The socioeconomic epidemiology of inherited retinal diseases in Portugal.

BACKGROUND: Inherited retinal diseases (IRDs) are a group of rare degenerative disorders of the retina that can lead to blindness from birth to late middle age. Knowing the target population and its resources is essential to better plan support measures. The aim of this study was to evaluate the socioeconomic characteristics of regions in Portugal where IRD patients reside to inform the planning of vision aid and rehabilitation intervention measures. RESULTS: This study included 1082 patients from 973 families, aged 3 to 92 years, with a mean age of 44.8 ± 18.1 years. Patients living with an IRD were identified in 190 of the 308 municipalities. According to this study, the estimated IRD prevalence in Portugal was 10.4 per 100,000 inhabitants, and by municipalities, it ranged from 0 to 131.2 per 100,000 inhabitants. Overall, regions with a higher prevalence of IRD have a lower population density (r=-0.371, p < 0.001), a higher illiteracy rate (r = 0.404, p < 0.001) and an overall older population (r = 0.475, p < 0.001). Additionally, there is a lower proportion of doctor per capita (r = 0.350, p < 0.001), higher social security pensions beneficiaries (r = 0.439, p < 0.001), worse water quality for human consumption (r=-0.194, p = 0.008), fewer audiences at the cinema (r=-0.315, p < 0.001) and lower proportion of foreign guests in tourist accommodations (r=-0.287, p < 0.001). CONCLUSION: The number of identified patients with IRD varied between regions. Using data from national statistics (PORDATA), we observed differences in socioeconomic characteristics between regions. Multiple targeted aid strategies can be developed to ensure that all IRD patients are granted full clinical and socioeconomic support.

Case report: Introduction of an Ahmed valve implant tube into the ciliary sulcus using a guide-wire.

BACKGROUND: Glaucoma is a progressive optic neuropathy and it is the main cause of irreversible blindness worldwide. Intraocular pressure (IOP) is the only modifiable one and trabeculectomy is commonly considered the surgical "gold standard" to decrease IOP. In particular cases, other kind of surgeries, as the Ahmed Glaucoma Valve (AGV) implant, are a reliable alternative. Usually, the silicone tube of AGV surgery is inserted into the anterior chamber. Sometimes it is necessary to place the AGV silicone tube in the ciliary sulcus, especially in cases of endothelial decompensation. This surgical procedure is not always easy to perform. CASE PRESENTATION: This article describes and presents a technique for inserting the AGV tube into the posterior chamber using a guide-wire, in a total of 12 cases are reported. This procedure has been mostly applied in a group of pseudophakic patients who need AGV placement to control elevated IOP. In exceptional situations, this procedure was applied in pseudophakic patients previously with AGV tube implanted in the anterior chamber and with loss of endothelial cells or in phakic patients with previously implanted AGV anterior chamber tube, simultaneously to cataract surgery. CONCLUSION: The purpose of these cases presentations attended at our service is to demonstrate the passage of the AGV silicone tube was performed with the aid of a guide wire.

The Visual Impairment of Inherited Retinal Diseases in Portugal as per the National Table of Disabilities.

Purpose: To evaluate the visual impairment of patients with inherited retinal diseases (IRDs), as per the national table of disabilities (TNI). Design: Retrospective, single-center cohort study. Participants: Patients with a clinical diagnosis of IRD were recruited at a referral center in Portugal. Methods: Demographics and clinical data were collected from each individual patient file. The estimated visual disability coefficient was calculated through the evaluation of 7 graduated categories: orbital or eyelid deformities, low vision, visual field change, loss of bi-foveolar fixation, oculomotor palsy, photophobia, and chronic conjunctivitis. The TNI provides minimum and maximum disability values for numerous conditions within each category, which were summed to calculate an overall summary disability coefficient for each patient. Main Outcome Measures: Demographic/clinical and estimated minimum and maximum visual disability coefficient according to the TNI for each patient. Results: This study included 253 patients from 214 families, aged 3 to 80 years, with a mean age of 39.8 ± 20.0 years. The mean estimated minimum and maximum visual disability coefficients as per the TNI were 0.6 ± 0.4 and 0.7 ± 0.4, respectively. The low vision was the single most frequent contributor category (21.7%) present in the calculation of visual impairment. Low vision and visual field changes were the most frequent double combination (18.2%), and the addition of loss of bi-foveolar fixation was the most frequent triple combination (8.3%). Conclusions: This study found that IRD patients had a significant visual disability, with the majority having a disability coefficient ≥0.6, which would qualify them for a "multipurpose disability medical certificate." Financial Disclosures: The authors have no proprietary or commercial interest in any materials discussed in this article.

