Biomedical subjects

C Cursiefen

Publications and source records attributed to C Cursiefen.

At least 37 records · Page 2Linked to original sources

PubMed2001

Orbital involvement in cherubism.

PURPOSE: To demonstrate the clinical, radiologic, and histopathologic features of a patient with orbital involvement in cherubism that prompted surgical treatment. DESIGN: Single interventional case report. INTERVENTION: Findings of the ophthalmic evaluation, computed tomography (CT) scans, intraoperative examination, and light microscopy of the specimens were analyzed. MAIN OUTCOME MEASURES: Globe displacement, orbital bony lesions detected on CT scans, histopathology, and postoperative results were assessed. RESULTS: A 27-year-old female was seen with a slowly progressive superonasal globe displacement and a temporal orbital mass bilaterally of 6 years' duration. She had a history of cherubism, but her cheeks and jaws had a normal appearance instead of the bilateral fullness of the lower half of the face typical of the disease. CT scans demonstrated multicystic bony lesions arising from the orbital floors bilaterally. The masses were excised using an anterior transcutaneous transseptal orbitotomy. Histopathology demonstrated numerous giant cells in a fibrovascular stroma, confirming the clinical diagnosis of cherubism. Postoperative recovery was complete. CONCLUSIONS: Orbital involvement in cherubism may develop beyond puberty, after stabilization or regression of the lesions in the jaws. Patients with cherubism should be routinely evaluated by an ophthalmologist.

Adult↗

PubMed2001

The negative response of the flash electroretinogram in glaucoma.

The existence of a negative ERG component following the b-wave has been known for a long time. Recently, in unilateral macaque experimental glaucoma, a negative response in flash electroretinograms under scotopic as well as photopic conditions has been shown to be greatly reduced or absent compared to the healthy fellow eye. The aim of this pilot study was to test whether a late negative electroretinogram component is reduced also in human glaucoma patients under different stimulus conditions. Dark-adapted ganzfeld flash electroretinograms were recorded after 30 min of dark using two test conditions, obtained as optimal in pilot studies on controls. Under the scotopic condition I white Xenon-flashes of intensity 0.53 Log photopic Td s were presented on a low white background of 1.38 Log scotopic Td. Under the more photopic condition II orange flashes of intensity -0.37 Log photopic Td s were presented on a blue-adapting background of 2.5 Log scotopic Td. Nine controls and 18 patients with advanced glaucoma were analyzed. The amplitude of the negative response was not significantly reduced in glaucoma patients (condition I: -28.5+/-23.7 microV; condition II: -25.2+/-19.7 microV) compared to controls (condition I: -41.4+/-36.6 microV; condition II: -31.3+/-26.2 microV). The peak latency of the responses under condition I and II did not differ significantly between patients and controls. Thus, the late negative electroretinogram component in ganzfeld flash electroretinograms obtained under scotopic and more photopic conditions does not seem to distinguish as easy between human controls and glaucoma patients as animal experiments suggest.

Dark Adaptation↗

PubMed2001

Pseudoexfoliation syndrome in eyes with ischemic central retinal vein occlusion. A histopathologic and electron microscopic study.

PURPOSE: To determine histopathologically the prevalence of pseudoexfoliation (PEX) material in eyes enucleated secondary to ischemic central retinal vein occlusion (CRVO) and to evaluate eyes with PEX material in the anterior segment and CRVO ultrastructurally for PEX deposits in the vicinity of central retinal vessels. These deposits could explain an association of CRVO and PEX. METHODS: All surgically enucleated eyes with secondary angle closure glaucoma due to rubeotic iris secondary to ischemic CRVO (1981-1998) available were re-analyzed light microscopically for the presence of PEX in the anterior segment (n=120; 76.9+/-8.5 years [range: 51-91]). Eyes with PEX in the anterior segment and available optic nerve cross sections were examined by electron microscopy for PEX material in the retrolaminar (n=7) and intralaminar central retinal vessels (n=3). All eyes surgically enucleated because of malignant melanoma of the choroid aged 70 years and older (1981-1998) with sections available served as age-matched controls (n=107; 76.4+/-5 years [range: 70-91]). RESULTS: PEX material was present light microscopically in the anterior segment in 12 of 120 eyes with CRVO (10%) compared to 2 of 107 age-matched eyes with choroidal malignant melanoma (1.9%; p<0.05). Electron microscopically, neither structural alterations of the vessel wall nor PEX deposits were found in association with central retinal vessels both in the intra- and retrolaminar areas in any of the 7 eyes with PEX in the anterior segment and CRVO analyzed. CONCLUSIONS: Histopathologically, PEX is significantly more common in eyes enucleated secondary to CRVO compared to eyes enucleated because of an intraocular tumor. This most likely is due to the secondary open angle glaucoma in eyes with PEX as a known risk factor for CRVO. According to the small number of optic nerves analyzed here, there seems to be no morphologically evident PEX vasculopathy in the central retinal vessels both within and immediately behind the lamina cribrosa in eyes with PEX in the anterior segment and CRVO potentially causing retinal venous thrombosis.

