Congenital Retinal Macrovessel Research Articles

Congenital Retinal Macrovessel and Cavernous Hemangioma in Cowden Syndrome - A Case Report and Review of Literature.

To describe a case of unilateral congenital retinal macrovessel with a retinal cavernous hemangioma in a patient with Cowden syndrome (CS). In addition, we summarize previously reported cases of ocular findings in CS in the literature. Observational case report and literature review. A 45-year-old white female with Cowden syndrome presented for routine ocular examination. She had a history of thyroid carcinoma, fallopian para-tubal cyst, chromophobe type renal cell carcinoma, multiple benign skin lesions, and macrocephaly. At presentation, she was asymptomatic with good vision. On dilated fundus examination, the right eye revealed a congenital retinal macrovessel and retinal cavernous hemangioma. Previously performed genetic testing revealed a pathogenic c.46dupT mutation in the PTEN gene which was consistent with CS. Our patient had a history of multiple malignancies and hamartomas consistent with her genetic diagnosis of CS. We incidentally discovered a congenital retinal macrovessel with a retinal cavernous hemangioma. Patients with CS should be referred for interdisciplinary evaluation including routine ocular examinations.

Congenital Retinal Macrovessel. A Case Report of a Rare Incidental Finding

Congenital retinal macrovessel (CRM) is a rare vascular abnormality of the macular region that is usually discovered incidentally.We present the case of a 57-year-old Libyan female patient with a CRM.The patient's left eye showed an abnormally large retinal vein crossing the foveal avascular region, Optical coherence tomographic angiography (OCTA) showed a large retinal vessel in the left eye branching superiorly at the edge of the fovea a vascular zone. The patient has no visual defect or macular thickening.CRM is an incidental finding that, with rare exceptions, does not cause any alteration to the patient's vision. They can be imaged by OCTA and need to be differentiated from other retinal pathologies.

Spontaneous Resolution of Macular Edema from a Congenital Retinal Macrovessel

Spontaneous Resolution of Macular Edema from a Congenital Retinal Macrovessel

Congenital retinal macrovessel with intracranial venous malformation in a pediatric patient: a case report

Congenital retinal macrovessel with intracranial venous malformation in a pediatric patient: a case report

Multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma: a case report

BackgroundTo report the results of multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma.Case presentationA 52-year-old female patient presented with progressive vision loss in the right eye. BCVA was 8/20 in the right eye and 18/20 in the left eye. Fundus examination of the right eye showed an aberrant retinal macrovessel arising from the inferior temporal major vein. It crossed the foveal area and overstepped to the superior retina. A “brunch of grapes” shaped retinal lesions arised from the macrovessel. Fluorescein angiography showed saccular lesions that filled slowly during the venous phase and became brightly hyperfluorescent saccular caps. SS-OCT of the right eye revealed a highly back-scattering hyper-reflective vessel across the fovea with shadow effect and adhesions between the vitreous and the aberrant macrovessel. It also revealed hypo reflective saccules with hyperreflective borders located in the inner retina corresponding to the cavernous retinal hemangioma. Retinal pigment epithelium undulations and vascular dilations at the level of Haller’s layer were observed in both eyes. Indocyanine green angiography revealed chroidal vascular dilatations in both eyes in the late phase. OCT-A showed the aberrant vessel emerging from the inferior temporal vein and splitting the foveal avascular zone horizontally. RCH appeared as small heterogeneous saccular flow areas associated with focal capillary hypo perfusion areas. Asymmetry and distorsion of the foveal avascular zone were also noticed.A diagnosis of retinal macrovessel associated with a retinal cavernous hemangioma was made.ConclusionsCongenital retinal macrovessels and retinal cavernous hemangioma are benign lesions. Their association is rare. Abnormal vascular development is likely to be responsible for their association. Swept source OCT and OCT angiography may be of a great contribution to better evaluate these retinal vascular disorders.

