An orbital perivascular epithelioid cell tumor (PEComa) in a 9-year-old boy: Case report and review of the literature.

Perivascular epithelioid cell tumors (PEComas) are a family of benign neoplasms characterized by smooth muscle and melanocytic differentiation. Orbital cases are rare. A 9-year-old male presented with a slowly growing orbital mass. Magnetic resonance imaging (MRI) revealed a well-defined orbital mass without intracranial extension. The microscopic appearance of the complete resection specimen showed large nests of epithelioid cells with wide cytoplasm containing melanin pigment and round to oval nuclei with mild cytonuclear atypia and low mitotic activity. Immunohistochemistry was positive for HMB45 and negative for melanA, smooth muscle actin, desmin and S-100 protein. Pangenomic RNA-sequencing identified an in-frame NONO-TFE3 rearrangement, and clustering data showed that the tumor's gene expression profile was grouped with other previously studied PEComas. A diagnosis of orbital pigmented PEComa with uncertain malignant potential associated with a NONO-TFE3 rearrangement was made. There was no recurrence after 1 year of follow-up.

[Effect of keratoplasty on the quality of life of keratoconus patients]. / Effet de la kératoplastie sur la qualité de vie des patients atteints de kératocône.

Corneal deformations caused by keratoconus produce high levels of optical aberration (OA). Despite appropriate optical correction, these alter the quality of vision and diminish the patient's quality of life, especially since the affected population is predominantly young and of working age. When thinning is too severe or corneal transparency too impaired, a corneal transplant may be considered. In this study, we compare the quality of life of patients with keratoconus in the early (stages 1 and 2) or advanced (stages 3 and 4) stages of the Krumeich classification, as well as patients who have had keratoconus treated by keratoplasty. Quality of life was assessed using the NEI-VFQ 25 questionnaire, the most widely used for keratoconus. An aberrometric examination (OQAS®; HD Analyser, Visiometrics, Terrassa, Spain) was also performed to assess patients' quality of vision. Our results show that keratoplasty provides an improvement in quality of life compared with advanced-stage keratoconus in the areas of distance (p=0.0083) and near vision (p=0.029) activities. This improvement also applies to Best-Corrected Visual Acuity (BCVA) (p=0.032) and transparency (OSI) (p=0.049). Our study shows that keratoplasty improves corneal transparency, and it is interesting to note that it improves patients' quality of life over the long term.

[Intraoperative opacification of a scleral-fixated Carlevale (Soleko®) intraocular lens]. / Opacification peropératoire d'un implant fixé à la sclère Carlevale (Soleko®).

Thermal shock can cause intraoperative opacification of a Carlevale (Soleko®) implant. This is a rare phenomenon which resolves spontaneously. It is crucial to recognise this phenomenon in order to avoid unnecessary and potentially harmful explantation decisions for the patient.

FIL SSF intraocular lens opacification after gas tamponade: A case series.

PURPOSE: To report three cases of postoperative opacification of sutureless scleral-fixed hydrophilic intraocular lens (FIL SSF IOL, Soleko, Italy) after gas tamponade. Two cases occurred after pars plana vitrectomy and one case after Descemet membrane endothelial keratoplasty. CASE REPORT: Two diabetic patients underwent a FIL SSF IOL implantation after posterior capsular rupture during cataract surgery. Rhegmatogenous retinal detachment (RRD) was observed in one patient during the initial surgery. A second patient developed a RRD five months after surgery. Both RRDs were treated with pars plana vitrectomy and perfluoroethane (C2F6) gas tamponade. A few days after the surgery, C2F6 was observed in the anterior chamber of both patients. Two months after gas tamponade, opacification of the anterior surface of the IOL was observed. The third patient was a 74-year-old woman, who underwent a combined Descemet membrane endothelial keratoplasty (DMEK) and FIL SSF IOL implantation. Two rebubblings with sulfur hexafluoride (SF6) retreatments were required due to corneal graft detachment. One month later, an opacification of the anterior surface of the IOL was observed. Explantation with implantation of iris-claw IOL was decided, which resulted in an improvement of BVCA. Analysis of the IOL showed a positive Von Kossa staining, indicating calcification of the IOL. We performed a review of all the cases of FIL SSF IOL implantation in our centers. The overall rate of FIL SSF IOL opacification was 2.1% (3/140). Amongst patients treated with gas tamponade, the rate of opacification was 27.3% (3/11). Although FIL SSF IOL implantation appears to be an effective option for the treatment of aphakia, caution should be exercised regarding the risk of opacification following gas tamponade, especially since these patients are at risk of retinal detachment.

