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Beim kongenitalen Brown-Syndrom handelt es sich um eine mechanisch bedingte Einschrankung der Hebung in Adduktion mit Parallelstand im Abblick. Nachdem als Ursache 1975 von Parks et al. eine von Brown angenommene Verkurzung der Obliquus-superior-Sehnenscheide ausgeschlossen wurde, wird uber diese spekuliert. Es wird dabei hauptsachlich eine Fehlinnervation diskutiert. Doch widerspricht v. a. die vollkommen normale Senkung in Adduktion dieser Annahme. Eine belegbare Ursache ist dagegen beim typischen Brown-Syndrom ein fibrotischer Strang. Hierbei handelt es sich – aufgrund der von Fink beschriebenen entwicklungsgeschichtlichen Befunde – mit hoher Wahrscheinlichkeit um einen atavistischen Obliquus superior. Der Strang hat – wie auch noch bei vielen Tieren der M. obliquus superior – seinen Ursprung in Trochleanahe an der Orbitawand. Er verlauft von dort am Hinterrand der Obliquus-superior-Sehne in diese integriert zum gemeinsamen Ansatz am Bulbus, wo er etwas hinter dem Aquator inseriert. Bei 2 Fallen mit atypischem Brown-Syndrom konnte ebenfalls als Ursache ein fibrotischer Strang gefunden werden. Die intraoperativ vorgefundene Lage der Insertion des Strangs befand sich jedoch bei dem ersten Fall vor dem Aquator, was dort die – den True-atypical-Fallen von Brown entsprechende Hebungseinschrankung auch in Abduktion erklarte. Beim zweiten Fall mit einer bisher noch nicht beschriebenen Variante, bei der der Strang von der Obliquus-superior-Sehne separiert verlief, inserierte der Strang weit hinter dem Aquator nasal des M. rectus superior, was bei diesem eine sprungartig bei uber 30° Adduktion aufgetretene vollstandige Hebungseinschrankung und das Y‑Phanomen im Aufblick mit einer bei Adduktion zunehmenden und bei Abduktion abnehmenden Exotropie erklarte. Als Therapie hat sich am besten die Teilexzision des fibrotischen Strangs von ca. 10 mm vom Ansatz aus am besten bewahrt. Die danach noch vorhandene Einschrankung der aktiven Hebung in Adduktion bildet sich bei postoperativ praktizierten Blickbewegungen nach Adduktion und oben meistens nach uber 1 Jahr zuruck. Ein fibrotischer Strang (atavistischer Obliquus superior) kann als Ursache fur das kongenitale Brown-Syndrom angesehen werden. Die unterschiedlichen Arten der Hebungseinschrankung lassen sich uber die Variabilitat der Insertion des Strangs erklaren. Die dabei im Einzelfall vorliegende Motilitatsstorung kann uber eine Teilexzision des Strangs vom Ansatz aus behoben werden – wenn auch die Normalisierung der aktiven Hebung meist uber 1 Jahr dauert.

Die Ablatio retinae nimmt aufgrund der ernsthaften Visusbedrohung fur Kinder und Jugendliche im Hinblick auf die noch lange Lebenserwartung eine Sonderstellung ein. In dieser Studie sollen charakteristische Befundmerkmale der Ablatio retinae im Kindes- und Jugendalter bis zum 20. Lebensjahr dargelegt werden. Es wurde ein Kollektiv von 259 Patienten ausgewahlt, die ein- oder beidseitig an Ablatio retinae erkrankt waren, zum Zeitpunkt der erstmaligen Diagnosestellung am zuerst bzw. einzig erkrankten Auge nicht alter als 20 Jahre waren und wenigstens 1‑mal in einem Zeitraum von 18 Jahren (1980 bis 1998) an der Augenklinik der Ludwig-Maximilians-Universitat Munchen operativ versorgt wurden. Dieses Patientenkollektiv wurde retrospektiv hinsichtlich der klinischen Befundmerkmale der erstmals diagnostizierten Ablatio retinae untersucht. Es wurde die Gruppe der zuerst bzw. einzig erkrankten Augen (259 Augen) betrachtet, die im Verlauf ebenfalls an Ablatio retinae erkrankten Partneraugen wurden hier ausgeschlossen (19 Augen). Die Zeitspanne zwischen dem Auftreten visueller Symptome und Diagnosestellung der Ablatio retinae betrug im Durchschnitt 9,6 Wochen, als haufigste Manifestationssymptomatik wurde von 36,3 % der Patienten ein Visusabfall angegeben. Bei 40,2 % der Patienten wurde die Netzhautablosung zufallig diagnostiziert. Am haufigsten (34,0 %) erstreckte sich die Ablatio uber 2 Quadranten, bei 27,0 % der Augen war sie (sub)total. Die Makula war bei 154 Augen (59,5 %) abgehoben. Ein oranaher Einriss trat mit 36,1 % als haufigste Foramenform auf. Riesenrisse (12,8 % aller Foramina) traten bevorzugt im oranahen Bereich auf, degenerative Rundforamen fanden sich insbesondere im Aquatorbereich und traten gehauft als Lochkette auf. Am haufigsten waren Foramen im temporal unteren Quadranten lokalisiert. Bei 22,4 % der Ablationes konnte – auch intraoperativ – kein Foramen aufgefunden werden. Eine primare PVR-Reaktion mindestens Stadium C fand sich bei 25,5 % der Ablationes. Im Kindes- und Jugendalter ermoglicht die charakteristisch verzogerte Diagnosestellung eine grosflachige Ausdehnung der Ablatio retinae mit haufiger Makulabeteiligung, hohem Anteil an (sub)totaler Ausdehnung und florider primarer PVR. Oranahe Einrisse jeglicher Art, Riesenrisse, multiple degenerative Rundforamen im Aquatorbereich sowie ein hoher Anteil nicht auffindbarer Foramen sind wesentliche Merkmale des juvenilen Ablatiobefundes.

