Posterior embryotoxon: its epidemiology and significance as a sign of glaucoma

Background: Posterior embryotoxon (PET) forms part of a spectrum of anterior chamber developmental anomalies, several of which are risk factors for glaucoma. Posterior embryotoxon also occurs as an isolated anomaly. This study was undertaken to determine the prevalence of PET and whether PET in isolation is a risk factor for glaucoma. Methods: Consecutive patients were examined for PET and their intraocular pressures recorded. Consecutively presenting patients having glaucoma were also examined for PET. All patients having PET or glaucoma were further examined by gonioscopy for evidence of other anterior chamber developmental anomalies. Results: PET occured in 47 (6.1 per cent) of 765 patients. There was no statistically significant difference between the intraocular pressure of patients having PET and those who did not. Patients having glaucoma did not have higher prevalence of PET than normals. Axenfeld's anomaly occurred in four of the non-glaucoma PET patients and in one of the glaucoma patients. Conclusions:PET in isolation does not appear to be a risk factor for glaucoma but might be an appropriate indication for gonioscopy. (Clin Exp Optom 1995; 78: 2: 74–78)     

Anterior chamber cleavage syndrome. A stepladder classification

This paper classifies the abnormalities of the anterior chamber cleavage syndrome (mesodermal dysgenesis of the iris and cornea). The anatomic findings are arranged in a tabular stepladder fashion which builds from simple to more complex combinations, most of which have been previously known by eponyms. There are three groups of anomalies: 1) peripheral, 2) central, and 3) combinations of the two. 1) The peripheral anomalies consist of a prominent Schwalbe's ring, iris strands to Schwalbe's ring, and hypoplasia of the anterior iris stroma. Developmental glaucoma is commonly present. 2) The essential feature of the central anomalies is a defect in the corneal endothelium and Descemet's membrane with an overlying corneal opacity. Additional components include central iridocorneal adhesions, keratolenticular approximation with cataract, and scleralization of the cornea. Chorioretinal anomalies, developmental glaucoma, and systemic malformations may be present. 3) Central and peripheral combinations may exist in the same eye, in both eyes of the same patient, or within the same family.     

剥脱性开角型青光眼与原发性开角型青光眼眼前节结构参数的比较

目的：比较剥脱性开角型青光眼（PXOAG）与原发性开角型青光眼（POAG）眼前节结构参数的差异。方法：病例对照研究。选取2012 年12 月至2016 年12 月住院治疗的连续PXOAG病例54 例（54 眼）作为PXOAG组,平均眼压为（28.8±7.9）mmHg（1 mmHg=0.133 kPa）。选取性别、年龄及眼压相匹配的POAG病例53 例（53 眼）作为POAG组,平均眼压为（26.3±7.4）mmHg。测定2 组患者角膜厚度、角膜内皮细胞密度、六角型细胞比例、前房深度及晶状体厚度等眼前节参数,并采用独立样本t 检验进行数据分析。结果：POAG组角膜厚度、角膜内皮细胞密度、六角型细胞比例、前房深度及晶状体厚度分别为（535±36）μm、 （2 538±356）/mm2、 （52±12）%、 （2.89±0.36）mm和（4.96±0.41）mm;PXOAG组相应参数分别为（523±41）μm、 （2 323±451）/mm2、 （52±14）%、 （2.79±0.60）mm和（4.98±0.42）mm。2 组患者角膜厚度、六角型细胞比例、前房深度及晶状体厚度比较差异无统计学意义（t =1.57、0.18、1.11、0.26,P ＞0.05）,而角膜内皮细胞密度比较差异有统计学意义（t =2.78,P =0.01）。结论：PXOAG与POAG相比,角膜内皮细胞密度较低,提示在临床治疗过程中应更加注意对角膜内皮的保护。     

Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature

Hyperimmunoglobulinemia E (Job''s) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job''s syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and Candida endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job''s syndrome.     

Overview and Diagnosis of Acute Retinal Necrosis Syndrome

Acute retinal necrosis (ARN) syndrome, also known as Kirisawa's uveitis, is one of the most serious ocular diseases, and is characterized by a combination of peripheral, confluent, necrotizing retinitis, retinal arteritis, and intraocular inflammation. ARN syndrome is caused by the herpesvirus family, including herpes simplex virus (HSV) and varicella-zoster virus (VZV). The diagnosis of ARN syndrome is fundamentally based on clinical appearance and the demonstration of viral infection. Recently, polymerase chain reaction techniques permit detection of very small amounts of viral DNA in intraocular specimens. This knowledge can help in both the diagnosis and design of therapeutic strategy for ARN syndrome. Here we review the clinical presentation and the current advances in the diagnosis of ARN syndrome.     

Susac's syndrome: First from India and youngest in the world

A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child presented with reduced level of consciousness and bilateral hypoacusis. The patient was lethargic. Ophthalmic examination showed branch retinal artery occlusion (BRAO). This finding was crucial to the diagnosis of Susac''s syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Magnetic resonance imaging of the brain also showed typical features. Thorough blood investigations did not reveal any other abnormality. Patient was treated with immunosuppressive to prevent her from developing severe sequelae of this disease. The child showed dramatic improvement in her systemic condition within 48 h of starting the treatment. This is the youngest ever and first case report from India.     

Retinal pigment epithelial abnormalities in fundus flavimaculatus: a light and electron microscopic study

Light, fluorescent, and scanning and transmission electron microscopic examinations of two postmortem eyes from a 24-year-old man with well-documented fundus flavimaculatus with atrophic macular degeneration revealed striking abnormalities in the retinal pigment epithelium (RPE). Beginning near the equator, scanning electron microscopy demonstrated a progressively marked heterogeneity in the size of the RPE cells. Surrounded by a fairly regular mosaic of relatively normal appearing cells, enormously enlarged hypomelanotic cells measuring up to 80 microns in diameter occurred in irregular aggregates that became more prevalent posteriorly. Diffusely and intensely PAS-positive, the RPE was packed with a granular substance with ultrastructural, autofluorescent, and histochemical properties consistent with an abnormal form of lipofuscin. The greatest concentration of lipopigment was noted posteriorly. Stains for acid mucopolysaccharide were only mildly positive. The clinical and fluorescein angiographic manifestations of fundus flavimaculatus are consistent with accumulation of a lipofuscin-like substance in the RPE. The massive amounts of lipopigment encountered in this young individual suggest that disordered lipopigment metabolism may play a major role in the pathogenesis of this retinal pigment epithelial disorder.