Bilateral Phlyctenular Keratoconjunctivitis in the Context of Hidradenitis Suppurativa: A Case Report and Literature Review.

PURPOSE: To report a case of severe bilateral phlyctenular keratoconjunctivitis (PKC) associated to hidradenitis suppurativa (HS). CASE REPORT: A 26-year-old male with reactivation of HS in the last few months presented with concurrent pain and vision loss secondary to bilateral PKC resistant to topical treatment. There were no other infectious or autoimmune disorders. Systemic immunosuppression was needed, with simultaneous improvement of the ophthalmological and dermatological findings. CONCLUSIONS: Different inflammatory eye diseases have been reported in the context of HS. Acute inflammation in HS reactivation would trigger an autoimmune response, acting as a common causal mechanism in this association. We have reported a new case of inflammatory eye disease - HS in the form of PKC, not previously described in the literature, and consistent with immune dysregulation where the systemic Staphylococcus aureus burden due to HS may act as an additional causal factor.

Bilateral neurosensory retinal detachment secondary to tadalafil treatment. / Desprendimiento neurosensorial retiniano bilateral secundario a tratamiento con tadalafilo.

A 53-year-old male with no systemic disorders, other than controlled arterial hypertension, presented with asymptomatic, bilateral neurosensory retinal detachment (NRD) detected during a routine revision. The patient reported the use of tadalafil (a phosphodiesterase-5 inhibitor [PDE5I]) for erectile dysfunction. Following suspension of the drug, subretinal fluid reabsorption was confirmed, with the persistence of chronic alterations in the optical coherence tomography (OCT) and the visual field. PDE5Is have ocular side effects, including exudative retinal detachment. Although no direct causal relationship has been confirmed, PDE5 inhibition at chorioretinal level produces vasodilatation, increased choroid hydrostatic pressure, and exudation into the subretinal space. In cases of NRD, a thorough assessment of the drug treatment history is crucial. Patients who use PDE5I drugs should be alerted to their potential ocular side effects.

Dual corneal involvement by endothelial and epithelial corneal dystrophies in Steinert's disease: A case of triple dystrophy.

INTRODUCTION: A case of dual corneal involvement due to Fuchs endothelial corneal dystrophy and epithelial basement membrane corneal dystrophy in a patient with Steinert's myotonic dystrophy type 1 is described, and a literature review on the triple association is made. CASE DESCRIPTION: A 52-year-old male diagnosed with myotonic dystrophy type 1 presented due to progressive bilateral vision loss during the past year. A full ophthalmological evaluation was made, with biomicroscopy, funduscopy, anterior segment optical coherence tomography, and endothelial cell count using specular microscopy. Exploration revealed bilateral superior palpebral ptosis, visual acuity 0.5 in the right eye and 0.3 in the left eye, and with an intraocular pressure of 11 and 10 mmHg, respectively. Biomicroscopy revealed map-dot-fingerprint lesions characteristic of epithelial basement membrane corneal dystrophy in both eyes, as well as abundant endothelial guttae due to Fuchs endothelial corneal dystrophy (stage II) and bilateral nuclear and posterior subcapsular cataracts. Specular microscopy in turn showed cell loss and a destructured endothelial map. Finally, anterior segment optical coherence tomography revealed the accumulation of epithelial basement membrane and hyperreflective endothelial excrescences corresponding to guttae. CONCLUSION: The association of Fuchs endothelial corneal dystrophy with myotonic dystrophy has been described and explained by a common genetic basis in the expansion of a CTG trinucleotide repeat, though this is the first reported case of the triple association of Fuchs endothelial corneal dystrophy, epithelial basement membrane corneal dystrophy, and myotonic dystrophy type 1. New mutations or still unknown genetic alterations could possibly explain the triple association reported in our case.

Retinal detachment with spontaneous dialysis of the ora serrata in a 13-year-old child with neurofibromatosis type 1: A case report.

A 13-year-old child diagnosed with neurofibromatosis type 1 who on a routine control presented with rhegmatogenous retinal detachment associated to dialysis of the ora serrata in the left eye (OS). There were no clinical signs or history of contuse ocular trauma. Neurofibromatosis produces alterations in fibroblasts of the cortex of the vitreous base. This results in deficient production of the collagen fibers that anchor the vitreous base to the pars plana and the peripheral neurosensory retina. Thus, suboptimal function of the fibroblasts explains spontaneous avulsion of the vitreous base. Such avulsion in turn is related to dialysis of the ora serrata.

Updating national diagnostic reference levels for interventional cardiology and methodological aspects.

