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Purpose: To explore whether varying degrees of vitreous haemorrhage (VH) and calcification act as risk factors for enucleation in patients with advanced retinoblastoma (RB). Methods: Advanced RB was defined by the international classification of RB (Philadelphia version). Basic information for retinoblastoma patients diagnosed as groups D and E in our hospital between January 2017 and June 2022 was reviewed by logistics regression models. Additionally, a correlation analysis was performed, excluding variables with a VIF (variance inflation factor) >10 from the multivariate analysis. Results: A total of 223 eyes diagnosed with RB were included in assessing VH and calcification; of these, 101 (45.3%) eyes experienced VH, and 182 (76.2%) eyes were found to have calcification within the tumour through computed tomography (CT) or B‐scan ultrasonography. Ninety‐two eyes (41.3%) were enucleated, of which 67 (72.8%) had VH and 68 (73.9%) calcification, both of which were significantly relevant to enucleation (p < 0.001*). Other clinical risk factors, such as corneal edema, anterior chamber haemorrhage, high intraocular pressure during treatment and iris neovascularization, correlated significantly with enucleation (p < 0.001*). Multivariate analysis included IIRC (intraocular international retinoblastoma classification), VH, calcification and high intraocular pressure during treatment as independent risk factors for enucleation. Conclusions: Despite identifying different potential risk factors for RB, there remains significant controversy concerning which patients require enucleation, and the degree of VH varies. Such eyes need to be evaluated carefully, and management with appropriate adjuvant therapy may improve the outcome of these patients. [ABSTRACT FROM AUTHOR]

Orbital floor fractures are commonly encountered, but the dislocation of the eyeball into the maxillary sinus is relatively rare. When it does occur, globe dislocation can have serious consequences, including vision loss, enucleation, and orbito-ocular deformity. Immediate surgical intervention is typically attempted when possible. However, severe comorbidities and poor general health can delay necessary surgery. In this report, we present the surgical outcomes of a 70-year-old woman who received delayed treatment for traumatic eyeball dislocation into the maxillary sinus due to a subarachnoid hemorrhage and hemopneumothorax. Additionally, we propose a treatment algorithm based on our clinical experience and a review of the literature. [ABSTRACT FROM AUTHOR]

Introduction: Retinoblastoma is a malignant tumor with a high cure potential when proper therapy is used. The purpose of this paper is to report the clinical features and outcomes of patients with retinoblastoma who were treated with a combination of local and systemic chemotherapy‐based protocols. Method: We retrospectively studied patients treated with systemic chemotherapy plus local treatment between 2003 and 2015 with a follow‐up ≥2 years. We correlated clinical and pathological characteristics with decimal visual acuity (VA) and death. Results: Among 119 patients, 60% had unilateral disease (UNI), and 52% were male. The median presentation age was 19.5 months, 10% had a positive family history, and the most frequent sign was leukocoria (68.8%). Advanced disease was more frequent in eyes with UNI (98.4%) than in eyes with bilateral retinoblastoma (BIL: 55.3%). Enucleation was performed in 97% of UNI eyes and in 55.8% of BIL eyes. The overall globe salvage was 26.6%, 44.25% of BIL eyes. Bilateral enucleation was required in 5%. High‐risk pathologic features occurred in 50% and 37% of eyes enucleated without and with neoadjuvant chemotherapy, respectively. High‐risk features were related to the presence of goniosynechiae in the pathologic specimen and were more frequent in children younger than 10 months or older than 40 months. Extraocular disease was present in 5% of patients, and the death rate related to metastasis of the tumor was 8%. The final VA was ≥ 0.7 in 72.8% and ≥0.1 in 91% of BIL patients. Conclusions: Treatment of retinoblastoma with conservative systemic‐based chemotherapy was associated with an excellent survival rate (92%). Albeit the low overall globe salvage rate, in BIL patients, approximately half the eyes were conserved, and a satisfactory functional visual result was achieved The evaluated protocol is an important treatment option, especially in developing countries. [ABSTRACT FROM AUTHOR]

Objective: To identify the characteristics of cornea transplant after transplantation. Methods: Cross-sectional, retrospective study with quantitative approach. Research was conducted at Banco de Olhos do Ceará and at three other private transplant centers in Fortaleza. Medical records of transplant patients from October 2020 to September 2022 were analyzed within one year of the procedure. Results: 300 medical records of patients undergoing corneal transplantation were collected, of which 289 (96.3%) corneas demonstrated good transparency. Regarding visual acuity, 62 transplants (20.7%) presented mild vision loss, with 129 (43%) presenting normal intraocular pressure. Conclusion: The characteristics of post-transplant corneas vary depending on the recovery phase and the success of graft integration. The tissue showed good transparency, with visual acuity with slight loss of vision. [ABSTRACT FROM AUTHOR]

Purpose: Enucleation is a common treatment modality performed for pediatric retinoblastoma patients, and the resultant defects are reconstructed using an ocular prosthesis. The prostheses are modified or replaced periodically, as the child develops due to orbital growth and patient‐error. The purpose of this report is to evaluate the replacement frequency of prostheses in the pediatric oncologic population. Methods: A retrospective review was completed by the two senior research investigators, of patients that had ocular prostheses fabricated following enucleation of their retinoblastoma from 2005 to 2019 (n = 90). Data collected from the medical records of the patient included the pathology, date of surgery, date of prosthesis delivery, and the replacement schedule of the ocular prosthesis. Results: During the 15‐year study period, 78 enucleated observations (ocular prosthesis fabricated) were included for analysis. The median age of the patients at the time of delivery of their first ocular prosthesis was calculated to be 2.6 years (range 0.3–18 years). The median time to the first modification of the prosthesis was calculated to be 6 months. The time to modification of the ocular prosthesis was further stratified by age. Conclusion: Pediatric patients require modification of their ocular prostheses throughout their growth and development period. Ocular prostheses are reliable prostheses with predictable outcomes. This data is helpful to set an expectation among the patient, parent, and provider. [ABSTRACT FROM AUTHOR]

Introduction: Ocular trauma is a significant cause of visual loss with long-term sequelae, including reduced quality of life and loss of productivity for young patients. Surgical treatment depends on the injury; enucleation and evisceration are viable options in nonrecoverable eyes. Our study aims to describe the epidemiological and clinical profiles of patients undergoing evisceration/enucleation due to ocular trauma in a tertiary care center in Medellin, Colombia, over 10 years. Methods: We conducted a cross-sectional descriptive study of eyes that were eviscerated or enucleated due to trauma at the SHospital Universitario San Vicente Fundación in Medellin, Colombia, between 2011 and 2021. We studied sociodemographic characteristics, factors related to ocular trauma, and the surgical procedure performed. Results: We studied 161 eyes of 160 patients, of which 2 were enucleated and 158 eviscerated due to ocular trauma. The male-to-female ratio was 7.9:1, and the mean age was 36.6 years (standard deviation = 18.3). 52.2% were due to aggression or violence; the most frequent mechanism was a gunshot wound (28.1%). 98.1% were classified as open ocular trauma, of which 84.5% were ruptured globes. Of the eyes with reported visual acuity, 93.3% did not perceive light. Of the 161 eyes, 58.4% suffered an orbital fracture, and 29.2% suffered cranioencephalic trauma. Primary evisceration was performed in 98.8%, with half of the patients operated in 2 days or less and 75% in 4 days or less. An orbital implant was used in 90.7%. Seventy-five percent of patients were followed up at 36 days. Conclusions: The majority of ocular trauma resulting in loss of the eyeball occurred in young men who had suffered aggression or violence and presented with ocular rupture, with primary evisceration and orbital implant being performed in most cases, in contrast to what is reported in international series where secondary evisceration is preferred, possibly due to the initial severity of the traumatized eyes. [ABSTRACT FROM AUTHOR]

ObjectiveTo report onset and progression of clinical signs of a neuroendocrine neoplasm (NEN) presumed metastatic to the choroid in a dog.Animals studiedA 7.5-year-old female spayed German shepherd dog mix referred for advanced imaging and evaluation of a subretinal mass in the right eye.ProceduresProcedures performed included general physical and ophthalmic examinations; ocular, orbital, and abdominal ultrasonography; thoracic radiographs; cranial magnetic resonance imaging; serologic testing for infectious agents; analysis of hematologic as well as serum and urine biochemical parameters; echocardiography; electrocardiography; cytologic assessment of lymph nodes; and histopathology and immunohistochemistry of the enucleated globe.ResultsExamination and imaging identified a pigmented mass within and expanding the superior choroid. Following enucleation, a choroidal NEN with tumor emboli in scleral blood vessels was diagnosed by histopathologic assessment and confirmed by immunohistochemical labelling. Despite extensive and repeated diagnostic testing over many months, a putative primary site was not identified until 19 months after the initial ocular signs were noted. At that time, a heart-base mass and congestive heart failure were highly suggestive of a chemodectoma.ConclusionThis comprehensive report of a NEN presumed metastatic to the choroid in a dog suggests that ocular disease can be a very early and solitary sign of NEN in the dog.

