[Imaging techniques in the evaluation of primary large vessel vasculitides: Part 2: duplex ultrasound, positron emission tomography, computed tomography, and ophthalmological methods]. / Bildgebende Techniken zur Diagnostik primärer Grossgefässvaskulitiden : Teil 2: Duplexsonographie, Positronenemissionstomographie, Computertomographie und ophthalmologische Diagnostik.

This article focuses on the clinical application and technical aspects of imaging methods which are used alternatively or additionally to angiography or magnetic resonance imaging in patients with Takayasu's arteritis or giant cell arteritis. Providing a high spatial resolution, duplex ultrasound is particularly suitable for the evaluation of peripheral arteries. With the exception of cranial arteries, positron emission tomography as a whole body examination is the best imaging modality for the assessment of inflammatory activity. Computed tomography is used for angiographic examinations and enables evaluation of wall thickening in large arteries. It is the method of choice in the case of emergencies due to aortic aneurysm or dissection. In addition to angiographic and ultrasound techniques, ophthalmological methods comprise biomicroscopy, including funduscopy and optical coherence tomography.

[Imaging techniques in the evaluation of primary large vessel vasculitides: part 1: angiography, interventional therapy, and magnetic resonance imaging]. / Bildgebende Techniken zur Diagnostik primärer Grossgefässvaskulitiden: Teil 1: Angiographie, interventionelle Therapie und Magnetresonanztomographie.

Imaging methods have become indispensable for the diagnosis and follow-up of giant cell arteritis and Takayasu's arterititis, in addition to physical examination and laboratory parameters. The choice of method is predominantly determined by clinical presentation and localization of the vascular territory to be examined. Furthermore, aspects of radiation protection, contrast media intolerance and other contraindications, as well as the varying costs of the different procedures need to be considered. This article reviews the clinical and morphological features of primary large vessel vasculitides which are fundamental to the identification of the disease and the assessment of inflammatory activity using imaging modalities. Angiography is the gold standard for the evaluation of stenotic lesions and can be combined with interventional treatment. Vessel wall thickening as a defining diagnostic criterion is outlined only by cross-sectional imaging. In addition to MR angiography, MRI techniques in particular enable vizualization of inflammatory processes in central as well as in peripheral arteries.

Detection of Treponema pallidum in the vitreous by PCR.

BACKGROUND: Ocular involvement of syphilis still poses a clinical challenge due to the chameleonic behaviour of the disease. As the serodiagnosis has significant limitations, the direct detection of Treponema pallidum (TP) in the vitreous represents a desirable diagnostic tool. METHODS: Real-time polymerase chain reaction (PCR) for the detection of TP was applied in diagnostic vitrectomies of two patients with acute chorioretinitis. Qualitative verification of TP by real-time PCR and melting point analysis according to a modified protocol was ruled out. Patients underwent complete ophthalmological examination with fundus photographs, fluorescein angiography, serological examination, antibiotic treatment and follow-up. RESULTS: In two cases of acute chorioretinitis of unknown origin, real-time PCR of vitreous specimens of both patients provided evidence of TP and was 100% specific. Initial diagnosis of presumed viral retinitis was ruled out by PCR of vitreous specimen. Patients were treated with systemic antibiotics and showed prompt improvement in visual function and resolution of fundus lesions. CONCLUSIONS: With real-time PCR, detection of TP in the vitreous was possible and delivered a sensitive, quick and inexpensive answer to a disease rather difficult to assess. In cases of acute chorioretinitis, the use of PCR-based assays of vitreous specimens in the diagnostic evaluation of patients is advisable. Although syphilitic chorioretinitis is a rare disease, PCR should include search for TP, as diagnostic dilemmas prolong definitive treatment in a sight-threatening disease.

[Ligneous conjunctivitis]. / Conjunctivitis lignosa.

CASE REPORT: We report the case of a 3-year-old female patient with therapy refractive recurrent conjunctivitis and membrane formation of the upper eyelid. After surgical removal the histological examination showed an image compatible with ligneous conjunctivitis. A manifest serum plasminogen deficiency (22 %) supported the diagnosis. TREATMENT: The treatment with corticosteroids, heparin-containing and fresh frozen plasma (FFP) eye drops, renewed surgical ablation with perioperative intravenous FFP administration and local cyclosporine A eye drops achieved a stable condition with low disease activity. CONCLUSION: The combination of these therapy approaches has been performed here for the first time and has not been described in the literature so far.

