Neurodegeneration with Brain Iron Accumulation Disorders and Retinal Neurovascular Structure.

BACKGROUND: The unique neurovascular structure of the retina has provided an opportunity to observe brain pathology in many neurological disorders. However, such studies on neurodegeneration with brain iron accumulation (NBIA) disorders are lacking. OBJECTIVES: To investigate NBIA's neurological and ophthalmological manifestations. METHODS: This cross-sectional study was conducted on genetically confirmed NBIA patients and an age-gender-matched control group. The thickness of retinal layers, central choroidal thickness (CCT), and capillary plexus densities were measured by spectral domain-optical coherence tomography (SD-OCT) and OCT angiography, respectively. The patients also underwent funduscopy, electroretinography (ERG), visual evoked potential (VEP), and neurological examination (Pantothenate-Kinase Associated Neurodegeneration-Disease Rating Scale [PKAN-DRS]). The generalized estimating equation model was used to consider inter-eye correlations. RESULTS: Seventy-four patients' and 80 controls' eyes were analyzed. Patients had significantly decreased visual acuity, reduced inner or outer sectors of almost all evaluated layers, increased CCT, and decreased vessel densities, with abnormal VEP and ERG in 32.4% and 45.9%, respectively. There were correlations between visual acuity and temporal peripapillary nerve fiber layer (positive) and between PKAN-DRS score and disease duration (negative), and scotopic b-wave amplitudes (positive). When considering only the PKAN eyes, ONL was among the significantly decreased retinal layers, with no differences in retinal vessel densities. Evidence of pachychoroid was only seen in patients with Kufor Rakeb syndrome. CONCLUSION: Observing pathologic structural and functional neurovascular changes in NBIA patients may provide an opportunity to elucidate the underlying mechanisms and differential retinal biomarkers in NBIA subtypes in further investigations. © 2023 International Parkinson and Movement Disorder Society.

Cellular senescence implication in mustard keratopathy.

Mustard agents are vesicants that were used in warfare multiple times. They are potent alkylating agents that activate cellular pathways of apoptosis, increase oxidative stress, and induce inflammation. Eyes are particularly susceptible to mustard exposure with a wide range of ocular surface damage. Three main categories of mustard-related eye injuries are acute, chronic, and delayed-onset manifestations. Mustard keratopathy (MK) is a known complication characterized by corneal opacification, ulceration, thinning, and neovascularization that can lead to severe vision loss and discomfort. Recently, a few reports demonstrated the role of senescence induction as a new pathological mechanism in mustard-related injuries that could affect wound healing. We ran the first murine model of delayed-onset MK and nitrogen mustard-induced senescence, evaluating the pathological signs of senescence in the cornea using beta-galactosidase staining. Our results suggest that nitrogen mustard exposure causes senescence in the corneal cells, which could be the underlying mechanism for chronic and late-onset ocular surface damage. We also found a significant correlation between the percentage of positive beta-galactosidase staining and the degree of fibrosis in the cornea. This provides valuable insight into the possible role of anti-senescence drugs in the near future for accelerating corneal healing and restricting fibrosis in patients with mustard keratopathy.

Usefulness of subtraction images for accurate diagnosis of pituitary microadenomas in dynamic contrast-enhanced magnetic resonance imaging.

BACKGROUND: Dynamic contrast-enhanced magnetic resonance imaging (MRI) is the modality of choice for the diagnosis of pituitary microadenomas; however, it may be associated with a relatively high false-negative rate, especially in small lesions. PURPOSE: To evaluate the usefulness of subtraction images for enhancing the visual detection of pituitary microadenomas. MATERIAL AND METHODS: In total, 50 patients with clinically established diagnosis hyperprolactinemia, acromegaly, and Cushing's disease were enrolled. Ten patients referred for brain MRI for reasons other than pituitary abnormality were selected as control group. Routine dynamic MRI of the pituitary gland and obtained subtraction MRI scans were scrutinized separately on different sessions by an experienced radiologist blinded to the study design and patient's data. The investigator's opinion on the presence or absence of a lesion and lesion size were collected. RESULTS: In patients with pituitary microadenoma, dynamic MRI images were reported positive in 42 (84%) patients and negative in 8 (16%). Subtraction images were described as positive in all patients (100%)-including all patients with negative dynamic MRI-and the difference was statistically significant (P=0.016). Undetected lesions on dynamic MRI had a mean size of 2.84 ± 1.79 mm (median= 2.20 mm, interquartile range=1.62-4.62 mm) and a significant inverse correlation was noted between lesion size and negative report of dynamic MRI (P=0.018). Brain MRI scans in the control group were reported negative for pituitary microadenoma in both dynamic contrast-enhanced and subtraction images. CONCLUSION: Subtraction images can successfully identify all lesions detectable with conventional dynamic MRI as well as improving visualization of lesions undetected on dynamic MRI, especially in small lesions.