The eyes provide themselves with immune tolerance. Frequent skin inflammatory diseases in young blind people suggest, nonetheless, that the eyes instruct a systemic immune tolerance that benefits the whole body. We tested this premise by using delayed skin contact hypersensitivity (DSCH) as a tool to compare the inflammatory response developed by sighted (S) and birth-enucleated (BE) mice against oxazolone or dinitrofluorobenzene at the ages of 10, 30 and 60 days of life. Adult mice enucleated (AE) at 60 days of age were also assessed when they reached 120 days of life. BE mice displayed exacerbated DSCH at 60 but not at 10 or 30 days of age. AE mice, in contrast, show no exacerbated DSCH. Skin inflammation in 60-day-old BE mice was hapten exclusive and supported by distinct CD8+ lymphocytes. The number of intraepidermal T lymphocytes and migrating Langerhans cells was, however, similar between S and BE mice by the age of 60 days. Our observations support the idea that the eyes instruct systemic immune tolerance that benefits organs outside the eyes from an early age. The higher prevalence of inflammatory skin disorders reported in young people might then reflect reduced immune tolerance associated with the impaired functional morphology of the eyes. [ABSTRACT FROM AUTHOR]

PurposeTo determine the usefulness of a comprehensive, targeted-capture next-generation sequencing (NGS) assay for the clinical management of children undergoing enucleation for retinoblastoma.DesignCohort study.ParticipantsThirty-two children with retinoblastoma.MethodsWe performed targeted NGS using the UCSF500 Cancer Panel (University of California, San Francisco, San Francisco, CA) on formalin-fixed, paraffin-embedded tumor tissue along with constitutional DNA isolated from peripheral blood, buccal swab, or uninvolved optic nerve. Peripheral blood samples were also sent to a commercial laboratory for germline RB1 mutation testing.Main outcome measuresPresence or absence of germline RB1 mutation or deletion, tumor genetic profile, and association of genetic alterations with clinicopathologic features.ResultsGermline mutation or deletion of the RB1 gene was identified in all children with bilateral retinoblastoma (n = 12), and these NGS results were 100% concordant with commercial germline RB1 mutation analysis. In tumor tissue tested with NGS, biallelic inactivation of RB1 was identified in 28 tumors and focal MYCN amplification was identified in 4 tumors (2 with wild-type RB1 and 2 with biallelic RB1 inactivation). Additional likely pathogenic alterations beyond RB1 were identified in 13 tumors (41%), several of which have not been reported previously in retinoblastoma. These included focal amplifications of MDM4 and RAF1, as well as damaging mutations involving BCOR, ARID1A, MGA, FAT1, and ATRX. The presence of additional likely pathogenetic mutations beyond RB1 inactivation was associated with aggressive histopathologic features, including higher histologic grade and anaplasia, and also with both unilateral and sporadic disease.ConclusionsComprehensive NGS analysis reliably detects relevant mutations, amplifications, and chromosomal copy number changes in retinoblastoma. The presence of genetic alterations beyond RB1 inactivation correlates with aggressive histopathologic features.

Introduction: Melanomas are malignant neoplasms that occur in various anatomical sites, including the eye. Ocular melanomas account for 5% of all melanomas and are mainly described in Caucasian and older individuals. This study describes the clinical and pathological characteristics of uveal (choroid) melanoma in a Caucasian patient. Case report: A 41-year-old Caucasian female patient, brown eyes, without history of ophthalmological diseases or family history of cancer experienced pain and loss of visual acuity in the left eye. On clinical examination, an increase of ocular pressure was detected. Ultrasound showed a mushroom-like neoformation. Moreover, magnetic resonance imaging showed a mass with spontaneous hypersignal on T1-weighted images, intense gadolinium enhancement, and marked hyposignal on T2-weighted images. The patient was referred to the Oncology Ophthalmology department for enucleation due to suspected uveal melanoma. Anatomopathological analysis revealed a blackened mass in the eyeball. Histologically, the mass comprised spindle cells (50%) and epithelioid cells (50%). A diagnosis of choroidal melanoma was established based on the identification of ophthalmoscopic, imaging, and histological characteristics of the tumor. Conclusion: Choroidal melanomas usually occur in males, clear-eyed, and older individuals. A wide variety of ocular lesions may mimic choroidal melanoma, which should be included in the differential diagnosis of choroidal nevus and peripheral hemorrhages.

BackgroundActivity in neurons drives afferent competition that is critical for the refinement of nascent neural circuits. In ferrets, when an eye is lost in early development, surviving retinogeniculate afferents from the spared eye spread across the thalamus in a manner that is dependent on spontaneous retinal activity. However, how this spontaneous activity, also known as retinal waves, might dynamically regulate afferent terminal targeting remains unknown.MethodsWe recorded retinal waves from retinae ex vivo using multi-electrode arrays. Retinae came from ferrets who were binocular or who had one eye surgically removed at birth. Linear mixed effects models were used to investigate the effects of early monocular enucleation on retinal wave activity.ResultsWhen an eye is removed at birth, spontaneous bursts of action potentials by retinal ganglion cells (RGCs) in the surviving eye are shorter in duration. The shortening of RGC burst duration results in decreased pairwise RGC correlations across the retina and is associated with the retinal wave-dependent spread of retinogeniculate afferents previously reported in enucleates.ConclusionOur findings show that removal of the competing eye modulates retinal waves and could underlie the dynamic regulation of competition-based refinement during retinogeniculate development.

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Purpose: The objective of this study was to better understand the onset of radiation retinopathy and secondary orbital tumors in the setting of retinoblastoma treated with radiation and chemotherapy., Methods: This was a case report., Results: Here, the authors present a 48-year-old woman with a history of bilateral hereditary retinoblastoma after enucleation of the left eye and radiation therapy to the right eye along with systemic chemotherapy. She underwent bladder leiomyosarcoma resection at the age of 24 years. In 2020, she presented with significantly delayed radiation retinopathy complicated by cystoid macular edema, and bevacizumab injections were initiated. An incidental benign lymphoproliferative tumor in the right lacrimal gland was found on B-scan ultrasound and was successfully excised., Conclusion: It is rare for radiation retinopathy to present with significant delay after local radiation treatment, with only two other cases found in the literature describing a similar late onset. In addition, there have been no other published cases of a secondary benign lymphoproliferative tumor in the setting of retinoblastoma treated with radiation and chemotherapy.