[Safety and efficiency of epithelium-off accelerated corneal cross-linking for progressive keratoconus in pediatric patients]. / Efficacité et tolérance du cross-linking cornéen épithélium-off accéléré dans le traitement du kératocône évolutif de l'enfant.

In adults, the management of keratoconus has evolved in recent years to achieve a well-codified treatment algorithm. The technique of cross-linking (CXL) has allowed us to stabilize the progression of keratoconus and has been largely developed. It is very effective, with few postoperative complications. Currently, there is no specific keratoconus management protocol for children. As we already know that keratoconus usually evolves more rapidly in children, we might consider whether a stabilizing treatment should be proposed as first-line therapy at the time of diagnosis. We carried out a retrospective study including patients less than 18 years of age with keratoconus who consulted the ophthalmology department at Edouard Herriot hospital in Lyon between 2013 and 2017. The main study parameter was whether or not CXL was performed. The other parameters were gender, age, ethnicity, eye rubbing, presence or absence of atopic disease, maximum keratometry (Kmax), minimal pachymetry, best corrected visual acuity (BCVA) and spherical equivalent. Forty-eight eyes of 34 patients were included. We found that two-thirds of the patients were Caucasian boys. Half of the patients had allergies, and over 60% of patients rubbed their eyes regularly. Only six percent of patients had a family history of keratoconus. The mean age of the patients was 14 (7-18) years at the time of diagnosis. Thirty-four eyes of 22 patients underwent CXL, for a total of 71% of our cohort. No postoperative complications occurred. After CXL, there was no significant difference in minimum pachymetry (455.6±37.25µm vs. 453.45±42.6µm after treatment (P=0.71)) or Kmax (50.23±7.17D vs. 50.99±7.01D after treatment (P=0.058)). There was a significant improvement in BCVA (from 0.30±0.3LogMar to 0.17±0.17LogMar after CXL (P=0.024)) and spherical equivalent (-1.91±2.1D to -2.54±1.89D after treatment (P=0.009)). The mean duration of follow-up was 32.2 months (12-59). CXL shows long-term disease stabilization in children with keratoconus. Nevertheless, this technique is indicated only for progressive keratoconus. Early diagnosis and management are essential in this population where the disease is rapidly changing. Treatment of atopy and performance of corneal topography when a child has irregular astigmatism should become automatic for early detection of this disease.

[Descemet's membrane detachment after phacoemulsification: Series of 9 cases]. / Décollement descemétique post-phakoémulsification : à propos de 9 cas.

PURPOSE: Descemet's membrane detachment (DMD) is a rare but potentially serious complication of cataract surgery. Although there are no consensual guidelines regarding the diagnosis or treatment of DMD, incorrect treatment may result in irreversible corneal changes with visual sequellae. The purpose of our study is to describe the diagnosis and treatment of DMD. METHODS: We report a series of 9 cases of DMD, their diagnosis, treatment and outcomes. We tested the HELP protocol retrospectively against our 9 real-life cases. RESULTS: Two cases recovered with simple medical management, 4 required air-bubble descemetopexy, and three required keratoplasty. Our study revealed that the main factor associated with poor outcomes is late diagnosis and management. CONCLUSION: Our series illustrates the importance of proactive management and timely diagnosis by performing anterior segment OCT in the setting of persistent postoperative corneal edema.

Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase.We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis.The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.

[Corneal endothelial cell therapy, a review]. / Thérapie cellulaire cornéenne endothéliale. Revue de la littérature.

