Retinal microvascular changes in patients with familial mediterranean fever: a study based on optical coherence tomography angiography / Alterações microvasculares da retina em pacientes com febre mediterrânea familiar: estudo baseado em angiotomografia de coerência óptica

ABSTRACT Purpose: In this study, we aimed to show whether a difference exists between retinal and choroidal microcirculation findings between patients with familial Mediterranean fever and healthy controls. Methods: Thirty-two patients with familial Mediterranean fever and 30 healthy controls were included in the study. All the patients underwent a complete ophthalmologic examination, including best-corrected visual acuity and intraocular pressure measurement. The AngioVue optical coherence tomography angiography device (Optovue, Fremont, CA) with split-spectrum amplitude-decorrelation angiography was used to evaluate and examine the retinal microvascular structure. Three-dimensional en face Optical coherence tomography angiography images were obtained by examining the macula using the 3 x 3 mm scanning protocol in the Angio Retina mode and the optic nerve using the 3 x 3 mm scanning protocol in the Angio Disk mode. All the patients' right eyes were examined. Results: A total of 62 subjects were included in the study, of whom 32 (53.3%) were female and 30 (46.7%) were male. No statistically significant difference was found between the two groups in terms of optic nerve head or radial peripapillary capillary vessel density. On examination, the superficial capillary plexuses were statistically similar between the two groups, but the deep capillary plexus vessel density in the parafovea, superior hemi, temporal, and superior areas were significantly lower in the patients with familial Mediterranean fever. Conclusions: We found that the capillary plexus vessel density was significantly lower in the parafovea, superior hemi, temporal, and superior regions in the patients with familial Mediterranean fever than in the control group. Therefore, OCTA, a noninvasive study, may be useful for understanding the systemic effects of familial Mediterranean fever.

RESUMO Objetivos: Este estudo teve como objetivo mostrar se há diferença entre os achados da microcirculação retiniana e coroidal entre pacientes com febre mediterrânica familiar e um grupo controle saudável. Métodos: Trinta e dois pacientes com febre mediterrânica familiar e 30 controles saudáveis foram incluídos neste estudo. Todos os pacientes foram submetidos a um exame oftalmológico completo, incluindo a acuidade visual melhor corrigida e medida da pressão intraocular. O aparelho AngioVue Optical coherence tomography angiography (Optovue, Fremont, CA) com angiografia de correlação de amplitude de espectro dividido foi utilizado para avaliar e examinar a estrutura microvascular da retina. As angiotomografias de coerência ópticas en face tridimensionais foram obtidas examinando o protocolo de varredura macular 3 x 3 mm (modo angio retina) e o nervo óptico com o protocolo de varredura 3 x 3 mm (modo angio-disco). Todos os olhos direitos dos pacientes foram examinados. Resultados: Foram incluídos neste estudo, 62 sujeitos, dos quais 32 (53,3%) eram do sexo feminino e 30 (46,7%) do sexo masculino. Não houve diferença estatisticamente significativa entre os dois grupos quanto à densidade dos vasos da cabeça do nervo óptico ou da densidade dos vasos capilares peripapilares radiais. Durante o exame, os plexos capilares superficiais foram estatisticamente semelhantes entre esses dois grupos, mas a densidade profunda dos vasos do plexo capilar nas areas parafovea, hemi superior, temporal e superior foram significativamente menores nos pacientes com febre mediterrânica familiar. Conclusões: Verificamos que a densidade dos vasos do plexo capilar foi significativamente menor nas regiões parafovea, hemi superior, temporal e superior em pacientes com febre mediterrânica familiar em comparação com o grupo controle. Portanto, pode ser útil usar a angiotomografia de coerência óptica, por tratar-se de um estudo não invasivo, para melhor compreensão dos efeitos sistêmicos da febre mediterrânica familiar.

Quantitative Evaluation of Pupil Responses in Patients with Prolactinomas Being Treated with Dopamine Agonists.

The aim of this study was to determine whether the dopamine agonist (DA) drug cabergoline used in the treatment of prolactinoma causes autonomic dysfunction by measuring static and dynamic pupillary responses. The study included 25 eyes from 25 patients who were receiving DA for the treatment of prolactinoma and 25 eyes from 25 healthy individuals. Static and dynamic pupillary responses were measured by automatic quantitative pupillometry. The scotopic pupillary diameter was found to be significantly higher in patients receiving DA medication compared with the control group, while pupil contraction time and pupillary dilatation latency were significantly lower. DA drug use changes static and dynamic pupillary responses, probably by increasing sympathetic tone. Pupillometry can be used as a non-invasive method to provide information about changes in the autonomic nervous system in patients receiving such drug therapy.