Purpose: Radiation-induced scleral necrosis (RISN) is a less frequent complication of brachytherapy for uveal melanoma, and may require surgical treatment in selected cases. We aimed to identify the prognostic factors for RISN treatment., Methods: All patients with brachytherapy for uveal melanoma treated at our institution between 01/1999 and 12/2016 who developed RISN were followed until 02/2021. Various parameters were evaluated through univariable and multivariable Cox regression analysis. The surgical intervention due to RISN was the principal outcome event of this study., Results: Of 115 patients in the final cohort, 51 individuals (44%) underwent RISN treatment (conjunctival revision [n = 2], patching [n = 46] or enucleation [n = 3]) at median 1.80 months after RISN occurrence. Significant RISN characteristics were summarized into a novel RISN severity scale - Grade I: largest diameter ≤ 5 mm and no progression; Grade II: largest diameter > 5 mm or any progression during the follow-up; Grade III: presence of uveal prolapse; and Grade IV: leakage through open eyewall perforation. In the multivariable analysis, the RISN severity scale (aHR = 2.37 per grade increase, p = 0.01) and the time between brachytherapy and RISN occurrence (<15 months, aHR = 6.33, p < 0.0001) were independently associated with the study endpoint. The RISN severity scale showed high diagnostic accuracy for prediction of RISN treatment (AUC = 0.869)., Conclusions: In our series, about the half of RISN cases underwent surgical treatment. The presented novel severity scale for RISN might become a helpful tool for clinical management of individuals with RISN. We recommend external validation of the diagnostic accuracy of the presented scale., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To employ a validated survey for evaluation of quality of life (QoL) outcomes and associated factors in a US cohort of adult patients with acquired anophthalmia wearing a prosthesis., Methods: A retrospective cohort study was performed at a single, US academic institution of patients cared for between 2012 and 2021. The electronic medical record database was queried for adult patients with a history of evisceration or enucleation surgery and placement of an orbital implant. Identified patients were asked to anonymously complete a validated QoL questionnaire from which the Global Ocular Prosthesis Score was assessed. Summary statistics were reported, and analysis of variance was used to determine surgical and demographic factors associated with QoL survey scores., Results: A total of 81 patients completed the questionnaire. Mean age was 45.8 years (range: 18-96 years), and 54.3% of patients identified as women. Enucleation was performed in 81.4% of eyes (n = 66), while the remaining 17.3% underwent evisceration (n = 14). The mean and median Global Ocular Prosthesis Score (out of 100) were 67.51 and 68.00, respectively. Multivariate analysis demonstrated the etiologies of glaucoma (95% confidence interval: 0.84-25.40, p = 0.04) and endophthalmitis (95% confidence interval: 0.41-23.72, p = 0.04) to be significantly associated with higher total Global Ocular Prosthesis Score. Several other factors including employment status, etiology of anophthalmia, gender, and level of education were also significantly associated with subdomain scores ( p < 0.05)., Conclusions: Among patients with anophthalmia wearing an ocular prosthesis, the etiology of anophthalmia was significantly associated with the Global Ocular Prosthesis Score. Employment status, etiology of anophthalmia, gender, and level of education were also observed to have statistically significant associations with subdomain scores. Knowledge of these factors moving forward may help the surgeon manage expectations, mitigate challenges, and promote higher QoL outcomes for patients with acquired anophthalmia., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To compare the clinical outcomes of children with unilateral retinoblastoma (Rb) and high-risk histopathology features (HRHF) following upfront enucleation with/without adjuvant chemotherapy, and investigate cases locally considered non-HRHF but converted to a standardized HRHF definition., Design: Retrospective multinational clinical cohort study., Methods: Children with Rb who presented to 21 centers from 12 countries between 2011-2020, and underwent primary enucleation were recruited. Centers retrieved clinical data and were asked to report detailed histopathology findings, as well as indicate cases defined locally as high-risk. For analysis, only unilateral cases with standardized HRHF, defined as retrolaminar optic nerve invasion, massive choroidal invasion, scleral invasion, anterior-segment involvement, and/or combined nonmassive choroidal and prelaminar/laminar optic nerve invasion, were included. Main outcome measures included orbital tumor recurrence, systemic metastasis, survival and number, and outcome of cases converted to standardized HRHF., Results: A total of 600 children presenting to 14 centers in 9 countries were included. Of these, 505 (84.2%) were considered locally as HRHF and received adjuvant chemotherapy. After a median follow-up period of 39.2 ± 1.6 months (range: 0.8-60.0 months), 36 (6.0%) had orbital tumor recurrence, 49 (8.2%) metastasis, and 72 (12.0%) children died. Children not receiving adjuvant chemotherapy were at significantly increased risk of orbital tumor recurrence, metastasis, and death (P ≤ .002). Of the study children, 63/600 (10.5%) were considered locally non-HRHF, but converted to standardized HRHF and included in the analysis. Of these, 6/63 (9.5%) had orbital tumor recurrence, 5/63 (7.9%) metastasis, and 6/63 (9.5%) children died. Isolated minor choroidal invasion with prelaminar/laminar optic nerve invasion was reported in 114 (19.0%) children, but considered locally as HRHF only in 68/114 (59.6%). Of these, 6/114 (5.3%) children developed metastasis and subsequently died, yielding a number needed to treat of 15., Conclusion: Based on this multinational cohort of children with Rb, we recommend the use of adjuvant chemotherapy following upfront enucleation and diagnosis of HRHF. Variation exists worldwide among centers when defining HRHF, resulting in adverse patient outcomes, warranting standardization., (Copyright © 2024 Elsevier Inc. All rights reserved.)

A 61-year-old man presented hyperacute endophthalmitis due to Proteus mirabilis after a pars plana vitrectomy. In the first examination (24 h after surgery), visual acuity (VA) was of hand movement, biomicroscopy showed edematous cornea, Tyndall ++++ and fibrin membrane, with vitritis and impossibility of visualizing retina details. Treatment with intravitreal injections was performed. Despite the treatment, the patient's symptoms worsened, and he began with poor general condition, fever, and leukocytosis, requiring hospitalization and intravenous treatment because of a diagnosis of secondary meningitis due to post-surgical endophthalmitis. The patient required enucleation of the affected eye without prosthesis placement. Even though post-surgical bacterial endophthalmitis is usually an infection confined to the eye, this clinical case demonstrates the possibility of the infection spreading to the rest of the body, potentially endangering the patient's life., Competing Interests: Declaration of competing interest None declared., (Copyright © 2024 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Objective: Histopathological analysis of the retinal pigment epithelial (RPE) changes in retinoblastoma (RB) cases who received pre-surgical chemotherapy., Design: Laboratory-based observational study., Methods: Five-year analysis was performed to identify Retinoblastoma cases who underwent enucleation after receiving systemic chemotherapy. Grossly, RPE cells were observed in flat preparation in small calottes by staining with fluorescein stain in the raw specimens. They were documented under the objective of compound microscope and compared with hematoxylin and eosin-stained slides in the permanent tissue sections., Results: Out of 51 cases of RB, post-chemotherapy enucleation was performed in 17 cases. Mean age of enucleation was 3.2 years. Endophytic RB (11 cases, 64.71%) was more common than the exophytic variety. Choroidal involvement was noted in 8 cases (47.06%), and optic nerve involvement was seen in 5 cases (29.4%). Focal and diffuse RPE changes were seen in one case each (5.88%). Central RPE cell changes near the cell nucleus were seen in all 17 cases (100%), which were documented by both fluorescein and Hematoxylin and eosin stain (100%). Drusens were observed in 8 cases (47.06%), and RPE proliferations were seen in 3 cases (17.65%)., Conclusion: The study highlights the characteristic histopathological RPE changes after systemic chemotherapy in RB cases. These changes may be attributable to cell nucleus damage after chemotherapy., (Copyright © 2024 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Objective: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) followed by massive unilateral uveal proliferation., Methods: Retrospective case report., Results: A 47-year-old female with history of metastatic ovarian carcinoma initially presented with bilateral vision loss and multifocal red patches on posterior poles consistent with BDUMP. Five years later, she presented with bilateral neovascular glaucoma and unilateral iris and ciliary body mass concerning for malignancy. Enucleation revealed diffuse uveal growth involving almost the entirety of the uveal tract., Conclusions: BDUMP can rarely be associated with uveal proliferation. Routine examinations are recommended to monitor for any changes concerning malignancy.

