Germline mutations in the von Hippel-Lindau (VHL) gene in patients from Poland: disease presentation in patients with deletions of the entire VHL gene.
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Adolescent↗
Biomedical subjects
M Starzycka
Publications and source records attributed to M Starzycka.
[Analysis of 100 cases of retinal detachment treated with conventional surgery in the years 1997-1998].
PURPOSE: The observed increasing number of severe cases of retinal detachment was the ground of the attempt to state the causes of this phenomenon. MATERIAL AND METHODS: Retrospective evaluation of 100 consecutive cases of retinal detachment operated on with conventional methods between September 1997 and October 1998 was performed. The following data were analysed: sex and age of patients, period between the first symptoms and time of diagnosis and surgery. The clinical characteristics of retinal surgery comprise: extent of detachment, macular involving, type and number of breaks, degenerations and PVR. RESULTS: In the majority of cases very severe retinal detachments were observed, including 3-4 quadrants, with large tears or multiple breaks, with PVR B or C. Duration of the detachment ranged from several days in very few cases to several months, on average lasting several weeks. Attachment of the retina was achieved in 80 cases, improvement of the visual acuity in 47. CONCLUSION: Our data indicate the necessity to improve the early diagnosis of retinal detachment and changes predisposing to its development.
Adolescent↗
[Strabismus and binocular vision disorders in premature infants with retinopathy treated with cryotherapy].
PURPOSE: To assess frequency of refraction errors, abnormal eye position and binocular vision disorders in children with retinopathy treated with cryotherapy. MATERIAL AND METHODS: Subjects of the study were 67 children, aged 2-7, treated with cryotherapy for active phase retinopathy. RESULTS: The largest group consisted of children with good visual acuity (48%). Refraction error was found in 94% of the examined eyes. Hypermetropia was more common (54%) than myopia (40%). Majority of the examined children had squint, and esotropia was much more frequent (52%). The deviation in 37% of children was over 10 degrees. In the group with birth weight under 1000 grams as well as in those with birth weight 1000-1500 grams the squint occurred in 48%, and in children weighing over 1500 grams in 4.0%. Only 54% of the studied children had normal binocular vision. CONCLUSIONS: Retinopathy of prematurity is a risk factor for development of refraction errors, strabismus and amblyopia. In infants with retinopathy of prematurity treated with cryotherapy it is necessary to have vision check-ups during their early childhood years.
Child↗
[Failures of brachytherapy in posterior uveal melanoma].
PURPOSE: To estimate the risk factors responsible for unsuccessful treatment of melanoma with episcleral plaque brachytherapy. MATERIAL AND METHODS: Based on 23 cases of choroidal melanoma, in which the eyes had to be enucleated after earlier brachytherapy, clinical characteristics of the tumors, type of plaque therapy and reasons of enucleation were analysed. Histopathologic examination of enucleated eyes was performed by standard techniques and immunohistochemical stains on formalin-fixed paraffinembedded tumor tissues. RESULTS: Tumor size is the most valuable risk factor for prognosis in plaque brachytherapy of melanoma. The direct indications for enucleation were: increase of tumor mass, optic disc invasion, consecutive glaucoma and extrascleral extension. Histopathological examination revealed 17 mixed-cell melanoma, 4 spindle-cell, 1 epithelioid and 1 necrotic. Post-irradiation necrosis was found in only 6 tumors. CONCLUSIONS: It is very difficult to predict the results of conservative treatment of melanomas basing on their clinical characteristics. The aggressive nature of the tumors and their resistance to irradiation seem to be the likely explanation of failures.
Adult↗
[Trans-scleral resection of the ciliary body and choroidal melanoma].
PURPOSE: To present our preliminary results of trans-scleral resection of ciliary body and choroidal melanoma. MATERIAL AND METHODS: 16 patients aged 20-73 years (mean 56 years) were treated from February 1999 to March 2000. There were 10 patients with ciliary body tumors, 2 with choroidal tumors and in 4 patients the tumor involved ciliary body and choroid. The mean basal diameter of tumors was 9.4 mm and the mean thickness was 7.2 mm. Trans-scleral resection was performed in all cases. Adjunctive radiotherapy using 106Ru plaques was applied in 7 cases. RESULTS: Histopathologically the diagnosis of melanoma was confirmed in all cases. In 10 cases there were spindle cell melanomas and in 6 mixed type. The intra-operative complications were vitreous hemorrhage in 3 patients, vitreous loss in three and both complications in one patient. Post-operative complications occurred in 3 cases of single instances each of retinal detachment, retinal edema and haemophthalmus. After surgery visual acuity did not change in 9 eyes, improved in 2 and deteriorated in 5 eyes. CONCLUSIONS: Our preliminary results confirm the opinion that trans-scleral resection could be a valuable method in the treatment of intraocular melanoma.
