Case report: Introduction of an Ahmed valve implant tube into the ciliary sulcus using a guide-wire.

BACKGROUND: Glaucoma is a progressive optic neuropathy and it is the main cause of irreversible blindness worldwide. Intraocular pressure (IOP) is the only modifiable one and trabeculectomy is commonly considered the surgical "gold standard" to decrease IOP. In particular cases, other kind of surgeries, as the Ahmed Glaucoma Valve (AGV) implant, are a reliable alternative. Usually, the silicone tube of AGV surgery is inserted into the anterior chamber. Sometimes it is necessary to place the AGV silicone tube in the ciliary sulcus, especially in cases of endothelial decompensation. This surgical procedure is not always easy to perform. CASE PRESENTATION: This article describes and presents a technique for inserting the AGV tube into the posterior chamber using a guide-wire, in a total of 12 cases are reported. This procedure has been mostly applied in a group of pseudophakic patients who need AGV placement to control elevated IOP. In exceptional situations, this procedure was applied in pseudophakic patients previously with AGV tube implanted in the anterior chamber and with loss of endothelial cells or in phakic patients with previously implanted AGV anterior chamber tube, simultaneously to cataract surgery. CONCLUSION: The purpose of these cases presentations attended at our service is to demonstrate the passage of the AGV silicone tube was performed with the aid of a guide wire.

Modified ex-PRESS technique versus Ahmed glaucoma valve as primary glaucoma surgery for hereditary transthyretin amyloidosis glaucoma.

BACKGROUND: To compare the efficacy of modified Ex-PRESS technique (ExP) versus Ahmed Glaucoma Valve (AGV) as primary surgery in hereditary transthyretin amyloidosis (ATTRv) secondary glaucoma. METHODS: Retrospective study at the national amyloidosis centre. Success was defined as an IOP ≥ 6 mmHg and ≤ 21 mmHg with no need for further glaucoma surgery or laser trabeculoplasty and without loss of light perception at the time of the last follow-up. Secondary outcomes included surgical complications, need for hypotensive drugs, and endothelial cell loss. Patients submitted to previous glaucoma surgery were excluded. RESULTS: We included 180 eyes of 150 patients, 121 in AGV and 59 in ExP group. No significant baseline differences were found between groups. At the time of last follow-up, both groups exhibited significant intraocular pressure (IOP) reduction (p < .001) and number of glaucoma medications (p < .001). Kaplan-Meyer analysis showed higher cumulative probability of success for AGV group (80.1% vs. 41.1%, p < .001) and higher mean time to failure (54.5 vs. 36.9 months, respectively, p < 0.001) at 60 months follow-up. AGV showed lower hazard (HR 0.21, 95% CI [0.111-0.407], p < .001) for failure. CONCLUSION: AGV and ExP are safe and effective techniques in the treatment of ATTRv secondary glaucoma. However, AGV's efficacy seems to be more durable.

Scheimpflug-Based Corneal Biomechanical Analysis As A Predictor of Glaucoma in Eyes With High Myopia.

Purpose: To address if corneal biomechanical behavior has a predictive value for the presence of glaucomatous optical neuropathy in eyes with high myopia. Patients and Methods: This observational cross-sectional study included 209 eyes from 108 consecutive patients, divided into four groups: high myopia and primary open-angle glaucoma (POAG) - HMG, n = 53; high myopia without POAG - HMNG, n = 53; non-myopic with POAG - POAG, n = 50; non-myopic and non-POAG- NMNG, n = 53. Biomechanical assessment was made through a Scheimpflug-camera-based technology. Receiver operating characteristic curves were made for the discrimination between groups. Multivariable logistic regression models were performed to address the predictive value of corneal biomechanics for the presence of glaucoma. Results: Areas Under the Receiver Operating Characteristic (AUROCs) above 0.6 were found in 6 parameters applied to discriminate between HMG and HMNG and six parameters to discriminate between POAG and NMNG. The biomechanical models with the highest power of prediction for the presence of glaucoma included 5 parameters with an AUROC of 0.947 for eyes with high myopia and 6 parameters with an AUROC of 0.857 for non-myopic eyes. In the final model, including all eyes, and adjusted for the presence of high myopia, the highest power of prediction for the presence of glaucoma was achieved including eight biomechanical parameters, with an AUROC of 0.917. Conclusion: Corneal biomechanics demonstrated differences in eyes with glaucoma and mainly in myopic eyes. A biomechanical model based on multivariable logistic regression analysis and adjusted for high myopia was built, with an overall probability of 91.7% for the correct prediction of glaucomatous damage.

