[Bilateral posterior persistent hyperplastic primary vitreous]. / Bilateraler posteriorer persistierender hyperplastischer primärer Glaskörper.

Bilateral persistent hyperplastic primary vitreous (PHPV) represents a rare entity of a congential malformation. This casuistic presents for the first time in the German literature the case of a 4-month-old child with bilateral posterior PHPV.

[Immunotherapy of uveal melanoma: vaccination against cancer. Multicenter adjuvant phase 3 vaccination study using dendritic cells laden with tumor RNA for large newly diagnosed uveal melanoma]. / Immuntherapie beim Aderhautmelanom: Vakzination gegen Krebs. Multizentrische adjuvante Phase-III-Impfstudie mit Tumor-RNA-beladenen dendritischen Zellen bei neu diagnostizierten, großen Uveamelanomen.

Uveal melanomas are the most common malignant tumors of the eye. With modern molecular biological diagnostic methods, such as chromosome 3 typing and gene expression analysis, these tumors can be categorized into highly aggressive (monosomy 3, class II) and less aggressive forms. This molecular biological stratification is primarily important for determining the risk of these tumors as no therapy is currently available that is able to prevent or delay metastases. A randomized study of patients with a poor prognosis (monosomy 3) is currently being carried out in order to determine whether a cancer vaccine prepared from autologous (patient's own) dendritic cells and uveal melanoma RNA can prevent or delay progression and further metastases of this extremely aggressive form of cancer. Inclusion in the uveal melanoma study, which hopes to provide a potential therapeutic option for patients, is only possible if patients are referred to an institution that is able to manufacture and provide this vaccination before the patient is operated on or treated with radiation. Untreated tumor material is necessary for producing the vaccine on an individualized patient basis.

[Successful brachytherapy of a clinically unapparent ciliary body melanoma]. / Erfolgreiche Brachytherapie bei klinisch inapparentem Ziliarkörpermelanom.

We report on a typical case of a ciliary body melanoma in a 59-year-old female, which was only noticed due to nonspecific unilateral vision disorders and which could only be seen in maximal mydriasis via a gonioscopic three-mirror lens. We further discuss tumor monitoring via ultrasound biomicroscopy (UBM) and execution and prognosis of Ru-106 brachytherapy.

[High-dose 106Ruthenium plaque brachytherapy for posterior uveal melanoma. A clinico-pathologic study]. / Hochdosisbrachytherapie des malignen Aderhaut- und Ziliarkörpermelanoms mit 106Ruthenium. Eine klinisch-pathologische Studie.

BACKGROUND: The purpose of this study was to report the multifactorial results of high-dose (106)Ruthenium plaque brachytherapy for (cilio-)choroidal melanoma and to confirm them by histological examinations. PATIENTS AND METHODS: 100 patients with choroidal or ciliochoroidal melanoma treated by high-dose 106Ruthenium plaque brachytherapy were followed-up for 5 years. 12 secondary enucleated eyes were compared to a non-irradiated matched group by light microscopy. RESULTS: The 5-year local tumour control rate was 93%, the 5-year survival rate 91%. Late radiogenic side effects occured as a retinopathy in 13%, as an optic neuropathy in 5% and as a secondary glaucoma in 3% of the patients. 14% had to be enucleated, 10% developed metastases. The histopathologic examination revealed significantly higher degrees of necrosis (p=0,041), balloon cell degeneration (p=0,025) and fibrosis (p<0,001) in the irradiated melanomas than in the control tumours. CONCLUSION: High-dose 106Ruthenium plaque brachytherapy turned out to be an effective treatment procedure for posterior uveal melanoma (not exceeding a prominence of 5,5 mm) with a high rate of local tumour control and a low rate of side effects.

Retinal redetachment after removal of intraocular silicone oil tamponade.

