Patient Presentation: A 23-year-old obese female was diagnosed with idiopathic intracranial hypertension (IIH) and referred to neurosurgery for ventriculoperitoneal shunt. A baseline ocular examination was performed prior to the procedure.
On examination, vision was 20/200 in the right eye, and 20/40 in the left eye. There was a right relative afferent pupillary defect. Slit lamp examination was normal.
A dilated fundus examination was performed demonstrating the following:
Retina
Case 81
Contributor: Dr. David Rabinovitch
Patient presentation: A 47-year-old man was referred to a tertiary retina clinic for a two-month history of “wavy lines” in his central vision OU. He denied flashes, floaters or a visual field deficit. His past medical history was significant for hypertension and migraines, managed with ramipril and Tylenol PRN, respectively. His ocular history was unremarkable.
On examination, his visual acuity was 20/30+2 in the right eye (OD) and 20/20-1 in the left eye (OS). IOP was within normal limits and there was no RAPD. Anterior segment examination was unremarkable. Fundus photographs and fundus autofluorescence images were obtained and shown below:


Question 2: What are the key features on the OCT imaging?
Next, OCT angiography images were taken. The right eye’s image is shown below:

Question 3: Is there any sign of choroidal neovascularization in the OCT-A above?
Question 4: What is the most likely diagnosis?
Question 5: What are other disease entities in the pachychoroid disease spectrum?
References:
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Warrow DJ, Hoang QV, Freund KB. Pachychoroid pigment epitheliopathy. Retina. 2013;33(8):1659–1672.
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Pereira A, Aldrees S, Pimentel MC, Yan P. Updated review: optical coherence tomography findings of the pachychoroid disease spectrum. Can J Ophthalmol. 2023 Feb;58(1):e33-e35.
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Karacorlu, M., Ersoz, M.G., Arf, S. et al. Long-term follow-up of pachychoroid pigment epitheliopathy and lesion characteristics. Graefes Arch Clin Exp Ophthalmol 256, 2319–2326 (2018). https://doi.org/10.1007/s00417-018-4144-0
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Tang J, Han X, Tang R, Li M, Wang Z, Zhao M, Qu J. Case series: pachychoroid pigment epitheliopathy transformed to polypoidal choroidal vasculopathy after long-term follow-up. BMC Ophthalmol. 2022 Jun 21;22(1):272.
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Cheung, C.M.G., Lee, W.K., Koizumi, H. et al. Pachychoroid disease. Eye 33, 14–33 (2019). https://doi.org/10.1038/s41433-018-0158-4
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Cheung, C.M.G., Dansingani, K.K., Koizumi, H. et al. Pachychoroid disease: review and update. Eye (2024). https://doi.org/10.1038/s41433-024-03253-4
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Kkaya S. Spectrum of pachychoroid diseases. Int Ophthalmol. 2018;38(5):2239–2246.
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Kishi S, Matsumoto H. A new insight into pachychoroid diseases: Remodeling of choroidal vasculature. Graefes Arch Clin Exp Ophthalmol. 2022;260:3405–3417.
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B Brown R, Mohan S, Chhablani J. Pachychoroid Spectrum Disorders: An Updated Review. J Ophthalmic Vis Res. 2023 Apr 19;18(2):212-229.
Learning Objectives:
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Understand the multimodal imaging features associated with pachychoroid pigment epitheliopathy.
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Understand the different disease entities in the pachychoroid disease spectrum.
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