Congenital Retinal Arterial Macrovessel and Congenital Hamartoma of the Retinal Pigment Epithelium

Souissi, K.; Afrit, M.A. El; Kraiem, Abdelhafidh

doi:10.3928/01913913-20060301-10

Cited by 10 publications

(3 citation statements)

References 2 publications

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“…Of the 38 articles in the literature, only 4 RVMs originating from a retinal artery are reported. [18][19][20][21][22][23][24] However, none of these 4 cases included an FA study to confirm the true arterial nature. Fluorescein angiography was available in 40 eyes (82%) in our series, and the venous origin of the RVM was confirmed in all cases (Figure 2).…”

Section: Discussionmentioning

confidence: 99%

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Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain

et al. 2018

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IMPORTANCE Congenital retinal macrovessel (CRM) is a rarely reported venous malformation of the retina that is associated with venous anomalies of the brain.OBJECTIVE To study the multimodal imaging findings of a series of eyes with congenital retinal macrovessel and describe the systemic associations. DESIGN, SETTING, AND PARTICIPANTSIn this cross-sectional multicenter study, medical records were retrospectively reviewed from 7 different retina clinics worldwide over a 10-year period (2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017). Patients with CRM, defined as an abnormal, large, macular vessel with a vascular distribution above and below the horizontal raphe, were identified. Data were analyzed from December 2016 to August 2017.MAIN OUTCOMES AND MEASURES Clinical information and multimodal retinal imaging findings were collected and studied. Pertinent systemic information, including brain magnetic resonance imaging findings, was also noted if available. RESULTSOf the 49 included patients, 32 (65%) were female, and the mean (SD) age at onset was 44.0 (20.9) years. A total of 49 eyes from 49 patients were studied. Macrovessel was unilateral in all patients. Color fundus photography illustrated a large aberrant dilated and tortuous retinal vein in all patients. Early-phase frames of fluorescein angiography further confirmed the venous nature of the macrovessel in 40 of 40 eyes. Optical coherence tomography angiography, available in 17 eyes (35%), displayed microvascular capillary abnormalities around the CRM, which were more evident in the deep capillary plexus. Of the 49 patients with CRM, 39 (80%) did not illustrate any evidence of ophthalmic complications. Ten patients (20%) presented with retinal complications, typically an incidental association with CRM. Twelve patients (24%) were noted to have venous malformations of the brain with associated magnetic resonance imaging. Of these, location of the venous anomaly in the brain was ipsilateral to the CRM in 10 patients (83%) and contralateral in 2 patients (17%), mainly located in the frontal lobe in 9 patients (75%). CONCLUSIONS AND RELEVANCEOur study has identified an association between macrovessels in the retina and venous anomalies of the brain (24% compared with 0.2% to 6.0% in the normal population). Thus, we recommend new guidelines for the systemic workup of patients with CRM to include brain magnetic resonance imaging with contrast. These lesions may be more accurately referred to as retinal venous malformations, which may raise awareness regarding potential cerebral associations.

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Section: Discussionmentioning

confidence: 99%

“…An RVM is in fact usually a retinal vein and rarely an artery. Of the 38 articles in the literature, only 4 RVMs originating from a retinal artery are reported . However, none of these 4 cases included an FA study to confirm the true arterial nature.…”

Section: Discussionmentioning

confidence: 99%

Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain

et al. 2018

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“…Furthermore, CSHRPE has been reported as a coincidental finding alongside Coat’s disease or the presence of a congenital retinal arterial macrovessel crossing the macular region in the same eye [ 12 - 13 ]. Despite these associations, the precise relationship between these conditions remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Multifocal Congenital Simple Hamartoma of the Retinal Pigment Epithelium: A Multimodal Imaging Case Study

Khadamy,

Elmi Sadr

2024

Cureus

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Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare benign tumor often detected incidentally during routine eye exams. We present a case of multifocal CSHRPE in a 32-year-old Hispanic woman, emphasizing the diagnostic challenges posed by its presentation and the pivotal role of multimodal imaging in accurate diagnosis. Despite initial difficulties due to a history of trauma and pigmented fundus, advanced imaging techniques, including optical coherence tomography (OCT), OCT angiography (OCTA), fluorescein angiography (FA), and indocyanine green angiography (ICGA), facilitated a precise diagnosis. Notably, OCTA revealed high signal intensity and flow at the largest nodule site while FA and ICGA exhibited characteristic blockage patterns. Moreover, smaller nodules exhibited OCT findings supporting the theory of islands of retinal pigment epithelium (RPE) cells proliferating ectopically within the retina. Our case underscores the importance of comprehensive imaging assessment in distinguishing CSHRPE from other lesions, contributing to a deeper understanding of this rare ocular condition.

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Congenital retinal macrovessel and foveal dysplasia of retinopathy of prematurity

et al. 2009

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LETTERS 277 cortex of hypermature lenses than in immature lenses. 4 In the case presented here, we speculated that the accumulated lobules of LEP embedded the retained lens material and kept it from breaking into small pieces, releasing soluble lens protein, and avoiding strong chemotactic activity for monocytes. 5 As time went by, the lens protein denatured to plentiful HMWP, and abundant macrophages laden with digested lens material fi nally led to the glaucoma.As for the right eye, the Soemmering's ring cataract had a hybrid content of retained lens material and LEP. It was diffi cult to eradicate the content only without also removing the capsule. Although en bloc excision of the after-cataract and simple PPV were performed, some complications still occurred, such as retained fragments, glaucoma, and endothelial injury. Fortunately, the second operation led to good results, including visual improvement. However, the left eye revealed no visual improvement owing to the long duration of elevated IOP.From this case, we learned that lens material-induced glaucoma might occur 25 years after cataract extraction. With fi ndings of severe LEP and aqueous cytology, radical and immediate surgical intervention should be performed to eliminate further visual loss.

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Congenital Retinal Arterial Macrovessel and Congenital Hamartoma of the Retinal Pigment Epithelium

Cited by 10 publications

References 2 publications

Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain

Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain

Multifocal Congenital Simple Hamartoma of the Retinal Pigment Epithelium: A Multimodal Imaging Case Study

Congenital retinal macrovessel and foveal dysplasia of retinopathy of prematurity

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