Abnormalities of retinal ganglion cell complex at optical coherence tomography in patients with type 2 diabetes: a sign of diabetic polyneuropathy, not retinopathy.

AIMS: To compare optical coherence tomography (OCT)-derived neuro-retinal parameters in patients with type 2 diabetes and non-diabetic controls and to evaluate their correlation with diabetic retinopathy (DR) and polyneuropathy (DPN). METHODS: One-hundred consecutive patients with type 2 diabetes were examined by spectral-domain (SD) OCT for evaluating ganglion cell complex (GCC) and retinal nerve fibre layer (RNFL) thickness and two new pattern-based quantitative measures of GCC damage, global and focal loss volume (GLV and FLV). Fifty sex- and age-matched non-diabetic subjects served as control. RESULTS: RNFL thickness (101.0±10.6 vs. 106.4±10.3 µm, P=0.003) was significantly lower and GLV (6.58±4.98 vs. 4.52±3.10 %, P=0.008) and FLV (1.90±1.97 vs. 0.89±0.84 %, P<0.0001) were significantly higher in diabetic versus control subjects. The OCT parameters did not differ significantly according to DR grade. Conversely, RNFL thickness was lower and GLV and FLV were higher in patients with versus those without DPN, and the extent of changes increased significantly with quartiles of DPN score. At both bivariate and multivariate analysis, OCT parameters, especially FLV, correlated significantly with DPN measures. CONCLUSIONS: The GCC is significantly affected in patients with type 2 diabetes and SD-OCT might represent a useful tool to detect DPN, but not DR in these individuals.

Pseudoexfoliation Syndrome and Pseudoexfoliation Glaucoma: A Review of the Literature with Updates on Surgical Management.

Pseudoexfoliation syndrome (PES) is a systemic disorder caused by progressive accumulation of extracellular material over various tissues. PES usually determines increased intraocular pressure, changes in the anatomical aspects of the optic nerve, and visual field alterations leading to the diagnosis of pseudoexfoliation glaucoma (PEG). Use of topical medical treatment usually leads to poor results in terms of long-term follow-up but many surgical techniques, such as Argon Laser or Selective Laser Trabeculoplasty, have been proposed for the management of PEG affected patients. The present paper is a review on the pseudoexfoliation syndrome and pseudoexfoliation glaucoma with an update on surgical management.

Pediatric Glaucoma: A Literature's Review and Analysis of Surgical Results.

The purpose of this paper is to review the surgical options available for the management of pediatric glaucoma, to evaluate their advantages and disadvantages together with their long-term efficacy, all with the intent to give guidelines to physicians on which elements are to be considered when taking a surgical decision. Currently there is a range of surgical procedures that are being used for the management of pediatric glaucoma. Within these, some are completely new approaches, while others are improvements of the more traditional procedures. Throughout this vast range of surgical options, angle surgery remains the first choice in mild cases and both goniotomy and trabeculotomy have good success rates. Trabeculectomy with or without mitomycin C (MMC) is preferred in refractory cases, in aphakic eyes, and in older children. GDIs have a good success rate in aphakic eyes. Nonpenetrating deep sclerectomy is still rarely used; nevertheless the results of ongoing studies are encouraging. The different clinical situations should always be weighed against the risks associated with the procedures for the individual patients. Glaucomatous progression can occur many years after its stabilization and at any time during the follow-up period; for this reason life-long assessment is necessary.

Twelve-Month Results of a Single or Multiple Dexamethasone Intravitreal Implant for Macular Edema following Uncomplicated Phacoemulsification.

The clinical efficacy of one or two intravitreal injections of a continued deliverance dexamethasone 700 µg implant in ten patients with persistent macular edema following uncomplicated phacoemulsification was evaluated. Complete ophthalmological examination and spectral domain optical coherence tomography were carried out. Follow-up was at day 7 and months 1, 2, 4, 6, 8, and 12. At baseline mean best corrected visual acuity was 62 Early Treatment Diabetic Retinopathy Study Chart letters, which showed statistically significant improvement at each follow-up, except at month 6, to reach 79 letters at month 12 (P = 0.018). Prior to treatment mean central foveal thickness was 622 µm, which showed statistically significant improvement at each follow-up to reach a mean value of 282 µm (P = 0.012) at month 12. Five patients received a second dexamethasone implant at month 7. Two patients were excluded from the study at months 4 and 8. Intraocular pressure remained stable during the study period with the exception of mild increase in two patients requiring topical therapy. In conclusion there was statistically significant improvement of best corrected visual acuity and mean central foveal thickness with one or two intravitreal dexamethasone implants over 12 months.

Retinitis pigmentosa: evaluation of the vestibular system with cervical and ocular vestibular evoked myogenic potentials and the video head impulse test.