[The German Acanthamoeba keratitis register: Initial results of a multicenter study]. / Das Deutsche Akanthamöbenkeratitis-Register : Erste Ergebnisse einer multizentrischen Erhebung.

BACKGROUND AND PURPOSE: In September 2011 the cornea section of the German Ophthalmological Society (DOG) established the first German Acanthamoeba keratitis registry. The data of this multicenter survey are being collected, compiled and evaluated at the Department of Ophthalmology at the Saarland University. The aim of this article is to present an intermediate report. PATIENTS AND METHODS: Data from 172 eyes with Acanthamoeba keratitis were collected during the last 10 years. For this interim report we actually evaluated 121 eyes (60.2 % female patients, average age 41.3 years) and collected the following data: date of onset of symptoms, date and method of diagnosis, initial diagnosis, anamnestic data, clinical symptoms and signs at diagnosis and during follow-up, conservative and surgical therapy. Criteria for inclusion in the Acanthamoeba registry was the established diagnosis of an Acanthamoeba keratitis with at least one of the methods described in this article. RESULTS: Acanthamoeba keratitis could be histologically proven in 55.3 % of the cases, via PCR in 25.6 %, with confocal microscopy in 20.4 % and using in vitro cultivation in 15.5 %. Clinical symptoms and signs in Acanthamoeba keratitis were pain in 67.0 %, ring infiltrates in 53.4 %, pseudodendritiform epitheliopathy in 11.7 % and keratoneuritis in 5.8 %. In 47.6 % of the cases the initial diagnosis was herpes simplex virus keratitis followed by bacterial keratitis in 25.2 % and fungal keratitis in 3.9 %. Acanthamoeba keratitis was the correct initial diagnosis in only 23.2 % of cases. The average time period between first symptoms and diagnosis was 2.8 ± 4.0 months (range 0-23 months). A triple therapy with Brolene® Lavasept® and antibiotic eye drops at least 5 ×/day was used in 54.5 % of eyes (n = 66). Penetrating keratoplasty was performed in 40.4 %, in 18 cases in combination with cryotherapy of the cornea. The mean graft diameter was 7.9 ± 1.1 mm (range 3.5-11.0 mm). The final visual acuity (Snellen visual acuity chart at 5 m) was comparable in the two groups of eyes with (5/40 ± 5/25) and without (5/32 ± 5/25) keratoplasty. CONCLUSION: Acanthamoeba keratitis is a rare and often very late diagnosed disease and two thirds of the cases were initially misdiagnosed. The early recognition of the typical symptoms is crucial for the prognosis of the disease. All ophthalmological departments in Germany are invited to submit further data of all confirmed cases (berthold.seitz@uks.eu), whether retrospectively or prospectively in order to generate an adequate standardized diagnostic and therapeutic approach for this potentially devastating disease.

Humoral immune response to Klebsiella capsular polysaccharides in HLA-B27-positive patients with acute anterior uveitis and ankylosing spondylitis.

Klebsiella is suggested to trigger ankylosing spondylitis (AS) and acute anterior uveitis (AAU) in HLA-B27-positive individuals. Previous investigations showed an increased antibody response to the Klebsiella capsular types K26, K36, and K50 in sera from HLA-B27-positive AS patients. In the present study the prevalence and titers of antibodies against Klebsiella capsular antigens were measured by means of an ELISA in 32 sera from HLA-B27-positive AAU patients either with (n = 10) or without AS (n = 22) and compared with sera from HLA-B27-negative AS-patients (n = 13). Sera from either HLA-B27-positive (n = 45) or negative (n = 40) healthy individuals served as control. Sera from HLA-B27-positive AAU with or without AS showed significantly higher antibody prevalence and IgG-titers against capsular antigens of the Klebsiella serotypes K26, K36, and K50 when compared with sera from HLA-B27-negative AS patients or with healthy controls. These results might be taken to indicate the predominance of these serotypes in the HLA-B27-associated AS and AAU.

Response to trimethoprim/sulfamethoxazole in Wegener's granulomatosis depends on the phase of disease.

