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Purpose: We evaluated the clinical features and retinal and disk perfusion characteristics by using optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) in a subset of giant cell arteritis (GCA) patients who manifested anterior ischemic optic neuropathy (AION), in a subset of GCA patients without ocular involvement, and in a control group composed of healthy controls. Methods: We performed an observational study on the eyes of GCA patients affected by arteritic AION both in acute and chronic phases, unaffected eyes of AION, eyes of GCA patients without ocular involvement, and in a control group of healthy eyes of healthy individuals. All patients underwent a complete ophthalmic examination and an OCT and OCTA of the macula and the disk. Results: The study evaluated 10 eyes of GCA patients with AION (AION group), 8 unaffected eyes of GCA patients with AION in another eye (unaffected eyes of AION group), 16 eyes of GCA patients without ocular involvement (non-ocular group), and 22 eyes of healthy patients (healthy group). The ganglion cell complex (GCC) superior and inferior thicknesses were significantly lower in the AION group compared to the unaffected eyes of the AION group (p = 0.045 and p = 0.034, respectively). All OCTA vascular density parameters of the optic disk analyzed in this study (optic nerve head (ONH) whole, superior, inferior, radial peripapillary capillary plexus (RPCP) whole, superior, inferior, lamina cribrosa (LC) whole, superior, inferior) resulted significantly lower in the AION group compared to the unaffected eyes group (p < 0.05 for all the comparisons). The ONH whole and inferior were statistically higher in the healthy group in comparison to the group of GCA patients without ocular involvement (p = 0.008 and p = 0.006, respectively). The ONH inferior was also statistically higher in the unaffected eyes of the AION group in comparison to the non-ocular group (p = 0.045). Regarding the OCTA macular vessel density parameters, the superficial capillary plexus (SCP), whole and inner, were statistically lower in the AION group compared with the unaffected eyes of the AION group. Conclusion: We found a profound vascular impairment in eyes affected by AION and areas of hypoperfusion in the eyes of patients with GCA without ocular involvement, good BCVA, and no clinically significant features. We hypothesized that these areas of lower vessel density might represent areas of subclinical hypoperfusion that cannot be detected ophthalmoscopically.
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Purpose: We report the successful surgical treatment of a case of spontaneous complete anterior crystalline lens luxation in a patient affected by retinitis pigmentosa (RP), associated with elevated intraocular pressure and pupillary block. Additionally, we review the current literature regarding the association between ectopia lentis and RP. Case description: A 44-year-old female RP patient presented to our emergency department reporting severe ocular pain in her left eye (LE) and sickness. She had no history of ocular trauma and did not report systemic disorders. The best corrected visual acuity at presentation was 1/20 in her LE, the intraocular pressure was 60 mmHg, and slit lamp examination showed in her LE a complete dislocation of the lens in the anterior chamber, with mydriasis, atalamia, and a pupillary block. The patient had been administered intravenous mannitol 18% solution and dorzolamide-timolol eye drops and was hospitalized for urgent lens extraction. Anterior segment optical coherence tomography and ultrasound biomicroscopy were performed before surgery. Decompressive 23-gauge pars plana vitrectomy and phacoemulsification were performed, and the capsular bag was removed due to marked zonular weakness, with deferred intraocular lens implant. Conclusions: Acute angle closure glaucoma in patients with RP may be rarely caused by spontaneous anterior lens dislocation. To our knowledge, this is the first report of spontaneous anterior lens dislocation in an RP patient, documented through photographs, anterior segment optical coherence tomography, and ultrasound biomicroscopy.