The aim of this study is to propose national diagnostic reference levels (DRL) for updating in the field of interventional cardiology and to include technical details to help plan optimization. Medical physics experts and interventional cardiologists from 14 hospitals provided patient dose indicators from coronary angiography and percutaneous coronary interventions. Information about X-ray system dose settings and image quality was also provided. The dose values from 30,024 procedures and 26 interventional laboratories were recorded. The national DRLs proposed for coronary angiography and percutaneous coronary interventions were respectively 39 and 78 Gy·cm2 for air kerma area product (PKA), 530 and 1300 mGy for air kerma at reference point (Ka,r), 6.7 and 15 min of fluoroscopy time and 760 and 1300 cine images. 36% of the KAP meters required correction factors from 10 to 35%. The dose management systems should allow these corrections to be included automatically. The dose per image in cine in reference conditions differed in a factor of 5.5. Including X-ray system dose settings in the methodology provides an insight into the differences between hospitals. The DRLs proposed for Spain in this work were similar to those proposed in the last European survey. The poor correlation between X-ray systems dose settings and patient dose indicators highlights that other factors such as operation protocols and complexity may have more impact in patient dose indicators, which allows a wide margin for optimization. Dose reduction technology together with appropriate training programs will be determinant in the future reduction of patient dose indicators.

Primary multifocal anaplastic large cell lymphoma with eyelid involvement: a case report / Linfoma anaplásico de grandes células primário multifocal com envolvimento de pálpebra: relato de caso

ABSTRACT Ocular adnexal involvement in CD30+ lymphoproliferative disorders is rare. We report the case of a 73-year-old woman with a relapsing primary cutaneous anaplastic large cell lymphoma on her eyelid. A systemic extension study excluded extracutaneous involvement. Systemic chemotherapy resulted in an optimal response, with complete regression of the cutaneous lesions. There has been no recurrence during the 2 years of follow-up.

RESUMO O acometimento ocular adicional nos distúrbios linfoproliferativos CD30+ é raro. Relatamos o caso de uma mulher de 73 anos com linfoma de grandes células anaplásicas primárias recidivantes em sua pálpebra. A avaliação sistêmica excluiu envolvimento extracutâneo. A quimioterapia sistémica resultou em uma resposta ótima, com regressão completa das lesões cutáneas. Não houve recidiva durante 2 anos de acompanhamento.

Primary multifocal anaplastic large cell lymphoma with eyelid involvement: a case report.

Ocular adnexal involvement in CD30+ lymphoproliferative disorders is rare. We report the case of a 73-year-old woman with a relapsing primary cutaneous anaplastic large cell lymphoma on her eyelid. A systemic extension study excluded extracutaneous involvement. Systemic chemotherapy resulted in an optimal response, with complete regression of the cutaneous lesions. There has been no recurrence during the 2 years of follow-up.

Outcomes of Aortic Valve Replacement According to Surgical Approach in Intermediate and Low Risk Patients: A Propensity Score Analysis.

BACKGROUND: Previous trials have shown that, among high-risk patients with aortic stenosis, survival rates are similar for transcatheter aortic-valve implantation (TAVI) and surgical aortic valve replacement. The study aimed to compare the outcomes of aortic valve replacement according to the adopted surgical approach in intermediate and low risk patients. METHODS: This is a retrospective, observational, cohort study of prospectively collected data from 421 patients undergoing isolated aortic valve replacement between 2011 and 2015. A multinomial logit propensity score model based on preoperative risk factors was used to match patients 1:1:1 between conventional replacement (CAVR), minimally invasive (MIAVR) and TAVI groups, resulting in 50 matched three cohorts. RESULTS: After multinomial logit propensity score, the three groups were comparable in terms of preoperative characteristics. Mean age and Logistic EuroSCORE I of CAVR, MIAVR and TAVI groups were (84.2±5.1 vs. 82.3±4.8 vs. 85.6±4.9 years; p=0.002) and (11.4±3.6% vs. 8.3±3.4% vs. 15.8±5.4%; p<0.001) respectively. Overall mortality rates were similar for the three patient cohorts at one year. There were no significant differences related to stroke to 30 days. In the TAVI cohort, pacemaker implantation for new-onset total atrioventricular block became necessary in 30% of patients (p<0.001) and 16% of patients had some degree of paravalvular aortic regurgitation, which was more than mild (p<0.001). Total length of stay was shorter in the TAVI group when compared with surgical groups (11.5±5.3 vs. 10.1±6.9 vs 8.5±3.7 days; p=0.023). After discharge, the survival rate follow-up (average follow up: 46.7 months) was 70%, 84% and 72% for three cohorts (log Rank x2=2.40, p=0.3). CONCLUSIONS: In our experience, the three aortic valve replacement approaches offer very good results. Differences in the rate of complications were found between groups. Depending on patient's characteristics the Heart-Team group must offer the best surgical approach for each patient.