ZusammenfassungDie Abklärung unklarer Sehstörungen ist ein schwieriger Prozess. Dem Swinging-Flashlight-Test kommt aufgrund fehlender Invasivität, schneller Durchführbarkeit und seinem großen differenzialdiagnostischen Wert eine Schlüsselrolle bei der Abklärung unklarer Sehstörungen zu – vorausgesetzt er wird korrekt durchgeführt.

The congenital Brown syndrome is characterized by a mechanical limitation of elevation in adduction, with an orthophoria in down gaze. Brown postulated a shortened superior oblique tendon sheath as the cause of the limitation but this was disproved by Parks et al. in 1975 and the origin of Brown syndrome remains unclear. In recent years, a congenital dysinnervation has been discussed; however, this does not explain the full spectrum of abnormalities and especially contradicts the unlimited depression in adduction seen in Brown syndrome.Surgical exploration in Brown true typical cases reveals a fibrotic strand, typically located at the posterior margin of the superior oblique tendon. This strand originates from the trochlear area and has a common insertion with the superior oblique tendon posterior to the equator into the globe. It may represent an atavistic superior oblique muscle as described by Fink in various animals. They do not have a trochlea but a superior oblique muscle originating in the anterior superior nasal orbit.A fibrotic strand was also surgically revealed in two cases of atypical Brown syndrome. In the first case an elevation deficit-as in Brown true atypical cases-also present in abduction could be explained by an unusual insertion of the fibrotic strand anterior to the equator. The second case showed a fibrotic strand which was completely separated from the superior oblique tendon and inserted far posterior to the equator nasal to the superior rectus muscle. This finding had not been previously described and explained the total elevation restriction which was suddenly in 30° adduction and the Y‑pattern exotropia which increased in adduction and decreased in abduction.A 10 mm excision of the fibrotic strand from the insertion gives the best results from all procedures. The residual limitation of active elevation in adduction improved with gaze exercises mostly after more than 1 year.The fibrotic strand, an atavistic superior oblique muscle, not only explains the typical Brown syndrome but also-by its variable insertion-different patterns of elevation deficits seen in atypical Brown syndrome. A 10 mm excision of the strand gives good functional results of abnormal head position (immediate in most cases) and even elevation in adduction (after 1 year in most cases).

Palisade endings are located at the myotendinous junction of extraocular muscles in most mammals. Irrespective of their unclarified function as motor or sensory nerve endings, a specialized role in convergence is proposed, based on their high number in the medial rectus muscle (MR). Further support comes from a study in monkey demonstrating that only the MR and inferior rectus muscle (IR) contain an additional population of palisade endings that express the calcium-binding protein calretinin (CR) in addition to choline acetyltransferase (ChAT). Here we studied, whether CR-positive palisade endings are present in human as well and confined to extraocular muscles most active during convergence. The systematic analysis of all eye muscles of 17 human specimen revealed that only the MR and IR contain an additional population of CR-positive palisade endings and multiple en-grappe endings, which target non-twitch muscle fibers along their whole length. Approximately 80% of all palisade endings in the MR expressed CR. Furthermore, the intrafusal muscle fibers of some muscle spindles in the MR were innervated by CR-positive annulospiral nerve endings that transmit the signals of muscle length changes to the brain. All extraocular muscles contained few thin CR-positive, but ChAT-negative nerve fibers, possibly representing free sensory or autonomic endings arising from the trigeminal ganglion. As in monkey, in the medial periphery of the human oculomotor nucleus ChAT-positive neurons were found to co-express CR. Therefore these neurons most likely represent the cell bodies of CR-positive palisade endings in the MR. Unlike in monkey, these neurons do not lie within a compact cell group, but are more scattered. In conclusion, the MR and IR in human contain two histochemically different populations of palisade and multiple endings that may contribute to ocular alignment and convergence in a different way.

