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Frontiers in Medicine 2023X-linked retinoschisis (XLRS) is a potential target for gene supplementation approaches. To establish potential structural and functional endpoints for clinical trials,...
INTRODUCTION
X-linked retinoschisis (XLRS) is a potential target for gene supplementation approaches. To establish potential structural and functional endpoints for clinical trials, a comprehensive understanding of the inter-eye symmetry, relationship between structural and functional parameters, and disease progression is vital.
METHODS
In this retrospective multicentre study, 118 eyes of 59 XLRS patients with mutations were assessed. Information from center databases included: variant; age at presentation; best-corrected visual acuity (BCVA), central retinal thickness (CRT), macular volume (MV) at presentation and at the last follow up; full-field electroretinogram (ERG) findings; presence of peripheral retinoschisis and complications (vitreous hemorrhage, retinal detachment); treatment with systemic or topical carbonic anhydrase inhibitors (CAI).
RESULTS
Inter-eye symmetry revealed strong correlation in CRT ( = 0.77; < 0.0001) and moderate correlations in MV ( = 0.51, < 0.0001) and BCVA ( = 0.49; < 0.0001). Weak or no correlations were observed between BCVA and structural parameters (CRT, MV). Peripheral retinoschisis was observed in 40 (68%), retinal detachment in 9 (15%), and vitreous hemorrhage in 5 (8%) patients, respectively. Longitudinal examinations (mean, 4.3 years) showed no BCVA changes; however, a reduction of the CRT ( = 0.02), and MV ( = 0.01) was observed. Oral and/or topical CAI treatment did not significantly alter the CRT ( = 0.34).
DISCUSSION
The XLRS phenotype demonstrates a strong CRT symmetry between the eyes within individual patients and stable BCVA over several years. BCVA exhibits a weak correlation with the morphological parameters of retinal thickness (CRT MV). In our cohort, longitudinal functional changes were not significant, likely attributed to the short average follow-up period. Furthermore, CAI treatment didn't influence both morphological and functional outcomes.
PubMed: 38351967
DOI: 10.3389/fmed.2023.1331889 -
American Journal of Ophthalmology Case... Mar 2024In this study, we report a patient who presented with both chronic myelocytic leukemia (CML) and Susac syndrome (SS).
PURPOSE
In this study, we report a patient who presented with both chronic myelocytic leukemia (CML) and Susac syndrome (SS).
OBSERVATIONS
A 45-year-old male diagnosed with CML in the blast phase sought consultation due to a deterioration in vision in his right eye. He also had hearing loss and severe migraneous headaches. Best corrected visual acuity was light perception and 20/20 in the right and left eyes, respectively. The slit lamp examination and intraocular pressure were within normal ranges for both eyes. Upon dilated fundoscopy, organized vitreous hemorrhage was observed in the right eye, while the left eye exhibited extensive sclerotic vessels with retinal neovascularization in the periphery. Ultrasound of the right eye showed tractional retinal detachment. Optical coherence tomography of the left retina showed thinning of the retina in temporal macula. Fluorescein angiography revealed a substantial nonperfused region in the peripheral left retina, accompanied by arterioarterial and arteriovenous collaterals, along with microaneurysms. MRI showed scattered foci of hyperintensity within the supratentorial white matter, mostly subcortical on T2-weighted and fluid-attenuated inversion-recovery. The patient received a diagnosis of SS and was subsequently referred to the neurology service for further assessment and potential treatment.
CONCLUSION AND IMPORTANCE
SS may manifest as a presentation of CML. It is advisable to conduct investigations for SS in CML patients experiencing neurological, ophthalmological, or otological symptoms.
PubMed: 38318442
DOI: 10.1016/j.ajoc.2024.101996 -
American Journal of Ophthalmology Case... Mar 2024To describe a case of retinal vaso-occlusive vasculitis with associated lid edema and conjunctivitis following intravitreal pegcetacoplan administration in a patient...
PURPOSE
To describe a case of retinal vaso-occlusive vasculitis with associated lid edema and conjunctivitis following intravitreal pegcetacoplan administration in a patient with geographic atrophy (GA).