Coronary artery aneurysm formation following implantation of a bioresorbable vascular scaffold for in-stent restenosis.

Coronary artery aneurysm (CAA) formation is a rare complication of coronary intervention that may develop after implantation of bare-metal or drug-eluting stents. The etiology of this entity appears to be multifactorial and its prognosis is poorly understood, but it has been associated with an increased risk of stent thrombosis. To date few cases of CAAs related to bioresorbable vascular scaffold (BVS) implantation have been reported, and the development of CAA after BVS implantation for the treatment of in-stent restenosis (ISR) has not been previously described. Here we present two cases of CAA formation after BVS, which represent the first demonstration of CAA formation after the use of BVS for ISR.

Optical Coherence Tomography Findings in Patients With Stent Thrombosis.

INTRODUCTION AND OBJECTIVES: Stent thrombosis (ST) is a rare but potentially serious complication. Optical coherence tomography (OCT) provides high-resolution images and additional information to angiography in the study of this event. METHODS: Prospective study of patients with ST undergoing reintervention with OCT imaging. RESULTS: The study included a total of 40 consecutive patients with ST. Mean age was 69 ± 13 years and 83% were male. Early ST (≤ 30 days) was observed in 16 patients and late ST (> 30 days) in 24 patients. Stent thrombosis occurred in 17 bare-metal stents and 23 drug-eluting stents. In 34 patients (85%), adequate OCT images were obtained at the time of the ST. The predominant mechanism in early ST was stent malapposition (39%). In late ST, high frequencies of uncovered (46%) and malapposed struts (17%) were observed, especially in patients with drug-eluting stents. Furthermore, the presence of neoatherosclerosis was very high (67%) in patients with late ST. After intervention, improvements were observed in malapposition length and the amount of residual thrombus. CONCLUSIONS: OCT allows identification of the underlying mechanisms potentially involved in ST. This imaging modality is helpful in guiding reintervention in these patients, which improves the area and length of malapposition, as well as the maximal residual thrombus area.

Diagnostic accuracy of a hybrid approach of instantaneous wave-free ratio and fractional flow reserve using high-dose intracoronary adenosine to characterize intermediate coronary lesions: Results of the PALS (Practical Assessment of Lesion Severity) prospective study.

OBJECTIVES: We sought to investigate the diagnostic accuracy of instantaneous wave-free ratio (iFR) and high-dose intracoronary adenosine fractional flow reserve (IC-FFR) compared with classical intravenous adenosine fractional flow reserve (IV-FFR) to assess coronary stenosis severity. The usefulness of two hybrid strategies combining iFR and high-dose IC-FFR was also evaluated. BACKGROUND: Physiological assessment of intermediate coronary stenoses to guide revascularization is currently recommended. METHODS: Consecutive real-world patients with angiographically intermediate coronary stenosis (40-80% diameter stenosis) were prospectively included in the PALS (Practical Assessment of Lesion Severity) study. In every target lesion iFR, high-dose IC-FFR and IV-FFR were systematically measured to assess the accuracy of an hybrid sequential approach combining iFR and IC-FFR. RESULTS: A total of 106 patients with 121 intermediate coronary lesions were analyzed. Both, iFR and IC-FFR showed a significant correlation with IV-FFR (iFR: r = 0.60, 95%CI 0.48-0.70; IC-FFR: r = 0.88; 95%CI: 0.83-0.92). High-dose IC-FFR provided lower FFR values than IV-FFR (0.81 ± 0.08 vs. 0.82 ± 0.09, P = 0.25). Using a receiver-operating-characteristic curve an optimal iFR threshold of 0.91 for the screening test was identified. A sequential test strategy (initial iFR followed by IC-FFR only in lesions with iFR <0.91) yielded an excellent diagnostic accuracy (96.7%, 95%CI 96.7-99.1%) with a sensitivity, specificity, positive and negative predicted values of 100%, 94.7%, 91.8%, and 100%, respectively. A hybrid approach using the previously described iFR gray zone (0.85-0.94) also provided an excellent diagnostic accuracy (95%, 95%CI: 89.5-98.1%). CONCLUSIONS: In patients with intermediate coronary lesions a hybrid strategy by using a sequential approach of iFR and high-dose IC-FFR, provided a very good diagnostic performance to identify physiologically significant stenoses. © 2017 Wiley Periodicals, Inc.

