AUGENHEILKUNDE 213/1999 – ISSUE 5

Abstracts:

S. 257-261 (Art. 414)

Mycophenolatemofetil in ocular immunological disorders. A survey of the Literature with 3 case reports
Alexander Reis, Thomas Reinhard, Rainer Sundmacher, Christoph Althaus, Adina Voiculescu, Bernd Kutkuhn

Background Up to now ophthalmologists have only a small number of substances in their therapeutic armamentarium for the treatment of ocular immunological disorders. These are very potent agents, and any rapidly proliferating cells are extremely sensitive to such agents, thus the possibility of severe side effects is great. Mycophenolatemofetil (MMF) has been shown to be effective in preventing acute graft rejection following clinical renal and cardiac transplantation with a low risk of adverse events. Likewise MMF was effective in the treatment of experimental immunological disorders. A review of literature was performed in Medline®.

Case reports We report on the effective use of MMF in a patient with ocular cicatricial pemphigoid, the switch from Cyclosporin A (CSA) therapy to MMF therapy following high-risk keratoplasty due to CSA allergy and about the combination therapy (CSA/MMF) in a patient following high risk keratoplasty, in whom CSA monotherapy was not sufficient to prevent allograft rejection.

Conclusions In these three cases MMF has been shown to be a safe and effective agent for the treatment of ocular immunological disorders. Whether these beneficial casuistic experiences will hold true will be dependend on the outcome of longterm studies underway.

Key words Mycophenolatemofetil - keratoplasty - immunosuppression

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S. 262-270 (Art. 423)

Clinical and histopathological aspects of 113 necrotizing malignant melanomas of the choroid
1. Clinical and histopathological features of necrotizing choroidal melanomas
Alexander A Bialasiewicz, Jin-Xue MA, Ulrich Schandig, Dietrich von Domarus, Gisbert Richard

Background Tumor necrosis may reflect a destructive immune reaction. Systematic and statistically significant comparative clinico-histopathologic studies have not yet been reported.

Patients and Methods 113 necrotizing choroidal melanomas (NCM) recruited from 701 enucleated globes 1967-1988 were resectioned, stained and compared to 100 choroidal melanomas without necrosis (CM), and data of 74 patients with a follow-up of more than 10 years were evaluated.

Results Statistically significant chracteristics of NCM were: patient age < 60 yrs. for NCM 27.4%, CM 46%; patient age in men for NCM was 64 yrs on average (CM: 58 yrs.), in women for NCM 67 yrs. (CM: 59 yrs.). Time elapsed between first symptoms and enucleation was < 12 months in 15.9% of NCM (89% for CM), and > 12 months in 23.9% of NCM (11% in CM). Mixed or epitheloid cell tumors was seen in 54.9% of NCM and 49% of CM, spindle cell tumors in 36.3% of NCM and 51% in CM. Advanced tumor stages T3 and T4 were present in 45.1% resp. 36.3% of NCM compared to 37% resp. 16% in CM. Scleral invasion was documented in 67.3% of NCM and 37% of CM, extrascleral dissemination in 43% of NCM and 16% of CM. Secondary glaucomas were seen in 62.2% of NCM and 6% CM, a penetration through Bruch's membrane in 61.0% of NCM and 46% of CM. Intratumoral hemorrhage was noted in 68.14% of NCM and 24% of CM, extratumoral bleeding in 23.9% of NCM and 0% CM. Inflammatory reactions in tumors were observed in 96.7% of NCM harboring >30% necrosis compared to 5% in CM, and extratumoral in 94.5% of NCM and 0% of CM. Intraocular extratumoral necrosis was seen in 23.9% of NCM and 0% of CM. There were no significant differences in the degree of pigmentation of the 90.3% pigmented NCM or of the 94% pigmented CM, neither in the tumor localization, being constantly behind the equator in 87% of cases. Survival of patients with NCM patients was 5 yeas and 9 months on average (5-year mortality rate 41.9%), and 74.3% were deceased from metastatic spread.

Conclusions Significant clinical and histopathological differences between necrotizing and non-necrotizing malignant melanomas of the choroid can be identified. The inflammatory reaction of NCM must be further elucidated, particularly with respect to the nature of tumor-infiltrating lymphocytes.