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Ectopia do Cristalino , Retinose Pigmentar , Humanos , Feminino , Adulto , Ectopia do Cristalino/complicações , Ectopia do Cristalino/diagnóstico , Retinose Pigmentar/complicações , Retinose Pigmentar/diagnóstico , Acuidade Visual , Facoemulsificação/métodos , Vitrectomia/métodos , Tomografia de Coerência Óptica/métodosRESUMO
PURPOSE: To report a case of macular hole and detachment occurring after the subretinal injection of Voretigene Neparvovec (VN) in a patient affected by atypical RPE65 retinal dystrophy with high myopia and its successful surgical management. CASE DESCRIPTION: We report a case of a 70-year-old man treated with VN in both eyes. The best corrected visual acuity (BCVA) was 0.7 LogMar in the right eye (RE) and 0.92 LogMar in the left eye (LE). Axial length was 29.60 mm in the RE and 30.28 mm in the LE. Both eyes were pseudophakic. In both eyes, fundus examination revealed high myopia, posterior staphyloma, and extended retinal atrophy areas at the posterior pole, circumscribing a central island of surviving retina. Both eyes were treated with VN subretinal injection, but a full-thickness macular hole and retinal detachment occurred in the LE three weeks after surgery. The patient underwent 23-gauge vitrectomy with internal limiting membrane (ILM) peeling and the inverted flap technique with sulfur hexafluoride (SF6) 20% tamponade. Postoperative follow-up showed that the macular hole was closed and the BCVA was maintained. CONCLUSIONS: Our experience suggests that patients with atypical RPE65 retinal dystrophy and high myopia undergoing VN subretinal injection require careful management to minimize the risk of macular hole and detachment occurrence and promptly detect and address these potential complications.
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Terapia Genética , Descolamento Retiniano , Distrofias Retinianas , Perfurações Retinianas , cis-trans-Isomerases , Humanos , Masculino , Perfurações Retinianas/etiologia , Perfurações Retinianas/genética , Perfurações Retinianas/cirurgia , Idoso , Descolamento Retiniano/genética , Descolamento Retiniano/etiologia , Descolamento Retiniano/cirurgia , cis-trans-Isomerases/genética , Distrofias Retinianas/genética , Vitrectomia , Acuidade Visual , Miopia/genéticaRESUMO
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PURPOSE: To describe the experience of our centre (Careggi University Hospital, Florence, Italy) in using a heads-up three-dimensional (3D) surgical viewing system in vitreoretinal surgery, making a comparison with the conventional microscope surgery. METHODS: We retrospectively analyzed data taken from 240 patients (240 eyes) with surgical macular diseases (macular hole and epiretinal membrane), retinal detachment or vitreous hemorrhage who underwent vitreoretinal surgeries, by means of the NGENUITY 3D Visualization System (Alcon Laboratories Inc., Fort Worth, TX, USA), in comparison with 210 patients (210 eyes) who underwent vitreoretinal surgeries performed using a conventional microscope. All surgeries were performed with standardized procedures by the same surgeons. We analyzed data over a follow-up period of 6 months, comparing the surgical outcomes (best-corrected visual acuity, anatomical success rate and postoperative complication rate) between the two groups. RESULTS: the 3D group included 74 patients with retinal detachment, 78 with epiretinal membrane, 64 with macular hole and 24 with vitreous hemorrhage. There were no significant differences in the demographic and clinical characteristics between the 3D group and the conventional group. We found no significant differences in outcome measures at three and six months follow-up between the two groups (p-value ≥ 0.05 for all comparisons). Surgery durations were similar between the two groups. CONCLUSIONS: In our experience, a heads-up 3D surgical viewing system provided comparable functional and anatomical outcomes in comparison with conventional microscope surgery, proving to be a valuable tool for vitreoretinal surgery in the treatment of different retinal diseases.
