Iris lymphoma—a systematic guide for diagnosis and treatment

Iris lymphomas are rare malignant neoplasms arising either as primary tumors in the iris or as secondary tumors involving the iris. We summarize previously published data and make recommendations for work-up strategies for cases of suspected iris lymphoma. Our objective is to provide a structured overview of the typical clinical symptoms and signs, the pathologic, ophthalmic, as well as hematologic work-up for diagnosis, treatment, and follow-up of iris lymphomas and offer a flowchart on how to diagnose and treat these tumors.     

The ophthalmologist B. Adamandiadis and the history of the "Adamandiadis-Beh?et syndrome"

This article presents the scientific and social work of Benedikt Adamandiadis and the history of the "Adamandiadis-Beh?et syndrome". The authors also attempt to describe the inestimable contribution to modern Greek ophthalmology. It is not the authors' intention to underscore Adamandiadis' importance as the discoverer of the syndrome - for him, the fulfillment of his duties as a scientist and a human being was always more important than personal fame -, but merely to correct an historical injustice.     

Histological observation of trabecular meshwork in a patient with Axenfeld-Rieger syndrome—a new theory for the mechanism of ectropion uvea in congenital glaucoma

AIM: To analyze the clinical efficacy of intravitreal injection of ranibizumab on diabetic macular edema (DME) with multifocal electroretinography (mfERG) and optical coherence tomography (OCT). METHODS: A total of 41 patients (41 eyes) with DME were treated with intravitreal injection of ranibizumab (IVR). The best corrected visual acuity (BCVA), mfERG results, and OCT were analyzed to compare to the baselines, 1wk, 1 and 3mo after operation. RESULTS: The BCVA was significantly improved in all eyes at each time point (P<0.001). The macular area leakage and edema were reduced 1wk and 1mo after IVR, and the central fovea thickness (CFT) was significantly reduced compared to baseline (P<0.001). The mfERG, two-dimensional and three-dimensional images all showed that the macular fovea (1 ring) response density decreased, and the fovea and macular area spikes significantly decreased or disappeared. The amplitude density of the P1 wave was increased, and the latency of the P1 wave was shortened than preoperation (P<0.001). At 1wk and 1mo after the operation, there was a negative correlation between the amplitude density of P1 waves and CFT. CONCLUSION: OCT and mfERG fully demonstrate the importance of IVR for DME patients from the macular morphology and function, especially the significance of mfERG in this disease.     

Is early surgery for congenital cataract a risk factor for glaucoma?

AIMS: To estimate the risk of aphakic glaucoma after lensectomy for congenital cataract and its association with surgery within the first month of life. METHOD: A retrospective case notes review was conducted of all patients who had lensectomy for congenital cataract during their first year of life at Great Ormond Street Hospital between 1994 and 1997. Patients with pre-existing glaucoma, anterior segment dysgenesis, and Lowe syndrome were excluded. The risk of aphakic glaucoma after surgery was estimated using Kaplan-Meier survival analysis. RESULTS: 80 patients, undergoing 128 lensectomies were eligible. Of these, six patients (nine eyes) were lost to follow up. Based on eye count, the risk of glaucoma by 5 years after lensectomy was 15.6% (95% CI 10.2 to 23.4). Based on patient count, the 5 year risk of glaucoma in at least one eye following bilateral surgery was 25.1% (95% CI 15.1 to 40.0). The incidence of glaucoma remained at a constant level for the first 5 years after surgery. After early bilateral lensectomy, within the first month of life, the 5 year risk of glaucoma in at least one eye was 50% (95% CI 27.8 to 77.1) compared to 14.9% (95% CI 6.5 to 32.1) with surgery performed later (log rank test, p = 0.012). There was no significant difference (Kolmogorov-Smirnov test: unilateral lensectomy p = 0.587, bilateral lensectomy p = 0.369) in 5 year visual outcomes between eyes operated before and after 1 month of age. CONCLUSION: Bilateral lensectomy during the first month of life is associated with a higher risk of subsequent glaucoma than with surgery performed later. The reason for this is unclear but it may be prudent, in bilateral cases, to consider delaying surgery until the infant is 4 weeks old. As the incidence of glaucoma is similar for each year after surgery, long term glaucoma surveillance is mandatory.     