Palisade endings are located at the myotendinous junction of extraocular muscles in most mammals. Irrespective of their unclarified function as motor or sensory nerve endings, a specialized role in convergence is proposed, based on their high number in the medial rectus muscle (MR). Further support comes from a study in monkey demonstrating that only the MR and inferior rectus muscle (IR) contain an additional population of palisade endings that express the calcium-binding protein calretinin (CR) in addition to choline acetyltransferase (ChAT). Here we studied, whether CR-positive palisade endings are present in human as well and confined to extraocular muscles most active during convergence. The systematic analysis of all eye muscles of 17 human specimen revealed that only the MR and IR contain an additional population of CR-positive palisade endings and multiple en-grappe endings, which target non-twitch muscle fibers along their whole length. Approximately 80% of all palisade endings in the MR expressed CR. Furthermore, the intrafusal muscle fibers of some muscle spindles in the MR were innervated by CR-positive annulospiral nerve endings that transmit the signals of muscle length changes to the brain. All extraocular muscles contained few thin CR-positive, but ChAT-negative nerve fibers, possibly representing free sensory or autonomic endings arising from the trigeminal ganglion. As in monkey, in the medial periphery of the human oculomotor nucleus ChAT-positive neurons were found to co-express CR. Therefore these neurons most likely represent the cell bodies of CR-positive palisade endings in the MR. Unlike in monkey, these neurons do not lie within a compact cell group, but are more scattered. In conclusion, the MR and IR in human contain two histochemically different populations of palisade and multiple endings that may contribute to ocular alignment and convergence in a different way.

Background Idiopathic intracranial hypertension (IIH) in children is a rare condition of unknown etiology and various clinical presentations. The primary aim of this study was to evaluate if our pediatric IIH study group fulfilled the revised diagnostic criteria for IIH published in 2013, particularly with regard to clinical presentation and threshold value of an elevated lumbar puncture opening pressure. Additionally we investigated the potential utilization of MR-based and fundoscopic methods of estimating intracranial pressure for improved diagnosis. Patients and methods Clinical data were collected retrospectively from twelve pediatric patients diagnosed with IIH between 2008 and 2012 and revised diagnostic criteria were applied. Comparison with non-invasive methods for measuring intracranial pressure, MRI-based measurement (MR-ICP) and venous ophthalmodynamometry was performed. Results Only four of the twelve children (33%) fulfilled the revised diagnostic criteria for a definite diagnosis of IIH. Regarding noninvasive methods, MR-ICP (n = 6) showed a significantly higher mean of intracranial pressure compared to a healthy age- and sex-matched control group (p = 0.0043). Venous ophthalmodynamometry (n = 4) showed comparable results to invasive lumbar puncture. Conclusion The revised diagnostic criteria for IIH may be too strict especially in children without papilledema. MR-ICP and venous ophthalmodynamometry are promising complementary procedures for monitoring disease progression and response to treatment.

Nystagmus is an involuntary, periodic eye movement caused by a slow drift of fixation which is followed by a fast refixation saccade (jerk nystagmus) or a slow movement back to fixation (pendular nystagmus). In childhood most cases are benign forms of nystagmus: idiopathic infantile, ocular or latent nystagmus. They arise at the age of 3 months, without oscillopsia and show the absence of the physiologic opto-kinetic nystagmus. A full ophthalmologic evaluation is all that is needed in most cases: albinism, macular or optic nerve hypoplasia and congenital retinal dystrophies are the most common forms of ocular nystagmus. Idiopathic infantile nystagmus can be hereditary, the most common and best analyzed form being a mutation of the FRMD7 gene on chromosome Xq26.2. The mutation shows a mild genotype-phenotype correlation. In all female carriers the opto-kinetic nystagmus is absent and half had mild nystagmus. Latent nystagmus is part of the infantile esotropia syndrome and shows the unique feature of change of direction when the fixing eye changes: it is always beating to the side of the fixing eye. There is no cure for infantile nystagmus but therapeutic options include magnifying visual aids or eye muscle surgery at the age of 6-8 y in patients with head turn. Less than 20% of childhood nystagmus are acquired and need further neurological and imaging work-up. Alarming signs and symptoms are: onset after the age of 4 months, oscillopsia, dissociated (asymmetric) nystagmus, preserved opto-kinetic nystagmus, afferent pupillary defect, papilloedema and neurological symptoms like vertigo and nausea. The most common cause is due to pathology of the anterior optic pathway (e.g. optic nerve gliomas). It shows the same clinical feature of dissociated nystagmus as spasmus nutans but has a higher frequency as in INO. Other forms of acquired nystagmus are due to brainstem, cerebellar or metabolic diseases.

Refraktionsfehler stellen im Kindesalter neben Schielen das haufigste Sehproblem dar. Die Indikationen fur eine Brillenverordnung umfassen die Amblyopieprophylaxe und -behandlung, Schielbehandlung, Myopiekorrektur und Unterstutzung bei Leseproblemen. Der zentrale Bestandteil der Brillenverordnung bei Kindern ist die objektive Refraktion in Zykloplegie, idealerweise mit dem Skiaskop. Eine leichte Unterkorrektur (maximal 0,5 dpt, falls ein Schielen vorliegt, und 1,0 dpt bei Orthophorie) ist nur bei der Hyperopie sinnvoll. Myopie, Anisometropie und Astigmatismus mussen voll korrigiert werden. Auf jedes Brillenrezept gehoren die Vermerke „MA = PD“ und „Kunststoffglaser“ sowie „Bifokalteil bis Pupillenmitte“, falls erforderlich. Der Aufklarung der Eltern kommt eine wichtige Rolle zu. Auf Tipps bei den einzelnen Schritten der Brillenanpassung und mogliche Fehlerquellen wird besonders eingegangen.