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PURPOSE: To evaluate the anatomical and functional outcomes of endoresection and adjuvant ruthenium (Ru)-106 brachytherapy for uveal melanoma (UM). METHODS: Retrospective case series of 15 UM patients (15 eyes) treated at our center (Careggi University Hospital, Florence). RESULTS: Six patients (40%) were male and nine were female (60%). The mean age of patients at the time of treatment was 61.6 years (±19.41). The mean BCVA at baseline was 20/50. In all cases UM originated from the choroid. The mean tumor thickness at baseline was 7.14 mm (±2.05), and the mean largest basal diameter was 11.2 mm (±1.92). A concurrent retinal detachment was diagnosed in 11 patients (73.3%). Two patients (13.3%) showed vitreous seeding at baseline. Eleven patients (73.3%) were treated with primary endoresection, while four patients (26.7%) were treated with a "salvage endoresection" after primary treatment failure (previous radiation treatment). The mean follow-up time was 28.9 months (±10.6). Thirteen out of fifteen patients were alive and showed no evidence of local recurrence or distance metastasis at the last follow-up visit. The treatment achieved local control of the disease in 14 out of 15 cases (93.3%). In one case, the patient underwent enucleation for disease recurrence. The overall survival rate at the end of the follow-up was 93.3%. The mean BCVA at last follow-up visit was 20/40. Treatment was well tolerated, without significant complications. CONCLUSIONS: Endoresection and adjuvant Ru-106 brachytherapy is a valuable conservative option for selected UM patients and can be used both as a primary treatment and as a salvage therapy. It can control melanoma and avoid enucleation, reduce radiation-related complications, and provide tumor tissue for chromosomal analysis and prognostic testing.
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In this review, we will focus on different non-invasive retinal imaging techniques that can be used to evaluate morphological and functional features in full-thickness macular holes with a prognostic purpose. Technological innovations and developments in recent years have increased the knowledge of vitreoretinal interface pathologies by identifying potential biomarkers useful for surgical outcomes prediction. Despite a successful surgery of full-thickness macular holes, the visual outcomes are often puzzling, so the study and the identification of prognostic factors is a current topic of interest. Our review aims to provide an overview of the current knowledge on prognostic biomarkers identified in full-thickness macular holes by means of different retinal imaging tools, such as optical coherence tomography, optical coherence tomography angiography, microperimetry, fundus autofluorescence, and adaptive optics.
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INTRODUCTION: Piggyback IntraOcular Lenses (IOLs), or supplementary secondary implant lenses, have been developed to provide a sufficient dioptric power in eyes with high refractive defects, which are not fully correctable after cataract surgery with single IOL in the range of powers available. These lenses can also be used for the correction of refractive errors that occurred for a wrong choice of the IOL power after cataract surgery. CASE DESCRIPTION: We report the case of a complete refractive success obtained in a patient with an abnormal cornea, with a central stable ectasia, with thinning, high myopic astigmatism and cataract, obtained with the implant of a primary posterior chamber IOL at the time of cataract surgery and a subsequent implant of a secondary piggyback, sulcus-based customized toric IOL (Camellens FIL 622-2 Toric Monofocal IOL, Soleko, Rome, Italy). CONCLUSIONS: This brief report demonstrates the utility of combining primary and piggyback IOLs implant for the correction of a complex spherical-cylindrical refractive defect in a case of abnormal cornea and cataract.
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Astigmatismo , Catarata , Lentes Intraoculares , Facoemulsificação , Humanos , Acuidade Visual , Implante de Lente Intraocular , Refração Ocular , Córnea/cirurgia , Astigmatismo/cirurgia , Astigmatismo/complicações , Catarata/complicaçõesRESUMO
PURPOSE: To describe the use of toric trans-plugs IOL to correct aphakia with significant corneal astigmatism. METHODS: Case report and literature review. RESULTS: A 62-year-old man with an history of aphakia in his left eye after a traumatic cataract extraction and significant corneal astigmatism was referred to our clinic. Surgery plan was cataract remnants removal and secondary IOL implantation of toric sutureless trans-scleral plugs fixated IOL (FIL SSF, Soleko, Italy). The postoperative course was uneventful. At 12 months, the IOL was well-centered and the optic alignment remained stable with minimal total astigmatism. The e uncorrected distance visual acuity (UDVA) was 20/25 Snellen while correct distance visual acuity (CDVA) was 20/20 with a manifest refraction of -0.25-50 40° D; the patient was satisfied by the visual outcome. CONCLUSION: This novel toric trans-scleral plug fixated lens provides valuable outcomes also for astigmatic correction for a comprehensive correction of the aphakia in patients with poor zonular support and high expectations for postoperative visual quality.