Aged↗

PubMed2001

[Transitory intrathoracic and -abdominal pressure elevation in the history of 64 patients with normal pressure glaucoma].

BACKGROUND: As one pathogenetic factor in normal-tension glaucoma an individually elevated sensitivity of lamina cribrosa regarding intraocular pressure fluctuations is postulated. Aim of this study was to evaluate patients with normal-tension glaucoma for the exposure to potential, clinically undetected transient elevations of intraocular tension due to increased intrathoracic and -abdominal pressure. PATIENTS AND METHODS: A survey of 64 patients of the "Erlanger Glaucoma registry" with normal-tension glaucoma (NTG) and 64 patients with primary open angle glaucoma (pOAG) as control group were performed with regard to activities respectively diseases causing intrathoracic or -abdominal pressure elevation (1. weight lifting, 2. playing high resistance wind instruments, 3. chronic asthma/cough, 4. obstruction of the urinary system, 5. constipation). Both groups were matched regarding age (median: 61 years), sex (24 male, 40 female), visual field defects (mean defect: NTG 4.4; pOAG 4.7), visual acuity (median 1.0 +/- 0.2) and systemic diseases (diabetes mellitus, hypertension, cardiac disease). RESULTS: Among patients with NTG there were 45% (29/64 patients) with activities respectively diseases causing intrathoracic or intraabdominal pressure elevation in their medical history compared to 11% (7/64) among patients with pOAG. Male patients with normal-tension glaucoma showed with 62% the highest frequency of such activities (among them 4/24 high resistance wind instrument playing, 5/24 urinary system obstructions, 4/24 long time weight lifting). Female patients with normal-tension glaucoma most frequently presented with a history of weight lifting (11/40). CONCLUSION: Patients with glaucomatous optic nerve atrophy without evident intraocular pressure elevation compared to patients with pOWG more frequently report activities or diseases causing intrathoracic/-abdominal pressure elevation in their medical history. This may suggest an additional pathomechanism in normal-tension glaucoma. Therefore patients may be adviced on these potential risk factors.

Abdomen↗

PubMed2001

[Standardized semiquantitative analysis of corneal neovascularization using projected corneal photographs--pilot study after perforating corneal keratoplasty before immune reaction].

BACKGROUND: A semiquantitative scheme for analysis of corneal neovascularization using projected corneal photographs is demonstrated and tested in a pilot study to analyze occurrence of corneal neovascularization in patients after perforating keratoplasty which subsequently developed transplant rejection. METHODS: Corneal photographs on the slit lamp with diffuse frontal illumination were obtained in a standardized technique. Slides were projected with 100 x magnification and analyzed twice with a 2 months interval. Corneal vessels were graded by two independent observers in each of 12 corneal sectors in a standardized fashion (grade 0: no vessels beyond limbus, 1: vessels between limbus and outer end of a double-running diagonal suture; 2: vessels between outer suture end and graft-host junction; 3: vessels reaching graft-host junction; 4: vessels within donor cornea). All patients with endothelial graft rejection of the prospective Erlangen non-high-risk keratoplasty study were included in a pilot study (1/1997-6/2000: 13 of 325; 4%). One patient without photographs available was excluded. Corneal photographs taken prior to surgery (n = 10), at the last 3 monthly-routine control before (10), at rejection episode (12) and one year later (10) were evaluated for corneal neovascularization. RESULTS: Interobserver correlation at the two assessments was 0.79 and 0.86 (Kendall's Tau B). Correlation between the assessments at the two analyses 2 months apart was 0.8. New vessels with diameter up to 6 microns can be detected. 8 of 12 analyzed patients (67%) with immune reaction after keratoplasty developed corneal neovascularization within 1 year after operation prior to transplant rejection in at least one corneal sector (2.1 +/- 1.9 sectors; 1-6). At time of rejection, new vessels reached the graft-host junction in 2 patients, in 1 patient vessels grew into the donor cornea, whereas in 8 the vessels were seen beyond the outer suture end without reaching host-graft junction (grade I: 1 patient). New vessels usually pointed to the outer suture ends of the double-running suture. CONCLUSIONS: Development of corneal neovascularization e.g. after keratoplasty can be assessed reliably using projected slides of corneal photographs at 100 x magnification. This method has the advantage of being more objective, precise and available compared to simple evaluation at the slit lamp. Postkeratoplasty corneal neovascularization seems to be common in non-high-risk eyes later developing transplant rejection. However, new vessels usually do not reach the host-graft junction. Whether neovascularization after keratoplasty demonstrates a risk factor for subsequent transplant rejection remains to be analyzed in a greater study.

