PURPOSE: Low intraocular pressure (IOP) measured by Goldmann applanation tonometry (GAT) is one of the ocular manifestations of Steinert's myotonic dystrophy. The goal of this study was to evaluate the corneal-compensated IOP as well as corneal properties (central corneal thickness and corneal hysteresis) in patients with myotonic dystrophy. METHODS: A total of 12 eyes of 6 patients with Steinert's myotonic dystrophy (dystrophy group) and 12 eyes of 6 age-, race-, and gender-matched healthy volunteers (control group) were included in the study. GAT, Dynamic Contour Tonometry (DCT-Pascal) and Ocular Response Analyzer (ORA) were used to assess the IOP. Central corneal thickness was obtained by ultrasound pachymetry, and corneal hysteresis was analyzed using the ORA device. In light of the multiplicity of tests performed, the significance level was set at 0.01 rather than 0.05. RESULTS: The mean (standard deviation [SD]) GAT, DCT, and corneal-compensated ORA IOP in the dystrophy group were 5.4 (1.4) mmHg, 9.7 (1.5) mmHg, and 10.1 (2.6) mmHg, respectively. The mean (SD) GAT, DCT, and corneal-compensated ORA IOP in the control group was 12.6 (2.9) mmHg, 15.5 (2.7) mmHg, and 15.8 (3.4) mmHg, respectively. There were significant differences in IOP values between dystrophy and control groups obtained by GAT (mean, -7.2 mmHg; 99% confidence interval [CI], -10.5 to -3.9 mmHg; P<0.001), DCT (mean, -5.9 mmHg; 99% CI, -8.9 to -2.8 mmHg; P<0.001), and corneal-compensated ORA measurements (mean, -5.7 mmHg; 99% CI, -10.4 to -1.0 mmHg; P=0.003). The mean (SD) central corneal thickness was similar in the dystrophy (542 [31] µm) and control (537 [11] µm) groups (P=0.65). The mean (SD) corneal hysteresis in the dystrophy and control groups were 11.2 (1.5) mmHg and 9.7 (1.2) mmHg, respectively (P=0.04). CONCLUSIONS: Patients with Steinert's myotonic dystrophy showed lower Goldmann and corneal-compensated IOP in comparison with healthy individuals. Since central corneal thickness and corneal hysteresis did not differ significantly between groups, the lower IOP readings documented in this dystrophy seem not to be related to changes in corneal properties.

Keywords: Intraocular pressure; Tonometry, ocular; Myotonic dystrophy; Ocular hypotension; Cornea; Corneal topography

INTRODUCTION: Pediatric cataracts constitute an important cause of low visual acuity. PURPOSE: The objective of the present work is to know the characteristics of this pathology in an ophthalmic attention unit of a developing country. METHODS: A retrospective, descriptive, transversal study was carried out in 328 cases of pediatric cataracts (children between 0 and 14 years). RESULTS: 190 (57.9%) patients were male and 138 (42.1%) female, 145 (44.2%) cases were congenital cataracts and 183 (55.8%) acquired cataracts; 160 (48.8%) presented unilateral affection and 168 (51.2%) bilateral. The treatment was surgical in 98.2% of the cases. The mean age at diagnosis of congenital cataract was 34.9 months. The mean age of presentation of the acquired ones was 76.6 months. The treatment was abandoned in 70.58% cases of congenital cataracts and 64.10% of acquired cataracts. In the congenital ones, 44% presented visual acuity in the range of 20/200 and 20/70 and in the acquired ones, 32.5% were better than 20/30. CONCLUSIONS: The data found was not encouraging due to the late detection and the abandon of the treatment, which are bad prognosis factors.