Importance: Adjuvant therapy is an important and effective treatment for retinoblastoma. However, there is a lack of head-to-head clinical trials comparing 3 vs 6 cycles of CEV chemotherapy (carboplatin, etoposide, and vincristine) for enucleated unilateral retinoblastoma with high-risk pathological features., Objective: To assess whether 3 cycles of CEV chemotherapy is noninferior to 6 cycles for enucleated unilateral retinoblastoma with high-risk pathological features., Design, Setting, and Participants: This double-center, randomized, open-label, noninferiority trial was conducted at 2 premier eye centers in China and included 187 patients who had undergone enucleation for unilateral retinoblastoma with high-risk pathological features (massive choroidal infiltration, retrolaminar optic nerve invasion, or scleral infiltration) between August 2013 and March 2024. The final date of follow-up was March 21, 2024., Interventions: Patients were randomly assigned to receive either 3 (n = 94) or 6 (n = 93) cycles of CEV chemotherapy regimen after enucleation., Main Outcomes and Measures: The primary end point was disease-free survival, with a noninferiority margin of 12%. Secondary end points encompassed overall survival, safety, economic burden, and the quality of life of children., Results: All 187 patients (median [IQR] age, 25.0 [20.0-37.0] months; 83 [44.4%] female) completed the trial. Median (IQR) follow-up was 79.0 (65.5-102.5) months. Five-year disease-free survival was 90.4% for the 3-cycle group vs 89.2% for the 6-cycle group (difference, 1.2% [95% CI, -7.5% to 9.8%]), which met the noninferiority criterion (P = .003 for noninferiority). The 6-cycle group experienced a higher frequency of adverse events, greater reduction in quality of life scores, and increased costs compared with the 3-cycle group., Conclusions and Relevance: Among patients with unilateral pathologic high-risk retinoblastoma, 3 cycles of CEV chemotherapy resulted in 5-year disease-free survival that was noninferior to 6 cycles of CEV chemotherapy., Trial Registration: ClinicalTrials.gov Identifier: NCT01906814.

Purpose: To investigate the timing of enucleation, treatment course, and outcome for retinoblastoma (RB) with optic nerve (ON) invasion on imaging., Study Design: Retrospective clinical study., Methods: Of the 160 patients with RB who presented to the National Center for Child Health and Development in Japan between 2005 and 2022, ON invasion on imaging at the initial presentation was seen in five patients. The clinical, computed tomography (CT), and magnetic resonance imaging (MRI) findings, and treatment courses were reviewed retrospectively., Results: MRI showed ON invasion in all five patients (three with unilateral RB, 2 with bilateral RB); in two patients CT detected no invasion. Enucleation was performed in four patients, three of whom underwent neoadjuvant therapy and one had a positive ON resection margin following the enucleation as initial treatment. One patient did not undergo enucleation due to cerebrospinal fluid dissemination. All enucleated patients underwent adjuvant chemotherapy. Four patients underwent radiotherapy. During follow-up (mean, 89.4 months), four patients survived and one died., Conclusion: MRI is recommended to evaluate ON invasion and determine the timing of enucleation for RB. The appropriate choice of neoadjuvant or adjuvant therapy would be helpful to avoid radiotherapy for RB with ON invasion on imaging., Competing Interests: Declarations. Conflict of interest: T. Onishi, None; S. Nishina, None; T. Yokoi, None; T. Yoshida, None; S. Hayashi, None; H. Anzai, None; N. Azuma, None; C. Kiyotani, None; K. Terashima, None; T. Yoshioka, None; H. Ogiwara, None; H. Fuji, None; M. Kitamura, None; Y. Tsutsumi, None., (© 2024. The Author(s).)

Abstract Background Parvimonas micra is known as a causative agent of chronic periodontal disease. This Gram-positive obligate anaerobic coccus was cultured from the ocular surface of blowout fracture-related orbital cellulitis progressing to panophthalmitis. Case presentation The patient was a woman in her fifties who had panic disorder and subsequently was a victim of domestic violence. These factors led to delayed consultation. At the initial visit to an ophthalmologist, the ocular surface of the right eye was covered with pus. Swelling of the upper and lower eyelids prevented the eyelid from closing and exophthalmos, severe corneal ulcer, panophthalmitis, and no light perception were observed. Head computed tomography revealed an old blowout fracture and chronic sinusitis with orbital cellulitis. P. micra were isolated from culture of pus samples from the sinus and from the ocular surface. Conclusions There is a possibility that P. micra invaded the orbit via the fragile bony site and caused orbital cellulitis, severe corneal ulcer, and panophthalmitis that required enucleation. In cases of coexisting old blowout fracture and chronic sinusitis, the chronic sinusitis should be treated as quickly as possible.

PurposeTo measure orbital volume using serial magnetic resonance imaging (MRI) scans to determine the effect of enucleation on orbital growth over time.MethodsThe medical records of patients who underwent unilateral enucleation for retinoblastoma with a minimum of 2 MRIs were retrospectively reviewed. Orbital asymmetry was calculated using MRI measurements. Nonlinear and linear mixed effect regression models were used to predict the effect of age at time of enucleation on degree of orbital asymmetry.ResultsA total of 27 patients were included (mean age at enucleation, 2 years; range, 2.5 months to 5 years). Age at scan (P = 0.046) and age at enucleation (P = 0.0006) were found to have a significant effect on orbital asymmetry. Change in orbital asymmetry over time was more pronounced after enucleation in children enucleated at

PurposeTo assess the ability of trabecular micro-bypass stents to improve aqueous humor outflow (AHO) in regions initially devoid of AHO as assessed by aqueous angiography.MethodsEnucleated human eyes (14 total from 7 males and 3 females [ages 52-84]) were obtained from an eye bank within 48 hours of death. Eyes were oriented by inferior oblique insertion, and aqueous angiography was performed with indocyanine green (ICG; 0.4%) or fluorescein (2.5%) at 10 mm Hg. With an angiographer, infrared and fluorescent images were acquired. Concurrent anterior segment optical coherence tomography (OCT) was performed, and fixable fluorescent dextrans were introduced into the eye for histologic analysis of angiographically positive and negative areas. Experimentally, some eyes (n = 11) first received ICG aqueous angiography to determine angiographic patterns. These eyes then underwent trabecular micro-bypass sham or stent placement in regions initially devoid of angiographic signal. This was followed by fluorescein aqueous angiography to query the effects.ResultsAqueous angiography in human eyes yielded high-quality images with segmental patterns. Distally, angiographically positive but not negative areas demonstrated intrascleral lumens on OCT images. Aqueous angiography with fluorescent dextrans led to their trapping in AHO pathways. Trabecular bypass but not sham in regions initially devoid of ICG aqueous angiography led to increased aqueous angiography as assessed by fluorescein (P = 0.043).ConclusionsUsing sequential aqueous angiography in an enucleated human eye model system, regions initially without angiographic flow or signal could be recruited for AHO using a trabecular bypass stent.

Purpose: To report a patient with bilateral scleritis who was initially treated for infectious scleritis, and subsequently diagnosed as granulomatosis with polyangiitis (GPA)., Method: Retrospective chart review., Result: A 48-year-old female with a known history of diabetes presented with pain, redness, and blurring of vision in the right eye. She was diagnosed as scleritis with retinal detachment and underwent vitrectomy, with silicone oil tamponade and intravitreal injections of antibiotics, and antifungal agents. She presented with active scleritis with additional multiple pus points. Several scleral biopsies failed to yield any microorganism and finally, the painful blind eye was enucleated. The enucleated specimen grew gram-positive bacteria which were identified as Staphylococcus arlettae by polymerase chain reaction (PCR)-based sequencing of the 16S rRNA gene. Nine months after the onset of symptoms in the right eye, the patient developed necrotizing scleritis in the left eye. Laboratory investigation revealed a positive cytoplasmic- anti-neutrophil cytoplasmic autoantibody, which was previously negative during the right eye involvement. She was diagnosed with GPA by a rheumatologist and treated accordingly., Conclusion: Retinal detachment may be associated with scleritis, and ANCA testing may not detect GPA in its early stages. Once infection has been excluded, clinicians should not be afraid to use high-dose immunosuppression instead of surgery to treat retinal detachment associated with scleritis.

