Photo Essay

Pigmentary Retinopathy and Chronic Subretinal Fluid
Associated with Pseudoxanthoma Elasticum and

Angioid Streaks

Matthew R. Starr, MD; Sophie J. Bakri, MD

Mayo Clinic Department of Ophthalmology, Rochester, MN, USA

J Ophthalmic Vis Res 2022; 17 (1): 152–155

PRESENTATION

We present the ophthalmic findings of a 57-year-
old female with pseudoxanthoma elasticum who
was initially diagnosed in her 20s after a biopsy
of abnormal neck lesions. She began to develop
visual problems decades later, but currently has
no other related medical problems, including any
evidence of coronary artery disease. Our patient
complained of bilateral metamorphopsia but with
preserved visual acuity of 20/25 in both eyes.
In addition to the angioid streaks, the patient
also had evidence of pigmentary changes at the
posterior pole [Figures 1A–1B]. Optical coherence
tomography imaging [Figures 1C–1F] revealed the
presence of bilateral subretinal fluid, shaggy
photoreceptors, and intraretinal hyperreflective
material concentrated within the outer retina that
was stable for five years [Figures 1 E & 1F].

DISCUSSION

Angioid streaks are breaks within a weakened
Bruch’s membrane. They are deep to the

Correspondence to:

Sophie J. Bakri, MD. Mayo Clinic, Department of
Ophthalmology, 200 First St, Southwest, Rochester
55905, MN, USA.
E-mail: bakri.sophie@mayo.edu
Received 23-07-2019; Accepted 02-04-2021

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Website: https://knepublishing.com/index.php/JOVR

DOI: 10.18502/jovr.v17i1.10183

neurosensory retina and typically bilateral,
irregular, and emanate from the optic disc.
Causes include Ehler–Danlos syndrome, Paget’s
disease, sickle cell, and other hemoglobinopathies,
idiopathic, and pseudoxanthoma elasticum.[1]
Vision is typically not affected by angioid
streaks unless patients develop choroidal
neovascularization with resultant macular edema
and retinal hemorrhages.[2] Following our patient
for 10 years (5 years with spectral domain OCT),
she has never had any evidence of a choroidal
neovascular complex on fluorescein angiography
and thus it was concluded that the subretinal fluid
was the result of malfunctioning retinal pigment
epithelium due to the pigmentary changes at the
posterior pole, a rare finding first described by
Zweifel and colleagues in 2011, who concluded
the fluid was due to a similar pathophysiology as
pattern dystrophy.[3] Clinicians should be aware of
the ophthalmic manifestations of pseudoxanthoma
elasticum which include angioid streaks which can
lead to choroidal neovascularization and severe
vision loss but also pigmentary changes at the
posterior pole that can lead to chronic subretinal
fluid and a milder effect on visual acuity.

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How to cite this article: Starr and Bakri. Pigmentary Retinopathy and
Chronic Subretinal Fluid Associated with Pseudoxanthoma Elasticum and
Angioid Streaks . J Ophthalmic Vis Res 2022;17:152–155.

152 © 2022 Starr, Bakri. THIS IS AN OPEN ACCESS ARTICLE DISTRIBUTED UNDER THE CREATIVE COMMONS ATTRIBUTION LICENSE | PUBLISHED BY KNOWLEDGE E

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Photo Essay; Starr, Bakri

Figure 1. Fundus images (A) with bilateral hyperpigmented lines emanating from the discs, consistent with angioid streaks (white
arrows). On FAF (B), the angioid streaks are easily appreciated (white arrows). Also seen are punctate areas of hyper- and hypo-
FAF surrounding the disc and within the fovea (asterisks). The hyper-FAF lesions are felt to be accumulations of photoreceptor
lipofuscin while the hypo-FAF lesions are thought to be related to subretinal drusenoid deposits. On OCT (C-F), there is evidence
of bilateral subretinal fluid (red arrows) consistent with chronic RPE damage that are stable over five years (C & D images taken
five years before E & F images).

JOURNAL OF OPHTHALMIC AND VISION RESEARCH Volume 17, Issue 1, January-March 2022 153



Photo Essay; Starr, Bakri

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

REFERENCES

1. Clarkson JG, Altman RD. Angioid streaks. Surv Ophthalmol
1982;26:235–246.

2. Nakagawa S, Yamashiro K, Tsujikawa A, Otani A,
Tamura H, Ooto S, et al. The time course changes of
choroidal neovascularization in angioid streaks. Retina
2013;33:825–833.

3. Zweifel SA, Imamura Y, Freund KB, Spaide RF. Multimodal
fundus imaging of pseudoxanthoma elasticum. Retina
2011;31:482–491.

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