Examining the Role of Early Diagnostic Imaging for Craniosynostosis in the Era of Endoscopic Suturectomy: A Single Institution Experience.

ABSTRACT: Endoscopic suturectomy is a minimally invasive surgical treatment for single-suture craniosynostosis in children between 1 and 4 months of age. This study sought to characterize the role played by diagnostic imaging in facilitating early surgical management with endoscopic suturectomy. The authors also characterized the overall diagnostic utility of imaging in patients assessed for abnormal head shape at their institution, regardless of surgical status. A retrospective cohort of children diagnosed with singlesuture synostosis undergoing either primary endoscopic suturectomy or open calvarial reconstruction at the authors' institution from 1998 to 2018 was first reviewed. Of 132 surgical patients, 53 underwent endoscopic suturectomy and 79 underwent open repair. There was no difference in the proportion of endoscopic and open surgery patients imaged preoperatively before (24.5% versus 35.4%; P = 0.24) or after (28.3% versus 25.3%; P = 0.84) craniofacial assessment. Stratifying by historical epoch (1998-2010 versus 2011-2018), there was also no difference found between preoperative imaging rates (63.6% versus 56.4%; P = 0.35). In another cohort of 175 patients assessed for abnormal head shape, 26.9% were imaged to rule out craniosynostosis. Positive diagnostic imaging rates were recorded for suspected unicoronal (100%), metopic (87.5%), lambdoidal (75.0%), sagittal (63.5%), multisuture (50%), and bicoronal (0%) synostosis. The authors conclude that the use of diagnostic imaging at their institution has not increased despite higher utilization of endoscopic suturectomy and need for expedient identification of surgical candidates.However, their results suggest that imaging may play a greater diagnostic role for suspected bicoronal, sagittal, and multi-sutural synostosis among sutural subtypes of synostosis.

Melanoma brain metastasis presentation, treatment, and outcomes in the age of targeted and immunotherapies.

BACKGROUND: Historically, the prognosis for patients who have melanoma brain metastasis (MBM) has been dismal. However, breakthroughs in targeted and immunotherapies have improved long-term survival in those with advanced melanoma. Therefore, MBM presentation, prognosis, and the use of multimodality central nervous system (CNS)-directed treatment were reassessed. METHODS: In this retrospective study, the authors evaluated patients with MBM who received treatment at Memorial Sloan Kettering Cancer Center between 2010 and 2019. Kaplan-Meier methodology was used to describe overall survival (OS). Recursive partitioning analysis and time-dependent multivariable Cox modeling were used to assess prognostic variables and to associate CNS-directed treatments with OS. RESULTS: Four hundred twenty-five patients with 2488 brain metastases were included. The median OS after an MBM diagnosis was 8.9 months (95% CI, 7.9-11.3 months). Patients who were diagnosed with MBM between 2015 and 2019 experienced longer OS compared to those who were diagnosed between 2010 and 2014 (OS, 13.0 months [95% CI, 10.47-17.06 months] vs 7.0 months [95% CI, 6.1-8.3 months]; P = .0003). Prognostic multivariable modeling significantly associated shortened OS independently with leptomeningeal dissemination (P < .0001), increasing numbers of brain metastases at diagnosis (P < .0001), earlier MBM diagnosis year (P = .0008), higher serum levels of lactate dehydrogenase (P < .0001), receipt of immunotherapy before MBM diagnosis (P = .003), and the presence of extracranial disease (P = .02). The use of different CNS-directed treatment modalities was associated with presenting symptoms, diagnosis year, number and size of brain metastases, and the presence of extracranial disease. Multivariable analysis demonstrated improved survival for patients who underwent craniotomy (P = .01). CONCLUSIONS: The prognosis for patients with MBM has improved within the last 5 years, coinciding with the approval of PD-1 immune checkpoint blockade and combined BRAF/MEK targeting. Improving survival reflects and may influence the willingness to use aggressive multimodality treatment for MBM. LAY SUMMARY: Historically, melanoma brain metastases (MBM) have carried a poor survival prognosis of 4 to 6 months; however, the introduction of immunotherapy and targeted precision medicines has altered the survival curve for advanced melanoma. In this large, single-institution, contemporary cohort, the authors demonstrate a significant increase in survival of patients with MBM to 13 months within the last 5 years of the study. A worse prognosis for patients with MBM was significantly associated with the number of metastases at diagnosis, previous exposure to immunotherapy, spread of disease to the leptomeningeal compartment, serum lactate dehydrogenase elevation, and the presence of extracranial disease. The current age of systemic treatments has also been accompanied by shifts in the use of central nervous system-directed therapies.

Cerebrospinal fluid diversion for leptomeningeal metastasis: palliative, procedural and oncologic outcomes.

