Retinal Vascular Tortuosity Index Change after Idiopathic Epiretinal Membrane Surgery: Does Internal Limiting Membrane Peeling Affect Retinal Vascular Tortuosity?

BACKGROUND: Idiopathic epiretinal membrane (iERM) surgery is one of the most commonly performed vitreoretinal surgeries, and the issue of internal limiting membrane (ILM) peeling in ERM surgery is still controversial. The aims of this study are to evaluate the changes in retinal vascular tortuosity index (RVTI) after pars plana vitrectomy for the iERM using optical coherence tomography angiography (OCTA) and to assess whether ILM peeling has an additional effect on RVTI reduction. METHODS: This study included25 eyes of 25 iERM patients who underwent ERM surgery. The ERM was removed without ILM peeling in 10 eyes (40.0%), and the ILM was peeled in addition to the ERM in 15 eyes (60.0%). The existence of the ILM after ERM peeling was checked with second staining in all eyes. Best corrected visual acuity (BCVA) and 6 × 6 mm en-face OCTA images were recorded before surgery and at the first month postoperatively. A skeleton model of the retinal vascular structure was created following Otsu binarization of en-face OCTA images using ImageJ software (1.52U). RVTI was calculated as the ratio of each vessel length to its Euclidean distance on the skeleton model using the Analyze Skeleton plug-in. RESULTS: The mean RVTI declined from 1.220 ± 0.017 to 1.201 ± 0.020 (p = 0.036) in eyes with ILM peeling and from 1.230 ± 0.038 to 1.195 ± 0.024 in eyes without ILM peeling (p = 0.037). There was no difference between the groups in terms of postoperative RVTI (p = 0.494). A statistically significant correlation was found between postoperative RVTI and postoperative BCVA (rho = 0.408, p = 0.043). CONCLUSIONS: The RVTI is an indirect indicator of the traction created by the iERM on retinal microvascular structures, and it was effectively reduced after iERM surgery. The postoperative RVTIs were similar in cases who underwent iERM surgery with or without ILM peeling. Therefore, ILM peeling may not have an additive effect on the loosening of microvascular traction and thus may be reserved for recurrent ERM surgeries.

A case series from a single family of familial retinal arteriolar tortuosity with common history of sudden visual loss.

PURPOSE: To report family members with familial retinal arteriolar tortuosity (FRAT) identified after sudden visual loss. OBSERVATIONS: A 15-year-old boy had sudden visual loss in his left eye while playing on a horizontal bar. He was referred to Nagoya City University Hospital from an eye clinic. The ophthalmologic examination showed retinal hemorrhage bilaterally. His best-corrected visual acuity (BCVA) was 20/17 in the right eye and 20/67 in the left eye. Bilateral retinal arteriolar tortuosity as well as retinal hemorrhage was seen. Since his mother with 54 years of age also had a history of retinal hemorrhage that improved spontaneously, fundus examination was performed, revealing tortuosity of the retinal arterioles. Consequently, the patient and his mother were diagnosed as FRAT. He was followed without intervention. Retinal hemorrhage gradually decreased and resolved after 3 months. The BCVA of his left eye gradually improved and reached 20/20 after 1 year. CONCLUSIONS AND IMPORTANCE: In this case, the family history was very useful for early diagnosis. Immediate and accurate diagnosis allowed the patient to be followed without intervention and achieve subsequent resolution of retinal hemorrhage and improved vision. FRAT should be considered in cases of sub-internal limiting membrane hemorrhages in young patients even in the presence of discrete retinal arteriolar tortuosity.

Acute Macular Neuroretinopathy in a Patient with Retinal Vascular Tortuosity.

We report a rare case of a young woman with acute macular neuroretinopathy (AMN) in the right eye and concomitant retinal vascular tortuosity in both eyes. A 19-years-old woman presented with a sudden loss of central vision in the right eye. Apart from flu-like infection 2 weeks before the onset of symptoms, she reported overall good health. She used oral contraceptive pills. Multimodal imaging techniques including color fundus photography, fundus autofluorescence, infrared reflectance imaging, fluorescein angiography, swept-source optical coherence tomography (SS-OCT), and visual field assessment were used for the diagnosis of AMN as well as disease monitoring during follow-up. At presentation, ophthalmoscopy revealed a reddish parafoveal lesion, while SS-OCT showed hyper-reflectivity in the outer plexiform and outer nuclear layers with a slightly disrupted inner segment/outer segment junction. All these imaging findings indicated AMN, but the interpretation was slightly difficult due to the presence of tortuous retinal arteries in both eyes. During the disease course, functional and morphological recovery was documented at 1- and 6-month follow-up. However, as the abnormal appearance of the retinal vessels did not change, congenital retinal vascular tortuosity was diagnosed. Since the pathogenesis of AMN has not been fully elucidated, there is currently no effective treatment. Numerous studies have emphasized a vascular origin and the key role of ischemia in AMN. Our rare case suggests that congenital tortuosity of the retinal vessels, although constituting a common finding in healthy individuals, may be involved in the pathophysiology of the disease.

