[Bilateral uveal melanoma: a case report]. / Bilaterales uveales Melanom: ein Fallbericht.

BACKGROUND: The occurrence of bilateral uveal melanoma is rare. This is the first reported case of bilateral melanoma of the choroidea in Austria. METHODS: The chart of a 62-year-old male patient with bilateral melanoma of the choroidea was reviewed. RESULTS: In October 1992, a patient was diagnosed with a choroidal melanoma in the left eye. After two treatments with a ruthenium applicator in 1992 and 1995, the left eye was enucleated in 1999 due to recurrent growth of the choroidal melanoma. In September 2005, the patient presented with a primary choroidal melanoma in the remaining right eye. Sonography of the liver as well as CCT and bone marrow scintigraphy showed no sign of metastatic disease. A ruthenium-106 brachytherapy was performed and the size and height of the melanoma showed decreases. In July 2006, the patient developed metastatic disease and died the following month due to metastasis of the liver. In the genetic analysis, monosomy 3 was detected in both melanoma, an amplification of c-MYC of chromosome 8 was detected in the melanoma of the right eye only. CONCLUSION: Bilateral uveal melanoma is a rare diagnosis. This case shows the importance of a thorough and recurrent examination of the second eye. The results of the genetic analysis are discussed.

[Foreign bodies of the iris as a rare differential diagnosis of malignant melanoma of the iris]. / Irisfremdkörper als seltene Differenzialdiagnose zum Irismelanom.

BACKGROUND: Lesions of the iris require a thorough examination to differentiate them from malignant melanoma because of the severe therapeutic consequences of the latter entity. METHODS: We observed two cases with a unilateral lesion on the iris, both suspicious of malignant melanoma of the iris. Ophthalmological, echographic and ultrasound biomicroscopic examinations were done. RESULTS: The cases of a 66-year-old man with a metallic foreign body and a 37-year-old man with a foreign body, both located on the iris, are presented. CONCLUSION: In both patients an unclear lesion of the iris, suspicious of a malignant melanoma of the iris, turned out to be a foreign body after ophthalmoscopic examination. Both echography and ultrasound biomicroscopy were carried out and proved to be useful to confirm the diagnosis. No ocular inflammations, siderosis or secondary glaucoma occurred in the described cases. Observation of dormant foreign bodies is sufficient.

Gonioscopic changes after implantation of a posterior chamber lens in phakic myopic eyes.

PURPOSE: To evaluate the effects of posterior chamber implantation of phakic intraocular lenses on angle structures and the correlation with intraocular pressure (IOP). SETTING: Department of Ophthalmology, University Hospital of Vienna, Vienna, Austria. METHODS: A Staar implantable contact lens (ICL) was implanted in 20 white patients (34 eyes) with high myopia. The patients were followed for a mean of 2.3 years (range 0.8 to 6.0 years). Two subgroups were created: One had a follow-up of 2.4 to 6.0 years and the other, 0.8 to 2.3 years. The mean spherical power of the implanted ICL was -16.8 diopters (D) (range -9.0 to -21.0 D). The angle structures were examined by gonioscopy using the Spaeth technique, and optical pachymetry and IOP were measured in dilated and undilated pupils. Trabecular pigmentation was measured semiquantitatively. The ICL models included a few prototypes (6 eyes), the V2 model (10 eyes), the V3 model (6 eyes), and the V4 model (12 eyes). RESULTS: The anterior chamber angle was between 11 and 20 degrees in 41% of eyes, between 21 and 30 degrees in 21%, larger than 30 degrees in 21%, and 10 degrees or smaller in 17%. An apparent iris root insertion was above Schwalbe's line in 6% of eyes, behind Schwalbe's line in 12%, at the scleral spur in 67%, and in a deep angle recess behind the scleral spur in 15%. The curvature of the iris was regular in 19 eyes and steep in 15 eyes. The mean pigmentation in all 4 quadrants was 1.3. (Grade 1 was defined as trace pigmentation.) The mean pachymetry from the endothelium to the ICL anterior surface was 2.7 mm and from the endothelium to the anterior surface of the crystalline lens, 3.2 mm. The mean IOP was 14.1 mm Hg in undilated pupils and 12.1 mm Hg in dilated pupils. There was no correlation between angle pigmentation and IOP. CONCLUSION: Trace pigmentation in the trabecular meshwork was common after ICL implantation. However, there was no direct influence on IOP. Long-term follow-up is necessary to evaluate the effect and progress of trabecular pigmentation in eyes with an ICL.

