Sea Fan Neovascularization Research Articles

A review of outcomes of laser photocoagulation for Goldberg stage 3 proliferative sickle cell retinopathy

Topic: A review of outcomes of laser photocoagulation for Goldberg stage 3 proliferative sickle cell retinopathy (PSR) in a Eye Foundation Hospital. Aims and objectives: To report regression of retina neovascularization and visual outcomes after prophylactic scatter retina laser photocoagulation for Goldberg stage 3 PSR in a tertiary private hospital in sub-Saharan Africa. Methods: A retrospective review of case files of patients who were treated with prophylactic scatter retina laser photocoagulation between January 2017 and June 2022 following a diagnosis of PSR in a Eye Foundation Hospital, Ikeja, Lagos, Nigeria, was done. Results: A total of 124 eyes of 62 patients with PSR were seen within the period under consideration. A majority of patients (49 (79.0%)) had hemoglobin (Hb) genotype SC, while 13 patients (21%) were Hb genotype SS. At presentation, 29 eyes (23.3%) had Goldberg stage 2 PSR, 55 eyes (44.4%) had Goldberg stage 3 PSR, 27 eyes (21.8%) had stage 4 PSR, while 13 eyes (10.5%) had stage 5 PSR. All 55 eyes (49.2%) with stage 3 PSR were treated with prophylactic retina laser photocoagulation. At the patients’ last clinic visit, 34 eyes (61.8%) had the same visual acuity (VA) as at presentation, 11 eyes (20%) had improvements in VA, while 10 eyes (18.2%) had worse VA. Laser treatment success (regression of seafan neovascularization) was seen in 50 eyes (90.9%), while five eyes (8.1%) had unsuccessful laser treatment. Conclusion: A high percentage (90.9%) of resolution of retina neovascularization secondary to PSR at stage 3 of Goldberg’s classification was seen after prophylactic scatter retina laser photocoagulation in our group of evaluated patients. Most patients (81.8%) maintained or had improvements in their visual acuities over the period of follow-up. More clinical research is required to further evaluate outcomes of laser photocoagulation for PSR.

Nonmydriatic Ultra-Widefield Fundus Photography in a Hematology Clinic Shows Utility for Screening of Sickle Cell Retinopathy

To determine the feasibility and accuracy of nonmydriatic ultra-widefield (UWF) fundus photographs taken in a hematology clinic setting for screening of sickle cell retinopathy (SCR) DESIGN: Prospective cohort study. This single-site study took place at the Johns Hopkins Sickle Cell Center for Adults and the Wilmer Eye Institute. The study population was 90 eyes of 46 consecutive adults with sickle cell disease (SCD). Bilateral nonmydriatic fundus photos taken by clinic personnel during the participants' routine hematology appointment were graded by 2 masked retina specialists at the Wilmer Eye Institute for the presence of nonproliferative SCR (NPSR) and proliferative sickle retinopathy (PSR). A third retina specialist adjudicated in cases of grader disagreement. All participants underwent the standard dilated fundus examination (DFE) within 2 years of acquisition of UWF photographs. The main outcome measure was the sensitivity and specificity of nonmydriatic UWF images for the detection of NPSR and PSR RESULTS: PSR was noted in 19 of 90 eyes that underwent DFE and in 9 of 67 gradable UWF images. Interrater agreement between the 2 graders was moderate, with κ=0.65 (range 0.43-0.87) for PSR. For gradable UWF photos, the sensitivity and specificity of detecting SCR using the nonmydriatic photos compared with the DFE were 85.2% and 62.5% for NPSR, respectively, and 69.2% and 100% for detection of PSR, respectively. One participant required ophthalmic therapy in both eyes for active sea-fan neovascularization. UWF imaging shows utility in screening for SCR and may help identify patients with PSR who require a DFE and who may benefit from treatment.