AIM: To evaluate frequency and risk factors of retinal redetachment after removal of intraocular silicone oil tamponade. METHODS: The study included 225 patients who consecutively underwent intraocular silicone oil removal at a mean interval of 10 months after pars plana vitrectomy had been performed by one of two surgeons. Mean follow up time was 17.37 (SD 14.40) months (range 3.02-67.42 months). RESULTS: In 57 of 225 (25.3%) patients, the retina detached after removal of silicone oil. Risk factors for retinal redetachment were the following: number of previously unsuccessful retinal detachment surgeries (p=0.0008); surgeon (p=0.007); visual acuity before silicone oil removal (p=0.009); incomplete removal of vitreous base (p=0.01); absence of an encircling band in eyes with proliferate vitreoretinopathy in which an inferior retinotomy had not been performed (p=0.01); and indication for pars plana vitrectomy. Rate of retinal redetachment was statistically (p>0.05) independent of the technique of silicone oil removal and duration of silicone oil endotamponade. CONCLUSION: Retinal redetachment after removal of silicone oil endotamponade can occur in approximately a fourth of patients, depending on the criteria to use and to remove silicone oil. Risk factors for recurrent detachment included the following: number of previously unsuccessful retinal detachment surgeries, surgeon, preoperative visual acuity, incomplete removal of the vitreous base, absence of an encircling band, and reason for pars plana vitrectomy. The rate of retinal redetachment is independent of the technique of silicone oil removal and duration of silicone oil endotamponade, with a minimal duration of silicone oil tamponade of about 3 months in the present study.

Intraocular pressure and silicone oil endotamponade.

PURPOSE: To evaluate intraocular pressure after instillation and eventual removal of silicone oil in patients undergoing pars plana vitrectomy combined with silicone oil endotamponade. METHODS: The study included 198 patients who underwent pars plana vitrectomy with silicone oil endotamponade (5,000 centistoke viscosity), in whom silicone oil was removed and in whom follow-up after oil removal was at least 3 months. All patients were operated on by one of two surgeons. RESULTS: After silicone oil instillation, intraocular pressure increased significantly (P < 0.001) from 12.9 +/- 4.4 mm Hg preoperatively to 16.1 +/- 5.5 mm Hg postoperatively. Intraocular pressure was statistically (P > 0.20) independent of the duration of silicone oil tamponade. Twenty percent of the 198 patients had at least one postoperative intraocular pressure measurement that was higher than 21 mm Hg. Main reasons for increased intraocular pressure were closed inferior iridectomy, iris neovascularisation, silicomacrophagocytic open-angle glaucoma secondary to silicone oil emulsification, and preoperative history of glaucoma. Glaucomatous optic nerve damage was detected in 14 (14 of 198, 7.1%) eyes, including 8 eyes with preoperative antiglaucoma treatment. Silicone oil emulsification occurring in 40 (40 of 198, 20.2%) patients did not statistically influence intraocular pressure after oil removal. Ocular hypotony occurred in 10 (10 of 198, 5.1%) patients after oil release leading to intraocular hemorrhages and loss of vision in 3 patients. CONCLUSION: Clinically significant increased intraocular pressure after pars plana vitrectomy with silicone oil endotamponade occurs relatively rarely, can usually be well controlled by topical antiglaucoma medication, and is reversible in most patients after oil removal. In patients with increased intraocular pressure and silicone oil endotamponade, oil removal may be preferred to invasive antiglaucoma surgery to reduce intraocular pressure.

Prognostic factors in ocular injuries caused by intraocular or retrobulbar foreign bodies.

OBJECTIVE: To evaluate prognostic factors associated with final visual outcome, development of posttraumatic infectious endophthalmitis, and occurrence of proliferative vitreoretinopathy in patients with penetrating ocular injuries caused by intraocular or retrobulbar foreign bodies (FBs). DESIGN: Clinic-based cross-sectional study. PARTICIPANTS: One hundred thirty patients presenting with penetrating ocular injuries caused by lacerations from FBs were operated on by one of two surgeons between 1989 and 1997. Follow-up time was an average of 20.84 +/- 20.76 months (median: 17.0 months). All FBs were located posterior to the lens. INTERVENTIONS: Pars plana vitrectomy; foreign body removal; additional surgical procedures according to the clinical situation. MAIN OUTCOME MEASURES: Postoperative visual acuity; posttraumatic infectious endophthalmitis; proliferative vitreoretinopathy. RESULTS: Occurrence of posttraumatic infectious endophthalmitis developing in seven patients (7/130 = 5.4%) was significantly (P = 0.026) associated with removal of the FB later than 24 hours after the accident and with the type of the FB (P < 0.01). Size (P = 0.37) of the FB, preoperative visual acuity (P = 0.62), presence of traumatic cataract (P = 0.75) or a retinal lesion by the FB (P = 0.16), age (P = 0.39), and gender (P = 0.46) did not show a statistically significant influence on the occurrence of endophthalmitis. Statistically significant risk factors for the development of proliferative vitreoretinopathy occurring in 27 patients (27 of 99 [27.6%] patients with a minimal follow-up of 3 months) were size of the FB (P < 0.001), preoperative visual acuity (P = 0.02), presence of a retinal lesion (P = 0.002), and traumatic cataract (P = 0.03). The time between FB removal and the accident was statistically marginally associated with the development of proliferative vitreoretinopathy (P = 0.07). Postoperative visual acuity depended significantly on size of the FB (P = 0.002), preoperative visual acuity (P < 0.001), presence of a retinal lesion (P = 0.049), and location of the retinal lesion (P < 0.001). Three eyes had to be enucleated because of endophthalmitis or phthisis bulbi. CONCLUSIONS: Prognosis in open-globe injuries with intraocular or retrobulbar foreign bodies depends on the size and type of the foreign body, presence and location of retinal lacerations, additional involvement of other intraocular structures, preoperative visual acuity, and timing of surgery. These factors may be important in preoperative counseling of the patient and for planning surgery.

