Mona Regina Schneider

Ocular Trauma Scores in paediatric open globe injuries

Background/aims To assess the predictive value and the applicability of Ocular Trauma Score (OTS) for paediatric injuries. Methods Retrospective case series of 71 open globe injuries in children less than 18 years of age with a minimum follow-up period of 1 year. The variables of the OTS, the Paediatric Penetrating OTS (POTS), lens injuries and anterior versus posterior segment injuries were analysed for their predictive values in terms of visual outcome. The applicability and the predictive values of OTS and POTS as a whole were then evaluated. Results Initial visual acuities, retinal detachments, wound locations (p<0.001 each), lens injuries (p=0.001), posterior segment injuries (p=0.002), traumatic cataracts (p=0.010), hyphaema (p=0.011) and vitreous haemorrhages (p=0.026) had significant impacts on visual outcome. The application of OTS proved difficult, as the presence of a mild degree of a relative afferent pupillary defect (RAPD) could not accurately be evaluated in all of our patients. Calculating the OTS without evaluation of RAPD renders it easily applicable for the initial examinations while remaining significantly prognostic (p<0.001). The predictions of the POTS correlated with the actual final visual acuities (p<0.001), but several POTS variables (ie, iris prolapse, age, organic injuries and delay of surgery >48 h) had only limited impacts on visual outcome. Conclusions The OTS has a high predictive value for visual outcome after open globe injuries in children, even without evaluation of RAPD.

Visual acuity after Gamma-Knife radiosurgery of choroidal melanomas

Background/aims To report on conservation of visual acuity after Gamma-Knife radiosurgery of choroidal melanoma. Methods A total of 189 patients with choroidal melanoma were treated with Gamma-Knife stereotactic single-fraction radiosurgery at a single institution between June 1992 and May 2010. The main outcome measure of our retrospective analysis was conservation of pretreatment visual acuity of 20/40 or better, 20/200 or better and counting fingers (CF) or better, over time of follow-up. Patient, tumour and treatment parameters were evaluated as potential risk factors for visual loss. Results Five years after treatment, the actuarial probability of keeping visual acuity better than 20/40, 20/200 and CF was 13%, 14% and 36%, respectively. The majority of patients (84.7%) encountered a deterioration of vision after treatment. The most important risk factors for visual loss were tumour height, longest basal diameter, distance to the optic disk and/or foveola, and retinal detachment before treatment. Treatment dose, and patient characteristics (age, sex, concurrent systemic diseases) were less important. Local tumour control rate was 94.4% after a median follow-up of 39.5 months. Conclusions Visual outcome after single-fraction Gamma-Knife radiotherapy is comparable with linear accelerator (LINAC) based fractionated stereotactic radiotherapy, inferior to proton beam radiotherapy, and depends primarily on tumour size, location and pre-existing retinal detachment.

Influence of single-fraction Gamma-Knife radiosurgery on ocular surface and tear function in choroidal melanoma patients

Aim To evaluate ocular surface and tear function in patients with choroidal melanoma treated with single-fraction radiosurgery. Methods 36 patients (median age 62 years; range 26–84 years) were enrolled between 2001 and 2006 at a single institution. They were treated with the Leksell Gamma Knife in one fraction with a median dose of 30 Gy (range 25–35 Gy). In both eyes of all patients treated subjective symptom score (visual analogue scale) was evaluated, central corneal sensitivity testing, Schirmer test without local anaesthesia, and corneal and conjunctival staining were performed before therapy and 3, 6, 12, 24 and 36 months thereafter. The respective untreated fellow eye served as control. Results Three months after radiosurgery, the subjective dry eye symptom score and lissamine green staining score of the ocular surface were significantly higher in the treated eyes compared with the fellow eyes (p<0.001, p=0.028, respectively). After 12 months, a significant difference between the treated and the fellow eyes in corneal sensitivity (p=0.041) and corneal fluorescein staining (p=0.002) was found when compared with pretreatment values. After 24 months Schirmer test values without local anaesthesia were significantly reduced in the treated eyes vis-à-vis untreated fellow eyes and pretreatment values (p=0.004). The dose applied to the lacrimal gland was significantly correlated to ocular surface staining scores (p=0.001) and Schirmer test values (p=0.026) at 24 months after irradiation. Conclusions Stereotactic single-fraction Gamma-Knife radiotherapy of choroidal melanoma with a median dose of 30 Gy significantly affected ocular surface and tear function and increased dry eye symptoms and signs.