Pigment deposits in the retrolental space of Berger: a rare feature of pigment dispersion syndrome? / Depósitos de pigmento no espaço de Berger: uma característica rara de síndrome de dispersão pigmentar?

ABSTRACT Pigment dispersion syndrome is associated with clinical features such as Krukenberg's spindles, trabecular pigmentation, Scheie's stripe and Zentmayer's ring. Another less common feature of this syndrome is retrolental pigment deposits due to anterior hyaloid detachment or a defect in the Wieger's ligament. We present two cases of pigment deposits on the posterior lens capsule. In both cases, there is bilateral dispersion of pigment throughout the anterior segment. The retrolental deposits are unilateral in the first case and bilateral in the second. Both patients report a history of ocular trauma. This is a possible important clinical sign of pigment dispersion syndrome, rarely described.

RESUMO A síndrome de dispersão pigmentar associa-se a sinais clínicos característicos como fuso de Krukenberg, hiperpigmentação da malha trabecular, linha de Scheie e anel de Zentmeyer. Um sinal menos comum dessa síndrome é o depósito de pigmento posterior ao cristalino, que ocorre por um descolamento da hialoide anterior ou um defeito no ligamento de Wieger. Apresentamos dois casos de depósitos de pigmento posterior à cápsula posterior do cristalino. Em ambos os casos, existia dispersão bilateral de pigmento por todo o segmento anterior. No primeiro caso, os depósitos eram unilaterais e, no segundo, estavam presentes em ambos os olhos. Este pode corresponder a um sinal potencialmente importante da síndrome de dispersão pigmentar, raramente descrito.

Ocular Surface Changes in Primary Open Angle Glaucoma Patients Treated With Topical Antihypertensive Drugs.

PRCIS: In this study, patients with glaucoma undergoing topical antihypertensive (TAH) drugs had changes in the ocular surface and more dry eye symptoms than controls. Clinicians should recognize the influence of TAH drops on exacerbating ocular surface disease. PURPOSE: The purpose of this study was to evaluate the ocular surface of eyes with glaucoma treated with TAH drugs. METHODS: Cross-sectional study that included eyes undergoing TAH drugs due to primary open angle glaucoma and controls. The parameters evaluated were: the basal tear flow (basic secretion test); the tear film osmolarity (TearLab); and the noninvasive break-up time, blink score, lipid layer thickness, tear meniscus height, and loss area of the meibomian glands, measured with the IDRA Ocular Surface Analyser. Presence of symptoms [Ocular Surface Disease Index (OSDI)], dry eye disease (DED, TFOS DEWS II criteria), and corneal fluorescein staining were assessed. RESULTS: We included 154 eyes (154 patients), 77 undergoing TAH drugs for glaucoma (group 1) and 77 of controls (group 2). The tear film osmolarity ( P =0.003) and the loss area of the meibomian glands ( P =0.004) were higher in group 1. The noninvasive break-up time ( P =0.005), lipid layer thickness ( P =0.006), and tear meniscus height ( P =0.001) were lower in group 1. The global OSDI score ( P <0.001), the proportion of eyes with severe disease ( P =0.002), according to the OSDI, and with DED ( P <0.001), according to the TFOS DEWS II criteria, were higher in group 1. The proportion of patients with corneal fluorescein staining was higher in group 1 ( P <0.001). There were no significant differences in eyes taking TAH drugs with and without preservatives ( P >0.127). CONCLUSIONS: DED, in patients with glaucoma, is a multifactorial disease, with a strong contribution from TAH drugs. These eyes had changes in almost every measured parameter, translating into the presence of more dry eye symptoms and corneal damage when compared with controls.