OBSERVATION
A 78 year old Caucasian woman presented with complaints of lid edema, conjunctival injection, loss of vision, and mild ocular discomfort eleven days after receiving an intravitreal pegcetacoplan injection in the left eye for geographic atrophy. Visual acuity on presentation was decreased to 20/400 from 20/200 previously in that eye. Eyelid edema and conjunctival injection were present with minimal anterior chamber reaction. Dilated fundus examination revealed hemorrhages throughout the retina and signs of retinal vasculitis. The patient subsequently developed hyphema and vitreous hemorrhage. Laboratory evaluations for common infectious and inflammatory causes including aqueous and vitreous cultures for bacteria and Herpes simplex PCR were normal or negative. A delayed hypersensitivity to pegcetacoplan was suspected and was treated with topical, oral subconjunctival and intravitreal steroids.
CONCLUSION
This index report illustrates a case of retinal vaso-occlusive vasculitis associated with intravitreal pegcetacoplan associated with lid edema and conjunctival injection and subsequent hyphema and vitreous hemorrhage. Therapy with steroids topically, systemically, periocularly and intravitreally were used to treat the inflammatory process and prevent further visual loss.
PubMed: 38298266
DOI: 10.1016/j.ajoc.2024.101999 -
Scientific Reports Jan 2024To investigate the sensitivity and potential application of steady-state flash visual evoked potentials (SSFVEP) in assessing the visual function of fundus diseases with...
To investigate the sensitivity and potential application of steady-state flash visual evoked potentials (SSFVEP) in assessing the visual function of fundus diseases with vitreous hemorrhage. 18 patients diagnosed with monocular vitreous hemorrhages in the fundus were examined the flash visual evoked potentials (FVEP) and SSFVEP in both eyes. The difference in the P2-wave amplitude of FVEP and the average amplitude of SSFVEP waveform between the diseased eyes and those without vitreous hemorrhage were statistically compared. There was no significant difference in the waveform of FVEP between both eyes. The amplitude of P2-wave from FVEP of the diseased eye was slightly lower than that without vitreous hemorrhage. However, the difference was not statistically significant (P = 0.111). The waveform of SSFVEP in the eye without vitreous hemorrhage showed a towering shape, while that of the diseased eye was flat. The average amplitude of SSFVEP in the diseased eye was statistically lower than that without vitreous hemorrhage (P = 0.036). The difference ratio of SSFVEP amplitude between both eyes was significantly greater than that of FVEP amplitude (P = 0.028). In some fundus diseases with vitreous hemorrhage, SSFVEP had a higher sensitivity than FVEP, providing a novel potential application for visual function assessment.
Topics: Humans; Vitreous Hemorrhage; Evoked Potentials, Visual; Fundus Oculi
PubMed: 38287026
DOI: 10.1038/s41598-023-47714-4 -
Case Reports in Ophthalmological... 2024We report the case of bilateral, subinternal limiting membrane crystalline deposits in a patient with Terson syndrome, describe the possible pathogenesis, and highlight...
BACKGROUND
We report the case of bilateral, subinternal limiting membrane crystalline deposits in a patient with Terson syndrome, describe the possible pathogenesis, and highlight management. . A 24-year-old male with a history of traumatic massive parenchymal and subdural frontal hemorrhage presented to our clinic seven months after a motor vehicle accident, prolonged hospitalization, and rehabilitation, complaining of decreased vision in both eyes. The Snellen visual acuity was 1/60 in the right eye, and 6/60 in the left eye. Fundus examination showed an organized white vitreous hemorrhage in both eyes with almost no view of the retina. The anterior segments were normal. He underwent a 25-gauge pars plana vitrectomy in both eyes. During the surgery, golden crescent-shaped sediment consisting of small crystals was observed under the internal limiting membrane in both eyes: anterior to the inferior temporal vascular arcade in the right eye and posterior to it in the left eye. Internal limiting membrane (ILM) peeling after staining with ILM-blue dye was performed in the left eye, where the finding involved the macula. One year after the surgery, visual acuity significantly improved to 6/8.5 on the right and 6/6 on the left. Epiretinal membrane formation was observed in the right eye, where ILM peeling was not performed.
CONCLUSION
Subinternal limiting membrane crystalline deposit finding is a rare condition. Consider performing internal limiting membrane peeling and sediment removal in cases with macular involvement. In cases where crystals are concentrated outside of the macula, follow-up may be considered.
PubMed: 38274844
DOI: 10.1155/2024/8225960 -
Retina (Philadelphia, Pa.) May 2024To evaluate features of infectious panuveitis associated with multiple pathogens detected by ocular fluid sampling.
PURPOSE
To evaluate features of infectious panuveitis associated with multiple pathogens detected by ocular fluid sampling.