Review: New horizons in retinoblastoma treatment: an updated review article.

Retinoblastoma (Rb) is a rare childhood intraocular malignancy with an incidence rate of approximately 9000 children per year worldwide. The management of Rb is inherently complex and depends on several factors. The orders of priorities in the treatment of Rb are saving life, globe salvage and vision salvage. Rarity and the young age at diagnosis impede conducting randomized clinical trials (RCTs) for new therapeutic options, and therefore pre-RCTs studies are needed. This review provides an overview of advances in Rb treatment options, focusing on the emergence of new small molecules to treat Rb. Articles related to the management and treatments of Rb were searched in different databases. Several studies and animal models discussing recent advances in the treatment of Rb were included to have a better grasp of the biological mechanisms of Rb. Over the years, the principles of management and treatment of Rb have changed significantly. Innovations in targeted therapies and molecular biology have led to improved patient and ocular survival. However, there is still a need for further evaluation of the long-term effects of these new treatments.

Huntington's disease and neurovascular structure of retina.

BACKGROUND: Retinal biomarkers in neurodegenerative disorders have attracted much attention in recent years. Recent studies have reported visual dysfunction in Huntington's disease (HD). However, little is known about retinal structural changes in HD. METHODS: A total of 50 subjects, including 25 motor-manifest HD patients and 25 gender- and age-matched controls, were enrolled. Unified Huntington's Disease Rating Score-Motor part was assessed in HD patients. Spectral-domain Optical Coherence Tomography (SD-OCT) was used to evaluate the macular thickness and peripapillary retinal nerve fiber layer (pRNFL). Superficial and deep capillary plexus densities were measured using OCT angiography (OCTA). To account for inter-eye correlation, generalized estimating equation (GEE) model was used. RESULTS: HD patients had a significant reduction in macular thickness in both inner and outer superior sectors and the inferior outer sector. Inferior pRNFLs were significantly decreased in thickness. There was no significant difference in retinal capillary plexus density between the two groups. Age and disease duration were negatively correlated with macular thickness in HD patients. However, the severity of motor involvement was not correlated with SD-OCT or OCTA parameters. CONCLUSIONS: We observed attenuated pRNFL and macular retinal thickness in patients with HD, independent of macular capillary plexus parameters. It can support the hypothesis that the retina may be a potential biomarker for monitoring the neurodegenerative process in HD.

Alterations in choroidal vascular parameters following panretinal photocoagulation using enhanced-depth imaging optical coherence tomography in diabetic retinopathy.