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Purpose: We investigated the chorioretinal microvascular changes in patients with retinitis pigmentosa (RP) by optical coherence tomography angiography (OCTA). Methods: Twenty-six patients (52 eyes) affected by RP were compared with 19 healthy controls (38 eyes). OCTA 3 mm × 3 mm macular scans were performed in all subjects. We evaluated the vessel density (VD) of the superficial capillary plexus (VD SCP), deep capillary plexus (VD DCP), choriocapillaris (VD CC), and choroid (VD choroid). We also evaluated the foveal avascular zone (FAZ) area, and the correlation between clinical and OCTA parameters. We also measured central retinal thickness (CRT) and subfoveal choroidal thickness (CT). Results: RP patients compared to healthy controls showed significantly lower VD SCP values (27.56% ± 15.37 vs. 49.39% ± 1.55; p-value < 0.0001), lower VD DCP values (38.43% ± 15.23 vs. 3.34% ± 0.26; p-value < 0.0001), lower VD CC values (46.02% ± 1.293 vs. 50.63% ± 0.4274; p-value = 0.0040), and lower VD choroid values (38.48% ± 15.23 vs. 3.34% ± 0.26; p-value < 0.0001). Even the FAZ area was significantly lower in RP patients (0.45 mm2 ± 0.35 vs. 0.26 mm2 ± 0.13; p-value < 0.0001). The FAZ area was larger with increasing age, both in control (r = 0.42; p = 0.012) and RP group (r = 0.46; p-value = 0.009). In RP patients, there was a statistically significant correlation between best-corrected visual acuity and VD SCP (r = 0.24, p-value = 0.04) and VD DCP (r = 0.52; p-value = 0.0004) and between subfoveal choroidal thickness and VD SCP (r = 0.43, p-value < 0.001) and VD DCP (r = 0.35, p-value < 0.001). Conclusions: In our study, OCTA reported relevant vascular alterations in RP patients in comparison with the healthy controls, in agreement with the published literature. These abnormalities were associated with choroidal atrophy and related to visual acuity loss. OCTA provided clinically significant information and may represent a reliable tool for the management of RP patients.
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PURPOSE: A case of bilateral choroidal metastasis from an adenoid cystic carcinoma of the submandibular gland is described. CASE DESCRIPTION: A 45-years-old woman with a history of metastatic adenoid cystic carcinoma presented with visual impairment in both eyes. Fundus images showed bilateral creamy-white choroidal masses. Optical coherence tomography revealed subretinal fluid with high reflective speckles and a "lumpy bumpy" anterior contour of the lesions. Fluorescein angiography showed a hypofluorescent pattern of the lesions in early arterial phases, and progressive late hyperfluorescence. A diagnosis of bilateral choroidal metastasis from adenoid cystic carcinoma was made. The patient was advised to underwent palliative chemotherapy, but she expired a few weeks after the diagnosis. CONCLUSION: Salivary gland carcinoma rarely metastasizes to the choroid, with few cases described in literature. In patients with a history of salivary glands tumor the possibility of choroidal metastatization should always be considered.