Ocular blood flow in patients infected with human immunodeficiency virus.

PURPOSE: Alterations of ocular blood flow may play a role in the pathophysiology of human immunodeficiency virus (HIV) related retinal microvasculopathy. In this study ocular blood flow was investigated in patients with HIV infection. DESIGN: In a prospective, cross-sectional study ocular blood flow was measured in 37 eyes of consecutive HIV- infected persons and compared with the data of age-matched healthy controls. This sample size was calculated based on an alpha-error of 0.5 and a beta-error of 0.8. METHODS: Macular white blood cell flow, fundus pulsation amplitude, and blood flow velocities in the retrobulbar vessels were measured with blue field entoptic technique, laser interferometry, and Doppler sonography, respectively. Immunologic and ophthalmologic status was evaluated from each patient. RESULTS: Mean CD4+ cell count of the HIV-infected persons was 206.8 +/- 145.6 cells/mm(3). In five patients HIV-related retinopathy was observed. A significant reduction in leukocyte density was seen in HIV infected persons (82.2 +/- 23.4) as compared with the control group (102.0 +/- 28.4; P =.019). The resistive index in the central retinal artery was higher in HIV infected patients (0.77 +/- 0.05) as compared with the controls (0.74 +/- 0.04; P =.04). The other hemodynamic parameters were not different between groups. No correlation of flow parameters and CD4+ cell count or HIV-related retinopathy was observed. CONCLUSIONS: Decreased macular leukocyte density was detected in HIV infected persons. Our study suggests that abnormal retinal hemodynamics in individuals infected with HIV may be involved in the pathogenesis of HIV-related microvasculopathy.

Contact lens induced keratitis associated with contact lens wear.

PURPOSE: To evaluate clinical characteristics, risk factors, management and outcome of contact lens induced keratitis (CLIK) associated with contact lens wear. METHODS: The study comprised all consecutive patients presenting with contact lens related presumed microbial keratitis during a 12-month period at our department. Detailed demographic data, type of contact lens, duration of lens wear, wearing schedule and lens hygiene were derived from a self-administered questionnaire. Severity of ulcer, corneal scrapings, treatment and final outcome were evaluated. RESULTS: 27 patients with CLIK, some of which may have been sterile peripheral infiltrates, due to contact lens wear were detected. In the majority of the cases patients had used disposable soft contact lenses (89%) and most of them had a daily wearing schedule (78%). 51.8% patients cleaned their lenses regularly. 6 (22%) applied no disinfection since their lenses were disposable on removal. In 3 of our cases with CLIK, penetrating keratoplasty had to be performed. CONCLUSION: Disposable contact lenses seem to have been a predisposing factor for contact lens induced keratitis also when used on a daily wear schedule. Insufficient contact lens care products and/or manufacturing characteristics may be responsible for CLIK, which is also observed in otherwise compliant contact lens users. In 3 of our patients with CLIK keratoplasty became necessary, indicating that contact lens induced keratitis may result in severe corneal complications.

Astigmatism correction with a foldable toric intraocular lens in cataract patients.