A patient with congenital retinal macrovessel and prepapillary vascular loop together at the same eye

A patient with congenital retinal macrovessel and prepapillary vascular loop together at the same eye

Congenital Retinal Macrovessel Strays into Deep Capillary Plexus

Congenital Retinal Macrovessel Strays into Deep Capillary Plexus

OCT Angiography Showing Congenital Retinal Macrovessel

OCT Angiography Showing Congenital Retinal Macrovessel

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

Congenital retinal macrovessel (CRM) is a rarely reported venous malformation of the retina that is associated with venous anomalies of the brain. To study the multimodal imaging findings of a series of eyes with congenital retinal macrovessel and describe the systemic associations. In this cross-sectional multicenter study, medical records were retrospectively reviewed from 7 different retina clinics worldwide over a 10-year period (2007-2017). 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. 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. Of 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%). Our 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.

Three Cases of Congenital Retinal Macrovessel, One Coexisting with Cilioretinal Artery.

In this report, we describe three cases of retinal macrovessel. Two of the three patients presented to our clinic for annual eye exam and had no visual complaints. The third patient presented because of vision loss in the left eye. Two patients had 20/20 best corrected visual acuity in both eyes and the third patient had 20/20 in the right eye, 20/25 in the left eye. Pupillary exams were normal. Slit-lamp examinations of the anterior segment were unremarkable. Fundus examination revealed macrovessels in the left eyes of two patients and in the right eye of one patient. The patients underwent complete ophthalmological examinations including color fundus photography for all three patients and optic coherence tomography, fundus autoflorescence, and fundus fluorescein angiography for two of the patients. Cilioretinal artery coexisting with macrovessel was seen angiography in one case. Congenital retinal macrovessel is a rare vascular condition. It is often unilateral and the vessel is an aberrantly large branch of the retinal arteries or veins. They may cross the fovea and their visual impact is minimal. The coexistence of congenital retinal macrovessel and cilioretinal artery is very rare. Visual impairment may occur in congenital retinal macrovessel due to retinal cavernous hemangioma, foveal cysts, central serous retinopathy, and other retinal vascular abnormalities.

Congenital retinal macrovessel associated with retinal peripheral telangiectasia and retinal ischaemia

Abstract Clinical case The case is presented of a 16 year-old girl with unilateral congenital retinal macrovessel associated with peripheral telangiectasia and retinal ischaemia. Discussion Congenital retinal macrovessel is a rare finding. To the best of our knowledge this is the first report of this unusual presentation of congenital retinal macrovessel. After 21 months of follow up, visual acuity remained stable, and no complications developed.

Macrovaso retiniano asociado a telangiectasias periféricas retinianas e isquemia retiniana

Clinical caseThe case is presented of a 16 year-old girl with unilateral congenital retinal macrovessel associated with peripheral telangiectasia and retinal ischaemia. DiscussionCongenital retinal macrovessel is a rare finding. To the best of our knowledge this is the first report of this unusual presentation of congenital retinal macrovessel. After 21 months of follow up, visual acuity remained stable, and no complications developed.

Multimodal Imaging Analysis in a Case with Congenital Fovea-Involving Retinal Macrovessel and Excellent Visual Acuity

Purpose Congenital retinal macrovessels (CRM) represent rare aberrant vasculature of the retinal vessels that can supply or drain the macula. In this report, the optical coherence tomography angiography features of a congenital retinal macrovessel are discussed. Methods The history and examination findings are presented alongside swept-source OCT angiography with corresponding B scan and en face OCT imaging. Patients The case is a 12-year-old female patient with excellent best-corrected visual acuity in both eyes. Results Swept-source OCT angiography demonstrated considerable loss of the foveal avascular zone at the levels of the superficial and deep capillary plexus. Discussion In this case there was no detrimental effect on vision despite anatomical loss of the foveal avascular zone.

Congenital retinal macrovessel associated with vitreous hemorrhage

A "congenital retinal macrovessel" (CRM) is an aberrant retinal vessel (frequently a vein) that traverses the central macula and supplies or drains both above and below the horizontal raphe. It is an uncommon entity that is usually disclosed on routine examination and may rarely cause a compromised visual acuity. We describe the case of an adolescent girl who underwent vitrectomy for vitreous hemorrhage and was found to have a CRM with vascular abnormalities. To our knowledge, this is the first report of vitreous hemorrhage secondary to a CRM.