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Carcinoma Adenoide Cístico , Neoplasias da Coroide , Carcinoma Adenoide Cístico/diagnóstico por imagem , Corioide , Neoplasias da Coroide/diagnóstico , Feminino , Angiofluoresceinografia , Humanos , Pessoa de Meia-Idade , Glândula SubmandibularRESUMO
PURPOSE: To describe a surgical approach to remove a retained subfoveal Densiron 68 bubble in a case of recurrent inferior retinal detachment. METHODS: A 23-gauge pars plana vitrectomy was performed to remove the subfoveal silicone oil bubble and to treat the retinal detachment. Through a midperipheral retinal tear, we reached the subfoveal space by extending the retinal detachment up to the macular region. We inserted in the subretinal space a Charles cannula protected with a silicone tip to catch and aspirate the bubble. Intraoperative optical coherence tomography successfully guided the surgical maneuvers. RESULTS: We achieved the complete removal of the bubble from the subretinal space, and this led to the restoration of the retinal morphology with functional improvement, although the fovea developed atrophy. CONCLUSION: We reported a surgical approach for the removal of a retained subfoveal Densiron 68 bubble that combines a macular detachment together with the aspiration of the bubble using a protected Charles cannula. This technique may provide an alternative approach in the management of this challenging complication.
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Descolamento Retiniano , Humanos , Óleos de Silicone , Acuidade Visual , Vitrectomia/métodosRESUMO
PURPOSE: The aim of this study is to report our experience in the management of type 1 retinopathy of prematurity (ROP) or aggressive posterior retinopathy of prematurity (APROP) in premature infants, who received intravitreal bevacizumab (IVB) injections either as first-line monotherapy or as rescue therapy following laser therapy. METHODS: Single-center retrospective study on 37 patients (74 eyes) affected by sight-threatening ROP that underwent treatment either with IVB alone or laser photocoagulation followed by IVB at the Neonatal Intensive Care Unit of Careggi University Hospital of Florence, between 2008 and 2015. RESULTS: Seventeen patients were males (45.9%) and 20 were females (54.1%). The mean gestational age was 24 weeks and the mean birth weight was 610 g. Fifty-six eyes (75.7%) of 28 patients were diagnosed as type 1 ROP and 18 eyes (24.3%) of nine patients as APROP. Sixty-six eyes of 33 patients received IVB as first-line monotherapy, eight eyes of four patients were treated with IVB after laser photocoagulation treatment, as rescue therapy. The mean postmenstrual age at treatment was 33.9 weeks. All the patients received bilateral injections. None of the infants required repeat injections. The mean follow-up time was 59.6 months. All the patients achieved a good response to treatment, showing the regression of the proliferative phase and a complete peripheral retinal vascularization within 2 months from the injection of bevacizumab. One patient with a bilateral ROP had only a partial resolution of a vitreous hemorrhage in the left eye. The procedures were well tolerated without local or systemic adverse events during the follow-up. No infants showed recurrences. Ocular motility alterations appeared in four patients (10.8%), with the absence of stereopsis in six cases (16.20%). After treatment, spherical equivalent values in cycloplegic refraction ranged from -4.0 D to +5.0 D, with a mean value of +1.75 D. All the patients showed a normal neuropsychomotor development. CONCLUSION: Our study confirms the effectiveness and safety of IVB in the treatment of ROP, both as monotherapy and rescue therapy after laser photocoagulation, according to the published literature.
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Retinopatia da Prematuridade , Inibidores da Angiogênese/efeitos adversos , Bevacizumab/efeitos adversos , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Injeções Intravítreas , Fotocoagulação a Laser/métodos , Masculino , Retinopatia da Prematuridade/tratamento farmacológico , Retinopatia da Prematuridade/cirurgia , Estudos RetrospectivosRESUMO
Purpose: To compare the refractive results between sutureless scleral fixation intraocular lens (IOLs) (Carlevale, Soleko) and suture-free scleral fixation three-piece IOLs (Sensar AR40, Johnson & Johnson) for secondary implantation in patients with IOL dislocation or aphakia. Methods: This is a monocentric retrospective study on 28 patients (28 eyes) with sutureless scleral fixation Carlevale IOL and 25 patients (25 eyes) with suture-free scleral fixation three-piece IOL. Best-corrected visual acuity (BCVA) evaluation, refractive measures and IOL tilt evaluation with anterior segment optical coherence tomography were conducted at one, three, six and twelve months after surgery. Point Spread Function (PSF) was measured using a total ocular aberrometer. Results: BCVA in both groups improved since the postoperative visit at 1 month and reached a stable value at 3 months At month 12, mean BCVA was 0.23 logMAR in group one and 0.32 logMAR in group two. Mean IOL tilt angle at 12 months was 2.76° ± 1.87 in group one and 2.51° ± 1.80 in group two. PSF at 12 months was 0.18 ± 0.09 in group one and 0.15 ± 0.05 in group two. There were no statistically significant differences (p > 0.05) for all comparisons. The post-operative complications were similar within the two groups. Conclusions: Our results show that secondary IOL implantation has similar visual and surgical outcomes when a sutureless Carlevale lens scleral fixation and a suture-free scleral fixation three-piece IOL are used.
