Search

Objective. To evaluate feasibility of chemoradiation as alternative for extensive surgery in patients with locally advanced vulvar cancer and to report on locoregional control, toxicity and survival. Methods. In a multicenter, prospective phase II trial patients with locally advanced vulvar cancer were treated with locoregional radiotherapy combined with sensitizing chemotherapy (capecitabine). Treatment feasibility, percentage locoregional control, survival and toxicity were evaluated. Results. 52 patients with mainly T2/T3 disease were treated according to the study protocol in 10 centers in the Netherlands from 2007 to 2019. Full dose radiotherapy (tumor dose of 64.8Gy) was delivered in 92% and full dose capecitabine in 69% of patients. Most prevalent acute >_ grade 3 toxicities were regarding skin/ mucosa and pain (54% and 37%). Late >_grade 3 toxicity was reported for skin/mucosa (10%), fibrosis (4%), GI incontinence (4%) and stress fracture or osteoradionecrosis (4%). Twelve weeks after treatment, local clinical complete response (cCR) and regional control (RC) rates were 62% and 75%, respectively. After 2 years, local cCR persisted in 22 patients (42%) and RC was 58%. Thirty patients (58%) had no evidence of disease at end of follow-up (median 35 months). In 9 patients (17%) extensive surgery with stoma formation was needed. Progression free survival was 58%, 51% and 45% and overall survival was 76%, 66%, 52% at 1,2, and 5 years. Conclusions. Definitive capecitabine-based chemoradiation as alternative for extensive surgery is feasible in locally advanced vulvar cancer and results in considerable locoregional control with acceptable survival rates with manageable acute and late toxicity. (c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).

Background. Decision making regarding adjuvant therapy for high-risk endometrial cancer is complex. The aim of this study was to determine patients' and clinicians' minimally desired survival benefit to choose chemoradiotherapy over radiotherapy alone. Moreover, influencing factors and importance of positive and negative treatment effects (i.e. attribute) were investigated.Methods. Patients with high-risk endometrial cancer treated with adjuvant pelvic radiotherapy with or without chemotherapy and multidisciplinary gynaecologic oncology clinicians completed a trade-off questionnaire based on PORTEC-3 trial data.Results. In total, 171 patients and 63 clinicians completed the questionnaire. Median minimally desired benefit to make chemoradiotherapy worthwhile was significantly higher for patients versus clinicians (10% vs 5%, p = 0.02). Both patients and clinicians rated survival benefit most important during decision making, followed by long-term symptoms. Older patients (OR 0.92 [95%CI 0.87 & ndash;0.97]; p = 0.003) with comorbidity (OR 0.34 [95% CI 0.12 & ndash;0.89]; p = 0.035) had lower preference for chemoradiotherapy, while patients with better numeracy skills (OR 1.2 [95%CI 1.05 & ndash;1.36], p = 0.011) and chemoradiotherapy history (OR 25.0 [95%CI 8.8 & ndash;91.7]; p < 0.001) had higher preference for chemoradiotherapy.& nbsp;Conclusions. There is a considerable difference in minimally desired survival benefit of chemoradiotherapy in high-risk endometrial cancer among and between patients and clinicians. Overall, endometrial cancer patients needed higher benefits than clinicians before preferring chemoradiotherapy.(c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http:// creativecommons.org/licenses/by/4.0/).

BackgroundVaginal brachytherapy is currently recommended as adjuvant treatment in patients with high-intermediate risk endometrial cancer to maximize local control and has only mild side effects and no or limited impact on quality of life. However, there is still considerable overtreatment and also some undertreatment, which may be reduced by tailoring adjuvant treatment to the patients’ risk of recurrence based on molecular tumor characteristics.Primary objectivesTo compare the rates of vaginal recurrence in women with high-intermediate risk endometrial cancer, treated after surgery with molecular-integrated risk profile-based recommendations for either observation, vaginal brachytherapy or external pelvic beam radiotherapy or with standard adjuvant vaginal brachytherapyStudy hypothesisAdjuvant treatment based on a molecular-integrated risk profile provides similar local control and recurrence-free survival as current standard adjuvant brachytherapy in patients with high-intermediate risk endometrial cancer, while sparing many patients the morbidity of adjuvant treatment and reducing healthcare costs.Trial designA multicenter, international phase III randomized trial (2:1) of molecular-integrated risk profile-based adjuvant treatment (experimental arm) or adjuvant vaginal brachytherapy (standard arm).Major inclusion/exclusion criteriaWomen aged 18 years and over with a histological diagnosis of high-intermediate risk endometrioid endometrial cancer after total abdominal or laparoscopic hysterectomy and bilateral salpingo-oophorectomy. High-intermediate risk factors are defined as: (i) International Federation of Gynecology and Obstetrics stage IA (with invasion) and grade 3; (ii) stage IB grade 1 or 2 with age ≥60 and/or lymph-vascular space invasion; (iii) stage IB, grade 3 without lymph-vascular space invasion; or (iv) stage II (microscopic and grade 1).EndpointsThe primary endpoint is vaginal recurrence. Secondary endpoints are recurrence-free and overall survival; pelvic and distant recurrence; 5-year vaginal control (including treatment for relapse); adverse events and patient-reported symptoms and quality of life; and endometrial cancer-related healthcare costs.Sample size500 eligible and evaluable patients.Estimated dates for completing accrual and presenting resultsEstimated date for completing accrual will be late 2021. Estimated date for presentation of (first) results is expected in 2023.Trial registrationThe trial is registered at clinicaltrials.gov (NCT03469674) and ISRCTN (11659025).