Modified Technique of Ex-PRESS® Filtration Device Combined with a Scleral Pocket for Hereditary Transthyretin Amyloidosis (hATTR) Secondary Open-Angle Glaucoma.

Purpose: To evaluate the effectiveness and safety of a modified approach using the Ex-PRESS® implant combined with a scleral pocket in the management of secondary open-angle glaucoma in hereditary transthyretin amyloidosis (hATTR) at our department. Methods: This was a retrospective analysis. The primary endpoints included Intraocular pressure (IOP) evaluation (baseline, 1st day, 1st week, 1, 3, 6, 12 months and at last follow-up) and number of hypotensive drugs (baseline, 6th, 12th months and at last follow-up). As secondary endpoints surgical complications, the need for additional glaucoma surgery and LogMAR BCVA were evaluated. Qualified and complete success were defined as ≥ 30% IOP decrease from baseline, with or without additional medications, respectively. The minimum follow-up was 12 months. Results: A total of 32 eyes were included with a mean follow-up of 2.4±2.9 years. IOP decreased significantly from baseline (27.4±4.4 mmHg) to 1st day (5.00±2.9 mmHg), 1st week (6.9±4.1 mmHg), 1st month (11.7±7.8 mmHg), 3rd month (11.6±6.1 mmHg), 6th month (13.1±6.8 mmHg), 12th month (12.0±3.5 mmHg) and last visit (11.8±2.4 mmHg), p<0.001. There was also a significant reduction in the number of antiglaucoma medications from baseline (3.8±0.6) and last follow-up (0.4±0.8), p<0.001. LogMAR BCVA remained stable (0.25±0.26 at baseline and 0.25±0.24 at last follow-up), p=0.767. Transient hypotony occurred in 17 eyes (53.1%), but only 11 (34.4%) exhibited anterior chamber shallowing and needed additional care, namely cycloplegic drops and viscoelastic injection. Complete surgical success was achieved in 22 eyes (68.8%) and qualified success in 6 eyes (18.8%). Four eyes (12.5%) needed additional glaucoma surgery. Conclusion: The modified ExPRESS® technique appears to be effective, especially when low levels of IOP are required. Additionally, fewer anti-glaucoma drugs were necessary. In the other hand, hypotony was a common side effect with this procedure, although all patients were properly handled, preserving the surgical outcomes.

XEN Gel Stent Implantation in Eyes With Previous Glaucoma Filtering Surgeries: A Case Series.

Background In this study, we aimed to analyze the efficacy and safety of ab interno XEN® gel stent (Allergan Inc., Dublin, Ireland) implantation in a series of eyes with open-angle glaucoma and a history of previous filtering glaucoma surgeries. Methodology This retrospective, single-center study included all eyes that underwent XEN gel implantation with a minimum follow-up of 18 months that had previously undergone a filtering glaucoma surgery. The main outcomes were intraocular pressure (IOP) variation (baseline, first day, first week, and first, third, sixth, 12th, and 18th months), the number of hypotensive eyedrops at 18 months, intraoperative and postsurgical complications, and the need for subsequent glaucoma surgery. Results A total of 10 eyes from nine patients were enrolled in the study. IOP significantly decreased from 24.0 ± 2.4 mmHg to 7.8 ± 1.6 mmHg on the first day, 9.4 ± 2.2 mmHg in the first week, and 12.3 ± 4.8 mmHg, 14.7 ± 4.3 mmHg, 13.3 ± 3.8 mmHg, 11.7 ± 1.9 mmHg, and 12.3 ± 1.9 at the first, third, sixth, 12th, and 18th month, respectively (p < 0.001), with a 49.5% reduction in IOP at the end of the follow-up. The number of hypotensive eyedrops decreased significantly from 3.5 ± 0.5 (3-4) to 0.7 ± 0.7 (0-2) (p = 0.026). No intraoperative or postsurgical complications were recorded. In total, seven (70%) eyes achieved controlled IOP of <18 mmHg without any anti-glaucomatous medications, and one (10%) eye with the use of topical prostaglandin. One (10%) eye was submitted to a surgical revision, and another needed an additional glaucoma surgery during follow-up, with appropriate IOP control at the last follow-up. Conclusions This case series aims to highlight that the XEN implant can be an option in eyes with IOP levels in the low 20s and previously failed filtering surgeries, with satisfactory efficacy and high safety.