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PURPOSE: To describe features of uveitis-glaucoma-hyphema (UGH) syndrome, using Anterior Segment-Optical Coherence Tomography (AS-OCT) and Ultrasound Biomicroscopy (UBM) and to evaluate the diagnostic role of AS-OCT as an imaging technique alternative to UBM. DESIGN: Retrospective case series. METHODS: Four eyes of 4 patients with UGH syndrome were analyzed. All patients reported previous uncomplicated cataract surgery with in-the-bag implantation of single-piece-intraocular lens (IOL). They underwent at presentation complete ophthalmological examination and imaging with slit-lamp anterior segment photographs, UBM and AS-OCT. RESULTS: Although AS-OCT did not allow to visualize the structures behind the iris, it displayed a contact between IOL (plate and/or haptics) and iris and IOL tilting in 3 out of 4 eyes. AS-OCT directly detected the cause of UGH syndrome in one eye, 2 eyes required some expedients to display the iris chafing, like scans in mydriasis and/or patient's gaze direction change. AS-OCT did not allow to appreciate the IOL-iris contact (showed by UBM technique) only in one eye, probably due to the change of patient position from supine to sitting, and consequent anteriorization of iris diaphragm. Furthermore AS-OCT showed fine details, as capsular bag collapse and indirect signs of haptic malposition in 3 out of 4 eyes. CONCLUSION: AS-OCT is a non-invasive technique that allows to determine IOL position and IOL-uveal contact in selected cases of UGH syndrome. Considering AS-OCT and UBM advantages and limitations, AS-OCT should be used as first imaging modality when clinical diagnosis is uncertain. When UGH diagnosis cannot be verified using AS-OCT, UBM should be performed.
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Glaucoma de Ângulo Aberto , Glaucoma , Doenças do Cristalino , Lentes Intraoculares , Uveíte , Glaucoma/cirurgia , Glaucoma de Ângulo Aberto/cirurgia , Humanos , Hifema/diagnóstico , Hifema/etiologia , Hifema/cirurgia , Doenças do Cristalino/cirurgia , Implante de Lente Intraocular/efeitos adversos , Lentes Intraoculares/efeitos adversos , Estudos Retrospectivos , Síndrome , Tomografia de Coerência Óptica , Uveíte/diagnóstico , Uveíte/etiologia , Uveíte/cirurgiaRESUMO
PURPOSE: To describe the findings of four patients (four eyes) with optic disc melanocytoma (ODM), using multimodal imaging. METHODS: Retrospective case series. RESULTS: On ocular ultrasonography ODMs appeared as hyperechogenic lesions with moderate-to-high internal reflectivity. On blue-light fundus autofluorescence, ODMs showed total hypoautofluorescence, while, on infrared reflectance images appeared as bright and well-marginated lesions. MultiColor composite images showed reddish-brown lesions with well-defined margins. Swept-source optical coherence tomography revealed elevated lesions covering the optic disc with an irregular hyperreflective surface, dishomogeneous internal structure with hyperreflective dots, and posterior shadowing. In all cases optical coherence tomography angiography detected intratumoral blood vessels which were not detectable with fluorescein angiography. CONCLUSION: Multimodal imaging in ODM might be useful both at presentation, increasing the diagnostic accuracy, and at follow-up, providing helpful details, that can help to rule out the possibility of malignant transformation and other ocular complications.