Cornea↗

PubMed2001

[Visual rehabilitation and intraocular pressure elevation due to immunological graft rejection following penetrating keratoplasty].

PURPOSE: Endothelial graft rejection and intraocular pressure elevation are the most common causes of graft failure following penetrating keratoplasty (PK). Aim of this study was to evaluate the visual rehabilitation and the development of intraocular pressure during and after graft rejection. PATIENTS AND METHODS: The study included 20 eyes of 20 patients (age 54.7 +/- 19.8 years) with endothelial graft rejection, that fulfilled the following inclusion criteria 1) graft rejection was diagnosed and treated in our department; 2) at least one year follow-up after graft rejection; 3) avascular corneal pathology. The mean follow-up was 23 +/- 14 months. According to the type of surgical procedure patients were classified in PK only (n = 15, one after cataract extraction), PK combined with extracapsular cataract extraction and intraocular lens (IOL) implantation (n = 1); PK combined with secondary IOL-implantation or IOL-exchange (n = 4). Standardized complete ophthalmological examinations were performed on a regular basis before, during the acute graft rejection und then regularly in a defined examination raster in an out-patient service with cornea specialization. RESULTS: The time interval between first symptom of 18 acute diffuse and 2 chronic focal graft rejection and start of treatment was 9 +/- 13 days. Best-corrected visual acuity (CVA) was 0.6 +/- 0.2 before graft rejection and decreased significantly at the time of diagnosis (0.2 +/- 0.2; p = 0.001). Six weeks after graft rejection CVA was 0.5 +/- 0.2 and remained almost stable until one year after rejection (0.6 +/- 0.3) in 16 patients with reversible graft rejection. Only 4 patients (20%) showed an irreversible graft failure requiring Re-PK. Intraocular pressure (IOP) was not elevated in 75% of the patients (n = 15) and did not need any antiglaucomatous treatment during and after the rejection phase. In 5 eyes (25%) (3 after PK combined with anterior chamber IOL-explantation and secondary posterior chamber IOL-implantation; 1 with secondary pseudoexfoliation glaucoma and 1 steroidal responder) IOP was elevated during graft rejection (26 +/- 7 mmHg), but was controlled by intensive topical antiglaucomatous treatment. CONCLUSION: Typically, the visual rehabilitation after graft rejection was good if the clinical signs were diagnosed just in time and treated adequately. There is no direct correlation between graft rejection and intraocular pressure elevation. However, the development of intraocular pressure elevation seems to be strongly associated with preexisting glaucoma, preexisting anterior synechiae and/or simultaneous anterior chamber lens implant removal. A careful patient management after PK plays an important role to prevent the development of irreversible graft failure due to graft rejection.

Acute Disease↗

PubMed2001

[Immunological graft reactions after penetrating keratoplasty - A prospective randomized trial comparing corneal excimer laser and motor trephination].