Modified Technique of Ex-PRESS® Filtration Device Combined with a Scleral Pocket for Hereditary Transthyretin Amyloidosis (hATTR) Secondary Open-Angle Glaucoma.

Purpose: To evaluate the effectiveness and safety of a modified approach using the Ex-PRESS® implant combined with a scleral pocket in the management of secondary open-angle glaucoma in hereditary transthyretin amyloidosis (hATTR) at our department. Methods: This was a retrospective analysis. The primary endpoints included Intraocular pressure (IOP) evaluation (baseline, 1st day, 1st week, 1, 3, 6, 12 months and at last follow-up) and number of hypotensive drugs (baseline, 6th, 12th months and at last follow-up). As secondary endpoints surgical complications, the need for additional glaucoma surgery and LogMAR BCVA were evaluated. Qualified and complete success were defined as ≥ 30% IOP decrease from baseline, with or without additional medications, respectively. The minimum follow-up was 12 months. Results: A total of 32 eyes were included with a mean follow-up of 2.4±2.9 years. IOP decreased significantly from baseline (27.4±4.4 mmHg) to 1st day (5.00±2.9 mmHg), 1st week (6.9±4.1 mmHg), 1st month (11.7±7.8 mmHg), 3rd month (11.6±6.1 mmHg), 6th month (13.1±6.8 mmHg), 12th month (12.0±3.5 mmHg) and last visit (11.8±2.4 mmHg), p<0.001. There was also a significant reduction in the number of antiglaucoma medications from baseline (3.8±0.6) and last follow-up (0.4±0.8), p<0.001. LogMAR BCVA remained stable (0.25±0.26 at baseline and 0.25±0.24 at last follow-up), p=0.767. Transient hypotony occurred in 17 eyes (53.1%), but only 11 (34.4%) exhibited anterior chamber shallowing and needed additional care, namely cycloplegic drops and viscoelastic injection. Complete surgical success was achieved in 22 eyes (68.8%) and qualified success in 6 eyes (18.8%). Four eyes (12.5%) needed additional glaucoma surgery. Conclusion: The modified ExPRESS® technique appears to be effective, especially when low levels of IOP are required. Additionally, fewer anti-glaucoma drugs were necessary. In the other hand, hypotony was a common side effect with this procedure, although all patients were properly handled, preserving the surgical outcomes.

Monitoring the Patient with Retinal Angiopathy Associated with Hereditary Transthyretin Amyloidosis: Current Perspectives.

Retinal angiopathy associated with hereditary transthyretin amyloidosis (ATTRv), if untreated, may lead to irreversible vision loss. Our purpose was to systematically review the clinical and imaging features of retinal angiopathy associated with ATTRv and assemble a monitoring approach for these patients. All types of original research studies reporting clinical and imaging findings on retinal angiopathy associated with ATTRv were included. The most common clinical findings were tortuous retinal vessels, microaneurysms, retinal hemorrhages, sheathing of retinal vessels, whitish amyloid deposits along retinal arteries, obliteration of retinal vessels, vitreous hemorrhage, retinal and iris neovascularization. The most relevant imaging findings were hyperautofluorescence of perivessel amyloid deposits; delayed arterial filling, vascular leakage, and retinal ischemia on fluorescein angiography; late hypercyanescence along the choroidal arteries on indocyanine green angiography; perivascular hyperreflective material, needle-shaped deposits on the retinal surface and macular edema on optical coherence tomography (OCT) and attenuated retinal vascular network on OCT-angiography. ATTRv patients should be strictly followed to detect and treat retinal angiopathy, avoiding complications. Both panretinal photocoagulation and intravitreal anti-vascular endothelial growth factor have been used to treat retinal angiopathy in ATTRv. In an individual that presents with retinal angiopathy of unknown etiology, ATTRv should be considered as in the differential diagnosis, even out of the initial core countries. The prognostic value of subclinical findings, namely in OCT-A, is not yet established.