Local tumour control and eye preservation after gamma-knife radiosurgery of choroidal melanomas

Background/aims To report on local tumour control and eye preservation after gamma knife radiosurgery (GK-RS) to treat choroidal melanomas. Methods A total of 189 patients with choroidal melanoma were treated with GK-RS, with treatment doses between 25 and 80 Grays. The main outcome measures of our retrospective analysis were local tumour control, time to recurrence, eye retention rate and the reason for and time to secondary enucleation. Patient-associated, tumour-associated and treatment-associated parameters were evaluated as potential risk factors. Results Local tumour control was achieved in 94.4% of patients. The estimated tumour control rates were 97.6% at 1 year, 94.2% at 5 years and 92.4% at 10 years after treatment. Recurrence was observed between 3.1 months and 60.7 months post-treatment (median: 13.5 months). Advanced tumour stage (Tumour, Node, Metastasis (TNM) 3–4) was the most important risk factor for recurrence (Fine-Gray model; subhazard ratio, SHR: 3.3; p=0.079). The treatment dose was not related to tumour recurrence. The eye preservation rate was 81.6% at 5 years after treatment, remaining stable thereafter. Twenty-five eyes (14.1%) had to be enucleated at between 17 days and 68.0 months (median: 13.9 months) after GK-RS, and advanced tumour stage (Cox model; p=0.005), treatment dose (p=0.048), pretreatment visual acuity (p=0.016), and retinal detachment (p=0.027) were risk factors for requiring enucleation. Conclusions GK-RS achieved a high tumour control rate, comparable to linear accelerator-based radiotherapy. Advanced TNM stage was a predictive risk factor for tumour recurrence and for secondary enucleation after GK-RS. Lower treatment doses were unrelated to tumour recurrence, although they were associated with an improved eye retention rate.

Ruthenium-106 plaque brachytherapy for uveal melanoma

Background To report on local tumour control, eye preservation and visual outcome after ruthenium-106 brachytherapy for uveal melanoma. Methods Medical records of 143 eyes with uveal melanoma, treated by ruthenium-106 brachytherapy between 1997 and 2012 at one single centre, were included. Primary outcome measures were local tumour control, eye preservation and visual outcome. The influence of patient, tumour and treatment parameters on outcome was analysed by time to event analysis and competing risk regression. Results The median overall follow-up was 37.9 months. Tumour control: recurrent tumour growth was observed in 17 patients. The estimated local tumour recurrence rate at 12, 24 and 48 months after irradiation was 3%, 8.4% and 14.7%, respectively. The only significant risk factors for tumour recurrence were age (p=0.046) and reduced initial visual acuity (VA, p=0.045). No significant difference could be shown for tumour size or tumour category (T1–T2 vs T3–T4), and for any other tumour or treatment parameters (including combined transpupillary thermo-therapy (TTT)). Eye preservation: The likelihood of keeping the eye 12, 24 and 48 months after irradiation was 97.7%, 94.7% and 91.8%, respectively. Most significant risk factors for secondary enucleation were initial VA (p<0.001), tumour height (p=0.002) and tumour category (p=0.015). Vision The chances of keeping VA of 20/200 or better at 1, 2 and 5 years after treatment were 86.4%, 80.8% and 61.7%, respectively. Patients receiving sandwich-TTT showed significantly worse visual outcomes. Conclusions Ruthenium-106 brachytherapy appears to be a useful treatment regarding tumour control, eye preservation and visual function. Adjunct sandwich therapy resulted in worse visual outcome.