Key words malignant melanoma - choroid - inflammation - survival - risk factors - secondary glaucoma - intraocular hemorrhage

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S. 271-277 (Art. 424)

Clinical and histopathological aspects of 113 necrotizing malignant melanomas of the choroid
2. Immunogenetic characterization of T-cell receptor-positive tumor-infiltrating lymphocytes and survival of patients with necrotizing melanomas of the choroid
Alexander A. Bialasiewicz, Jin-Xue Ma, Gisbert Richard

Background T-cell receptor (TCR) gene analysis of tumor infiltrating lymphocytes (TIL) has been recognized to play a pivotal role in the immunosurveillance of solid neoplasms.

Patients and methods Therefore, 113 necrotizing choroidal melanomas (NMM) were compared to 100 non-necrotizing melanomas (MM) histologically and immunohistochemically.

Results NMM showed TIL more frequently (76.11% vs. 21%, p < 0.001). Va/Vb- (70.3% of NMM) and Vg1- (39.2% of NMM) and Vd1- (52.7% of NMM) TCR+ cells were distributed focal or diffuse, and correlated with tumor volume, diameter, and scleral invasion. 74.33% of NMM patients had died after 240 months (5 year survival: 58.1%). Mortality was not significantly associated with TIL (p < 0.33) or Va/Vb-TCR+ cells (p < 0.2), however, survival was significantly increased with evidence of Vg1- and Vd1-TCR+ cells (p < 0.026). Conclusions Va/Vb-, Vg1- and Vd1-TCR+ tumor infiltrating lymphocytes can be demonstrated in choroidal melanomas. This is a basis for functional studies providing a rationale for the evaluation of immmunotherapeutic modalities.

Key words tumor-infiltrating lymphocytes - inflammation - melanoma - survival

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S. 278-283 (Art. 426)

Surgical management of periocular basal cell carcinoma using frozen section control and immediate plastic reconstruction - indications and methods
Leonard M Holbach, Anselm Jünemann, Samir Muhammad

Purpose To review our first experiences with the surgical removal of basal cell carcinoma (BCC) utilizing frozen section control and immediate plastic reconstruction.

Patients and Methods We analyzed the accuracy of clinical diagnosis, methods of surgical excision, plastic repair and recurrence rates in 162 consecutive patients who underwent surgical excision utilizing either frozen section (np106) or biomicroscopic control (np56) between January 1991 and June 1996. Specimens used for intraoperative frozen-section monitoring were also fixed, processed, and sectioned for permanent sections and served as postoperative controls for the frozen sections (np43).

Results The accuracy of clinical diagnosis was 86% (np189). In 106 patients with BCC excised with frozen section control, there were no recurrences reported after a mean follow-up of 2.9 years. The mean surgical defect measured 55% of total eyelid length (range 10%-100%). The incidence of morphea pattern was 34%. Intraoperative re-excisions due to frozen sections positive for tumor were necessary in 31% of cases. Permanent postoperative sections of the tissue that had been used for intraoperative frozen sections confirmed in 97.5% of patients (np43) the preliminary findings made with frozen sections. In 56 patients with BCC excised with biomicroscopic control, three tumors recurred (5%) after a mean follow-up of 4.4 years. The mean surgical defect measured 42% of total eyelid length (range 16%-100%). The incidence of morphea type was 23%.

Conclusion Surgical removal utilizing frozen section control and immediate plastic repair appears to represent a reliable and effective option in the management of selected patients with periocular BCC.

Key words Periocular basal cell carcinoma - intraoperative frozen section control - immediate plastic repair

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S. 284-292 (Art. 429)

Clearance of liposome-incorporated ciprofloxacin after intravitreal injection in rabbit eyes
Burkhard Wiechens, Rea Krausse, Joachim B Grammer, Doris Neumann, Uwe Pleyer, Gernot IW Duncker

Background Ciprofloxacin (CIP) is a fluoroquinolone-antibiotic with a high antimicrobial activity against all pathogens causing bacterial endophthalmitis. After intravitreal injection, however, elimination half-life of this antibiotic is only 2.2 hours. To prolong intraocular bioavailability this study was performed to incorporate CIP into liposomes and to determine its clearance from the vitreous after intravitreal injection.