Contains fulltext : 229838.pdf (Publisher’s version ) (Open Access) OBJECTIVE: The PORTEC-4a trial investigates molecular-integrated risk profile guided adjuvant treatment for endometrial cancer. The quality assurance programme included a dummy run for vaginal brachytherapy prior to site activation, and annual quality assurance to verify protocol adherence. Aims of this study were to evaluate vaginal brachytherapy quality and protocol adherence. METHODS: For the dummy run, institutes were invited to create a brachytherapy plan on a provided CT-scan with the applicator in situ. For annual quality assurance, institutes provided data of one randomly selected brachytherapy case. A brachytherapy panel reviewed and scored the brachytherapy plans according to a checklist. RESULTS: At the dummy run, 15 out of 21 (71.4%) institutes needed adjustments of delineation or planning. After adjustments, the mean dose at the vaginal apex (protocol: 100%; 7 Gy) decreased from 100.7% to 99.9% and range and standard deviation (SD) narrowed from 83.6-135.1 to 96.4-101.4 and 8.8 to 1.1, respectively. At annual quality assurance, 22 out of 27 (81.5%) cases had no or minor and 5 out of 27 (18.5%) major deviations. Most deviations were related to delineation, mean dose at the vaginal apex (98.0%, 74.7-114.2, SD 7.6) or reference volume length. CONCLUSIONS: Most feedback during the brachytherapy quality assurance procedure of the PORTEC-4a trial was related to delineation, dose at the vaginal apex and the reference volume length. Annual quality assurance is essential to promote protocol compliance, ensuring high quality vaginal brachytherapy in all participating institutes.

In this article, we will review major therapeutic advances in neonatology over the past ten years. We will discuss the antenatal administration of magnesium sulfate, the interest of hypothermia in the context of hypoxic ischaemic encephalopathy, the benefits and modalities of placental transfusion, less invasive techniques for ventilation and administration of the surfactant, possibilities to fortify breast milk and the concept of developmental care. These therapeutic advances are sometimes based on new therapeutics, sometimes on new concepts and, sometimes, on new less invasive techniques. They have made it possible to optimize the care of premature babies but also of term newborns.Dans cet article, nous allons passer en revue les grandes avancées thérapeutiques dans le domaine de la néonatologie au cours des dix dernières années. Nous traiterons de l’administration anténatale du sulfate de magnésium, de l’intérêt de l’hypothermie contrôlée dans le cadre de l’encéphalopathie d’origine hypoxo-ischémique, des bénéfices et modalités de la transfusion placentaire, des techniques moins invasives pour la ventilation et pour l’administration du surfactant, de la fortification du lait maternel et du concept de soins de développement. Ces avancées thérapeutiques reposent tantôt sur des nouveautés thérapeutiques, tantôt sur de nouveaux concepts et, parfois, sur de nouvelles techniques moins invasives. Elles ont permis d’optimiser la prise en charge des prématurés, mais aussi des nouveau-nés à terme.

