AUGENHEILKUNDE 213/1999 – ISSUE 2

Abstracts:

S. 067-070 (Art. 383)

Ocular growth after successful glaucoma surgery in the first year of life - follow-up data for primary congenital glaucoma
Thomas S Dietlein, Philipp C Jacobi, Günter K Krieglstein

Background Echographic biometry of the ocular axial length is a helpful criterion in diagnosis and follow-up of primary congenital glaucoma. However, quantitative assessment of ocular growth following successful primary glaucoma surgery in the first year of life is hardly to find in literature.

Patients and Methods In 36 eyes from 21 patients (mean age 4.4B2.4 months) with primary congenital glaucoma A-scan biometry was performed under general anesthesia before and 7.2B4.2 months following successful primary glaucoma surgery and retrospectively summarized. Results Preoperative axial length was 21.7B2.5 mm, postoperative axial length was 22.4B1.6 mm. Ocular growth was significantly stronger in eyes with a preoperative axial length < 20 mm (pp0.0012) and in children younger than 3 months at surgery (pp0.0004).

Conclusions Preoperative axial length and age are basic factors for the interpretation of ocular growth following glaucoma surgery in primary congenital glaucoma. Temporary cessation of ocular growth is a frequent finding after successful pressure-reducing surgery in eyes with axial length > 22 mm and in children aged 3 months or older.

Key words Congenital glaucoma - buphthalmia - sonography - axial length - intraocular pressure

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S. 071-073 (Art. 382)

Cataract surgery in patients with retinitis pigmentosa
Susanne Schumacher, Michael JM Groh, Gottfried OH Naumann

Background Because of the retinal alterations visual acuity of patients with retinitis pigmentosa is more affected by loss of contrast than in normal persons. Therefore cataract in retinitis pigmentosa (mostely posterior subcapsular cataract) leads earlier to decreasing visual acuity than is expected from the lens opacity. In a retrospective study we have looked up all patients with retinitis pigmentosa and cataract which were operated between 1989 and 1997 at our department.

Patient and methods Between 1989 and 1997 50 eyes of 36 patients (21 female, 15 male; mean age 51B11.4 years) with the diagnosis of retinitis pigmentosa and cataract underwent extracapsular cataractextraction and IOL-implanation. In a retrospective study we studied all case histories of these patients. Mean follow up time was 1.2B1.2 years.

Results Preoperative visual acuity was 0.1B0.1, best postoperative visual acuity was 0.3B0.2. Visual field was not affected by cataract-surgery. Postoperative complications were not found. 2/3 of all patients were satisfied withe the postoperative visual acuity, 1/3 of all patients did not give comments.

Conclusion In patients with retinitis pigmentosa cataract surgery leads to significant increase of visual acuity. Cataract extraction should not be with held from patients with retinal dystrophy.

Key words retinitis pigmentosa - cataract - cataract surgery - success - indication

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S. 074-080 (Art. 376)

Radiotherapy alone for severe, progressive thyroid-associated orbitopathy: long-term results with comparison of scoring systems
Gabriele-Charlotte Gusek-Schneider, Michael Heinrich Seegenschmiedt, Anselm Jünemann, Ludwig Keilholz, Wolfgang Becker, Johannes Hensen

Background Therapeutic results after radiotherapy in thyroid associated orbitopathy (TAO) often are not comparable, because either different therapeutic methods at the same time or different scores were used in the evaluation. This study focusses on radiotherapy alone by means of different evaluation scores.

Patients and methods 60 patients (49 women, 11 men) received standard external beam radiotherapy (20 Gy: 10 fractions of each 2 Gy) as ultima ratio after failing different other therapies of thyroid associated orbitopathy. The mean interval from beginning of the symptoms to the radiotherapy was 17B36 months (between 6 and 240 months). The follow up was documented - classified by means of 4 different scores - before radiotherapy, 6-12 weeks, 1 year after radiotherapy and at last follow up. The changes of symptom categories or grades of the different scores were analysed.

Results Significant changes of the ophthalmic scores were observed when comparing the endpoints at 6-12 weeks, and at 1-year follow up after radiotherapy. The "classical" Werner score at 12 months follow up did not correlate well with the other TAO scores: American thyroid association (ATA) scoring system, Stanford scoring system, International ophthalmopathy index, while all other TAO scores revealed a high correlation among each other. According to the Orbitopathy Index (OI) of Grußendorf an improvement from 14.2 points to 6.0 points was achieved. Soft tissue involvement and corneal involvement demonstrated the highest response rate (83/87%) , extraocular muscle involvement and proptosis a good response rate (69/70%). No long-term complications were observed.