[Age-related characteristics of retinal vessels in aging (literature review).]

The changes of retinal vascular caliber and microvascular topography reflect the cumulative response to ageing, the influence of cardiovascular risk factors, inflammation, endothelial dysfunction. With objective to perform a systematic review of the studies which evaluate retinal vessels in ageing, we conducted the search of published reports (2003-2020). The review provided the evidence of narrowing of the caliber of retinal arterioles and venules in ageing; inverse relationship has been found in ethnically heterogeneous populations in a wide age range from 4th to 9th decade. The age dynamics of arteriovenous ratio (AVR) is evaluated less consistently. The available data showed the loss of complexity of the retinal microcirculatory bloodstream in elderly age, which might lead to a decrease in functional activity of microcirculation; however the studies are limited for systematic conclusions. The large population studies in Russia on this topic are practically absent. The researches of the microvascular retinal bloodstream in aging using the automatic analysis of the modern range of indicators, are relevant in the Russian population and will provide new data.

Cerebral cavernous malformation type 1 with retinal blood vessel tortuosity and KRIT1 gene mutation.

Cerebral cavernous malformations (CCMs) represent a relatively rare and heterogeneous clinical entity with mutations identified in three genes. Both sporadic and familial forms have been reported. We present a young female patient with episodic paresthesia and headaches, but without acute neurological deficits. Her mother had a hemorrhaged cavernoma surgically removed 21 years ago. Cranial magnetic resonance imaging revealed multiple cavernous malformations in the size of a few millimeters and the ophthalmologic exam detected retinal blood vessel tortuosity in the proband. Targeted exome sequencing analysis identified a nonsense mutation in exon 16 of the KRIT1 gene, which resulted in a premature stop codon and a truncated protein underlying the abnormal development of cerebral and retinal blood vessels. This mutation with pathogenic significance has been reported before. Our case points to the importance of a thorough clinical and molecular work up despite the uncertain neurological complaints, since life style recommendations, imaging monitoring and genetic counseling may have major significance in the long term health of the patient.

Retinal vascular tortuosity in schizophrenia and bipolar disorder.

The micro-vasculature of retina and brain share common morphological, physiological, and pathological properties. Retina being easily accessible, retinal vascular examination provides an indirect assessment of cerebral vasculature. Considering the high prevalence of vascular morbidity in SCZ and BD a few studies have examined retinal vascular caliber and have reported increased retinal venular caliber in schizophrenia (SCZ). Retinal vascular tortuosity could serve as a better structural measure than caliber as it is static and less susceptible to pulse period variations. However, to date, no study has examined retinal vascular tortuosity in SCZ and bipolar disorder (BD). Hence, we examined retinal vascular tortuosity in comparison with healthy volunteers (HV). We included 255 subjects (78 HV, 79 SCZ, and 86 BD) in the age range of 18 to 50 years. Trained personnel acquired images using a non-mydriatic fundus camera. To measure the average retinal arteriolar tortuosity index (RATI) and retinal venular tortuosity index (RVTI), we used a previously validated, semi-automatic algorithm. The results showed significant differences across the three groups in RATI but not in RVTI; both BD and SCZ had significantly increased RATI compared to HV. There was also a significant difference between SCZ and BD, with BD having higher RATI. If shown to be of predictive utility in future longitudinal studies, it has the potential to identify patients at risk of development of adverse vascular events. As retinal vascular imaging is non-invasive and inexpensive, it could serve as a proxy marker and window to cerebral vasculature.

Multiple retinal hemorrhages in bilateral congenital venous tortuosity.