We prospectively studied trimethoprim/sulfamethoxazole (T/S) in inducing remission in 'initial phase' Wegener's granulomatosis (WG), and in sustaining remission in generalized WG, in 72 patients in various disease stages. Nineteen patients with initial phase WG received T/S (2 x 960 mg/day). Another 24 patients with generalized WG received the same dose of T/S (group A) and were compared with 21 patients receiving no further treatment after standard therapy (group B). Eight patients were given T/S plus low-dose prednisone (group C). Eleven of 19 patients (58%) with initial phase WG achieved complete or partial remission lasting a median 43 months (range 6-88 months). Of the remaining eight (42%), five showed local disease progression, and three developed generalized WG. In group A (T/S alone, generalized WG), 10/24 (42%) suffered a relapse after a median 13 months (range 4-58 months). In group B (generalized WG, no further treatment) 29% of patients relapsed after a median 22.5 months (range 18-26 months). All eight patients treated with T/S plus low-dose prednisone (group C) suffered serious relapse after 2-24 months. T/S induced long-term remission in > 50% of patients with initial phase WG; however, neither T/S alone nor T/S plus low-dose prednisone sustained remission in generalized WG.

Distribution of TFF peptides in corneal disease and pterygium.

The central cornea of 10 cadavers and 33 patients suffering from keratoconus, herpetic keratitis, Fuchs' dystrophy and pterygium were analysed focusing on the expression of TFF peptides by means of reverse transcription polymerase chain reaction and immunohistochemistry. TFF1 and TFF3 transcripts were detected in healthy corneae as well as in pterygia. Only TFF3 mRNA was transcribed in keratoconus, Fuchs' dystrophy and herpetic keratitis. Immunohistochemistry revealed absence of all three TFF peptides in healthy corneae but production of TFF3 in each of the diseased corneae. In pterygia both TFF1 and TFF3 synthesis was detectable in goblet cells. The absence of TFF peptide production in the healthy cornea indicates that TFF3 secretion is induced in different corneal diseases by yet unknown stimuli. Here TFF3 synthesis can be interpreted as a protection mechanism, because all corneal diseases analysed are characterized by progressive tissue destruction. TFF1 and TFF3 production by goblet cells in pterygia is comparable to the healthy conjunctiva suggesting that TFF peptides do not play a significant role in the pathogenesis of pterygia.

Ultrasound biomicroscopic imaging in intermediate uveitis.

BACKGROUND: Clinical examination of the region of the eye mainly affected in patients with intermediate uveitis is difficult and often hampered by media opacities. In that perspective ultrasound biomicroscopy (UBM) promises to be a valuable additional diagnostic tool. METHODS: UBM was performed at a sound frequency of 50 MHz on 26 eyes of 13 patients with intermediate uveitis in order to determine configuration of pars plana, peripheral retina, and vitreous. Findings of ophthalmoscopy with scleral indentation and UBM were compared. RESULTS: In 18 of 26 eyes pathological structures such as membraneous or fluffy vitreous condensations were identified by UBM. Among these UBM revealed pathological findings which were not visible on funduscopy in nine eyes. Most importantly, vitreoretinal adhesions with traction on the retina were imaged in four eyes. However, in three eyes vitreous opacities being visible on funduscopy were not identified by UBM. CONCLUSION: UBM seems to be a valuable diagnostic technique for the evaluation of patients with intermediate uveitis. Longitudinal studies will have to determine the relevance of UBM findings for the individual clinical course and their influence on therapeutic decisions.

Expression of adhesion molecule CD44 on human corneas.

AIMS: This study was undertaken to confirm the distribution and expression of the molecule CD44 on human corneas under normal and pathological conditions. METHODS: Fifty eight corneal buttons from adult patients suffering from various corneal diseases and four normal corneas were included in this study. Frozen sections were stained immunohistochemically with monoclonal antibodies against human CD44 using an APAAP method and observed under a light microscope. RESULTS: In normal corneas CD44 was predominantly expressed on the membranes of basal epithelial cells and on the keratocytes, as well as on the vascular endothelial cells of the corneal limbi, but was not expressed on corneal endothelial cells. Enhanced expression of CD44 was observed on the epithelium of corneas with inflammation and allograft rejection. In a number of abnormal conditions including allograft rejection, corneal trauma, primary and secondary corneal endothelial decompensation the remaining endothelial cells stained positively for CD44. However, in some corneas of keratitis, keratoconus, and dystrophy the endothelium which appeared relatively integral in morphology and amount remained CD44 negative. CONCLUSIONS: These results suggest that CD44, the hyaluronate receptor, may play an important role in corneal cell-cell and cell-matrix interactions. Its regulation is closely related to corneal inflammatory reactions. The induction of CD44 on corneal endothelium might play a potential role in compensatory processes when corneal endothelial cells are injured.