Ziel ist es, die im SLO-mfERG objektiv nachweisbaren retinalen Funktionsminderungen mit den in der Mikroperimetrie unter Anwendung unterschiedlicher Stimulusintensitaten messbaren Skotome in Vergleich zu setzen. Bei 4 Patienten mit Morbus Stargardt wurden ein SLO-mfERG und eine SLO-Mikroperimetrie durchgefuhrt. Das SLO-mfERG wurde in einem Messfeld von 24° Durchmesser am hinteren Augenpol unter simultaner Fixationskontrolle abgeleitet. Die Stimulation erfolgte mit einem Helium-Neon-Laser, die Darstellung des Augenhintergrundes mit einem Infrarotlaser. Die SLO-Mikroperimetrie wurde mit einem Stimulus, dessen Winkelgrose dem Goldmann-III-Stimulus entspricht, bei Intensitaten von 0 dB, 12 dB und 20 dB durchgefuhrt. Es wurde die Amplitudenreduktion im SLO-mfERG mit der Skotomgrose bei verschiedenen Stimulusintensitaten in der SLO-Mikroperimetrie korreliert. Die in der SLO-mfERG Untersuchung topographisch zuordenbaren Funktionsminderungen zeigten eine enge Ubereinstimmung mit der Grose der Skotome in der SLO-Mikroperimetrie bei Anwendung der Kombination Prufmarke Goldmann III/20dB. Sowohl das SLO-mfERG als auch die SLO-Mikroperimetrie stellen eine sensitive Untersuchungstechnik zur Quantifizierung retinaler Funktionsdefizite dar und eignen sich zur Durchfuhrung einer detaillierten Netzhautfunktionsdiagnostik.

For all in-patients a fundamental change of hospital financing based only on diagnosis related groups (DRGs) is planned in Germany for 2004. Patients are grouped into different DRGs according to their coded diagnoses, operations and procedures. Hence, the quality of coding is essential for adequate reimbursement of cases and thus for hospital financing. The legal framework of coding is supplied by the so-called "general and special coding guidelines for coding diseases and procedures", presented jointly by the associations representing hospitals and public and private health insurance companies in early 2002. These German coding standards regulate how coding must be performed in daily practice and therefore have a major influence on the grouping of patients into DRGs. Because of their importance this paper describes the practical application of current coding standards. Specifically in Ophthalmology the problem exists that for many procedures adequate coding numbers are still missing, thus impairing further development of the DRG system.

Hintergrund. Ziel der Untersuchung ist es, Kosten und Nutzen einer elektronischen Krankenakte denen der traditionellen Papierversion gegenuberzustellen. Methoden. Am Beispiel einer Universitats-Augenklinik mit 140 Betten werden theoretische Kosten und Nutzen im Sinne von vermiedenen direkten Kosten bei der geplanten Einfuhrung einer elektronischen Krankenakte verglichen. Ergebnisse. Im vorgestellten Beispiel betragt der gut quantifizierbare, zusatzliche Nutzen einer elektronischen Krankenakte 192.000 DM pro Jahr. Dem stehen Kosten von jahrlich 234.000 DM bei 4 Jahren Abschreibung der erforderlichen Zusatzausstattung gegenuber. Somit treten durch eine elektronische Patientenakte im Vergleich zur Krankenakte in Papierform jahrlich 42.000 DM zusatzliche Gesamtkosten auf. In der Analyse verschiedener Szenarios ergibt sich bei Erhohung der Abschreibungsfrist auf 6 Jahre ein Fehlbetrag von lediglich 8.667 DM. Bei hoheren Lohnkosten reduziert sich der Fehlbetrag weiter, beim Wegfall von einzelnen Nutzenfunktionen der elektronischen Krankenakte erhoht er sich. Auf der Nutzenseite stehen einige nicht direkt quantifizierbare Nutzen fur Forschung, Lehre und Qualitatssicherung sowie fur einen zuverlassigeren Datenzugriff. Diese durften das Nutzen-Kosten-Verhaltnis groser eins werden lassen. Schlussfolgerungen. Nur ein Teil der Vorteile einer elektronischen Patientenakte ist gut quantifizierbar in Form von eingesparten Kosten. Die im dargestellten Beispiel verbleibenden Mehrkosten einer elektronischen Patientenakte werden durch eine Reihe von zusatzlichen Nutzeffekten mehr als aufgewogen. Aufgrund von Schwierigkeiten bei der Quantifizierung konnen diese jedoch nur qualitativ aufgefuhrt werden.