BACKGROUND: Leptomeningeal metastasis (LM) occurs in 3-5% of patients with solid metastatic tumors and often portends a severe prognosis including symptomatic hydrocephalus and intracranial hypertension. Cerebrospinal fluid (CSF) shunting can provide symptomatic relief in this patient subset; however, few studies have examined the role of shunting in the palliation, prognosis and overall oncologic care of these patients. OBJECTIVE: To identify and evaluate risk factors associated with prognosis after CSF diversion and assess surgical, symptomatic and oncologic outcomes in this population. METHODS: A retrospective study was conducted on patients with solid-malignancy LM treated with a shunt at a NCI-designated Comprehensive Cancer Center between 2010 and 2019. RESULTS: One hundred and ninety patients with metastatic LM underwent CSF diversion. Overall survival was 4.14 months from LM diagnosis (95% CI: 3.29-4.70) and 2.43 months (95% CI: 2.01-3.09) from shunting. Karnofsky performance status (KPS) at time of shunting and brain metastases (BrM) number at LM diagnosis demonstrated significant associations with survival (HR = 0.66; 95% CI [0.51-0.86], p = 0.002; HR = 1.40; 95% CI [1.01-1.93] per 10 BrM, p = 0.04, respectively). Eighty-three percent of patients experienced symptomatic relief, and 79% were discharged home or to rehabilitation facilities post-shunting. Post-shunt, 56% of patients received additional systemic therapy or started or completed WBRT. Complications included infection (5%), symptomatic subdural hygroma/hematoma (6.3%), and shunt externalization/removal/repair (8%). Abdominal seeding was not identified. CONCLUSIONS: CSF diversion for LM with hydrocephalus and intracranial hypertension secondary to metastasis can achieve symptomatic relief, hospital discharge, and return to further oncologic therapy, with a complication profile unique to this pathophysiology. However, decision-making in this population must incorporate end-of-life goals of care given limited prognosis.

Orbital Apex Syndrome Secondary to Invasive Aspergillus Infection: A Case Series and Literature Review.

BACKGROUND: Invasive fungal sinusitis carries high morbidity and mortality and often poses a diagnostic challenge. Orbital apex syndrome (OAS) is not an uncommon presentation in the setting of invasive fungal sinusitis. Delays in diagnosis and appropriate treatment can result in permanent visual dysfunction and, potentially, death. We present 2 cases of OAS secondary to invasive sinus aspergillosis, detailing the diagnostic process, treatment, and outcome for both patients. Subsequently, we present a review of the literature and combined analysis of our 2 patients plus 71 cases from previously published reports. METHODS: Literature review was performed to identify demographic, diagnostic, clinical, and treatment data of patients with OAS caused by Aspergillus species. RESULTS: The review resulted in 52 included articles with 71 patients, plus our 2 reported patients, leading to a total of 73 subjects included in the analysis. The average age of patients at presentation was 59.9 years. A combination of visual disturbance and pain (headache and/or periocular pain) was the most common presentation reported (46 cases; 63%). Diabetes mellitus was reported in 15 cases (21%), with more than half specifically noted to have poorly controlled diabetes. After diabetes, the second most common cause of immunocompromise was chronic steroid use (n = 13; 18%). Empiric antifungal treatment was started in 10 patients (14%), while 25 patients (34%) were first treated with systemic steroids due to a concern for an inflammatory etiology. Time to diagnosis from initial presentation was on average 7.4 weeks (range of 0.3-40 weeks). Approximately 78% of the cases (57 of 73) had biopsies with histology that confirmed Aspergillus fungal morphology, and 30/73 (41%) had diagnostic fungal cultures. The majority of the cases received monotherapy with intravenous (IV) amphotericin B (36 patients; 49%) and IV voriconazole (19 patients; 26%), with a combination of the 2 or more antifungal agents being used in 11 patients (15%). Forty patients (55%) showed signs of clinical improvement with treatment, while 33 (45%) patients did not experience any improvement or continued to deteriorate, and 23 (32%) died in the course of their reported follow-up. CONCLUSIONS: The present cases illustrate well the challenge in the diagnosis and treatment of OAS due to invasive sinus aspergillosis. Our review and analysis of 73 cases support the notion that a high index of suspicion leading to early biopsy with histology and fungal culture is paramount for diagnosis. Early empiric antifungal treatment and debridement can potentially reduce morbidity and mortality.

Surgical Management of Complex Syndromic Craniosynostosis: Experience With a Rare Genetic Variant.