Timing of retinal redetachment after removal of intraocular silicone oil tamponade.

PURPOSE: To evaluate the interval between removal of intraocular silicone oil tamponade and retinal redetachment after pars plana vitrectomy, and to investigate factors influencing the length of the interval. PATIENTS AND METHODS: The retrospective study included 42 eyes of 42 consecutive patients who experienced a retinal redetachment after silicone oil had been removed 8.0+/-6.2 months after an initial pars plana vitrectomy including intraocular silicone oil (5,000 centistokes) tamponade. Pars plana vitrectomy had been performed for proliferative vitreoretinopathy caused by complicated rhegmatogenous retinal detachment. RESULTS: The retina redetached 2 days to 5.5 months after silicone oil removal (mean +/- SD, 1.3+/-1.4 months; median, 18 days). Thirteen (30%) of all 42 redetachments occurred in the first 9 days, 21 (50%) of all 42 retinal redetachments occurred in the first 18 days, and 32 (75%) of all 42 retinal redetachments occurred in the first 50 days. The interval between silicone oil removal and retinal redetachment was statistically (by analysis of variance) independent of the method of silicone removal (transpupillary drainage vs via pars plana sclerotomies), refractive error of the eye (P = .62), time between initial pars plana vitrectomy and silicone oil removal (P = .99), visual acuity before silicone oil removal (P = .26), type of anesthesia (P = .69), gender (P = .80), and age (P = .48) of the patients. CONCLUSION: The risk of retinal redetachment decreases steeply with increasing time after silicone oil removal. Three to 5 months after oil removal, retinal redetachment becomes unlikely. The time of retinal redetachment is statistically independent of the method of silicone oil removal, refractive error, time between the preceding pars plana vitrectomy and silicone oil removal, visual acuity before silicone oil removal, type of anesthesia, and gender and age of the patents. These data may be important for scheduling reexaminations and for counseling patients in their planned activities after removal of intraocular silicone oil tamponade.

Retinal detachments by squash ball accidents.

PURPOSE: To evaluate the characteristics of retinal detachments caused by squash ball accidents. METHODS: Twenty-six patients had a retinal detachment after a squash ball hit their eyes. RESULTS: Characteristics of the 26 eyes with retinal detachment were large retinal tears parallel to the corneoscleral limbus located close to the ora serrata usually in the temporal superior fundus quadrant (in 14 [54%] of the 26 eyes) and in the temporal inferior quadrant (in seven eyes [27%]); primarily intact vitreous with a traumatic avulsion of the vitreous base in approximately one half the patients; relatively slow progression of the retinal detachment; and additional damage to the choroid and retinal pigment epithelium in the posterior fundus. The patients were young and did not have severe myopia. Reattachment of the retina was achieved in 22 (85%) of the 26 patients. In 11 patients (42%), visual acuity outcome was 20/40 or better with best correction. CONCLUSIONS: Retinal detachments after squash ball accidents show different characteristics with worse prognosis than do ordinary rhegmatogenous detachments. Protective eyewear should be worn when playing squash.

[Intraocular silicone oil tamponade. A clinico-pathologic study of 36 enucleated eyes]. / Intraokuläre Silikonöltamponade. Eine klinisch-pathologische Studie an 36 unukleierten Augen.