Ahmed valve for secondary glaucoma in patients with hereditary transthyretin amyloidosis.

OBJECTIVE: To evaluate the surgical outcomes of patients with hereditary transthyretin amyloidosis (TTR-FAP) who underwent Ahmed glaucoma valve (AGV) implantation. METHODS: A retrospective cohort study was performed on patients with a diagnosis of TTR-FAP secondary glaucoma, who underwent AGV implantation in our department, between November 2010 and July 2019. The cumulative probability of treatment success was measured with Kaplan-Meier survival analysis. The primary outcome was success, defined as intraocular pressure (IOP) ≥ 6 mmHg and ≤21 mmHg with or without medication, with no need for further glaucoma surgery and without loss of light perception at last follow-up. Secondary outcomes were postoperative IOP, number of IOP-lowering medications, and rates of complications. RESULTS: The study included 114 eyes of 87 patients. The mean follow-up duration was 3.81 ± 2.11 years (y) [range, 1.00-8.28 y]. Compared to the preoperative values, the mean IOP was reduced from 28.20 ± 7.01 to 12.87 ± 3.76 mmHg at the final visit (p < 0.001), with a reduction in the number of medications from 3.89 ± 0.66 to 1.86 ± 1.43 (p < 0.001). Early and late postoperative complications occurred in 20 (17.09%) and 9 (7.89%) eyes, respectively. Kaplan-Meier analysis indicated probabilities of success of 0.98 at 1 y, 0.97 at 2 y, 0.95 at 3 y, 0.89 at 4 y, 0.77 at 5 y and 0.72 at 6 y. The linear correlation analysis showed a correlation between some characteristics of the natural history of TTR-FAP patients and AGV implantation success. CONCLUSION: Although glaucoma in TTR-FAP patients is very difficult to manage, AGV implantation is an effective and relatively safe procedure.

Corneal Biomechanics for Ocular Hypertension, Primary Open-Angle Glaucoma, and Amyloidotic Glaucoma: A Comparative Study by Corvis ST.

BACKGROUND: To evaluate biomechanical parameters of the cornea provided by Corvis ST in patients with ocular hypertension, primary open-angle glaucoma, and amyloidotic glaucoma and to compare with healthy controls. METHODS: This was a cross-sectional study of patients with ocular hypertension, primary open-angle glaucoma, and amyloidotic glaucoma that underwent Corvis ST imaging. Primary outcome was the comparison of corneal biomechanical parameters between study groups after adjusting for age, gender, Goldmann intraocular pressure (GAT-IOP), and prostaglandin analogues medication. Secondary outcome was the comparison of different IOP measurements in each group. RESULTS: One hundred and eighty-three eyes from 115 patients were included: 61 with primary open-angle glaucoma, 32 with amyloidotic glaucoma, 37 with ocular hypertension and 53 were healthy controls. Amyloidotic glaucoma group had smaller radius (p=0.025), lower deflection amplitude at highest concavity (p=0.019), and higher integrated radius (p=0.014) than controls. Ocular hypertension group had higher stiffness parameter at first applanation (p=0.043) than those with primary open-angle glaucoma, and higher stress-strain index (p=0.049) than those with amyloidotic glaucoma. Biomechanically corrected intraocular pressure was significantly lower than Goldmann intraocular pressure in group with primary open-angle glaucoma (p=0.005) and control group (p=0.013), and Goldmann intraocular pressure adjusted for pachymetry in group with primary open-angle glaucoma (p=0.01). CONCLUSION: Eyes with amyloidotic glaucoma have more deformable corneas, while eyes with ocular hypertension have less deformable corneas. These findings may be linked to the susceptibility to glaucomatous damage and progression. There were significant differences between Goldmann applanation tonometry and biomechanically corrected intraocular ocular pressure provided by Corvis ST.

Successful intravenous thrombolysis in a 14-year-old boy with ischemic stroke.

