Foveal Hypoplasia: Diagnosis Using Optical Coherence Tomography Angiography

Sánchez-Vicente, J.L.; Contreras-Díaz, M.; Llerena-Manzorro, L.; Rueda, T. Rueda; López-Herrero, F.; Molina-Socola, F.E.; Muñoz-Morales, A.; Rúa-Franch, Enrique Rodríguez de la

doi:10.1097/icb.0000000000000440

Cited by 9 publications

(5 citation statements)

References 7 publications

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“… 7 The absent FAZ of both the SVC and DVC are also similar findings seen in other etiologies of foveal hypoplasia. 8 , 9 , 10 In a small sample of foveal hypoplasia subjects, Pakzad-Vaezi et al 9 observed that the FAZ's in the DVC of eyes with albinism were less developed than both normal eyes and eyes with idiopathic foveal hypoplasia. They also noted that the eyes with albinism tended to have worse visual acuity compared to eyes with idiopathic foveal hypoplasia and raised the question as to whether this was due in part to a less developed FAZ.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic correlation of aniridia: Optical coherence tomography angiography and histopathologic observations

Therattil

Naranjo

Hsu

et al. 2020

American Journal of Ophthalmology Case Reports

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Purpose To describe optical coherence tomography angiography (OCTA) findings in a patient with aniridia and correlate with representative histopathology. Observations OCTA images of the macula of a pediatric aniridic patient, who has nystagmus and impaired vision bilaterally, demonstrate a complete absence of the foveal avascular zone (FAZ) in both the superficial and deep vascular complexes (SVC and DVC). In addition, larger superficial blood vessels were found to be abnormally diving from the SVC into the DVC. Similarly, immunofluorescence with confocal microscopy imaging of a retinal histopathology specimen from a 2 month old aniridic patient demonstrated larger vessels diving in the same manner. Conclusions and importance This study highlights the clinical, imaging and histopathologic findings of aniridia. Supine OCTA imaging, performed during examination under anesthesia, allowed for visualization of retinal microvasculature in eyes with nystagmus. The histopathology images helped validate OCTA findings that, with further investigation, may lead to new information about the development of abnormal retinal microvasculature.

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Section: Discussionmentioning

confidence: 99%

Clinicopathologic correlation of aniridia: Optical coherence tomography angiography and histopathologic observations

Therattil

Naranjo

Hsu

et al. 2020

American Journal of Ophthalmology Case Reports

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“…OCT angiography is a simple, fast and non-invasive method for imaging the retinal blood vessels in the macular area. In foveal hypoplasia, it can reveal the lack of a foveal avascular zone in the superficial and deep capillary layers (Pakzad-Vaezi et al, 2017;Sánchez-Vicente et al, 2018). In our case, the patient had the absence of a foveal avascular zone in both eyes.…”

Section: Discussionmentioning

confidence: 50%

Isolated Foveal Hypoplasia: A Case Report

Ivanovska Adjievska,

Buckoska

2023

MEDIS

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Foveal hypoplasia is a retinal disorder in which the foveal pit of the macula lutea is incompletely developed and is characterized by nystagmus and low visual acuity. It can manifest itself in isolation, without a clear etiology, or associated with other conditions such as albinism, aniridia, Stickler Syndrome, optic nerve hypoplasia, microphtalmus, etc. Modern retinal imaging technologies are key to diagnosis of this rare retinal disorder. We present a case of a 19-year-old woman with poor distance and near vision and nystagmus since childhood, without other associated diseases. Visual acuity was 0.5 Snellen decimal units in both eyes, with present latent nystagmus. The anterior segment was normal, while an absent foveal reflex was found on fundus examination. Optical coherence tomography (OCT) imaging confirmed the absence of foveal depression in the macular area. OCT-angiography (OCTA) was performed, which confirmed the absence of a foveal avascular zone in the macula. According to the proposed grading system parameters for foveal hypoplasia, this case corresponds with the most severe (grade 4) degree of foveal hypoplasia. Foveal hypoplasia can be associated with numerous etiological factors, and visual acuity can vary depending on the development of foveal photoreceptors and structural gradation. For this reason, especially in children with reduced visual acuity of unknown etiology and the existence of nystagmus, it is recommended to perform additional examinations and use multimodal imaging techniques (OCT and OCTA) in order to make a timely and accurate diagnosis. Management of this disorder includes treatment of the associated ocular and systemic conditions, refractive correction, treatment of amblyopia and use of low vision aids.

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“…Our patient had no foveal pit, with persistence of all the inner retinal layers and outer plexiform layer, outer nuclear layer widening, and outer segment lengthening in the foveolar area, which makes it a grade 2 with a visual acuity of 20/32 according to Thomas et al [2]. FH and FH are commonly bilateral [4–6]. In our case, the differential diagnosis includes an epiretinal membrane, which may simulate the absence of the foveal pit [7].…”

Section: Discussionmentioning

confidence: 85%

Fovea Plana in a 9-Year-Old Boy Presenting with Decreased Vision in the Left Eye

et al. 2019

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Patient: Male, 9 Final Diagnosis: Fovea plana Symptoms: Visual acuity loss Medication: — Clinical Procedure: — Specialty: Ophthalmology Objective: Congenital defects/diseases Background: Foveal hypoplasia (FH) is a congenital disorder, generally associated with other conditions. Case Report: A 9-year-old boy presented with moderately decreased vision in the left eye. Fundus examination showed an absence of macular reflection and no foveal pit was seen on optical coherence tomography. Fluorescein angiography demonstrated the absence of a foveal avascular zone. Conclusions: This is a rare case of a unilateral fovea plana associated with a visual impairment.

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Foveal Hypoplasia: Diagnosis Using Optical Coherence Tomography Angiography

Cited by 9 publications

References 7 publications

Clinicopathologic correlation of aniridia: Optical coherence tomography angiography and histopathologic observations

Clinicopathologic correlation of aniridia: Optical coherence tomography angiography and histopathologic observations

Isolated Foveal Hypoplasia: A Case Report

Fovea Plana in a 9-Year-Old Boy Presenting with Decreased Vision in the Left Eye

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