BACKGROUND AND PURPOSE: Nonmechanical trephination has been established as an advantageous procedure in penetrating keratoplasty (PK) for avascular corneal diseases and has been performed successfully in more than 1000 eyes at our institution. The purpose of this study was to compare incidence, type and reversibility of immunological graft reactions after mechanical and nonmechanical trephination for PK. PATIENTS AND METHODS: As part of a prospective randomized clinical trial 179 eyes of 76 females and 103 males were included, that had primary central PK without previous intraocular surgery between 10/1992 and 12/1997 for Fuchs' dystrophy (diameter 7.5 mm, n=73) or keratoconus (8.0 mm, n=106). The recipient and donor trephinations were performed from the epithelial side using either a motor trephine (Microkeratron, Geuder, n=91) or an 193-nm excimer laser (MEL60, Aesculap-Meditec, 1.5 x 1.5 mm spot mode, 16 - 24 mJ/pulse, repetition rate 25/s) along round metal masks with 8 "orientation teeth/notches" (n=88). As a routine, donor oversize was 0.1 mm. 128 patients (72 %) had PK only, 51 patients had simultaneous cataract extraction and PC-IOL implantation (triple procedure). Wound closure was achieved using a 16-bite double running diagonal suture by one surgeon (G.O.H.N.). In 53 % of procedures short-term-preserved donor tissue, in 47 % organ-cultured tissue was used. The mean patient/donor age was 51 +/- 17/57 +/- 19 years with nonmechanical trephination and 50 +/- 19/58 +/- 20 years with mechanical trephination. Incidence, type and reversibility of endothelial immunologic graft reactions were recorded continuously by clinical follow-up in an outpatient service with cornea specialization (after 6 weeks, then every 3 months until after suture removal, then every half year). RESULTS: During a mean follow-up of 40 +/- 15 months, 7.3 % of eyes developed an immunological endothelial graft reaction. Acute diffuse (5.6 %) were more frequent than chronic focal reactions (1.7 %). Only 1.7 % of grafts became irreversibly cloudy. More than 80 % of all graft reactions occurred later than one year (on average 23 +/- 13 months) after PK. Neither incidence (p=0.82, Chi square test) nor cumulative 3-year-rate of immunological graft reactions (p=0.91, LogRank test) differed significantly comparing nonmechanical (6.8 % or 7.3 %) and mechanical (7.7 % or 5.6 %) trephination. In eyes with keratoconus (6.6 %, 16 +/- 8 months) graft reactions did not occur more frequently (p=0.68) but earlier (p=0.02) than in eyes with Fuchs' dystrophy (8.2 %, 31 +/- 11 months). Donor age, post-mortem-time and preservation period did not significantly affect the rate of graft reactions in our study setting. CONCLUSIONS: Besides well-established optical advantages, nonmechanical trephination using the excimer laser seems to have no immunologic drawbacks in contrast to conventional mechanical trephination. Donor parameters appear to have no major impact on the incidence of graft reactions after non-high-risk PK. Rather, an adequate postoperative therapy with topical steroids, an informed patients' compliance, a consequent long-term follow-up in a clinical out-patient service with corneal specialization and a good education of and cooperation with the referring ophthalmologists are of utmost importance for the clear corneal graft in the long run.

Adolescent↗

PubMed2000

Oculocerebral non-Hodgkin's lymphoma with uveal involvement: development of an epibulbar tumor after vitrectomy.

Primary ocular lymphoma is the ocular manifestation of primary oculocerebral non-Hodgkin's lymphoma. We describe a 79-year-old woman with a 7-year history of bilateral uveitis and subsequent central nervous system lymphomas. Repeated diagnostic vitrectomy during the following 5 years failed to demonstrate intraocular lymphoma cells. Within 9 months after the second vitrectomy, an epibulbar tumor developed in the limbal region of the left eye at the site of the sclerotomy. The eye, blind and painful due to secondary angle-closure glaucoma, was enucleated. Histopathologically, the globe showed a diffuse large B-cell non-Hodgkin's lymphoma extending from the ciliary body outward through the sclerotomy. We conclude that, following vitrectomy, a primary ocular lymphoma may extend through the sclerotomy lesion and present as an epibulbar tumor. Uveal involvement may occur in oculocerebral non-Hodgkin's lymphoma.

Aged↗

PubMed2000

Migraine and tension headache in high-pressure and normal-pressure glaucoma.