We report a healthy 14-year-old boy with an acute left middle cerebral artery stroke, treated 2 hours after the onset of symptoms with intravenous recombinant tissue plasminogen activator (r-TPA). Recanalization of the middle cerebral artery was documented with transcranial Doppler during the first 5 minutes of intravenous r-TPA perfusion, and progressive recovery of the neurological deficits occurred. Although lack of evidence regarding safety and efficacy in children precludes the recommendation of systematic use of r-TPA in pediatric stroke, we propose that this option should be considered and discussed with the parents, especially in older children presenting within 3 hours in centers with experience in adult thrombolysis.

Transscleral Cyclophotocoagulation in Familial Amyloidotic Polyneuropathy Secondary Glaucoma after Ahmed Valve Implantation: A Case Series Study.

PURPOSE: To investigate the outcomes of transscleral cyclophotocoagulation (TSCPC) after Ahmed glaucoma valve (AGV) implantation failure in patients with familial amyloidotic polyneuropathy (FAP) secondary glaucoma. MATERIALS AND METHODS: In this retrospective study, all patients with secondary FAP glaucoma who underwent AGV implantation between 2010 and 2019 in our tertiary center were assessed. Among all, those patients who needed TSCPC to control intraocular pressure (IOP) after AGV were selected. Demographic data, value of IOP, best-corrected visual acuity (BCVA), number of antiglaucomatous medications, surgical complications, and need for retreatment were collected. RESULTS: From a total of 124 eyes submitted to AGV implantation, 13 eyes (10.48%) needed TSCPC to control IOP. The median age at TSCPC was 49.50 years (43; 55.75), and the median period between AGV implantation and TSCPC was 1.63 years (1.00; 3.65). There was a significant decrease of IOP after the procedure, from 24.33 ± 4.76 to 11.33 ± 2.90 mm Hg at last visit (p < 0.001). A reduction in the number of antiglaucomatous medications was also noticed, from 3.92 ± 0.29 to 1.25 ± 1.42 (p < 0.001). There was no difference in BCVA (p = 0.502). After the procedure, there was an exuberant anterior chamber (AC) reaction in two eyes (16.7%), and one case developed a neurotrophic ulcer (8.3%). There was no need for re-treatment. CONCLUSION: Ahmed glaucoma valve implantation is a promising surgical procedure to treat patients with PAF secondary glaucoma, showing good results. For refractory cases, TSCPC seems to be effective and safe to control IOP. CLINICAL SIGNIFICANCE: There are only a few studies in current literature that address amyloid secondary glaucoma, maybe because it is a rare pathology worldwide. However, it has a high incidence in certain regions, including the north of Portugal. For the record, this is the first study describing the tool of cycloablative procedures in this type of glaucoma. HOW TO CITE THIS ARTICLE: Vieira R, Marta A, Figueiredo A, et al. Transscleral Cyclophotocoagulation in Familial Amyloidotic Polyneuropathy Secondary Glaucoma after Ahmed Valve Implantation: A Case Series Study. J Curr Glaucoma Pract 2021;15(1):32-35.

Use of Therapeutic Hypothermia in Sudden Unexpected Postnatal Collapse: A Retrospective Study.

