[Cardiac amyloidosis: State of art in 2022]. / Les amyloses cardiaques : état des lieux en 2022.

The 3 main types of cardiac amyloidosis are linked to two protein precursors: AL amyloidosis secondary to free light chain deposits in the context of monoclonal gammopathy (mainly of undetermined significance or myeloma) and transthyretin amyloidosis (ATTR), comprising wild-type transthyretin amyloidosis (ATTRwt for wild type) and hereditary transthyretin amyloidosis (ATTRv for variant). These diseases are underdiagnosed and highly prevalent in common cardiac phenotypes in recent studies (heart failure with preserved ejection fraction, severe aortic stenosis, hypertrophic cardiomyopathy). Myocardial amyloid infiltration affects all cardiac structures and clinically promotes predominantly heart failure, conductive disorders and cardioembolic events. The search for extracardiac signs makes it possible to arouse diagnostic suspicion. Electrocardiogram, echocardiography and cardiac MRI can suspect cardiac amyloidosis. The diagnostic confirmation follows a simple algorithm including a systematic search for monoclonal gammapathy and a disphosphonate scintigraphy. Histological proof is necessary in case of AL or ATTR amyloidosis with concomitant monoclonal gammopathy in order to initiate specific treatment. Due to the late disease onset in ATTRv, genetic testing must be routine in all cases of ATTR. These diseases are no longer perceived as incurable since recent therapeutic innovations. A better knowledge of the disease is more than ever necessary.

[Ocular toxicity of targeted therapies with MEK inhibitors and BRAF inhibitors in the treatment of metastatic cutaneous melanoma]. / Toxicité oculaire des thérapies ciblées anti-MEK et anti-BRAF dans le traitement des mélanomes cutanés métastatiques.

INTRODUCTION: Cutaneous melanoma is a malignant tumor, which develops from dermal melanocytes. Targeted therapies have changed the therapeutic management of metastatic melanoma and improved the survival rate. Among the various targeted therapies, MEK inhibitors and BRAF inhibitors have demonstrated efficacy, but they may lead to ocular toxicity. The goal of this study was to assess the incidence of ocular complications caused by the use of MEK inhibitors and BRAF inhibitors and to report their clinical features and therapeutic management. MATERIAL AND METHODS: This retrospective, observational, descriptive, single center study was conducted between May 2015 and December 2019 and included all patients with metastatic cutaneous melanomas treated with MEK inhibitors and BRAF inhibitors in whom ophthalmic toxicity was suspected. The data collected were demographic data (age, sex), the type of MEK inhibitors and BRAF inhibitors used, the length of time from melanoma diagnosis, mean duration of ophthalmological follow-up, time differential between starting therapy and the emergence of ocular complications, initial and final logMAR visual acuity, biomicroscopic examination of the anterior segment, dilatated fundus examination, and treatment administered. RESULTS: Fifty-four eyes of 27 patients with a mean age of 61.3±14.3 were included. The mean time delay between melanoma diagnosis and initiation of treatment was 23.2±8 months. Twenty patients (74%) were treated with a combination of MEK inhibitors and BRAF inhibitors (trametinib/dabrafenib), 5 patients (19%) were treated with MEK inhibitor monotherapy (cobimetinib), and 2 patients (7%) were treated with BRAF inhibitor monotherapy (vemurafenib). The mean duration of ophthalmological follow-up was 77.8±29 days, and the delay between the start of therapy and the emergence of symptoms was 87.2±78 days. The mean initial visual acuity was 0.075±0.13 logMAR, and the final visual acuity was 0.01±0.03 logMAR. Twelve patients (44%) developed ocular complications due to the targeted therapy. In the patients who received combination trametinib/dabrafenib, 5 patients (18.5%) developed clinical signs of uveitis, from acute anterior uveietis to panuveitis, and 2 patients (7.4%) developed bilateral central serous chorioretinopathy; in the patients who received cobimetinib, 4 patients (14.8%) developed bilateral central serous chorioretinopathy; and one patient (3.7%) who received vemurafenib developed acute anterior uveitis. For these 12 patients with ophthalmic side effects, temporary discontinuation of therapy was chosen for six patients (22.2%), three patients (11.1%) received half the initial dose, and for three patients (11.1%), normal dosing was continued. CONCLUSION: The two main side effects of targeted therapies are uveitis for BRAF inhibitors and central serous chorioretinopathy for MEK inhibitors. A multidisciplinary approach including ophthalmologists, dermatologists and oncologists is essential in order to adapt treatment in the advent of these ocular complications.