Post-mortem HLA tissue typing of retinal pigment epithelial cells.

Retinal pigment epithelial cells (RPE) were derived from bulbi of cornea donors and maintained in culture. The time-span between donor's death and cell cultivation ranged from 2 to 122 h. The mean numbers of hours was 25.6 (n = 130). After IFN-gamma stimulation, cells were serologically typed for class I and class II antigens. Unclear class I allospecificities were verified by one-dimensional isoelectric focusing. Data from the serological typing of lymphocytes and those of the serological and biochemical typing of RPE from the same donors were compared in 22 cases. There was a discrepancy of less than 5%, whereby either the typing on lymphocytes could not identify some specificities declared as blanks or the RPE typing had failed to clearly define a specificity. Our data show that the strategy adopted here is very successful for tissue typing post mortem, thus increasing the number of available HLA-matched corneas and consequently reducing the number of corneal graft rejections.

[The magnetic resonance tomographic signs of Wegener's granulomatosis in the head area]. / Magnetresonanztomographische Kennzeichen der Wegenerschen Granulomatose im Kopfbereich.

MRI of the head was performed in 25 patients suffering from Wegener's granuloma. 23 patients showed evidence of mucosal thickening in the paranasal sinuses, the middle and inner ears and in the mastoid cells; these were characterised by low signal intensity of T1-weighted and high signal intensity of T2-weighted images. In 10 patients there were granulomas in the paranasal sinuses and in the orbits which showed low signal intensity of both T1-weighted and T2-weighted images. In 4 patients, additional images were obtained after the intravenous injection of Gd-DTPA. In 2 patients this resulted in non-homogeneous contrast accumulation in the granuloma. In 7 patients there were signal changes in the brain which were typical of infarcts. The complete extent of bone destruction in the facial skeleton was visible only by CT.

ANCA in ocular inflammatory disorders.

400 patients presenting with active inflammation of the conjunctiva, sclera, orbita, uvea or retina were cANCA negative during active disease, however, pANCA were found in 15%. Another group of 100 patients suffering from Wegener's granulomatosis (WG) showed in 72 cases one or multiple ocular inflammatory symptoms. They were frequently cANCA-positive. Contrary to some other reports dealing with ocular inflammation, we find a high specificity of cANCA for Wegener's granulomatosis and, therefore, recommend cANCA-testing in all ocular inflammatory disorders, which may be difficult to differentiate from a manifestation of WG.

Orbital involvement in Wegener's granulomatosis.

Orbital granulomas may complicate the course of Wegener's granulomatosis (WG). Granulomas that grow and compress the optic nerve may lead to blindness. Careful magnetic resonance imaging (MRI) is recommended for early detection and follow-up of intraorbital granulomas. Sufficient systemic immunosuppressive treatment should be given as usual for Wegener's granulomatosis. In order to preserve vision orbital decompression may be necessary in rapidly progressive pseudotumor of the orbit.

[Postmortem evaluation of corneal astigmatism after astigmatism oriented penetrating keratoplasty]. / Postmortale Evaluation des kornealen Astigmatismus nach Astigmatismus-orientierter perforierender Keratoplastik (AOpKP).

BACKGROUND: Reduction of astigmatism following penetrating keratoplasty can be achieved if the graft is oriented according to astigmatism parameters in donor and host (AOPKP). Postmortem evaluation of these parameters is therefore essential. The aim of our investigation was to compare postmortem measurements using a hand-held keratometer with those of the living donor's astigmatism. METHOD: The 72-year-old female patient had undergone AOPKP on her right eye. After death the astigmatism in the right eye was evaluated 4.5 h postmortem using a hand-held keratometer. After explantation, the eyeball was examined by computer-assisted topography (TMS-1). RESULTS/CONCLUSION: We could show that hand keratometry in situ is reliable for evaluating astigmatism in donor eyes after death. As far as our AOPKP study is concerned, these results are of great interest. Reduction of postoperative astigmatism following penetrating keratoplasty is only possible if data on astigmatism of the donor and host corneas are available.

[Bilateral simultaneous acute amaurosis in neuritis nervi optici. Initial manifestation of encephalomyelitis disseminata]. / Bilaterale simultane akute Amaurose bei Neuritis nervi optici. Erstmanifestation einer Encephalomyelitis disseminata.