INTRODUCTION: Sudden and unexpected postnatal collapse is a rare event with potentially dramatic consequences. Intervention approaches are limited, but hypothermia has been considered after postnatal collapse. The aim of this study was to analyse sudden and unexpected postnatal collapse cases that underwent therapeutic hypothermia in the five Portuguese hypothermia centres. MATERIAL AND METHODS: In this multicentre, retrospective and descriptive study, clinical, ultrasonography, amplitude-integrated electroencephalography and brain magnetic resonance findings of newborns with postnatal collapse that underwent therapeutic hypothermia are reported (2010 - 2018). Statistical analysis was performed by using IBM SPSS Statistics version 21. RESULTS: Twenty-two cases of sudden and unexpected postnatal collapse were referred for therapeutic hypothermia (82% outborn), all ≥ 36 weeks, with Apgar 5´ ≥ 8. Collapse occurred during the first two hours in 73% (all < 24 hours), 50% during skin-to-skin care, 55% related to feeding and 23% during co-bedding. Moderate-severe encephalopathy and severe acidosis were observed (median: Thompson score 16, pH 6.90, base deficit 22 mmol/L). Amplitude-integrated electroencephalogram was abnormal in 95% and magnetic resonance imaging showed severe brain injury in 46%. The mortality rate was 50%. A possible cause was identified in 27%. DISCUSSION: The incidence rate of 2.7 sudden cases of postnatal collapse per 100 000 births, is possibly under-estimated. All infants suffered the collapse in the first day, mostly within the first two hours, as reported before. Possible causes were identified in less than a third of cases, but multiple predisposing conditions were identified, suggesting that prevention may be possible. Newborn positioning and skin-to-skin care have been the most discussed practices. A significant proportion of infants had poor outcomes. Lower Thompson score, electroencephalogram amplitude normalization and normal magnetic resonance imaging seemed to indicate better outcomes. Although conclusive trials on therapeutic hypothermia after postnatal collapse are not available, its use has been considered individually. No severe adverse effects directly related to hypothermia were registered in this study, but the results do not allow drawing meaningful conclusions. CONCLUSION: In our national sample of 22 infants who suffered sudden and unexpected postnatal collapse and underwent therapeutic hypothermia, a significant proportion had poor outcomes. Absolute conclusions from our experience with hypothermia in postnatal collapse cannot be drawn, but systematic reporting of cases and long-term clinical evaluation would facilitate understanding of the real benefits of hypothermia. As this procedure has not been validated with clinical trials for this indication, its use should be considered on a case-by-case approach. The potentially avoidable nature of unexpected postnatal collapse is evident from its association with certain behaviours and risk factors. Surveillance practices during the first hours should be implemented, whilst the benefits of breastfeeding and skin-to-skin care should continue to be widely promoted.

Introdução: O colapso pós-natal súbito inesperado, apesar de raro, condiciona potenciais consequências dramáticas. As intervenções terapêuticas são limitadas, mas a hipotermia induzida tem sido considerada após estes eventos. O objetivo deste estudo foi analisar os casos de colapso pós-natal súbito inesperado submetidos a hipotermia induzida nos cinco centros portugueses que a realizam. Material e Métodos: Estudo descritivo retrospetivo multicêntrico dos recém-nascidos submetidos a hipotermia induzida após colapso pós-natal entre 2010 e 2018. Foram analisadas as variáveis clínicas, a monitorização por eletroencefalograma de amplitude integrada e imagem por ultrassonografia e a ressonância magnética cerebral. A análise estatística foi efetuada com apoio do IBM SPSS Statistics version 21. Resultados: Foram submetidos a hipotermia terapêutica por colapso súbito 22 recém-nascidos, 82% outborn, todos com 36 ou mais semanas de gestação e Apgar 5´ ≥ 8. A situação ocorreu nas primeiras duas horas de vida em 73% (todos com menos de 24 horas de vida), 50% no contacto pele-a-pele, 55% associados à amamentação e 23% durante partilha de cama. Os recém-nascidos observados apresentaram encefalopatia moderada a grave e acidose grave (mediana: Thompson 16, pH 6,90, défice bases 22 mmol/L). Entre os recém-nascidos, 95% registaram alteração no eletroencefalograma e 46% padrões graves de ressonância cerebral. A taxa de mortalidade foi de 50%. Identificaram-se possíveis causas em 27%. Discussão: Estimou-se uma incidência de 2,7 casos de colapso pós-natal súbito inesperado por cada 100 000 nascimentos, um valor possivelmente subestimado. O colapso ocorreu no primeiro dia em todas as crianças, a maioria nas primeiras duas horas, tal como descrito em publicações anteriores. Identificaram-se possíveis causas em menos de um terço dos casos, mas múltiplas condições predisponentes foram referidas, o que sugere a possibilidade de adoção de medidas preventivas. O posicionamento do recém-nascido e o contacto pele-a-pele têm sido as práticas mais discutidas. Uma proporção significativa das crianças teve uma evolução desfavorável. Um desfecho mais positivo parece ter ocorrido nos casos em que se verificaram valores inferiores na escala de Thompson, normalização do eletroencefalograma de amplitude integrada e ressonância magnética normal. Embora não estejam disponíveis ensaios conclusivos sobre a utilização da hipotermia terapêutica após o colapso pós-natal, o seu uso tem sido considerado individualmente. Nesta revisão não foram observados efeitos adversos diretamente relacionados com o procedimento, mas os resultados não permitem obter conclusões significativas. Conclusão: Na nossa amostra nacional de 22 crianças que sofreram colapso súbito pós-natal e submetidas a hipotermia terapêutica, uma proporção significativa teve uma evolução desfavorável. A nossa experiência e a raridade da entidade clínica não permitem delinear conclusões precisas sobre a aplicação da hipotermia induzida no colapso pós-natal súbito inesperado, pelo que se considera essencial a prevenção. O benefício desta terapêutica poderá ser clarificado através do registo sistemático dos casos e do seguimento a longo prazo das crianças. Embora não existam ensaios clínicos que permitam a sua validação após estes eventos, a hipotermia induzida deve ser uma opção a considerar individualmente. A associação do colapso pós-natal com determinados comportamentos e fatores de risco evidenciam a sua potencial prevenção. Devem ser implementadas estratégias de monitorização nas primeiras horas de vida que permitam simultaneamente a contínua promoção da amamentação e do contacto pele-a-pele.