PURPOSE: To investigate the alteration of choroid in patients with very severe non-proliferative diabetic retinopathy (NPDR) or early proliferative diabetic retinopathy (PDR) following panretinal photocoagulation (PRP). METHODS: Thirty-nine eyes of 21 patients with very severe non-proliferative diabetic retinopathy (NPDR) (19 eyes) and early proliferative diabetic retinopathy (PDR) (20 eyes) were recruited. Enhanced-depth imaging optical coherence tomography at baseline, 1, and 6 months after PRP was employed to measure choroidal parameters including total choroidal area (TCA) and choroidal vascular index (CVI). RESULTS: In eyes with very severe NPDR, subfoveal TCA decreased non-significantly at month 1, which increased significantly at month 6 (539 ± 131µm2, 502 ± 134µm2, and 598 ± 168µm2 at baseline and months 1 and 6, respectively; P = 0.003). Subfoveal CVI increased at month 1 and then decreased at month 6 (68.25 ± 3.05, 69.74 ± 3.62, and 67.84 ± 1.77 at baseline and months 1 and 6, respectively; P < 0.001). A reverse pattern occurred in eyes with early PDR, a non-significant increase in TCA at month 1 followed by a decrease at month 6 (497 ± 95µm2, 514 ± 133µm2, and 425 ± 95µm2 at baseline and months 1 and 6, respectively; P = 0.011). CVI decreased at month 1 and remained relatively stable at month 6 (69.34 ± 3.11, 68.33 ± 3.41, and 68.50 ± 5.04 at baseline, and months 1 and 6, respectively; P = 0.023). Alteration of choroidal thickness was not statistically significant in both groups. CONCLUSION: Eyes with very severe NPDR and early PDR exhibit a reverse pattern regarding choroidal indices after PRP.

Ruthenium-106 plaque radiotherapy for uveal melanoma: analysis of tumor dimension and location on anatomical and functional results.

BACKGROUND: To report the long-term outcomes of Ru-106 plaque radiotherapy in eyes with uveal melanoma (UM) and to assess the effect of tumor thickness and location on final outcomes. METHODS: Medical records of 234 patients undergoing Ru-106 plaque radiotherapy for UM were reviewed, and the visual outcome, globe preservation, and patient survival were evaluated. The results of 2 groups were compared: 1. between thin (small and medium-sized, thickness < 7 mm, 148 eyes [63.2%]) and thick (thickness ≥ 7 mm, 86 eyes [36.8%]) tumors, and 2. between large (largest basal diameter [LBD] > 12 mm, 109 eyes [46.6%]) and medium/small (LBD ≤ 12 mm, 125 eyes [53.4%]). In addition, a comparison of the juxtapapillary location in 46 eyes (19.7%) versus tumors arising elsewhere and between tumors with and without ciliary involvement in 48 eyes (21.5%) were done. RESULTS: The patients were followed for a median of 54.2 months (range: 6-194.5 months). After adjusting for baseline visual acuity (VA), there was no significant association between final VA and different dimension and tumor location groups. Final globe preservation was 91.9%, and there was no significant difference between different dimension- and ciliary body involvement groups regarding anatomical success rate. The juxtapapillary tumors had lower globe preservation (80.4% vs .94.7%, p = 0.002). The hazard ratio (HR) for enucleation in juxtapapillary tumors was HR = 6.58 (95-CI: 3.84 to 11.21). The overall metastasis rate was 6.8%, with no significant difference in juxtapapillary tumors (4.3% vs.7.4%, p = 0.455). CONCLUSIONS: Ru-106 plaque radiotherapy is an effective treatment for thick and large UM. With this type of treatment, the globe preservation rate is lower in juxtapapillary tumors, but there is no significant difference in the metastasis rate.

Optic Nerve Head Vessel Density Changes from Graves' Disease without TED to TED Dysthyroid Optic Neuropathy: Does Optic Nerve Head Ischemia Play a Role?

PURPOSE: To compare the retinal peripapillary capillary (RPC) vessel density (VD) between 5 groups of patients on the spectrum of thyroid eye disease (TED). METHOD: This is a prospective comparative study looking at 5 groups of patients who were consecutively included from January 2018 to March 2021. They were composed of: Healthy volunteers (39 eyes and 20 subjects), patients with Graves' disease without TED (26 eyes and 13 patients), mild TED (28 eyes and 14 patients), moderate-severe TED (30 eyes and 17 patients), and TED with dysthyroid optic neuropathy (DON) (21 eyes and 12 patients). Ocular and periocular examination, visual field indices, RPC-VD (with optical coherence tomography angiography), and retinal nerve fiber layer, and macular ganglion cell complex thickness were recorded. RESULT: An initial insignificant (0.5 < p < 0.9) rise in the peripapillary-VD (pp-VD) and whole image-VD (wi-VD) from the healthy subject to the Graves' disease without TED was followed by a significant (p = 0.001) fall in RPC-VD in different severity grades of the TED. Paired comparison between the 5 groups showed that the statistically significant fall from the Graves' disease group occurred in the moderate-severe and DON groups (0.001 ≤ p ≤ 0.04). No variable significantly affected the VD (0.08 ≤ p ≤ 0.9). A lower wi-VD and pp-VD were significantly (0.001 ≤ p ≤ 0.009) correlated with the impaired optic nerve functional and structural tests. The sensitivity and specificity of wi-VD (81% and 76%) and pp-VD (69% and 71%) for detecting the DON were statistically significant (p < 0.001). CONCLUSION: Despite an insignificant rise in the wi- and pp-VD from the healthy volunteers to the patients with Graves' disease without TED, VD showed a declining trend in the course of patients with TED, which was statistically significant in the moderate-severe TED and DON groups.