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Purpose: Optical coherence tomography angiography (OCTA) is a non-invasive and objective tool for the evaluation of the retinal microvascular changes in Fabry disease (FD). We investigated changes in retinal vasculature in FD patients, and the possible correlation with systemic parameters, by using OCTA, and reviewed the current status of literature. Methods: Thirteen FD patients (eight females, five males, mean age 49.85 ± 14.7 years) were compared with 13 age- and sex-matched healthy controls. OCTA 3 × 3 mm macular scans were performed in all subjects. We evaluated the vessel density and vessel perfusion in distinct macular areas (whole, inner, and outer) of both the superficial capillary plexus (SCP VD and SCP VP) and of the deep capillary plexus (DCP VD and DCP VP). We also evaluated the foveal avascular zone (FAZ) metrics (area, perimeter, and circularity), and correlation between systemic and OCTA parameters. A literature review on the current understanding of OCTA in FD is then presented. Results: FD patients showed significantly lower SCP VD values in the whole area (17.37 ± 2.08 mm-1 vs. 18.54 ± 1.21 mm-1; p-value 0.022), as well as in the outer area (17.46 ± 2.10 mm-1 vs. 19.08 ± 1.14 mm-1; p-value 0.002), but not in the inner. Even the DCP VD was significantly lower in all the imaged areas: whole (17.75 ± 3.93 mm-1 vs. 19.71 ± 1.20 mm-1; p-value 0.024), outer (18.25 ± 4.17 mm-1 vs. 20.33 ± 1.20 mm-1; p-value 0.023), and inner (19.54 ± 4.17 mm-1 vs. 21.96 ± 1.55 mm-1; p-value 0.011). There were no significant differences in vessel perfusion parameters (both SCP VP and DCP VP ones) and FAZ. No significant correlations were found between the OCTA parameters and systemic parameters (maximal left ventricular wall thickness and glomerular filtration rate) in FD patients. Conclusions: OCTA can be considered as a promising non-invasive tool, which enables a quantitative evaluation of retinal vascular involvement in FD, despite the varying data reported in literature. Our results support the use of OCTA as an objective tool to evaluate retinal vascular abnormalities in FD. The utility of OCTA in FD needs to be validated by longitudinal studies taking into account the overall progression of the disease.
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PURPOSE: Fabry disease retinal vascular involvement has been widely reported, with narrowing of the retinal arterioles, dilation and irregularity of the retinal veins, and exaggerated tortuosity of the retinal vessels. We evaluated retinal vessel diameter in Fabry disease, by means of a dedicated software, aiming to provide a quantitative marker of retinal vascular network abnormalities in Fabry disease patients. MATERIAL AND METHODS: Observational case-control study evaluating different branches of vessels, peripapillary vessels (group A), temporal vascular arcades (group B), and second-order collaterals of the temporal arcades (group C). We obtained the vessel diameters values from eye fundus digital images of eight Fabry disease patients and eight age-sex matched controls, using a semiautomatic software. Mann-Whitney test was used to compare the Fabry disease group versus the control group. RESULTS: The difference between the average diameters of all the types of vessels considered were significantly smaller in Fabry disease patients compared to healthy controls, resulting in a decrease in size (mm) of 10.9% for group A, 7.8% for group B, and 7.4% for group C. The most evident difference between Fabry disease patients and controls was found in the largest vessels. CONCLUSION: A computer-assisted analysis of retinal vessel diameter in Fabry disease by means of dedicated software showed narrower retinal arteries in Fabry disease patients than in controls. Our data support the use of semiautomatic assessment of retinal vessel attenuation as an objective and reproducible method to evaluate retinal vascular alterations in Fabry disease, providing a clinical non-invasive tool for early diagnosis and disease monitoring.