PURPOSE: To analyze the association of normal-pressure glaucoma and migraine. METHODS: In a prospective study, 154 patients with glaucoma (56 normal-pressure subgroup and 98 high-pressure glaucoma subgroup), 55 patients with ocular hypertension, and 75 control subjects were analyzed by means of a standardized questionnaire based on International Headache Society criteria. RESULTS: According to the questionnaire, 46 patients (17%) were classified as suffering from migraine and 20 (7%) from tension headache (episodic and chronic). The prevalence of headache, migraine, and tension headache did not vary significantly among control subjects, patients with ocular hypertension, and patients with glaucoma, but migraine was significantly more common in patients with normal-pressure glaucoma (28%) compared with control subjects (12%; P<.05) and patients with high-pressure glaucoma (10%; P<.01). CONCLUSION: The results suggest an association of normal-pressure glaucoma and migraine and a potential, common vascular etiology of both diseases.

Adolescent↗

PubMed2000

Ocular findings in Fryns syndrome.

PURPOSE: To demonstrate the ocular histopathologic findings in Fryns syndrome, a multiple congenital anomaly syndrome, with characteristic features including Dandy-Walker malformation, cleft palate, diaphragmatic hernia, lung hypoplasia, distal limb anomalies and polyhydramnios. The prevalence is about 0.7 per 10,000 births. Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. METHODS: Case report. The ocular histopathologic and ultrastructural findings in a male fetus with Fryns syndrome who died immediately after his birth at 26th week of gestation are shown. RESULTS: An abnormal Descemet's membrane was found in addition to "cloudy corneae". Electron microscopy demonstrated absence of the banded collagen fibrils in Descemet's membrane, indicating corneal endothelial dysfunction. Otherwise, the eye was morphologically normal for its age; none of the other reported ocular features of Fryns syndrome were found. CONCLUSION: Corneal endothelial dysfunction might cause abnormal composition of anterior Descemet's membrane and could contribute to the "cloudy cornea" known to occur in Fryns syndrome.

Abnormalities, Multiple↗

PubMed2000

[Immunophenotype classification of macular corneal dystrophy: first case report of immunophenotype I A outside of Saudi Arabia. A clinical histopathological correlation with immunohistochemistry and electron microscopy].

BACKGROUND: Macular corneal dystrophy can be classified into three different immunophenotypes according to presence and distribution of sulfated keratan sulfate (SKS) in the cornea and serum levels of SKS. Immunophenotype I A has yet only been reported in patients with macular corneal dystrophy from Saudi Arabia. MATERIAL AND METHODS: Case report of a patient with macular corneal dystrophy type I A from Germany. Indirect immunohistochemistry for sulfated keratan sulfate was performed on the corneal buttons obtained after perforating keratoplasty (monoclonal antibodies 3D12/H7 and 5-D-4). The serum concentration of SKS was determined using the monoclonal antibody 5-D-4 in a serum antigen-inhibition assay. Immunogold labeling with the 5-D-4 antibody was performed to allow ultrastructural localization of SKS in the cornea. RESULTS: The patient demonstrated the typical clinical picture of macular corneal dystrophy. Serum levels of SKS were not detectable. Positive immunohistochemistry for SKS was found only within single keratocytes of both corneae without staining of the stroma (3D12/H7 and 5-D-4). Immunogold labeling localized SKS within stromal keratocytes and proved this patient to suffer from macular corneal dystrophy immunophenotype I A. CONCLUSION: Macular corneal dystrophy immunophenotype I A can be found outside Saudi Arabia in a German patient. All three immunophenotypes of macular corneal dystrophy are present in corneal buttons from German patients.

Adult↗

PubMed2000

[Synopsis of various electrophysiological tests in early glaucoma diagnosis--temporal and spatiotemporal contrast sensitivity, light- and color-contrast pattern-reversal electroretinogram, blue-yellow VEP].