Spatial distribution of diabetic capillary non-perfusion.

OBJECTIVE: To evaluate the distribution of capillary non-perfusion (CNP) in superficial and deep capillary plexuses (SCP and DCP) in eyes with diabetic retinopathy (DR). METHODS: In this retrospective case series, macular optical coherence tomography angiography (OCTA) images were obtained from eyes with DR without diabetic macular edema (DME). The area of CNP in SCP and DCP was delineated using an automated approach after excluding the foveal avascular zone (FAZ) and major retinal vessels. The distribution and spatial correlation of the CNP in each layer were analyzed. RESULTS: Forty-three eyes of 27 patients with DR with a mean age of 59.10 ± 9.05 years were included. The mean CNP area in SCP was statistically significantly higher than DCP (0.722 ± 0.437 mm2 vs. 0.184 ± 0.145 mm2 , respectively, p < .001). There was a statistically significant association between mean BCVA (0.28 ± 0.21 logMAR) and CNP area in DCP (p = .01). After automated subtraction of CNP areas in DCP from SCP, 25.43 ± 15.05% of CNP areas in the DCP had co-localized CNP areas in SCP. The CNP percentage was statistically significantly different between the concentric rings on foveal center, both in SCP and in DCP (both p < .001) showing a decreasing trend from the outer ring toward the center. CONCLUSION: In DR, SCP is more ischemic than DCP. This is in contrast to the previously described oxygenation-dependent ischemic cascade following acute retinal vascular occlusions. This study provides further insight into the retinal ischemia in DR.

Overuse of brain CT scan for evaluating mild head trauma in adults.

INTRODUCTION: CT scan is crucial in evaluating head trauma. However, its inappropriate use will cause unnecessary radiation exposure to patient and financial burden to health systems. Our aim is appraising amount of brain CT scans performed in our Emergency Department (ED) for evaluating mild head trauma which are not indicated according to four standardized guidelines as well as analyzing contributing factors. METHODS: This was a descriptive prospective study. We included randomly selected adult patients under 75 years old with minor head trauma evaluated by brain CT scan at our ED. For all patients, we completed a checklist including demographic data, mechanism of trauma, specialty of the requesting physician, and whether the patient meets the brain CT guidelines criteria. Brain CT overuse was defined as scans performed for patients without criteria of any of the standardized guidelines. RESULTS: We evaluated 170 patients. The mean age of patients was 38.38 ± 19.73 years old. The most common mechanism of trauma was falling (37.6%). The overall brain CT scan overuse was 15.3%. Most of the overused scans were performed in younger patients, and patient's age was inversely correlated to overuse. There was no significant difference based on the mechanism of trauma and the specialty of requesting physician. DISCUSSION: Our study accentuates the high frequency of brain CT scan overuse, leading to unnecessary radiation exposure and financial burden on healthcare systems. We emphasize that using a guideline for requesting brain CT scan can eliminate unnecessary scans along with detecting patients with important decisive damages.

Culture-proven endogenous endophthalmitis: microbiological and clinical survey.