A 28-year-old male presented to the emergency room suffering an ocular burn injury from a welding rod. Given the mechanism of injury, severe delayed injury of the ocular adnexa occurred, requiring enucleation, partial exenteration of the superior orbit, and extensive reconstruction. Histopathology of the affected tissue was analyzed. This is the first report that details the clinical course of a patient with delayed high amperage and low voltage electrical burn injury.

Objective: To assess the impact of evolving criteria for group E retinoblastoma on ocular survival outcomes., Design: A retrospective observational study., Methods: Single-institution consecutive case series of patients with advanced intraocular retinoblastoma (groups D and E) were classified based on International Intraocular Retinoblastoma Classification (IIRC) and International Classification of Retinoblastoma (ICRB) criteria. The main outcomes measured were ocular survival, frequency of histopathologic risk factors (HRF), and the need for adjuvant therapy., Results: A total of 332 eyes of 298 patients were classified into group D (150, 45%) and E eyes (182, 55%) based on IIRC criteria. ICRB classification resulted in upstaging of 57 group D eyes (17%) to group E. Eyes that were upstaged to group E from D in the ICRB classification (E1) differed significantly, with a greater proportion undergoing primary enucleation (17 of 57, 30%) than those that were not (10 of 93, 11%) (p = 0.003). Similar significant differences were observed between group E2 and E3 eyes (p < 0.0001). Ocular survival according to Kaplan-Meier estimates at 12 months of 79%, 59%, 49%, and 1% differed significantly between all groups (ICRB D, E1, E2, and E3, respectively)., Conclusion: Proposed new subgrouping of group E eyes into E1, E2, and E3 based on clinical criteria is based upon natural history of tumor progression and is predictive of ocular survival. Preservation of the existing lower boundaries for group E by ICRB and IIRC offers the possibility of reanalyzing existing published data., Competing Interests: Footnotes and Disclosure Supported Support provided by The Operation Eyesight Universal Institute for Eye Cancer (SK) and Hyderabad Eye Research Foundation (SK), Hyderabad, India. The funders had no role in preparing, reviewing, or approving the manuscript. No conflicting relationship exists for any author., (Copyright © 2024 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the effectiveness of preventing metastasis for each major treatment modality for iris melanoma., Design: Retrospective case series., Participants: Three hundred consecutive eyes with iris melanoma at a single tertiary referral centre for ocular oncology., Methods: Retrospective analysis of eyes with iris melanoma, both with (n = 69 eyes) and without (n = 231 eyes) ciliary body extension, was undertaken for metastasis-free survival at 5, 10, and 20 years based on type of treatment, including globe-sparing surgical resection (n = 169 eyes), plaque radiotherapy (n = 74 eyes), or enucleation (n = 57 eyes)., Results: For the total population, 5-, 10-, and 20-year metastasis-free survival rates were 95%, 93%, and 87%, respectively, and there was no difference in metastatic rates for tumours with versus without ciliary body extension (p = 0.95). Noninferiority was demonstrated for surgical resection and plaque radiotherapy, with metastasis-free survival rates of 98%, 97%, and 94% for surgical resection and 94%, 94%, and 89% for plaque radiotherapy (p = 0.002). The rates for globe salvage were 94%, 92%, and 90% for surgical resection and 94%, 86%, and 86% for plaque radiotherapy (p = 0.003). However, metastasis-free survival was worse in patients who underwent enucleation (86%, 67%, and NA; p < 0.001)., Conclusions: Metastasis-free survival and globe salvage following plaque radiotherapy and surgical resection are not inferior to either, but eyes undergoing enucleation demonstrated a lower metastasis-free survival, likely because enucleation is performed for larger, more extensive melanomas, often with secondary glaucoma. In this analysis, iris melanoma with ciliary body involvement did not increase the risk of metastasis., (Copyright © 2023 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

The role of experience in the development and maintenance of emergent network properties such as cortical oscillations and states is poorly understood. To define how early-life experience affects cortical dynamics in the visual cortex of adult, head-fixed mice, we examined the effects of two forms of blindness initiated before eye opening and continuing through recording: (1) bilateral loss of retinal input (enucleation) and (2) degradation of visual input (eyelid suture). Neither form of deprivation fundamentally altered the state-dependent regulation of firing rates or local field potentials. However, each deprivation caused unique changes in network behavior. Laminar analysis revealed two different generative mechanisms for low-frequency synchronization: one prevalent during movement and the other during quiet wakefulness. The former was absent in enucleated mice, suggesting a mouse homolog of human alpha oscillations. In addition, neurons in enucleated animals were less correlated and fired more regularly, but no change in mean firing rate. Eyelid suture decreased firing rates during quiet wakefulness, but not during movement, with no effect on neural correlations or regularity. Sutured animals showed a broadband increase in depth EEG power and an increased occurrence, but reduced central frequency, of narrowband gamma oscillations. The complementary-rather than additive-effects of lid suture and enucleation suggest that the development of emergent network properties does not require vision but is plastic to modified input. Our results suggest a complex interaction of internal set points and experience determines mature cortical activity, with low-frequency synchronization being particularly susceptible to early deprivation., Competing Interests: The authors declare no competing financial interests., (Copyright © 2024 the authors.)

The patient is a 2-year-old male. The family consulted the Department of Ophthalmology, Shanghai Ninth People's Hospital, after noticing a white reflection in the pupil area of the child's right eye for 6 days. Following a thorough ocular and systemic examination, the patient was diagnosed with retinoblastoma (Group E, cT2bN0M0) of the right eye. The right eye was enucleated and classified as pathological stage pT3cN0M0. Postoperatively, systemic intravenous chemotherapy with the VEC regimen was administered. Genetic testing revealed a germline mutation in the RB1 gene: c.874 (exon9) delT (p.Tyr292fsTer9), necessitating close monitoring of the socket during follow-up visits. Three months after the operation, fundus examination revealed yellow-white lesions in the left eye, and bilateral retinoblastoma was diagnosed (Group E in the right eye, Group C in the left eye). Based on the ICRB and pTNM stages, the patient underwent six rounds of systemic intravenous chemotherapy and three rounds of cryotherapy in the left eye. No recurrence was detected with a 4-year follow-up. The patient was initially diagnosed with unilateral retinoblastoma, but later developed the disease in the contralateral eye during treatment, which was a case of metachronous bilateral retinoblastoma.

Purpose: To describe our experience over 6 years using a new high-density polyethylene-based spherical integrated porous orbital implants (Oculfit)., Methods: This is an observational retrospective case series study analyzing all cases requiring Oculfit implants between February 2015 and September 2021. Clinical information regarding the population included, the characteristics of the implant, and the outcomes and complications during the follow-up were noted. The success of the implant was defined according to anatomical and functional parameters., Results: The study analyzed 90 cases of anophthalmic patients. The main causes for enucleation or evisceration were ocular decompensations (36.7%) and neoplasms (27.8% uveal melanoma and 7.8% retinoblastoma). Anatomical success was identified in 63 (70.0%) cases, functional success in 79 (87.8%) and complete success (anatomical + functional) in 61 (67.7%) cases. Factors associated with the functional success were age and exposure of the primary orbital implant. Complications appeared in 11 (12.2%) cases, which were completely resolved without sequelae in 4 (4.4%). Orbital explant was required in 5 (5.6%) cases., Conclusion: In our experience, Oculfit can be considered a useful alternative among the currently available options for orbital implants and has a good efficacy/safety profile., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

During outdoor work in April 2022, a 48-year-old man was stabbed by a tree branch and underwent intraocular foreign body extraction and repair of the scleral wound with sutures and amniotic membrane graft at a local hospital. Steroid therapy with prednisone was prescribed after a diagnosis of uveitis. Vitrectomy was performed in June 2023; a fungal culture was positive, and ITS sequencing identified the organism as Paradictyoarthrinium diffractum. Empiric antifungal therapy did not have an effect, and, because of deterioration of the condition, the left eye was enucleated in October 2023. P. diffractum is a mangrove host-specific saprophytic fungus that has not been reported in humans., Competing Interests: Declarations of competing interest The authors have no competing interests to declare., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

