Gerold Kolling

Final Report of the Early vs. Late Infantile Strabismus Surgery Study (ELISSS), a Controlled, Prospective, Multicenter Study

Background: The optimal age for surgery for infantile esotropia is controversial. Proponents of early surgery believe that further loss of binocular vision can be prevented by early surgery, a minority believes that binocular vision can even be restored by early surgery. The ELISSS compared early with late surgery in a prospective, controlled, non-randomized, multicenter trial. Methods: Fifty-eight clinics recruited children aged 6–18 months for the study. Each clinic operated all eligible children either ‘early’, i.e. at age 6–24 months, or ‘late’, i.e. at age 32–60 months. At baseline the angle of strabismus, refraction, degree of amblyopia and limitation of abduction were assessed. Intermediate examinations took place every six months. Children were evaluated at age six in the presence of independent observers. Primary endpoints were (i) level of binocular vision, (ii) manifest angle of strabismus at distance and (iii) remaining amblyopia. Secondary endpoints were number of operations, vertical strabismus, angle at near and the influence of surgical technique. Results: A total of 231 children were recruited for early and 301 for late surgery. Age at entry examination was 11.1 months (SD 3.7 months) in the early group and 10.9 (SD 3.7) months in the late group. Refraction, amblyopia and limitation of abduction were distributed equally in the early and late groups, but the angle of strabismus was slightly larger in the early group. Dropout-rates were 26.0% in the early and 22.3% in the late group. At age six, 13.5% of the early vs. 3.9% of the late group recognized the Titmus Housefly; 3.0% of the early and 3.9% of the late group had stereopsis beyond Titmus Housefly. No significant difference was found for angle of strabismus. 35.1% of the early group and 34.8% of the late group did not have an angle between 0° and 10°, the thresholds set for re-operation. For ratio of the visual acuities (remaining amblyopia) there was a small but significant advantage for the early group. There was hardly any correlation between the baseline parameters and the primary endpoints. Children scheduled for early surgery had first been operated at 20 (SD 8.4) months, but 8.2% had not been operated at age six. Children scheduled for late surgery had been operated at 49.1 (SD 12.7) months, but 20.1% had not been operated at age six. The number of operations per child was 1.18 (SD 0.67) in the early and 0.99 (SD 0.64) in the late group. Age at recruitment, age that strabismus reportedly had started and refraction at entry examination were similar among operated and non-operated children. Only the angle of strabismus at entry predicted, to some extent, whether a child had been operated at age six. Discussion: Children operated early had better gross stereopsis at age six as compared to children operated late. They had been operated more frequently, however, and a substantial number of children in both groups had not been operated at all.

The Diagnostic Value of Optic Disc Evaluation in Acute Elevated Intracranial Pressure

PURPOSE Ophthalmologists often are asked to evaluate the optic disc for evidence of acute increased intracranial pressure (ICP). The authors studied the incidence of papilledema in a population of patients with a documented acute increase in ICP. METHODS Included in this study were 37 patients with acute elevated ICP due to a spontaneous hemorrhage or craniocerebral trauma. In all patients, the ICP was monitored continuously. Fundus examination was performed twice daily on at least 7 consecutive days. RESULTS According to the level and duration of the ICP, the patients were divided into three groups. Group 1 included 13 patients who had a slightly elevated ICP (range, 20-30 mmHg) on at least 3 consecutive days. In this group, 3 of 13 patients demonstrated venous congestion on the fifth or sixth day. No swelling of the optic disc was seen in this group. Group 2 included seven patients with an elevated ICP, with values ranging from 30 to 70 mmHg lasting for at least 3 consecutive days. In this group, one patient had a blurred disc margin on the sixth day. Group 3 included 17 patients with shortlasting ICP values, ranging from 30 to 60 mmHg for less than 72 hour. Neither papilledema nor abnormalities of fundus vessels were seen in this group. CONCLUSION Papilledema in acute elevation of ICP is an uncommon event. Its absence does not preclude the presence of ICP elevation.