Keywords: Eye diseases; Cataracts; Child; Developing countries

PURPOSE: To compare the achieved refractive outcomes of patients undergoing cataract surgery with intraocular lens (IOL) power calculation performed by conventional immersion ultrasound (US) or partial coherence interferometry (PCI). METHODS: Prospective, comparative case series. Patients undergoing cataract surgery were randomly divided in two groups with regard to the IOL power calculation method. Group 1 had calculations performed by PCI (IOL Master; Carl Zeiss Meditec), while US was used in Group 2 (Ultrascan; Alcon), using the Holladay 1 formula. Differences between target and achieved refractions were then compared. RESULTS: The study comprised 120 eyes from 79 patients. Biometry with PCI was used in 50 eyes of 33 patients, and US was used in 70 eyes of 46 patients. Mean age of patients in the PCI Group was 69.8 ± 13.1 years (range 11 - 85) and 70.0 ± 9.3 (45 - 86) in the US Group (P=0.7165). Mean axial length measured by PCI was 23.22 ± 1.00 mm (range 21.01 - 25.45) and that by US was 23.22 ± 1.06 mm (20.05 - 25.78) (P=0.9110). Mean absolute error in the PCI group was 0.15 ± 0.33 D (range -0.65 - 0.9) and that in the US group was 0.26 ± 0.48 D (-1.05 - 1.76). All eyes in the PCI group and 94.3% of those in the US group were within 1.00 D of the planned refraction. CONCLUSION: Although both PCI and US yielded good prediction in IOL power calculation, the PCI group tended to show better accuracy and improved refractive outcome.

Keywords: Diagnostic techniques, ophthalmological; Preoperative care; Cataract extraction; Lens implantation, intraocular; Refractive errors

PURPOSE: To analyze the results in patients reoperated from congenital and essential esotropia. METHODS: A retrospective chart review of 393 patients who underwent surgery from 2000-2004 was performed. Subjects were divided into two groups: Congenital esotropia (91patients) and essential esotropia (302 cases). RESULTS: Among congenital cases we had 9 reoperations (9.9%). There were undercorrections (3.3%), overcorrections (2.2%), anisotropia (V) (1.1%), hypotropia (1.1%) and dissociatd vertical divergences (2.2%). Among the essential cases, there were 31 (10.3%) reoperations due to undercorrections (n=6.6%), overcorrections (n=2%) and hypotropias (1.7%). CONCLUSIONS: Outcomes reoperations rates were 9.9% and 10.2% between congenital and essential esotropias with a higher rate of undercorrections. Amblyopia in both groups and deviations higher than 50∆ in essential esotropias seems to be the most important factors for poor results.

Keywords: Esotropia; Esotropia; Oculomotor muscles; Ophthalmologic surgical procedures; Vision, binocular; Reoperation

PURPOSE: To describe the procedures used in developing Clinical and Regulatory Protocols for primary care teams to use in the management of the most common scenarios of impaired vision in Southern Brazil. METHODS: A retrospective review of 1.333 referral forms from all primary care practitioners was performed in Ribeirão Preto city, during a 30-day period. The major ophthalmic diagnostic categories were evaluated from those referrals forms. The Clinical and Regulatory Protocols development process was held afterwards and involved scientific cooperation between a university and the health care system, in the form of workshops attended by primary care practitioners and regulatory system team members composed of health care administrators, ophthalmologists, and professors of ophthalmology and social medicine. RESULTS: The management of impaired vision was chosen as the theme, since it accounted for 43.6% of the ophthalmology-related referrals from primary care providers of Ribeirão Preto. The Clinical and Regulatory Protocols developed involve distinctive diagnostic and therapeutic interventions that can be performed at the primary care level and in different health care settings. The most relevant clinical and regulatory interventions were expressed as algorithms in order to facilitate the use of the Clinical and Regulatory Protocols by health care practitioners. CONCLUSIONS: These Clinical and Regulatory Protocols could represent a useful tool for health systems with universal access, as well as for health care networks based on primary care and for regulatory system teams. Implementation of these Clinical and Regulatory Protocols can minimize the disparity between the needs of patients with impaired vision and the treatment modalities offered, resulting in a more cooperative health care network.

Keywords: Vision disorders; Delivery of health care; Public Health; Health Services; Primary health care; Health systems; Clinical protocols