Corneal Biomechanics for Ocular Hypertension, Primary Open-Angle Glaucoma, and Amyloidotic Glaucoma: A Comparative Study by Corvis ST.

BACKGROUND: To evaluate biomechanical parameters of the cornea provided by Corvis ST in patients with ocular hypertension, primary open-angle glaucoma, and amyloidotic glaucoma and to compare with healthy controls. METHODS: This was a cross-sectional study of patients with ocular hypertension, primary open-angle glaucoma, and amyloidotic glaucoma that underwent Corvis ST imaging. Primary outcome was the comparison of corneal biomechanical parameters between study groups after adjusting for age, gender, Goldmann intraocular pressure (GAT-IOP), and prostaglandin analogues medication. Secondary outcome was the comparison of different IOP measurements in each group. RESULTS: One hundred and eighty-three eyes from 115 patients were included: 61 with primary open-angle glaucoma, 32 with amyloidotic glaucoma, 37 with ocular hypertension and 53 were healthy controls. Amyloidotic glaucoma group had smaller radius (p=0.025), lower deflection amplitude at highest concavity (p=0.019), and higher integrated radius (p=0.014) than controls. Ocular hypertension group had higher stiffness parameter at first applanation (p=0.043) than those with primary open-angle glaucoma, and higher stress-strain index (p=0.049) than those with amyloidotic glaucoma. Biomechanically corrected intraocular pressure was significantly lower than Goldmann intraocular pressure in group with primary open-angle glaucoma (p=0.005) and control group (p=0.013), and Goldmann intraocular pressure adjusted for pachymetry in group with primary open-angle glaucoma (p=0.01). CONCLUSION: Eyes with amyloidotic glaucoma have more deformable corneas, while eyes with ocular hypertension have less deformable corneas. These findings may be linked to the susceptibility to glaucomatous damage and progression. There were significant differences between Goldmann applanation tonometry and biomechanically corrected intraocular ocular pressure provided by Corvis ST.

Pupillometry: An objective test to assess endocular hereditary transthyretin amyloidosis.

OBJECTIVE: To automatically study the pupillary light reflex in patients with hereditary transthyretin-associated amyloidosis (hATTR). METHODS: Prospective cross-sectional observational study in patients with hATTR with unilateral scalloped iris. Pupillary light reflex of scalloped iris eyes (21 eyes) were compared with non-scalloped iris eyes (21 eyes, paired eyes of the same patients) and also with a control group of 20 healthy eyes, using static and dynamic pupillometry with the Metrovision® MonPack One. RESULTS: No patient presented evident neurological involvment of the cranial nerves. No significant differences were found in the pupillary diameters under standardized lighting conditions (static pupillometry) among groups. In dynamic pupillometry, the amplitude of contraction, the velocity of contraction and the velocity of dilation were statistically significantly lower in eyes with scalloped iris, comparing both with the contralateral non-scalloped iris eyes (p < 0.001 for all) and with eyes from healthy subjects (p < 0.05 for all). CONCLUSION: A scalloped iris reflects a more advanced endocular hATTR and it is associated with an altered pupillary light reflex. Pupillometry may be a quick, simple, and portable test to objectively evaluate ocular amyloid deposition in hATTR eyes. Pupillary light reflex may not be reliable to evaluate neurological dysfunction in these patients.

Ahmed valve for secondary glaucoma in patients with hereditary transthyretin amyloidosis.