Bulbuserhalt und Funktion nach Endoresektion und Gamma-Knife Radiochirurgie uvealer Melanome

SummaryEyes with uveal melanoma sometimes need surgical intervention cause of radiation induced retinal complications. We report visual outcome, complications and globe preservation rates. 13 patients (10 male, 3 female) with a mean age of 64 years (range 42–88 years) were treated by single fraction Gamma Knife radiosurgery in 12 patients, two had previous thermotherapy and in one patient Ruthenium 106 brachytherapy was administered. All were subsequently endoresected by pars plana vitrectomy and silicone oil tamponade. In six patients period was less than six months (median, 2 months) and in seven patients period was more than six months (median, 19 months). Mean tumor base was 12.5 mm (range 8.3–20 mm), mean tumor prominence 9 mm (4.4–16.5 mm). Visual acuity was LP-20/400 in five, 20/200–20/40 in seven and 20/20 in one patient before radiation. Globe preservation was achieved in 12/13 (92 %), in 13/13 total tumor regression without any recurrence was achieved. Visual acuity after surgery was NLP in five patients, in seven patients LP-20/400 and in one patient 20/200. No clinically significant difference in visual acuity was seen in the group with endoresection earlier or later than six months. Seven additional operations after primary vitreoretinal surgery had to be performed (four vitrectomies, one enucleation and one lavage of the anterior chamber). Two patients developed distant metastases. Our data show that combined vitreoretinal surgery and endoresection after radiation are an acceptable management option for preservation of the globe and the residual visual function in uveal melanomas with retinal detachment or vitreous bleeding, yet visual function is determined by radiation induced side effects and the duration of the retinal detachment.ZusammenfassungIn den letzten Jahren hat die Endoresektion uvealer Melanome nach Vorbestrahlung als Therapieoption vor allem großer Tumore einen zunehmenden Stellenwert gewonnen. In dieser konsekutiven Fallstudie werden die Ergebnisse früher Resektionen denen später Resektionen (mehr als sechs Monate nach Bestrahlung) nach stereotaktischer Radiotherapie gegenüber gestellt. Insgesamt 13 PatientInnen (10 Männer, 3 Frauen) mit einem mittleren Alter von 64 Jahren (42–88 Jahre) wurden in diese Studie inkludiert. Bei 12 PatientInnen wurde eine Gamma-Knife Radiochirurgie durchgeführt, davon waren zwei mit Thermotherapie vorbehandelt. In einem Fall wurde primär eine Brachytherapie mit Ruthenium 106 durchgeführt. Die mittlere Tumorbasis betrug 12,5 mm (8,3–20 mm), die mittlere Tumorprominenz 9 mm (4,4–16,5 mm). Der Visus lag vor der Bestrahlung bei fünf PatientInnen zwischen Lichtprojektion und 0,05, zwischen 0,1 und 0,5 bei sieben PatientInnen und 1,0 bei einem Patienten. Bei allen PatientInnen wurde eine Vitrektomie mit anschließender Endoresektion und Silikonöltamponade nach initialer Bestrahlung durchgeführt. Primäre Ergebnisparameter waren Bulbuserhalt und Funktion nach Endoresektion. Eine totale Tumorregression ohne Rezidiv wurde in allen Augen, ein Erhalt des Bulbus wurde in 12/13 (92%) Augen erzielt. Der Visus lag bei insgesamt sieben PatientInnen zwischen Lichtprojektion und 0,05, bei einer/m PatientIn bei 0,1, insgesamt fünf PatientInnen zeigten kein Lichtempfinden. Bezüglich Visus und anatomischen Ergebnis bestand kein Unterschied zwischen der Gruppe mit früher (weniger als sechs Monaten nach Bestrahlung, sechs PatientInnen) und später (mehr als sechs Monate) Endoresektion. Bei sechs Augen war eine weitere Vitrektomie erforderlich, bei einem eine Vorderkammerspülung. Ein Auge musste enukleiert werden. Zwei PatientInnen entwickelten Metastasen im Beobachtungszeitraum. Eine Gamma-Knife Bestrahlung in Kombination mit Endoresektion ist eine erfolgreiche Behandlungsstrategie im Hinblick auf Bulbuserhalt und Erhalt einer Restfunktion bei großen Tumoren oder Tumoren mit Begleitamotio, die Sehfunktion wird durch radiogene Schäden bzw. Dauer der Netzhautabhebung definiert.

