Retinal nerve fiber layer thickness in children with β-thalassemia major

Purpose. Retinal nerve fibre layer (RNFL) is a sensitive structure, which is affected by anaemia due to hypoxia. A timely detection of RNFL thinning may aid preventing devastating complications. Optical coherence tomography (OCT) measures RNFL thinning with accuracy and helps in detecting thinning of the retinal layer in anaemic patients. This study was destined to evaluate thinning of RNFL in anaemic patients and their correlation with the haemoglobin level. Methods. It was a prospective comparative study. Total of 151 patients were included in this study. Patients with retinal diseases were excluded from this study. After initial evaluation, haematological and ophthalmological parameters were measured. RNFL was measured with OCT and corroborated with the Hb level and analysed accordingly. EPI and SPSS softwares were used for detail analysis and the correlation between RNFL thinning and the Hb level. Initially, each eye was separately assigned a value (0, 1, and 2) (normal, borderline, and abnormal, respectively) as per the severity of thinning, and then, the sum of the scores of both eyes were considered as a separate variable, and a multiple linear regression analysis was performed with the independent variables. Results. RNFL thinning was found to be significant in each group of patients. There was a strong correlation of RNFL thinning with degree of anaemia. Conclusions. Thalassaemia, iron deficiency anaemia, and anaemia of chronic diseases are associated with the significant damage to RNFL. Degree of anaemia is the most important parameter for such thinning of the RNFL layer.

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“…Sex distribution among the study population was found to corroborate with various previous studies [13][14][15].…”

Section: Gender Distributionsupporting

confidence: 90%

“…Mean age of this study population was much higher (23.6 years) compared to other studies like by Uzun et al [13] where the mean age of the study population was much younger (average 13.7 years).…”

Section: Age Distributioncontrasting

confidence: 64%

Retinal Nerve Fibre Layer Thinning in Patients with Thalassaemia, Iron Deficiency Anaemia, and Anaemia of Chronic Diseases

Datta

Baidya

Banerjee

et al. 2020

Journal of Ophthalmology

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“…As a result, various ocular manifestations can be seen in β-thalassemia patients including color vision anomalies, night blindness, cataract, visual field defects, decreased visual acuity, and optic neuropathy. 1 We did not find any eye findings to be detected yet in our study patients.…”

Section: Discussioncontrasting

confidence: 64%

“…β-thalassemia syndromes include a group of hereditary blood disorders characterized by the development of anemia as a result of a combination of hemolysis, ineffective erythropoiesis, and iron overload due to defective β-globulin chain synthesis. 1 The World Health Organization reports that thalassemia is the most common chronic and genetic disease in 60 countries and affects about 10,000 people per year. 2 Intensive chronic anemia, growth retardation, bone disorders, hepatosplenomegaly, and congestive heart failure are the main clinical features of this disease.…”

Section: Introductionmentioning

confidence: 99%

Optical coherence tomography angiography findings in young β-thalassemia patients

Kazancı

Korkmaz

Can

2020

European Journal of Ophthalmology

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Purpose: The purpose was to evaluate retinal vascular parameters by optical coherence tomography angiography in β-thalassemia major patients. Methods: Thirty-three patients with β-thalassemia major (study group) and 29 healthy children (control group) were enrolled in the study. All subjects underwent a complete ocular examination. The mean foveal avascular zone, non-flow area, foveal avascular zone perimeter, acircularity index of foveal avascular zone, foveal density, the superficial capillary plexus, and deep capillary plexus were scanned using 6 × 6 mm optical coherence tomography angiography scans centered on the macula. Superficial capillary plexus and deep capillary plexus were also scanned centered on the optic disk. We collected data on histories of patients, and hemoglobin and ferritin were also studied from both groups. Results: The mean age was 13.85 ± 4.69 years (range: 4–21 years) in β-thalassemia major group and 12.59 ± 3.66 years (range: 6–18 years) in the control group. The mean foveal avascular zone value was 0.265 ± 0.11 mm2 in the study group and 0.296 ± 0.12 mm2 in the control group. The mean non-flow area value was 0.468 ± 0.12 mm2 in the study group and 0.479 ± 0.14 mm2 in the control group ( p > 0.05). Differences in the mean values for foveal density and acircularity index were statistically significant between the study group and control group ( p < 0.05, p = 0.026, and p = 0.026, respectively). Superficial capillary plexus and deep capillary plexus were not a significant difference between the study and control groups in 6 × 6 mm scans on macula and 4.5 × 4.5 mm scans on optic disk area ( p > 0.05). Acircularity index was negatively correlated ( r = −0.292, p = 0.026), and foveal density was positively correlated with hemoglobin ( r = 0.292, p = 0.026). Conclusion: By using optical coherence tomography angiography, we detected foveal microvascular changes in young β-thalassemia patients before significant ocular anomalies development.

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“…According to previous studies, we hypothesized that in TDT patients, the tissue iron deposition could promote the neuronal cells death determining a thinning in ganglion cell complex and retinal nerve fiber layer, confirmed by the close correlation between the increased ferritin levels and the reduced thickness of the retinal nerve fiber layer [ 28 , 30 , 31 ].…”

Section: Discussionmentioning

confidence: 87%

Retinal and Choriocapillaris Vascular Changes in Patients Affected by Different Clinical Phenotypes of β-Thalassemia: An Optical Coherence Tomography Angiography Study

Cennamo

Montorio

Mazzella

et al. 2021

Biology

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In this cross-sectional study we assessed the vascular alterations in retinal and choriocapillaris perfusion in patients affected by β-thalassemia, by means of optical coherence tomography angiography (OCTA). A total of 124 eyes of 62 patients (mean age 44.74 ± 5.79 years old) affected by β-thalassemia (transfusion dependent thalassemia (TDT), non-transfusion dependent thalassemia (NTDT) and minor) were compared to 40 eyes of twenty healthy subjects. We evaluated the vessel density (VD) in superficial capillary plexus, deep capillary plexus, radial peripapillary capillary, choriocapillaris and the foveal avascular zone area. The TDT group showed a statistically significant reduction in retinal and choriocapillaris VD respect to controls and the other groups (p < 0.05). No statistically significant difference was found in OCTA parameters between β-thalassemia minor and controls. The NTDT group showed a significant reduction in VD in deep capillary plexus respect to controls and β-thalassemia minor. Significant negative correlations were shown in TDT group between foveal avascular zone and hemoglobin (r = −0.437, p = 0.044) and between ferritin levels and VD of choriocapillaris (r = −0.431, p = 0.038). The OCTA parameters provided a deeper understanding on retinal and choriocapillaris vascular impairment affected by tissue hypoxia levels and the oxidative stress in different clinical phenotypes of the β-thalassemia.

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Retinal nerve fiber layer thickness in children with β-thalassemia major

Abstract: In this study, we observed RNFL was thinner in patients with β-thalassemia major. Thinning of RNFL did not correlate with hemoglobin or ferritin levels.

Cited by 7 publications

References 35 publications

Retinal Nerve Fibre Layer Thinning in Patients with Thalassaemia, Iron Deficiency Anaemia, and Anaemia of Chronic Diseases

Retinal Nerve Fibre Layer Thinning in Patients with Thalassaemia, Iron Deficiency Anaemia, and Anaemia of Chronic Diseases

Optical coherence tomography angiography findings in young β-thalassemia patients

Retinal and Choriocapillaris Vascular Changes in Patients Affected by Different Clinical Phenotypes of β-Thalassemia: An Optical Coherence Tomography Angiography Study

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