PURPOSE: To determine the efficacy and rotational stability of a toric posterior chamber silicone intraocular lens (IOL) to correct preoperative astigmatism in cataract patients. SETTING: Department of Ophthalmology, University Hospital of Vienna, Vienna Austria. METHODS: Between 1993 and 1998, foldable toric single-piece plate-haptic silicone IOLs were implanted in 37 eyes (30 patients). The cylindrical IOL power was 2.00 diopters (D) (n = 29), 3.50 D (n = 7), or 4.00 D (n = 1). Phacoemulsification was performed through a scleral or a corneal sutureless self-sealing incision. Outcomes of Snellen visual acuity (without, with spherical, and with best correction), refractive and keratometric astigmatism, and IOL rotation after early postoperative (mean 15.9 days +/- 10.1 [SD]) and long-term (mean 20.3 +/- 16.6 months) follow-ups were evaluated. RESULTS: At last follow-up, 31 eyes (83.8%) had a spherically corrected and 34 (91.9%) a best corrected visual acuity of 0.5 (20/40) or better. Mean preoperative refractive and keratometric astigmatism was 2.68 and 2.70 D, respectively. At the last postoperative follow-up, mean refractive astigmatism was reduced to 0.84 D; keratometric astigmatism was 2.30 D. In 7 eyes (18.9%), the IOL axis was rotated a maximum of 25 degrees. In all 37 eyes, the axis of the toric IOL remained within 30 degrees of rotation. CONCLUSIONS: Early postoperative and long-term follow-ups showed effective and stable correction of astigmatism after implantation of a foldable toric posterior chamber silicone IOL.

Local tumor control and morbidity after one to three fractions of stereotactic external beam irradiation for uveal melanoma.

BACKGROUND AND PURPOSE: To evaluate prospectively local tumor control and morbidity after 1-3 fractions of stereotactic external beam irradiation (SEBI) in patients with uveal melanoma, unsuitable for ruthenium-106 brachytherapy or local resection. MATERIAL AND METHODS: This phase I/II study includes 62 selected patients with uveal melanoma. The mean initial tumor height was 7.8+/-2.8 mm. With the Leskell gamma knife SEBI, 41 patients (66%) were irradiated with two equal fractions of 35, 30 or 25 Gy/fraction, 14 patients (22%) were treated with three fractions of 15 Gy each, and seven patients (11%) with small tumor volumes below 400 mm(3) were treated with one fraction of 45 Gy. The mean total dose was 54+/-8 Gy. The minimal follow-up period was 12 months, and the median follow-up was 28.3 months. Data on radiation-induced side-effects were analyzed with the Cox proportional hazards model for possible risk factors. RESULTS: Local tumor control was achieved in 98% and tumor height reduction in 97%. The mean relative tumor volume reductions were 44, 60 and 72% after 12, 24 and 36 months, respectively. Seven patients developed metastases (11%). Secondary enucleation was performed in eight eyes (13%). Morbidity was significant in tumors exceeding 8 mm in initial height; it was comparable and acceptable in those smaller. In the stepwise multiple Cox model, tumor localization, height and volume, planning target volume (PTV), total dose and patient age were identified as the strongest risk factors for radiation-induced lens opacities, secondary glaucoma, uveitis, eyelash loss and exudative retinal detachment. In this model, the high-dose volume irradiated with more than 10 Gy/fraction was the strongest risk factor for radiation-induced uveitis. CONCLUSIONS: Stereotactic external photon beam irradiation and a total dose of 45-70 Gy delivered in one to three fractions are highly effective at achieving local tumor control in uveal melanoma. Further clinical studies using smaller fraction doses, and consequent smaller high-dose volumes, are justified to optimize dose and fractionation. Fractionated stereotactic irradiation has a challenging potential as an eye-preserving treatment in uveal melanoma.

Ocular arterial blood flow of choroidal melanoma eyes before and after stereotactic radiotherapy using Leksell gamma knife: 2 year follow up.