Materials and methods CIP was incorporated into multilamellar vesicles by mechanical dispersion. 0.1 ml of this suspension (equiv. 273.6 µg CIP) was injected into the midvitreous of pigmented rabbit eyes (Chinchilla-bastards). One day, 3 and 14 days after the injection intravitreal concentration of CIP was determined by means of high-pressure-liquid-chromatography after dissolution of the liposomes by ultrasound. At the same intervals serum concentration of the antibiotic was examined as well.

Results Within 24 hours intravitreal concentration fell to 18.0 µg/ml. Three days after the injection the concentration of CIP was 6.9 µg/ml. This is still above the minimal inhibitory concentration (MIC90) of the most common ocular pathogens. At 14 days CIP was not detectable in the vitreous any more. The serum concentration was between 0.04 µg/ml and 0.07 µg/ml. 3 and 14 days after injection no CIP could be detected.

Conclusions This study shows that the incorporation of CIP into liposomes can be achieved in sufficient doses by mechanical dispersion method. After intravitreal application the bioavailability of the antibiotic can be markedly improved. Even after 3 days the intravitreal levels were above the MIC90 of the most common endophthalmitis pathogens.

Key words ciprofloxacin - fluoroquinolones - intravitreal injection - clearance - antibiotic - liposome incorporation - endophthalmitis

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S. 293-300 (Art. 410)

Cutting edges after automatic lamellar keratotomy
Frank Wilhelm, Thomas Gießmann, Renate Hanschke, Gernot Duncker

Background In order to perform an automatic lamellar keratotomy microkeratomes are used, which differ from each other in several technical details. This study was done to examine, whether there are characteristics of the cutting edge, typical of each device, and whether there are some correlations between the cutting quality and technical parameters.

Methods In Germany seven different keratomes are used today. We perfomed the procedure of automatic lamellar keratotomy by using each of them on 8 fresh enucleated pig eyes and we examined the corneal tissue by means of scanning electron microscopy. The cutting edges were judged by their smoothness and sharpness.

Results A dominantly smooth cutting edge was found on the corneas cut by the MKM-System, the Universal Keratome and the BK-Microkeratome Set, while the Automatic Corneal Shaper, the Microtech-Mikrokeratom, the Rotor-Keratom and the Schwind-Mikrokeratom mostly produced a saw-toothed edge.

Conclusions The quality of the cutting edge may be influenced by the relationship of the speed of the pass and the rate of blade oszillation / rotation. Therefore it seems that a lower feed during one oszillation / rotation results in a more smooth pattern of the cutting edge.

Key words Refractive surgery - automatic lamellar keratotomy - microkeratomes - scanning electron microscopy

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S. 301-305 (Art. 422)

A new surgical approach for reconstruction of the orbit
Rüdiger Marmulla, Herbert Niederdellmann, Birgit Lorenz, Ralf Dammer, Christoph Niederdellmann

Background Complex posttraumatic malpositions of the orbital walls require repositioning osteotomy. Computer tomography, stereolithography models and tele-X-rays are used in planning. However, the precision achieved in the planning phase could not so far be translated to patients (1). The Surgical Segment Navigator SSN is the first highly precise computer-assisted system to transfer laboratory planning data concerning the repositioning osteotomy of orbital walls to a surgical site.

Materials and methods The SSN is based on infrared technology such as the Surgical Tool Navigator STN and the Surgical Microscope Navigator SMN manufactured by Carl Zeiss. Laboratory planning data are transferred to the surgical site by measurements with infrared transmitters which are checked by an infrared camera.

Results A surgical planning can be carried out exactly using the Surgical Segment Navigator. Moreover, the SSN displays hidden levels of an extensive bone segment which are not visible via a bicoronary approach (e.g. orbital floor and facial wall of the maxillary sinus) clearly on monitor and helps to navigate the complete segment.
Conclusions The Surgical Segment Navigator is the first computer-assisted system for highly precise repositioning osteotomy of the orbital walls.

Key words Reconstruction of the orbit - posttraumatic enophthalmos - Surgical Segment Navigator - computer-assisted surgery - surgical planning - repositioning osteotomy

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S. 306-308 (Art. 407)

Diode laser cyclophotocoagulation of secondary glaucoma caused by anterior, necrotizing scleritis
Torsten Schlote, Jörg Mielke, Manfred Zierhut, Benedikt Jean, Hans-Jürgen Thiel

Patient A 60-year-old female patient presented with recurrent anterior, necrotizing scleritis with inflammation and a newly developed secondary glaucoma in the right eye. Anterior uveitis occurred some years before. Severe scleral thinning was circumferentially present and focal scleral ectasia was found. Physical examination revealed no systemic association of scleritis. Immunosuppressive therapy with metotrexate was initiated and control of scleritis achieved. Intraocular pressure elevation persisted and was refractory to glaucoma medication. Diurnal pressure curve showed IOP-values of 40 mm Hg despite the use of systemic carbonic anhydrase inhibitors. Visual acuity was 20/50 in the right and 20/25 in the left eye.

