Fall 2017 Newsletter

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Fall 2017 Newsletter
7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: It’s So Dry

Ho Kashani
Presenter: Tiffany Ho, MD Discussant: Amir H. Kashani, MD, PhD
 

History

  • 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

Exam Findings

  • BCVA: 20/60 OD; 20/25 OS
  • IOP 19 OU
  • Pupils: Round, reactive, no rAPD
  • SLE OU: trace NSC and anterior syneresis
  • DFE OU:
Figure 1
Figure 1: Fundus photographs of right and left eye showing confluent soft drusen and pigmentary changes within the macula.

 

Figure 2
Figure 2: (A) OCT macula of the right eye showing large central pigment epithelial detachment (PED) with hyperreflective material beneath RPE and adjacent drusenoid PEDs. No intra- or subretinal fluid observed. Normal choroid (B) OCT macula of left eye with similar findings as right eye with smaller central PED.

 

Differential Diagnosis of Chorioretinal Conditions with Pigment Epithelial Detachments (PED)

  • Age-Related Macular Degeneration
  • Central Serous Chorioretinopathy
  • Polyploidal Choroidal Vasculopathy
  • Subtypes of PED
    • Drusenoid
    • Serous
    • Fibrovascular
    • Hemorrhagic

Additional Investigations

  • Ancillary testing to determine whether choroidal neovascularization is present
Figure 3
Figure 3: Fluorescein angiography of right eye at initial presentation. (A) Late arterial or early laminar phase with hypofluorescent rim (indicating hypopigmentation or RPE atrophy) surrounding hyperfluorescent center (suggesting pigment clumping). (B) Arteriovenous transit phase without leakage. (C) Late phase with staining within PED without leakage (hot spots or plaques).

 

Figure 4
Figure 4: Indocyanine green angiography of right eye showing irregular scalloped borders of drusenoid PED at initial presentation. Hypofluorescent during early and late frames suggesting that choroidal neovascularization (CNV) is not present.
  • Eight months after initial presentation. Visual acuity: 20/200 OD; 20/80 OS.
Figure 5
Figure 5: Figure 5: OCT Macula at eight-month follow-up. (A) Right eye with larger central drusenoid PED with vitelliform lesions at PED apex. (B) Left eye with enlarging central drusenoid PED with vitelliform lesions at top of apex with possible subretinal fluid.

 

  • 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
Figure 6
Figure 6: Fundus photograph of both eyes showing increased pigmentary changes (hyperpigmentation in macula).

 

Figure 7
Figure 7: OCT macula. (A) Right eye showing PED collapse with pockets of intraretinal fluid overlying PED and intraretinal RPE migration. (B) Left eye showing PED with red asterisk indicating focal RPE disruption (possible RPE aperture).

 

Figure 8
Figure 8: (A) Fluorescein angiography of left eye showing late staining of drusen, no leakage, and window defect temporal to macula (blue arrow) (B) ICG of left eye showing no clear CNV and window defect temporally (blue arrow).

 

Diagnosis

  • 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

References

  • 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.

Contact

Section Editors

  • Vivek Patel, MD, Associate Professor of Clinical Ophthalmology, Program Director, vivek.patel@med.usc.edu
  • Jesse Berry, MD, Assistant Professor of Clinical Ophthalmology, Associate Program Director, jesse.berry@med.usc.edu
  •  

    Produced by: Monica Chavez, John Daniel and Dr. Vivek Patel
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