Biometric Predictability in Combined Cataract Surgery and Ahmed Glaucoma Valve Implantation Depending on Tube Position.

PURPOSE: To compare the predictability of biometric results in patients undergoing cataract surgery combined with Ahmed glaucoma valve (AGV) implantation according to tube position. METHODS: A retrospective cohort study was performed in patients who underwent phacoemulsification surgery combined with AGV implantation with the tube in posterior (group PC) and anterior (group AC) chamber, between November 2012 and April 2020. The main outcome was the mean biometric prediction error, according to tube position, using different formulas. RESULTS: The study included 49 eyes of 36 patients, 23 eyes in group PC and 26 eyes in group AC. Gender (p=0.774), age (p=0.822), type of glaucoma (p=0.168), preoperative correct distance visual acuity (p=0.139), axial length (p=0.765), anterior chamber depth (p=0.351), keratometry (p=0.577) and intraocular lens power (p=0.608) were similar between groups. Only preoperative intraocular pressure was higher in group PC (p=0.005). The mean prediction errors using Haigis, SRK/T, Hoffer Q, Holladay 1, Barrett Universal II, Kane and Hill RBF formulas were all positive in group PC (hyperopic) and all negative (myopic) in group AC. In group PC, there was no significant difference in prediction error between these formulas (p>0.05). In group AC, the formula with the worst prediction error was Haigis (p=0.001), and the best was Barrett Universal II (p=0.043). CONCLUSION: The biometric predictability and expected final refraction in phacoemulsification surgery combined with AGV implantation are modified by the position of the tube.

The Role of Multimodal Approach in the Assessment of Glaucomatous Damage in High Myopes.

PURPOSE: To compare the optic disc structure and peripapillary retinal function between high myopes with and without glaucoma and to address the differential role of papillary optical coherence tomography angiography (OCT-A) and circumpapillary microperimetry (cpMP) on the diagnosis and prognosis of this patients. PATIENTS AND METHODS: It is a cross-sectional study including 30 high myopic patients (60 eyes), divided into 15 with (GG) and 15 without glaucoma (NGG). Demographic and clinical data were collected from patient records. Papillary structure (peripapillary retinal nerve fiber layer thickness, ppRNFLTs) and vascularization (small vessel densities, SVD´s) were assessed with SD-OCT (RTVue XR Avanti, with AngioVue system, Optovue®). cpMP was carried out with the MP-3 microperimeter (Nidek®). RESULTS: The GG were older, had lower best-corrected visual acuities and higher intraocular pressures and axial lengths (p<0.001). The GG showed lower values in all ppRNFLTs (p<0.05), lower values in all SVDs (p<0.001), except the SVD-inside disc (p=0.638) and lower retinal sensitivities within all cpMPs (p<0.001). The adjusted analysis computing the best two parameters per exam revealed that the anatomical model including the ppRNFLT-inferior and ppRNFLT-temporal and the vascular model including SVD-inferior and SVD-superior had the best discrimination power between groups, with cross-validated AUROCs of 0.9599 and 0.9921, respectively. CONCLUSION: Despite the apparent superiority of the papillary vascular study, a multimodal approach including the papillary anatomic and circumpapillary microperimetric assessments can be the new way on the diagnosis and prognosis of glaucoma in high myopia.

Two siblings with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome.

PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) is a benign sporadic syndrome of unknown cause. We report 2 siblings diagnosed with this syndrome. The second case started crisis simultaneously with recurrence of crisis after a 3-year free interval in her brother. This temporal relation suggests environmental factor acting in genetically predisposed children.