Retinal Microvascular Changes in Patients with Multiple Myeloma: A Study Based on Optical Coherence Tomography Angiography.

Purpose: Ocular microvascular networks and variables were analyzed using optical coherence tomography angiography (OCTA) in patients with multiple myeloma (MM) who had no pathological findings in their routine ophthalmologic examinations.Methods: The study included 31 patients with a diagnosis of MM and 30 healthy controls. The ophthalmologic examination findings and OCTA measurements of the participants were prospectively analyzed. We evaluated the superficial capillary plexus (SCP) vessel density (VD) and deep capillary plexus (DCP) VD in macular region, radial peripapillary capillary (RPC) VD, optic nerve head (ONH) VD and the foveal avascular zone (FAZ) area.Results: The samples were gender-balanced, and there were no significant differences in age or gender between the MM and control groups. From the OCTA, all the ONH-VD measurements, except for the peripapillary and superotemporal parameters, were found to be significantly lower in MM patients than in the control group; the same was found for the whole image, inferonasal, superonasal, and superotemporal RPC-VD values; for all the SCP-VD values, except for the inferior hemi and temporal; and for all the DCP-VD values. It was also observed that the deep FAZ area was wider in the MM group than in the control group.Conclusions: We detected decreased VD in deep and superficial macular retinal areas, papillary, peripapillary regions, suggesting decreased blood flow and possible ischemia in MM patients. Therefore, obtaining information on ischemia by using a noninvasive and easily measurable method such as OCTA, may be beneficial in terms of follow-up and treatment but this needs to be supported by further, larger studies.

The effectiveness of automatic pupillometry as a screening method to detect diabetic autonomic neuropathy.

PURPOSE: This study aimed to compare static and dynamic pupil responses of diabetic patients with and without nonproliferative diabetic retinopathy (DR) and normal healthy individuals under different lighting conditions via quantitative automated pupillometry. METHODS: Forty patients with DM with nonproliferative DR (group 1), 40 patients with DM without DR (group 2), and 40 healthy controls (group 3) underwent a complete ophthalmologic examination. Static pupillometry [scotopic pupil diameter (PD), mesopic PD, low photopic PD, and high photopic PD] and dynamic pupillometry (resting PD, contraction amplitude, latency, duration, velocity of contraction, dilatation latency, and duration and velocity at rest) were measured via automatic quantitative pupillometry. RESULTS: Analysis of variance revealed that scotopic PD [F(2, 117) = 6.42; p = 0.02], mesopic PD [F(2, 117) = 3.20; p = 0.04], and low photopic PD [F(2, 117) = 4.86; p = 0.009] were significantly different among the groups. Scotopic PD and low photopic PD were significantly lower in group 1 than in group 2 (p = 0.03 and p = 0.03, respectively). Meanwhile, the resting diameter, velocity of pupil contraction, and velocity of pupil dilatation were found to be significantly lower (p = 0.02, p = 0.01, and p = 0.008, respectively), and the duration of pupil contraction was significantly higher in group 1 than in group 3 (p = 0.03). CONCLUSION: Both DM patients with and without nonproliferative DR exhibited pupillary involvement. Automated pupillometry may be an easily applicable, noninvasive screening option for reducing mortality and morbidity rates associated with diabetic autonomic neuropathy.

Ophthalmic manifestations in patients with ectodermal dysplasia syndromes.

BACKGROUND: Ectodermal dysplasia (ED) is a disorder that results from abnormal formation of at least two of the four major ectodermal derivatives in the developing embryo. The ectoderm of the embryo forms the skin, teeth, hair and nails, sweat glands and part of the eyes. OBJECTIVES: The aim of this article is to reveal ophthalmologic symptoms and signs as multidisciplinary, reliable criteria for ectodermal dysplasia. MATERIAL AND METHODS: In this retrospective study, 24 patients with ED were analyzed from the recorded data. Ophthalmological examination of the patients, who had previously received the diagnosis of ED in the dental department, was done. During the examination, ocular symptoms related to tear film, corneal changes, lacrimal duct, periorbital hyperpigmentation, alteration lashes and eyebrows were evaluated. RESULTS: The age ranged between 3-45, and the mean and standard deviation (Mean ± SD) was 15.8 ± 7.4 years. The number of males was 13 (54.2%) and females, 11 (45.8%). Eighteen patients (75.0%) suffered from ocular complaints related to the ocular surface. In 11 of the patients with ED, there were dry eye symptoms. While the mean age of cases with eye involvement was 17.5, it was 23.1 in cases with dry eye symptoms. CONCLUSIONS: In the study, it was observed that, in patients with ED, ocular complaints, particularly dry eye symptoms, may increase as age advances.