Reduction in ionizing radiation exposure during minimally invasive anterior plate osteosynthesis of distal radius fracture: Naive versus deliberate practice.

This study aimed to decrease surgeon exposure to ionizing radiation through a new learning technique, "deliberate practice", which consists in improving performance by setting goals with feedback. The hypothesis was that exposure to ionizing radiation during distal radius fracture surgery using the minimally invasive plate osteosynthesis (MIPO) technique decreased faster with "deliberate" practice than with "naïve" practice. Radiographic dosimetry was measured in the first 30 fractures operated on by MIPO by 6 surgeons. The first 3 surgeons operated "naively" (Group 1) and the next 3 according to the "deliberate" procedure (Group 2). Group 2 received weekly feedback (number of exposed hands, number of fluoroscopic views, exposure duration, and X-ray dose). An expert, using fluoroscopic images and surgical videos, provided suggestions for improvement. Mean number of exposed hands was 23.66 in Group 1 and 1.9 in Group 2. Mean number of fluoroscopic views was 78.31 and 35.0, respectively. Mean X-ray exposure time was 74.34 and 32.89 s, respectively. Mean dosimetry was 1.40 mGy (and 0.59 mGy, respectively. The hypothesis was thus confirmed: dosimetry decreased faster in Group 2 than in Group 1. Teaching this deliberate practice should be generalized, to decrease the growth phase and increase the plateau phase of the learning curve.

[Ocular syphilis, the rise of a forgotten disease: Retrospective study of 18 cases diagnosed at Amiens University Hospital]. / La syphilis oculaire, retour d'une maladie oubliée : étude rétrospective de 18 cas diagnostiqués au CHU d'Amiens.

OBJECTIVES: A disturbing resurgence of syphilis has been observed in the past few years. Ocular involvement of syphilis is infrequent. The goal of our study was to analyze the demographic data and clinical features and to analyze visual outcomes in cases of ocular syphilis at Amiens UH between January 1, 2015 and December 31, 2019. MATERIAL AND METHODS: This descriptive, observational, single-center study included a retrospective cohort of patients who were diagnosed with ocular syphilis. The data collected were demographic data (age, sex and sexual orientation), history of risky sexual behavior, HIV status and potential co-infections, stage of syphilis, chief complaint, initial and final logMAR visual acuity, biomicroscopic examination of the anterior segment, dilatated fundus examination, extraocular clinical manifestations and treatment initiated. RESULTS: Twenty-four eyes of eighteen patients (17 men and 1 woman) with a mean age of 48±12 were included in the study. 9 patients were homosexual, and 9 were heterosexual. A history of risky sexual behavior was noted in 6 patients (33.3%), and 4 patients (22%) were HIV positive. 2 patients (11.1%) had primary syphilis, 14 patients (77.7%) had secondary syphilis and 2 patients (11.1%) had tertiary syphilis. All patients were symptomatic, and vision loss was the main ophthalmologic symptom. The mean initial visual acuity was -0.55±0.56 logMAR, and the final visual acuity was 0.04±0.07 logMAR. Posterior uveitis was the predominant type of involvement (42%), and 9 patients presented with neurosyphilis. 11 patients (61.1%) showed extraocular clinical manifestations. 9 patients (50%) received subcutaneous ceftriaxone 2g, 6 patients (33.3%) received daily intravenous benzylpenicillin G, 2.4 million IU, and 3 patients (16.6%) were treated with oral doxycycline 200mg. CONCLUSION: Ocular syphilis remains a diagnostic and therapeutic challenge because of the various ocular manifestations it provokes. Since this pathology can result in severe damage, every clinician who diagnoses uveitis should consider the possibility of syphilis so as to avoid any delay in treatment. Even though ocular syphilis remains a rare clinical entity, it is a potentially devastating infection.

Corneal haze post photorefractive keratectomy.