Sea-fan neovascularization of the optic disk in branch retinal vein occlusion

Sea-fan neovascularization of the optic disk in branch retinal vein occlusion

Chronic myeloid leukemia revealed by bilateral proliferative retinopathy: A case report and literature review

Bilateral proliferative retinopathy is a rare complication of chronic myeloid leukemia (CML) as a few case reports have been published to date. In this case report, a 32-year old diabetic female presented with history of bilaterally decreased vision. Ophthalmologic examination showed bilateral proliferative retinopathy (i.e., retinal detachment, vitreous hemorrhage, pre-retinal fibrosis and the presence of bilateral peripheral capillary dropout with multiple retinal sea fan neovascularization) for which panretinal laser photocoagulation and vitrectomy were planned. During the preoperative workup, complete blood count revealed hyperleukocytosis. Later on, the karyotype analysis identified Philadelphia chromosome, confirming the diagnosis of CML. Hence, it was an interesting case where bilateral proliferative retinopathy directed to the diagnosis of CML. Therefore, proliferative retinopathy may be the first presentation of CML.

Deep Learning Detection of Sea Fan Neovascularization From Ultra-Widefield Color Fundus Photographs of Patients With Sickle Cell Hemoglobinopathy

Adherence to screening for vision-threatening proliferative sickle cell retinopathy is limited among patients with sickle cell hemoglobinopathy despite guidelines recommending dilated fundus examinations beginning in childhood. An automated algorithm for detecting sea fan neovascularization from ultra-widefield color fundus photographs could expand access to rapid retinal evaluations to identify patients at risk of vision loss from proliferative sickle cell retinopathy. To develop a deep learning system for detecting sea fan neovascularization from ultra-widefield color fundus photographs from patients with sickle cell hemoglobinopathy. In a cross-sectional study conducted at a single-institution, tertiary academic referral center, deidentified, retrospectively collected, ultra-widefield color fundus photographs from 190 adults with sickle cell hemoglobinopathy were independently graded by 2 masked retinal specialists for presence or absence of sea fan neovascularization. A third masked retinal specialist regraded images with discordant or indeterminate grades. Consensus retinal specialist reference standard grades were used to train a convolutional neural network to classify images for presence or absence of sea fan neovascularization. Participants included nondiabetic adults with sickle cell hemoglobinopathy receiving care from a Wilmer Eye Institute retinal specialist; the patients had received no previous laser or surgical treatment for sickle cell retinopathy and underwent imaging with ultra-widefield color fundus photographs between January 1, 2012, and January 30, 2019. Deidentified ultra-widefield color fundus photographs were retrospectively collected. Sensitivity, specificity, and area under the receiver operating characteristic curve of the convolutional neural network for sea fan detection. A total of 1182 images from 190 patients were included. Of the 190 patients, 101 were women (53.2%), and the mean (SD) age at baseline was 36.2 (12.3) years; 119 patients (62.6%) had hemoglobin SS disease and 46 (24.2%) had hemoglobin SC disease. One hundred seventy-nine patients (94.2%) were of Black or African descent. Images with sea fan neovascularization were obtained in 57 patients (30.0%). The convolutional neural network had an area under the curve of 0.988 (95% CI, 0.969-0.999), with sensitivity of 97.4% (95% CI, 86.5%-99.9%) and specificity of 97.0% (95% CI, 93.5%-98.9%) for detecting sea fan neovascularization from ultra-widefield color fundus photographs. This study reports an automated system with high sensitivity and specificity for detecting sea fan neovascularization from ultra-widefield color fundus photographs from patients with sickle cell hemoglobinopathy, with potential applications for improving screening for vision-threatening proliferative sickle cell retinopathy.