Congenital Retinal Macrovessel: a Case Report

We report a rare clinical case of a 66-year-old Caucasian male with slightly decreased vision in his left eye. After comprehensive ophthalmic examination and multimodal imaging with fundus photography, spectral domain optical coherence tomography (OCT), fluorescein angiography (FA), and a 30-2 visual field tests, he was diagnosed with congenital retinal macrovessel crossing the fovea avascular zone in his left eye. His right eye is unremarkable on ophthalmic exams. Congenital retinal macrovessel refers to an aberrant vessel, usually a vein, which transverse the macula and supplies both sides of the horizontal raphe. Various retinal abnormalities or clinical complications have been reported in association with congenital retinal macrovessel, such as cystoid macular edema, fovea cysts, macular exudates, hemorrhage, serous retinal detachment and branch vein occlusion. In our patient, there were not significant clinical complications. The reduction in the vision of the left eye could be explained by irregularity and slight enlargement of the foveal avascular zone (FAZ). In addition, patient's congenital macrovessel may have caused a relative angioscotoma, which could also affect the visual acuity (VA).

Congenital retinal macrovessel with macular edema: An unusual presentation

Congenital retinal macrovessel (CRM) is a rare developmental condition in which a large vein, artery, or occasionally an artery and a vein together, cross the macular region. Most often, this aberrant vessel originates from a branch of the inferotemporal vein, with branches crossing the horizontal raphe. CRMs do not affect vision unless there are associated foveolar cysts, foveal ectopia, pigmentary changes at fovea, macular hemorrhage, exudates, serous macular detachment, or the macrovessel crosses the fovea. We present a case of a 13-year-old male patient with congenital macular artery crossing the fovea, associated with macular edema and decreased visual acuity. In this case report, we highlight the fundus fluorescein angiography findings and optical coherence tomography evaluation of the patient.

Congenital Retinal Macrovessel with Normal Visual Acuity: A Case Report

Congenital retinal macrovessel is a large aberrant vessel discovered as an incidental finding. These aberrant vessels are mostly single, unilateral veins. CRMs do not affect vision unless there are associated foveolar cysts, foveal ectopia, pigmentary changes at fovea, macular haemorrhage, exudates, serous macular detachment or the macrovessel crosses the fovea. Thorough clinical evaluation and investigations are valuable in diagnosis and follow-up of such cases and aid in timely management of associated complications. In this report, we have discussed a case of CRM with unaffected visual acuity. To the best of our knowledge, such a case of CRM crossing fovea but sparing vision and findings substantiated by Spectral Domain Optical Coherence Tomography (OCT) has not been documented earlier.

Intravitreal bevacizumab in congenital retinal macrovessel with retinal arteriolar macroaneurysm

Congenital retinal macrovessel (CRM) refers to an aberrant vessel, usually a vein, which traverses the macula and supplies both sides of the horizontal raphe. It is a rare condition, mostly asymptomatic and discovered on routine examination. We describe a case of both arterial and venous CRM with a macroaneurysm along the arterial CRM that presented with decreased vision due to prominent lipid exudation at the macula. Treatment with intravitreal bevacizumab resulted in a favourable anatomical as well as functional outcome. To the best of our knowledge, this is the first report of this unusual presentation of CRM, and its successful management with intravitreal bevacizumab.

Congenital retinal macrovessel: An atypical presentation with low vision and macular thickening

Case reportWe report a case of a 35-year-old male patient with no medical history, who experienced decreased vision in his left eye that he noticed by chance. After a complete ophthalmic examination, he was diagnosed with congenital retinal macrovessel with macular thickening. DiscussionCongenital retinal macrovessels are rare vascular anomalies, in which the diagnosis is usually incidental as their visual impact is minimal. In the rare cases where there is a significant visual impairment, this is due to macular hemorrhages, foveal cysts, serous macular detachment, or the course of the vessel itself through the foveal avascular zone.

Macrovaso congénito retiniano: presentación atípica con baja visión y engrosamiento macular

Case reportWe report a case of a 35 year old male patient with no medical history, who experienced decreased vision in his left eye that he noticed by chance. After a complete ophthalmic examination, he was diagnosed with congenital retinal macrovessel with macular thickening. DiscussionCongenital retinal macrovessels are rare vascular anomalies, in which the diagnosis is usually incidental as their visual impact is minimal. In the rare cases where there is a significant visual impairment, this is due to macular hemorrhages, foveal cysts, serous macular detachment, or the course of the vessel itself through the foveal avascular zone.