BACKGROUND The aim of our study was a detailed morphological and functional assessment of macular foramen stages II-IV by scanning laser ophthalmoscopy (SLO) and fundus perimetry including a correlation with clinical findings. METHODS AND MATERIALS Included in this prospective study were 78 patients where SLO imaging and fundus perimetry were performed with the SLO-105 (Rodenstock) prior to pars plana vitrectomy for macular foramen on one eye. Both the size and shape the of macular foramen were assessed. Deep (0 dB) and relative (12 dB) scotomata were tested using the Goldmann II stimulus in the 20 degrees field of the SLO. Imaging was performed with the infrared laser and confocal aperture C2. RESULTS The size of macular foramen ranged from 140 microns to 800 microns (median: 420 microns) and 20 (26%) had irregular, polycyclic borders. In 21 patients (27%) a bright sector could be seen just outside the hole. Deep scotomata were notably smaller than the visible macular hole in 4 patients (6%) and 18 deep scotomata (25%) extended beyond the hole. There was a weak inverse correlation between the size of deep, relative scotomata and the diameter of the macular holes with the visual acuity (r = -0.46; r = -0.39 or r = -0.53), but no correlation with the duration of symptoms could be demonstrated. CONCLUSION Polycyclic borders were seen as a new feature of some idiopathic macular foramen. This may be due to asymmetric traction or adhesions on the macular area. Also, deep scotomata either smaller or larger than the visible macular hole were found.

PURPOSE: To report the incidence of macular changes following pars plana vitrectomy with peeling of the internal limiting membrane (ILM) for idiopathic macular hole. DESIGN: Prospective consecutive series. METHODS: In a prospective study 105 eyes of 105 patients underwent vitrectomy for idiopathic macular holes. Surgery consisted of a standard three-port vitrectomy, induction of a posterior hyaloid detachment, removal of epiretinal membranes including the ILM, fluid-air exchange and intraocular gas tamponade (15% hexafluoroethane (C2F6) gas mixture) followed by head-down positioning for at least five days. No adjuvants were used during surgery. In addition to the clinical examination, static microperimetry using a Rodenstock scanning laser ophthalmoscope (SLO-105) was performed pre- and 6 or 12 weeks postoperatively. The stimulus size was 0.2 degrees (Goldmann II), intensities employed were 0 and 12 dB. For all tests, 20-degree fields were used. RESULTS: Anatomic closure of macular holes was achieved in 92 (87.6%) of 105 patients by one operation. Eight patients underwent a successful second procedure. The closure rate after two operations was 95.2%. Best corrected visual acuity increased from a median of 0.2 (range 0.05 to 0.5) preoperatively to a median of 0.5 (range 0.05 to 1.0) postoperatively. Anatomical macular changes were found in 8 (7.6%) patients: There were two cases of macular edema following secondary cataract extraction and six cases of retinal pigment epithelium changes. Formation of postoperative epiretinal membranes or late reopenings were not noted. Small, mostly asymptomatic paracentral scotomata were seen in 59 (56.2%) of 105 patients. CONCLUSION: Anatomical changes of the macula following vitrectomy with removal of the ILM are infrequent. However, paracentral scotomata observed in our series might be caused by a trauma to the nerve fibers during ILM peeling. To achieve reliable results a standardized procedure for microperimetry should be developed.

Hintergrund: Mikroperimetrische Untersuchungen nach operativer Extraktion chorioidaler Neovaskularisationsmembranen (CNV) bei altersbedingter Makuladegeneration (AMD) haben gezeigt, das ein tiefes Skotom im Bereich des resultierenden Pigmentepithelverlustes entsteht. Bei der Laserbehandlung nach MPS-Kriterien ist ebenfalls von einem kompletten Funktionsverlust im behandelten Areal auszugehen. Der operative Aufwand ist gegen die moglichen funktionellen Ergebnisse beider Therapieformen abzuwagen. Patienten und Methode: Funf nach MPS-Kriterien gelaserte Patienten mit groser subfovealer klassischer CNV bei AMD wurden Patienten mit vergleichbarem Ausgangsbefund aus einem Kollektiv von 78 operierten Patienten gegenubergestellt. Pra- und posttherapeutisch erfolgte die Bestimmung des Visus nach ETDRS-Kriterien, des Vergroserungsbedarfes anhand der ZEISS-Tafeln sowie eine funduskontrollierte SLO-Mikroperimetrie des relativen (12 dB) und tiefen (0 dB) Skotoms. Ergebnisse: Die Rezidivrate beider Gruppen (OP 2/5; ALK 1/5) ist bei der kleinen Fallzahl ahnlich. Die Visusveranderung der operierten Patienten (Mittelwert: pra 0,08; 6 Wo 0,09; 3 Mo 0,15; zuletzt 0,13) ist geringfugig besser als die der gelaserten Patienten (pra 0,06; 6Wo 0,07; 3 Mo 0,08; zuletzt 0,12). Bei rezidivfreier chirurgischer Extraktion ist eine Verkleinerung des tiefen Skotoms (Mittelwert: 6 Wo Faktor 0,6; letzte Kontrolle Faktor 0,8) moglich. Bei der Laserbehandlung kommt es tendenziell zu einer Vergroserung des tiefen Skotoms (Mittelwert: 6Wo Faktor 2,3; letzte Kontrolle Faktor 2,2). Schlusfolgerung: Angesichts der geringfugigen funktionellen Vorteile sehen wir die CNV-Extraktion subfovealer Membranen nicht generell als Therapie der ersten Wahl an. Lediglich bei kleinen subfovealen Membranen mit kurzem zeitlichem Verlauf und bei juxtafovealer CNV konnte der geringeren Skotomgrose nach chirurgischer Extraktion eine funktionelle Bedeutung zukommen. Unabhangig von der Therapieform ergibt sich ein eindeutiger Vorteil hinsichtlich der Versorgungsmoglichkeit mit Vergrosernden Sehhilfen gegenuber dem Spontanverlauf.