Conclusion According to this study there are indications that external beam radiotherapy is a suitable therapy even after pretreatment and a longer course of TAO. The OI, the ATA and the Stanford scoring systems lead to simular results in the assessment of thyroid orbitopathy.

Key words thyroid associated orbitopathy - scores - radiotherapy - quality assurance

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S. 081-086 (Art. 385)

Fundus autofluorescence in patients with macular dystrophies, malattia leventinese, dominant drusen and age-related drusen
Andrea von Rückmann, Karl-Georg Schmidt, Fredrick W Fitzke, Alan C Bird, Karl Wilhelm Jacobi

Purpose The lack of histopathological material has placed limitation on our knowledge on the composition of focal deposits in eyes with macular dystrophies, malattia leventinese, dominant drusen and age related macular degeneration. This study was designed to study the composition of focal deposits in these eyes by documenting fundus autofluorescence in vivo.

Methods Fundus autofluorescence was documented in 343 eyes of 199 subjects with macular dystrophies, malattia leventinese, dominant drusen and age-related macular degeneration using a laser scanning ophthalmoscope (Zeiss, Oberkochen; excitation wavelength: 488 nm, barrier filter at 521 nm).

Results Autofluorescence of focal deposits was increased in eyes with macular dystrophies. In eyes with malattia leventinese and dominant drusen autofluorescence intensity of focal deposits showed a wide spectrum. In contrast, autofluorescence of age-related drusen was within normal limits. Background autofluorescence intensity was increased in eyes with macular dystrophies and within normal limits in eyes with malattia leventinese, dominant drusen and age-related drusen.

Conclusion The technique of in-vivo recording of fundus autofluorescence allows the differential diagnosis between macular dystrophies/ malattia leventinese, dominant drusen/ age related drusen when otherwise not possible.

Key words lipofuscin - fundus autofluorescence - laser scanning ophthalmoscope - macular dystrophy - malattia leventinese - dominant drusen - age related drusen

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S. 087-092 (Art. 363)

Wolfgang Stock, his ophthalmopathological collection, and the progress in glaucoma therapy in the second half of the 20th century
Jens Martin Rohrbach, Torsten Schlote, Hans-Jürgen Thiel

Background As long as Wolfgang Stock was head of the University Eye Clinic Tübingen (1921-1952), he founded a voluminous collection of ophthalmopathological slides, which reflect the knowledge about and possible therapies for various eye diseases at that time.

Material The "Collection Stock" contains ca. 450 glaucoma specimens. Of these, 50 were chosen at random and investigated macroscopically and microscopically. Each specimen was supplied with a (main) diagnosis. For comparison, 50 eyes with glaucoma enucleated between 1992 and 1997 were examined.

Results Nowadays, "glaucoma enucleation" is performed nearly exclusively because of secondary angle closure glaucoma. In contrast, the "Collection Stock" contains various eyes with 1. primary glaucoma without previous operation, 2. congenital glaucoma, 3. lens induced glaucoma, 4. aphakic glaucoma, and 5. complications after glaucoma surgery. No case of diabetic neovascular glaucoma or PEX-glaucoma was found.

Conclusions Due to improvement in ophthalmological care, development of various pressure-lowering and anti-inflammatory drugs, new surgical techniques and, probably most important, introduction of the operation microscope, major advances concerning glaucoma therapy and avoidance of complications have been achieved in the last 50 years. Thus, the spectrum of "glaucoma enucleations" has changed dramatically, and certain types of (open angle) glaucoma Wolfgang Stock was very familiar with have in modern ophthalmopathological practice almost fallen into oblivion.

Key words Wolfgang Stock - Glaucoma - Enucleation - Histology - History of Ophthalmology

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S. 093-096 (Art. 381)

Correlation of glucose and lactate concentrations in human corneas and organ-culture medium
Claudia Redbrake, Andrea Frantz, Sabine Salla

Background Metabolic changes of the human donor cornea during organ-culture are not at all reflected by the endothelium. Therefore metabolic investigations have become of increasing interest. It was the aim of this study to determine the correlation between glucose and lactate in storage medium and within the cornea itsself and to find thereby an additional parameter for glucose metabolism during organ-culture.

Methods Glucose and lactate were examined in 166 organ-culture medium samples as well as in 106 human corneas by enzymatical optical methods. Investigations were carried out after 1,7,15, 21 and 28 days of organ-culture.

Results Glucose consumption was highest during the first two weeks of organ-culture. Glucose concentrations showed a good linear correlation between medium samples and the cornea (rp0,923). The correlation coefficient for lactate was worse (rp0,733)

Conclusion Glucose and lactate levels in the organ-culture medium can be used as a marker for glucose metabolism in the cornea.