BACKGROUND: Of the three glaucoma-defining criteria intraocular pressure, optic-nerve damage, and visual field damage, the latter is a late symptom. Therefore, in order to improve an early sensory diagnosis, new tests are necessary. It is the aim of the present paper to test new sensory methods, to rank them in an order of sensitivity, and to base them on possible pathophysiological mechanisms. PATIENTS AND METHODS: The tests were carried out in subjects of the Erlangen Glaucoma registry: Normals, patients with ocular hypertension, and patients with open-angle glaucoma without or with field defects. The tests are designed to preferentially probe the function of different groups of ganglion cells. Psychophysical methods: Temporal contrast sensitivity in a ganzfeld as "Erlangen flicker test" and spatio-temporal contrast sensitivity to test Magno-cell function. Electrophysiological methods: Pattern-reversal electroretinogram with a luminance-contrast pattern to test Magno-cell function, color-contrast pattern electroretinogram for Parvo-cell function, and blue-on-yellow visual evoked potential to test the "blue-sensitive" pathway. RESULTS: The most sensitive test is the temp.CS, it is significantly reduced in OHT (p < 0.01). The spatio-temp.CS is reduced in perimetric stages (p < 0.01). The BY-VEP is altered in the preperimetric stage (p < 0.01), the PR-ERG in perimetric stages (p < 0.01). The CC-ERG is reduced in even later stages. These results are in agreement with the hypothesis that tests selective for non-redundant neurons are of early diagnostic value. Multivariate analyses increase the early diagnostic value when different functions are tested in combination. CONCLUSIONS: When a particular test is taylored to the the special needs of certain groups of ganglion cells sensory defects can be observed before the occurrence of optic-nerve damage (OHT). The most sensitive psychophysical test is the "Erlangen flicker test" which is a screening test selective for M cells. The most sensitive electrophysiological test is the BY-VEP testing the blue-sensitive ganglion cells.

Color Perception↗

PubMed2000

Immunohistochemical localization of vascular endothelial growth factor, transforming growth factor alpha, and transforming growth factor beta1 in human corneas with neovascularization.

PURPOSE: To analyze presence and distribution of vascular endothelial growth factor (VEGF), transforming growth factor (TGF)alpha, and TGFbeta1 in human corneas with neovascularization due to different corneal diseases. METHODS: Indirect immunohistochemistry for VEGF, TGFalpha, and TGFbeta1, was performed on paraffin-embedded corneas obtained by keratoplasty. Corneas from each of the four main groups of histopathologic diagnoses associated with corneal neovascularization were analyzed (scarring after keratitis, graft rejection/insufficiency, acute necrotizing keratitis, scarring after mechanical/chemical injury). Subclassification of inflammatory infiltrates was done using immunohistochemistry for CD3 (T-lymphocytes) and CD68 (macrophages). RESULTS: The analyzed angiogenic factors were detectable in corneas from all four histopathologic groups in a similar distribution; capillary endothelial cells, stromal and intravascular inflammatory cells (T-lymphocytes, macrophages), and basal corneal epithelial cells stained positive for the tested angiogenic factors. CONCLUSION: The angiogenic factors VEGF, TGFalpha, and TGFbeta1 are detectable in human corneas with neovascularization. Their distribution is quite uniform in different corneal diseases, resulting in corneal angiogenesis. An antiangiogenic therapy inhibiting corneal neovascularization by antagonizing angiogenic factors would have to counteract several angiogenic factors.

Antigens, CD↗

PubMed2000

Reduced recovery of temporal contrast sensitivity after flicker stress in patients with glaucoma.

PURPOSE: To compare recovery of temporal contrast sensitivity after flicker stress between control subjects and patients with glaucoma. METHODS: Forty-three patients with primary open-angle glaucoma and secondary open-angle or normal-tension glaucoma of the Erlangen Glaucoma Registry and 30 healthy control subjects had their temporal contrast sensitivity measured at 37 Hz and their recovery time determined after monocular exposure to 30 seconds of 58% contrast full-field flicker. Recovery time of temporal contrast sensitivity was measured as the time interval from cessation of flicker until twice the threshold value was reached. RESULTS: Patients with glaucoma had a significant reduction in temporal contrast sensitivity (P < 0.001). Recovery time was also significantly longer in patients with glaucoma (23.8 +/- 24.1 seconds) than in control subjects (7.6 +/- 2.6 seconds; P < 0.001). There was a significant positive correlation between recovery time and mean visual field defect (0.5; P < 0.001) and a significant negative correlation between recovery time and neuroretinal rim area of the optic disc (0.32; P = 0.04). CONCLUSIONS: Recovery of temporal contrast sensitivity after flicker stress is impaired in some patients with glaucoma. This might be the result of functional impairment of the inner retina, outer retina, or both in glaucoma.