Bilateral Tonic Pupils with Evidence of Anti-Hu Antibodies as a Paraneoplastic Manifestation of Small Cell Lung Cancer

Introduction: Ophthalmological manifestations of systemic malignancies can be either direct, metastatic or paraneoplastic. The latter are remote effects of carcinoma, often caused by autoantibodies. Ophthalmological manifestations include cancer-associated retinopathy, melanoma-associated retinopathy, opsoclonus-myoclonus syndrome or motility disorders due to effects on the neurological system. A unilateral tonic pupil is usually a benign finding but has also been described in the context of paraneoplastic syndromes, in some cases associated with anti-Hu antibodies. Case Reports: The authors describe 2 patients with bilateral symptomatic tonic pupils due to a paraneoplastic syndrome. Both patients had been treated for small cell lung cancer and had evidence of anti-Hu antibodies (autoantibodies against nuclei of neural cells) in serum and cerebrospinal fluid. Both had typical pupillary findings and hypersensitivity to diluted pilocarpine. The first patient also had sensory neuronopathy, the second affection of several cranial nerves. Discussion: To the best of our knowledge, to date no case of bilateral tonic pupils has been published due to a paraneoplastic disorder with evidence of autoantibodies. This is surprising, as it is probable that autoantibodies in paraneoplastic disorders affect both ciliary ganglions in a similar way.

The relevance of stereopsis for motorists: a pilot study

Abstract. Purpose: To study the influence of stereoscopic depth perception on automobile driving performance. Methods: Ten patients with strabismus and defective stereopsis were compared with ten healthy controls with respect to their performance in a series of automobile driving manoeuvres. The two groups were individually matched as to age, annual miles driven, years of licence holding and type of vehicle owned. After an ophthalmologic examination the subjects in each group performed the following series of driving tests: (1) stopping in front of an obstacle, (2) reversing into a parking space, (3) driving through a slalom course, (4) estimating the relative positions of two cars. All tests were performed binocularly and monocularly (with the non-dominant eye covered). Results: Only in the slalom test did the normal subjects perform significantly better than the stereo-deficient subjects (odds ratio 10.5; P<0.01). In estimating position, normal subjects actually performed significantly worse (odds ratio 0.091; P<0.01). A significant distance ratio of 2.5 (95% CI 1.1–5.5; P=0.033) of the monocular with respect to the binocular performance of the normal subjects was found for the stopping task only, while the subjects with defective stereopsis showed no difference between their monocular and binocular performance. Conclusion: In this study, stereopsis had a positive effect on driving performance only in dynamic situations at intermediate distances.

Immune-mediated retinopathy in a patient with stiff-man syndrome

Abstract · Background: Stiff-man syndrome is a rare neurological disorder characterised by rigidity and violent spasms of the body musculature. In the majority of patients, presence of antibodies against glutamic acid decarboxylase (GAD), the enzyme synthesizing γ-aminobutyric acid (GABA), suggests an autoimmune attack against GABA-ergic inhibitory neurons. We report a 32-year-old patient with stiff-man syndrome and anti-GAD antibodies who developed subacute progressive loss of vision in the right eye, and in the left eye 18 months thereafter. · Methods: Ophthalmological work-up included electro-retinogram (ERG), visual evoked potentials (VEP) and fluorescein angiography. Antiretinal antibodies were investigated using an indirect immunofluorescence technique on frozen sections of macaque retina with patient´s serum and FITC-conjugated goat antihuman immunoglobulin. Staining with monoclonal anti-GAD65 antibodies and with serum from three healthy normals served as controls. · Results: Visual acuity of both eyes decreased to 0.16 within a span of 6 weeks. Perimetry revealed a central scotoma in the visual field of both eyes. VEP and flash ERG were progressively disturbed on the right eye. On the left eye, initially only pattern ERG and photopic responses were abnormal. Follow-up recordings revealed widespread pathology of photopic single and flicker responses. Immunofluorescence revealed strong reactivity of the inner plexiform layer and to a lesser extent staining of the outer plexiform layer at dilutions of 1:1000 with patients serum. The same retinal staining pattern was obtained with monoclonal anti-GAD65 antibodies. · Conclusions: These findings suggest autoimmune retinopathy, mediated by anti-GAD65 autoantibodies as the underlying cause of visual loss.

Absence of the fourth cranial nerve in congenital Brown syndrome

Purpose:  To elucidate the aetiology of congenital Brown syndrome.

The Early vs Late Infantile Strabismus Surgery Study: do sources for bias exist in this non-randomised trial?

BACKGROUND The Early vs Late Infantile Strabismus Surgery Study Group investigates whether early or late surgery is preferable in infantile convergent strabismus, in a non-randomised, prospective, multicentre clinical trial. The current state of the study after end of recruitment is reported here, focusing on the question of possible sources for bias in this non-randomised trial. METHOD The prognostic factors were analysed at baseline in order to check for imbalances between the two treatment groups. Reasons for possible differences are discussed. RESULTS There is no evidence for clinically relevant inhomogeneities between the two groups concerning the distribution of the three prognostic factors spherical equivalent, degree of amblyopia, and limitation of abduction. The fourth prognostic factor, horizontal angle of squint, differs significantly between the two groups. CONCLUSION In the analysis of the final results we may have to account for differences in angle of squint at baseline by its inclusion as a covariate or by stratification.

