[Patient with breast cancer and bilateral serous retinal detachment].
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Biomedical subjects
Publications and source records attributed to E Messmer.
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BACKGROUND: The purpose of this study was to measure the activity of the sympathetic nervous system in patients with central serous chorioretinopathy (CSC) by power spectral analysis (PSA) of heart-rate (RR-interval) variability, a noninvasive method that reflects the balance of the sympathetic-vagal interaction. METHODS: The following four different groups of patients were measured; group 1, acute CSC (n = 11); group 2, acute recurrent CSC (n = 7); group 3, chronic persistent CSC (n = 4); group 4, complete remission of CSC (n = 9). The data recorded for these 31 patients (29 men and 2 women), with an average age of 44 years were compared with those noted for a group of 15 age-matched healthy individuals. The sympathetic-vagal balance is expressed by the ratio of the low-frequency component (LF) to the high-frequency component (HF) of the power spectrum. RESULTS: Significant differences in mean LF/HF ratios were found as follows for all but one of the sub-groups as compared with the normal controls (LF/HF = 1.1): group 1, LF/HF = 5.5 (P < 0.01); group 2, LF/HF = 5.4 (P < 0.05); group 3, LF/HF = 4.2 (P = 0.1); and group 4, LF/HF = 3.0 (P < 0.01). There was also a significant difference between active CSC and inactive CSC (P < 0.05). CONCLUSIONS: These results support the view that the pathogenesis of CSC is related to an increase in the sympathetic activity of the autonomic nervous system. Furthermore, the LF/HF ratios seem to correlate with the activity of the disease.
PURPOSE: Intraoperative retinopexy currently is performed transsclerally during surgery for retinal detachment by means of cryocoagulation, which usually results in much damage to the tissue. In this study, we investigated the potency of transscleral chorioretinal laser photocoagulation in achieving retinopexy with less breakdown of the blood-retina barrier and resulting in decreased protein leakage and reduced dispersion of the retinal pigment epithelium. METHODS: A continuous wave (CW) Nd:YAG laser with a custom-made optical tip was used. The tip consists of an optical fiber protected by a steel casing for indentation of the sclera. Eight patients underwent the procedure. RESULTS: Three to six pulses of 5.2-6.6 W and exposure duration of 300-500 mesec per spot were used to obtain mild to moderate photocoagulation; no retinal complications were observed. An average of 45 coagulations were obtained per patient. One patient showed mild scleral thermal effects. All retinas were attached after surgery, and seven remained attached at the 6-month follow-up examination. CONCLUSION: Transscleral chorioretinal irradiation with the CW Nd:YAG laser is a suitable method for performing retinopexy. Because no thermal damage to the sclera occurred, this method seems to be a safe and effective procedure.
PURPOSE: To compare the relative toxicities of six systemic immunosuppressive drugs and systemic corticosteroids used to treat patients with severe ocular inflammatory disease and to identify factors influencing their occurrence. METHODS: The authors reviewed the clinical records of 602 patients with ocular inflammatory disease treated with immunosuppressive drug therapy and/or systemic corticosteroids for adverse systemic effects while undergoing therapy. Proportional hazards regression analysis was performed to identify demographic and clinical factors that influence the occurrence of drug toxicity in these patients. RESULTS: Immunosuppressive drug treatment was more likely to result in discontinuation of therapy because of toxic side effects than was corticosteroid treatment. However, unlike many of the side effects of corticosteroid treatment, the side effects of immunosuppressive therapy were reversible with reduction in dosage or discontinuation of the drug. Gastrointestinal symptoms and hematologic abnormalities accounted for the majority of reported side effects of the immunosuppressive medications. Neuro-psychiatric and endocrine side effects were common in patients taking prednisone. In 17 patients treated with prednisone, pathologic fractures developed, which involved the hips and the spine. Female sex and age older than 60 years also were identified as factors associated with intolerance to drug therapy in the authors' study population. Race and type of systemic ocular disease were not significant factors influencing tolerance to drug therapy. CONCLUSION: These findings suggest that when properly administered and monitored for adverse effects, most immunosuppressive agents used in the current study have similar risk profiles with relatively few serious therapeutic mishaps and largely reversible side effects. In contrast, corticosteroids can result in permanent disabilities as a result of long-term treatment.