Adaptation, Ocular↗

PubMed1999

Adenoma of the nonpigmented ciliary epithelium mimicking a malignant melanoma of the iris.

Adenomas of the nonpigmented ciliary epithelium are rare tumors and are difficult to differentiate from amelanotic malignant melanomas of the ciliary body. We describe a 62-year-old woman with a nonpigmented, vascularized iris tumor with small satellites involving the anterior chamber angle. Clinically, an amelanotic, tapioca, malignant melanoma of the iris was suggested although the blood-aqueous barrier was intact. A block excision of 7.5 mm, a tectonic corneoscleral graft, and a simultaneous extracapsular cataract extraction with implantation of a posterior chamber lens were performed. Histopathologic and electron microscopic studies revealed an adenoma of the nonpigmented ciliary epithelium. Postoperative visual acuity was 20/40 OD after 6 weeks. An adenoma of the nonpigmented ciliary epithelium may mimic an amelanotic malignant melanoma of the iris and should be considered in its differential diagnosis.

Adenoma↗

PubMed1999

Recurrent macular corneal dystrophy type II 49 years after penetrating keratoplasty.

Recurrence of macular corneal dystrophy after keratoplasty is rare. We report light microscopic, immunohistochemical, electron microscopic, and serologic findings in a 78-year-old woman who underwent regrafting 49 years following the first penetrating keratoplasty. Examination of the corneal button revealed deposits of glycosaminoglycans in the graft beneath the Bowman layer, throughout the stroma, and in the endothelium with positive staining for antigenic keratan sulfate. By transmission electron microscopy, intracellular and extracellular deposits of a fibrillogranular material were detected in the stroma, Descemet membrane, and endothelium. The serum level of antigenic keratan sulfate was normal. Our findings indicate that macular corneal dystrophy type II may show late recurrence after penetrating keratoplasty with intense deposition of antigenic keratan sulfate in all corneal layers.

Aged↗

PubMed1999

Ocular findings in ichthyosis follicularis, atrichia, and photophobia syndrome.

Ichthyosis follicularis, atrichia, and photophobia (IFAP) are typical features of a rare neuroichthyosis termed IFAP syndrome. We demonstrate the ultrastructural findings of the eyes from a 33-year-old patient with IFAP syndrome. Clinically, eyebrows and eyelashes were absent from birth, and photophobia was noted at the age of 1 year. The globes measured 28 and 29 mm, respectively, and both eyes showed a posterior staphyloma. Histopathologically, bilateral centrally located subepithelial avascular corneal scarring with secondary corneal amyloid deposition was found. In addition to already described ocular abnormalities in IFAP syndrome we demonstrate ultrastructural anomalies of desmosomes and tonofilaments in corneal epithelium; defects of basement membrane, Bowman layer, and anchoring fibrils; secondary corneal amyloid deposition; and keratocyte degeneration. A defective tear film, recurrent atopic keratoconjunctival inflammations, or a primary anomaly of corneal epithelial adhesion are potential causes for the corneal defects. Photophobia is most likely due to corneal abnormalities.

Abnormalities, Multiple↗

PubMed1999

Recurrent optic nerve head infarctions associated with combined factor V Leiden- and factor II:G20210A-mutation.

PURPOSE: To demonstrate the association of bilateral recurrent optic nerve head infarctions with thrombophilia due to combined factor V:R506Q- and factor II:G20210A-mutation. METHODS: Case report. We examined a 55-year-old man with a two-year history of three segmental optic nerve head infarctions. Visual acuity was 20/80 on the left and 20/25 on the right eye. RESULTS: Ophthalmologic, cardiologic, radiologic, neurologic and hematologic-immunologic examinations were unremarkable except for increased APC-resistance (APC ratio: 1.4; normal value >2) due to heterozygous factor V:R506Q-mutation and high factor II-levels due to factor II:G20210A-mutation. Therapy with coumarin was instituted at INR 2.0-3.0 and no relapse has occurred over the past 1-year period. CONCLUSION: Combined occurrence of thrombogenic factor II:G20210A-mutation and factor V:R506Q-mutation may be causally linked to recurrent optic nerve head infarctions.

Factor V↗