AIMS: To evaluate the effect of high dose stereotactic radiotherapy on the ocular blood flow of patients with uveal melanoma. METHODS: Colour Doppler imaging (CDI) was used to measure blood flow velocity and vascular resistance in the ophthalmic, short posterior, and central retinal arteries of nine patients suffering from uveal melanoma. The measurements were taken before, 6 months, 1 year, and 2 years after stereotactic radiotherapy. Irradiation was performed with the Leksell gamma knife with the 59 (41-66.5) Gy total marginal dose divided in two equal fractions. CDI results were compared with age and sex matched healthy control eyes. RESULTS: At each time of measurement, blood flow velocity in the central retinal artery of the affected eyes was significantly reduced whereas vascular resistance was only increased at the 2 year follow up. Blood flow velocity and vascular resistance in the short posterior arteries of melanoma eyes were also only significantly altered at the 2 year follow up. Blood flow velocity and vascular resistance in the ophthalmic artery of melanoma eyes were not changed at all follow ups. CONCLUSIONS: In the melanoma eyes, blood flow velocity in the central retinal artery is reduced. High dose stereotactic radiotherapy with the Leksell gamma knife and a 59 (41-66.5) Gy total marginal dose in two fractions leads to a significant reduction of blood flow and a significant increase in resistance variables in the small ocular arteries within 2 years.

Control of cytomegalovirus retinitis after combination antiretroviral therapy.

PURPOSE: To report on AIDS patients having combination antiretroviral therapy whose cytomegalovirus (CMV) retinitis remained inactive after discontinuation of anti-CMV maintenance therapy. METHODS: We describe the course of CMV-retinitis in 3 patients with AIDS after initiation of combination antiretroviral therapy. RESULTS: After cessation of anti-CMV therapy no relapse of CMV-retinitis has been observed for up to 18 months. Two of the patients developed new CMV-retinitis in the first months after initiation of combination therapy, nevertheless after further improvement of immunological parameters retinitis remained stable without anti-CMV therapy. CONCLUSION: The sustained immunological effects of combination therapy are possibly sufficient enough to provide protection against CMV-retinitis.

Fractionated stereotactic radiotherapy with linear accelerator for uveal melanoma--preliminary Vienna results.

PURPOSE: To study local tumor control and radiogenic side-effects after fractionated stereotactic radiotherapy for uveal melanoma. PATIENTS AND METHODS: Between June 1997 and February 1998, 21 patients suffering from uveal melanomas have been treated with stereotactic 6 MeV LINAC (Saturne 43, General Electric, France) in conjunction with a stereotactic frame system (BrainLAB, Germany). Immobilization of the eye was ensured with an optical fixation system which was proven reliable. During radiotherapy, movements of the irradiated eye were controlled on a monitor and documented by video recording. All patients co-operated very well with the optical fixation system. In 1164 measurements, the median value of horizontal deviation of the diseased eye during treatment was 0.3 mm (range: 0 to 1.3 mm). Median vertical deviation was 0.2 mm (range: 0 to 1.2 mm). For all patients, mean tumor prominence before treatment was 6.0 +/- 2.2 mm. In 20 patients, the total dose of 70 Gy (at 80%) was delivered in 5 fractions within 10 days. In one patient with a ciliary body tumor, the total dose of 70 Gy was divided into 7 fractions for better sparing of the anterior eye segment. RESULTS: After a follow-up of at least 6 months, local tumor control was seen in all eyes. Mean tumor thickness reduction after 3, 6 and 9 months was 7%, 13% and 31%, respectively. Up to now, only mild subacute side-effects located in the anterior eye segment have been noticed. CONCLUSION: Optical fixation of the eye allows high precision stereotactic radiotherapy with small safety margins. Fractionated stereotactic radiotherapy and 70 Gy total dose delivered in 5 fractions seems to be appropriate for local tumor control in uveal melanoma. Further long-term studies with extended number of patients will be necessary to conclude on the use of linac-based fractionated stereotactic radiotherapy for uveal melanoma.

Three-year clinical outcome after penetrating keratoplasty for keratoconus with the guided trephine system.