Adult↗
[Transpupillary thermotherapy combined with 106Ru as a method of managing choroidal melanoma].
BACKGROUND: A study was carried out to evaluate the effectiveness of transpupillary thermotherapy (TTT) with diode laser applied as adjuvant treatment to 106Ru brachytherapy in choroidal melanoma. These two methods coined as "sandwich therapy" are complementary and allow to decrease the dose of radiation. MATERIAL AND METHODS: Between November 1996 and December 1997, 45 patients with choroidal melanoma were treated simultaneously with 106Ru and TTT. There were 18 males and 27 females, aged 20 to 78 (mean 57). There were 7 tumors with height of 5 mm or less, 28 ranging 5.43-8.63 mm and 10 with height 9 mm or more. The anterior tumor margin was located in the equator area or posterior to it in 71.1% of cases. The laser treatment was delivered using spots 2-3 mm in size, at a mean power 600 mW, for about 1 minute. The mean radiation dose was 60 Gy to the tumor apex. The follow-up ranged from 12 to 24 months. RESULTS: Reduction in tumor height by at least 30% was exhibited in 35 (77.8%) tumors, in one evaluated as positive result it was 27%. There was no improvement after treatment in 4 cases, 2 eyes were enucleated. A significant relationship was found between tumor thickness and the result of treatment. CONCLUSION: Our preliminary observations suggest that TTT combined 106Ru brachytherapy is an effective method of treatment. A longer follow-up is needed to assess final local results and the impact of treatment on survival.
Adult↗
[Diagnostic problems in intraocular melanoma].
PURPOSE: To evaluate accuracy of the diagnosis, rate of advancement and therapeutic possibilities of intraocular malignant melanomas, basing on our one-year clinical material. MATERIAL AND METHODS: The total of 288 consecutive patients with suspicion or diagnosis of intraocular malignant melanoma, referred in the year 1997 to the Department of Ophthalmology CM UJ, were analysed. There were 121 males and 167 females, aged 22-76, mean 50. The period of time between the patient's first ocular symptoms and his visit to the ophthalmologist ranged between 7 days and several years. Besides routine ophthalmological examination each patient underwent USG and, if necessary AF, ICG, CT and IMR. RESULTS: Diagnosis of malignant melanoma was confirmed in 141 cases. Among these patients in 69 brachytherapy was applied, in 20 surgical resection of the tumor and in 52 enucleation. In 111 cases other following diseases were diagnosed: iris cysts, deficient iridectomy, iris nevus, choroidal nevus, metastatic tumors, age-related macular degeneration, myelinated nerve fibres, Leber-Coats syndrome, toxoplasmosis and granulomatous uveitis. In 36 cases the diagnosis was not exact and those patients remained under periodic clinical observation. CONCLUSIONS: Our study shows that in many patients intraocular malignant melanoma is diagnosed too late and in these cases enucleation is the only method of treatment. The most diagnostic challenges are connected with differentiation of choroidal melanoma from choroidal nevus and exudative form of age-related macular degeneration.
Adult↗
[Brachytherapy in choroidal melanoma].
BACKGROUND AND PURPOSE: Brachyterapy in the management of intraocular tumors was introduced in the Department of Ophthalmology in Cracow in the late sixties, soon after it was applied by Stallard. For many years, similar to other oncological centers, we used 60Co and in 1994 we started with 106Ru and at the end of 1997 with 125J. The aim of this paper is to present our experiences with 106Ru and 125J brachytheray in posterior uveal melanoma. MATERIAL AND METHODS: Our studies comprised 164 patients with choroidal melanoma, treated in the years 1995-1997 with 106Ru and 19 patients treated between December 1997 nad April 1998 with 125J plaque brachytherapy. There were 93 women and 90 men, aged 18-86. Tumor thickness was beneath 6 mm in 85 cases, 6-9 mm in 63 and above 9 mm in 35. In 121 eyes anterior margin of the tumor was located in equatorial region or posterior to it, 62 the tumor situated anterior to the equator attached to the ciliary body. The usual dosage was 60-100 Gy to the tumor apex; in 33 eyes transpupillary thermotherapy (TTT) with diode laser was added. The follow-up ranges from 3 months to 3 years after 106Ru plaque brachytherapy and 1-3 months after 125J. RESULTS: Criteria of the treatment efficacy were decrease of tumor thickness at least 10%, increase of its density and vessel obliterations. Among 164 patients treated with 106Ru improvement was achieved in 84 (51.2%) of cases, stabilization in 50 (30.5%) and negative results in 30 (18.3%). Enucleation was performed in 16 (9.7%) cases. Among 19 patients treated with 125J preliminary evaluation indicates positive reaction for treatment in 14 cases. CONCLUSIONS: Our studies confirm the opinion that brachytherapy is a method of choice in the management of many cases of posterior uveal melanoma. There are significant relationships between the results of treatment and the size of tumor and its location. The adequate choice of the kind of radioactive isotope in the plaque is very important.