Corneal haze represents subepithelial corneal fibrosis, a manifestation of a pathological healing process. It occurs as a result of an epithelial-stromal lesion involving a break in the epithelial barrier. It is an inflammatory response that involves the migration, multiplication and differentiation of keratocytes into mature myofibroblasts, causing loss of corneal transparency. Although it is a transient phenomenon, this complication is feared following refractive photokeratectomy (PRK), because it can cause alterations in the quality of vision, refractive regression and decreased visual acuity. The severity of these symptoms is correlated with the severity of the corneal haze, which can be assessed clinically or by objective means such as corneal densitometry measurement. The frequency and severity of corneal haze increase with the depth of photoablation in PRK and are therefore increased during the treatment of severe ametropia. Considering that no consensus exists, the application of mitomycin C (MMC) intraoperatively and topical corticosteroids postoperatively are conventionally used to inhibit collagen synthesis, sometimes in combination with various protocols depending on the center or surgeon. This review of the literature reports the current knowledge on corneal haze, in order to better understand it and optimise its prevention in the context of a decreased MMC supply, which has occurred in the past and could recur in the future.

[Refractive surgery for hyperopia]. / Hypermétropie et chirurgie réfractive.

Hyperopia is a refractive error in which light is focused behind, instead of on, the retina. Clear vision can be obtained by accommodation, but in the long run, this results in eye strain. Hyperopia can be classified as low [≤2.00 diopters (D)], moderate (2.00-4.00 D) and high (>4.00 D). Detailed preoperative evaluation is necessary and essential to obtain good postoperative results. Various surgical techniques can be proposed to correct this ametropia. The main techniques used act either by modifying the corneal curvature with the Excimer laser or by implanting a phakic intraocular lens. The anatomical peculiarities of the hyperopic eye (small corneal diameter, short axial length, narrow anterior chamber or large kappa angle) make refractive surgery for hyperopia a considerable challenge. Large optical ablation zones now allow correction of high hyperopia by reducing the risks of optical aberrations and regression. The patient must be informed and understand the postoperative course, which differs from that of surgery for myopia.

Eyelid Chemical Burns: A Multidisciplinary And Challenging Approach.

During second- and third-degree eyelid sulfuric acid burns, many surgeons prefer to wait until primary wound separation occurs before grafting. However, this approach may miss the chance to recover the eyelids and can cause ectropion, resulting in delayed eyeball healing with exposure keratitis. We propose that early eyelid release and grafting makes a significant difference in long-term outcomes and improves eyeball healing. Here, we present the case of a woman who presented second- and third-degree burns of the eyelids secondary to physical domestic assault with acid, who had an early surgical management with a full-thickness skin graft. Ten days after surgery, we found that the graft had survived totally, and the donor site of the right arm had already healed. Eyelids were successfully grafted and the functions of both eyelids were well recovered, allowing complete cover of the eyeball. Two months after surgery, functional and cosmetic results were satisfying, with no postoperative lagophthalmos or difficulties with exposure-related problems. Case reports of eyelid chemical burns are very few. No specific and codified management of eyelid chemical burns was found in the literature search. This case report demonstrated that a multidisciplinary approach led by both ophthalmologists and plastic surgeons must be decided early (<6h) in order to achieve synergistic and coordinated management between the eye and the eyelid. There is a significant improvement in ocular healing with early excision and grafting of eyelids after sulfuric acid burn.

En cas de brûlure du 2ème ou du 3ème degré des paupières par acide sulfurique, de nombreux chirurgiens préfèrent attendre la séparation spontanée de l'escarre avant de greffer. Cette stratégie comporte le risque d'une cicatrisation défectueuse source d'ectropion, d'occlusion incomplète et de kératite. Nous conjecturons qu'une excision-greffe précoce améliore le pronostic à long terme de ce type de brûlure. Nous présentons le cas d'une femme victime d'une agression intra-familiale à l'acide sulfurique, souffrant de brûlure des 2ème et 3ème degrés des paupières traitée par excision-greffe de peau totale précoce. À J10, la greffe était totalement intégrée et le site donneur (bras droit) était cicatrisé. La fonction palpébrale était normale et l'occlusion oculaire complète. Ces bons résultats persistaient à 2 mois, sans lagophtalmie ni défaut d'occlusion, avec un aspect esthétique correct. Les rapports de brûlures chimiques des paupières sont peu fréquents et il nous n'avons pas trouvé de protocole dans la littérature. Ce cas clinique illustre la nécessité d'une analyse précoce (dans les 6h) par ophtalmologiste et plasticien afin de définir une stratégie coordonnée vis à vis du globe oculaire et de la paupière. L'excision-greffe précoce améliore la pronostic oculaire après brûlure par acide sulfurique.