Genetik des Aderhautmelanoms

SummaryUveal melanoma is a rare sporadic tumor with high mortality. Despite all advances in the treatment of this malignancy, mortality rate has not improved over the past decades. Tremendous advances in research on the development of uveal melanoma and metastatic disease have changed our understanding of this disease over the past years. New insights into the genetic and molecular fundamentals of uveal melanoma show first impacts in the clinical management of affected patients, and there is reason for hope, that the development of new treatments will improve the still poor prognosis. The objective of this work is to provide an overview on our current understanding of clinical genetics, cyto- and molecular genetics, to explain recent research in this field and to describe how recent findings have already begun to change the treatment of uveal melanoma patients.ZusammenfassungDas Aderhautmelanom ist ein seltener sporadischer Tumor mit einer hohen Mortalitätsrate. Trotz aller Fortschritte in der Behandlung des Tumors hat sich die Überlebensrate der PatientInnen seit Jahrzehnten nicht verbessert. Die Erforschung der Grundlagen der Entstehung und Metastasierung des Aderhautmelanoms hat in den letzten Jahren rasche Fortschritte gezeigt. Das verbesserte Verständnis der genetischen und molekularen Grundlagen dieses Tumors zeigt nun erste Auswirkungen auf die klinische Praxis, und es besteht Hoffnung auf die Entwicklung verbesserter Therapien und eine verbesserte Überlebensprognose. Die vorliegende Arbeit gibt einen Überblick über die klinische Genetik, die Zytogenetik und die Molekulargenetik des Aderhautmelanoms, schildert neuere Forschungsergebnisse in diesen Teilbereichen und beschreibt die Auswirkungen in der Betreuung ophthalmo-onkologischer Patienten.

