L. Krause

Lymphoproliferative lesions of the ocular adnexa: Analysis of 112 cases

OBJECTIVE Lymphoproliferative lesions of the ocular adnexa were analyzed to examine (1) the suitability of the Revised European-American Lymphoma (REAL) classification for the subtyping of the lymphomas in these sites; (2) the predictive value of the REAL classification for the evolution of these tumors; and (3) the frequency and prognostic impact of tumor type, location, proliferation rate (Ki-67 index), p53, CD5 positivity and the presence of monoclonality within these tumors. DESIGN Retrospective review. METHODS The clinical, histomorphologic, immunohistochemical, and molecular biologic (polymerase chain reaction [PCR]) features of lymphoid proliferations of the ocular adnexa were studied. STUDY MATERIALS: The ocular adnexal lymphoproliferative lesions were located as follows: orbit in 52 patients (46%), conjunctiva in 32 patients (29%), eyelid in 23 patients (21%), and caruncle in 5 patients (4%). RESULTS Reactive lymphoid hyperplasia was diagnosed in 12 cases and lymphoma in 99 cases; 1 case remained indeterminate. The five main subtypes of lymphoma according to the REAL classification were extranodal marginal-zone B-cell lymphoma (64%), follicle center lymphoma (10%), diffuse large cell B-cell lymphoma (9%), plasmacytoma (6%), and lymphoplasmocytic lymphoma (5%). Age, gender, and anatomic localization of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years (mean, 3.3 years). Extent of disease at time of presentation was the most important clinical prognostic factor: advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and with lymphoma-related death (P < 0.001). Histomorphologic features and immunohistochemical markers positively correlating with disseminated disease at presentation, stage at final follow-up, and occurrence of lymphoma-related death included cytologic atypia (P < 0.001), MIB-1 proliferation rate (P < 0.001), and tumor cell p53 positivity (P < 0.001). The MIB-1 proliferation rates greater than 20% in extranodal marginal-zone B-cell lymphoma corresponded to at least stage II lymphoma (P < 0.05). CONCLUSION The REAL classification is suitable for the subdivision of the ocular adnexal lymphomas. The MIB-1 proliferation rate and p53 positivity may aid the prediction of disease stage and disease progression, whereas PCR can support the diagnosis and reduce the number of histologically indeterminate lesions.

Primary vitrectomy for rhegmatogenous retinal detachment: an analysis of 512 cases

BackgroundPrimary pars plana vitrectomy (PPPV) has gained widespread popularity in the treatment of rhegmatogenous retinal detachments (RRD). However, the surgical procedure is still flawed by a significant rate of anatomical and functional failures. The study was conducted to analyse the risk factors for a dissatisfying postoperative outcome.MethodsWe carried out a retrospective study of 512 cases of PPPV with a minimum follow-up of 3 months from one institution over a 9-year period. Preoperatively, 24.8% of patients (127 out of 512) were pseudophakic, 16.4% (84 out of 512) highly myopic, 19.9% (102 out of 512) had preoperative proliferative vitreoretinopathy (PVR) and 14.6% (75 out of 512) had vitreous haemorrhage.ResultsThe follow-up period ranged from 3 to 108 months (median 14.8). Retinal reattachment was achieved with one operation in 70.7% (362 out of 512) and after one or more operations in 97.5% of cases (499 out of 512). The major reasons for redetachments were new retinal breaks, followed by a combination of new breaks and PVR, and PVR without apparent breaks. Postoperative visual acuities of ≥0.1 and ≥0.4 were achieved in 82.8% (424 out of 512) and 48.2% (247 out of 512) respectively. Out of 376 phakic patients at study entry, 66.4% (250 out of 376) underwent cataract surgery either in combination with PPPV or during the postoperative course. Factors that were significantly associated with either anatomical or functional failure included duration of symptoms, low preoperative visual acuity, myopia, amblyopia, hypotony, macular detachment, preoperative PVR, extent of detachment, involvement of inferior quadrants, no detectable breaks, large breaks, breaks posterior to the equator, surgeon, level of surgical training, endocryotherapy, and combined scleral buckling surgery.ConclusionPrimary pars plana vitrectomy is still flawed by a relatively high primary redetachment rate following the initial procedure. The advantages of the technique are a high final reattachment rate and relatively good functional results in a subset of patients with more complicated types of RRD. The risk factors for postoperative failures following PPPV for RRD match to a large extent those following scleral buckling surgery (SBS). Future improvements of the technique will have to focus on modifiable risk factors, such as details of the surgical procedures, surgical training and case selection, to distinguish it from SBS.