PURPOSE: To report causative organisms, visual outcomes, and associated medical conditions in endogenous endophthalmitis (EE). METHODS: Microbiological and medical reports of patients with culture-proven EE referred to Farabi Eye Hospital, Tehran, Iran, from January 2008 to January 2018 were reviewed. RESULTS: A total of 144 cases (171 eyes) were included in the present study. Candida albicans (30.6%) and Staphylococcus aureus (27.1%) were the most prevalent microbial causes. Diabetes mellitus (43.8%) and IV drug abuse (10.4%) were the leading associated systemic conditions. Age of the patients (P = 0.002) and presenting corrected distance visual acuity (CDVA) (P < 0.001) were correlated with postoperative CDVA. Patients with bilateral involvement had significantly lower mean logMAR (P = 0.002), and patients with hypopyon had significantly higher mean logMAR (P = 0.006). Eyes with gram-negative bacillus cultures had a significantly worse logMAR than eyes with gram-positive cocci (P = 0.009). CONCLUSION: Candida albicans and S. aureus were the most common causes of EE, and diabetes mellitus was the most common associated medical condition. EE is associated with poor visual outcome, especially in older patients, those with worse visual acuity at presentation, and those with positive cultures for gram-negative bacilli.

Simultaneous photorefractive keratectomy and accelerated collagen cross-linking in high-risk refractive surgery (Tehran protocol): 3-year outcomes.

PURPOSE: To evaluate safety and efficacy of performing simultaneous photorefractive keratectomy (PRK) and collagen cross-linking (CXL) in myopic patients with preoperative risk factors for developing keratectasia. METHODS: Seventeen eyes of 15 patients with at least one of the following risk factors were recruited: central keratometry (Kmax) between 48 and 50, difference between inferior, superior corneal power (I-S value) between 1.4 and 1.9 and corneal thickness between 450 and 480 µm. Upon final stage of standard PRK, 0.02% mitomycin was applied for 30-50 s, and then, accelerated CXL was performed for 5 min. Pre- and postoperative Oculus Pentacam® imaging for keratometry values, measurement of uncorrected distance visual acuity (UDVA) and corrected distance visual acuity (CDVA) were done for all patients. RESULTS: Mean follow-up time was 32.08 ± 7.79 months (range 25-49 months). Mean age of patients was 28.78 ± 3.80 years. Mean postoperative spherical equivalent was + 0.19 ± 0.42 (- 0.5 to + 1.0 [D]). Mean UDVA and CDVA improved from 0.9062 ± 0.485 log MAR and 0.0148 ± 0.043 log MAR to 0.0173 ± 0.040 log MAR and 0.0057 ± 0.023 log MAR, respectively (P = 0.011, P = 0.735). Mild degree of early postoperative stromal haze was seen which did not persist more than 6 months. There was no late stromal haze, corneal ectasia or other major postoperative complication in the follow-up period. CONCLUSION: Combined PRK and accelerated CXL is an efficient and safe procedure for high-risk refractive surgery candidates, with no increased risk of persistent corneal haze.

Segmentation of COVID-19 pneumonia lesions: A deep learning approach.

Background: Lung CT scan has a pivotal role in diagnosis and monitoring of COVID-19 patients, and with growing number of affected individuals, the need for artificial intelligence (AI)-based systems for interpretation of CT images is emerging. In current investigation we introduce a new deep learning-based automatic segmentation model for localization of COVID-19 pulmonary lesions. Methods: A total of 2469 CT scan slices, containing 1402 manually segmented abnormal and 1067 normal slices form 55 COVID-19 patients and 41 healthy individuals, were used to train a deep convolutional neural network (CNN) model based on Detectron2, an open-source modular object detection library. A dataset, including 1224 CT slices of 18 COVID-19 patients and 9 healthy individuals, was used to test the model. Results: The accuracy, sensitivity, and specificity of the trained model in marking a single image slice with COVID-19 lesion were 0.954, 0.928, and 0.961, respectively. Considering a threshold of 0.4% for percentage of lung involvement, the model was capable of diagnosing the patients with COVID-19 pneumonia, with a sensitivity of 0.982% and a specificity of 88.5%. Furthermore, the mean Intersection over Union (IoU) index for the test dataset was 0.865. Conclusion: The deep learning-based automatic segmentation method provides an acceptable accuracy in delineation and localization of COVID-19 lesions, assisting the clinicians and researchers for quantification of abnormal findings in chest CT scans. Moreover, instance segmentation is capable of monitoring longitudinal changes of the lesions, which could be beneficial to patients' follow-up.

