Posterior polar annular choroidal dystrophy

Gala, Jignesh Manshibhai; Narayanan, Raja

doi:10.1136/bcr-2019-230495

Cited by 4 publications

(23 citation statements)

References 5 publications

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“…Patients present with similar findings; however, the morphology of the atrophy seen with PPACD is oriented in a ring-like fashion around the macula, although some argue the 2 disease processes should be considered a single entity. 2,[4][5][6] In addition, the appearance of the fundus and the clinical presentation have some similarities to RP, more specifically sectoral RP. We believe that the electroretinogram findings, clinical examination, and multimodal imaging findings help exclude the diagnosis of sectoral RP.…”

Section: Resultsmentioning

confidence: 99%

Posterior Polar Hemispheric Choroidal Dystrophy: Case Report and Presentation of Genetic Screening

Tabbaa

Daroszewski

Sobol

2022

Journal of VitreoRetinal Diseases

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Purpose: Posterior polar hemispheric choroidal dystrophy is a rare dystrophy affecting the choroid and retina with a characteristic clinical appearance that distinguishes it from a broad variety of other retinal diseases. The disease process has a morphology described in the literature that preferentially affects the outer macula and spares the fovea with no arteriolar attenuation or optic nerve pallor. Methods: This case report illustrates the use of multimodal retinal imaging, visual field testing, electroretinogram, and genetic testing of a patient who we believe fits the clinical pattern established by previous studies describing this condition. Results: Fundus imaging in addition to further imaging modalities such as fluorescein angiography helped further characterize this disease process and aided in the diagnosis. In addition, genetic testing revealed unique allele variants found in this patient. Conclusions: By taking a multifaceted approach to the diagnosis of retinal pathology, clinicians can make informed decisions regarding patient care.

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Section: Resultsmentioning

confidence: 99%

Posterior Polar Hemispheric Choroidal Dystrophy: Case Report and Presentation of Genetic Screening

Tabbaa

Daroszewski

Sobol

2022

Journal of VitreoRetinal Diseases

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“…PPACD has been described in fewer than 10 patients, including the one reported here . This report describes the development of posterior subcapsular cataract and retinal neovascularization.…”

Section: Discussionmentioning

confidence: 70%

“…Different electroretinographic responses have been described in PPACD. Rod-cone dysfunction has been described in 2 other patients, normal rod function with cone dysfunction was reported in 3 patients, and normal rod and cone function was described in 2 patients . In the last case, reduced cone photoreceptor density was observed by adaptive optics.…”

Section: Discussionmentioning

confidence: 97%

Retinal Neovascularization in Posterior Polar Annular Choroidal Dystrophy

2023

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“…To date, only a few cases of PPACD have been reported in the literature. 1 , 3 , 4 , 5 Of these, one case reported by Forte et al was followed up for long-term prognosis. 3 The aforementioned PPACD case involved a 32-year-old man who exhibited an annular pattern of retinal atrophy in both eyes, similar to that in our case.…”

Section: Discussionmentioning

confidence: 99%

“…Although not mentioned, previous reports have also revealed dilated choroidal large vessels in OCT images. 4 , 5 While the pathophysiology accounting for the dilation of choroidal large vessels remains unclear, the decreased choroidal blood flow velocity detected in LSFG indicated that the dilated choroidal vessels were consequence of the congestion of choroidal blood flow. It is possible that the choroidal blood vessels are dilated due to the atrophy of the surrounding tissues or vascular wall components per se in the atrophic lesions.…”

Section: Discussionmentioning

confidence: 99%