Patients with syndromic craniosynostosis (CS) can present with both intracranial and extracranial manifestations. Extracranial features include proptosis, exorbitism, and midface hypoplasia. Intracranial manifestations can include elevated intracranial pressure (ICP), brainstem compression, foramen magnum stenosis or jugular foramen hypoplasia with resultant venous hypertension and anomalous drainage. While fronto-orbital advancement, cranial vault remodeling, and posterior fossa decompression are standard surgical approaches to normalizing orbito-cranial volume and morphology, associated hydrocephalus, anomalous venous drainage, and tonsillar herniation often affect the timing, safety, and selection of corrective interventions. The surgical decision-making to circumvent venous emissaries, effectively time treatment of hydrocephalus, and address posterior versus anterior pathology primarily has not been widely described in the literature, and is important in the development of guidelines in these complex cases. In this report, we describe the surgical management of a patient with Jackson-Weiss syndrome presenting with delayed, but rapidly progressive bilateral lambdoid CS, severe proptosis, midface hypoplasia, elevated ICP, hydrocephalus, tonsillar ectopia, and severe venous hypertension with anomalous drainage. We review the literature related to management of complex synostosis and present our surgical decision-making in the setting of complex syndromic synostosis to aid in the formation of guidelines toward approaching these cases.

Impact of a Multidisciplinary Craniofacial Clinic for Patients With Craniofacial Syndromes on Patient Satisfaction and Outcome.

OBJECTIVE: Multidisciplinary clinics are becoming widely utilized. Given the number of patients with craniofacial syndromes evaluated at our institution, and the burden of assessment by multiple subspecialists, we created an American Cleft Palate-Craniofacial Association-certified Craniofacial Multidisciplinary Clinic (CMC) composed of a nurse practitioner, neurosurgeon, plastic surgeon, otolaryngologist, oromaxillofacial surgeon, geneticist, pulmonologist, occupational therapist, dentist, and child life specialist to improve patient experience, lessen the burden of assessment, decrease time to surgery, and improve patients' understanding of the diagnosis and treatment plan specifically for patients with complex craniofacial syndromes. We reviewed the impact of this clinic after 1 year of implementation. DESIGN: Retrospective review was performed to identify patients with craniofacial syndromic diagnoses seen by the neurosurgery department before and after implementation of the CMC from February 2017 to present. SETTING: The CMC is an outpatient clinic based in a tertiary care academic institution. PATIENTS: Chart review was performed to identify demographic, diagnostic, clinical, and treatment data. We assessed clinic experience, and the impact on quality of clinical and surgical care was assessed via survey. We compared this cohort to patients with similar craniofacial syndromes treated prior to the CMC. Thirty patients seen at the CMC were identified, and data from a comparable cohort of 30 patients seen prior to the clinic's inception was reviewed. RESULTS: Our CMC survey response rate was 67% (n = 20/30) for the CMC patients. Second opinions sought by parents prior to CMC was higher (mean = 0.85, range: 0-3) than for patients seen at the CMC (mean = 0.16, range: 0-1). Mean time to surgery before the CMC was 10.1 months (range: 1-15) compared to 4 months (range: 3-5) after implementation. Parents agreed that they felt well-informed about their diagnosis (n = 18/20, 90%), and that the presence of a plastic surgeon (19/20, 95%) and a nurse practitioner (17/20, 85%) were valuable in coordination of their care. Following surgery, 76% (n = 13/17) of patients who received surgery were happy with the outcome, 76% (n = 13/17) were happy with the appearance of the scar, and 95% (n = 19/20) would recommend the CMC to others. CONCLUSION: Multidisciplinary evaluation of patients with complex craniofacial conditions provides comprehensive, efficient, and effective care, as well as improved parent satisfaction and knowledge base.

Ultrafast Cortical Gain Adaptation in the Human Brain by Trial-To-Trial Changes of Associative Strength in Fear Learning.

In fear conditioning, more efficient sensory processing of a stimulus (the conditioned stimulus, CS) that has acquired motivational relevance by being paired with an aversive event (the unconditioned stimulus, US) has been associated with increased cortical gain in early sensory brain areas (Miskovic and Keil, 2012). Further, this sensory gain modulation related to short-term plasticity changes occurs independently of aware cognitive anticipation of the aversive US, pointing toward implicit learning mechanisms (Moratti and Keil, 2009). However, it is unknown how quickly the implicit learning of CS-US associations results in the adaptation of cortical gain. Here, using steady-state visually evoked fields derived from human Magnetoencephalography (MEG) recordings in two experiments (N = 33, 17 females and 16 males), we show that stimulus-driven neuromagnetic oscillatory activity increases and decreases quickly as a function of associative strength within three or four trials, as predicted by a computationally implemented Rescorla-Wagner model with the highest learning rate. These ultrafast cortical gain adaptations are restricted to early visual cortex using a delay fear conditioning procedure. Short interval (500 ms) trace conditioning resulted in the same ultrafast activity modulations by associative strength, but in a complex occipito-parieto-temporo-frontal network. Granger causal analysis revealed that reverberating top-down and bottom-up influences between anterior and posterior brain regions during trace conditioning characterized this network. Critically, in both delay and trace conditioning, ultrafast cortical gain modulations as a function of associative strength occurred independently of conscious US anticipation.SIGNIFICANCE STATEMENT In ever-changing environments, learned associations between a cue and an aversive consequence must change under new stimulus-consequence contingencies to be adaptive. What predicts potential dangers now might be meaningless in the next situation. Predictive cues are prioritized, as reflected by increased sensory cortex activity for these cues. However, this modulation also must adapt to altered stimulus-consequence contingencies. Here, we show that human visual cortex activity can be modulated quickly according to ultrafast contingency changes within a few learning trials. This finding extends to frontal brain regions when the cue and the aversive event are separated in time. Critically, this ultrafast updating process occurs orthogonally to aware aversive outcome anticipation and therefore relies on unconscious implicit learning mechanisms.