Intravitreal Bevacizumab for Proliferative Sickle Retinopathy: A Case Series

Purpose: To report outcomes of intravitreal bevacizumab therapy for proliferative sickle retinopathy (PSR). Methods: A retrospective, interventional case series. Five eyes of 5 patients with PSR were managed with intravitreal bevacizumab therapy over a 13-year period at a single institution. Results: Four patients had sickle cell-hemoglobin SC disease and 1 had sickle cell-beta thalassemia disease. Four of the patients treated with intravitreal bevacizumab injection were treated for recurrent vitreous hemorrhage and 1 was treated for new peripheral sea fan neovascularization. In those patients treated for vitreous hemorrhage, there was improvement in visual acuity as early as 2 weeks after treatment. Only 2 of the patients had documented recurrent vitreous hemorrhage during the period of follow-up after the initial injection. In 1 patient, the vitreous hemorrhage did not recur until 13 months after the injection. All patients showed an anatomic response to intravitreal bevacizumab therapy with partial regression of the peripheral sea fan neovascularization. All patients tolerated the injections without any complications. Conclusions: Intravitreal bevacizumab injections appear to be well tolerated and may be an effective treatment of PSR. Regression of peripheral sea fan neovascularization and decreased duration of vitreous hemorrhage may be observed. Large-scale randomized controlled trials are needed to further clarify the role of bevacizumab in PSR.

Peripheral Sea-Fan Neovascularization as the Initial Manifestation of Systemic Lupus Erythematosus Retinopathy

Purpose: To Describe a case of Sea-fan Neovascularization in a SLE patient. Method: Case report A 35 year old female who underwent a right hemicolectomy for gangrene gut of undetermined cause was referred to the Ophthalmology department for fundus examination. A Complete ocular examination along with Fluorescein Angiography was done. Fundus examination revealed neovascularization in the temporal quadrant of her right eye. Fluorescein angiography revealed a sea-fan neovascularization corresponding to the neovascular tuft. Hb electrophoresis revealed no abnormal hemoglobin. ANA and anti-dsDNA tests were positive. She was referred to the immunology department where she was diagnosed as a case of SLE according to the SLE diagnostic criteria of the American Rheumatology Association. Conclusion: Sea-fan neovascularization can be seen in SLE. To the best of our knowledge this is the first time sea-fan neovascularization is described in a case of SLE. It also demonstrates the diagnostic difficulty of SLE when it presents without the usual manifestations and the importance of a thorough Ophthalmological

Intravitreal Ranibizumab for Stage IV Proliferative Sickle Cell Retinopathy: A First Case Report.

Purpose. To present the case of a 27-year-old male patient with stage IV proliferative sickle cell retinopathy, treated with one intravitreal injection of ranibizumab, showing regression of the neovascularization and no recurrence at the 9-month follow-up. Methods. A 27-year-old male patient presented with blurred vision and floaters in the right eye since three days. His best corrected visual acuity was 6/18. Ophthalmological examination and fluorescein angiography revealed proliferative sickle cell retinopathy stage IV with vitreous hemorrhage and sea fan neovascularization, as well as ischemic areas at the temporal periphery. Results. The patient was treated with one intravitreal injection of ranibizumab, presenting improvement in the visual acuity from 6/18 to 6/6, resolution of vitreous hemorrhage, and regression of the neovascularization. Additionally, he underwent scatter laser photocoagulation at the ischemic areas. At the 9-month follow-up there was no recurrence, while no adverse effects were noticed. Conclusions. Intravitreal ranibizumab may be a useful adjunct to laser photocoagulation in the management of proliferative sickle cell retinopathy and may permit some patients to avoid pars plana vitrectomy for vitreous hemorrhage.

Bevacizumab presurgical treatment for proliferative sickle-cell retinopathy-related retinal detachment.

To report the use of presurgical intravitreal bevacizumab in the context of proliferative sickle-cell retinopathy with retinal detachment. Intravitreal bevacizumab was injected 3 days before the surgical procedure for traction retinal detachment. Vitrectomy, membrane peeling, endolaser, and SF6 gas tamponade were performed. A 37-year-old African American woman presented with hemoglobin sickle-cell disease and temporal retinal detachment with bullous subretinal fluid extending through the fovea associated with an area of active sea-fan retinal neovascularization with preretinal hemorrhage and retinal traction, with 3 associated retinal breaks. The sea-fan neovascularization associated with the traction retinal detachment and the resultant retinal breaks appeared more fibrotic and les vascular than was noted prior to the pre-operative bevacizumab injection. Segmentation and dissection were performed with minimal bleeding, and retinal traction was relieved without difficulty. This was believed to be atypical in the experience of the surgeons. One month postoperatively, vision measured 20/50, and the retina remained attached. Further study is necessary to clarify the role of anti-vascular endothelial growth factor in the treatment of proliferative sickle cell retinopathy associated retinal detachment.