PURPOSE: To evaluate the influence of the oblique muscles surgical weakening on the horizontal alignment in the primary position (PP) and its efficacy on the correction of the "A" and "V" anisotropies. METHODS: In order to study the influence of bilateral superior oblique muscles (SO) weakening on the horizontal alignment in PP, we analyzed the files of 12 patients who underwent only that operation; no other muscle was operated on. We took the opportunity of those 12 patients to analyze the effect of their operation on the correction of "A" incomitance. For evaluating the effect of the inferior oblique muscles (IO) weakening on the correction of the "V" pattern, we analyzed retrospectively the files of 67 anisotropic patients who underwent a bilateral SO weakening. In 10 of them, the only operation was the oblique muscles weakening and, in 57 patients, the horizontal recti were also operated on for the horizontal deviations in primary position. These patients were divided into two groups: 50 were esotropic and 17 exotropic. There was not any mixed anisotropy. RESULTS: The mean value of the preoperative "V" incomitance of the 50 esotropic patients was 24.25∆ ± 10.15∆; the mean postoperative correction was 15.56 ∆ ± 8.74∆. The mean correction between the PP and upgaze was 7.52∆ ± 7.47∆ and from the PP to downgaze was 8.56∆ ± 9.21∆. The same values of the 17 exotropic patients was: preoperative 31.88∆ ± 9.4∆; primary position to upgaze was 13.11∆ ± 4.9∆ and primary position to downgaze 14.11∆ ± 12.48∆. The mean preoperative value of the "A" incomitance among the 12 patients who underwent isolated SO weakening was 30.50∆ ± 19.25∆ and the postoperative was of 9,92∆, therefore a mean correction of 22.58∆ ± 17.54∆. Among these ones, in 5 there was no alteration of the deviation in primary position, in 4 there was an exo-effect and in 3 there was an eso-effect. The mean alteration of the deviation in PP was an exo-shift of 2.25∆. CONCLUSIONS: 1) There was no difference in the relative correction of "V" pattern between the esotropic and exotropic patients, although the absolute numbers were greater in the exotropic ones. 2) The correction of the "V" pattern was between 65 and 75 % from the initial values. 3) The IO weakening corrected similarly the anisotropia between the superior and inferior components among the esotropic patients, but among the exotropic ones the correction was greater in the inferior component. 4) The SO weakening did not cause an eso-effect in the deviation in primary position.

Keywords: Oculomotor muscles; Ophthalmologic surgical procedures; Strabismus

INTRODUCTION: Ipsilateral rotational penetrating autokeratoplasty technique may be an alternative to minimize the problem of long waiting lists for transplantation due to keratoconus. PURPOSE: To report the visual outcomes in keratoconus patients after ipsilateral rotational penetrating autokeratoplasty technique (IRPAK) associated with a crescent-shape resection of 0.5 mm of the inferior cornea. METHODS: According to a prospective, longitudinal, interventional, analytic study, the authors investigated the visual results of 15 patients (18 eyes) with keratoconus, who underwent an ipsilateral rotational penetrating autokeratoplasty, by two coincident trepanations superiorly and with a difference of 0.5 mm inferiorly. After resection of this corneal crescent, a 180° rotation of the corneal disk was performed, which was fixed with 24 sutures diametrically opposed. The patients were followed-up for a period of 24 months. RESULTS: Comparing data of the first and second postoperative years to preoperative data, there were: significant improvements in visual acuity, vision lines and spherical equivalent; reduction of topographic astigmatism and maximum and mean corneal curvatures. There was also a decrease in corneal thickness associated to a reduction of corneal endothelial cell count. CONCLUSION: There was an improvement of ophthalmologic parameters 6, 12 and 24 months after ipsilateral rotational penetrating autokeratoplasty associated with a crescent-shape resection of 0.5 mm of the inferior cornea, but along the follow-up there was reduction of visual acuity with increased astigmatism.

Keywords: Keratoconus; Keratoplasty, penetrating; Cornea; Vision, binocular; Transplantation, autologous; Visual acuity; Follow-up studies

PURPOSE: The primary objective of this case series was to get electroretinographic data in subjects with sickle cell disease. The secondary purpose was to identify ocular changes, their frequency and stage of evolution. METHODS: 90 patients aged between 8 and 75 years were evaluated. They were subjected to complete ophthalmological examination, indirect ophthalmoscopy, retinography, angiography and electroretinography. Electroretinograms with amplitude of wave b less than 400 mW were considered subnormal. Prevalence study was conducted using the Chi-square test, P<0.001. RESULTS: The electroretinogram was subnormal in 27 (30%) of 90 patients, but of these, only 4 (4.4%) had proliferative retinopathy. The sign of comma in 95% of individuals, the retinal vascular tortuosity in 36.6% and black sunburst in 24.4% were the most frequent changes. CONCLUSION: No correlation between sickle cell disease and electroretinographics changes, as described in other studies, were noted. On the other hand, the ocular findings corroborate the previous researches.