BACKGROUND: Previous histological studies have shown that intraocular silicone oil induces irreversible changes in ocular tissues, especially the retina. The purpose of this study was to analyze, in a larger group of enucleated eyes, changes in intraocular tissue after silicone oil injection, dependent on intraocular pressure, how long the oil was in the eye, and the viscosity of intraocular silicone oil. PATIENTS AND METHODS: We did histological examinations on 36 enucleated globes with intraocular silicone oil after vitreoretinal surgery and compared them with 68 enucleated globes treated with buckle and encircling band using immunohistochemistry (n = 5) and electron microscopy (n = 7). For statistical evaluation we used the chi(2) test and analysis of variance. RESULTS: After silicone oil injection we observed a more pronounced reduction in corneal endothelial cells (58%), more frequent closed chamber angle (86%), atrophy of the ciliary body (80%) (P < 0.05), proliferative vitreoretinopathy (89%), and glaucomatous atrophy of the optic nerve (56%) (P < 0.01). The retinae showed independent of the use of silicone oil a loss of inner and outer segments of photoreceptors and of ganglion cells and thinning and rareficaton of all other retinal layers. Globes with silicone oil revealed vacuoles both free and incorporated by macrophages in all layers of the retina. Similar vacuoles were seen in the optic nerve, choroid, retinal pigment epithelium, ciliary body, iris, chamber angle and the corneal endothelium. Silicone oil vacuoles were seen in the retina and optic nerve by 1 month after surgery in two eyes with high intraocular pressure (42 mmHg). Six of eight eyes with normal intraocular pressure levels showed retinal vacuoles, 3 of them after 2 months. Vacuoles in the optic nerve were found in eight of nine eyes with intraocular instillation of 1000 mPa silicone oil. There was no clinicohistopathological correlation between the presence of vacuoles in the retina or optic nerve and the duration and viscosity of intraocular silicone oil. CONCLUSIONS: This study suggests that vacuoles in eyes with silicone oil instillation can be found in the retina after 4 weeks. The period of intraocular silicone oil should be limited to 3-6 months.

Chronic postoperative endophthalmitis following cataract extraction and intraocular lens implantation. Report on nine patients.

Between 1987 and 1992, nine patients (three men and six women; mean age, 75 years) presented with chronic postoperative endophthalmitis after cataract extraction and intraocular lens (IOL) implantation. Eight patients were referred from another institution. The interval between IOL implantation and the beginning of intraocular inflammation averaged 5.6 months (range, 1-19 months). The referral diagnosis was "toxic lens" syndrome in seven patients. The main clinical findings were a hypopyon in the anterior chamber (n = 8) and in the capsule bag (n = 2), and "fibrosis" of the lens capsule (n = 7). In eight patients a vitrectomy was performed, combined with removal of the IOL in six cases and with a total posterior capsulectomy in five cases. Pathogenic organisms were identified in vitreous aspirate (6/8), in culture (3/3), and on histopathological examination (4/5) of lens capsule and included coagulase-negative staphylococci (n = 3, Staphylococcus epidermidis), Propionibacterium acnes (n = 1), Streptococcus viridans (n = 1), Rhodococcus erythropodes and R. luteus (n = 1), and one hyphomycete (Alternaria alternata). In chronic recurrent intraocular inflammations following IOL implantation, an infectious agent should be excluded by diagnostic and therapeutic pars plana vitrectomy including removal of the IOL and posterior capsulectomy.

Human retinal pigment epithelium in vitro: influence of low oxygen tension, glucose and insulin.

Retinal pigment epithelium (RPE) cells migrating through the damaged retina into the vitreous body seem to play an important role for the pathogenesis of proliferative vitreoretinopathy (PVR) and other proliferative retina diseases. It is so far not known how the RPE cells are able to survive in the vitreous body without contact to the blood vessels of the choroid supplying them with oxygen and nutritive substances. To answer this question, we studied growth characteristics and sensitivity to glucose and insulin of human RPE cells, incubated with reduced oxygen partial pressure. In the first study, RPE cultures of 58 postmortem human eyes were grown with 5% O2/5% CO2 and with standard conditions (20% O2/5% CO2). The growth was assessed in five graded stages. Our data show that human RPE grows better under 5% oxygen than under 20% O2 (p < 0.0001). In consideration of this effect, we cultivated, in a further study, pigment epithelium of 49 postmortem human eyes with 5% oxygen and with 4 different glucose concentrations with and without addition of insulin. We found that glucose in higher concentrations was a potent stimulator of growth, whereas insulin was a modest stimulator when used alone. The combination of glucose and insulin was significantly more effective (p = 0.01) in the period of the first 7 days. These results suggest that proliferation of human RPE cells can be increased by oxygen reduction, glucose and insulin. These interactions may help in understanding the pathophysiology of retina damage and proliferative retina diseases like PVR.