Gender differences in open globe injuries in children

SummaryBackgroundOpen globe injuries are characterized by at least one full-thickness wound of the eyewall (cornea and sclera). Their causes and prognosis vary strongly with the country in which they occur. This study gives an overview about the injury circumstances and visual outcome in children in the catchment area of the University Department of Ophthalmology of Graz (Styria, parts of Burgenland, and Carinthia).Material and methodsThe patient records of all children < 18 years of age who were operated on between 1992 and 2011 at the University Department of Ophthalmology of Graz because of open globe injuries were analyzed. Epidemiologic data as well as the functional and anatomical outcome were evaluated with a special focus on gender differences. Only children with a follow-up of at least 1 year were included in the analysis of the visual outcome.ResultsWe observed a preponderance of males (p < 0.001) in our collective of 104 children; 81 (77.9 %) patients were boys, and 23 (22.1 %) were girls. There was a gender-specific difference of the age at injury: the girls were injured at a significantly younger age than the boys (median: 6 versus 11 years, p = 0.001). The patients sustained 76 (73.1 %) penetrating injuries, 19 (18.3 %) injuries with an intraocular foreign body, 6 (5.8 %) ruptures, and 3 (2.9 %) perforating injuries. The most frequent injury objects were tools (n = 21, 20.2 %), sticks and arrows (n = 18, 17.3 %), and glass fragments (n = 12, 11.5 %). Of 88 patients with recorded final visual acuity (FVA), 52 (59.1 %) recovered a vision of at least 20/40; 14 (15.9 %) went unilaterally blind. The FVA significantly improved (p < 0.001) compared with the initial visual acuity.ConclusionThe majority of victims were teenage boys, and the most frequent injury objects were tools. Injuries in girls occurred at a younger age than those in boys.ZusammenfassungHintergrundBulbuseröffnende Augenverletzungen sind charakterisiert durch mindestens eine Wunde, die die Hornhaut oder Sklera in ihrer gesamten Dicke durchbricht. Für ihre Ursachen und ihre Prognose gibt es deutliche länderspezifische Unterschiede. Diese Studie gibt einen Überblick über die Verletzungsumstände und den Endvisus von Kindern im Einzugsgebiet der Universitätsaugenklinik Graz (Steiermark, Teile von Burgenland und Kärnten).Material und MethodeWir analysierten die Daten von allen Kindern unter 18, die zwischen 1992 und 2011 aufgrund einer bulbuseröffnenden Verletzung an der Universitätsaugenklinik Graz operiert wurden. Epidemiologische Daten, sowie das funktionelle und anatomische Endergebnis wurden, mit besonderer Begutachtung der Geschlechtsunterschiede, evaluiert. In die Analyse des Endvisus inkludierten wir nur Kinder mit einer Nachbeobachtungszeit von mindestens einem Jahr.ResultateIn unserem Kollektiv von 104 Kindern befanden sich signifikant mehr Buben (81 = 77,9 %) als Mädchen (23 = 22,1 %, p < 0,001). Ein geschlechterspezifischer Unterschied trat auch im Verletzungsalter auf: Die verletzten Mädchen waren deutlich jünger als die verletzten Buben (Median sechs und elf Jahre, p = 0,001). Wir operierten 76 (73,1 %) penetrierende Verletzungen, 19 (18,3 %) mit intraokularem Fremdkörper, sechs (5,8 %) Rupturen und drei (2,9 %) perforierende Verletzungen. Die häufigsten Verletzungsobjekte waren Werkzeuge (n = 21, 20,2 %), Stecken und Pfeile (n = 18, 17,3 %) und Glassplitter (n = 12, 11,5 %). Von den 88 Patienten mit dokumentiertem Endvisus betrug die Sehschärfe bei 52 (59,1 %) zumindest 0,5, 14 (15,9 %) erblindeten. Der Endvisus war signifikant besser als der Aufnahmevisus (p < 0,001).SchlussfolgerungDie Mehrzahl der Verletzten waren männliche Teenager, die häufigsten Verletzungsobjekte Werkzeuge. Die Mädchen waren zum Zeitpunkt der Verletzung signifikant jünger als die Buben.

Tumor parameters predict the risk of side effects after ruthenium-106 plaque brachytherapy of uveal melanomas

Background To report on radiation-related side effects and complications after ruthenium-106 plaque brachytherapy of uveal melanomas. Methods Medical records of 143 eyes with uveal melanoma, treated by ruthenium-106 brachytherapy between 1997 and 2012 at a single center, were analyzed. We evaluated the occurrence of radiation-related side effects on the anterior and posterior segment of the eye. The influence of patient, tumor and treatment parameters on outcome was analyzed by multivariate time to event analysis considering competing risks. Results The median overall follow-up was 37.9 months. After treatment, the estimated risk at 12, 24 and 48 months for developing anterior segment complications was 25.3%, 37.5% and 50.3% for cataract formation and 5.4%, 6.4% and 8.1% for secondary glaucoma, respectively. The estimated risk for the occurrence of posterior segment complications 12, 24 and 48 months after treatment was 3.1%, 6.7% and 18.3% for radiation retinopathy, 18.3%, 27.1% and 42.6% for radiation maculopathy and 16.5%, 21.0% and 32.8% for radiation neuropathy, respectively. The risk of an increase in retinal detachment after treatment was 14.7%, 14.7% and 17.4% at 12, 24 and 48 months, respectively. The risk of vitreous hemorrhage occurring after treatment was 6.2%, 8.1% and 12.7%, and the risk of tumor vasculopathy was 15.4%, 17.4% and 19.0%. Scleral necrosis was observed in one patient. Conclusion Radiation-related side effects and complications are common among patients treated with ruthenium brachytherapy for uveal melanoma. However, the risk for those largely depends on individual tumor parameters. Before treatment, patients should be informed of their specific risks to develop various side effects. Patient information before treatment should cover not only general information about the treatment and possible complications and side effects but should also give details on the specific risks of the patient in her individual situation. This also includes elucidating the patients individual resources and expectations and her willingness for long-term regular follow-up examinations and secondary adjunct treatments.