Clinical predictive factors and imaging biomarkers of treatment response to half dose PDT in patients with chronic central serous chorioretinopathy.

PURPOSE: To determine the clinical and imaging biomarkers of the response to half-dose photodynamic therapy (HD-PDT) in patients with central serous chorioretinopathy (CSC) METHODS: Clinical records and baseline ophthalmic images of 67 chronic CSC patients who underwent HD-PDT were assessed. In addition to demographic data, optical coherence tomography (OCT), fluorescein angiography (FA) and fundus autofluorescence (FAF) images were analyzed for specific biomarkers. The patients were categorized to early responder and late responder based on the time needed for complete resolution of subretinal fluid after PDT (less than 1 month vs. more than 1 month). The baseline clinical and imaging biomarkers were compared between the two groups. RESULTS: Seventy-three eyes of 67 patients were included in the study. The mean response time to PDT was 1.63 ± 1.48 months with 82.2% (60/73) of eyes categorized as early responder. The mean response time to PDT in delayed-response group was 4.15±1.51 months. In multivariate analysis, delayed response to PDT was associated with lacking history of systemic corticosteroid consumption, lacking history of pretreatment with eplerenone or acetazolamide before PDT and presence of hyperreflective foci in baseline OCT images (all p values < 0.05). There was no association between final visual outcome and late response to PDT. CONCLUSION: The presence of inflammatory biomarkers such as hyperreflective foci in baseline OCT images might be indicative of resistance to PDT. Moreover, the effect of pretreatment with mineralocorticoid antagonist on the response to PDT in chronic CSC should be explored in future prospective studies.

Fluorescein versus Indocyanine Green Angiography Guided Half-Dose Photodynamic Therapy for Chronic Central Serous Chorioretinopathy.

Purpose: To compare the outcomes of fluorescein angiography (FA)-guided and indocyanine green angiography (ICGA)-guided half-dose photodynamic therapy (PDT) in patients with chronic central serous chorioretinopathy (CSC). Methods: In this retrospective comparative study, medical records of eyes with chronic CSC who underwent half-dose PDT were reviewed. A retina specialist performed FA-guided half-dose PDT, and the other performed ICGA-guided treatment. The success of applying PDT in the resolution of subretinal fluid was compared between the FA- and ICGA-guided methods. Results: Eighty-two eyes of 73 patients (41 eyes in each group) received half-dose PDT. After half-dose PDT, a significant improvement in the best-corrected visual acuity (BCVA) was found at the time of the last follow-up in both groups (both P < 0.001), with no significant intergroup difference. Central subfield and subfoveal choroidal thicknesses decreased significantly in both groups at the last follow-up (all P < 0.05), with no significant differences between the groups. Subretinal fluid (SRF) resolved in all eyes, and no persistent SRF was detected during the follow-up period. Conclusion: FA-guided and ICG-guided half-dose PDT may have similar efficacy for the treatment of chronic CSC.

Double Retina Sign: A New Optical Coherence Tomography Finding in Combined Hamartoma of the Retina and Retinal Pigment Epithelium.

Purpose: To describe a case of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with peculiar optical coherence tomography (OCT) findings. Methods: Case report. Results: A 7-year-old girl with a history of decreased visual acuity in the left eye since early childhood presented with pigmented epiretinal membrane in favor of CHRRPE based on clinical and paraclinical findings. In OCT images, an area of retinal defect was noted, and the retina doubled up on itself near the defect (double retina sign). Conclusion: Careful examination of OCT images in patients with CHRRPE can reveal new findings.

Stem Cell Therapy in Stargardt Disease: A Systematic Review.

This article aimed to review current literature on the safety and efficacy of stem cell therapy in Stargardt disease. A comprehensive literature search was performed, and two animal and eleven human clinical trials were retrieved. These studies utilized different kinds of stem cells, including human or mouse embryonic stem cells, mesenchymal stem cells, bone marrow mononuclear fraction, and autologous bone marrow-derived stem cells. In addition, different injection techniques including subretinal, intravitreal, and suprachoroidal space injections have been evaluated. Although stem cell therapy holds promise in improving visual function in patients with Stargardt disease, further investigation is needed to determine the long-term benefits, safety, and efficacy in determining the best delivery method and selecting the most appropriate stem cell type.