PURPOSE: To compare the occurrence of malignancy in patients with severe ocular inflammatory disease treated with systemic corticosteroids alone or with systemic immunosuppressive drugs with or without systemic corticosteroids. METHODS: The clinical records of 543 patients with ocular inflammatory disease treated with systemic corticosteroids and/or immunosuppressive chemotherapy were reviewed in a retrospective cohort study. Characteristics of patients treated with corticosteroids alone were compared with characteristics of patients treated with immunosuppressive agents with or without preceding corticosteroid treatment. The rates of malignancy after initiation of drug therapy were compared using an exact test for incidence rate data. RESULTS: Compared with patients treated with corticosteroids alone, patients treated with immunosuppressants with or without corticosteroid treatment were older and had more severe systemic disease. During a total of 1261 person-years of follow-up, a malignancy developed in five patients. The rate of malignancy in the immunosuppressant group (4 malignancies during 968 person-years of follow-up) was not significantly different from the rate in the corticosteroids alone group (1 malignancy during 293 person-years of follow-up) (P > 0.90, exact test for incidence rate data). CONCLUSION: These findings do not support the hypothesis of an increased risk of malignancy in patients with severe ocular inflammatory disease who are treated with systemic immunosuppressive agents compared with patients treated with systemic corticosteroids.
A total of 11 patients exhibiting in 11 eyes a subfoveal or juxtafoveal choroidal neovascular membrane due to age-related macular degeneration (AMD) were treated with interferon alpha-2a (Roferon) according to a protocol that was designed for an independent, randomized, open-label pilot study to evaluate the efficacy and safety of this therapy. However, none of the patients agreed to be randomized and all elected to be treated. The average follow-up period after treatment was 8.2 months. In 6 of 7 eyes with classic choroidal neovascularization the membrane increased in size during and after treatment. Of these 6 eyes, 5 lost more than 6 lines of visual acuity. In 2 of 4 eyes with occult neovascularization the lesion enlarged. In the other 2 eyes the lesion became atrophic in 1 case and remained stable in size in the other. Visual acuity decreased by 3-9 lines in 3 eyes with occult neovascularization. Of the 11 patients, 2 discontinued interferon treatment after 8 weeks because of severe adverse reactions. From the data collected thus far in this study and in the majority of published series, we conclude that interferon alpha-2a in the dose range of 3-6 million IU given three times weekly over 8-12 weeks is neither effective nor safe for the treatment of subfoveal neovascularization in AMD.
BACKGROUND: Muir-Torre syndrome (MTS) is a hereditary genodermatosis associated with internal malignancies. METHODS: We report the histological case of an atypical sebaceous gland adenoma of the palpebral conjunctiva in a 42-year-old female patient. RESULTS: The combination of this adenoma with atypical adenomatous hyperplasia of the endometrium and a strong family history of gastrointestinal and urogenital cancers led to the diagnosis of MTS. CONCLUSION: We present a case where the diagnosis of an atypical solitary sebaceous gland adenoma of the palpebral conjunctiva combined with a conspicuous personal and family history led to the diagnosis of MTS.
Laser treatment of subfoveal neovascular membranes secondary to age-related macular degeneration (AMD) has been proven to be beneficial with regard to long-term contrast sensitivity and central scotoma size, but significant visual loss occurs immediately after treatment and it is therefore not generally accepted. Alternative treatment modalities should be evaluated. The observation made by Dr. W. Fung that patients with subretinal neovascular membranes may benefit from systemic treatment with the angiogenesis inhibitor interferon alpha-2a has stimulated much interest in this new treatment modality. We present four patients suffering from a subfoveal neovascular membrane who have been treated with this drug. In three cases the membrane was of an occult type, in one case there was a combination of classic and occult choroidal neovascularization. In all patients there had been progressive visual loss during the last 6 months prior to treatment. Visual acuity stabilized in three cases with occult neovascularization. In one case with classic choroidal neovascularization the membrane increased in size during treatment and a further drop of visual acuity was noted. Despite the fact that serious side effects could not be observed in our patients, the results do not allow to recommend Interferon for widespread use in the treatment of subretinal neovascular membranes, especially because the number of patients was small and the follow up time short. Its efficacy needs to be demonstrated by randomized clinical trials.
Fifty-six globes that had to be enucleated following ruthenium plaque therapy were examined histopathologically. These eyes account for 10% of all uveal melanomas treated at the University Eye Clinic Essen up until 1985. All but one revealed at least some supposedly viable tumor cells. The most prominent findings within the tumors were tumor cell necrosis, vacuolization and balloon cell degeneration, vascular obstruction and fibrosis of the tumor stroma with accumulation of pigmented macrophages. Tumor necrosis was complete or nearly complete in five cases. Tumor regression correlated with cell type and pigmentary characteristics of the tumor, with epithelioid and heavily pigmented tumor cells being more radiosensitive. Tumor regression was inhomogeneous, possibly due to polyclonality, with tumor cells of varying radiosensitivity, or due to patchy areas of vascular obliteration. Among other ocular structures, extensive subretinal gliosis, chorioretinal atrophy and scarring of the sclera within the field of radiation were observed. Scleral necrosis was present in only five cases and was limited to areas in which the tumor had infiltrated the deep scleral layers. The findings described were considered to reflect radiation injury rather than spontaneous tumor regression when compared to 70 control eyes that had been enucleated without prior treatment for uveal melanoma.