METHODS
Single-center, retrospective, consecutive case series of patients with aqueous/vitreous polymerase chain reaction testing with >1 positive result in a single sample from 2001 to 2021.
RESULTS
Of 1,588 polymerase chain reaction samples, 28 (1.76%) were positive for two pathogens. Most common pathogens were cytomegalovirus (n = 16, 57.1%) and Epstein-Barr virus (n = 13, 46.4%), followed by varicella zoster virus (n = 8, 28.6%), Toxoplasma gondii (n = 6, 21.4%), herpes simplex virus 2 (n = 6, 21.4%), herpes simplex virus 1 (n = 6, 21.4%), and Toxocara (n = 1, 3.6%). Mean initial and final visual acuity (logarithm of the minimum angle of resolution) were 1.3 ± 0.9 (Snellen ∼20/400) and 1.3 ± 1.1 (Snellen ∼20/400), respectively. Cytomegalovirus-positive eyes (n = 16, 61.5%) had a mean final visual acuity of 0.94 ± 1.1 (Snellen ∼20/175), whereas cytomegalovirus-negative eyes (n = 10, 38%) had a final visual acuity of 1.82 ± 1.0 (Snellen ∼20/1,320) ( P < 0.05). Main clinical features included intraocular inflammation (100%), retinal whitening (84.6%), immunosuppression (65.4%), retinal hemorrhage (38.5%), and retinal detachment (34.6%).
CONCLUSION
Cytomegalovirus or Epstein-Barr virus were common unique pathogens identified in multi-PCR-positive samples. Most patients with co-infection were immunosuppressed with a high rate of retinal detachment and poor final visual acuity. Cytomegalovirus-positive eyes had better visual outcomes compared with cytomegalovirus-negative eyes.
Topics: Humans; Retrospective Studies; Male; Female; Panuveitis; Middle Aged; Aqueous Humor; Eye Infections, Viral; Polymerase Chain Reaction; Visual Acuity; Adult; Aged; DNA, Viral; Vitreous Body; Cytomegalovirus; Young Adult; Toxoplasma
PubMed: 38271688
DOI: 10.1097/IAE.0000000000004037 -
BMC Ophthalmology Jan 2024The importance of communicating the anterior chamber and vitreous cavity for managing malignant glaucoma (MG) is widely recognized. This study investigated the impact of...
BACKGROUND
The importance of communicating the anterior chamber and vitreous cavity for managing malignant glaucoma (MG) is widely recognized. This study investigated the impact of improved minimally invasive anterior vitrectomy (IAV) on the prognosis of MG.
METHODS
This retrospective interventional study included patients with MG who underwent conventional surgery or improved minimally IAV in Nanchang Aier Eye Hospital between January 2011 and April 2021. For the improved step, a small amount of triamcinolone acetonide was injected into the vicinity of the iris. Then, the residual vitreous body adhering to triamcinolone acetonide was excised. Comparisons were made using repeated measures ANOVA, t-test, and chi-squared test.
RESULTS
Thirty-one eyes from 26 patients were included: 15 eyes from 13 patients in the conventional group and 16 eyes from 13 patients in the IAV group. The 1-week, 1-month, and 3-month intraocular pressure (IOP) and the 3-month mean central anterior chamber depth were comparable between the two groups (all P > 0.05). The conventional group showed one eye with intraoperative vitreous hemorrhage and two eyes with postoperative re-shallowing of the anterior chamber; such events did not occur in the IAV group, and none developed corneal endothelial decompensation, IOL deviation, suprachoroidal hemorrhage, or retinal detachment during treatment and follow-up.
CONCLUSION
Patients with MG who undergo improved minimally IAV might have similar postoperative IOP and central anterior chamber depth compared with conventional surgery but with reduced complications such as intraoperative vitreous hemorrhage and postoperative re-shallowing of the anterior chamber. Improved minimally IAV might be an alternative surgery for MG.
Topics: Humans; Vitrectomy; Retrospective Studies; Triamcinolone Acetonide; Vitreous Hemorrhage; Glaucoma; Prognosis; Anterior Chamber; Blood Loss, Surgical
PubMed: 38267918
DOI: 10.1186/s12886-024-03310-2 -
Journal of Personalized Medicine Dec 2023Neovascular glaucoma (NVG) secondary to proliferative diabetic retinopathy (PDR) is a devastating ocular disease with poor prognosis. Intravitreal ranibizumab injection...