Keywords: Hemoglobin SC disease; Retina; Retinal diseases; Retinal vessels; Electroretinography

PURPOSE: To report the results of Descemet stripping endothelial keratoplasty (DSEK) using the TAN EndoGlideTM device to facilitate the insertion of the endothelial membrane. METHODS: Prospective clinical study that included nine patients presenting corneal edema secondary to endothelial dysfunction. Best corrected visual acuity, refraction, central corneal thickness, endothelial cell density and complications were analyzed after a six-month follow-up. RESULTS: There was a significant improvement in the corneal edema and visual acuity in 7 patients (77.78%). The best corrected visual acuity ranged between 20/40 and 20/200. The average density of endothelial cells in six months varied between 1,305 cells/mm² and 2,346 cells/mm² with an average loss of 33.14% cells. Detachment of part of the graft was observed in one eye (11.11%) and primary failure of the endothelial transplantation occurred in 2 eyes (22.22%). CONCLUSION: The device TAN EndoGlideTM facilitates the introduction of the graft in Descemet stripping endothelial keratoplasty.

Keywords: Corneal transplantation; Corneal edema; Corneal endothelial cell loss; Descemet membrane; Descemet stripping endothelial keratoplasty

PURPOSE: A microdevice for the treatment of refractory glaucoma is presented. The underlying concepts, its mechanisms of action and the surgical technique for implanting are explained and the results are analyzed. The microdevice was developed and the surgeries were performed at the Maldonado-Bas Eye Clinic (Cordoba, Argentina), under the rules established in the protocol approved by the provisions of the National Administration of Drugs, Food and Medical Technology 430/7. File No.:1-47-25-649-07-1. METHODS: In a prospective study, following the protocol, 16 eyes with refractory glaucoma were included and operated. Intraocular pressure <21mmHg with or without additional medication was considered successful. The follow-up was one year. Averages, percentages and their 95% confidence bands were calculated. Analysis of variance for repeated measures was used to compare averages. RESULTS: The average preoperative intraocular pressure was 32.81 mmHg, SD ± 10.94 mmHg in a range of 14 to 50 mmHg. The average post-surgical intraocular pressure at one year was 12.43 mmHg, SD ± 2.85 mmHg in a range of 7 to 19 mmHg. The difference between the pre-and post-surgery average intraocular pressure was 20.38 mmHg. The number of successes was 14 eyes (87.5%, confidence interval (CI) 95% 61.6% - 98.6%). The number of failures was two eyes (12.5%, CI 95% 1.43% - 38.4%). CONCLUSIONS: The results show that the microdevice is successful for the treatment of refractory glaucoma.

Keywords: Glaucoma; Filtering surgery; Intraocular pressure; Trabeculectomy; Aqueous humor; Optical devices; Sclera; Sclerostomy

Hemangiomas are the most common benign tumors of infancy. Despite their self-limited course, infantile capillary hemangiomas can impair vital or sensory functions as vision and cause cosmetic deformity. The usual treatments include oral/intralesional steroids, alpha interferon, cytotoxins, pulsed dye laser and cosmetic surgery resection. These treatments are not free of multiple complications and toxic side effects. This report describes the case of a 3-month-old female baby with progressively increasing hemangioma of the left upper eyelid impinging over the visual field. The hemangioma promptly responded to low-dose oral propranolol. A clinical response was noticed few days after the beginning of the treatment, with regression to 1/4 of its original size in 45 days of treatment, and to less than 1/10 after 8 months, free of any major side effects.