Method Diode laser cyclophotocoagulation (Oculight SLx 810 nm, Iris Medical Instruments Inc. California, USA) was performed under general anaesthesia using reduced parameters for application (12 laser spots, 1 second, 1,25 W). No complications occurred during and after laser application. Postoperatively, intraocular pressure was within normal range between 14 and 18 mm Hg. No reactivation of scleritis or uveitis was seen.

Conclusion In our experience, diode laser cyclophotocoagulation is effective and safe in treating secondary glaucoma associated with anterior, necrotizing scleritis with inflammation and uveitis using reduced parameters for application.

Key words scleritis - secondary glaucoma - cyclophotocoagulation - therapy

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S. 309-311 (Art. 408)

Axial displacement of IOL and visual impairment
Andreas Frohn, Wolfgang Fink, Hans Jürgen Thiel

Background After a cataract surgery procedure with vitreous loss a vitreous strand remained in the wound. An unusual visual impairment was observed.

Patient A shift in refraction depending on the pupillary diameter was observed. In miosis the refraction was estimated to c1,0 sph, in mydriasis P1,0 sph/P0,5 cyl, respectively.

Method We presumed that the vitreous strand between the iris and the intraocular lens caused an axial displacement of the IOL during the change from miosis to mydriasis and v.v. Therefore the vitreous strand was surgically eliminated. After surgery the refractive changes by pupillary actions were overcome. In a computer simulation (ray tracing), the amount of axial displacement of the IOL was estimated to 1,5 mm.

Conclusion This paper reminds of considering other reasons for loss of VA than CME, e.g. axial displacement of IOL. An adequate method of examination is the measurement of refraction in miosis and mydriasis.

Key words capsular rupture - vitreous string - vision distortion - ray tracing - macular edema

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S. 312-313 (Art. 417 DF)

Focal mucinosis of the eyelid
Jens Martin Rohrbach, Harald Preßler, Tzonka Djelebova, Stefan Schüller

Purpose To present a case of focal mucinosis of the eyelid.

Patient A male patient of Turkish origin born in 1982 desired therapy in 1995 for an asymptomatic tumour of the left medial upper lid which had been present probably since birth. The tumour was reduced with argon laser photocoagulation. Fourteen months later, in 1997, the tumour had enlarged so that a surgical excision was performed which histologically was interpreted as an unspecific inflammation. In 1998, the tumour had grown again at the original site, and there were additional three pedunculated tumours nearby at the lid margin. All tumours were surgically excised. Histology showed empty spaces in the upper dermis which contained some connective fibers, benign appearing fibroblasts and some leukocytes. The PAS-reaction and S100-immunohistology were negative while the lesion stained positive for alcianblue. The epidermis was intact. A diagnosis of a focal mucinosis of the eyelid was established.

Conclusions Focal mucinosis of the eyelid seems to be exceedingly rare. It is probably caused by a local overproduction of hyaluronic acid of unknown etiology. Our patient demonstrates that focal mucinosis might perhaps be congenital. Moreover it may recur if excision is not complete. Focal mucinosis should be included in the differential diagnosis of long-lasting, asymptomatic lid tumours.

Key words Focal Mucinosis - Eyelid - Hyaluronic acid

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S. 315-319 (Art. 436)

Prognosis of keratoplasty in keratitis phlyctenulosa peracta
Carsten Meyer, Hans-Jürgen Meyer

Background In spite of clear transplants the results of keratoplasty are often disappointing. We looked for possible reasons for these failures.

Patients and methods We analyzed in 204 eyes of 115 patients the factors influencing the prognosis. Our results are based on medical records and on demanded reports of the treating ophthalmologists. Patients were divided after different aspects (method of operation, beginning of the disease, operation of the first or second eye).