[Signs, symptoms, and clinical forms of cataract in adults]. / Sémiologie et formes cliniques de la cataracte chez l'adulte.

Cataract is a partial or total opacification of the crystalline lens. In adults, cataract is acquired; the most common form is the age-related cataract. Assessment of the functional impact of a cataract is clinical. The common symptom is loss of visual acuity, but other symptoms (photophobia, monocular diplopia, myopic shift, change in color vision, etc.) may be found depending on the anatomical distribution of the opacities (nuclear, posterior subcapsular, cortical). Diagnosis is based on slit-lamp examination after pupillary dilation. This allows classification of the opacities according to their anatomical distribution and can help direct any etiologic work-up. A number of potential causes should be ruled out before concluding that a cataract is age-related. Cataracts may be iatrogenic, associated with other ocular or systemic disease, or induced by ocular trauma. Knowledge of the signs, symptoms, and clinical forms of cataract helps to establish proper indications for cataract surgery in accordance with preferred practice patterns in ophthalmology.

[Functional results and prognostic factors in open-globe ocular trauma with presenting visual acuity of no-light perception]. / Résultats fonctionnels et facteurs pronostiques des traumatismes oculaires à globe ouvert avec acuité visuelle initiale à perception lumineuse négative.

PURPOSE: To analyse the clinical ocular characteristics and determine prognostic factors for functional recovery in eyes presenting with no light perception (NLP) after open globe ocular trauma. MATERIALS AND METHODS: In this retrospective study were included all the patients with no light perception after open globe trauma who presented to Amiens University Hospital between October 2014 and June 2018. RESULTS: Fifteen eyes of 15 patients were included in this study. The main mechanism of the trauma was globe rupture (80 %, n=12). The most common location was zone III (66 %, n=10). The wound size was greater than 10mm in 9 patients (60 %). The ocular lesions included expulsion of the crystalline lens or posterior chamber intraocular lens, hyphema, retinal detachment, vitreous hemorrhage and ciliochoroidal lesions. Damage to the ciliary body was a negative prognostic factor for functional recovery (P=0.04). Nine patients remained with no light perception, whereas 6 patients experienced an improvement in visual acuity (2.3 logMAR in 3 patients, 0.7 logMAR in 1 patient, 0.4 logMAR in 1 patient and 0.2 logMAR in 1 patient). These 6 patients had undergone posterior vitrectomy due to vitreoretinal involvement (P<0.001). CONCLUSION: In the case of open globe trauma with no light perception on presentation, a functional recovery is possible if there is no irreversible anatomical damage.

[Cyclodialysis, a therapeutic challenge: Review of the literature on current practices]. / Cyclodialyse : un challenge thérapeutique, revue de la littérature sur les pratiques actuelles.

INTRODUCTION: Cyclodialysis is a rare condition that is difficult to manage. We present the case of a woman with a cyclodialysis complicated by chronic hypotony requiring two surgeries to achieve reattachment of the ciliary body. We also report the results of a review of the literature regarding the treatment of this condition. DESCRIPTION: This is a 46-year-old woman with history of trauma to the right eye. Examination revealed an intra-ocular pressure (IOP) of 7mmHg, a shallow anterior chamber and signs of chronic hypotony on fundus examination (vascular tortuosity, hypotony maculopathy) due to an extensive 360° cyclodialysis, confirmed by ultrasound biomicroscopy. Transcleral cryotherapy as a first-line approach did not achieve reattachment of the ciliary body. Secondary pars plana vitrectomy with gas tamponade (C2F6) reattached the ciliary body and restored the intraocular pressure (12mmHg) and normal fundus appearance. The patient recovered corrected visual acuity of 20/20. DISCUSSION: To our knowledge, there is no standardized management for cyclodialysis. The study of the literature available on the Medline database showed that direct cyclopexy remains the most common treatment, followed by vitrectomy with internal tamponade. Neither the extent nor the duration of the cyclodialysis can predict the visual recovery, which can be major even after weeks of hypotony. CONCLUSION: The management of cyclodialysis is not well-defined; it remains a true therapeutic challenge.