Late preterm infants are born between 34 weeks of amenorrhea and 36 weeks 6 days. Late preterms represent the largest proportion of premature infants (about 75 %). Late prematurity is increasing in recent decades. While studies initially focused on mortality and morbidity related to very preterm birth, the late preterms have been the subject of increased attention over the past 15 years. Late preterm infants have an increased risk of respiratory complications, infections, feeding problems, hypothermia and hypoglycemia. Neonatal, infant and during adulthood mortalities are significantly higher in late preterm than in term infants. In addition, late preterm infants carry an increased risk of long-term morbidities, such as neurodevelopmental delay, cerebral palsy, chronic respiratory or metabolic diseases. This review highlights the evidence that late preterm infants are high risk newborns and require adapted follow-up.Les enfants nés entre 34 semaines d’aménorrhée et 36 semaines 6 jours sont dans la période de prématurité tardive. Ils sont également appelés «late-preterm». Ces enfants représentent près de 75 % des naissances prématurées dans les pays industrialisés. Cette prématurité tardive est en augmentation croissante sur les dernières décennies. Alors qu’initialement les études se concentraient essentiellement sur la mortalité et les morbidités liées à la grande prématurité, la population des prématurés tardifs a fait l’objet d’une attention accrue ces 15 dernières années. Il est ainsi démontré que ces enfants présentent un risque accru de complications respiratoires, d’infections, de problèmes d’alimentation, d’hypothermie et d’hypoglycémie. La mortalité néonatale, infantile et jusqu’à l’âge adulte des late-preterm est significativement plus élevée que chez les nouveau-nés à terme. De plus, les morbidités à long terme, tels que le retard neurodéveloppemental, l’infirmité motrice cérébrale, les pathologies respiratoires chroniques ou métaboliques, sont significativement plus élevées. A travers cette revue de la littérature, nous revoyons ces risques, qui contribuent à faire des «late preterm» une population fragile et nécessitant un suivi adapté.

Background: Although women with endometrial cancer generally have a favourable prognosis, those with high-risk disease features are at increased risk of recurrence. The PORTEC-3 trial was initiated to investigate the benefit of adjuvant chemotherapy during and after radiotherapy (chemoradiotherapy) versus pelvic radiotherapy alone for women with high-risk endometrial cancer. Methods: PORTEC-3 was an open-label, international, randomised, phase 3 trial involving 103 centres in six clinical trials collaborating in the Gynaecological Cancer Intergroup. Eligible women had high-risk endometrial cancer with FIGO 2009 stage I, endometrioid-type grade 3 with deep myometrial invasion or lymph-vascular space invasion (or both), endometrioid-type stage II or III, or stage I to III with serous or clear cell histology. Women were randomly assigned (1:1) to receive radiotherapy alone (48·6 Gy in 1·8 Gy fractions given on 5 days per week) or radiotherapy and chemotherapy (consisting of two cycles of cisplatin 50 mg/m2 given during radiotherapy, followed by four cycles of carboplatin AUC5 and paclitaxel 175 mg/m2) using a biased-coin minimisation procedure with stratification for participating centre, lymphadenectomy, stage of cancer, and histological type. The co-primary endpoints were overall survival and failure-free survival. We used the Kaplan-Meier method, log-rank test, and Cox regression analysis for final analysis by intention to treat and adjusted for stratification factors. The study was closed on Dec 20, 2013, after achieving complete accrual; follow-up is ongoing. PORTEC-3 is registered with ISRCTN, number ISRCTN14387080, and ClinicalTrials.gov, number NCT00411138. Results: 686 women were enrolled between Nov 23, 2006, and Dec 20, 2013. 660 eligible patients were included in the final analysis, of whom 330 were assigned to chemoradiotherapy and 330 were assigned to radiotherapy. Median follow-up was 60·2 months (IQR 48·1–73·1). 5-year overall survival was 81·8% (95% CI 77·5–86·2) with chemoradiotherapy versus 76·7% (72·1–81·6) with radiotherapy (adjusted hazard ratio [HR] 0·76, 95% CI 0·54–1·06; p=0·11); 5-year failure-free survival was 75·5% (95% CI 70·3–79·9) versus 68·6% (63·1–73·4; HR 0·71, 95% CI 0·53–0·95; p=0·022). Grade 3 or worse adverse events during treatment occurred in 198 (60%) of 330 who received chemoradiotherapy versus 41 (12%) of 330 patients who received radiotherapy (p