Results A frequently associated cataract and the method of surgery (aphakia, one- or two-step, triple procedure) has no influence on the prognosis. However, age of onset and density of corneal scars are crucial. This is especially evident in eyes first operated on. In case of early onset (before the age of five) visual acuity only reached 0.21 average postoperatively, whereas patients with late onset gained visual acuity of 0.59. The fellow eye (np89) had postoperative visual acuity of 0.48. In patients with poor postoperative visual improvement (^ two lines) high myopia was apparent in 50%.

Conclusion For estimating the prognosis besides early childhood amblyopia we must take into account high myopia induced by formdeprivation (FDM). Exact evaluation of patients history and preoperative ultrasound biometry are recommended.

Key words Keratitis phlyctenulosa peracta - keratoplasty - age of onset - formdeprivation myopia - preoperative biometry

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S. 320-325 (Art. 435)

External trabecular excision (ETE) - as an alternative to filtering procedures in patients with primary open angle glaucoma
Jörg Schmidt, Peter Kroll, Gabriele Beate Kuba

Background Postoperative complications concerning glaucoma filtering surgery (trabeculectomy, goniotrepanation) often include hypotonia that may lead to athalamia or choroidal detachment, which are difficult to handle. Cystic non filtering blebs are due to postinflammatory reactions, and may limit the success of filtering surgery. Aim of the study was to compare the success and the complications of a new operating technique, which will be described, with those of usual glaucoma filtering surgery.

Patients and methods In 24 open angle glaucoma patients with mean intraocular pressure of 28.12 mm Hg (±8.6) we performed external trabecular exzision in 25 eyes since june 1997. Preoperative visual acuity and peak intraocular presure were compared retrospectively in all eyes with the values of the first postoperative day, in 22 eyes after one month and in 17 eyes after 3 months.

Results Intraocular pressure measured between 0 mm Hg and 16 mm Hg on the first postoperative day (7.64 mm Hg ±4.3), after one month between 10 mm Hg and 30 mm Hg (17.81 mm Hg±5.5) and after 3 months between 9 mm Hg and 26 mm Hg (15.29 mm Hg±4.2). After 1 month 10 of 22 (45%) and after 3 months 7 of 17eyes (42%) required antiglaucomatous drugs; 3 eyes needed gonitrepanation (2 weeks, 1 month, 3 months after ETE). Concerning postoperative complications, we observed 6 choroidal detachments, once erythrocoytes in the anterior chamber, twice hyphemata, twice inflammatory reaction in the anterior chamber, two flat anterior chambers and twice a positive seidel test.

Conclusion Complications after ETE are similar to those after filtering surgery. Postoperative intraocular pressure dip after ETE in most eyes was not as pronounced as after goniotrepanation or trabeculectomy, and postoperative complications were all reversible. 45% of the eyes again needed antiglaucomatous drugs after one month and 42% after 3 months. A prospective long-term study has to verify the success respectively the complications of ETE.

Key words External trabecular exzision - glaucoma filtering surgery - primary open angle glaucoma

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S. 326-330 (Art. 430)

Behaviour of intraocular eyepressure after Nd:YAG-laser-capsulotomy - a critical judgment by prophylactic application of a topical carbonic anhydrase-II-inhibitior dorzolamide hydrochloride (Trusopt®)
Claudius Lachmann, Klaus-Michael Jörg, Reinhard Trinkmann

Background The aim of the present study was to examine the influence of the topical carbonic anhydrase-II-inhibitor dorzolamide hydrochloride (Trusopt®) in regarding to behaviour of intraocular eyepressure after Nd:YAG-laser-capsulotomy.

Patients and methods The study was carried out as a double-blind-test. In a period of nine months, 120 patients underwent Nd:YAG-laser-capsulotomy. One group was given dorzolamide hydrochloride (Trusopt®), the other group (placebo) remained without medication. The intraocular pressure was measured bevor capsulotomy and four hours post-op. For statistical evaluation the t-test of not-associated spot-checks was performed.

Results The prophylactic use of the topical carbonic anhydrase-II-inhibitor dorzolamide hydrochloride (Trusopt®) after nd:YAG-laser-capsulotomy shows a highly significant lowering of intraocular pressure, i.e. 1.09 mm Hg in the average.