Pigment deposits in the retrolental space of Berger: a rare feature of pigment dispersion syndrome? / Depósitos de pigmento no espaço de Berger: uma característica rara de síndrome de dispersão pigmentar?

ABSTRACT Pigment dispersion syndrome is associated with clinical features such as Krukenberg's spindles, trabecular pigmentation, Scheie's stripe and Zentmayer's ring. Another less common feature of this syndrome is retrolental pigment deposits due to anterior hyaloid detachment or a defect in the Wieger's ligament. We present two cases of pigment deposits on the posterior lens capsule. In both cases, there is bilateral dispersion of pigment throughout the anterior segment. The retrolental deposits are unilateral in the first case and bilateral in the second. Both patients report a history of ocular trauma. This is a possible important clinical sign of pigment dispersion syndrome, rarely described.

RESUMO A síndrome de dispersão pigmentar associa-se a sinais clínicos característicos como fuso de Krukenberg, hiperpigmentação da malha trabecular, linha de Scheie e anel de Zentmeyer. Um sinal menos comum dessa síndrome é o depósito de pigmento posterior ao cristalino, que ocorre por um descolamento da hialoide anterior ou um defeito no ligamento de Wieger. Apresentamos dois casos de depósitos de pigmento posterior à cápsula posterior do cristalino. Em ambos os casos, existia dispersão bilateral de pigmento por todo o segmento anterior. No primeiro caso, os depósitos eram unilaterais e, no segundo, estavam presentes em ambos os olhos. Este pode corresponder a um sinal potencialmente importante da síndrome de dispersão pigmentar, raramente descrito.

Influence of bromazepam on cortical interhemispheric coherence.

Benzodiazepines are among the most commonly prescribed medications due to their therapeutic efficacy in reducing anxiety and inducing sleep. Consequently, they have been widely employed in the pharmacological treatment of several disorders. Nevertheless, few studies have analyzed the effects of bromazepam in electroencephalographic activity (EEG). The present study aimed at investigating the modulatory effects of this drug on brain dynamics. Specifically, the effects of bromazepam (3mg) on EEG coherence were tested in a double-blind experiment. The sample, consisting of 10 healthy subjects (5 male and 5 female), was submitted to ten minutes of EEG recording. The electrophysiological measure (coherence) was analyzed across three experimental conditions: bromazepam, placebo 1, and placebo 2. Results indicate that bromazepam significantly increases cortical interhemispheric coherence.

The effects of bromazepam on the early stage of visual information processing (P100).

The early stages of visual information processing, involving the detection and perception of simple visual stimuli, have been demonstrated to be sensitive to psychotropic agents. The present study investigated the effects of an acute dose of bromazepam (3 mg), compared with placebo, on the P100 component of the visual evoked potential and reaction time. The sample, consisting of 14 healthy subjects (6 male and 8 female), was submitted to a visual discrimination task, which employed the "oddball" paradigm. Results suggest that bromazepam (3 mg) impairs the initial stage of visual information processing, as observed by an increase in P100 latency.

Consecutive bilateral decompression retinopathy after mitomycin C trabeculectomy: a case report.

BACKGROUND: After a successful trabeculectomy, a sudden intraocular pressure decrease may alter the intracranial to intraocular pressure ratio and cause decompression retinopathy. Frequent Valsalva maneuvers may also play a role in its pathogenesis. This condition may manifest as multiple retinal hemorrhages, edema of the optic disc, macular edema, or a sudden decrease in visual acuity postoperatively. Outcomes for patients are usually good, with spontaneous resolution occurring within a matter of weeks. It has been rarely reported in the literature as a bilateral condition. CASE PRESENTATION: We present a case of consecutive bilateral decompression retinopathy in a 54-year-old severely obese Caucasian woman (body mass index 37 kg/m(2)) with open angle glaucoma and a poor history of medical therapeutic compliance, who chose surgical treatment based on her inability to consistently use ocular drops. Our patient underwent a trabeculectomy with mitomycin C in both eyes, with surgeries taking place 3 months apart. After the first surgery, 2 weeks postoperatively, she complained of decreased visual acuity. Examination of her right eye fundus revealed multiple retinal hemorrhages and disc edema. There was a similar pattern in her left eye, this time including maculopathy. Her visual acuity and fundoscopic changes resolved spontaneously over a period of a month in both cases. Currently, our patient has well-controlled bilateral intraocular pressure, ranging between 14 and 16 mmHg, without hypotensive medication. CONCLUSIONS: Decompression retinopathy is a potential complication after glaucoma surgery, but has rarely been described as a bilateral consecutive condition. A comprehensive approach could help to anticipate its occurrence and manage it.