Rapidly Progressive Multisutural Craniosynostosis in a Patient With Jackson-Weiss Syndrome and a De Novo FGFR2 Pathogenic Variant.

Little is currently known about the mechanisms by which pathogenic variants of FGFR2 produce changes in the FGFR protein and influence the clinical presentation of affected individuals. We report on a patient with a de novo pathogenic variant of FGFR2 and a phenotype consistent with Jackson-Weiss syndrome who presented with delayed, rapidly progressive multisutural craniosynostosis and associated medical complications. Using 3-dimensional modeling of the FGFR protein, we provide evidence that this variant resulted in abnormal dimerization and constitutive activation of FGFR, leading to the Jackson-Weiss phenotype. Knowledge regarding the correlation between genotype and phenotype of persons with FGFR2-related craniosynostosis has the potential to allow for anticipation of medical complications, institution of early treatment, and improved clinical outcomes.

Comparison of Structural and Functional Features in Primary Angle Closure and Open Angle Glaucomas.

PRCIS: Using a large data set, we showed structural and functional differences between primary angle closure glaucoma (PACG) and primary open angle glaucoma (POAG). Primary angle closure glaucoma has relative structural preservation and worse functional loss inferiorly. PURPOSE: To identify structural and functional differences in PACG and POAG. MATERIALS AND METHODS: In this large cross-sectional study, differences in structural and functional damage were assessed among patients with POAG and PACG with optical coherence tomography and reliable visual field testing. RESULTS: In all, 283 patients with PACG and 4110 patients with POAG were included. Despite similar mean deviation on visual fields (mean [SD] -7.73 [7.92] vs. -7.53 [6.90] dB, P =0.72), patients with PACG had thicker global retinal nerve fiber layer (RNFL), smaller cup volume, smaller cup-to-disc ratio, and larger rim area than POAG (77 [20] vs. 71 [14] µm, 0.32 [0.28] vs. 0.40 [0.29] mm 3 , 0.6 [0.2] vs. 0.7 [0.1], 1.07 [0.40] vs. 0.89 [0.30] mm 2 , P <0.001 for all), while patients with POAG had more pronounced inferior RNFL thinning (82 [24] vs. 95 [35] µm, P <0.001). In a multivariable analysis, hyperopia [odds ratio (OR): 1.24, confidence interval (CI): 1.13-1.37], smaller cup-to-disc ratio (OR: 0.69, CI: 0.61-0.78), thicker inferior RNFL (OR: 1.15, CI: 1.06-1.26) and worse mean deviation (OR: 0.95, CI: 0.92-0.98) were associated with PACG. Functionally, POAG was associated with superior paracentral loss and PACG with inferior field loss. After adjusting for average RNFL thickness, PACG was associated with more diffuse loss than POAG (total deviation differences 1.26-3.2 dB). CONCLUSIONS: Patients with PACG had less structural damage than patients with POAG despite similar degrees of functional loss. Regional differences in patterns of functional and structural loss between POAG and PACG may improve disease monitoring for these glaucoma subtypes.

Multimodal Imaging Characteristics and Correlation to Outcomes in Patients with CRAO Presenting to a Large Academic Center.