Keywords: Hemangioma; Hemangioma, capillary; Propranolol; Case reports; Infant, newborn

Waardenburg syndrome (WS) type I is a non-progressive auditory-pigmentary disorder comprising congenital sensorineural hearing loss and pigmentary disturbances of the iris, hair, and skin, along with dystopia canthorum (lateral displacement of the inner canthi). Affected individuals may have higher risk of: neural tube defects, cleft lip and palate, limb abnormalities, and Hirschsprung disease. The diagnosis is clinical and should be considered if the individual has two major or one major plus two minor criteria. PAX3 is the only known gene associated to the syndrome. Nevertheless, its use is mostly for genetic counseling. Regarding different diagnosis, we may list: other causes of non-progressive auditory-pigmentary disorder comprising congenital sensorineural hearing loss, other types of Waardenburg syndrome, piebaldism, albinism, vitiligo and Teitz syndrome. This paper presents a case of an eleven year old boy with deafness and ophthalmologic alterations, based on his files and exams. It reinforced the importance of the ophthalmologist contributing for the diagnosis of this rare systemic disease, as it includes some ophthalmologic alterations. We remind that the early diagnosis allows adequate stimulation for the hearing loss, as well as preventive measures in case of pregnant women affected by genetic counseling.

Keywords: Waardenburg´s syndrome; Iridocyclitis; Eyebrows; Eyelids; Nose; Paired Box transcription factors; Humans; Male; Child; Case reports

The authors report the case of a 5 year-old boy who up to 2 years old presented normal eyes, when his right eye started to deviate upward and laterally, until be hidden under the superior lid. At the surgery, a strong passive limitation to infraduction of this eye was felt. He had already been operated on in another clinic, but the surgeon could not succeed in hooking his superior rectus. With great difficulty, the only thing that we could do was a free tenotomy of the superior rectus. As the eye was equilibrated in a moderate abduction, we performed an 8 mm recession of the lateral rectus. As there was still a small hypertropia and exotropia postoperatively, we performed in a second operation an eight millimeters resection of the medial rectus, a recession with anterior transposition of the inferior oblique and an advancement of the inferior rectus according to the Romero-Apis technique, in order to avert circulatory problems to the anterior segment of the eye. As he presented a small blepharoptosis, we did, in a third surgery, a tarsectomy according to the Fasanella-Servat technique, with good result. He ended with good eye position, almost no limitation of the horizontal movements and - 3 limitation of up and down ductions. The magnetic resonance imaging showed an altered image of the superior rectus, suggesting fibrosis post myositis.

Keywords: Oculomotor muscles; Strabismus; Blepharoptosis; Fibrosis; Ophthalmologic surgical procedures; Case report; Human; Male; Child

A case of a 53 year-old female patient who developed branch retinal vein occlusion after six 3 mg/kg Infliximab infusions for rheumatoid arthritis treatment is reported. Hematological disorders, cardiopathy and chronic systemic hypertension evaluation were negative. The relationship between the infliximab therapy and the development of retinal vein occlusion raises awareness of the possibility of a potential side effect of this medication.

Keywords: Rheumatoid arthritis; Antibodies, monoclonal; Antibodies, monoclonal; Retinal vein occlusion; Case reports

Retinopathy of prematurity (ROP) is related to oxygen-regulated vascular endothelial growth factor and to insulin-like growth factor-I. After premature birth, supplemental oxygen induces a retinal hyperoxic condition with vasoconstriction and to a definitive interruption of retinal vasculogenesis. Peripheral ischemia may stimulate retinal neovascularization and the onset of additional ROP-related complications. The natural course of the disease may result in irreversible blindness if not promptly diagnosed and attended. Recently, a significant increase in the prevalence of ROP has been observed in survival rates of preterm infants, especially in emerging-economy countries in Latin America, Asia, and Eastern Europe. This article addresses the main preventive measures in ROP.

Keywords: Retinopathy of prematurity; Blindness; Risk factors

The pattern electroretinogram is an electrophysiological test that assesses the function of inner retinal layers, particularly the ganglion cells layer of retina, using a reversing checkerboard or grating pattern that produces no change in average luminance over time. The normal pattern electroretinogram is composed of a proeminent positive component (P50) and a large later negative component (N95). Since structural damage that compromises the retinal ganglion cell layer can lead to pattern electroretinogram changes, particularly in the N95 amplitude, the test can be useful in the treatment of a number of anterior visual pathway diseases. In this article, we review the methods for recording pattern electroretinogram and its usefulness in the diagnosis and management of diseases including inflammatory, hereditary, ischemic and compressive lesions of the anterior visual pathway.

Keywords: Electroretinography; Optic nerve injuries; Retina; Optic nerve diseases; Pattern recognition, visual; Vision Disorders; Visual Pathways