OBJECTIVE: Retinitis pigmentosa (RP) represents a group of inherited disorders in which abnormalities of the photoreceptors lead to progressive visual loss. Night blindness, peripheral visual field loss, and eventual total blindness represent typical visual damage of such disease. No study has previously evaluated the presence of a "latent" vestibular deficit in patients with RP. STUDY DESIGN: Prospective study with caloric test, cervical vestibular evoked myogenic potentials (C-VEMPs), ocular vestibular evoked myogenic potentials (O-VEMPs), and video head impulse test (v-HIT). SETTING: Tertiary referral center. PATIENTS: 16 patients suffering from RP. INTERVENTION: Evaluation of vestibular dysfunction with caloric test, C-VEMPs, O-VEMPs, and the measurement of the vestibular-ocular reflex (VOR) using the v-HIT. RESULTS: Only five patients with RP showed normal values in all the vestibular tests performed. Three patients had an evident deficit at the caloric test, whereas eight (50%) of them had a normal caloric test but a pathological response in at least one of the other vestibular tests performed. No patient of the study showed a bilateral otolith or ampullary dysfunction. CONCLUSION: Our patients with RP unexpectedly showed pathological responses in at least one of the vestibular tests performed. Nowadays, in patients affected by RP, a vestibular diagnostic protocol must include VEMPs and v-HIT to confirm the vestibular damage and to identify selective damage of the vestibular nerve.

Potential regulatory molecules in the human trabecular meshwork of patients with glaucoma: immunohistochemical profile of a number of inflammatory cytokines.

Glaucoma occurs when there are imbalances between the production and the drainage of the eye liquid. The vast majority of the aqueous humor leaves the eye through the trabecular meshwork (TM). The cause of hypertonicity may be due to an alteration in the thickness of the TM. In the majority of cases the molecular changes that determine primary open­angle glaucoma (POAG) are unclear. However, it has been hypothesized that the significant increase in the extracellular matrix (ECM) of the fibrillary bands in the TM is associated with possible inflammatory conditions. In this study the tissue distribution of interleukin (IL)­6, IL­1ß, transforming growth factor-ß1 (TGF­ß1), vascular endothelial growth factor (VEGF) and tumor necrosis factor α (TNF­α) was analyzed in TM samples from patients with POAG by immunohistochemistry. Seven specimens from patients with POAG and three control tissues were analyzed by immunohistochemistry using specific antibodies against these cytokines. Morphological changes in the TM, such as increased cell content, macrophages, fibrosis and accumulation of neutrophils, were observed by transmission electron microscopy. In human TM tissues, an evident immunoreactivity for IL­6, IL­1ß and TNF­α was observed in patients with POAG when compared with the control subjects, indicating that these cytokines may be correlated with disease activity. TM endothelial cells secrete a number of factors and cytokines that modulate the functions of the cells and the ECM of the conventional outflow pathway. In the TM in glaucoma, macrophages produce cytokines, including IL­6, IL­1ß and TNF­α, leading to an acute inflammatory response and recruitment of other immune cells, including T lymphocytes. In addition, TGF­ß1 regulates and induces the expression of IL­6 in TM that indirectly induces angiogenesis by stimulating VEGF expression. The present results support previous evidence that suggests that growth factors and cytokines can induce ECM remodelling and alter cytoskeletal interactions in the TM.

Usher's Syndrome: Evaluation of the Vestibular System with Cervical and Ocular Vestibular Evoked Myogenic Potentials and the Video Head Impulse Test.

OBJECTIVE: The Usher's syndrome (USH) is composed of a group of inherited disorders characterized by a dual sensory impairment of the audiovestibular and visual systems. Despite the established hearing loss, few authors have investigated vestibular dysfunction in these patients.The aim of this article is to investigate otolith or ampullary dysfunction in a group of patients affected by USH by means of a diagnostic protocol using caloric vestibular tests, cervical vestibular evoked myogenic potentials (C-VEMPs), ocular vestibular evoked myogenic potentials (O-VEMPs), and video head impulse test (v-HIT) to show any selective damage of the vestibular nerve and also to identify if it is present in patients with a previous diagnosis of USH Type II. STUDY DESIGN: Prospective study with C-VEMPs, O-VEMPs, and v-HIT. SETTING: Tertiary referral center. PATIENTS: Fifteen patients with USH. INTERVENTION: Evaluation of otolith dysfunction with caloric test, C-VEMPs, and O-VEMPs and the measurement of the vestibular-ocular reflex using the v-HIT. RESULTS: Only three cases showed normal values of all the vestibular tests performed. O-VEMPs and C-VEMPs appeared pathologic in nine and seven cases, respectively. V-HITs showed ampullary dysfunction in 10 patients. In our study, eight of the 11 patients belonging to the group of USH Type II showed a pathologic response to at least one of the vestibular tests performed. CONCLUSION: Today, in patients affected by USH, any vestibular diagnostic protocol must include VEMPs and v-HIT to confirm the vestibular damage, identify selective deficit of the vestibular nerve, and provide useful information for a correct classification of USH.