PURPOSE: To characterize a large modern cohort of patients with central retinal artery occlusion (CRAO) by describing presenting features and outcomes relating to manually segmented optical coherence tomography (OCT) features, angiographic reperfusion, and visual recovery. DESIGN: Retrospective clinical cohort study. METHODS: Patients with CRAO (ICD-10: H34.1) initially presenting to a tertiary referral center between January 2017 and December 2021 were included. Demographics, eye exam findings, fundus photographs, OCT, and fluorescein angiography (FA) were analyzed. Main outcome measures included total and inner retinal thickness on macular OCT, reperfusion, visual outcomes, and development of neovascularization. RESULTS: 145 eyes of 144 patients with mean age at of 69.4±13.6 years were included. The mean time to presentation was 1.6±4.2 days, with 19% examined within 4.5 hours and 26% within 6 hours of vision loss. 19% had cilioretinal artery (CLRA) sparing. Mean initial visual acuity (VA) was 1.68±1.10 LogMAR (CLRA sparing) compared to 2.53±0.58 LogMAR (non-CLRA sparing), P<0.001. 32% had elevated inflammatory makers. Out of 47 eyes with final fluorescein angiography, one-third showed some reperfusion. Final vision was 1.40±1.16 LogMAR (CLRA sparing) compared to 2.46±0.81 (non-CLRA sparing), P<0.001. A third of patients improved in VA in both groups, 27% of patients gained more than 2 lines of vision in the CLRA sparing group and 36% in the non-CLRA sparing group. 17% improved to better than 20/200 in CLRA-sparing and 4% in non-CLRA sparing. 11% developed neovascularization all in non-CLRA sparing. In a multiple linear regression, VA at presentation was associated with regaining vision of 2 lines or more (OR=2.603, P=0.007). OCT showed progressive thinning over time, reaching lowest measurements at 6 months, and stabilizing thereafter. CONCLUSIONS: In this modern cohort of acute CRAO patients, presentation to a tertiary facility within 12 hours of symptoms was seen in almost half of the patients. Final visual acuity improved in almost a third of the patients, however, vision better than the legal blindness limit was rare (∼5%). Interestingly, a third of patients had some mild elevation of systemic inflammatory markers. Better visual acuity at presentation was associated with visual gain, while baseline OCT values had poor correlation with final outcome.

Occlusive Retinal Vasculitis in a Pediatric Patient With Kikuchi-Fujimoto Disease and Sickle Cell Trait.

A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.].

Brain MRI changes in degenerative cervical myelopathy: a systematic review.

BACKGROUND: Degenerative cervical myelopathy (DCM) is the most common cause of adult spinal cord dysfunction globally. Associated neurological symptoms and signs have historically been explained by pathobiology within the cervical spine. However, recent advances in imaging have shed light on numerous brain changes in patients with DCM, and it is hypothesised that these changes contribute to DCM pathogenesis. The aetiology, significance, and distribution of these supraspinal changes is currently unknown. The objective was therefore to synthesise all current evidence on brain changes in DCM. METHODS: A systematic review was performed. Cross-sectional and longitudinal studies with magnetic resonance imaging on a cohort of patients with DCM were eligible. PRISMA guidelines were followed. MEDLINE and Embase were searched to 28th August 2023. Duplicate title/abstract screening, data extraction and risk of bias assessments were conducted. A qualitative synthesis of the literature is presented as per the Synthesis Without Meta-Analysis (SWiM) reporting guideline. The review was registered with PROSPERO (ID: CRD42022298538). FINDINGS: Of the 2014 studies that were screened, 47 studies were identified that used MRI to investigate brain changes in DCM. In total, 1500 patients with DCM were included in the synthesis, with a mean age of 53 years. Brain alterations on MRI were associated with DCM both before and after surgery, particularly within the sensorimotor network, visual network, default mode network, thalamus and cerebellum. Associations were commonly reported between brain MRI alterations and clinical measures, particularly the Japanese orthopaedic association (JOA) score. Risk of bias of included studies was low to moderate. INTERPRETATION: The rapidly expanding literature provides mounting evidence for brain changes in DCM. We have identified key structures and pathways that are altered, although there remains uncertainty regarding the directionality and clinical significance of these changes. Future studies with greater sample sizes, more detailed phenotyping and longer follow-up are now needed. FUNDING: ODM is supported by an Academic Clinical Fellowship at the University of Cambridge. BMD is supported by an NIHR Clinical Doctoral Fellowship at the University of Cambridge (NIHR300696). VFJN is supported by an NIHR Rosetrees Trust Advanced Fellowship (NIHR302544). This project was supported by an award from the Rosetrees Foundation with the Storygate Trust (A2844).

Progressive Choriocapillaris Changes on Optical Coherence Tomography Angiography Correlate With Stage Progression in AMD.