We investigated whether magnetic resonance imaging can visualize the distribution in the vitreous cavity via eye drops of ophthalmic solutions, gadolinium-based contrast agent, and 17 O-water, and to clarify the usefulness of ultra-heavily T2-weighted sequences in the research of intraocular distribution. Five different solutions (V-ROHTO, TRAVATANZ, gadobutrol, H 2 17 O, and saline) were administered to excised pig eye specimens. The samples were scanned using T1 mapping, T2 mapping, 3D T2-weighted (echo times (TE): 500, 3200, and 4500 ms), a half-Fourier single-shot turbo-spin echo sequence (HASTE; TE: 440 and 3000 ms), and 3D-real inversion-recovery before eye drops administration. Subsequently, we used a plastic dropper to drop a 0.5 mL solution each, and images were obtained up to 26 h later. Temporal changes in the T1 and T2 values of the anterior chamber and vitreous cavity were compared. The other sequences were evaluated by determining temporal signal changes as signal intensity ratio (SIR) compared to "No drop." The T1 and T2 values of samples treated with gadobutrol and H 2 17 O decreased over time. The SIR of samples treated with gadobutrol and H 2 17 O showed remarkable changes in the 3D T2-weighted images, whereas no remarkable temporal changes were observed in the other solutions. Longer TEs resulted in remarkable changes. We demonstrated that visualization of distribution in the vitreous cavity via eye drops could be achieved with excised pig eyes using gadobutrol and H 2 17 O, but not with ophthalmic solutions. Ultra-heavily T2-weighted sequences may be promising for the early and highly sensitive visualization of the intraocular distribution of eye drops., (© 2024. The Author(s).)

Sensory development is a complex process that can influence physiological and pathological factors. In laterally-eyed mammals, monocular enucleation (ME) during development and the subsequent lack of external sensory stimuli can result in permanent morphological and physiological changes. Malnutrition, especially in early life, also can cause permanent morphofunctional changes due to inadequate nutrient intake in both hemispheres. This study investigated the effects of early (postnatal day 7) ME and malnutrition during the suckling period on cortical excitability in adulthood (110-140 days of life). For this, we compared the speed propagation of cortical spreading depression in the occipital and parietal cortex of malnourished and well-nourished adult rats, previously suckled small-sized litters with three pups (L3/dam) medium-sized litters with six pups (L6/dam), and large-sized litters with twelve pups (L12/dam). The CSD velocity was augmented by the ME in the contralateral side of the removed eye in the parietal and occipital cortex. These findings suggest that visual sensory input deprivation is associated with permanent functional changes in the visual pathways, which can alter cortical excitability and lead to modifications in CSD propagation., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background/aims: Anterior segment ischaemia (ASI) is a rare but serious complication of strabismus surgery, which may be caused by damage to the anterior ciliary arteries that run along the rectus muscles. To avoid ASI, clinical praxis is to operate on a maximum of two rectus muscles at a time. The aim of this study was to perform a detailed study of the contribution of the four ocular rectus muscles to the perfusion of the anterior segment using laser speckle contrast imaging (LSCI)., Methods: The four rectus muscles were successively detached during enucleation in nine patients with suspected uveal melanoma. Perfusion of the anterior segment was monitored with LSCI during the detachment of each of the rectus muscles, and after the ophthalmic artery had been severed., Results: Perfusion of the anterior segment, measured in the paralimbal tissue, decreased gradually as the four rectus muscles were detached, showing a decrease to 98% when the first (lateral rectus) muscle was detached, to 88% when the second (medial rectus), and 69% when the third (inferior rectus) muscles were detached. The decrease was more pronounced and statistically significant when the fourth (superior rectus) muscle was detached (to 28%, p=0.0102)., Conclusion: LSCI is a valuable perfusion monitoring tool, as it has the capability to visualise the gradual decrease in anterior segment perfusion as the rectus muscles are successively detached. Further studies are needed to determine how many rectus muscles can be safely detached during strabismus surgery without risking ASI., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To retrospectively investigate clinical characterization and the long-term postoperative outcomes of retinoblastoma (RB) patients receiving enucleation with primary orbital implantation in early infancy (0-6 months old)., Methods: The clinical and follow-up data of 42 RB patients receiving enucleation with primary orbital implantation in early infancy at Beijing Tongren Hospital from December 2009 to January 2020 were analysed. The average follow-up time was 83 months. The patient group included 24 males and 18 females, 30 unilateral and 12 bilateral cases. A total of 44 eyes with 10 in stage D and 34 in stage E underwent 40 unilateral and 2 bilateral surgeries. 17 RB eyes received hydrogel and 27 RB eyes received hydroxyapatite implants. This study was performed by following the guideline of STROBE., Results: Enucleation combined with primary orbital implantation promoted survival and was safe with few and minor complications such as increased secretion, upper eyelid ptosis, and sunken eye sockets which were not affected by stages, lateralities, or implant materials. 55-80% RB patients exhibited satisfactory appearance and obvious or moderate motility of orbital implants according to the evaluation by doctors and family members. Family members were likely more optimistic about the appearance and more pessimistic about motility of the orbital implantation than doctors did.The quality of life was high as indicated by PedsQL3.0 or PedsQL4.0 scores ( ≧ 90 for > 75% patients). It was not affected by the stages, laterality, and implant materials, nor affected by the appearance and motility of the implants., Conclusions: The outcomes of the combination of enucleation and primary orbital implantation for pertinent RB patients in early infancy are generally satisfactory with few and minor complications, high safety, appearance, and overall quality of life. Enucleation combined with primary orbital implantation in early infancy benefits pertinent RB patients in appearance, survival, and quality of life., (© 2024. The Author(s).)

Backgrounds: Iris nodules are frequently noted as clinical manifestations of neurofibromatosis type 1 but the other intraocular manifestations are rare. The purpose of this study is to present a patient with a phthisic eye who underwent enucleation for a cosmetic reason after 15-year follow-up and also to review 14 patients with enucleation described in the literature., Case Presentation: A 17-year-old man with neurofibromatosis type 1 from infancy underwent the enucleation of phthisic left eye and also had the resection of eyelid subcutaneous mass lesions on the left side for a cosmetic reason. He had undergone four-time preceding surgeries for eyelid and orbital mass reduction on the left side in childhood and had developed total retinal detachment 10 years previously. Pathologically, the enucleated eye showed massive retinal gliosis positive for both S-100 and glial fibrillary acidic protein (GFAP) in the area with involvement of the detached retinal neuronal layer, together with a more fibrotic lesion along the choroid which were, in contrast, negative for both S-100 and GFAP. The choroid, ciliary body, and iris did not show apparent neurofibroma while episcleral neurofibroma was present., Literature Review: In review of enucleated eyes of 14 patients in the literature, buphthalmic eyes with early-onset glaucoma on the unilateral side was clinically diagnosed in 9 patients who frequently showed varying extent of hemifacial neurofibromatosis which involved the eyelid and orbit on the same side. Pathologically, neurofibromas in varying extent were found in the choroid of 12 patients. One patient showed choroidal malignant melanoma on the left side and fusiform enlargement of the optic nerve on the right side suspected of optic nerve glioma. The phthisic eye in another patient showed massive retinal gliosis similar to the present patient., Conclusions: In summary of the 15 patients with neurofibromatosis type 1, including the present patient, buphthalmic or phthisic eyes with no vision were enucleated for cosmetic reasons and showed choroidal neurofibroma in most patients and massive retinal gliosis in two patients including the present patient., (© 2024. The Author(s).)