PURPOSE: To determine the long-term clinical outcome after keratoplasty with the guided trephine system in keratoconus eyes. METHODS: In a prospective study, all consecutive cases of penetrating keratoplasty had trephination performed with the guided trephine system, with which both donor and recipient cornea are trephined from the epithelial side with a same-sized blade. For wound closure, a double running antitorque suture technique with 10-0 nylon was used. Uncorrected and best-corrected Snellen visual acuity, subjective refraction, and astigmatism by keratometry were evaluated after final suture removal, 2 and 3 years postoperatively. RESULTS: In the 31 patients (31 eyes) enrolled, mean best-corrected visual acuity improved from 0.72 +/- 0.16 (20/30) after final suture removal to 0.88 +/- 0.15 (20/25) 3 years postoperatively (P < .001). The mean spherical equivalent increased from -0.86 +/- 2.10 diopters after final suture removal to -2.35 +/- 2.65 diopters 3 years postoperatively (P < .001). Mean keratometric astigmatism decreased from 4.68 +/- 1.76 diopters after final suture removal to 3.57 +/- 1.37 diopters 3 years postoperatively (P = .001). Furthermore, an increase in mean keratometric levels with time (P = .01) was observed and associated with myopic shift (r(s) = -.46, P = .008). CONCLUSION: With the guided trephine system, we attained favorable visual results, with prolonged improvement of visual acuity during the entire follow-up period. Our data show low and decreasing degrees of corneal astigmatism over time. During the follow-up period, a myopic shift was found after final suture removal. Nevertheless, this technique of performing same-sized grafts reduces postoperative residual myopia.

Evaluation of cytomegalovirus retinitis management. Outcome of four years of cytomegalovirus therapy.

Cytomegalovirus (CMV) is the most common pathogen of opportunistic viral infections in patients with the acquired immunodeficiency syndrome. In this study, we assessed the therapeutic outcome of our treatment regimen of CMV retinitis by analysing retrospectively 33 consecutive patients. The clinical utility of CMV cultures from blood, urine and throat specimens obtained at the time of diagnosis was additionally evaluated. Treatment started with ganciclovir (GCV) therapy. In case of relapsing retinitis, re-induction therapy was initiated, and if unsuccessful, the patient was switched to foscarnet. Patients developing resistant retinitis despite foscarnet therapy were offered a GCV-foscarnet combination therapy. Under primary GCV therapy, the median first stable interval of the whole group was 202 days (mean 238 days). Twenty-five out of 33 CMV retinitis patients (76%) responded to initial GCV therapy. Eleven of these patients showed relapsing retinitis that could be stabilised in 3 patients solely with combination therapy. Eight patients did not respond to primary GCV therapy. Three of them improved with foscarnet, but 3 patients did not respond to either treatment. In 18 (56%) out of 32 patients, CMV cultures yielded positive results. Considering our series, we may conclude that in the majority of patients primary or secondary viral resistance can be overcome by dose increase, switching to the alternative drug or a combination therapy.

Ophthalmic and genetic screening in pedigrees with familial adenomatous polyposis.

PURPOSE: To study the possible association between ophthalmic findings, genetic status, and clinical course of the disease in Austrian pedigrees with familial adenomatous polyposis (FAP). METHODS: Thirty-nine members of 16 consecutive FAP families with 20 affected patients and 19 relatives with a 50% a priori risk to develop the disease were examined ophthalmologically. The intestinal status of all persons was established by colonoscopy. Direct or indirect molecular genetic analysis, or both, was possible in eight of the 16 FAP families. RESULTS: Congenital hypertrophy of the retinal pigment epithelium (CHRPE) was discovered in 15 (75%) of the 20 persons affected by familial adenomatous polyposis. Five (25%) of the patients with an established FAP were CHRPE-negative. Four of the 19 at-risk individuals were CHRPE-positive. According to DNA analysis, five of the 19 at-risk relatives had a high risk to develop a manifest disease. The ophthalmoscopic tests were in complete agreement with the molecular risk estimation. Furthermore, the combined results of endoscopy and ophthalmoscopy suggested a relationship between a positive CHRPE status and the severity of FAP. CONCLUSIONS: Ophthalmic examinations facilitate predictive diagnosis in FAP patients and first-degree relatives, permitting a noninvasive, highly reliable risk assessment. When present, CHRPE lesions are a reliable clinical marker for FAP in CHRPE-positive families. In CHRPE negative families, negative ophthalmic examinations are of no predictive value. The CHRPE status can add information about the location of the genetic mutation. The combination of an ophthalmic examination with DNA analysis and endoscopy improves the risk assessment of FAP carriers.