Conclusions It was demonstrated that a single dose of dorzolamide hydrochloride (Trusopt®) after Nd:YAG-laser-capsulotomy could lower intraocular pressure significantly. Prophylactical usage of dorzolamide hydrochloride (Trusopt®) is a useful method to prevent the increase of intraocular pressure and thus to protect the eye against harzadous damage.

Key words glaucoma - intraocular pressure - Nd:YAG-laser-capsulotomy - dorzolamide

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S. 331-340 (Art. 444)

Diffus malignant melanomas of the uvea
A clinico-pathologic study of 39 patients
Ulrike C Braun, Volker C Rummelt, Gottfried OH Naumann

Background Focal malignant melanomas involving only the iris have a very good prognosis. Involvement of the iris and /or diffus growth makes the prognosis worse. The prognostic parameters of diffusely growing malignant anterior uveal melanomas are analysed.

Patients and methods From 1981-1995 serial sections of 39 eyes, 23 women and 16 men, of diffusely growing malignant uveal melanomas were histologically examined. Patients were between 9 and 80 years old (median 56 years). Follow-up was between 2 months and 15 years. At the end of the study, 13 patients had died from metastasis, all showed involvement beyond the iris. Four patients died from other causes.

Results The 39 diffus malignant melanomas were categorized into 10 affecting only the iris, 18 iris and ciliary body and 11 involving iris, ciliary body and choroid. 14 spindle- cell, 23 mixed-cell and 2 epitheliod- cell tumors were identified. We observed between 0 and 4 mitoses in 40 high-power fields. Only 4 melanomas showed than 100 tumor-infiltrating lymphocytes in 20 high-power fields. Nine tumors showed extra-scleral spread, nine displayed vascular networks. Univariate Kaplan-Meier survival curves showed as significant prognostic factors: localisation in the iris only (pp0.0008), spindle cell type (pp0.0002) and absence of vascular networks (pp0.01). This was confirmed by multivariate Cox analysis.

Conclusion Patients with diffuse malignant melanoma confined to the iris alone have an excellent prognosis for survival. Diffuse tumor cell spread can be diagnosed at the slitlamp with high magnification. Conservative follow-up is acceptable, as long as ciliary body involvement is ruled out. Only an eye with medically uncontrollable secondary glaucoma should be enucleated. Anti-glaucoma filtering procedures are contraindicated because of the danger of extraocular spreading malignant cells.

Key words malignant melanoma - anterior uvea - diffusely growing malignant uveal melanomas - vessel parameter - vascular network - iris melanoma - iris- and ciliary body melanoma - iris- ciliary body- choroidal melanoma - extrascleral spread - malignant melanocyte

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S. 341-346 (Art. 432)

Peak pulse blood volume and topical antiglaucomatous drugs in a cynomolgus monkey high tension glaucoma model
Karl-Georg Schmidt, Andrea v. Rückmann, Dieter Eisenmann, Daniel Y Stegman, Thomas W Mittag

Background The present study was designed to investigate the effect of topical antiglaucoma drugs on Peak Pulse

Blood Volume (PPBV; German abreviation: PGBV) in cynomolgus monkey eyes without (CMNG; German abreviation: RA-KTL) and with lasersurgically induced glaucoma (CMG; German abreviation: RA-LHDG).

Methods CMG unilaterally received 2-3 laser treatments so as to develop the lasered-eye glaucoma model. Intraocular pressure (IOP; German abreviation: IOD) and Ocular Pulse Amplitude were measured and PPBV was determined until the glaucoma model had stabilized. Consecutively topical antiglaucoma drugs (epinephrine, paraaminoclonidine, pilocarpine, timolol) were investigated in 4-8 animals in CMNG and CMG eyes. Results IOP and PPBV were not significantly altered in CMNG. In the CMG eyes epinephrine and paraaminoclonidine did not significantly alter IOP or PPBV, whereas pilocarpine and timolol sig. (p < 0.01) reduced IOP and significantly (p < 0.05) increased PPBV.

Conclusion With respect to improved PPBV in the CMG eye the rank order of drug effectiveness is timolol > pilocarpine > paraaminoclonidine > epinephrine.

Key words Experimental primary open angle glaucomas - intraocular vascular system - ocular pulse amplitude - peak pulse blood volume - topical glaucoma drugs

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S. 347-350 (Art. 390)

Peak pulse blood volume during manipulation of local perfusion parameters
Karl-Georg Schmidt, Andrea v. Rückmann, Volker Klingmüller, Lutz E Pillunat, Thomas W Mittag

Background Choroidal autoregulation is discussed controversially. The present study was designed to investigate for the effect of standardized manipulation of local perfusion parameters on choroidal hemodynamics in cynomolgus monkey eyes with lasersurgically induced glaucoma (CMG; German abreviation: RA-LHDG).