BACKGROUND: Although women with endometrial cancer generally have a favourable prognosis, those with high-risk disease features are at increased risk of recurrence. The PORTEC-3 trial was initiated to investigate the benefit of adjuvant chemotherapy during and after radiotherapy (chemoradiotherapy) versus pelvic radiotherapy alone for women with high-risk endometrial cancer. METHODS: PORTEC-3 was an open-label, international, randomised, phase 3 trial involving 103 centres in six clinical trials collaborating in the Gynaecological Cancer Intergroup. Eligible women had high-risk endometrial cancer with FIGO 2009 stage I, endometrioid-type grade 3 with deep myometrial invasion or lymph-vascular space invasion (or both), endometrioid-type stage II or III, or stage I to III with serous or clear cell histology. Women were randomly assigned (1:1) to receive radiotherapy alone (48·6 Gy in 1·8 Gy fractions given on 5 days per week) or radiotherapy and chemotherapy (consisting of two cycles of cisplatin 50 mg/m 2 given during radiotherapy, followed by four cycles of carboplatin AUC5 and paclitaxel 175 mg/m 2) using a biased-coin minimisation procedure with stratification for participating centre, lymphadenectomy, stage of cancer, and histological type. The co-primary endpoints were overall survival and failure-free survival. We used the Kaplan-Meier method, log-rank test, and Cox regression analysis for final analysis by intention to treat and adjusted for stratification factors. The study was closed on Dec 20, 2013, after achieving complete accrual; follow-up is ongoing. PORTEC-3 is registered with ISRCTN, number ISRCTN14387080, and ClinicalTrials.gov, number NCT00411138. RESULTS: 686 women were enrolled between Nov 23, 2006, and Dec 20, 2013. 660 eligible patients were included in the final analysis, of whom 330 were assigned to chemoradiotherapy and 330 were assigned to radiotherapy. Median follow-up was 60·2 months (IQR 48·1-73·1). 5-year overall survival was 81·8% (95% CI 77·5-86·2) with chemoradiotherapy versus 76·7% (72·1-81·6) with radiotherapy (adjusted hazard ratio [HR] 0·76, 95% CI 0·54-1·06; p=0·11); 5-year failure-free survival was 75·5% (95% CI 70·3-79·9) versus 68·6% (63·1-73·4; HR 0·71, 95% CI 0·53-0·95; p=0·022). Grade 3 or worse adverse events during treatment occurred in 198 (60%) of 330 who received chemoradiotherapy versus 41 (12%) of 330 patients who received radiotherapy (p

BACKGROUND: Although women with endometrial cancer generally have a favourable prognosis, those with high-risk disease features are at increased risk of recurrence. The PORTEC-3 trial was initiated to investigate the benefit of adjuvant chemotherapy during and after radiotherapy (chemoradiotherapy) versus pelvic radiotherapy alone for women with high-risk endometrial cancer. METHODS: PORTEC-3 was an open-label, international, randomised, phase 3 trial involving 103 centres in six clinical trials collaborating in the Gynaecological Cancer Intergroup. Eligible women had high-risk endometrial cancer with FIGO 2009 stage I, endometrioid-type grade 3 with deep myometrial invasion or lymph-vascular space invasion (or both), endometrioid-type stage II or III, or stage I to III with serous or clear cell histology. Women were randomly assigned (1:1) to receive radiotherapy alone (48·6 Gy in 1·8 Gy fractions given on 5 days per week) or radiotherapy and chemotherapy (consisting of two cycles of cisplatin 50 mg/m 2 given during radiotherapy, followed by four cycles of carboplatin AUC5 and paclitaxel 175 mg/m 2) using a biased-coin minimisation procedure with stratification for participating centre, lymphadenectomy, stage of cancer, and histological type. The co-primary endpoints were overall survival and failure-free survival. We used the Kaplan-Meier method, log-rank test, and Cox regression analysis for final analysis by intention to treat and adjusted for stratification factors. The study was closed on Dec 20, 2013, after achieving complete accrual; follow-up is ongoing. PORTEC-3 is registered with ISRCTN, number ISRCTN14387080, and ClinicalTrials.gov, number NCT00411138. RESULTS: 686 women were enrolled between Nov 23, 2006, and Dec 20, 2013. 660 eligible patients were included in the final analysis, of whom 330 were assigned to chemoradiotherapy and 330 were assigned to radiotherapy. Median follow-up was 60·2 months (IQR 48·1-73·1). 5-year overall survival was 81·8% (95% CI 77·5-86·2) with chemoradiotherapy versus 76·7% (72·1-81·6) with radiotherapy (adjusted hazard ratio [HR] 0·76, 95% CI 0·54-1·06; p=0·11); 5-year failure-free survival was 75·5% (95% CI 70·3-79·9) versus 68·6% (63·1-73·4; HR 0·71, 95% CI 0·53-0·95; p=0·022). Grade 3 or worse adverse events during treatment occurred in 198 (60%) of 330 who received chemoradiotherapy versus 41 (12%) of 330 patients who received radiotherapy (p

Previously, we showed that case-specific non-linear finite element (FE) models are better at predicting the load to failure of metastatic femora than experienced clinicians. In this study we improved our FE modelling and increased the number of femora and characteristics of the lesions. We retested the robustness of the FE predictions and assessed why clinicians have difficulty in estimating the load to failure of metastatic femora. A total of 20 femora with and without artificial metastases were mechanically loaded until failure. These experiments were simulated using case-specific FE models. Six clinicians ranked the femora on load to failure and reported their ranking strategies. The experimental load to failure for intact and metastatic femora was well predicted by the FE models (R2= 0.90 and R2= 0.93, respectively). Ranking metastatic femora on load to failure was well performed by the FE models (τ = 0.87), but not by the clinicians (0.11 < τ < 0.42). Both the FE models and the clinicians allowed for the characteristics of the lesions, but only the FE models incorporated the initial bone strength, which is essential for accurately predicting the risk of fracture. Accurate prediction of the risk of fracture should be made possible for clinicians by further developing FE models.