The follow-up of central scotomas and fixation – next to visual acuity – are important parameters for the evaluation of new therapies in AMD.

Patients with solar retinopathy often complain of minute central scotomas and are handicapped when reading. The purpose of this study was to verify scotomas that are too small to be detected by standard perimeters and to analyze patients' reading patterns. Methods: Nineteen patients (12 female, 7 male, aged 5–46 years) with acute solar retinopathy after watching a solar eclipse on 12 October 1996 underwent scanning laser ophthalmoscope (SLO) microperimetry within 10 days after exposure using stimulus size Goldmann I (0.11 °) with the 20 ° field. Size and depth of scotomas were measured. Eye movements during reading were recorded on videotape. Follow-up was at 1 and 6 months. Results: Thirty-one eyes (7 patients unilateral, 12 bilateral) showed scotomas. Four eyes showed anatomic changes in the retinal pigment epithelium but no functional loss. VA was 0.16 to 0.5 in 5 eyes, 26 had VA of 0.8–1.2. Scotomas could be detected in all eyes with subjective impairment. Scotoma size varied from 0.3 to 1.7 ° (1 patient 6 °). Fortyfour percent were deep scotomas (0 dB). All defects improved at 1 and at 6 months; 25 % were no longer detectable. Reading speed was reduced in 75 % of eyes (42 % at 6 months): 200–860 signs/min, median: 510, normal L 660 (at 6 months: 350–920 signs/min, median 670). This was especially due to increased number of regressions (in 81 % of eyes, 21 % at 6 months). The frequency and width of saccades were no different from normal subjects. Conclusion: Minute scotomas (diameter = 0.3 °) can be detected with the SLO. All patients showed objective improvement of their field defect up to 6 months, even when this was not noted by the patient or thought to be due to habituation. Small scotomas can dramatically reduce reading performance.

Background: North Carolina macular dystrophy (NCMD) is a rare autosomal dominant maculopathy with highly variable expressivity. Genetic analysis of an American family consisting of 247 members out of which 96 were affected with NCMD allowed chromosomal assignment of the NCMD locus to 6q14-q16.2. Few families with NCMD are known in Europe, one of these is living in Germany. By routine investigation, a second family affected with NCMD was detected in Germany. As some authors still doubt the good prognosis of this disease, our results should be added to the experience of others. Patients and methods: In a total of 18 family members from three generations between the age of 2 and 65 years, clinical investigations and genetic analysis was carried out. Some individuals had additional examinations such as colour contrast sensitivity, EOG, ERG, and microperimetry. Results: Ten of 18 family members turned out to be affected. All grades of NCMD were present with great variability. Visual acuity ranged from 0.32 to 1.0 and did not correlate to the grade of the disease or to the age of the person. In those patients who underwent microperimetry, central fixation was confirmed. Genetic linkage analysis further narrowed the region harbouring the NCMD locus and supported the assumption that the central areolar pigment epithelial dystrophy (CAPED) is an allelic disorder. Conclusion: Similar visual acuity in three generations of NCMD patients supports the observation that NCMD is not a progressive disorder. If geographic atrophy is found in a patient with good visual acuity, NCMD should be considered and genetic analysis should be carried out.

Background The coding of ophthalmological diagnoses is a long-standing problem that has recently been highlighted by the planned introduction of ICD 10. Methods Using an alphanumerical classification system for the first time, ICD 10 offers the space needed for necessary expansion without leading once again to dislocations in the numbering system. The FoxPro® 2.5 database system was used to create a program based on an expanded ICD 10 that can be applied from DOS® and Windows Results ICD 10 was expanded by the addition of a numerical fifth digit, thus increasing the number of classifiable ophthalmological disorders by a factor of 4,2 from 390 to 1635. The new program greatly facilitates the handling of this substantial amount of data. Conclusions The enhanced diagnosis coding achieved with the expanded ICD 10 permits nosologically exact statistics to be kept for use as a basis for further scientific work.

The incidence of various benign and malignant tumors is increased in patients with Noonan syndrome compared with the general population. We present a 9-year-old boy with the typical features of Noonan syndrome and an acute nonaxial proptosis of the right eye. An ultrasound scan, a computed tomography scan, and magnetic resonance imaging raised suspicion of rhabdomyosarcoma. Biopsy confirmed the diagnosis. The tumor was consequently treated with combined chemotherapy and radiotherapy. Rhabdomyosarcoma is a rare but important differential diagnosis of tumor formation in children with Noonan syndrome and may arise in the orbit.

Many patients with heterophoria report on symptoms related to impaired vision. To investigate whether these symptoms are provoked by saccades this study examines whether in heterophoria effects on intrasaccadic and postsaccadic vergence movements are linked to effects on visual performance. Visual acuity was measured in 35 healthy subjects during fixation and immediately after asymmetric diverging saccades. Binocular position traces were recorded by video-oculography. Subjects with exophoria showed larger intrasaccadic divergence amplitudes, which in turn led to smaller postsaccadic divergence amplitudes. Visual acuity did not depend on heterophoria or vergence amplitudes. The results suggest that compensating for exophoria requires increased convergence activity as compared to orthophoria or compensated esophoria. Visual acuity seemed relatively robust with respect to postsaccadic vergence movements.