In France, endothelial dysfunction represents approximately one half of the indications for corneal transplants performed each year. However, the use of endothelial keratoplasty is limited by the technical difficulty of the procedure, a shortage of available grafts, and the potential for graft failure or rejection. These limitations are driving researchers to develop new, less invasive, and more effective therapies. Corneal endothelial cell therapy is being explored as a potential therapeutic measure, to avoid the uncertainty associated with grafting. The human cornea is an ideal tissue for cell therapy. Due to its avascular and immunologically privileged characteristics, transplanted cells are better tolerated compared with other vascularized tissues and organs. Advances in the field of stem cell engineering, particularly the development of corneal epithelial stem cell therapy for the treatment of severe ocular surface disease, have aroused a massive interest in adapting cell therapy techniques to corneal endothelial cells. This chapter, based on a review of the literature, aims at educating the reader on the latest research in the field of corneal endothelial cell therapy.

[Descemet's Membrane Endothelial Keratoplasty. Indication, surgical technic, postoperative management and review of literature]. / Descemet's Membrane Endothelial Keratoplasty. Indication, technique chirurgicale, gestion postopératoire et revue de la littérature.

Endothelial keratoplasty is currently the preferred method for the treatment of endothelial dysfunctions and dystrophies. Descemet Membrane Endothelial Keratoplasty (DMEK), described by Gerrit Melles in 2006, is performed by selectively replacing the damaged endothelium with a healthy counterpart. It leads to a faster visual recovery and better refractive outcomes with a limited risk of rejection compared to Descemet's Stripping Automated Endothelial Keratosplasty (DSAEK), which includes a thin stromal layer. Open debate still exists between DMEK and DSAEK. This article aims to provide a literature review and enlighten the reader on the DMEK technique, its results and complications.

[Prospective, monocentric, uncontrolled study of efficacy, tolerance and adherence of cyclosporin 0.1 % for severe dry eye syndrome]. / Étude prospective, monocentrique, non contrôlée de l'efficacité, de la tolérance et de l'adhésion au traitement par ciclosporine 0,1 % au cours des sécheresses oculaires sévères.

PURPOSE: To evaluate the efficacy, tolerability and treatment adherence of Ikervis® (Santen, SAS) (ciclosporine 0.1 %) for first line therapy or following treatment with Restasis® (Allergan, Inc.) (ciclosporine 0.05 %) for severe dry eye syndrome. MATERIAL AND METHODS: A prospective, monocentric, uncontrolled study was conducted between January 2012 and March 2015 on 110 eyes of 55 patients with severe dry eye on first line therapy or previously treated with Restasis® who required the introduction of Ikervis®. Patients' quality of life was assessed before and after treatment was started using a standardized questionnaire (Ocular Surface Disease Index© [OSDI]), clinical efficacy was quantified at the slit lamp, by measurement of the Break Up time Test (BUT) and the Oxford classification. Tolerability and adherence to treatment were measured using a simple questionnaire. RESULTS: A total of 72 eyes of 37 patients were included. Etiologies of dry eye syndrome were dominated by Sjögren syndrome (32 %) and severe ocular surface conditions (48 %). The mean age was 57.7 years (±17.45) and mean follow-up was 458 days (±292). The mean BUT increased by 2.043seconds [1.522-2.563] (P<0.0001). Corneal/conjunctival involvement evaluated by the Oxford classification was also improved with a difference in level of 1.68 [1.290-2.071] (P<0.0001). Ocular Surface Disease Index© (OSDI) decreased by 21.7 [16.372-27.024] (P<0.0001). Treatment tolerability was moderate, with more than 50 % of patients experiencing pain on instillation. Overall satisfaction with treatment was good, with more than 60 % of patients feeling better after initiation of treatment. CONCLUSION: Ikervis® is an effective treatment of severe dry eye. Its indications tend to evolve towards less severe dry eye. However, the tolerability profile remains poor, and an improvement in this would be desirable.

[Management of post-traumatic aphakia and aniridia: Retrospective study of 17 patients undergoing scleral-sutured artificial iris intraocular lens implantation. Management of aphakia-aniridia with scleral-sutured artificial iris intraocular lenses]. / Prise en charge de l'aphakie et de l'aniridie post-traumatiques. Étude rétrospective de 17 patients opérés d'implants intraoculaires suturés à la sclère à iris artificiel. Gestion de l'aphakie-aniridie par implants suturés à la sclère à iris artificiel.