PURPOSE: To report a new family with Blau syndrome caused by CARD15/NOD2 mutation. DESIGN: Observational case series. METHODS: Detailed clinical evaluation in three affected relatives with Blau syndrome. Haplotype and mutation analysis of the CARD15/NOD2 gene were performed. RESULTS: Ocular manifestations identified in the proband include bilateral band keratopathy, cataract, iritis, vitritis and severe granulomatous choroidopathy. The mother and one brother of the proband exhibit the same characteristic organ involvements of this disease. Haplotype analysis from the pericentromeric region on chromosome 16 identified a common haplotype in all affected relatives that is absent in unaffected relatives. Sequencing analysis revealed a heterozygous pathogenic mutation in the CARD15/NOD2 gene, the previously reported p.R334W substitution. CONCLUSION: Blau syndrome is a rare autosomal-dominant disease that can lead to severe visual impairment. The search of a CARD15/NOD2 mutation could be helpful in the differential diagnosis of childhood uveitis.

We report the case of an immunocompetent 78-year-old woman who developed cytomegalovirus (CMV) retinitis after a single intravitreous injection of triamcinolone acetonide (IVTA). Review of medical records. The patient with macular edema secondary to branch retinal vein occlusion developed peripheral retinitis with hemorrhagic and inflammatory vascular sheathing 3 months after IVTA. A presumptive diagnosis of viral retinitis was confirmed by polymerase chain reaction (PCR) of the aqueous humor tap. The PCR test was positive for CMV DNA. The patient slowly responded to intravenous ganciclovir and oral valganciclovir. After therapeutic vitrectomy for intercurrent vitreous hemorrhage, and while still under treatment, the retinitis resolved completely with final visual acuity of 20/25. CMV retinitis can occur after local immunosuppression with IVTA in an immunocompetent patient with no other systemic risk factors.

Purpose: To study the prevalence of polypoidal choroidal vasculopathy (PCV) in Caucasian patients with occult choroidal neovascularization (CNV); to study the clinical spectrum of PCV in Caucasians and the outcome after laser photocoagulation of such lesions. Methods: (1) A consecutive series of 374 eyes of Caucasian patients at least 58 years old, presenting occult CNV, presumed to have age-related macular degeneration (AMD) on fluorescein angiography (FA) were further characterized by indocyanine green angiography (ICGA) to determine the frequency of PCV. (2) The funduscopic, FA and ICGA findings in a cohort of 36 Caucasian patients with PCV were analyzed. (3) The outcome after laser photocoagulation was studied in 14 PCV eyes with a minimum follow-up of 6 months. Results: (1) Fourteen of 374 eyes (4%) presenting occult CNV in patients at least 58 years old were diagnosed as PCV by means of ICG-A. (2) A polypoidal lesion was found in the macula in 22 of 45 PCV eyes, in the peripapillary area in 16 of 45, under the temporal vascular arcade in 6 of 45 and in the midperiphery in 6 of 45. Large or soft drusen were observed in 15 of 45 eyes with PCV. (3) Regression of fundus signs without persisting polyps 6 months after laser photocoagulation was obtained in 5 of 5 treated peripapillary lesions but in only 5 of 9 treated macular or arcade lesions. Conclusion: Polypoidal choroidal vasculopathy is not rare in Caucasian patients presenting with occult choroidal neovascularization. The fundus abnormalities seen in such eyes overlap with the typical manifestations of AMD. Whereas the prognosis after photocoagulation of peripapillary polypoidal lesions appears to be relatively good, it is more guarded for macular or arcade lesions.

AIMS/BACKGROUND--This study was undertaken to confirm the association between diffuse retinal pigment epitheliopathy (DRPE) and systemic corticosteroid therapy. This finding can be of help in determining an aetiological factor in DRPE and associated diseases. Corticosteroids may contribute to the development of leakage in the presence of a retinal pigment epitheliopathy or central serous chorioretinopathy. METHODS--Cases of DRPE were collected from the files of members of the European Fluorescein Angiography Club. There were 34 who developed their ophthalmic symptoms while being treated with corticosteroids. RESULTS--DRPE developed in 34 patients from 10 European eye clinics during systemic treatment with corticosteroids. Thirteen patients were treated with corticosteroids after allotransplantation, 21 other patients developed ocular symptoms while treated with steroids for serious systemic disorders. Symptoms occurred in some patients when the daily corticosteroid dosage was elevated, and the visual complaints ameliorated or even disappeared sometimes on discontinuation of the corticosteroid therapy. CONCLUSION--Corticosteroids can damage the retinal pigment epithelial barrier and predispose a patient to serous retinal detachment, whereas psychological stress may play a role in the development of central serous chorioretinopathy. A decrease of the daily corticosteroid dosage may help to diminish the visual symptoms.