Reliability of physiological assessment of coronary stenosis severity using intracoronary pressure techniques: a comprehensive analysis from a large cohort of consecutive intermediate coronary lesions.

AIMS: We sought to assess the reliability of the most frequently used intracoronary physiologic indices (including intravenous adenosine FFR [IV-FFR], intracoronary low-dose adenosine FFR [LD-IC-FFR], intracoronary high-dose adenosine FFR [HD-IC-FFR], Pd/Pa and iFR). We also sought to analyse factors affecting their reproducibility in a real-world patient population. METHODS AND RESULTS: A total of 91 lesions in 86 consecutive patients were included. Measurements of all physiological indices were repeated within a systematic standardised prospective protocol. All measured indices showed excellent test-retest reliability, with intraclass correlation coefficient (ICC) over 0.96. IV-FFR showed the highest coefficient of variation (CV) values among the studied measurements (Pd/Pa: 0.05; iFR: 0.10; LD-IC-FFR: 0.10; HD-IC-FFR: 0.08; IV-FFR: 0.12). Pd/Pa was significantly less variable than the other indices. On multivariate analysis, female gender, distal lesion location, history of hypertension or kidney failure, as well as presentation as an acute coronary syndrome, were associated with more variability in all physiological intracoronary measurements. CONCLUSIONS: The reliability of most frequently used intracoronary physiologic indices is high. Clinical and anatomic factors significantly influence the reliability of these physiologic indices.

Drug-eluting balloons in coronary interventions: the quiet revolution?

INTRODUCTION: Drug-eluting balloons (DEB) may be considered as a 'quiet revolution' in percutaneous coronary interventions. Early-generation DEB eluting paclitaxel proved to be very effective in animal models to reduce neointimal hyperplasia. Areas covered: Review of DEB efficacy in patients with coronary de novo lesions and in-stent restenosis (ISR). Expert opinion: Many randomized clinical trials and meta-analyses have demonstrated the value of DEB in patients with ISR. In this setting, DEB are safe and effective with clinical and angiographic results superior to plain balloon angioplasty and at least equivalent to first generation drug-eluting stents (DES). In selected 'de novo' lesions (bifurcation lesions, small vessels, diffuse disease, myocardial infarction) DEB represent an attractive alternative although additional evidence in these 'niche' indications is still required before a widespread clinical utilization can be recommended. Recently, new generation DEB have become available, offering interesting new possibilities (paclitaxel and also sirolimus) for coronary interventions. Further studies are required to compare the results of novel generation DEB with those of second-generation DES.

Atrioventricular Septum Pseudoaneurysm As Late Complication After Repeated Mitral Valve Replacement.

We report the case of a pulsatile mass found in a patient who presented for a routine echocardiogram. The mass turned out to be an exceedingly rare mitral-subannular pseudoaneurysm involving the membranous atrioventricular septum with systolic expansion protruding into right atrium, discovered late after repeated multiple valve replacement surgery. Although these pseudoaneurysms may present asymptomatically, surgical intervention might be indicated because of the risk of rupture. This report describes this rare finding, discusses possible pathophysiological mechanisms, and underscores the importance of multimodality imaging to achieve correct identification and delimitation to guide surgical intervention in such cases.

Mother-and-child catheter-facilitated optical coherence tomography: A novel approach to improve intracoronary imaging.

BACKGROUND: Our aim was to prospectively analyze the usefulness of mother-and-child catheter approach with the GuideLiner® (GL) catheter extension system to overcome some potential limitations associated with the use of optical coherence tomography (OCT). METHODS: We included consecutive patients referred to our institution for coronary angiogram in whom OCT image acquisition was obtained using GL. From February 2015 to May 2016, a total of 19 procedures (18 patients) were prospectively included. RESULTS: In more than two thirds of the procedures, the OCT catheter was unable to cross the coronary stenosis prior to the use of GL. The right coronary artery was the most frequently involved coronary vessel (63%). The main reason for using GL (47% of cases) was poor engagement of the coronary ostium and lack of support of the guiding catheter (47%). Eventually, in 18 out of 19 procedures, a good quality OCT image was obtained and, when indicated, successful percutaneous coronary intervention with stent implantation was performed. Of note, there were no serious complications related to the use of GL during OCT imaging. CONCLUSIONS: Optical coherence tomography through a catheter extension system is a useful technique able to overcome some of the limitations associated with this imaging technique ensuring adequate blood clearance and thus optimal image quality.