The subconjunctival use of cetuximab and bevacizumab in inhibition of corneal angiogenesis.

PURPOSE: To investigate the effects of cetuximab and bevacizumab on experimental rat model of corneal angiogenesis. METHODS: The right eyes of 28 male Sprague-Dawley rats were included in silver nitrate cauterization-induced corneal angiogenesis model. They were divided into four groups: (1) silver nitrate cauterization-induced and 0.15 ml serum physiologic was given to the angiogenesis group, (2) bevacizumab was given 1.25 mg to the bevacizumab group, (3) cetuximab was given 5 mg to the cetuximab group, and (4) 1.25 mg bevacizumab plus 5 mg cetuximab were given to the bevacizumab + cetuximab group. All eyes were exposed to the treatment on days 1, 4, and 7 of the experiment, and drugs were given subconjunctivally. The left eyes were untreated and used as sham. On day 8, the treated eyes were evaluated biomicroscopically. Then, the rats were sacrificed, and corneal specimens were prepared for histopathologic examinations. RESULTS: The degree of angiogenesis inhibition was observed as 50.8% in bevacizumab, 54.3% in cetuximab, and 15.8% in bevacizumab + cetuximab groups by biomicroscopic evaluation. According to the histopathological and immunohistochemical findings obtained from the present study, the amount of angiogenesis was determined to have decreased considerably in both the bevacizumab and cetuximab groups; also, relatively less inhibiton was observed in the bevacizumab + cetuximab group. CONCLUSION: Subconjunctival injection of cetuximab and bevacizumab is effective in reducing corneal angiogenesis in silver nitrate cauterization induced angiogenesis model of rats. Further investigation is needed to assess the potential side-effects of the drugs, especially cetuximab.

Intravitreal bevacizumab combined with panretinal photocoagulation in the treatment of open angle neovascular glaucoma.

PURPOSE: To evaluate the clinical efficacy of intravitreal bevacizumab (IVB) combined with panretinal photocoagulation in patients with open angle neovascular glaucoma (NVG). METHODS: Nine patients (9 eyes) with NVG participated in this study. Patients received IVB (1.25 mg) as the initial treatment for NVG and were followed up for at least 4 months. IVB was offered as the first treatment of choice to patients with NVG. Panretinal photocoagulation was performed as soon as feasible after the second week and completed in all patients the fourth week after IVB. The main outcome measures are resolution of INV, inhibition of peripheral anterior synechia (PAS), and controllability of intraocular pressure (IOP). RESULTS: The mean follow-up period was 5.6+/-1.4 months (range, 4-9 months). The mean IOP before treatment was 35.1+/-9.7 mmHg (range, 24-56) under medication before IVB treatment. After IVB and after combined treatment, the mean IOP was reduced to 22.8+/-8.1 mmHg (range, 9-33) and 13.0+/-4.0 mmHg (range, 7-20), respectively. The mean referral INV was 3.6+/-0.4 grade (range, 3-4) and reduced to 1.6+/-0.4 (range 1-2) grade after IVB and 0.6+/-0.8 (range 0-2) grade after combined therapy. By IVB, combined panretinal photocoagulation recurrence of INV was not observed. CONCLUSIONS: In NVG, IVB treatment can reduce iris and angle neovascularization and inhibits further PAS formation temporarily. Panretinal photocoagulation inhibits neovascularization constantly. Therefore, management of open angle NVG is more feasible with bevacizumab combined with panretinal photocoagulation.

Incidence, clinical findings and management of intraoperative floppy iris syndrome associated with tamsulosin.

