An unusual presentation of peripapillary pachychoroid syndrome

Hubschman, Sasha; Hou, Kirk; Sarraf, David; Tsui, Irena

doi:10.1016/j.ajoc.2022.101338

Cited by 6 publications

(7 citation statements)

References 16 publications

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“…Peripapillary pachychoroid syndrome (PPS) is a relatively new addition to the pachychoroid spectrum, with few cases reported overall. [ 55 ] In PPS, patients demonstrate intra or subretinal fluid in the region nasal to the macula and choroidal thickening near the optic disk, rather than the fovea. [ 3 , 56 ] The disease tends to occur in elderly males [ 1 , 56 , 57 , 58 , 59 ] and may present bilaterally.…”

Section: Resultsmentioning

confidence: 99%

“…Some authors have speculated that PPS may have a similar pathogenesis as CSCR, wherein papillary choroidal congestion results in RPE dysfunction and subretinal fluid accumulation. [ 55 , 56 ] Some have proposed that fluid from the nasal choroid may leak into the retinal space via membrane defects in the external limiting membrane (ELM), [ 55 ] while others purport that the peripapillary fluid pocket signifies the fluid entry site from the choroidal vasculature into the retina. [ 60 ] Recent evidence has led authors to theorize that anastomotic connections may also play a role in PPS pathogenesis.…”

Section: Resultsmentioning

confidence: 99%

“…[ 60 ] Recent evidence has led authors to theorize that anastomotic connections may also play a role in PPS pathogenesis. [ 43 , 55 ] Peripapillary anastomoses, in combination with the lack of RPE around the optic nerve head, may allow for the direct transmission of choroidal hydrostatic pressure to the inner retinal layers in this region. [ 57 ]…”

Section: Resultsmentioning

confidence: 99%

“…Treatment efficacy for PPS varies. Recent cases have resolved spontaneously [ 55 ] or with PDT laser. [ 60 ] A case of a bilateral PPS, initially treated with dorzolamide eye drops and eplerenone tablets, ultimately achieved retinal fluid resolution and visual acuity improvement after treatment with dexamethasone drops.…”

Section: Resultsmentioning

confidence: 99%

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Pachychoroid Spectrum Disorders: An Updated Review

Brown

Mohan

Chhablani

2023

JOVR

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Pachychoroid disease spectrum is a recent term that has been associated with an increasing number of phenotypes. This review discusses updated findings for each of the typical pachychoroid entities (central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, peripapillary pachychoroid syndrome, and focal choroidal excavation), as well as two relatively new additions (peripapillary pachychoroid neovasculopathy and peripheral exudative hemorrhagic chorioretinopathy). Here, we discuss the potential pathogenic mechanisms for these diseases and relevant imaging updates. Finally, we argue for a consistent classification scheme for these entities.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

See 2 more Smart Citations

Pachychoroid Spectrum Disorders: An Updated Review

Brown

Mohan

Chhablani

2023

JOVR

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“…A recent study by Iovino et al [ 8 ] supports the use of PDT for PPS since in their cohort, best-corrected VA significantly improved from baseline to month 3. On the other hand, in a recent PPS case report, it is emphasized that CME in PPS may show significant fluctuations and sometimes observation should be the initial approach especially if vision is not considerably affected [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity

Bouzika

Georgalas

Sotirianakou

et al. 2022

Case Reports in Ophthalmological Medicine

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Two cases with peripapillary pachychoroid syndrome (PPS) along with the challenges concerning correct diagnosis and treatment are presented. In the first case, the patient presented with painless unilateral gradual visual loss. Fundoscopy and optical coherence tomography (OCT) revealed cystoid macular edema (CME) in the left eye (LE), extending from the temporal optic disc margin towards the fovea, with no additional findings. Enhanced-depth imaging- (EDI-) OCT provided additional information and increased choroidal thickness nasally to the macula and pachyvessels in the outer choroidal layer, findings supportive of PPS. Photodynamic therapy (PDT) was applied at the leakage sites. Two months later, CME and subretinal fluid (SRF) had resolved, and VA had significantly improved. In the second case, a patient presented with reduced vision and metamorphopsia bilaterally over the previous 5 days. Fundoscopy revealed CME in both eyes. OCT confirmed the presence of CME in the papillomacular area in the right eye; similarly, CME was recorded in the macula of the LE with SRF located subfoveally. EDI-OCT showed increased choroidal thickness in both eyes. Treatment was administered, originally with dorzolamide eye drops along with eplerenone tablets, and then dexamethasone eye drops that eventually led to significant anatomic and functional improvement. It is important for ophthalmologists to be able to recognize the unique clinical entity of PPS, as its resemblance to disorders with similar features may lead to misdiagnoses and unnecessary, or even incorrect, interventions.

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