BACKGROUND: Bilateral simultaneous acute amaurosis as a primary manifestation of demyelinating disease is extremely rare. PATIENT: The clinical course of a 24-year-old patient who initially presented with a bilateral complete loss of vision is demonstrated. Morphologically both optic discs appeared slightly blurred and prominent. Otherwise there were no anterior and posterior segment abnormalities. Examination of the cerebrospinal fluid revealed an increased number of cells and protein without oligoclonal bands. On MRI multiple white matter lesions were visible. Laboratory tests showed no specific abnormalities, especially with respect to infectious or vasculitic diseases. Under intensive steroid therapy (initially 1000 mg prednisolone/day), visual acuity recovered almost completely. Nine months after onset of the disease visual acuity was 1.0 in both eyes. CONCLUSIONS: Even in patients with a fulminant onset of the disease almost complete visual recovery is possible. Differential diagnosis should rule out vasculitic autoimmune optic neuritis, infections, tumors, processes of the paranasal sinuses, toxic, and hereditary causes.

[Astigmatism-oriented perforating keratoplasty. A possibility for minimizing postoperative astigmatism?]. / Astigmatismusorientierte perforierende Keratoplastik. Eine Möglichkeit zur Minimierung des postoperativen Astigmatismus?

BACKGROUND: This pilot study examined whether astigmatism of donor corneas can be evaluated postmortem, and whether there is an effect on the astigmatism following keratoplasty. METHODS: The corneoscleral rims of donors were marked after measurement by a hand keratometer in the 12 o'clock position. We examined 38 patients after penetrating keratoplasty and divided them into three groups. The 12 o'clock position of the donor cornea was fixed in the same position as the recipient (group A, n = 12), or the donor corneas were transplanted with the axis in opposition to the axis of the recipient (group B, n = 12) or in the same orientation (group C, n = 14). Development of postoperative astigmatism was compared with controls (group D, n = 40). RESULTS: In group B we found a new orientation of the astigmatism axis within 2-4 months; in group C the axis maintained the original orientation of the recipient. Mean postoperative astigmatism was 3.5 +/- 1.5 dpt in group A, 2.1 +/- 1.3 dpt in group B, 5.8 +/- 1.6 dpt in group C, and 3.4 +/- 1.5 dpt in group D. After suture removal the axis orientation persisted in all groups. CONCLUSION: This study shows the possibility of evaluating corneal astigmatism in donor eyes. The astigmatism of the donor cornea itself directly influences the postoperative axis of astigmatism in recipient eyes. Astigmatism after corneal grafting can be reduced by orienting the donor astigmatism against the axis of the recipient.

[Wound or suture insufficiency complicating penetrating keratoplasty]. / Schwerwiegende Wund- und Nahtinsuffizienzen nach perforierender Keratoplastik.

BACKGROUND: With regard to penetrating keratoplasty methods to culture corneal donor tissues, microsurgical techniques, HLA typing and understanding of basic mechanisms in inflammation and especially graft rejection, and postoperative treatment schedules have been improved in recent years. This now enables successful penetrating keratoplasty in many more patients than previously performed. However, in rare cases relevant problems in wound closure may appear. PATIENTS AND METHODS: A standardized treatment protocol was applied to 1,253 penetrating keratoplasties performed in a single center. Patients were continuously followed up. Simple leakage after corneal grafting was not further analyzed if conservative treatment or additional sutures achieved sufficient wound closure. In 21 cases, however, the causes of large wound dehiscence after corneal grafting were analyzed. RESULTS: During the first week early problems in suturing penetrating keratoplasty in five patients were associated with the instability of the recipients' corneal stroma (stromal thinning in keratoconus or corneal herpes, suture problems in keratomalacia, active herpes keratitis, corneal burns, or rheumatic diseases). Long-term complications in 16 patients were associated with alcoholism, herpes keratitis, rheumatic disorders or traumatic suture defects. Overall, nine patients lost functionally or even anatomically one eye because of wound dehiscence after corneal grafting. CONCLUSIONS: When penetrating keratoplasty is indicated, special attention should be given to (1) the compliance of the patient, (2) sufficient treatment of herpes keratitis or other infections, (3) adequate immunosuppression in autoimmune corneal inflammation, (4) double running continuous sutures as primary suture with sometimes additional single sutures to stabilize the graft, and (5) surgery in time.