OBJECTIVE: To evaluate the surgical outcomes of patients with hereditary transthyretin amyloidosis (TTR-FAP) who underwent Ahmed glaucoma valve (AGV) implantation. METHODS: A retrospective cohort study was performed on patients with a diagnosis of TTR-FAP secondary glaucoma, who underwent AGV implantation in our department, between November 2010 and July 2019. The cumulative probability of treatment success was measured with Kaplan-Meier survival analysis. The primary outcome was success, defined as intraocular pressure (IOP) ≥ 6 mmHg and ≤21 mmHg with or without medication, with no need for further glaucoma surgery and without loss of light perception at last follow-up. Secondary outcomes were postoperative IOP, number of IOP-lowering medications, and rates of complications. RESULTS: The study included 114 eyes of 87 patients. The mean follow-up duration was 3.81 ± 2.11 years (y) [range, 1.00-8.28 y]. Compared to the preoperative values, the mean IOP was reduced from 28.20 ± 7.01 to 12.87 ± 3.76 mmHg at the final visit (p < 0.001), with a reduction in the number of medications from 3.89 ± 0.66 to 1.86 ± 1.43 (p < 0.001). Early and late postoperative complications occurred in 20 (17.09%) and 9 (7.89%) eyes, respectively. Kaplan-Meier analysis indicated probabilities of success of 0.98 at 1 y, 0.97 at 2 y, 0.95 at 3 y, 0.89 at 4 y, 0.77 at 5 y and 0.72 at 6 y. The linear correlation analysis showed a correlation between some characteristics of the natural history of TTR-FAP patients and AGV implantation success. CONCLUSION: Although glaucoma in TTR-FAP patients is very difficult to manage, AGV implantation is an effective and relatively safe procedure.

XEN Gel Stent Implantation in Eyes With Previous Glaucoma Filtering Surgeries: A Case Series.

Background In this study, we aimed to analyze the efficacy and safety of ab interno XEN® gel stent (Allergan Inc., Dublin, Ireland) implantation in a series of eyes with open-angle glaucoma and a history of previous filtering glaucoma surgeries. Methodology This retrospective, single-center study included all eyes that underwent XEN gel implantation with a minimum follow-up of 18 months that had previously undergone a filtering glaucoma surgery. The main outcomes were intraocular pressure (IOP) variation (baseline, first day, first week, and first, third, sixth, 12th, and 18th months), the number of hypotensive eyedrops at 18 months, intraoperative and postsurgical complications, and the need for subsequent glaucoma surgery. Results A total of 10 eyes from nine patients were enrolled in the study. IOP significantly decreased from 24.0 ± 2.4 mmHg to 7.8 ± 1.6 mmHg on the first day, 9.4 ± 2.2 mmHg in the first week, and 12.3 ± 4.8 mmHg, 14.7 ± 4.3 mmHg, 13.3 ± 3.8 mmHg, 11.7 ± 1.9 mmHg, and 12.3 ± 1.9 at the first, third, sixth, 12th, and 18th month, respectively (p < 0.001), with a 49.5% reduction in IOP at the end of the follow-up. The number of hypotensive eyedrops decreased significantly from 3.5 ± 0.5 (3-4) to 0.7 ± 0.7 (0-2) (p = 0.026). No intraoperative or postsurgical complications were recorded. In total, seven (70%) eyes achieved controlled IOP of <18 mmHg without any anti-glaucomatous medications, and one (10%) eye with the use of topical prostaglandin. One (10%) eye was submitted to a surgical revision, and another needed an additional glaucoma surgery during follow-up, with appropriate IOP control at the last follow-up. Conclusions This case series aims to highlight that the XEN implant can be an option in eyes with IOP levels in the low 20s and previously failed filtering surgeries, with satisfactory efficacy and high safety.

Biometric Predictability in Combined Cataract Surgery and Ahmed Glaucoma Valve Implantation Depending on Tube Position.

PURPOSE: To compare the predictability of biometric results in patients undergoing cataract surgery combined with Ahmed glaucoma valve (AGV) implantation according to tube position. METHODS: A retrospective cohort study was performed in patients who underwent phacoemulsification surgery combined with AGV implantation with the tube in posterior (group PC) and anterior (group AC) chamber, between November 2012 and April 2020. The main outcome was the mean biometric prediction error, according to tube position, using different formulas. RESULTS: The study included 49 eyes of 36 patients, 23 eyes in group PC and 26 eyes in group AC. Gender (p=0.774), age (p=0.822), type of glaucoma (p=0.168), preoperative correct distance visual acuity (p=0.139), axial length (p=0.765), anterior chamber depth (p=0.351), keratometry (p=0.577) and intraocular lens power (p=0.608) were similar between groups. Only preoperative intraocular pressure was higher in group PC (p=0.005). The mean prediction errors using Haigis, SRK/T, Hoffer Q, Holladay 1, Barrett Universal II, Kane and Hill RBF formulas were all positive in group PC (hyperopic) and all negative (myopic) in group AC. In group PC, there was no significant difference in prediction error between these formulas (p>0.05). In group AC, the formula with the worst prediction error was Haigis (p=0.001), and the best was Barrett Universal II (p=0.043). CONCLUSION: The biometric predictability and expected final refraction in phacoemulsification surgery combined with AGV implantation are modified by the position of the tube.