Histological findings in a failed corneal riboflavin-UVA collagen cross-linking performed for progressive keratoconus.

PURPOSE: To report the histological and immunohistochemical findings in a cornea removed from a patient who had undergone collagen cross-linking (CXL) with riboflavin and ultraviolet-A for progressive keratoconus. CXL was performed following the Siena protocol. Two years post-CXL, a visual acuity impairment in the treated eye secondary to corneal stromal opacity had occurred, together with corneal thinning and flattening. METHODS: The excised cornea was formalin-fixed, paraffin-embedded, and examined microscopically. Deparaffinized 4-µm sections were stained with hematoxylin-eosin and Masson trichrome. Further tissue sections were subjected to immunohistochemical evaluation of CD34 and Ki-67 antigens. RESULTS: Histologically, there was no scar tissue in the failed cornea. The biomicroscopic stromal opacity corresponded microscopically to an acellular area, devoid of keratocytes, and to compaction of the lamellar collagen. Amorphous, weakly eosinophilic interlamellar deposits, extending from the anterior to the posterior two thirds of the stroma, were noted. CONCLUSIONS: CXL is a promising procedure for the treatment of progressive keratoconus with minimal reported side effects. In the present case, we speculate that the short corneal soaking time (15 minutes according to the Siena protocol) may have resulted in inefficient ultraviolet-A blocking, thermal injury, and deeper keratocyte death. Inadequate keratocyte stem cells reservoir could also play a role in individual cases.

Retinal pigment epithelium, age-related macular degeneration and neurotrophic keratouveitis.

Age-related macular degeneration (AMD) is the leading cause of impaired vision and blindness in the aging population. The aims of our studies were to identify qualitative and quantitative alterations in mitochondria in human retinal pigment epithelium (RPE) from AMD patients and controls and to test the protective effects of pigment epithelium-derived factor (PEDF), a known neurotrophic and antiangiogenic substance, against neurotrophic keratouveitis. Histopathological alterations were studied by means of morphometry, light and electron microscopy. Unexpectedly, morphometric data showed that the RPE alterations noted in AMD may also develop in normal aging, 10-15 years later than appearing in AMD patients. Reduced tear secretion, corneal ulceration and leukocytic infiltration were found in capsaicin (CAP)-treated rats, but this effect was significantly attenuated by PEDF. These findings suggest that PEDF accelerated the recovery of tear secretion and also prevented neurotrophic keratouveitis and vitreoretinal inflammation. PEDF may have a clinical application in inflammatory and neovascular diseases of the eye.

Alterações eletrofisiológicas na doença de Oguchi / Electrophysiological findings in Oguchi disease

Descrever as alterações eletrofuncionais em um caso raríssimo da Doença de Oguchi. Paciente do sexo feminino, italiana de 17 anos de idade se queixava de cegueira noturna. A resposta escotópica de bastonetes, do ERG era não registrável. A resposta escotópica ao estímulo branco forte demonstrava uma diminuição de amplitude da onda B. As respostas ao flicker de 30Hz e ao EOG eram dentro dos limites da normalidade. Era presente o fenômeno de Mizuo-Nakamura. Os exames eletrofuncionais são muito importantes no diagnóstico de certeza da doença de Oguchi. É nítida, no presente caso, a discordância entre EOG e ERG. Considerando a função dos bastonetes, as respostas normais do EOG contrastam com a ausência de respostas dos bastonetes em condições escotópicas no ERG. Mais estudos são necessários para entender o complexo mecanismo eletrofuncional dessa doença e melhor definir a origem dos componentes sensíveis à luz do EOG...

To describe the electrophysiological alterations in a very rare case of Oguchi's disease. A 17-year-old italian girl complaining of night blindness underwent complete ophthalmological exams, including electrophysiological tests. Rod responses were nondetectable in full-field electroretinogram (ERG). The photopic ERG funtions, including the 30 Hz flicker ERG response was normal, while the scotopic b-wave was diminished in amplitude. The electrooculography (EOG) ratios within the normal range were 208% in the right eye and 222% in the left eye. The Mizuo-Nakamura phenomenon was present. The electrophysiological tests are important tools in Oguchi's disease diagnosis. In the present case, it's clear the non correspondance between EOG and ERG. Considering the rod function, the normal EOG ratio contrast with non-detectable rod ERG responses. More studies are necessary to understand the compless electrofuntional mecanism of the disease helping to understand the origin of the light-sensitive component of the EOG...