Purpose: To examine histological characteristics and differences between drusen beneath the retinal pigment epithelium (small hard drusen) located in the macula and located in the parapapillary region., Methods: We histomorphometrically examined human eyes enucleated due to uveal melanomas or secondary angle-closure glaucoma., Results: The study included 106 eyes (age, 62.6 ± 15.2 years) with macular drusen (n = 7 globes) or parapapillary drusen (n = 29 eyes) and 70 eyes without drusen. In all drusen, periodic-acid-Schiff-positive material was located between the RPE basal membrane and the inner collagenous layer of Bruch's membrane (BM). Macular drusen as compared with parapapillary drusen had lower height (15.2 ± 10.1 µm versus 34.3 ± 19.8 µm; P = 0.003), while both groups did not differ significantly in basal drusen width (74.0 ± 36.3 µm versus 108.7 ± 101.0 µm; P = 0.95). Eyes with macular drusen and eyes without drusen did not differ significantly in BM thickness (2.74 ± 0.44 µm versus 2.55 ± 0.88 µm; P = 0.57) or in RPE cell density (35.4 ± 10.4 cells/480 µm versus 32.8 ± 7.5 cells/480 µm; P = 0.53), neither in the drusen region nor in the drusen vicinity, while BM thickness (4.60 ± 1.490 µm; P < 0.001) and RPE cell density (56.9 ± 26.8 cells/480 µm; P = 0.005) were higher at the parapapillary drusen. Eyes with macular drusen, eyes with parapapillary drusen, and eyes without drusen did not differ significantly in choriocapillaris density (all P > 0.10) and thickness (all P > 0.35). Limitations of the study, among others, were a small number and size of drusen examined, diseases leading to enucleation, lack of serial sections, limited resolution of light microscopy, and enucleation-related and histological preparation-associated artefacts., Conclusions: The findings of this study, also taking into account its methodological limitations, suggest that macular drusen and parapapillary drusen shared the morphological feature of periodic-acid-Schiff-positive material between the RPE basal membrane and BM and that they did not vary significantly in choriocapillaris thickness and density. RPE cell density and BM thickness were higher in parapapillary drusen than in macular drusen., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

A novel form of injury associated with obstructive sleep apnea (OSA) that was comorbid with obesity hypoventilation syndrome and severe daytime somnolence is reported in a 55-year-old woman, manifesting as severe ocular and extraocular muscle injuries sustained from suddenly falling asleep and colliding with a sharp object, resulting in surgical enucleation of the right eye and orbital implant. The literature on injuries (falls, motor vehicle accidents) related to OSA and excessive day time sleepiness is reviewed, along with the literature on injuries from OSA-related parasomnias. The diverse health hazards, including physical injury, associated with OSA-excessive daytime sleepiness, are emphasized, further encouraging the need to educate primary care providers on early detection of OSA with prompt treatment intervention., Citation: Baker N, Schenck CH, Golden E, Varghese R. A case of accidental self-enucleation caused by obstructive sleep apnea. J Clin Sleep Med . 2024;20(8):1395-1397., (© 2024 American Academy of Sleep Medicine.)

Purpose: Retinoblastoma, a curable childhood cancer, has been identified as a tracer cancer in the WHO Global Initiative for Childhood Cancer. To document the outcomes of children with retinoblastoma in South Africa, treated as per the first prospective standard national treatment guidelines for childhood cancer in South Africa., Patients and Methods: All children diagnosed with retinoblastoma between 2012 and 2016 in five South African pediatric oncology units were treated with a standard treatment on the basis of the International Society of Pediatric Oncology-Pediatric Oncology in Developing Countries guidelines for high-income settings. Treatment included focal therapy with/without chemotherapy, or enucleation with/without chemotherapy, and orbital radiotherapy, depending on enucleated eye histology. The end point was survival at 24 months, using Kaplan-Meier curves with log-rank (Mantel-Cox) and chi-square (χ2) tests with respective P values reported., Results: A total of 178 children were included in the study; 68% presented with unilateral disease. The median age was 27 months (range 0-118 months) with a male:female ratio of 1:0.75. The overall survival was 79% at 24 months with significant association with stage at diagnosis ( P < .001) and older age over 2 years as opposed to younger than 2 years ( P < .001). Causes of death were disease progression/relapses in 90% (34 of 38) and unknown in 2% (1 of 38), whereas treatment abandonment was 1.7% (3 of 178)., Conclusion: Efficacy with national treatment guidelines was confirmed, and feasibility of implementing standard national childhood cancer treatment guidelines was documented, involving multidisciplinary teams in South Africa. Outcome was significantly associated with stage at diagnosis and age.

Purpose: The purpose of the study is to analyze the structural and functional alterations of the lacrimal gland and its tear secretion post-enucleation in Wistar rats. Method: Adult male Wistar rats (n = 15) of 8-week-old were randomly assigned into three groups viz. control, sham, and experimental group (n = 5). Rats of the experimental group were subjected to enucleation of the right eye. Under aseptic surgical conditions, with sterilized forceps, right eyeball of the rats of experimental group was exposed completely out of the socket by applying pressure on the lateral canthus of the eye. Enucleation was then achieved after holding the optic nerve tightly. The surgical procedure was similar in sham group without enucleation. Quantity of tears (Basal and reflex) secreted in both eyes in rats of all groups were measured by using Schirmer's strip, pre- and post-three weeks of enucleation. The lacrimal gland was harvested to analyze histopathological (structural) alterations. Results: Pr- and post-enucleation there was no significant difference observed in the tear volume across the groups. Histopathology of the lacrimal glands from all groups showed preserved lobular architecture with serous acini arranged in lobules, intralobular and interlobular ducts, interstitial fibro collagenous tissue. There was no glandular distortion and atrophy in experimental group. Conclusion: Enucleation do not co-relate or affect the tear volume and lacrimal gland acinar microstructural changes in an anophthalmic socket co-relating to the contralateral normal functional eye. [ABSTRACT FROM AUTHOR]

Background: Parvimonas micra is known as a causative agent of chronic periodontal disease. This Gram-positive obligate anaerobic coccus was cultured from the ocular surface of blowout fracture-related orbital cellulitis progressing to panophthalmitis.Case Presentation: The patient was a woman in her fifties who had panic disorder and subsequently was a victim of domestic violence. These factors led to delayed consultation. At the initial visit to an ophthalmologist, the ocular surface of the right eye was covered with pus. Swelling of the upper and lower eyelids prevented the eyelid from closing and exophthalmos, severe corneal ulcer, panophthalmitis, and no light perception were observed. Head computed tomography revealed an old blowout fracture and chronic sinusitis with orbital cellulitis. P. micra were isolated from culture of pus samples from the sinus and from the ocular surface.Conclusions: There is a possibility that P. micra invaded the orbit via the fragile bony site and caused orbital cellulitis, severe corneal ulcer, and panophthalmitis that required enucleation. In cases of coexisting old blowout fracture and chronic sinusitis, the chronic sinusitis should be treated as quickly as possible. [ABSTRACT FROM AUTHOR]

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In order to simulate apoptotic processes in the central nervous system and retina, one eyeball was enucleated in each of the male Wistar rats (n = 10) aged 10–12 weeks. Simultaneously, in one of the series of experiments (n = 5), 100 000 PKH67-labeled mesenchymal stem cells (MSCs) in 100 µL of buffer solution were injected parabulbarically on the side of an intact eye. In another series of experiments on rats (n = 5), immediately after enucleation, on one side of the eye, 100 000 PKH67-labeled MSCs in 100 µL of buffer solution were injected intranasally into the upper part of the nasal cavity. Three days after enucleation, brain and retinal slices were prepared on a cryostat. In both series of experiments, the distribution of fluorescent MSCs was determined in the mesencephalic quadruplet bodies, diencephalic lateral geniculate body, and intact eye's retina. It was inferred that intranasal perineural implantation of MSCs, due to its simplicity and efficiency, is a promising alternative method of MSC delivery into the retina of patients with signs of dystrophic and degenerative processes. [ABSTRACT FROM AUTHOR]

Rationale: Primary adenocarcinoma of the lacrimal gland is rare, and its presentation as corneal perforation is even rarer. Corneal perforation is an ocular emergency that warrants urgent ophthalmic surgery, yet complete staging of lacrimal gland tumor pre-operatively is essential for optimal oncological management. Patient’s concerns: A 57-year-old man presented with left eye pain was found to have left eye proptosis and fleshy tissue mass around the eyeball. Uveal tissue was prolapsing over the perforated keratitis cornea, and the eye was full of discharge. Diagnosis: Bedside ultrasound B-scan confirmed vitreous haze, and emergency contrast computed tomography (CT) revealed soft tissue density mass (>40 mm) molding around the left globe and optic nerve without any rim enhancing abscess. Left exogenous endophthalmitis from exposure keratopathy secondary to proptosis caused by the bulky lacrimal tumor located in the confined orbital cone was diagnosed. Interventions: Emergency enucleation surgery of the left eyeball was done for this painful blind eye to control the infection from spreading. Orbital walls were biopsied intra-operatively, and tumor staging was completed by positron emission tomography- CT scan and magnetic resonance imaging. Without evidence of metastasis, left orbital exenteration was followed by adjuvant orbital chemoradiotherapy for the sake of close proximity of resection margin. Outcomes: Left exenterated orbit was fully epithelialized at around 2 months, and there was no recurrence of the disease up to present at the 1 year follow-up. Lessons: Thorough workup on the staging of the disease to minimize the number of operations for oncological patients is always a top priority, yet it may not always be possible as in our case presenting with corneal perforation.