We present a case of a previously healthy 52-year-old woman with acute onset of scotomas in both eyes. It was associated with headache and vomiting for the past 1 week after a cold. The best-corrected visual acuity was 20/20 in both eyes. The examination of bilateral fundus revealed a venous tortuosity and mild dilatation in the posterior pole, with pre- and intraretinal hemorrhages. However, vasculatures were normally straight in the mid-peripheral and peripheral retina of both eyes. Hemorrhages showed complete resolution without treatment. There was no change in the appearance and shape of the vasculature. Ophthalmologists should be aware of congenital venous tortuosity as a possible cause of retinal hemorrhages.

Williams Syndrome with Ocular Manifestations

PURPOSE: To report two cases of Williams syndrome with ocular manifestations CASE SUMMARY: A 5-year-old boy who was diagnosed with Williams syndrome visited our hospital for ophthalmic examination. Best corrected visual acuity (BCVA) in both eyes was 0.6. He had myopic astigmatism and 8 prism diopters of esotropia. Oval-shaped pupil with a stellate pattern of the iris and increased retinal vascular tortuosity were seen in both eyes. Another case of an 8-year-old boy with Williams syndrome also had myopia in both eyes. BCVA was 0.7 in the right eye and 0.4 in the left eye. A stellate pattern of the iris and increased retinal vascular tortuosity were also seen in both eyes. CONCLUSIONS: Williams syndrome, deletion of 7q11.23, has ocular anomalies including a stellate pattern of the iris, refractive errors, amblyopia, and strabismus. Therefore, careful ophthalmic examination should be considered when children are diagnosed with Williams syndrome.

Síndrome de Bonnet-Wybur-Mason: dos polos de un espectro / The Bonnet-Wyburn-Mason syndrome: two poles of one spectrum

Se comunican los casos de dos pacientes con síndrome de Bonnet, Wyburn-Mason en quienes existía un aneurisma cirsoide de la retina de diferente grado de desarrollo. En uno, el cuadro oftalmoscópico era obvio; en tanto que en el otro la manisfestación fundamental era una tortuosidad vascular acentuada y en quien solo la angiografía fluoresceínica del fondo ocular mostró sutiles cambios compatibles con una malformación arteriovenosa localizada. Otro elemento inusual en el comportamiento de este tipo de malformaciones fue la asociación a una coartación de la aorta torácica y multiples anomalías esqueléticas sencillas, así como la obstrucción de un segmento muy desarrollado de la malformación arteriovenosa con infarto hemorrágico retiniano e involución posterior de parte de ella

We communicate the cases of two patients with syndrome of Bonnet, Wyburn-Mason who had cirsoide aneurysms of the retina of differnt degrees of development. In one, the ophthalmoscopic picture was obvious, while on the other, The primary manifestation was a marked vascular tortuosity and in which only the ocular fundus fluorescein angiography showed subtle changes consistent with a located arteriovenous malformation. Another unusual element in the behavior of this type of malformations was a coarctation of the thoracic aorta and multiple skeletal anomalies simple associated, as well as the obstruction of a highly developed portion of the retinal arteriovenous malformation with hemorrhagic infarction and consecutive involution of part of it

Retinal vascular tortuosity in obstructive sleep apnea.

PURPOSE: Endothelial dysfunction and vascular disease are common in obstructive sleep apnea (OSA). We sought to examine the retinal vascular manifestations of OSA. METHODS: Nine consecutive patients with OSA underwent ophthalmic examination regardless of any ocular complaints. Seven patients without OSA matched for demographics were used as controls. Fundus photographs from both eyes were used to quantitate retinal vascular tortuosity of the temporal arterial and venous arcades using ImageJ digital analysis software. The tortuosity of each vessel from the optic disc rim to the crossing point of a 5 disc diameter (5DD) circle and 10 disc diameter (10DD) circle centered on the optic disc were quantitated. RESULTS: The mean age of patients with OSA in the study was 52 years ± SD of 10 years and 67 years ± SD of 10 years in the control group. The apnea-hypopnea index in patients with OSA ranged from 12 to 102 events/hr of sleep. The nadir saturation during sleep in patients with OSA ranged from 60% to 87%. There was no significant difference in the frequency of diabetes or hypertension between the groups. Total tortuosity was increased at the 5DD (P = 0.011) and 10DD (P = 0.004) marks. Arterial tortuosity was significantly increased at the 10DD mark (P = 0.016). Venular tortuosity was increased at both the 5DD (P = 0.001) and 10DD (P = 0.028) marks. CONCLUSION: Patients with OSA have increased retinal vascular tortuosity as compared to matched controls. Increased tortuosity of the retinal vasculature may be a novel association with OSA. A larger prospective study will be necessary to further explore this relationship and its clinical significance.