The diurnal variation of intraocular pressure—the most important symptom for early detection and follow-up of the glaucomas

Hans Goldmann in 1958 emphasized the importance of the diurnal curve of intraocular pressure for very early detection of glaucoma. He stressed from the investigations of Leydhecker (1959) that there is a 10-year interval between early detection of chronic glaucoma and the first signs of neuronal defects or even later, functional problems. Roberto Sampaolesi (1974) finally has proven the important innovation from his great number of diurnal curves. Of course, a precise diurnal curve (Sampaolesi asked for eight measurements) is almost impossible by the ophthalmologist’s office or even within the hospital. We need the cooperation of the patient (like we do this in blood pressure measurement or even diabetes control). This requires an easy-to-handle “self-tonometer”, which allows measurement at any time of the day, even in different positions of the patient's body. Due to some difficult technical problems to be solved, it took until 1988 before the “automatic self-tonometer” was available. This was first tried in three space missions (Spacelab D1, 1985; Spacelab D2, 1993; German-Russian MIR-Mission 1992) allowing for the first time to register the enormous increase of intraocular and intracranial pressure after entering into microgravity. Some years later, the first instrument for clinical application was available, allowing a large series of dense diurnal curves, confirming Goldmann's and Sampaolesi's hypothesis. The technical solution, application, and results with this new instrument are described.     

The association of unilateral congenital glaucoma and congenital lower lid entropion: causal or casual?

This case documents unilateral congenital glaucoma associated with congenital lower lid entropion. A 2-year-old female infant was referred for evaluation and treatment of right-side buphthalmos caused by congenital glaucoma associated with bilateral congenital lower lid entropion that was prominent on the right side and present at birth. Examination disclosed a lower eyelid entropion of the right side that was treated surgically by reinserting the disinserted retractor aponeurosis to anterior inferior tarsal border. After three weeks, the patient was successfully treated with primary combined trabeculotomy-trabeculectomy for congenital glaucoma. The entropion of the left lower lid was asymptomatic and did not require any surgery. Buphthalmos caused by congenital glaucoma may be associated with congenital lower lid entropion and the association may be causal or coincidental.     

No evidence for a genetic blueprint: The case of the “complex” mammalian photoreceptor

Despite the intensity of the search for genes causing inherited retinal degenerations over the past 3 decades, of the approximately 200 disease genes identified to date, all appear to be ordinary housekeeping genes specifying proteins playing basic structural and functional roles in the mature photoreceptor cells. No genes or genetic elements have been identified which can be construed as having a specific morphogenic role, directing the development of the cytoarchitecture of any particular retinal cell. The evidence suggests that the cytoarchitecture of the retinal photoreceptors, although enormously complex, arises from the self-organization of the cells constituents without any regulation or direction from an external genetic blueprint.     

The significance of the complement system for the pathogenesis of age-related macular degeneration — current evidence and translation into clinical application

Background  

Dysregulation of the complement system has been shown to play a major role in the pathogenesis of age-related macular degeneration (AMD).     

Leber’s congenital amaurosis and the role of gene therapy in congenital retinal disorders

Leber’s congenital amaurosis (LCA) and recent gene therapy advancement for treating inherited retinopathies were extensive literature reviewed using MEDLINE, PubMed and EMBASE. Adeno-associated viral vectors were the most utilised vectors for ocular gene therapy. Cone photoreceptor cells might use an alternate pathway which was not reliant of the retinal pigment epithelium (RPE) derived retinoid isomerohydrolase (RPE65) to access the 11-cis retinal dehydechromophore. Research efforts dedicated on the progression of a gene-based therapy for the treatment of LCA2. Such gene therapy approaches were extremely successful in canine, porcine and rodent LCA2 models. The recombinant AAV2.hRPE65v2 adeno-associated vector contained the RPE65 cDNA and was replication deficient. Its in vitro injection in target cells induced RPE65 protein production. The gene therapy trials that were so far conducted for inherited retinopathies have generated promising results. Phase I clinical trials to cure LCA and choroideremia demonstrated that adeno-associated viral vectors containing RPE genes and photoreceptors respectively, could be successfully administered to inherited retinopathy patients. A phase III trial is presently ongoing and if successful, it will lead the way to additional gene therapy attempts to cure monogenic, inherited retinopathies.     

Current practice trends for lacrimal gland neurotoxin in the management of epiphora—a BOPSS survey

Graefe's Archive for Clinical and Experimental Ophthalmology - Injection of botulinum neurotoxin A (BoNTA) to the lacrimal gland (LG) offers a simple and effective treatment in the management...