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Doença de Fabry/diagnóstico , Doenças Retinianas/diagnóstico , Vasos Retinianos/patologia , Adulto , Idoso , Estudos de Casos e Controles , Diagnóstico por Computador , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
We retrospectively analyzed data from records of 48 patients (48 eyes) treated with gamma-knife (n = 18) or Ruthenium-106 brachytherapy (n = 30) for uveal melanoma, in our Ocular Oncology Unit between December 2013 and September 2019, with the aim to evaluate treatment outcomes, and incidence and risk factors for secondary glaucoma. Patients demographics and tumor characteristics at diagnosis were recorded. Follow-up data were collected regarding local tumor control, treatment complications, enucleation need, metastases occurrence and survival status. The median follow-up period was 33.7 months in the gamma-knife group and 26.2 months in the brachytherapy group. The mean tumor thickness, the largest basal diameter and the tumor volume were significantly higher in the gamma-knife group than in the brachytherapy group. The local tumor control rate was 100% in the brachytherapy group and 77.8% in the gamma-knife group. In the gamma-knife group, six patients were enucleated, no patient treated with brachytherapy underwent enucleation. The overall survival rate was 96.7% in the brachytherapy group and 94.44% in the gamma-knife group. Secondary glaucoma occurred in 10 patients after gamma-knife and in one patient after brachytherapy: it should be emphasized that larger lesions were treated with gamma-knife, whereas smaller tumors were selected for brachytherapy. We found a significative correlation of tumor thickness (P value = 0.043) and volume (P value = 0.040) with secondary glaucoma occurrence after gamma-knife treatment. Moreover, secondary glaucoma significantly correlated with radiation retinopathy in the gamma-knife group (P value = 0.009). This study shows preliminary clinical results that could be useful for further studies with more patients and longer follow-up.
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Braquiterapia/efeitos adversos , Glaucoma/etiologia , Melanoma/complicações , Radiocirurgia/efeitos adversos , Neoplasias Uveais/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Braquiterapia/métodos , Feminino , Glaucoma/fisiopatologia , Humanos , Masculino , Melanoma/cirurgia , Pessoa de Meia-Idade , Radiocirurgia/métodos , Estudos Retrospectivos , Neoplasias Uveais/cirurgiaRESUMO
INTRODUCTION: We report about a large retinal capillary hemangioma (RCH) with exudative retinal detachment and a macular fold, treated with Ruthenium-106 brachytherapy (Ru-106 BT) and scleral buckling surgery, followed by pars plana vitrectomy (PPV), for the removal of macular tractions. CASE DESCRIPTION: A 17-year-old boy was referred to our Ocular Oncology Unit for a large RCH in the left eye. BCVA was hand motion. The RCH measured 4.9 × 6.85 mm in basal diameters and 4.0 mm in thickness and was located in the mid-peripheral temporal retina. It was surrounded by extensive subretinal exudation, forming an exudative retinal detachment, with a retinal fold that extended from the lesion to the optic disc. We performed Ru-106 BT and at the moment of the plaque removal we placed a radial buckle with the aim to unbend the retinal fold. At 3-months follow-up the exudation decreased, we achieved the opening of the peripheral side of the retinal fold, but the macula was still detached. We decided to perform a lens sparing PPV, macular peeling and air tamponade, to remove the vitreoretinal tractions ab interno and to try to complete the opening of the macular fold. After 1-month BCVA was counting fingers, the retina appeared attached, also in the macular area, but the retinal fold remained partially close in the macular side. After 6 months the tumor was inactivated, the macula remained attached, unfortunately, the macular fold remained partially close. CONCLUSION: Ru-106 BT and scleral buckling concurrent approach can be an effective treatment modality in selected cases of large RCHs, followed by PPV to remove eventual vitreo-retinal tractions.