Proposed scoring system for assessment of functional impairment due to visual field defects

INTRODUCTION The purpose of the study is the development of a scoring system for the assessment of functional impairment due to visual field defects, based on the Esterman grid, but adapted to the existing system in the Federal Republic of Germany. METHODS The score areas and the evaluation rules were altered iteratively, until the functional score by the new system matched as much as possible the score obtained from the existing system of geometrical defect patterns. RESULTS The visual field is divided into 100 score areas, which are smaller in the visual field center and in its lower region, respectively. The areas inside the reference isopter are counted and taken as total function in percent. Areas with monocular perception are rated with 3/4 points. Functional impairment is defined as the difference of total function to 100%. For 9 of 20 defect patterns, the functional scores are equal. Another six patterns differ by 1-4%. The remaining 5 of 20 patterns differ by 6-8% in either direction. CONCLUSION The new system is easier to apply, more consistent, and covers irregular defect patterns. The level of functional impairment is nearly identical with both systems. A transition to the scoring system would considerably increase the certitude and the quality of assessment especially in difficult cases.ZusammenfassungEinführungFür die Begutachtung von Gesichtsfeldausfällen in der Bundesrepublik Deutschland soll ein System entwickelt werden, das auf einer Flächenbewertung ähnlich dem Esterman-Gitter beruht.MethodikIterativ wurden Wertungsflächen und -regeln verändert, bis die Funktionsminderung nach dem neuen System dem derzeit gültigen mit geometrischen Defektmustern möglichst nahe kam.ErgebnisseDas Gesichtsfeld wird in 100 Wertungsflächen eingeteilt, die in der Mitte und auch in der unteren Hälfte deutlich dichter liegen. Die von der Referenzisoptere eingeschlossenen Flächen werden gezählt und als Gesamtfunktion in Prozent gewertet. Monokular gesehene Flächen werden dabei zu 3/4 gewertet. Die Differenz der Gesamtfunktion zu 100% ist die Funktionsminderung. Für 9 von 20 Defektmustern ergibt sich keine Änderung in der Prozentzahl der Funktionsminderung. Bei weiteren 6 Mustern beträgt die Abweichung 1% bis 4%, Bei den restlichen 5 von 20 Mustern beträgt die Abweichung 6 bis 8% nach oben oder unten.SchlussfolgerungDas neue System ist einfacher in der Anwendung, konsistenter und berücksichtigt auch unregelmäßige Defektmuster. Ein Übergang auf das Punktesystem würde die Sicherheit und die Qualität der Begutachtung insbesondere bei schwierigen Fällen deutlich erhöhen.AbstractIntroductionThe purpose of the study is the development of a scoring system for the assessment of functional impairment due to visual field defects, based on the Esterman grid, but adapted to the existing system in the Federal Republic of Germany.MethodsThe score areas and the evaluation rules were altered iteratively, until the functional score by the new system matched as much as possible the score obtained from the existing system of geometrical defect patterns.ResultsThe visual field is divided into 100 score areas, which are smaller in the visual field center and in its lower region, respectively. The areas inside the reference isopter are counted and taken as total function in percent. Areas with monocular perception are rated with 3/4 points. Functional impairment is defined as the difference of total function to 100%. For 9 of 20 defect patterns, the functional scores are equal. Another six patterns differ by 1–4%. The remaining 5 of 20 patterns differ by 6–8% in either direction.ConclusionThe new system is easier to apply, more consistent, and covers irregular defect patterns. The level of functional impairment is nearly identical with both systems. A transition to the scoring system would considerably increase the certitude and the quality of assessment especially in difficult cases.