Epidemiology and clinical manifestations of Adamantiades-Behçet disease in Germany – Current pathogenetic concepts and therapeutic possibilities

© Blackwell Verlag GmbH • www.blackwell.de • 1610-0379/2006/0401-0049 JDDG | 1 2006 (Band 4) Introduction Historical background and nomenclature In his third book of “Epidimion” Hippocrates of Kos (460–377 BC) described a disease with symptoms comparable to the cardinal symptoms of Adamantiades-Behcet disease (ABD): “Many patients had aphthous ulcerations in the mouth. There were numerous purulent genital ulcers, as well as furuncles, which were external and internal, as well as in the groins. There was a watery inflammation of the eye that was chronic and painful as well as growths on the eyelids – external and internal – which impaired eyesight in many patients. (...) There were also larger ecthymata and large herpetic lesions in many patients. “ In 1930 Benediktos Adamantiades (1875–1965), a Greek ophthalmologist, presented a lecture describing findings in an 18-year-old patient, for the first time grouping ocular symptoms, oral aphthae, and genital ulcers together as symptoms of a single clinical entity. He later added vascular involvement as a fourth cardinal symptom and in a review article presented the first classification of the disease by describing its ocular, mucocutaneous, and systemic forms [1]. The chief symptom, which according to Adamantiades was hypopyon, appears at various intervals, causing more or less lasting permanent damage and ultimately resulting in blindness. Hulusi Behcet (1889–1948), a Turkish dermatologist, first described in 1937 patients with recurrent lesions affecting the mouth, genitals, and eyes, adding a catalogue of symptoms including periodontitis, jaw cysts, acneiform skin changes, and erythema nodosum as well as arthralgia. Behcet’s intense work on the disease and his conviction about its autonomous character finally brought this puzzling clinical picture to the attention of the scientific community. CME

Incidence and clinical characteristics of symptomatic choroidal metastasis from lung cancer

Purpose:  To determine the clinical characteristics of symptomatic choroidal metastasis (CM) resulting from metastatic lung cancer.

Interferon alfa-2a in the Treatment of Ocular Adamantiades-Behçet’s Disease

Adamantiades-Behcet’s disease (ABD) is a chronic relapsing multisystemic vasculitis involving small and large vessels. The etiology of the disease still remains unknown. Relapsing ocular involvement is one of the major manifestations of Adamantiades-Behcet’s disease‚ characterized by iritis (Fig. 1)‚ uveitis‚ retinal occlusive vasculitis (Fig. 2)‚ and optic nerve neuropathy that often lead to blindness if untreated. Retinal detachment and secondary glaucoma are severe complications of occlusive retinal vasculitis. Surgical intervention of these complications often leads to a recurrence of the inflammation limiting the surgical results. In the case of AdamantiadesBehcet’s disease with eye involvement‚ a combination of systemic corticosteroids and cyclosporine A is currently the treatment of choice‚ although severe side effects‚ such as Cushing syndrome‚ osteoporosis or renal failure and hypertension frequently occur. Interferon alfa has been shown to be an effective treatment in mucocutaneous ABD. It has also been suggested to improve ocular lesions. We examined the effects of interferon alfa-2a (6-9 Mio IU 3x/week) in a case series of 24 patients with ocular involvement.

Ten-year results of transscleral resection of large uveal melanomas: local tumour control and metastatic rate

Aims To describe the long-term tumour control and metastatic rate after transscleral resection (TSR) of large uveal melanomas in a single-centre study. Methods The sample included 210 patients with large uveal melanomas. Univariate analysis of local tumour control and metastatic risk by Kaplan–Meier survival curves and log-rank testing. Cox proportional HR analysis with forward and backward selection was used to identify independent prognostic factors in patients submitted to TSR of a large uveal melanoma. Results A residual tumour was diagnosed in 6% of the patients. The 5- and 10-year local tumour recurrence rates were 24% and 32%, respectively. Older age, a large basal tumour diameter, the lack of adjuvant ruthenium brachytherapy and retinal detachment led to a 2.6, 2.4, 4.4 and 7.8 times higher risk of melanoma recurrence, respectively. The 5- and 10-year metastatic rates were 28% and 44%, respectively, and were statistical significantly affected by extraocular spread, tumour thickness and local tumour recurrence. Conclusions TSR is an alternative to enucleation for the treatment of large uveal melanomas. Results should improve with better patient selection and more effective methods of adjuvant radiotherapy.

Ocular involvement is associated with HLA-B51 in Adamantiades–Behçet's disease

PurposeTo evaluate the association of HLA-B51 and ocular involvement in Adamantiades–Behçets disease.MethodsWe retrospectively analysed all patients with Adamantiades–Behçets disease examined in our Department of Ophthalmology since 1982. All patients fulfilled the criteria of the International Study Group for Behçets disease. We included 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years.ResultsThe mean age at the first manifestation was 23 years; full disease was noted at 32 years. The mean age at the time of eye involvement was 30 years. Most of the patients were of Turkish (n=73) or German (n=34) origin. A total of 56% patients developed eye involvement. Forty-nine out of 76 HLA-B51-positive patients (64.5%) and 26 out of 60 HLA-B51-negative patients (43.3%; P=0.014) developed ocular involvement.ConclusionMore than the half of the patients with Adamantiades–Behçets disease evaluated in our department developed ocular involvement. There was a statistically significantly higher frequency of HLA-B51 in these patients.