Recurrent epiretinal membranes are a major contributing factor to functional and visual failures after pars plana vitrectomy for proliferative vitreoretinal disorders. In this study 222 consecutive eyes that have had pars plana vitrectomy for proliferative diabetic retinopathy (operated in 1986 und 1987) were evaluated retrospectively. The study focuses on a subgroup of 108 consecutive eyes all with preoperative tractional retinal detachment and all operated with primary silicone oil injection. A minimum follow up of six months was present in 90% of these cases. Permanent visual success was observed in 40% of the eyes, most of which had dense vitreous hemorrhage preoperatively. Multivariant analysis revealed that among several potential risk factors recurrent epiretinal membranes as well as silicone oil were significantly associated with visual failure. Because of the high incidence of recurrent epiretinal membranes associated with poor functional results silicone oil injection following pars plana vitrectomy should be employed restrictively in patients with proliferative diabetic retinopathy.
DNA samples from 92 unrelated patients with bilateral retinoblastoma were analyzed by Southern blot hybridization with cDNA and genomic clones of the retinoblastoma (RB-1) gene. Qualitative and quantitative evaluation of the Southern blot patterns showed a deletion of all or part of the RB-1 gene in 15 patients. Deletion hot spots were not detected. The study shows that 16% of germ cell mutations are detectable by Southern blot hybridization, but that densitometric analysis is required in most cases.
Epigenetic models for tumor formation assume that oncogenic transformation results from changes in the activity of otherwise normal genes. Since gene activity can be inhibited by DNA methylation, and inactivation of tumor suppressor genes is a fundamental process in oncogenesis, we investigated the methylation status of the retinoblastoma suppressor gene (RB gene) on chromosome 13, in blood and tumor cells from 21 retinoblastoma patients. Using methylation-sensitive restriction enzymes and a cloned DNA probe for the unmethylated CpG island at the 5' end of RB gene, we obtained evidence of hypermethylation of this gene in a sporadic unilateral retinoblastoma tumor. The closely linked esterase D gene and a CpG-rich island on chromosome 15 were not affected. We suggest that changes in the methylation pattern of the RB gene play a role in the development and spontaneous regression of some retinoblastoma tumors.
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Transscleral coagulation of the ciliary body was performed by means of the continuous wave Neodymium: YAG laser in 39 eyes with various types of secondary glaucoma that was not controlled by medical therapy. Three days later, the mean intraocular pressure measured 54% of the pre-laser pressure; 3 months later it was 52%, 6 months later 47%, and 12 months later 58%. In 11 of 14 eyes (78.6%) treated in an attempt to preserve the remaining visual function, the pressure proved to be under control (less than or equal to 20 mmHg) 6 months after treatment. Major complications, such as hyphema or bleeding into the vitreous, were rare (7/39) and occurred only in the eyes with neovascular glaucoma.
Six families with retinoblastoma in more than one member were investigated with DNA markers linked to the retinoblastoma locus because direct analysis had not disclosed the gene defect. In all of the families we could identify the affected chromosome and predict the genetic risk with a high level of confidence (90 to 99%). In one patient the test helped to detect tumour development earlier than usually possible. Several subjects were found not to carry a mutation, thus obviating frequent ophthalmological examinations under anaesthesia as would be necessary otherwise. These results show that linkage analysis can be successfully applied to genetic counselling in families with hereditary retinoblastoma.
A 33-year-old woman underwent bone-marrow transplantation following radiation and chemotherapy for chronic myelocytic leukemia (CML); immunosuppressive therapy was continued for graft-versus-host disease. Five months after successful transplantation, she developed necrotizing retinitis in both eyes with rapid progression over the following weeks. Due to her immunosuppressed state the patient developed pneumonia and died. Postmortem evaluation of the retinal lesions in both eyes disclosed infection by Toxoplasma gondii, which was also found in the brain and myocardium. Multiple viable toxoplasmic cysts were observed at the transition zone from a necrotic to a normal retina. Additionally, cysts of Toxoplasma gondii a normal retina. Additionally, cysts of Toxoplasma gondii were seen in the adjacent intact retina and in areas of necrosis with almost complete absence of retinal or choroidal inflammation. Toxoplasmosis should therefore be considered along with fungi and viruses in the differential diagnosis of necrotizing retinochoroiditis in immunocompromised patients.
A case of bilateral complete cryptophthalmos combined with other malformations is described. The clinical appearance, histology, and the results of computed tomography and electro-ophthalmological investigations are communicated.