Adolescent↗
[Risk factors for age-related macular degeneration].
PURPOSE: To present the results of examinations of the risk factors for age-related macular degeneration (AMD) carried out in the last 3 years. MATERIAL AND METHODS: Our clinical material comprised 119 patients, 69 women and 50 men, aged 43-85, mean age 70 years. Using classification according to the worse eye, there were 20 patients with drusen, 27 with atrophic changes and 72 with exudative form and AMD. The following risk factors were evaluated: age, sex, body mass index, history of general medical conditions, cigarette smoking, sun exposure, family history of AMD and ocular conditions such as iris color, lens opacities, hyperopia, gerontoxon and changes in retinal vessels. RESULTS: The significant relationships were found between the development of AMD and the age of patients, as well as between the advanced forms of AMD and the history of cardiovascular diseases and sclerotic changes in retinal vessels, 87% of examined patients have light iris and 52% body mass index above 26. CONCLUSIONS: The studies confirmed the role of age in the development of AMD and indicated cardiovascular disturbances, increased body mass index and light iris as the possible risk factors for AMD that are most worth further studying. The special attention should be also paid to drusen as the risk factor of AMD.
Adult↗
[Age related macular degeneration as a cause of blindness].
PURPOSE: To present our studies concerning the causes of blindness in age-related macular degeneration, including the relationship with risk factors for this disease. MATERIAL AND METHODS: A total number of 158 eyes with AMD in 99 patients were examined. Among them there were 21 cases with visual acuity 0.1 or less in the better eye. This group of patients underwent a detailed analysis in order to determine the factors responsible for visual impairment in comparison to the patients with better visual function. RESULTS AND CONCLUSIONS: Blindness is connected mostly with exudative AMD. There are no significant differences in prevalence of risk factors for AMD between the group of patients with visual loss and that of patients with visual acuity above 0.1. The majority of patients referred to the ophthalmologist too late and on account of that is should be necessary to perform prophylactic examinations of the people above sixty.
Aged↗
[Epidemiology and risk factors for age related macular degeneration].
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Age Factors↗
[Use of cryotherapy in retinopathy of prematurity].
PURPOSE: To present our experiences in cryotherapy for ROP. MATERIAL AND METHODS: From October 1991 to August 1995, transscleral cryotherapy was applied in 128 eyes of 70 babies with ROP. There were 34 girls and 36 boys with birth weight between 650 g and 1990 g and gestational age from 24 to 36 weeks. In above 90% of cases, ROP reached zone II and stage 3 with "plus" disease. In 84% of the eyes the extent of ROP was greater than 5 clock hours. Prethreshold severity was diagnosed in 7 and threshold in 110 eyes. In 11 eyes these categories could not be seventy determined. The chronologic age of infants at cryotherapy ranged from 8 to 22 weeks, mean 12 weeks. In all cases, cryotherapy was carried out under general anesthesia using a technique described in CRYO-ROP study. No serious complications during or after cryotherapy were observed. RESULTS: Favorable structural outcome was found in 119 eyes of 70 treated infants. In 82 eyes of 46 infants, with at least 12-month follow-up examination, also functional outcome was evaluated, basing on the examination with the Teller Acuity Card Procedure; the results were favorable in 57% of the eyes. Structural and functional outcomes were in agreement in 47 of the 82 eyes and discordant in 33. CONCLUSIONS: Our experience confirms the benefit of cryotherapy in the treatment of active ROP. In most cases cryotherapy should be applied in threshold ROP but in some cases especially in those with very rapid progression it should be done earlier. Because of the unpredictability of the natural course of ROP it is essential to use the scheme of ophthalmological examinations proposed by CRYO-ROP Study.
Cryosurgery↗
[Immunologic tests in patients with idiopathic uveitis--preliminary reports].