Key words Cornea - organ-culture - biochemistry - metabolism

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S. 097-103 (Art. 374)

Atypical findings in Schimmelpenning-Feuerstein-Mims-syndrome
Carsten Framme, Mary Asiyo-Vogel, Gerd-Otto Bastian

Background The Schimmelpenning-Feuerstein-Mims-syndrome is a phacomatosis of unknown pathogenesis and is presumed to be based on alterations in early embryogenesis. The syndrome is expressed by congenital nevi characterized by hyperplasia of the sebaceous glands and papillary acanthosis. Other manifestations include ophthalmic, neurologic, cardiovascular, skeletal and urogenital involvement. Eye-findings are variable and may affect all parts of the organ. All patients have a nevus sebaceus of the skin and many of them are described to suffer from seizures and/or mental retardation and/or abnormalities of the central nervous system/skull. The atypical constellation of findings in our case makes it of interest to report and give a short review.

Patient A 17-year-old boy with Schimmelpenning-Feuerstein-Mims-syndrome has been followed up by us since birth.

Results Beside an unilateral combined nevus sebaceus and verrucosus of the face, neck and skull and an aplasia of a rib there were marked ocular symptoms. The affected right eye showed a dermoid, a wide lid-opening, microcornea, stenosis of the lacrimal ducts, a high myopic fundus, the optic disc with an inverse conus and a posterior scleral staphyloma. There were no seizures or mental retardation combined with normal radiologic findings of the central nervous system and the skull.

Conclusion The presented findings in our patient with Schimmelpenning-Feuerstein-Mims-syndrome emphasized the high variability of the expression of this disease, which may lead to difficulties in establishing correct diagnosis to prevent unnecessary examinations and inadequate therapies.

Key words linear nevus sebaceus - nevus verrucosus - phacomatosis - syndrome of Schimmelpenning-Feuerstein-Mims

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S. 104-107 (Art. 372)

Anterior corneal degeneration with potentially autosomal dominant inheritance: clinical findings, pathology and differential diagnosis
Anja R Singer, Stefan Pahl, Hans-Martin Lang, Klaus W Ruprecht

Background We report on three cases of a progressive anterior corneal degeneration with clinical characteristics of limbal insufficiency occuring in three persons in three consecutive generations of one family. The disease has not yet been described in literature.

Patients A woman aged 68, her 51 years old son and his 19 years old son are affected by the disease, which suggestes autosomal dominant inheritance. None of the affected family members suffers from any form of collagenosis, endocrine insufficiency, chronic dermatosis, alteration of fat metabolism or other relevant systemic diseases. There is no accompanying disease of lids and conjunctiva except of a keratoconjunctivitis sicca with alteration of the mucine phase. The youngest affected member has a binocular anterior polar cataract.In the eldest affected member we performed penetrating keratoplasty.

Histopathology revealed thinning of corneal epithelium, moderate intra- and subepithelial granulocytary infiltration, a marked hyaline thickening of Bowman's membrane. The adjacent stroma showed accumulation of granulocytes and an increased number of capillaries and fibroblasts. Posterior stroma, Descemet's membrane and endothelium were normal. There were no goblet cells in the central epithelium.

Conclusions The appearance of the disease in three consecutive generations (strongly suggesting autosomal dominant inheritance) and the progressivity are characteristics of a dystrophical process. Development of a circular pannus and infiltration of epithelium and stroma with granulocytes however classify the disease as a degeneration, clinically and pathologically similar to Salzmann's nodular degeneration and to autosomal dominantly inherited keratitis.

Key words cornea - corneal dystrophies - degeneration - Salzmann - hereditary disease - dominantly inherited keratitis

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S. 108-111 (Art. 384)

Atypical findings in Schimmelpenning-Feuerstein-Mims-syndrome
Ulrich Schönherr

Background The Schimmelpenning-Feuerstein-Mims-syndrome is a phacomatosis of unknown pathogenesis and is presumed to be based on alterations in early embryogenesis. The syndrome is expressed by congenital nevi characterized by hyperplasia of the sebaceous glands and papillary acanthosis. Other manifestations include ophthalmic, neurologic, cardiovascular, skeletal and urogenital involvement. Eye-findings are variable and may affect all parts of the organ. All patients have a nevus sebaceus of the skin and many of them are described to suffer from seizures and/or mental retardation and/or abnormalities of the central nervous system/skull. The atypical constellation of findings in our case makes it of interest to report and give a short review.

Patient A 17-year-old boy with Schimmelpenning-Feuerstein-Mims-syndrome has been followed up by us since birth.

Results Beside an unilateral combined nevus sebaceus and verrucosus of the face, neck and skull and an aplasia of a rib there were marked ocular symptoms. The affected right eye showed a dermoid, a wide lid-opening, microcornea, stenosis of the lacrimal ducts, a high myopic fundus, the optic disc with an inverse conus and a posterior scleral staphyloma. There were no seizures or mental retardation combined with normal radiologic findings of the central nervous system and the skull.