Purpose: We investigated the association between inner choroid flow deficit percentage (IC-FD%) using swept-source optical coherence tomography angiography (SS-OCTA) and progression of AMD. Methods: Retrospective, observational study including 64 eyes (42 participants) with early or intermediate AMD at baseline. Participants had two or more consecutive swept-source optical coherence tomography angiography covering a period of at least 18 months. Demographics, visual acuity, and AMD staging based on Beckman classification were reviewed. OCT was analyzed for hyperreflective foci, subretinal drusenoid deposits, hyporeflective drusen cores, and subfoveal choroidal thickness. IC-FD% was measured within the central 3- and 6-mm using a 16-µm slab, after compensation and binarization (Phansalkar method). Mixed-effects Cox regression models assessed the association between imaging biomarkers and AMD progression. Results: During follow-up (37 ± 9 months), 4 eyes with early AMD (31%) progressed to intermediate AMD and 30 (59%) eyes with intermediate AMD developed late AMD (19 geographic atrophy; 11 wet AMD). Baseline hyporeflective drusen core was associated with geographic atrophy development (P < 0.01), whereas greater IC-FD% (3-mm) was associated with wet AMD (P = 0.03). Time-varying analysis showed that faster subfoveal choroidal thickness reduction and IC-FD% (6-mm) increase were associated with geographic atrophy onset (P < 0.05), whereas IC-FD% (3-mm) increase was associated with wet AMD (P = 0.03). Notably, greater IC-FD% increases in the 3 mm (area under the curve = 0.72) and 6 mm (area under the curve = 0.89) were better predictive of wet AMD and geographic atrophy development, respectively. Conclusions: Our longitudinal IC-FD% assessment emphasizes the role of progressive choriocapillaris changes as a biomarker for AMD progression. Our findings support that widespread choriocapillaris alterations (6 mm) may precede progression to geographic atrophy, whereas more central choriocapillaris loss (3 mm) may provide an ischemic stimulus for wet AMD.

Decreased Macular Choriocapillaris Perfusion Correlates with Contrast Sensitivity Function in Dry Age-Related Macular Degeneration.

PURPOSE: To investigate the relationships between contrast sensitivity (CS), choriocapillaris perfusion, and other structural OCT biomarkers in dry age-related macular degeneration (AMD). DESIGN: Cross-sectional, observational study. PARTICIPANTS: One hundred AMD eyes (22 early, 52 intermediate, and 26 late) from 74 patients and 45 control eyes from 37 age-similar subjects. METHODS: All participants had visual acuity (VA) assessment, quantitative CS function (qCSF) testing, macular OCT, and 6 × 6-mm swept-source OCT angiography scans on the same day. OCT volumes were analyzed for subretinal drusenoid deposits and hyporeflective drusen cores, and to measure thickness of the outer nuclear layer. OCT angiography scans were utilized to calculate drusen volume and inner choroid flow deficit percentage (IC-FD%), and to measure the area of choroidal hypertransmission defects (HTDs). Inner choroid flow deficit percentage was measured from a 16-µm thick choriocapillaris slab after compensation and binarization with Phansalkar's method. Generalized linear mixed-effects models were used to evaluate the associations between functional and structural variables. MAIN OUTCOME MEASURES: To explore the associations between qCSF-measured CS, IC-FD%, and various AMD imaging biomarkers. RESULTS: Age-related macular degeneration exhibited significantly reduced qCSF metrics eyes across all stages compared with controls. Univariate analysis revealed significant associations between various imaging biomarkers, reduced qCSF metrics, and VA in both groups. Multivariate analysis confirmed that higher IC-FD% in the central 5 mm was significantly associated with decreases in all qCSF metrics in AMD eyes (ß = -0.74 to -0.25, all P < 0.05), but not with VA (P > 0.05). Outer nuclear layer thickness in the central 3 mm correlated with both VA (ß = 2.85, P < 0.001) and several qCSF metrics (ß = 0.01-0.90, all P < 0.05), especially in AMD eyes. Further, larger HTD areas were associated with decreased VA (ß = -0.89, P < 0.001) and reduced CS at low-intermediate frequencies across AMD stages (ß = -0.30 to -0.29, P < 0.001). CONCLUSIONS: The significant association between IC-FD% in the central 5 mm and qCSF-measured CS reinforces the hypothesis that decreased macular choriocapillaris perfusion contributes to visual function changes in AMD, which are more pronounced in CS than in VA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

The Use of Additional Facedown Positioning with Silicone Oil Tamponade for the Treatment of Retinal Re-detachment.

PURPOSE: To highlight a potential alternative to additional surgery for management of retinal re-detachment through the use of additional facedown positioning with silicone oil tamponade. METHODS: Retrospective case-series of two patients evaluated with examination, multimodal imaging, including fundus photography, optical coherence tomography (OCT), and fluorescein angiography. RESULTS: In case 1, a 70-year-old female patient underwent surgery for a full-thickness macular hole with associated macula-off retinal detachment, but experienced a recurrent detachment and underwent a second surgery with silicone oil placement. Another recurrent detachment was found. The case was managed conservatively with face-down positioning, resulting in resolution of subretinal fluid and improvement in vision. At follow-up, the retina remained attached with stable vision. In case 2, a 25-year-old male patient underwent a surgical repair for PVR retinal detachment with a scleral buckle, cryotherapy, and external drainage. After multiple re-detachment surgeries with retinectomy and oil placement there was another tractional re-detachment of the fovea was noted. Management was with facedown positioning and follow-up evaluation showed resolution of the subretinal fluid and improvement in vision with stability for greater than 2 months. CONCLUSIONS: For recurrent retinal re-detachments with silicone oil in place, an additional week of facedown positioning can result in anatomic success and be a viable alternative or bridge to invasive surgical interventions. This approach may have greatest utility for patients who are poor surgical candidates without new peripheral pathology.