AIM: This study aimed to evaluate the immune system function in patients with uveitis of unknown aetiology. METHODS: The clinical material comprised 19 patients with endogenous uveitis. In all cases the following immunological tests were performed: serum immunoglobulins A, G, M, total IgE, circulating immune complexes, complement components C3c and C4-all determined by laser nephelometry; antinuclear antibodies assessed with indirect immunofluorescence method using HEp-2 cell lines; and antineutrophil cytoplasmic antibodies using indirect immunofluorescence test. In 4 cases lupus anticoagulant was measured with APTT and dRVVT assays. RESULTS: Among our 19 examined patients immunological abnormalities were found in 12 cases. Changes in immunoglobulin concentrations were found in 8 cases. In 4 patients abnormalities of the complement system were observed. Antinuclear antibodies with speckled pattern in indirect immunofluorescence were present in 7 cases. CONCLUSION: In a proportion of patients with endogenous uveitis mild immunological abnormalities were present, suggesting an autoimmune background of the disease. Studies of the immunological profile can therefore help in better evaluation of the patients. It remains to be determined whether the observed immunological alterations are of any importance in the pathogenesis of the studied disease.
Adolescent↗
[Conventional methods in retinal detachment surgery].
PURPOSE: To evaluate the usefulness of conventional methods in retinal detachment (RD) surgery basing on the relationship between factors characterizing clinical picture of the RD, the type and course of surgical procedures and the retinal reattachment. MATERIALS AND METHODS: 252 patients (252 eyes), operated on in the last 5 years in our clinic were enrolled in the studies. There were 120 men and 132 women, aged 9 to 83, mean 53. In all cases scleral buckling procedures with silicone band or sponge and cryocoagulation of the breaks were used. The relationship between retinal reattachment and the following parameters were examined: visual acuity, retinal degenerations, myopia, extent of the detachment, number and kinds of breaks, macular involvement, state of vitreous, PVR, intraocular pressure, extent of scleral buckling, subretinal fluid drainage and intra- or post-operative complications. RESULTS: Retinal reattachment was achieved in 82% of the eyes with one operation and additionally in 7% after reoperation. The significant relationship was found between: visual acuity, intraocular pressure, extent of the detachment, number of breaks, PVR, extent of scleral buckling and retinal reattachment. CONCLUSION: PVR is a significant cause of failure in RD surgery with conventional methods and PVR grade C is the threshold beyond which the percentage of reattachments decreases to about 50%. Value of other risk factors, determining severity of RD is not certain for prognosis. Cerclage with silicone band is a method of choice in the majority of RD with PVR grade B and C. Our observations do not confirm the reports of severe complications caused by cerclage, which might be connected with excessive tightening of the band.
Adolescent↗
[Secondary retinal detachment].
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Humans↗
[Late results of surgical treatment of retinal detachment by the cerclage method using a silicone band].
Presented are the results of control examination performed in 121 patients in the period of 3 to 8 years after retinal detachment operation by the silicone band encircling method. Evaluated were: the anatomical reattachment, function of the eye and the cosmetic effect. The retina reattached in 63.2 p.c. of eyes. The visual acuity for far was at least 0.1 in over one half (55 p.c.) of eyes and for near 2.0 or more in 78.5 p.c. Among the persons examined by means of Amsler's test and Panel D-15 test a correct response was attained by -- respectively -- 44.7 p.c. and 76.1 p.c. of the examinees. A very good cosmetic effect was achieved in 44.9 p.c. and a good one in 47.2 p.c. of eyes.
Adolescent↗
[Reoperations after the treatment of retinal detachment].
Presented is an analysis of the clinical material comprising 100 patients in whom in the period of 1980-1989 one performed reoperations of retinal detachment. It was established that the most frequent causes--as well surgical failures as recurrences--were the vitreo-retinal proliferations and the insufficient closing of the holes. Reattachment of the retina after the second operation was attained in 42 p.c. of the eyes and after the third one in additionally 7 p.c. In cases with a reattached retina a very good visual acuity for far was found in 1/4 and for near in 1/2 of operated eyes. The results obtained are encouraging enough for the performance of reoperations.
Adolescent↗
[Value of studying the intraocular pressure in patients operated on for retinal detachment].
The IOP was checked in 263 eyes operated for retinal detachment--before, during and after surgery. Statistically lower values of the IOP were observed in eyes with retinal detachment in comparison with the healthy fellow-eye and in cases with an extensive and prominent detachment. There was no interdependence between the values of the IOP--even showing considerable oscillations--and the occurrence of complications, the normalization of the postoperative IOP and the results of the treatment. The measurements of the IOP in the course of retinal detachment operation may have a significance in determining the indications for a drainage of the subretinal fluid and in evaluation of the degree of the retinal invagination.
Adolescent↗