PERIPHERAL VASCULAR ABNORMALITIES IN β-THALASSEMIA MAJOR DETECTED BY ULTRA WIDE-FIELD FUNDUS IMAGING.

To describe peripheral retinal vascular abnormalities in two patients with β-thalassemia major. Visual acuity testing, slit-lamp biomicroscopy, dilated fundus examination, ultra wide-field fundus photography, and fluorescein angiography were performed in two consecutive patients with β-thalassemia major. Both patients had excellent visual acuity (20/20 and 20/25). Anterior segment examination was unremarkable. There were no lenticular changes noted in either patient. Dilated fundus examination was only remarkable for peripheral neovascular changes with preretinal hemorrhage in one patient and white without pressure in the other patient. Ultra wide-field fluorescein angiography revealed peripheral ischemia, vascular remodeling, active neovascularization, and arteriovenous anastomosis at the junction of perfused and nonperfused peripheral retina. One patient had sea fan neovascularization with active vitreous hemorrhage requiring peripheral laser photocoagulation. As with patients with sickle cell disorder, patients with β-thalassemia major may require surveillance for peripheral vascular abnormalities, with treatment of these abnormalities before they result in permanent visual loss.

Retinopathy in patients with sickle cell trait

Background Sickle hemoglobinopathies are among the most prevalent genetic disorders in the United States. Sickle cell trait (hemoglobin AS) is the most common genotype and has traditionally been considered a benign condition. Systemic and ocular complications are seen infrequently in patients with sickle cell trait. In the presence of concomitant systemic diseases or trauma, however, marked retinopathy can occur. The presence of retinopathy in patients with sickle cell trait necessitates a comprehensive medical workup, including serologic testing to rule out systemic conditions. Case reports Two cases are presented. The patient in case 1 is a 44-year-old black man with a positive history of sickle cell trait and uncontrolled hypertension. Best-corrected visual acuities (BCVAs) were 20/20 in the right eye (O.D.) and the left eye (O.S.). Retinal evaluation found vitreo-retinal fibrotic scaffolding and “sea-fan” neovascularization. The patient in case 2 is a 45-year-old black woman who presented after a stroke 1-month prior. Her medical history was positive for hypertension, hypercholesterolemia, a recently diagnosed aneurismal dilation of the ascending thoracic aorta, and sickle cell trait. BCVAs were 20/20 O.D., and 20/20 O.S. A single dot hemorrhage was evident O.D., and an inferior-temporal branch retinal artery occlusion with an adjacent area of sea-fan neovascularization was observed O.S. Conclusion Sickle cell disease remains a major public health concern because of its associated significant morbidity and mortality. Sickle cell trait is considered benign in a healthy patient. However, under conditions of stress, concomitant systemic diseases, or trauma leading to hypoxia, sickle cell trait can become a pathologic risk factor.

Retinitis pigmentosa associated with peripheral sea fan neovascularization

To describe a case with retinitis pigmentosa associated with sea fan type retinal neovascularization. Complete ocular examination including fluorescein angiography was performed in a 9-year-old girl. Ophthalmoscopically, in addition to arteriolar narrowing and bone corpuscular pigmentation of both retinae, a vascular lesion with surrounding intraretinal exudation was noted in the upper equatorial region of the right eye. On fluorescein angiography, the lesion stained in the form of a sea fan neovascularization. Sea fan type of neovascularization can be seen in association with retinitis pigmentosa. Fluorescein angiography is important in identifying the exact nature of such a lesion.