Diagnostic occlusion test in cases of unilateral strabismus sursoadductorius

PURPOSE To investigate the diagnostic occlusion test (OT) according to Marlow in strabismus sursoadductorius (StS; also known as ‘unilateral superior oblique palsy’) and its implications for surgical treatment. METHODS In 67 cases of StS, angles of strabismus were measured before and after patching the affected eye for three days. Horizontal, vertical and torsional deviations were measured in front of the tangent screen of Harms in nine directions of gaze. RESULTS After OT, most cases of StS showed the following distribution of vertical deviation (VD) on horizontal versions: setting the VD in 30° adduction to 100%, VD in primary position (PP) was 50%, VD in abduction 14%, excyclotropia in PP about 40% of the amount of VD. Those StS-cases showing the same distribution of VD on horizontal versions before and after OT were called ‘typical’ ones (27% of the patients). However, 73% of the patients showed a different distribution before OT: 68% VD in PP, 36% VD in abduction, excyclotropia of 20% in PP. After OT these cases converted to the normal pattern described above: VD in PP was 53% and VD in 30° abduction was 19%, excyclotropia in PP was 36%. CONCLUSION In ‘typical’ StS-cases, OT is not necessary. ‘Atypical’ cases, however, can be converted into ‘typical’ ones: they get more incomitant VD on horizontal versions and greater excyclotropia in PP. As VD in adduction remains almost the same in both groups after OT, surgery can be based on the angle in adduction. In individual ‘atypical’ patients the amount of oblique muscle surgery has to be modified after OT.

Qualitätsanforderungen an die Untersuchung des Farbsinns

Assessing colour vision comprises a wide spectrum of methods, many of which are practical and highly informative. Given this methodological variety this review aims to help select the most appropriate test and how to correctly execute it, thus achieving the highest quality.Some aspects of the physiology of colour vision are covered as far as is necessary for a basic understanding of colour testing methodology and possible pitfalls. For congenital colour anomalies most pertinent are questions of occupational aptitude. For acquired colour deficiencies assessing colour vision supplements diagnostics of the retina and the visual pathway, allowing both early diagnosis and/or monitoring. For both these fields colour tests provide different kinds of evidence and need to be adequately selected. Methodical artefacts due to both equipment design properties and testing procedures are highlighted so they can be avoided. A form is presented for recording colour examination results commensurate with quality objectives. Finally, a tabular overview of 19 common colour vision tests is provided.ZusammenfassungDie Untersuchung des Farbensehens in der Praxis umfasst ein breites Spektrum handlicher und diagnostisch informativer Methoden. In dieser Vielfalt kommt es auf die zielführende Auswahl und richtige Handhabung der einzelnen Verfahren an. In dieser Übersicht soll die sinnvolle Anwendung der Methoden und die Vermeidung typischer Fehler aufgezeigt werden, um die Einhaltung von Qualitätsstandards der guten klinischen Praxis zu erleichtern.Einführend werden einige Aspekte der Physiologie des Farbensehens angesprochen, die für das Verständnis der Untersuchungsmethoden, ihrer Möglichkeiten und ihrer Fehler wichtig sind. Bei angeborenen Störungen wird die Eignung für Berufe und Tätigkeiten geprüft, welche Anforderungen an das Farbensehen stellen. Bei erworbenen Störungen ergänzt die Untersuchung des Farbensehens die Diagnostik von Erkrankungen der Netzhaut, der Sehbahn sowie des Kortex und bietet Möglichkeiten zur Früherkennung und zu Verlaufskontrollen. Bei angeborenen und erworbenen Farbsinnstörungen liefern Farbtests unterschiedliche Befunde und müssen differenziert eingesetzt und beurteilt werden, ein breites Methodenspektrum wird diesbezüglich dargestellt. Fehlerquellen infolge technischer Merkmale der Apparaturen und solchen beim Ablauf der Untersuchungen werden erläutert. Als Vorlage für zuverlässige Dokumentation wird ein Musterformular angegeben. Eine Tabelle mit vielen derzeit in Deutschland kommerziell erhältlichen Geräten und Tests schließt die Darstellung ab.AbstractAssessing colour vision comprises a wide spectrum of methods, many of which are practical and highly informative. Given this methodological variety this review aims to help select the most appropriate test and how to correctly execute it, thus achieving the highest quality.Some aspects of the physiology of colour vision are covered as far as is necessary for a basic understanding of colour testing methodology and possible pitfalls. For congenital colour anomalies most pertinent are questions of occupational aptitude. For acquired colour deficiencies assessing colour vision supplements diagnostics of the retina and the visual pathway, allowing both early diagnosis and/or monitoring. For both these fields colour tests provide different kinds of evidence and need to be adequately selected. Methodical artefacts due to both equipment design properties and testing procedures are highlighted so they can be avoided. A form is presented for recording colour examination results commensurate with quality objectives. Finally, a tabular overview of 19 common colour vision tests is provided.