Case Study: It’s So Dry
|Presenter: Tiffany Ho, MD
||Discussant: Amir H. Kashani, MD, PhD
- 56-year-old Caucasian female
- History of drusen presents to retina clinic for second opinion
- Patient reports decreased vision and distortion of vision in right eye
- BCVA: 20/60 OD; 20/25 OS
- IOP 19 OU
- Pupils: Round, reactive, no rAPD
- SLE OU: trace NSC and anterior syneresis
- DFE OU:
Differential Diagnosis of Chorioretinal Conditions with Pigment Epithelial Detachments (PED)
- Age-Related Macular Degeneration
- Central Serous Chorioretinopathy
- Polyploidal Choroidal Vasculopathy
- Subtypes of PED
- Ancillary testing to determine whether choroidal neovascularization is present
- Eight months after initial presentation. Visual acuity: 20/200 OD; 20/80 OS.
- Three years after initial presentation s/p intravitreal Eylea x3 and Lucentis x1 OD and Lucentis x1 OS. Visual acuity: 20/50 OD and 20/80 OS
- Non-neovascular AMD (intermediate AMD) with bilateral drusenoid PED
- Eight months: Bilateral enlargement of bilateral PED with possible appearance of subretinal fluid on OCT Macula without CNV see on FA/ICG and unresponsive to anti-VEGF therapy
- Three years: Collapse of PED with focal RPE atrophy suggesting RPE aperture without clear CNV
Pathophysiology of AMD
- Metabolic disorder of RPE and photoreceptors
- Accumulation of granular lipid rich material between plasma and basement membrane of RPE (basal laminar deposits)
- Accumulation of lipofuscin deposits in Bruch’s membrane (basal linear deposits or soft drusen)
- Progressive accumulation of lipids creates a hydrophobic environment
- RPE pumping becomes stressed causing accumulation of fluid and debris leading to RPE detachment from Bruch’s
Treatment (AAO Practice Guidelines for Non-Neovascular AMD)
- Smoking cessation
- Monitoring with Amsler Grids
- AREDS2: intermediate AMD or advanced AMD in one eye
- Close monitoring for CNV with multimodal imaging studies (FA, ICG, OCT, OCT-A)
Prognosis and Future Directions
- Drusenoid PEDs associated with non-neovascular AMD (especially intermediate rather than high-risk AMD)
- Better visual prognosis than other PEDs
- Subset of Age Related Eye Disease (AREDS) Study #28
- 5 percent prevalence of drusenoid PEDs within study
- At median of eight years, 42 percent of patients developed advanced AMD with 19 percent developing geographic atrophy and 23 percent neovascular AMD
- Patients usually experience decline in visual acuity (loss of 26 letters from 20/30 to 20/60)
- In comparison, original AREDS study showed that risk of progressing from intermediate to advanced AMD over five years, 6 percent with one eye involvement and 26 percent with two eye involvement
- Life cycle of drusenoid PED
- Drusenoid PED usually develop and enlarge on background of large confluent soft drusen and hyperpigmentation
- Collapse of PED through unknown mechanism
- Development of hypopigmentation
- Final phase: central geographic atrophy
- Roquet W, Roudot-Thoraval F, Coscas G SG. Clinical features of drusenoid pigment epithelial detachment in age related macular degeneration. Br J Ophthalmol. 2004;88:638-43.
- Tan, A; Simahae, D; Balaratnasingam, C; Dansingani, K; Yannuzzi LA. A Perspective on the Nature and Frequency of Pigment Epithelial Detachments. Am J Ophthalmol. Elsevier Inc.; 2016;172:13-27.
- Querques G, Capuano V, Costanzo E, Corvi F, Querques LEA, Introini UGO, et al. Retinal Pigment Epithelium Aperture: A Previously Unreported Finding in the Evolution of Avascular Pigment Epithelium Detachment. Retina. 2016;36(12):65-72.
- Mrejen S, Sarraf D, Mukkamala SRIK. Multimodal Imaging of Pigment Epithelial Detachment: A Guide to Evaluation. Retina. 2013;33:1735-62.
- Zayit-soudry S, Moroz I, Loewenstein A. Retinal Pigment Epithelial Detachment. Surv Ophthalmol. 2007;52(3):227-43.
- Amd NE, Amd I, Bressler SB, Bressler NM. Age-Related Macular Degeneration. Fifth Edit. Retina. Elsevier Inc.; 2000. 1150-1182 p.
- Sikorski BL, Bukowska D, Kaluzny JJ, Szkulmowski M, Kowalczyk A, Wojtkowski M. Drusen with Accompanying Fluid. Ophthalmology. Elsevier Inc.; 2011;118(1):82-92.
- Vivek Patel, MD, Associate Professor of Clinical Ophthalmology, Program Director, email@example.com
- Jesse Berry, MD, Assistant Professor of Clinical Ophthalmology, Associate Program Director, firstname.lastname@example.org
Produced by: Monica Chavez, John Daniel and Dr. Vivek Patel