[Ocular manifestations of selected zoonoses in humans]. / Uber okuläre Manifestationen ausgewählter Zoonosen beim Menschen.

A report is given on the epidemiology and clinical signs of some selected zoonoses that may be of significance for ocular infections in man: brucellosis, leptospirosis, Lyme borreliosis, lymphocytic choriomeningitis, Newcastle Disease, ornithosis (chlamydiosis), rabies, Streptococcus suis infection, larva migrans ocularis by Toxocara canis or Baylisascaris procyonis, toxoplasmosis and tularemia.

[Morphometric and qualitative changes in corneal endothelium in primary chronic open angle glaucoma]. / Morphometrische und qualitative Veränderungen des Hornhautendothels bei primär chronischem Offenwinkelglaukom.

In a prospective study begun in March 1986, specular-microscopic endothelial cell photographs were taken in non-selected patients with primary, chronic, open-angle glaucoma who had not had intraocular surgery. Age-matched cataract eyes with normal intraocular pressure served as the control group. Standard specular microscopic examinations (Bio Optics LSM 2000 and Clinical Specular Microscope Camera) to evaluate the endothelial cell count, as well as the morphology and quality of the endothelium, revealed the following results. In 3204 normal eyes with normal intraocular pressure (10-20 mmHg), the cell count was observed to be age-dependent and to have an average value of 2293 +/- 394 cells/mm2; the cells generally had a regular arrangement. In 302 eyes with primary, chronic, open-angle glaucoma with intraocular pressures between 19 and 32 mmHg, there was a significant (p less than 0.001) reduction, in cell count to 1582 +/- 248 cells/mm2. The cell picture was composed of clearly enlarged, but regularly arranged cells. A statistical evaluation (matched age groups, etc.) was carried out using the Mann-Wilcoxon rank test.

[Epiphora as the leading symptom of Urbach-Wiethe syndrome in a sibling pair]. / Epiphora als Leitsymptom des Urbach-Wiethe-Syndroms bei einem Geschwisterpaar.

Urbach-Wiethe-Syndrom. syn. Hyalinosis cutis et mucosae is a rare, probably autosomal-recessively inherited disease. In the first weeks of life it begins with typical hoarness due to hyalin deposits in larynx and with increasing age leads to different changes in mucous membrane and later on in cutis. Etiology and pathogenesis are unknown. The treatment is only symptomatic. In our casuistic we present two sisters, who are constantly full of tears. Either show different stage of the typical representation of Hyalinosis cutis et mucosae. We demonstrate progression and penetrance of the disease and clinical alterations in eyes, skin and mucous membrane.

[Morphometric and qualitative changes in corneal endothelium in pseudoexfoliation syndrome]. / Morphometrische und qualitative Veränderungen des Hornhautendothels bei Pseudoexfoliationssyndrom.

In a prospective study begun in March 1986, in 182 eyes with pseudoexfoliation syndrome (PES) specular microscopic endothelial cell photographs were taken with direct and non-direct specular microscopy in non-selected patients who had not undergone intraocular surgery. Age-matched cataract eyes with normal intraocular pressure served as a control group. Standard specular microscopic examination (Bio Optics LSM 2000 A and Clinical Specular Microscope Camera) to evaluate the endothelial cell count and the morphology and quality of the endothelium yielded the following results. In 4432 control eyes with normal intraocular pressure the cell count was observed to be age-dependent and to have an average value of 2302 +/- 394 cells/mm2, the cells generally had a regular arrangement. In 123 eyes with pseudoexfoliation syndrome there was a significant (p less than 0.001) reduction in cell count to 1812 +/- 297 cells/mm2. In 59 eyes with PES and manifest glaucoma there was a significant (p less than 0.001) reduction of the corneal endothelium to 1482 +/- 267 cells/mm2. The cell picture was composed of enlarged, regularly arranged cells. In addition, such endothelial changes as incomplete cell lesions, cell detritus and appositions were six times as frequent as in normal eyes. In 32% of the eyes affected by PES we found manifest glaucoma. The statistical evaluation (matched age groups ect.) was carried out by means of the Mann-Withney-Wilcoxon rank test.