Megan Patterson, OD Battle Creek Veterans Affairs Medical Center Ocular Disease Resident American Academy of Optometry Residents Day Submission

Title: The Thick of Things: Advancement In Technology Provides A New Outlook On An Old Disease

Abstract: This case features a patient who displays pachychoroid pigment epitheliopathy, which falls within a newly described spectrum of macular disorders. This outline includes case data, advanced diagnostic procedures, and management of the aforementioned disorder.

I. Case History • Demographics: 30-year-old Caucasian male • Chief complaint: Blurry vision OS and distortion in left visual field starting 1 week prior • Ocular History: Myopia and astigmatism • Medical History: Post traumatic stress disorder • Medications: Multivitamin, vitamin B complex, melatonin II. Pertinent Findings • Entrance Testing: • BCVA - OD: 20/20, OS: 20/20 • Pupils - equal, round, and reactive to light OD & OS, no relative afferent pupillary defect • EOMs - full and smooth OU, no pain on eye movement • Confrontations - full to finger counting OD & OS • Amsler grid - OD: normal, OS: metamorphopsia greatest temporally • Tonometry - OD: 16mmHg, OS: 17mmHg @ 12:59 pm • Slit Lamp Evaluation: • Cornea - clear OD & OS • Conjunctiva - clear OD & OS • Anterior Chamber - deep & quiet OD & OS • Iris - normal OD & OS • Lens - clear OD & OS • Posterior Segment Findings: • OD: snailtrack superior, all other findings including macular health within normal limits • OS: 1 DD area of elevation just nasal to the fovea with a central yellowish appearance surrounded by pigment changes, all other findings within normal limits • Macular EDI-OCT - OD: subfoveal choroidal thickness of 333 μm, all other findings within normal limits, OS: subfoveal choroidal thickness of 393 μm, with an ~560 μm focal area of RPE hyperplasia nasal to fovea • Color fundus photos - OD: within normal limits, OS: small yellowish appearance surrounded by pigment changes nasal to fovea • FAF photos - OD: within normal limits, OS: small area of RPE changes showing both hypo- and hyperautofluorescence nasal to fovea • FANG - OD: within normal limits, OS: small hyperfluorescent dot nasal to fovea III. Differential Diagnosis • Pachychoroid pigment epitheliopathy • Central serous chorioretinopathy • Early age-related macular degeneration • Chorioretinal neovascular membrane IV. Diagnosis and Discussion • Case Diagnosis: Pachychoroid pigment epitheliopathy • Following a retinal consult and multimodal imaging • Secondary Diagnoses: Myopia and astigmatism • Discussion: • Pachychoroid clinical spectrum is a group of macular disorders that share similar choroidal findings • These findings include increased choroidal thickness, dilated outer choroidal vessels (“pachyvessels”), and attenuation and thinning of the choriocapillaris and Sattler’s layer • Central serous chorioretinopathy, pachychoroid pigment epitheliopathy, and pachychoroid neovasculopathy comprise this spectrum of disorders • Advancement in imaging technology has aided in the discovery of this recently described spectrum by allowing us to better visualize the pathological changes that occur within the choroid • Such imaging includes enhanced-depth imaging OCT, swept-source OCT, en face OCT, and OCT angiography • One recent study found the mean subfoveal choroidal thickness in normal eyes to be approximately 270 μm using en face SS-OCT • Pachychoroid pigment epitheliopathy is thought to be an atypical manifestation of central serous chorioretinopathy, specifically without a serous macular detachment • Upon clinical examination, patients will present with reduced fundus tessellation, retinal pigment epithelial abnormalities overlying areas of choroidal thickening, and fundus autofluorescence abnormalities • Pachychoroid neovasculopathy is considered a late complication of pachychoroid pigment epitheliopathy or chronic central serous chorioretinopathy • This can further develop into polypoidal choroidal vasculopathy • A multimodal imaging approach is the most effective way to properly diagnosis these pachychoroid disorders V. Treatment and Management • Once diagnosed, pachychoroid pigment epitheliopathy is monitored closely with repeat OCT’s and ancillary imaging as needed, which was how the patient in this case was managed • Following stability of the condition, time between follow-up appointments can be gradually extended • Extensive patient education on monitoring of home Amsler grid and being cognizant of any changes to central vision is imperative • At this time, anti-VEGF treatment is the mainstay treatment if the condition progresses into pachychoroid neovasculopathy • Photodynamic therapy has been proposed as a possible alternative/superior treatment, but much further research is needed regarding therapeutic intervention for pachychoroid disorders VI. Conclusion • Advancements in technology and imaging has made it possible to visualize pathological changes within the choroid that were not easily discerned in the past • Central serous chorioretinopathy, a once thought idiopathic disease, appears to actually stem from an underlying pachychoroid disorder • These same pachychoroid changes are thought to cause pachychoroid pigment epitheliopathy, a somewhat similar macular disease • More research is necessary to better understand the etiology, pathophysiology, and treatment of the pachychoroid clinical spectrum

References:

Adhi M, Duker JS. Optical Coherence Tomography. Current Opinion in Ophthalmology. 2013;24(3):213- 221.

Dolz-Marco R, Dansingani KK, Freund KB. Identifying the Pachychoroid Phenotype. Review of Ophthalmology. 2016 July.

Dolz-Marco R, Dansingani KK, Freund KB. The Pachychoroid Clinical Spectrum. Retina Today. 2016May/June.

Fujiwara A, Morizane Y, Hosokawa M, Kimura S, Kumase F, Shiode Y, Doi S, Hirano M, Toshima S, Hosogi M, Shiraga F. Factors affecting choroidal vascular density in normal eyes: Quantification using en face swept-source optical coherence tomography. American Journal of Ophthalmology. 2016 July.

Gallego-Pinazo R, Dolz-Marco R, Gómez-Ulla F, Mrejen S, Freund KB. Pachychoroid Diseases of the Macula. Medical Hypothesis, Discovery and Innovation in Ophthalmology. 2014;3(4):111-115.

Heiferman M, Simonett J, Fawzi A. En Face OCT Imaging in Retinal Disorders: An emerging technique offers innovative insights. Retinal Physician. 2015 June.

Michalewska Z, Michalewski J, Nawrocki J. Swept-source OCT: Wide-field simultaneous choroid, retina, and vitreous visualization. Retina Today. 2013 Sept.

Spaide RF. Applications for OCT Enhanced Depth Imaging. Retina Today. 2011 Sept.

Warrow DJ, Hoang QV, Freund KB. Pachychoroid pigment epitheliopathy. Retina. 2013 Sep;33(8):1659- 72

Yun S, Adelman RA. Enhanced Depth Imaging of Spectral Domain Optical Coherence Tomography in Age- Related Macular Degeneration. Ophthalmology Management. 2014 June;70.