PURPOSE: To evaluate the long-term outcomes of artificial iris intraocular lenses sutured to the sclera for managing traumatic aphakia and aniridia. METHODS: All consecutive cases receiving a Morcher® combination implant from June 2008 to February 2016 in Edouard-Herriot Hospital (Lyon, France) were included in this single-center retrospective study. Visual acuity, subjective degree of glare, quality of life and surgical complications were evaluated. RESULTS: Seventeen eyes of 17 patients were included, among which 82% were male. The mean age was 42 years. The injuries consisted of 23.5% contusion and 70.5% open globe injuries, of which 41% were globe ruptures. There was one postoperative case. A penetrating keratoplasty was performed at the same time for eight eyes. The mean follow-up was 32 months. Best-corrected visual acuity improved in 41.2%, remained the same in 17.6% and decreased in 41.2% of our cases. Distance vision averaged 1±0.25 line better and near vision 2.2±0.32 lines better when visual acuity was quantifiable before surgery. Glare improved in 80% of patients and remained stable in 20%, decreasing on average from 3.3/5 [min. 3-max. 4; SD: 0.48] before surgery to 1.9/5 [min. 0-max. 4; SD: 1.197] after surgery. Regarding the esthetic results, 78% of the patients declared themselves reasonably to very satisfied; 57% reported no limitation of activities of daily living, and 43% reported mild limitation. Ocular hypertension and glaucoma, found in 40% of eyes, were the main postoperative complications. CONCLUSION: Implantation of prosthetic iris device combined with an intraocular lens appears to be safe and effective in reducing glare disability and improving visual acuity. Close, long-term monitoring is essential for the success of this surgery.

[Limbal stem cell deficiency management. A review]. / Traitement du déficit en cellules souches limbiques. Revue de la littérature.

Limbal stem cell deficiency is predominantly caused by severe eye burns resulting in a decreased or a complete ablation of the regenerative potential of these stem cells. The inability to reconstruct the corneal epithelium further leads conjunctivalization of the gimbal-epithelial barrier. These abnormalities collectively result in the progressive opacification of the cornea responsible for blindness that is driven by chronic corneal ulceration and neovascularization. The underlying pathology of the cornea affects the homeostasis of the neighboring conjunctiva, eyelids, and tear film. Therefore, the ocular reconstruction to treat limbal stem cell deficiency is quite prolonged and involves a continued treatment plan. The management of limbal stem cell deficiency has undergone a multitude of changes over the past several decades. The understanding of limbal anatomy and physiology, as well as therapeutic advances in the stem cell field have propelled the development of new treatments offering new hope to severely disabled patients. Cultivated limbal epithelial and oral mucosal epithelial transplantations are therefore viable alternatives that could be utilized for the treatment of limbal stem cell deficiency.

[Leber-Coats' disease: A retrospective study of 10 patients]. / Maladie de Leber-Coats : étude rétrospective sur 10 cas.

INTRODUCTION: Retrospective study analyzing clinical characteristics and treatment of children affected by Leber-Coats' disease. METHOD: Children with Leber-Coats' disease seen in Edouard-Herriot Hospital, Lyon, between January 2004 and September 2013 were included. The following clinical characteristics were reported: sex, age at diagnosis, presenting symptom, Shields' stage, treatment used, and visual outcomes. RESULTS: Ten children were included in our study. Mean age at diagnosis was 5.5 years. The disease was unilateral in 9/10 cases. The presenting symptom was loss of vision in 7/10 cases, strabismus in 3/10 cases, and microphthalmos in 1 case. The diagnosis was made fortuitously during a follow-up of familial congenital cataract in 2 patients. No cases of leukocoria were seen. Shields' stages were distributed as follows: stage 1, 2A and 2B: 1 case each, stage 3A1: 7 cases, stage>3A1: no cases. Laser photocoagulation was used up to stage 2B, combined with cryotherapy in stage 3A. Stage 1 and 2A were associated with relatively good final visual acuity. Significant macular lipid exudate (stage>2A) was associated with a worse prognosis. CONCLUSION: These epidemiological data help us to better characterize these patients' prognosis. Early diagnosis seems to improve visual outcomes.