Endocrine dysfunction is a rare but known cause of benign intracranial hypertensio (BIH) in adults. Here we describe a rare case of BIH in the pediatric age group associated with autoimmune hyperthyroidism. A 12-year-old girl presented with a 3-month history of headaches. Ophthalmic examination revealed bilateral papilledema. The ocular findings were otherwise normal, with no exophthalmos. Cranial and orbital magnetic resonance imaging was unremarkable. Lumbar CSF opening pressure in recumbent and relaxed position was elevated (31 cm water). Thyroid hormones fT3 and fT4 were elevated while TSH was completely suppressed. As TSH receptor stimulating antibodies (TSHR-Ab) were elevated Graves' disease was diagnosed. Thyroid suppressive therapy with carbimazole was initiated and supplemented by propranolol. As hyperthyroidism improved over two weeks the headaches subsided and the papilledema slowly resolved over the next 2 months. This case illustrates that hyperthyroidism should be considered as a cause of BIH in children.

The Vision Screener is a new, commercial version of the Power Refractor, an off-axis, hand-held video refractor to screen for amblyogenic refractive errors. The aim of our study was to determine the reproducibility of the measurements, compare them to cycloplegic refraction, and evaluate the sensitivity and specificity for the detection of amblyogenic refractive errors.Included in the study were 161 preschool children, age 0.5-7.2 years without manifest strabismus10 degrees , who attended the outpatient clinic. After three measurements with the Vision Screener (version 4.3.15) and one measurement with +3 dpt glasses, the child underwent cycloplegic refraction.The reproducibility of the three non-cycloplegic measurements was +/- 0.5 dpt in 85% and +/- 1 dpt in more than 95% of the children. Accommodation reduced the manifest hyperopia that could be measured with the Vision Screener by up to 3 dpt in some children. Compliance with the +3 dpt glasses increased from 20% in those under one year of age to75 % in those over three. The glasses did increase the sensitivity from 70% to 80% but decreased the specificity from 80% to 65%.The Vision Screener is easy to handle and gives reproducible results. The sensitivity and specificity are between 70 and 80%. The +3 dpt glasses are only helpful in a few patients. The latest software version with a new algorithm is now under evaluation.

Contour interaction, the detrimental effect of flanking features on the discrimination of optotypes, has been studied mainly close to the visual acuity limit. We were interested to know how these results compare with those for the detection of targets. According to the simplest model of contour interaction, comparable detection effects would be expected. The case for low-level masking would be further strengthened if the form and nature of the dependence on flank separation and flank polarity followed that typically found in studies of lateral spatial masking [Vision Res. 33, 993 (1993)]. Landolt Cs subtending a visual angle of 0.25 degrees, 0.5 degrees, and 1.0 degrees were presented and contrast thresholds for detecting the presence of the Landolt C and discriminating its orientation were measured in five normal subjects as a function of flank separation and flank polarity. The results obtained for the relationship between detection and discrimination depend on the size of the target used. For small letters, discrimination but not detection was significantly affected by flanking bars. For large letters, detection and discrimination were affected to the same extent. However, in this case the effectiveness of opposite-polarity flanks and the finding that facilitation occurred at close, not far, flank separations suggests that the simplest explanation in terms of masking may not be applicable.

Schielstellungen und Motilitatsstorungen der Augen konnen in vielfaltiger Weise mit intraokularen Erkrankungen und Storungen der Lider und der Orbita gemeinsam auftreten. Vor einer moglichen kombinierten Operation der Augenmuskeln und der Adnexen oder einer Kombination mit intraokularen Eingriffen ist die Indikation unter verschiedenen funktionellen Aspekten zu betrachten. Nur so lassen sich unnotige Eingriffe vermeiden und die Dosierung der einzelnen Operationen richtig wahlen. Auserdem lasst sich das Doppelbildrisiko minimieren, um so letztendlich fur den Patienten das gunstigste Operationsergebnis zu erzielen.

Traditionally, contour interaction has been investigated at the visual acuity limit using a Landolt C and flanking bars, performance being quantified in terms of a percent correct measure. More recently, it has been shown that the properties of the contour interaction are different when larger stimuli are used: Contour interaction is not polarity specific, and spatial frequency tuning for an unflanked C is broader. Here we quantify contour interaction for stimuli 5× larger than the resolution limit in terms of contrast thresholds. We show that polarity of bars has little effect on unfiltered stimuli but does show very different effects on the spatial-frequency-tuning curves for discrimination of the Landolt C. This explains the polarity dependence of crowding at the visual acuity limit and its independence for larger unfiltered targets. Thus the underlying filtering function is composed of more than one mechanism, affected differently depending on the relative polarity of the test and flank contours.