Unplanned Reoperation following Vitreoretinal Surgery.

Purpose: To evaluate the incidence of unplanned return to the operating room following vitreoretinal surgery and assess the reasons. Methods: In this retrospective case series, medical records of all patients who underwent vitreoretinal surgery were reviewed to determine the incidence and reasons of early (<30 days postoperatively) and late (≥30 days postoperatively) unplanned reoperations after the surgery. Results: A total of 488 eyes of 468 patients with a mean age of 55.84 ± 18.23 years were included. Fourteen percent (68/488) of eyes required one or more unplanned reoperation following their primary surgery. These include 3.9% (19/488) for the early and 10.0% (49/488) for the late reoperation. The most common primary reason for baseline surgery was rhegmatogenous retinal detachment (RRD) without proliferative vitreoretinopathy (PVR, 38.2%), followed by RD with PVR (23.5%), and tractional RD (TRD, 19.1%). Unplanned reoperations were most common in RD with PVR (19.3%), RRD without PVR (17.2%), and TRD (14.4%). Overall, the most common reasons of the first unplanned reoperation were repeated RD with PVR (27.9%), repeated RD (19.1%), and the presence of silicone oil (SO) in the anterior chamber (AC) (10.3%). For early unplanned reoperations, SO in AC, postoperative endophthalmitis, and persistent hyphema were the most common causes. Repeated RD with PVR was the most prevalent cause of late unplanned reoperations (34.7%). In the multivariate analysis, preoperative best-corrected visual acuity (BCVA) was significantly lower in eyes with unplanned reoperation than in eyes without (P = 0.011). Conclusions: Unplanned reoperation following vitreoretinal surgery is not very common, and occurs mostly in the setting of PVR, RRD, and TRD. Lower preoperative BCVA may indicate an increased chance of future unplanned reoperation(s).

Targeted chemotherapy in retinoblastoma: A step forward from patient survival to globe survival.

BACKGROUND/ OBJECTIVES: Despite being the most common intraocular malignancy in childhood, there is a substantial disparity between developing and developed countries in terms of patient and globe survival in retinoblastoma (Rb). The current study intends to determine patient and globe survival before and after the introduction of the new targeted treatment modalities in a developing country. METHODS: Medical records of 350 patients (516 eyes) with retinoblastoma referred to a tertiary referral center for Rb in Tehran, Iran, were reviewed. In order to compare patient and globe survival before and after the availability of the new treatment modalities, including intra-arterial and intravitreal chemotherapy, the patients were divided into group 1 (2001-2007) and group 2 (2008-2018) based on the calendar period of diagnosis. RESULTS: Two-hundred-twenty-three eyes of 149 patients and 293 eyes of 201 patients were categorized into groups 1 and 2, respectively. The 5-year patient survival was 97% across the current survey, and the overall survival rate was 96% in group 1 and 99% in group 2 (P = 0.08). Overall, 50% of eyes with retinoblastoma underwent enucleation, which was the primary in 63% (116/184) of the unilateral and 30% (99/322) of the bilateral cases. Primary enucleation was significantly lower in group 2 (35%) in contrast to group 1 (50%) (P < 0.001). In addition, globe survival improved significantly in the International Classification of Retinoblastoma Groups D (17% in group 1 vs. 66% in group 2, P < 0.001) and E (1% in group 1 vs. 23% in group 2 P < 0.001) during the two timelines. In enucleated eyes, despite the increased rate of prelaminar involvement in group 2 (13% vs. 2% in group 1, P = 0.003), the rate of high-risk histopathologic findings was similar between the two groups. CONCLUSION: Similar to developed countries, the application of new targeted treatment modalities, including intra-arterial and intravitreal chemotherapy, has been associated with significantly improved globe survival in Rb patients. However, it should be noted that even with the availability of these novel treatment options, the decision for on-time enucleation should not be deferred.