Methods CMG unilaterally received 2-3 laser treatments so as to develop the lasered-eye glaucoma model. Intraocular pressure (IOP; German abreviation: IOD) and Ocular Pulse Amplitude (OPA) were measured and Peak Pulse Blood Volume (PPBV; German abreviation: PGBV) was determined.

Results In CMG PPBV was not significantly (sig., p > 0.05) altered in laser-treated eyes - despite a sig. (p < 0.05) increase in IOP. Untreated contralateral control eyes of monkeys with an IOP-increase > 5 mm Hg in laser-treated eyes showed a sig. (p < 0.05) increase in PPBV.

Conclusion Unchanged PPBV in laser-treated eyes of CMG and an increase in PPBV in control eyes point at a bilateral autoregulatory compensatory mechanism, which is more manifest in eyes with IOPs in the normal range.

Key words Experimental open angle glaucomas - choroid - ocular pulse amplitude - peak pulse blood volume - autoregulation

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S. 351-354 (Art. 425)

Pars plana vitrectomy for Borrelia burgdorferi endophthalmitis
Petra Meier, Rosemarie Blatz, Martina Gau, F.-Bernhard Spencker, Peter Wiedemann

Background Ocular manifestations of Lyme borreliose present with unusual forms of conjunctivitis, keratitis, optic nerve disease, uveitis, vitritis and rarely endophthalmitis.

Case report A 57-year-old man working as logger in Saxony-Anhalt suffering from an endophthalmitis on his left eye was referred to us. The vision of his left eye was intact light perception and hand motions. The slit-lamp examination revealed severe inflammation of the anterior chamber with hypopyon, posterior synechiae, and opacity of the posterior lens capsule. Funduscopy showed no red reflex, no retinal details. In the local hospital serum analysis was performed and showed in Western-Blot IgM- and IgG-antibodies against Borrelia burgdorferi. Despite of intravenous application of ceftriaxon for 14 days panuveitis persisted, and endophthalmitis developed when antibiotic therapy was finished.

Results During pars plana vitrectomy a sharply delineated cystic lesion containing yellowish fluid was revealed, and cremy yellow fluid was aspirated. Microscopically in hematoxylineosin stained slides of the aspirate structures consistent with Borrelia burgdorferi were found. Postoperatively vision increased to 1/15. Despite of a second intravenous ceftriaxon treatment for 14 days we observed a retinal vasculitis in the follow up of 6 months.

Conclusions Despite intravenous ceftriaxon-therapy borrelia burgdorferi must have survived in the vitreous body. Further investigations are required with respect to the use of other antibiotics or immunsuppressives.

Key words Borrelia burgdorferi - endophthalmitis - pars plana vitrectomy - retinal vasculitis

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S. 355-357 (Art. 434)

Optic neuropathy following erythema infectiosum
Helmut Wilhelm, Christiane Hartmann, Vera Boesche-Abele

Background Optic neuropathies can not always easily be classified as neuritis or ischemic disease.

Case report A 39 year old woman suffered from unilateral optic neuropathy 9 days after the acute onset of a general disease with erythema and joint swellings. The optic disc swelling and visual fields resembled an anterior ischemic optic neuropathy, however, a marked improvement was achieved by steroid treatment. The general disease proved to be erythema infectiosum.

Conclusions Erythema infectiosum as a frequent children's disease causes very rarely central nervous involvement, optic neuropathy has not been reported yet. In cases of atypical optic neuropathies search for an underlying systemic disease is of value.

Key words Optic neuropathy - steroid treatment - erythema infectiosum

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S. 358-361 (Art. 437)

Uveitis-masquerade syndrome of M.Gaucher
Causal treatment with alglucerase-substitution-therapy
Katharina Dann, Christoph Althaus, Andreas Kersten, Stephan vom Dahl, Rainer Sundmacher

Background Gaucher's disease, a sphingolipidose transmitted by autosomal-recessive inheritance, is caused by a deficiency of the lysosomal enzyme b-glucocerebrosidase which is responsible for hydrolysation of glucocerebroside to ceramid and glucose. Thus glucocerebroside is accumulated in the reticuloendothelial cells of spleen, liver and bone marrow and pathognomonic Gaucher's cells are formed.