The Transition to Ophthalmology Residency: A National Survey of the Combined Ophthalmology PGY-1 Program.

Background In 2017, the Accreditation Council for Graduate Medical Education announced all ophthalmology residency programs would provide a combined transitional or joint preliminary program for first postgraduate year (PGY-1) residents, with mandatory implementation by 2023. Purpose This study aimed to survey ophthalmology residency program directors, postgraduate year 2 (PGY-2) ophthalmology residents who were a part of the first, official combined ophthalmology PGY-1 year, and postgraduate year 3 (PGY-3) residents who were a PGY-1 resident the year prior to integration to evaluate characteristics and perspectives on the combined ophthalmology PGY-1 year. Methods A national, internet survey-based study approved by the Association of University Professors of Ophthalmology (AUPO) was disseminated to the AUPO listserv of program directors (PDs) and PGY-2 and PGY-3 ophthalmology residents from July to August 2022 and then again April to June 2023. Results Twenty-six PDs completed the survey (response rate 20.3% out of 128 PDs). Forty-one PGY-2 ophthalmology residents who underwent the combined ophthalmology PGY-1 year and 33 PGY-3 ophthalmology residents also completed the survey. Most PGY-1 curricula focused on exposure to comprehensive ophthalmology and provided indirect ophthalmoscope, slit lamp, and refraction skills training to residents. Early exposure to fundamentals and clinical workflows were commonly cited benefits to the integration. When PDs were surveyed about how well-prepared PGY-1 residents who went through the combined year are for the PGY-2 relative to the prior year's class, 16 (61.5%) responded "better prepared." PGY-2 residents also reported a relatively higher level of clinical preparedness and familiarity with ophthalmology co-residents than PGY-3 residents. Several areas of improvement cited by both PDs and residents were identified including a dedicated didactic curriculum and more time in ophthalmology during the PGY-1 year. Conclusions We found an overall net benefit from the integration of the combined ophthalmology PGY-1 year. Benefits include early exposure to clinical skills and knowledge specific to ophthalmology, leading to increased confidence and preparedness for the rigorous transition to ophthalmology residency. We also identified many areas for improvement to optimize the PGY-1 year including a formal curriculum and additional time in ophthalmology. Programs should work closely with their residents, faculty, and non-ophthalmology PDs to refine the PGY-1 for the benefit of future ophthalmologists.

Defining Occult High-Risk Cysts of the Pineal Region: A Case Series.

BACKGROUND: Absence of hydrocephalus on neuroimaging may impart a false sense of security for patients with pineal cysts. In this case series, we characterize a subset of patients with pineal cysts having an occult presentation. Unifying features of worsening paroxysmal headaches suggesting intermittent obstructive hydrocephalus and radiographic evidence of third ventricular invagination characterize these patients as high risk. OBJECTIVE: To define features of occult, high-risk pineal cysts and outcomes of endoscopic cyst fenestration. METHODS: Charts were retrospectively reviewed for patients with pineal cysts evaluated at our institution between 2018 and 2021 who underwent endoscopic cyst fenestration. To capture cysts presenting as occult, patients were excluded if hydrocephalus was noted at presentation. Relevant clinical history, imaging, operative data, and clinical outcomes were reviewed. RESULTS: Of 50 pineal cyst patients, 4 satisfied inclusion criteria. All patients presented with worsening paroxysmal headaches. In addition, 75% (3/4) also experienced intermittent syncope. Patients exhibited no hydrocephalus (n = 3) or fluctuating ventricular size on longitudinal imaging (n = 1). In all cases, high-resolution sagittal 3-dimensional T2 magnetic resonance imaging demonstrated invagination of the cyst anteriorly into the posterior third ventricle. All patients underwent endoscopic cyst fenestration with complete symptom resolution (mean follow-up of 20.6 months; range 3.5-37.4 months). CONCLUSION: The clinical history for occult, high-risk pineal cysts is notable for worsening paroxysmal headaches and episodic alterations of consciousness suggesting intermittent obstructive hydrocephalus. Because ventricular size can appear normal on standard imaging protocols, clinical suspicion should trigger workup with high-resolution magnetic resonance imaging designed to detect these cysts. Endoscopic cyst fenestration is a safe and efficacious management strategy.