The Role of Multimodal Approach in the Assessment of Glaucomatous Damage in High Myopes.

PURPOSE: To compare the optic disc structure and peripapillary retinal function between high myopes with and without glaucoma and to address the differential role of papillary optical coherence tomography angiography (OCT-A) and circumpapillary microperimetry (cpMP) on the diagnosis and prognosis of this patients. PATIENTS AND METHODS: It is a cross-sectional study including 30 high myopic patients (60 eyes), divided into 15 with (GG) and 15 without glaucoma (NGG). Demographic and clinical data were collected from patient records. Papillary structure (peripapillary retinal nerve fiber layer thickness, ppRNFLTs) and vascularization (small vessel densities, SVD´s) were assessed with SD-OCT (RTVue XR Avanti, with AngioVue system, Optovue®). cpMP was carried out with the MP-3 microperimeter (Nidek®). RESULTS: The GG were older, had lower best-corrected visual acuities and higher intraocular pressures and axial lengths (p<0.001). The GG showed lower values in all ppRNFLTs (p<0.05), lower values in all SVDs (p<0.001), except the SVD-inside disc (p=0.638) and lower retinal sensitivities within all cpMPs (p<0.001). The adjusted analysis computing the best two parameters per exam revealed that the anatomical model including the ppRNFLT-inferior and ppRNFLT-temporal and the vascular model including SVD-inferior and SVD-superior had the best discrimination power between groups, with cross-validated AUROCs of 0.9599 and 0.9921, respectively. CONCLUSION: Despite the apparent superiority of the papillary vascular study, a multimodal approach including the papillary anatomic and circumpapillary microperimetric assessments can be the new way on the diagnosis and prognosis of glaucoma in high myopia.

Return of phacoemulsification after emergency status related to COVID-19: experience of a tertiary referral center.

PURPOSE: To present a protocol of priority criteria for phacoemulsification after the backlog due to severe acute respiratory syndrome coronavirus (SARS-CoV)-2 pandemic status. SETTING: Ophthalmology department of Centro Hospitalar e Universitário do Porto (CHUP), Oporto, Portugal. DESIGN: Cross-sectional, nonrandomized, retrospective study. METHODOLOGY: Data of all patients waiting for cataract surgery were analyzed at the beginning of May 2020, after 2 months without performing elective surgery. The waiting time since surgical inscription was considered an independent and overriding factor. In addition, higher priority was given to patients with white or brunescent cataracts and patients with low visual acuity: corrected distance visual acuity (CDVA) of 20/200 or less in binocular patients or CDVA of 20/63 or less in monocular patients. Criteria of medium priority included patients who remained with anisometropia and patients with glaucoma or low to moderate risk for chronic angle closure. Data of scheduled surgeries in the following months were then analyzed. RESULTS: A total of 717 patients were waiting for phacoemulsification. One hundred ninety-one patients (26.64%) were on the waiting list more than 4.5 months; the medium waiting time was 3.51 ± 1.57 months. According to both priority criteria and waiting time, 348 (48.6%) were categorized as priority cases. A total of 158 patients (22.0%) met the highest priority; 61 patients (8.5%) met the medium priority criteria. In 129 patients (18.0%), priority was considered based solely on higher waiting time. This algorithm allowed surgeons to operate on all priority cases within the first 3 months. CONCLUSIONS: The presented protocol showed to be effective, providing a timely surgical opportunity for priority cases.

Blood in a Haab Stria.