To evaluate the frequency of diabetic retinopathy in Lubumbashi (Democratic Republic of Congo).Descriptive survey including 369 diabetic patients examined in our department of Ophthalmology of Lubumbashi from May 2006 to March 2008. Rates of glycaemia and blood pressure were noted. All the patients had a complete ophthalmological examination including visual acuity measurement, anterior biomicroscopy, intraocular pressure and fundus examination after pupil dilation.At the time of ocular examination, the mean age of diabetic patients was 57 years (range 16 to 85) with a sex ratio of 1.2 males for 1 female. The frequency of type 1 diabetic patients was 28% and of type 2, 72%. High blood pressure was found in 42% of the patients. The frequency of diabetic patients (all forms included) was 13% and diabetes-induced blindness was 0.27%. In our study, the frequency of diabetic retinopathy increased with the patient's age and the duration of the diabetes. Diabetic retinopathy was more frequently found in adults. We did not find correlation between the level of glycaemia and the incidence of diabetic retinopathy.From our epidemiological study, diabetic retinopathy is a concern for adult public health. It requires organized screening and early treatment to prevent blindness of those diabetic patients.

We report a case of bilateral optic neuropathy associated with the CREST syndrome, a variant of scleroderma. Immunosuppressive therapy achieved significant improvement of the visual symptoms for two years. The pathophysiological mechanisms underlying this unique association remain unclear.

Contains fulltext : 79904.pdf (Publisher’s version ) (Closed access) PURPOSE: To evaluate the long-term outcome after surgery and radiotherapy for patients with sinonasal cancer and assess late toxicity, with special emphasis on hypothalamic-pituitary dysfunction. METHODS AND MATERIALS: A retrospective analysis of 168 patients treated for sinonasal cancer in a single institute between 1986 and 2006. A more detailed analysis was performed on a subgroup of 76 patients with adenocarcinoma or squamous cell carcinoma treated with curative intent. Long-term survivors were evaluated for late toxicity by a multidisciplinary team using the late effects of normal tissues (LENT SOMA) scoring system. Additional endocrinologic tests were performed for assessment of hypothalamic-pituitary function. RESULTS: Five-year actuarial local control and overall survival rates were 62% and 35% for all patients and 64% and 42% for the subgroup with squamous cell carcinoma and adenocarcinoma. In multivariate analysis, T stage was the only significant factor predicting local relapse (79% at 5 years for T1-T3 vs. 53% for T4; p = 0.006). Sinonasal mucosal melanomas had the highest rate of regional failure (33% at 5 years). Thirteen of 21 patients (62%) evaluated at the late morbidity clinic had hormonal disturbances, of whom 5 (24%) had definitive evidence of hypopituitarism with multiple hormonal deficiencies. CONCLUSION: Local failure is the dominant cause of treatment failure for patients with sinonasal cancer, with T4 stage the only independent predictor. Because of a high rate of radiation-induced hypopituitarism, we recommend endocrinologic surveillance for these patients.

Specific fundus changes have been reported in patients with membranoproliferative glomerulonephritis type II (MPGN type II). We studied the clinical course of this retinopathy in four patients who all had a long follow-up with several fundus examinations. Sequential observation was indicative of a slow progression of the retinopathy. Most eyes maintained in the chronic stages a nearly normal visual acuity, and a full visual field despite the existence of marked drusen and atrophic changes. The prognosis however must be somewhat guarded, since choroidal neovascularization developed in three eyes and caused bilateral severe visual loss in one patient.

We describe a case of an acute symptomatic and non-progressive visual field constriction in one eye of a 19-year-old female with optic disc drusen. The fellow eye showed slowly progressive visual field defects. A limited or retrolaminar infarction of the optic nerve head seems a plausible cause of the acute visual field loss. The slow progression in the fellow eye is most probably due to mechanical compression by the drusen. The case is documented with Humphrey perimetry during a follow-up of 11 years and with Color Doppler Imaging (CDI) of the optic nerve.

Three patients with systemic lupus erythematosus (SLE) associated with anti-phospholipid antibodies are reported. All three had severe vaso-occlusive disease: the first had an unilateral vaso-occlusive retinopathy, the second an unilateral central retinal venous obstruction, and the third a bilateral ischemic choroidopathy. The association of these occlusive ocular vascular diseases with the presence of the lupus anticoagulant and other associated factors in SLE is discussed.