Adverse events associated with femtosecond laser-assisted cataract surgery reported to the MAUDE database.

PURPOSE: To report real-world complications associated with femtosecond laser-assisted cataract surgery (FLACS). SETTING: Deidentified database. DESIGN: Retrospective review. METHODS: The U.S. Food and Drug Administration's Manufacturer and User Facility Device Experience (MAUDE) database was culled for adverse events involving ophthalmic femtosecond laser during cataract surgery from January 2010 to January 2020. Reports in which the narrative description was inadequate to determine the nature of the complication were excluded. Each report was reviewed for complications, which were subsequently assigned to a complication category as determined by these authors. The number of events per year was determined and compared. RESULTS: Between January 2010 and January 2020, the MAUDE database search yielded 2927 reports involving femtosecond laser, of which 1927 met inclusion criteria and were included for analysis. These reports revealed 2704 complications: 1115 capsule tears (41.2%), 329 of which required vitrectomy, 372 instances of suction loss, 321 other capsulotomy-related complications, and 432 corneal or incisional complications. Rare complications included 54 reports of miosis, 16 reports of endophthalmitis, and 4 reports of toxic anterior segment syndrome. The number of events in the MAUDE database increased year over year between 2011 and 2017, peaking in 2017 at 303 events. CONCLUSIONS: By drawing on real-world data in the MAUDE database, this study identified the complications of greatest importance in clinical practice. Surgeons should be aware of the spectrum of possible complications to best prepare for all potential outcomes and to effectively counsel patients.

Effective Low-Cost Ophthalmological Screening With a Novel iPhone Fundus Camera at Community Centers.

Ophthalmologic care is inaccessible to many people due to a variety of factors, including the availability of providers, cost of equipment for ophthalmologic care, and transportation to clinics and appointments. Because many causes of blindness are both highly prevalent and preventable once identified, it is essential to address gaps in care for underserved populations. We developed a novel 3D-printed mobile retinal camera. In this study, we organized recurring student-run screening events around New York City that took place in community centers and churches, at which we utilized our device to take retinal images. Our screening events reached a diverse population of New Yorkers, disproportionately those with lower household income, many of whom had not had recent eye exams. To validate the device for use in telehealth ophthalmologic visits, we transmitted the images to a remote ophthalmologist for evaluation and compared the result with an on-site attending physician's dilated eye exam. The subjective assessment indicated that 97% of images captured with the mobile retinal camera were acceptable for telehealth analysis. Remote image assessment by achieved 92% sensitivity and 83% specificity in detecting optic disc cupping, compared to the gold-standard on-site dilated eye exam. In addition, the device was portable, affordable, and able to be used by those with relatively little ophthalmologic training. We have demonstrated the utility of this affordable mobile retinal camera for telehealth ophthalmologic evaluation during community screening events that reached an underserved population to detect disease and connect with long-term care.

The 100 Most Mentioned Glaucoma Articles Online With Highest Altmetric Attention Scores.

PRCIS: Characteristics of the most mentioned glaucoma articles on the internet were analyzed, allowing a better understanding of the dissemination of glaucoma research to the general public. PURPOSE: The aim was to determine the 100 most mentioned articles on the internet in the field of glaucoma and analyze their characteristics. MATERIALS AND METHODS: We identified the top 100 glaucoma articles with the highest Altmetric Attention Score (AAS), an automatically calculated metric for monitoring social media. Each article was evaluated for several characteristics including year of publication, title, journal name, journal impact factor (IF), article topic, article type, affiliation, and online mentions (news, blog, policy, Twitter, Facebook, etc.). Correlation analysis was conducted for AAS with these characteristics. RESULTS: The selected 100 articles came from 44 journals with more than half (56%) published in ophthalmology-specific journals. There was no significant correlation between IF and number of articles in a specific journal or AAS (P>0.1), but the number of articles in the top 100 was higher for ophthalmology journals with a higher IF (P<0.05). Original study was the most common study type (87%), of which clinical observation study was the most common subgroup (40%). Epidemiology/risk factor and basic science were the most common article topics (each 24%), followed by medical treatment (13%). Article topics regarding medical treatment had a significantly greater AAS than other topics (P<0.05). Of the top 5 articles, more than half (60%) were related to "Lifestyle choice" topics. CONCLUSIONS: There was no association between journal IF and AAS, consistent with previous studies. 90% of journals that had articles in the top 100 had a Twitter page. "Lifestyle choice" activities and other modifiable risk factors attracted significant online attention regarding glaucoma studies, with two of the top three most mentioned articles related to dietary intake. The present study thus provides a better understanding of online engagement with glaucoma research and the dissemination of this research to the general public.