PURPOSE: To determine the risk ratios and incidence of intraoperative floppy iris syndrome (IFIS) during cataract surgery in patients using tamsulosin, and to assess management strategies for IFIS. METHODS: We performed a non-randomized, observational, prospective study, in which 594 eyes of 579 patients undergoing cataract surgery were enrolled. Surgeons were masked to the patients' drug history. Usage or non-usage of tamsulosin, duration of tamsulosin use, presence or absence of IFIS, management of IFIS and intraoperative complications were recorded in the patients' theatre notes. RESULTS: Twelve of 15 (80%) IFIS patients were taking systemic tamsulosin. Twelve of 23 (52%) patients using tamsulosin showed features of IFIS. The odds ratios (ORs) and relative risk (RR) ratios show strong positive correlations between tamsulosin use and IFIS. The ORs and RR ratios and the 95% confidence intervals (CIs) are as follows: OR 206.5 (95% CI 50.9-836.5); RR 99.3 (95% CI 30.0-327.8). There were no statistically significant differences between patients with or without IFIS, who were using tamsulosin, in terms of age or duration of tamsulosin use (p > 0.05). Seven eyes (46.6%) with IFIS were successfully managed with epinephrine. Eight eyes (53.4%) with IFIS needed iris hooks. CONCLUSIONS: Patients using tamsulosin appear to be at high risk of IFIS during cataract surgery. The occurrence of IFIS may not be affected by duration of tamsulosin use or age. Epinephrine may be effective in approximately 50% of eyes with IFIS. The iris hook procedure represents an effective management strategy in IFIS.

Persistent triamcinolone acetonide particles on the posterior lens capsule after intravitreal injection.

We report a rare case in which triamcinolone acetonide particles gathered on posterior lens capsule after injection of intravitreal triamcinolone acetonide. Intravitreal injection of triamcinolone acetonide is useful in the treatment of macular edema; however, accumulation of triamcinolone acetonide particles on the posterior lens capsule may decrease visual acuity and requires surgical treatment.

Conjunctival-limbal autograft, amniotic membrane transplantation, and intraoperative mitomycin C for primary pterygium.

We evaluated and compared the safety and efficacy of the conjunctival-limbal autograft, amniotic membrane transplantation, and intraoperative application of mitomycin C in 94 patients undergoing primary pterygium surgery and concluded that conjunctival-limbal autografting and amniotic membrane methods were more effective and safer than intraoperative mitomycin C.

Pseudoxantoma elasticum, as a repetitive upper gastrointestinal hemorrhage cause in a pregnant woman.

Pseudoxantoma elasticum is a rare, hereditary, multisystemic disease affecting the skin, eye, and cardiovascular system. A twenty-eight-year-old female has presented to emergency unit with the complaint of gastrointestinal hemorrhage. This patient, who had been monitored in the gastroenterology clinic more than 10 times in the past 8 years, noted a repetitive hemorrhage during her previous pregnancy in her history. The examination of the patient revealed the following signs and symptoms: atrophy in the epithelium of the retina pigment; typical angioid streaks and peau d'orange finding in the fundus; thinning of the retinal nerve fiber in OCT (optic coherence tomography); bilateral and reticular papillary lesions with yellowish-color in the neck region (plucked chicken appearance); presence of bleeding foci in fundus; and nephrocalcinosis in kidneys. In light of these symptoms, the patient was diagnosed with pseudoxantoma elasticum. Skin biopsy confirmed the pseudoxantoma elasticum diagnose. PXE is an uncommon, hereditary disease. Early diagnosis of pseudoxantoma elasticum cases, is important for minimalizing systemic complications and informing the other family members through genetic counseling.

Pediatric orbital multifocal cavernous hemangiomas associated with bilateral arachnoid cysts of the middle cranial fossa. Case report and review of the literature.

Cavernous hemangiomas of the orbit are benign vascular growths that commonly occur in adults and account for 6% of all intraorbital tumors. Multifocal intraorbital cavernous hemangiomas are quite rare. The reported incidence of arachnoid cysts accounts for only 1% of lesions that occupy intracranial space, and they are nearly always sporadic and single. So far, the authors have not encountered any study reporting the coexistence of bilateral arachnoid cysts of the middle cranial fossa and orbital multifocal cavernous hemangiomas. In this report, they describe a 10-year-old boy with such a hemangioma that included the eyelid, conjunctiva, and retrobulbar space. His was the first case of a surgically treated pediatric orbital multifocal cavernous hemangioma associated with bilateral arachnoid cysts of the middle cranial fossa. An association between arachnoid cysts, intracranial cavernous malformations, and bilateral arachnoid cysts and metabolic disorders has been reported. The authors report on the coexistence of orbital multifocal cavernous hemangiomas and bilateral arachnoid cysts of the middle cranial fossa. Based on these observations, they believe that all patients with bilateral temporal arachnoid cysts should be screened for genetic disorders. In addition, the possibility of orbital cavernous malformations should be kept in mind for the follow-up period, and patients should be evaluated for possible symptoms of this condition.