PURPOSE: Noninvasive two-photon imaging of a living mammalian eye can reveal details of molecular processes in the retina and RPE. Retinyl esters and all-trans-retinal condensation products are two types of retinoid fluorophores present in these tissues. We measured the content of these two types of retinoids in monkey and human eyes to validate the potential of two-photon imaging for monitoring retinoid changes in human eyes. METHODS: Two-photon microscopy (TPM) was used to visualize excised retina from monkey eyes. Retinoid composition and content in human and monkey eyes were quantified by HPLC and mass spectrometry (MS). RESULTS: Clear images of inner and outer segments of rods and cones were obtained in primate eyes at different eccentricities. Fluorescence spectra from outer segments revealed a maximum emission at 480 nm indicative of retinols and their esters. In cynomolgus monkey and human retinal extracts, retinyl esters existed predominantly in the 11-cis configuration along with notable levels of 11-cis-retinol, a characteristic of cone-enriched retinas. Average amounts of di-retinoid-pyridinium-ethanolamine (A2E) in primate and human eyes were 160 and 225 pmol/eye, respectively. CONCLUSIONS: These data show that human retina contains sufficient amounts of retinoids for two-photon excitation imaging. Greater amounts of 11-cis-retinyl esters relative to rodent retinas contribute to the fluorescence signal from both monkey and human eyes. These observations indicate that TPM imaging found effective in mice could detect early age- and disease-related changes in human retina.

PurposeTo evaluate the cellular nature of and diagnostic terminology used in connection with acquired retinal "vasoproliferative tumors."DesignRetrospective clinicopathologic study.MethodsClinical records and microscopic slides of 4 enucleated globes were reviewed. Special stains and immunohistochemical probes for CD31, CD34, p53, glial fibrillary acidic protein (GFAP), CD163, and Ki67 (cell replication) were employed; ultrastructural and fluorescence in situ hybridization (FISH) analyses were performed.ResultsTumors were located inferotemporally in middle-aged patients. They were uniformly composed of compacted elongated, GFAP-positive spindle cells (due to intermediate filaments identified ultrastructurally) with a Ki67 index of less than 1%. Rosenthal fibers and eosinophilic granular bodies were observed. Hyalinized periodic acid-Schiff-positive vessels were widely separated. CD31 and CD34 revealed a sparse microvasculature. Tumor-associated exudate spread predominantly subretinally. The retinal pigment epithelium had undergone extensive placoid fibrous metaplasia with focal ossification. P53 upregulation, BRAF-KIAA gene rearrangement, and IDH1R132H mutation typically associated with low-grade astrocytic neoplasms were absent.ConclusionsRetinal "vasoproliferative" tumors have been mischaracterized, because they actually display a paucity of microvessels. Proliferating fibrous astrocytes with a very low proliferation index predominate, without immunohistochemical or genetic evidence favoring a neoplasm. Subretinal exudate appeared capable of provoking widespread fibrous metaplasia of the pigment epithelium that was mainly responsible for secondary retinal damage. The term "reactive retinal astrocytic tumor" is proposed as more appropriate for this entity. In carefully selected progressive lesions, consideration should be given to earlier surgical intervention before extensive subretinal exudate accumulates and pigment epithelial proliferation with fibrous metaplasia ensues.

Purpose: Fitting a customized ocular prosthesis for anophthalmic patients entails an artisanal labour‐exhausting process and is standardly based on impression moulding of the socket, which may be anatomically inaccurate. The objective of the study was to design an impression‐free socket mould with three‐dimensional (3D) technology. Methods: The ex vivo anophthalmic socket models included one silicone, one fresh pig cadaver head and three fresh‐frozen human cadaver heads. After intra‐socket application with iodine substance, five observers obtained eighteen low‐dose cone beam computed tomography (CBCT) scans and one observer one high‐dose CBCT scan of each model. The observers designed non‐impression 3D moulds of the socket with 3D software. For the human cadaver sockets 3D geometric models of the ocular prosthesis were rendered from the 3D mould of the socket and the mirrored cornea of the contralateral eye. Results: The posterior surface of the 3D mould was highly accurate, with a mean absolute deviation of 0.28 mm, 0.53 mm, 0.37 mm and mean upper deviation of 0.53 mm, 0.86 mm, 1.17 mm, respectively, for the phantom, pig and human model. The intra‐ and interobserver repeatability and reproducibility of the 3D moulds and designs was good (<0.35 mm). The largest variation in the 3D geometric model was found at the junction of the 3D mould and mirrored cornea. Conclusion: 3D design of an impression‐free geometric model for an ocular prosthesis with low‐dose CBCT is highly accurate in ex vivo anophthalmic socket models. This novel method is a critical step towards the manufacturing of 3D printed ocular prostheses and requires validation in anophthalmic patients. [ABSTRACT FROM AUTHOR]

Background: Retinoblastoma (RB) is an intraocular malignant tumor detected in early childhood with variable global impact. Histopathological classification of the tumor in enucleated globes with RB is the key for the decision of adjuvant chemotherapy use. We aim to validate the use of adjuvant chemotherapy in cases with combined pre-laminar/intralaminar optic nerve (ON) invasion and focal choroidal invasion according to the American Joint Committee on Cancer (AJCC) 8th classification., Methods: This is a retrospective study conducted at King Abdulaziz University Hospital (KAUH) and King Khalid Eye Specialist Hospital (KKESH) in Riyadh, Saudi Arabia of all RB cases who underwent enucleation over 22 years (2000 to 2021). The histopathological findings were reviewed to identify the enucleated globes classified as pT2a tumors, as an inclusion criterion. Basic demographic and clinical data were collected via chart review Simple descriptive and basic statistical analysis of the data was used where applicable., Results: Thirty-one patients who had an enucleated globe with RB that fit into the above classification were included. Sixteen were males and 15 were females. The median age was 14 months (IQR = 14 months). Most of the patients (93.5%) had no family history of RB. The commonest presentation was leukocoria in 87.1% followed by squint in 32.3%. Fourteen patients (45.2%) were treated by enucleation alone while 17 patients (54.8%) received adjuvant chemotherapy. Out of these, 7 patients had unilateral RB and the remaining 10 patients had bilateral RB. None of our patients developed recurrence or metastatic disease irrespective of the indication for adjuvant chemotherapy use after a maximum period of follow up reaching 17.84 years and a median of 10.6 years (IQR = 5.92)., Conclusions: In patients with 8th AJCC histopathological classification of pT2a, chemotherapy following enucleation might not be justified. The outcome in our untreated group of patients did not differ from the treated group with the absence of metastasis after a relatively long period of follow up with a median exceeding 10 years in both groups. Therefore, the risk and benefit of post enucleation adjuvant chemotherapy in the treatment of unilateral RB should be carefully decided and discussed with the primary caregivers taking into consideration the most recent evidence and recommendations in the literature., (© 2024. The Author(s).)