Invasive fungal infection in neonatal intensive care units: a multicenter survey.

This study assesses the epidemiology of invasive fungal infection (IFI) in Portuguese Neonatal Intensive Care Units (NICUs) and compares the effectiveness and safety of antifungal therapies. A survey concerning the period 2005-2010 was carried out in NICUs of Greater Lisbon. Among 10 473 admitted neonates, 44 cases were identified, 29 among extreme low birth weight neonates (65.9%). Cumulative incidence rate was 0.42% (95%CI 0.309-0.559). A central vascular catheter was present before IFI in all cases. Candida albicans and Candida parapsilosis were the most frequent isolates. The initial antifungic was fluconazole in 22 cases and liposomal amphotericin B (L-AmB) in 18. Therapy was switched in 10 patients on fluconazole and 3 on L-AmB. Case fatality rate was 11.4% (95%CI 4.39-23.91). No serious adverse drugs reactions (SADRs) or clinical side effects were observed. The knowledge of the local epidemiology helps to identify adequate prophylactic and treatment strategies.

Source Imaging of P300 Visual Evoked Potentials and Cognitive Functions in Healthy Subjects.

The P300 event-related potential (EPR) is regarded as a neurophysiological indicator of cognitive processing of a stimulus. However, it is not known whether the P300 is a unitary component recorded on the scalp as a result of the activity of a specific intracerebral structure, or if it represents the sum of underlying components that may reflect the activation of broadly distributed intracerebral structures. The objective of the present experiment was to investigate possible correlations among the source(s) involved in the generation of the P300 and their possible neurocognitive function. The visual-evoked potential (VEP) was elicited by the oddball paradigm and analyzed after employment of sLORETA (standardized low-resolution electromagnetic tomography). The window of the P300 wave encompasses the period during which the response to the target and nontarget condition differs significantly (≈375 ms to ≈465 ms, with a peak at ≈422.5 ms). The results showed sequential and what appeared to be logical activation patterns of specific structures (specific for the processing of the stimulus used here) after presentation of the target stimulus. The peak of the P300 wave represented activation of the parahippocampal gyrus, which is responsible for upgrading memory in response to a target stimulus.

Protocolo de assistência do serviço de condicionamento físico para indivíduos com doenças neuromusculares e musculoesqueléticas / Assistance protocol physical conditioning department for individuals with neuromuscular and musculoskeletal diseases

O condicionamento físico supervisionado por profissionais de Educação Física inserido em um programa de reabilitação para pacientes com distúrbios neuromusculares e musculoesqueléticos visa o aumento da força muscular, melhora da coordenação motora, equilíbrio dinâmico, capacidade aeróbia, capacidade funcional e da aptidão física voltada à saúde. Trata-se de importante intervenção para prevenir e atenuar a sinergia entre a perda de função física e a exacerbação de várias comorbidades que comprometem a independência funcional, modificar fatores de risco, aumentar a qualidade de vida e longevidade com redução da mortalidade. O objetivo desse artigo é apresentar o protocolo de assistência do Serviço de Condicionamento Físico do Instituto de Medicina Física e Reabilitação do Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo (IMREA-HCFMUSP), elaborado para que a intervenção de exercícios físicos seja adequada, respeitando o perfil e limitações físicas desses pacientes, bem como na observação de seus marcadores clínicos.

The Physical Conditioning Department supervised by physical education professionals included in the rehabilitation program for neuromuscular and musculoskeletal disorders aims to increase muscular strength, improve motor capacity, dynamic balance, aerobic capacity, functional capacity and physical fitness for health. It is an important function to prevent and attenuate a synergy between physical function and the exacerbation of several comorbidities, compromising functional independence, removing risk factors, increasing quality of life and longevity with mortality. The objective of this article is to present the physical conditioning protocol of the Institute of Physical Medicine and Rehabilitation of the Hospital das Clínicas of the Faculty of Medicine of the University of São Paulo (IMREA-HCFMUSP), elaborated so that the intervention of physical exercises is adequate, respecting the profile and physical limitations of these patients, as well as in the observation of their clinical markers.