Incidence and outcome of endophthalmitis over a 13-year period

OBJECTIVE The aim of the study was to assess the functional results and long-term visual prognosis in patients treated for acute endophthalmitis (AE). DESIGN Observational clinical series. PARTICIPANTS Evaluation of 120 eyes of 114 patients with AE treated between 1991 and 2004. METHODS This retrospective institutional analysis included 120 eyes of 114 patients (52 male, 68 female) with AE and a minimum follow-up of half a year. The patients had a mean follow-up of 23 months (6-135 months) and a mean age of 61 years (8 months-94 years) at the time of treatment. Treatment included intraocular injection of antibiotics alone (n = 18, 15%) and vitrectomy combined with intraocular and topical postoperative antibiotics (n = 85, 70%). RESULTS The most frequent complications were vitreous or retinal hemorrhages (n = 17, 14%), retinal detachment (n = 17, 14%), choroidal detachment (n = 3, 3%), secondary glaucoma (n = 7, 6%), and recurrent endophthalmitis (n = 3, 3%). Four eyes had to be enucleated because of recurrent and uncontrollable infection. Positive microbiological results were achieved in 67 of 88 specimens (76%). The most common isolate was Staphylococcus. At the end of follow-up, visual acuity was reduced (more than 2 lines) in 18 eyes (15%), stable in 22 (18%), and improved (more than 2 lines) in 72 eyes (60%). CONCLUSIONS AE is a rare but severe disease with a potentially deleterious outcome in affected eyes. In our series, 78% of all eyes had stable or improved postoperative visual acuity following various treatment regimens, depending on the severity of each case.

[Epidemiology and clinical aspects of Adamantiades-Behçet disease in Gemany. Current data].

Of the 721 documented patients in the German Registry for Adamantiades-Behçet disease (registered charity), 258 were of German and 308 of Turkish descent, along with 30 other countries of origin. The prevalence in Germany is 0.9:100,000. Manifestation of the disease was predominantly in the third decade of life (median age: 26.5 years) and in 10.7% the disease onset was under 16 years of age. The full clinical picture developed on average in 2.9 years (median 3 months). Patients of Turkish descent showed androtopism in contrast to those of German descent (female:male  1.9:1), which was also detected in the whole collective in a ratio of 1.4:1. In 12.4% there was a family history with differences between German and Turkish patients (3.8% versus 14.6%) as well as in patients with disease onset in young and adult age (25.0% versus 7.3%). Most frequent features included oral aphthae (98.5%), cutaneous lesions (81%), genital ulcers (64.7%), ocular manifestations (51.6%), arthritis (52.4%) and positive pathergy test (30.8%). Turkish patients suffered significantly more often from eye manifestations compared with Germans, while in German patients prostatitis/epididymitis and gastrointestinal involvement were more frequently documented. As serious complications arose blindness 6.8%, meningoencephalitis 4.0%, severe arthritis 2.6%, fatal outcome 1.2%, hemoptysis 1.1% and gastrointestinal perforation 0.5%. The HLA-B5 antigen was positive in 58.1% and showed an association with eye manifestations. The relative risk of HLA-B5 positive individuals is high in both Germans (6.57) and Turks (5.81).

Epidemiologie und Klinik des Morbus Adamantiades-Behçet in Deutschland

Of the 721 documented patients in the German Registry for Adamantiades-Behçet disease (registered charity), 258 were of German and 308 of Turkish descent, along with 30 other countries of origin. The prevalence in Germany is 0.9:100,000. Manifestation of the disease was predominantly in the third decade of life (median age: 26.5 years) and in 10.7% the disease onset was under 16 years of age. The full clinical picture developed on average in 2.9 years (median 3 months). Patients of Turkish descent showed androtopism in contrast to those of German descent (female:male  1.9:1), which was also detected in the whole collective in a ratio of 1.4:1. In 12.4% there was a family history with differences between German and Turkish patients (3.8% versus 14.6%) as well as in patients with disease onset in young and adult age (25.0% versus 7.3%). Most frequent features included oral aphthae (98.5%), cutaneous lesions (81%), genital ulcers (64.7%), ocular manifestations (51.6%), arthritis (52.4%) and positive pathergy test (30.8%). Turkish patients suffered significantly more often from eye manifestations compared with Germans, while in German patients prostatitis/epididymitis and gastrointestinal involvement were more frequently documented. As serious complications arose blindness 6.8%, meningoencephalitis 4.0%, severe arthritis 2.6%, fatal outcome 1.2%, hemoptysis 1.1% and gastrointestinal perforation 0.5%. The HLA-B5 antigen was positive in 58.1% and showed an association with eye manifestations. The relative risk of HLA-B5 positive individuals is high in both Germans (6.57) and Turks (5.81).