Purpose : To evaluate the safety and efficacy of photodynamic therapy with verteporfin (PDT) for subfoveal classic choroidal neovascularization (CNV) related to punctate inner choroidopathy (PIC) or presumed ocular histoplasmosis-like syndrome (POHS-like). Methods : Retrospective review of 16 eyes from 14 patients with subfoveal classic CNV associated with PIC or POHS-like and treated with PDT. Results : The mean visual acuity increased from 4.5/10 (range: 1/10-9/10) to 7/10 (range: 2/10-10/10) after a mean follow-up of 21 months (range: 8-32 months) and a mean number of 2 PDT (range: 1-6). Visual acuity remained stable or improved in 13 of the 16 eyes (81%) and decreased in three. Conclusion : This nearly two-year follow-up study suggests that PDT could be helpful for patients with subfoveal classic CNV related to PIC or POHS-like.

PURPOSE: To evaluate the effect of photodynamic therapy on subfoveal neovascular membrane related to type 2A idiopathic juxtafoveolar retinal telangiectasia. DESIGN: Interventional case series. METHODS: Retrospective review of four eyes of four patients who underwent photodynamic therapy for subfoveal neovascular membrane secondary to idiopathic juxtafoveolar retinal telangiectasia. Ocular photodynamic therapy with verteporfin was performed in all cases using standard protocols. Results are given in terms of final visual acuity and neovascular membrane activity based on clinical examination, fluorescein and indocyanin green angiography, and, in two cases, optical coherence tomography. RESULTS: Baseline visual acuity of 20/30 and 20/40 (x2) was maintained in three patients after one, two, and three sessions of photodynamic therapy respectively, and a follow-up of 23, 21, and 9 months. Leakage specific to the subfoveal neovascular membrane ceased on the fluorescein angiography. In the other patient, the final vision decreased from 20/50 to 20/200 after four sessions of photodynamic therapy and a follow-up of 14 months. Although there was still mild persistent leakage on the fluorescein angiography, neovascular membrane size was unchanged, and no subretinal fluid was demonstrated on optical coherence tomography. CONCLUSIONS: Data from this case series suggest that photodynamic therapy may be effective in managing subfoveal neovascular membrane associated with idiopathic juxtafoveolar retinal telangiectasia, which usually carries a poor visual prognosis. Prospective study is required to confirm the beneficial effect of this treatment.

Retrospective study of 17 patients (30 eyes) with diffuse retinal epitheliopathy followed for at least 5 years. The study is based on clinical data, ophthalmoscopic findings and fluorescein angiograms. The patients (16 men and 1 woman) are all caucasian with a mean age of 43 years. The disease is bilateral in 13 patients. We report an association with systemic corticosteroid treatment after kidney transplantation in 10 cases. Nine patients have arterial hypertension. Two patients are diabetic and 2 present high level of psychological stress. After a mean follow-up of 12 years, a loss of vision of at least 3 lines is noted in 17 eyes. The decrease in vision is related to an enlargement of the pigment epithelium atrophy to the fovea (15 eyes) or with permanent retrofoveolar serous detachment (2 eyes).

Multifocal choroiditis (MC) is an idiopathic choroidal inflammatory disease affecting young subjects. Secondary choroidal--and often central--neovascularisation is a frequent complication leading to a poor visual prognosis. Photodynamic therapy (PDT) has now proven to be successful to treat classic subfoveal choroidal neovascularisation in age-related macular degeneration and in pathologic myopia. We describe the treatment applied to classic choroidal neovascularisation in two young women with MC, two eyes with subfoveal neovascular membrane and one eye in which new vessels encroach the foveal avascular zone. PDT has been useful in the three reported eyes, with stable or improved visual acuity. In two of them, it even made the membrane retract and become extrafoveal, allowing a secondary treatment using conventional laser.

Cancer may affect the eye and orbit as a direct result of metastatic neoplastic infiltration, compression, or circulating antibodies involving paraneoplastic retinal degeneration. A metastatic tumor to the uvea is the most common form of an intraocular metastatic process. The choroid is the most common site for uveal metastasis; metastases to the ciliary body, iris, retina, optic disk, and vitreous are rare. Approximately one-third of patients have no history of primary cancer at the time of ocular diagnosis. Breast and lung carcinomas for women and lung and gastrointestinal carcinomas for men most commonly metastasize to the eye and orbit. The short-term prognosis for vision is usually good after an individualized therapeutic approach (chemotherapy, hormonal therapy, external beam radiotherapy, or plaque radiotherapy), but the systemic prognosis is poor. The visual paraneoplastic syndromes encompass several distinct clinical and pathological entities including carcinoma-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and bilateral diffuse melanocytic uveal proliferation (BDUMP). The CAR syndrome affects photoreceptors, MAR is thought to affect bipolar cell function, and BDUMP targets the uveal tract. Identification of circulating antibodies against retinal proteins (recovering, 23-kDa retinal protein; 46-kDa and 60-kDa retinal proteins) serves to recognize the paraneoplastic nature of the patient's symptoms, which frequently develop before the cancer is diagnosed. Anecdotal therapeutic responses are described after systemic steroids, immunoglobulin injection, and plasmapheresis. Recognition of their visual symptoms and ocular findings should alert the ophthalmologist to the possibility of cancer and systemic evaluation should be pursued.