Influência do genótipo da hemoglobinopatia falciforme nas manifestações retinianas em pacientes de um hospital universitário

Objetivo: Identificar as alterações retinianas em pacientes com hemoglobinopatia falciforme e comparar sua prevalência nos diferentes genótipos da doença em uma população de pacientes de hospital universitário de Porto Alegre - Brasil. Métodos: Realizou-se exame oftalmológico com avaliação fundoscópica e estudo do genótipo de pacientes portadores de hemoglobinopatia falciforme. Resultados: Foram examinados 94 olhos de 47 pacientes com doença falciforme: 17 pacientes do sexo masculino e 30 feminino, com média de idades de 22,4 anos (± 16,8 anos). 20 pacientes possuíam genótipo SS, 10 possuíam SC, 10 possuíam AS, e 7 SThal. As alterações retinianas encontradas foram: hemorragia tipo "salmon patch" em 1 olho, hiperpigmentação tipo "black sunburst" em 12 olhos, neovascularização tipo "sea fan" em 3 olhos, hemorragia vítrea em 1 olho e descolamento de retina em 1 olho. O grupo SC foi o que demonstrou ter maior freqüência de alterações (60%), e quando comparado com o grupo SS mostrou 4,0 vezes mais chance de apresentar retinopatia (p<0,05).Todos os 5 olhos com retinopatia proliferativa possuíam genótipo SC. Conclusões: A freqüência de alterações fundoscópicas no grupo estudado bem como a gravidade das alterações (retinopatia proliferativa) são maiores nos pacientes heterozigotos de genótipo SC, quando comparados com os demais genótipos.

Peripheral retinal nonperfusion associated with essential thrombocytosis

PURPOSE: To describe a patient with retinal complications of essential thrombocytosis. METHOD: Case report. A 24-year-old man with acute vitreous hemorrhage of the right eye underwent ocular and systemic examinations to elucidate the origin of this condition. RESULTS: Fluorescein angiography of the right eye showed an avascular peripheral retina with marked capillary nonperfusion, arteriovenous anastomosis, and sea fan neovascularization. Blood studies showed thrombocytosis without other associated systemic diseases. The peripheral retinal capillary nonperfusion and sea fan neovascularization were treated with dye-laser photocoagulation, and aspirin, 80 mg daily, was given for systemic treatment. CONCLUSIONS: In our patient, the avascular retina was associated with essential thrombocytosis, which may cause an avascular peripheral retina with neovascularization and vitreous hemorrhage in otherwise healthy persons.

A Randomized Clinical Trial of Scatter Photocoagulation of Proliferative Sickle Cell Retinopathy

A randomized prospective clinical trial of argon laser scatter photocoagulation therapy for proliferative sickle cell retinopathy was performed on 116 patients (174 eyes) in Kingston, Jamaica. Ninety-nine eyes were treated with scatter photocoagulation and 75 eyes served as controls. The average length of follow-up was 42 months for the control eyes and 47 months for the treated eyes. Prolonged loss of visual acuity was statistically significantly reduced in the treated eyes. The incidence of vitreous hemorrhage was also significantly reduced in the treated eyes after controlling for the previously defined risk factors of vitreous hemorrhage and extent of proliferative sickle cell retinopathy at entry into the study. There were no complications associated with argon laser scatter photocoagulation. Scatter photocoagulation of proliferative sickle cell retinopathy is currently the most effective and safe way to treat patients with sea fan neovascularization.

Scatter Retinal Photocoagulation for Proliferative Sickle Cell Retinopathy

We treated sea-fan neovascularization in 19 patients (21 eyes) who had proliferative sickle cell retinopathy with localized scatter photocoagulation. We placed burns of light to moderate intensity adjacent to 45 sea fans in a scatter fashion. In general, treatment extended from 1.5 mm posterior to 1.5 mm anterior to the sea fan and one clock hour to each side of each lesion. Flat sea fans responded dramatically to treatment, with complete regression in 24 of 28 lesions. Elevated sea fans (especially large ones) responded less rapidly, with complete regression in only four of 17 lesions. Localized scatter photocoagulation is valuable in treating patients with early proliferative sickle cell retinopathy.