anemia • Medications: fish oil 1000mg, folic acid, multivitamin, vitamin C 500 mg, and vitamin D. History of Hydroxyurea treatment. • Allergies: penicillin • Surgeries: no ocular or medical surgeries

II. Pertinent Findings • Best- corrected: OD 20/20 OS 20/30 PH no improvement OU 20/20 • Preliminary findings: (-) RAPD, EOM full, CVF were full to finger counting • Refraction: OD -0.25/-0.50x110 BCVA: 20/20 OS -0.25/-1.25x055 BCVA: 20/30 with add of +2.25 OD 20/20 OS 20/30 OU 20/20 • Anterior segment: Mild and nasal OU otherwise unremarkable • Intraocular pressures: OD 11mmHg OS 13 mmHg by Goldmann - Posterior Segment: OD: ONH was pink with healthy rim tissue and a CD ratio of 0.50. Macula was flat with pigmentary changes and vasculature was normal. Non-proliferative sickle cell retinopathy (black sunburst lesion) and fibrotic changes were noted temporally. There was no retinal tears, holes, or detachments. OS: ONH was pink with healthy rim tissue and a CD ratio of 0.55. An area of retinal thinning juxtafoveal extending to the periphery was noted. There was no sickle cell retinopathy and no retinal tears, holes, or detachments. • Other testing: SD-OCT macular cube, HD 21 line, and OCTA 6x6 OD: resolved vitreomacular traction (VMT), complete PVD, and ERM OS: dome-shaped elevated macula with a temporal juxtafoveal staphyloma and extrafoveal schisis encroaching on to the fovea

III. Differential Diagnosis Dome-shaped macula • Morphological anterior protrusion of the macula relative to the chorioretinal cavity1 • Unknown cause, but associated with posterior staphyloma found in high myopia1 • Abnormal configuration can cause reduced vision2 Macular pucker • ERM develops as a result of glial proliferation and can impact visual acuity3 • In severe cases, membrane can thicken and contract resulting in a thickened retina3 • In this case, SD-OCT showed an anterior protrusion of the along with the IV. Diagnosis and discussion • Diagnosis: Atypical presentation of dome-shaped macula with a temporal retinal staphyloma complicated with an extrafoveal schisis in OS • Patient had minimal myopia without history of refractive surgeries but displayed common posterior segment complications of high myopia • Posterior staphyloma in high myopia is due to abnormal collagen fibers4 o Smaller, immature and fewer cross-linkages results in increased elasticity and susceptibility to stretching4 o Patient may have been born with physiologically abnormal fibers resulting in the existing temporal staphyloma • Dome shaped macula is usually seen in highly myopic patients with a staphyloma1 o Possible mechanisms: adaptation to decrease defocus at macula, vitreomacular traction, resistance to growth of staphyloma, or localized choroidal thickening1 o Similarly, presence of the staphyloma in this case may trigger the mentioned mechanisms to cause the development of a dome-shaped macula

V. Treatment and Management • Complications of dome-shaped macula: foveal detachment2 , SRD and CNVM1 • Complications of posterior staphyloma: CNVM, , macular holes4 • Close monitoring with SD-OCT is needed for early detection of retinal complications that can have severe impact on vision • Patient was monitored every 3 months with DFE for complications due to sickle cell retinopathy OU and with SD-OCT for progression of extrafoveal schisis, complications due to dome-shaped macula and staphyloma OS • Management plan: Due to extrafoveal schisis progression with visual disturbance, patient was referred to retinal specialist for consultation

VI. Conclusions • case highlights an atypical presentation of dome-shaped macula associated with a posterior staphyloma can occur in patients without pathological myopia • SD-OCT is necessary in the diagnosis and management of this condition • Patients require close monitoring for associated complications, including visual disturbances, progression of retinoschisis, serous detachment, and CNVM1,4

References 1. Errera, M. H., Michaelides, M., Keane, P. A., Restori, M., Paques, M., Moore, A. T., et al. (2014). The extended clinical phenotype of dome-shaped macula. Graefe's Archive for Clinical and Experimental = Albrecht Von Graefes Archiv Fur Klinische Und Experimentelle Ophthalmologie, 252(3), 499-508. 2. Gaucher, D., Erginay, A., Lecleire-Collet, A., Haouchine, B., Puech, M., Cohen, S. Y., et al. (2008). Dome-shaped macula in eyes with myopic posterior staphyloma. American Journal of Ophthalmology, 145(5), 909-914. 3. Stevenson, W., Prospero Ponce, C. M., Agarwal, D. R., Gelman, R., & Christoforidis, J. B. (2016). : Optical coherence tomography-based diagnosis and classification. Clinical Ophthalmology (Auckland, N.Z.), 10, 527-534. 4. Hsiang, H. W., Ohno-Matsui, K., Shimada, N., Hayashi, K., Moriyama, M., Yoshida, T., et al. (2008). Clinical characteristics of posterior staphyloma in eyes with pathologic myopia.American Journal of Ophthalmology, 146(1), 102-110