Update on herpetic retinal necrosis, including cytomegalovirus retinitis.

We report on four patients, from three different families, with Senior-Loken syndrome (SLS). They were unusual in that they reached end-stage renal failure (ESRF) only during the fifth or sixth decade. SLS is an autosomal-recessive disorder defined by the association of nephronophthisis and retinal dystrophy. Affected individuals invariably progress to ESRF, usually before the age of 20 years. The diagnosis was based on typical clinical presentation and characteristic renal histology, that is, a picture of chronic interstitial nephritis with pronounced thickening and multilayering of tubular basement membranes. Deterioration of renal function was slow, leading to ESRF between the ages of 42 and 56 years. Retinal dystrophy, already symptomatic during childhood in two patients, led to severe visual impairment in all. In contrast with four cases of SLS recently reported in very young patients, the NPH1 gene (the main gene responsible for nephronophthisis) was not deleted in our two tested patients. We conclude that SLS should be considered in adults who suffer from both chronic interstitial nephropathy and retinal degeneration. Whether the SLS is a variant of nephronophthisis and whether early- and late-onset renal failure in SLS is accounted for by genetic or allelic heterogeneity remain to be determined.

A high-grade diffuse T cell lymphoma, initially simulating bilateral panuveitis, was diagnosed by analysis of a vitreous biopsy specimen and a breast tumor in a 57-year-old woman. It responded favorably to aggressive chemotherapy before it relapsed in leukemic transformation. This case emphasizes the misleading initial symptoms of primary intraocular lymphoma and the role of immunophenotyping in the diagnosis and classification of lymphoproliferative ocular disorders. The presentation and management of uveal lymphoid neoplasia are discussed.

A severe case of retinal vascular occlusion in a 31 year old white female is reported. Its origin is attributed to the primary antiphospholipid syndrome. The diagnostic criteria and the treatment of the primary antiphospholipid syndrome are revised and discussed. When the etiology of a retinal vascular occlusion is not assessed by the initial work-up, especially if the initial presentation consists of a severe occlusive disease, antiphospholipid antibodies should be checked.

A history of recurrent corneal erosion (RCE) in 3 brothers with juvenile X-linked Alport's syndrome (AS), whereas their 2 brothers without AS were unaffected, suggested a possible association between AS and RCE. A survey, among 39 patients with AS, revealed that 6 of them had suffered from RCE, supporting the association of the two conditions. RCE has to be considered as an additional ocular manifestation of AS, with a prevalence of 15 to 21%.

Thirty-three patients who underwent low dose of radiation therapy for subfoveal choroidal membranes were studied after a mean follow-up of 16 months. The evaluated parameters were the visual outcome and the choroidal neovascularization evolution. In this study, the efficacy of low-dose radiation delivered to the macular region as an alternative treatment in subfoveal membrane was limited.

Retrospective study of 15 cases of central retinal vein occlusion in young patients. A research for risk factors was performed. 4 patients had a systemic disease, and 6 had thrombophilic hematological conditions like blood hyperviscosity and increased platelet aggregation. No inflammatory disease was found. The visual prognosis was good in 7 out of 12 patients with a follow-up of more than 3 months. Chronic macular edema and neovascular glaucoma were responsible for the bad visual prognosis in 5 cases.

A Purtscher-like retinopathy occurred in a 41-year-old woman who underwent the excision of a pterygium after a retrobulbar injection of 3 ml of lidocaine HCl. Visual acuity increased from 20/200 on the first postoperative day to 20/25, 12 weeks later. The exudates and the hemorrhages progressively disappeared. However, a relative, localized, paracentral scotoma and an afferent pupil defect still persist. The pathophysiology of this Purtscher-like retinopathy is discussed.

Report of 5 cases of central serous choroidopathy (CSC) following kidney transplantation. The authors compare the clinical features, the fluorescein angiography, the color vision, the visual field and the electrophysiological tests of these 5 cases with 7 control patients. In the pathogenesis of the CSC, the authors briefly discuss about the risk factors associated with the transplantation.

Retrospective study of 19 cases of opportunistic infections of the chorioretina in patients with the Acquired Immunodeficiency Syndrome. We observed 14 cases of CMV retinitis, 2 cases of toxoplasmic chorioretinitis, 1 case of cryptococcal choroiditis and 2 cases of herpes zoster retinitis. Review of the clinical, angiographical and histopathological aspects of these infections. Review of the vital and visual prognosis after treatment.