For all in-patients a fundamental change of hospital financing based only on diagnosis related groups (DRGs) is planned in Germany for 2004. Patients are grouped into different DRGs according to their coded diagnoses, operations and procedures. Hence, the quality of coding is essential for adequate reimbursement of cases and thus for hospital financing. The legal framework of coding is supplied by the so-called "general and special coding guidelines for coding diseases and procedures", presented jointly by the associations representing hospitals and public and private health insurance companies in early 2002. These German coding standards regulate how coding must be performed in daily practice and therefore have a major influence on the grouping of patients into DRGs. Because of their importance this paper describes the practical application of current coding standards. Specifically in Ophthalmology the problem exists that for many procedures adequate coding numbers are still missing, thus impairing further development of the DRG system.

The essential feature of the Parry-Romberg syndrome is atrophy of the subcutaneous tissues on one side of the face. It is a progressive, rather self-limiting disease whose pathogenesis is still unknown. Ocular manifestations include enophthalmos, eyelid atrophy, pupillary and iris abnormalities, fundus changes, and intraocular inflammations, as well as disturbances of ocular motility. Signs of inflammation in the initial stages of this case suggest that the clinical picture could be the result of a secondary atrophy, following an active inflammatory process. The progressive, acquired type of hemifacial atrophy was first described by Parry in 1825 and later by Henoch and Romberg in 1845, and it was suggested to be a form of localized scleroderma. The French expression for this is sclerodermie en coup de sabre. The disease usually begins in the first 2 decades of life and can proceed rapidly. Females are more often affected than males and most cases appear to be sporadic. Apart from ocular manifestations, bone and cartilage are involved if the disease starts in the period of growth.

Background: For a better understanding of motor and sensory adaptations in cyclodeviations, subjective and objective ocular torsion have to be measured under the same conditions. The search coil technique and videooculography allow natural viewing but only assess relative cycloduction, the dynamics of torsion over a short period of time. Cycloposition, on the other hand, can be measured by analysing the position of the foveola relative to the optic disc with fundus photographs but only in nonphysiological viewing. The aim of the study was to develop a technique that allows natural viewing conditions during fundus cyclometry. Methods: The scanning laser beam of the SLO was deflected by 90° with a semitransparent mirror in front of the patient's eyes. The patient was able to look through the semitransparent mirror with both eyes into the room, e.g. at Harms' tangent screen. The infrared SLO images the central retina via the mirror through the undilated pupil. Digital image analysis quantifies the cycloposition of the eye. Controlled head movements while fixating the centre of Harms' tangent screen allow measurements in reproducible gaze positions. Results: The semitransparent mirror reduces SLO image brightness, but image quality is sufficient for cyclometry after contrast enhancement. The laser light can be vaguely perceived by the patient but does not interfere with natural viewing. Reproducibility of the measurement is within ±1°SD. Conclusion: Our modification of SLO fundus cyclometry allows direct measurements of cycloposition in natural viewing conditions. This opens a new field for investigations of cyclodeviations and their sensory and motor adaptations.

To report the occurrence of paracentral scotomata after pars plana vitrectomy for idiopathic macular holes.In 15 patients static microperimetry using a Rodenstock scanning laser ophthalmoscope (SLO-105) was performed preoperatively and 6 or 12 weeks postoperatively (stimulus size 0.2 degrees (Goldmann II), employed intensity 0 and 12 dB, 20 degrees fields in all tests). Surgery consisted of standard three port vitrectomy including removal of epiretinal membranes and the inner limiting membrane.Postoperative paracentral scotomata were detected in areas that were tested normally before surgery. They were mostly located temporally and/or inferiorly and often appeared like nerve fibre bundle defects. The greatest dimension varied from 1.2 degrees to 4.0 degrees (360-1200 microm), smallest dimension from 0.25 degrees to 2.0 degrees (75-600 microm). In three patients more than one scotoma was observed.Small, mostly asymptomatic, paracentral scotomata as a complication after vitrectomy for idiopathic macular hole have not been reported in the literature so far. Whether they are caused by trauma to the nerve fibres during surgery or other factors remains unknown.

Editor,—Visual deterioration caused by watching a solar eclipse has been recognised from the time of Plato. The aetiology of solar retinopathy has been attributed to photochemical effects, which may be enhanced by elevated tissue temperature.1Typically, a small yellow spot may be noted in the foveolar area immediately after exposure. Histopathological studies of solar retinal lesions revealed retinal pigment epithelium (RPE) and photoreceptor damage.1 2 We present two patients with acute, severe solar retinopathy after observation of the total eclipse on 11 August 1999. Funduscopic findings were accompanied by optical coherence tomography (OCT) investigation of the macula. ### CASE REPORTS #### Case 1 An 18 year old man presented 24 hours after watching the total eclipse without appropriate protection. Total exposure time was approximately 20 minutes. During direct viewing the patient kept his right eye closed. One hour after observation he noted blurred vision and a central scotoma on the left …

A recent report suggests that amblyopes are deficient in processing local orientation at supra-threshold contrasts. To determine whether amblyopes are also poor at integrating local orientation signals, we assessed performance for an orientation integration task in which the orientations of static signals are integrated across space. Our results show that amblyopic visual systems can integrate local static oriented signals with the same level of efficiency as normal visual systems. Although internal noise was slightly elevated, there was no indication that fewer samples were used to achieve optimal performance. This finding suggests normal integration of local orientation signals in amblyopia.