We describe a case of an asymptomatic and spontaneous intracorneal hemorrhage in an adult with congenital glaucoma and blood collected in a Haab stria.

Anterior Chamber Flare as an Objective and Quantitative Noninvasive Method for Oculopathy in Transthyretin V30M Amyloidosis Patients.

PURPOSE: Assess the aqueous humor flare in transthyretin V30M amyloidosis patients (ATTRV30M). MATERIALS AND METHODS: This is a retrospective, cross-sectional, noninterventional comparative study including 28 ATTRV30M patients with a unilateral scalloped iris. For comparative analysis, the fellow eye, the nonscalloped iris eye, from each patient was used as control. All patients underwent aqueous humor flare meter and intraocular pressure (IOP) measurements. RESULTS: Mean aqueous humor flare was significantly higher in the eyes with the scalloped iris than the control group with the nonscalloped iris (14.1 ± 2.2 versus 6.5 ± 0.9 pc/ms, respectively). No significant differences in IOP were found in the scalloped iris eyes than those in the nonscalloped iris control group (17.1 ± 0.8 versus 16.8 ± 0.7 mmHg, respectively). No significant correlation was not found between the flare and the IOP value within groups. CONCLUSIONS: In this study, aqueous humor flare values in the scalloped iris eyes may be a valid marker for controlling the stage of the oculopathy in ATTRV30M patients.

Consecutive bilateral decompression retinopathy after mitomycin C trabeculectomy: a case report.

BACKGROUND: After a successful trabeculectomy, a sudden intraocular pressure decrease may alter the intracranial to intraocular pressure ratio and cause decompression retinopathy. Frequent Valsalva maneuvers may also play a role in its pathogenesis. This condition may manifest as multiple retinal hemorrhages, edema of the optic disc, macular edema, or a sudden decrease in visual acuity postoperatively. Outcomes for patients are usually good, with spontaneous resolution occurring within a matter of weeks. It has been rarely reported in the literature as a bilateral condition. CASE PRESENTATION: We present a case of consecutive bilateral decompression retinopathy in a 54-year-old severely obese Caucasian woman (body mass index 37 kg/m(2)) with open angle glaucoma and a poor history of medical therapeutic compliance, who chose surgical treatment based on her inability to consistently use ocular drops. Our patient underwent a trabeculectomy with mitomycin C in both eyes, with surgeries taking place 3 months apart. After the first surgery, 2 weeks postoperatively, she complained of decreased visual acuity. Examination of her right eye fundus revealed multiple retinal hemorrhages and disc edema. There was a similar pattern in her left eye, this time including maculopathy. Her visual acuity and fundoscopic changes resolved spontaneously over a period of a month in both cases. Currently, our patient has well-controlled bilateral intraocular pressure, ranging between 14 and 16 mmHg, without hypotensive medication. CONCLUSIONS: Decompression retinopathy is a potential complication after glaucoma surgery, but has rarely been described as a bilateral consecutive condition. A comprehensive approach could help to anticipate its occurrence and manage it.

Bilateral proliferative retinopathy as the initial presentation of chronic myeloid leukemia.

The authors report a rare case of a 48-year-old male with chronic myeloid leukemia (CML) who initially presented with a bilateral proliferative retinopathy. The patient complained of recent visual loss and floaters in both eyes (BE). Ophthalmologic evaluation revealed a best corrected visual acuity (BCVA) of 20/50 in the right eye and 20/200 in the left eye (LE). Fundoscopy showed the presence of bilateral peripheral capillary dropout with multiple retinal sea fan neovascularisations, which were confirmed on fluorescein angiography. Full blood count revealed hyperleukocytosis, thrombocytosis, anemia, and hyperuricemia. Bone marrow aspiration and biopsy showed the reciprocal chromosomal translocation t (9;22), diagnostic of CML. The patient was started on hydroxyurea, allopurinol and imatinib mesylate. He received bilateral panretinal laser photocoagulation and a vitrectomy was performed in the LE. The patient has been in complete hematologic, cytogenetic, and major molecular remission while on imatinib and his BCVA is 20/25 in BE.