Clinical Efficacy of Preoperative and Intraoperative Intravitreal Ranibizumab as Adjuvant Therapy of Ahmed Glaucoma Valve Implantation Combined with Vitrectomy in the Management of Neovascular Glaucoma with Diabetic Vitreous Hemorrhage.
Neovascular glaucoma (NVG) secondary to proliferative diabetic retinopathy (PDR) is a devastating ocular disease with poor prognosis. Intravitreal ranibizumab injection (IVR) has been used as adjuvant therapy of surgical interventions preoperatively or intraoperatively. This study aimed to determine the efficacy and safety of combined IVR as adjuvant therapy in treating NVG with vitreous hemorrhage (VH) in PDR. A total of 39 NVG patients with VH (39 eyes) received IVR 3 to 5 days before surgery, and then they were assigned to either pars plana vitrectomy (PPV) + Ahmed glaucoma valve (AGV) implantation (Group 1, = 22) or PPV + AGV implantation + intraoperative IVR (Group 2, = 17). Patients were followed up for at least 9 months. Intraocular pressure (IOP), anti-glaucoma medications, best corrected visual acuity (BCVA), surgical success rates and postoperative complications were compared. Results showed that IOP decreased promptly after surgery and was notably maintained at a mid-term follow-up in both groups, and no significant differences were observed (all > 0.05). Additional intraoperative IVR significantly reduced postoperative recurrent VH and iris neovascularization ( = 0.047, = 0.025, respectively). There was no remarkable difference in postoperative anti-glaucoma medications, BCVA and complications between two groups (all > 0.05). In conclusion, preoperative and intraoperative IVR as adjuvant therapy of AGV implantation combined with PPV could be a safe and effective treatment for NVG with VH in PDR. An additional intraoperative anti-VEGF injection could significantly reduce postoperative VH and iris neovascularization.
PubMed: 38248719
DOI: 10.3390/jpm14010018 -
Medicine Jan 2024Vitreous hemorrhage (VH) is one of the main causes of vision loss in diabetic retinopathy (DRP). Early surgery increases the visibility of the retina, allowing early...
Vitreous hemorrhage (VH) is one of the main causes of vision loss in diabetic retinopathy (DRP). Early surgery increases the visibility of the retina, allowing early recognition of DRP complications and additional treatments. One of the most important reasons affecting success after surgery is recurrent vitreous hemorrhage (RVH). We aimed to investigate the risk factors for RVH after early 25G vitrectomy in diabetic VH. Eighty eyes of eighty patients who underwent early 25G PPV surgery with a diagnosis of VH due to proliferative diabetic retinopathy (PDR) were included in this retrospective study. Vision acuity changes and intraocular pressure (IOP) changes were compared. The effect of arterial hypertension (HT), coronary artery disease (CAD), preoperative antiglaucomatous usage, and anticoagulant usage on RVH was investigated. A value of P < .05 was accepted as statistically significant. Postoperative RVH was observed in 18 (22.5%) patients. There was no correlation between the age of the patients and the development of postoperative RVH (r = -0.197, P = .08). The rate of HT and the mean HbA1C levels were found to be higher in the patients who developed RVH than in those who did not (P = .04 and < 0.001, respectively). The presence of CAD, preoperative glaucoma disease, and the use of anticoagulants did not have any effect on RVH (P = .229, 0.843, 0.932, respectively). HT and increased HbA1c were found to be risk factors for RVH in VH patients who underwent 25G vitrectomy in the early period in our study.
Topics: Humans; Vitreous Hemorrhage; Vitrectomy; Diabetic Retinopathy; Retrospective Studies; Glycated Hemoglobin; Retina; Risk Factors; Diabetes Mellitus
PubMed: 38241585
DOI: 10.1097/MD.0000000000036963 -
Radiology Case Reports Mar 2024Tuberous sclerosis complex (TSC) is a genetic disorder that causes noncancerous growths and tumors in various body systems. This case report discusses an unusual eye...
Tuberous sclerosis complex (TSC) is a genetic disorder that causes noncancerous growths and tumors in various body systems. This case report discusses an unusual eye condition called retinal astrocytic hamartoma (RAH) in a TSC patient, along with a brain tumor called subependymal giant cell astrocytoma (SEGA). These conditions, linked to TSC gene mutations, can be atypical in size and cause complications like vitreous hemorrhage. Surgical treatment is generally effective. Recognizing similarities between SEGA and RAH can help with early detection and comprehensive care for TSC patients.
PubMed: 38226050
DOI: 10.1016/j.radcr.2023.11.039