Case report In May 1997 a 42-year-old female patient presented with left-sided intermediate uveitis of unknown origin and decreasing visual acuity to perception of hand movements and intact projection of light since September 1996. To exclude a systemic disease a thorough medical examination - showing hepatoslpenomegaly, anemia, thrombocytopenia and bone lesions - was iniciated and revealed advanced M. Gaucher (Type I) by bone marrow punction. Intravenous therapy with alglucerase was administered promptly. After five months of treatment vitreous opacities resolved almost completely and visual acuity increased to 0,7.

Conclusions The dramatic improvement occurred under treatment with alglucerase after no response to steroid treatment. Thus intermediate uveitis with severe vitritis can be interpreted as uveitis masquerade syndrome with M. Gaucher. To the best of our knowledge, this is the first case of M. Gaucher presenting initially as intermediate uveitis and showing successful clinical improvement during administration of alglucerase.

Key words Intermediate uveitis - M. Gaucher - alglucerase treatment

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S. 362-366 (Art. 433)

Juvenile neuronal ceroid-lipofuscinosis (Batten's disease)
Birte Neppert, Bianka Kemper

Background The neuronal ceroid-lipofuscinosis (NCL) belongs to progressive neurodegenerative disorders of childhood with both ophthalmologic and neurologic symptoms. In the most common type in Germany, the juvenile type, the ophthalmological examination is essential for an early diagnosis.

Patient A 5-year-old boy had exhibited a loss of visual acuity, visual field and colour perception in his pre-school age. His clinical features and electrophysiologic data are presented. The final diagnostic clues were drawn from the neuropediatric and cytologic examinations.

Results This patient shows the typical clinical feature of the juvenile NCL with a relatively rapid visual loss with bull's eye maculopathy in the pre-school- to early school age. His electroretinogram was abolished, and his EEG showed pathologic results. The diagnosis could be established by electron microscopy of his lymphocytes. Epilepsy and intellectual defects are expected to set in only few years later.

Conclusions Although there is no therapy, the ophthalmologist should be aware of this rare entity because prognostic counselling, social help and perhaps genetic counselling could be offered to the families. In many cases, a prenatal diagnosis is possible.

Key words bull's eye maculopathy - ceroid-lipofuscinosis - electroretinogram - visual evoked potentials - electron microscopy of lymphocytes - storage disease - gene defect - genetic counselling

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S. 367-369 (Art. 421 DF)

The removal of a Loa Loa worm in a Westafrican tourist
Helmut G Sachs, Markus Heep, Veit-Peter Gabel

Background Loa Loa is a chronic parasitemic disease which is endemic in the tropical rain forests of western africa. Vector of this disease is a mancrove fly with the name Chrysops. Besides the eye worm and skin affections a systemic infection with microfilariae is common.

Patient A Westafrican tourist from Bangibe showed up at the university eye clinic. His complaints were a red eye and a mobile subconjunctival tumor (Fig. 1) that showed vermiform movements. The worm was transparent and 4-5 cm in length. After topical anaesthesia and the attempt to paralyse the worm (1) with Pilocarpine 2% it vanished. Two days later the patient showed up in the morning for the planned blood test. No worm was visible at that time but at noontime the blood test was carried out and at that time the worm was visible in the nasal conjunctiva. This time the worm was removed without delay under topical anaesthesia. The worm was fixed with a forceps through the conjunctiva which was opened for 0.5 cm. The worm was grasped with a second forceps and drawn out under massive vermiform movement. Systemic therapy was recommended with Hetrazan (Diethylcarbamazine) using Corticosteroides and Antihistamine to minimize allergic side effects by the therapy due to the systemic microfilariae blood load. Eosinophilia was 8%.

Conclusions A subconjuctival Loa Loa worm can be removed under topical anaesthesia by fixing it with a forceps through the conjunctiva and opening it and grasping the worm with a second forceps. According to our experience the paralysation with Pilocarpine cannot be realized. Careful systemic therapy avoiding reported allergic side effects with Hetrazan which is not available in Germany is necessary.

Key words Loa loa - Filariasis - Eye-Diseases-parasitology - Loiasis

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