Conclusion The presented findings in our patient with Schimmelpenning-Feuerstein-Mims-syndrome emphasized the high variability of the expression of this disease, which may lead to difficulties in establishing correct diagnosis to prevent unnecessary examinations and inadequate therapies.

Key words linear nevus sebaceus - nevus verrucosus - phacomatosis - syndrome of Schimmelpenning-Feuerstein-Mims

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S. 112-116 (Art. 379)

Reversible night blindness in vitamin A deficiency due to pancreatic carcinoma
Alexander A Bialasiewicz, Ralph Wiezorrek, Gisbert Richard

Background Vitamin A deficiency is a leading cause of blindness in childhood in the developing countries. In developed countries xerophthalmia occures mainly as secundary hypovitaminosis due to malabsorption in chronic alcoholism or intestinal disorders.

Patient and Methods A 24-year-old female patient complained of night blindness since one week. There was a history of pancreas tail resection combined with radiation due to a papillary low grade cystic carcinoma at the age of 15. During a review period of 9 years of follow-up there was a slow disseminated metastatic spread including the liver. Despite a progressive tumor cachexia the patient was practising a profession. Visual acuity was 20/20 OU with normal visual fields. Dark adaptation was reduced to one log unit and scotopic electroretinography was extinguished. Serum vitamin A level was reduced to 75 ng/ml (normal range 400-700 ng/ml).

Results Under vitamin A substitution dark adaption and scotopic electroretinography normalized and night blindness disappeared within 7 days.

Conclusions In malabsorption syndromes, especially in combination with liver manifestations, xerophthalmia should be considered as a potential complication. Vitamin A substitution should be administered if necessary. In cases of malignant tumors differential diagnosis includes the cancer associated retinopathy (CAR).

Key words Vitamin A - vitamin A deficiency - pancreatic carcinoma - night blindness - xerophthalmia

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S. 117-120 (Art. 378)

Conjunctival cysts of the orbit: clinical features and histopathology of four cases
Torsten Schlote, Gunda Nagel, Pia V Vecsei, Volker Herzau, Jens M Rohrbach

Background Only a few reports concerning congenital conjunctival cysts of the orbit (primary nonkeratinized epithelial cysts) have been published.

Patients We describe four further cases of conjunctival cysts of the orbit, which were observed between 1995 and 1997 in Tübingen and Vienna. Histologically, all cysts were lined by nonkeratinizing epithelium with goblet cells. Epidermal appendeages were found in the wall of one cyst. The patients' age was between 1 and 32 years. Clinically, the prominent symptom was swelling of the upper eyelid, persisting in three patients for more than several years. In one patients diplopia and restriction of eye movements developed within several weeks.

Conclusions The cases we described are in accordance with the cases reported in the literature. Often a swelling of the upper eyelid persisted for several years, before the cyst has become symptomatic predominantly in an adult patient. In contrast to dermoid or epidermoid cysts, there is no bony erosion and the cysts are located in the superior to nasal orbit. These cysts are probably more common as it is represented in the literature.

Key words Conjunctival cyst - orbit

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S. 121-122 (Art. 352 DF)

Acute preretinal hemorrhage as presenting clinical sign of eventually life-threatening subarachnoid hemorrhage
Wido M Budde, Anselm Jünemann

Background To report on a patient with acute preretinal and intraretinal bleedings as first clinical signs of a life-threatening subarachnoid hemorrhage.

Patient A 36-year-old, so far healthy woman complained of decreased vision, which had started eight hours before. She presented herself in good general condition. Ophthalmoscopic evaluation showed intraretinal hemorrhages in both eyes and a preretinal hemorrhage in the left eye. During the examination, the patient fainted and suffered generalized seizure. Cranial computed tomography revealed a subarachnoid hemorrhage originating from an internal carotid artery aneurysm.

Conclusion Acute intraretinal and preretinal bleedings in apparently healthy patients can be precursors of eventually life-threatening complications of subarachnoid hemorrhages.

Key words subarachnoid hemorrhage - ocular hemorrhage

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S. 123-124 (Art. 370)

Observation on pH-values of commercially available artificial tears
Susanne Pitz, Manfred Haber, Norbert Pfeiffer

Background The pH-values of artificial tears have not been examined up to now.

Materials and methods The pH-values of 20 commercially available artificial tears were determined.

Results pH-values measured from 4.82 to 8.39. One sixth showed pH-values above physiological values.

Conclusion A remarkable proportion of commercially available artificial tears deviate from the physiological range of the pH. It does not seem advisable to apply those in eyes with damaged anterior segments.

Key words tear fluid - artificial tears - pH

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