Recent Submissions

  • Zebrafish optokinetic reflex: minimal reporting guidelines and recommendations

    Rodwell, Vanessa; Thomas, Mervyn (2023-12-20)
    Optokinetic reflex (OKR) assays in zebrafish models are a valuable tool for studying a diverse range of ophthalmological and neurological conditions. Despite its increasing popularity in recent years, there are no clear reporting guidelines for the assay. Following reporting guidelines in research enhances reproducibility, reduces bias, and mitigates underreporting and poor methodologies in published works. To better understand optimal reporting standards for an OKR assay in zebrafish, we performed a systematic literature review exploring the animal, environmental, and technical factors that should be considered. Using search criteria from three online databases, a total of 109 research papers were selected for review. Multiple crucial factors were identified, including larval characteristics, sample size, fixing method, OKR set-up, distance of stimulus, detailed stimulus parameters, eye recording, and eye movement analysis. The outcome of the literature analysis highlighted the insufficient information provided in past research papers and the lack of a systematic way to present the parameters related to each of the experimental factors. To circumvent any future errors and champion robust transparent research, we have created the zebrafish optokinetic (ZOK) reflex minimal reporting guideline.
  • Visual field deficits in albinism in comparison to idiopathic infantile nystagmus

    McLean, Rebecca; Sheth, Viral (2024-02-01)
    Purpose: This is the first systematic comparison of visual field (VF) deficits in people with albinism (PwA) and idiopathic infantile nystagmus (PwIIN) using static perimetry. We also compare best-corrected visual acuity (BCVA) and optical coherence tomography measures of the fovea, parafovea, and circumpapillary retinal nerve fiber layer in PwA. Methods: VF testing was performed on 62 PwA and 36 PwIIN using a Humphrey Field Analyzer (SITA FAST 24-2). Mean detection thresholds for each eye were calculated, along with quadrants and central measures. Retinal layers were manually segmented in the macular region. Results: Mean detection thresholds were significantly lower than normative values for PwA (-3.10 ± 1.67 dB, P << 0.0001) and PwIIN (-1.70 ± 1.54 dB, P < 0.0001). Mean detection thresholds were significantly lower in PwA compared to PwIIN (P < 0.0001) and significantly worse for left compared to right eyes in PwA (P = 0.0002) but not in PwIIN (P = 0.37). In PwA, the superior nasal VF was significantly worse than other quadrants (P < 0.05). PwIIN appeared to show a mild relative arcuate scotoma. In PwA, central detection thresholds were correlated with foveal changes in the inner and outer retina. VF was strongly correlated to BCVA in both groups. Conclusions: Clear peripheral and central VF deficits exist in PwA and PwIIN, and static VF results need to be interpreted with caution clinically. Since PwA exhibit considerably lower detection thresholds compared to PwIIN, VF defects are unlikely to be due to nystagmus in PwA. In addition to horizontal VF asymmetry, PwA exhibit both vertical and interocular asymmetries, which needs further exploration.
  • Phenotypic features determining visual acuity in albinism and the role of amblyogenic factors

    McLean, Rebecca; Sheth, Viral (2024-02-01)
    Albinism is a spectrum disorder causing foveal hypoplasia, nystagmus, and hypopigmentation of the iris and fundus along with other visual deficits, which can all impact vision. Albinism is also associated with amblyogenic factors which could affect monocular visual acuity. The foveal appearance in albinism can range from mild foveal hypoplasia to that which is indistinguishable from the peripheral retina. The appearance can be quickly and easily graded using the Leicester Grading System in the clinic. However, interquartile ranges of 0.3 logMAR for the grades associated with albinism limit the accuracy of the grading system in predicting vision. Here, we discuss the potential role of nystagmus presenting evidence that it may not be a major source of variability in the prediction of visual acuity. We also show that interocular differences in visual acuity are low in albinism despite high levels of amblyogenic factors indicating that active suppression of vision in one eye in albinism is uncommon.
  • Advances in the treatment of keratoconus: Epithelial-On (EPI-On) Corneal-Collagen Cross-Linking (CXL) and CXL-Plus Procedures

    Ageed, Ahmed (2024-01-03)
    Keratoconus (KC) incidence is on the increase. The advent of corneal-collagen cross-linking (CXL) has revolutionized the management of KC. This systematic review looks at the efficacy and complications of two novel treatments within CXL: Epithelial-On (Epi-On) and CXL-plus procedures. Two separate literature searches were carried out up until July 1, 2021. Articles only published in the last two years were included to ensure that only the most recent articles were reviewed. A total of 15 articles were selected for this review. There were varied results regarding the efficacy of Epi-On. No significant difference was found between Epi-On and standard Epithelial-Off (Epi-Off) CXL. However, it was found that Epi-On was inferior to standard CXL in terms of reducing KMAX. There was a higher risk of progression in patients treated with Epi-On CXL, with an increased rate of patients requiring re-treatment due to the advancement of their KC. While some studies report CXL-plus procedures demonstrate long-term efficacy and safety, a considerable number of studies advise caution, reporting a significant deterioration in corrected distance visual acuity (CDVA). Consequently, a question persists regarding the safest and most efficacious approach, given the lack of robust large randomized controlled trials (RCTs) in the current literature.
  • Achromatopsia-visual cortex stability and plasticity in the absence of functional cones

    McLean, Rebecca (2023-10-03)
    Purpose: Achromatopsia is a rare inherited disorder rendering retinal cone photoreceptors nonfunctional. As a consequence, the sizable foveal representation in the visual cortex is congenitally deprived of visual input, which prompts a fundamental question: is the cortical representation of the central visual field in patients with achromatopsia remapped to take up processing of paracentral inputs? Such remapping might interfere with gene therapeutic treatments aimed at restoring cone function. Methods: We conducted a multicenter study to explore the nature and plasticity of vision in the absence of functional cones in a cohort of 17 individuals affected by autosomal recessive achromatopsia and confirmed biallelic disease-causing CNGA3 or CNGB3 mutations. Specifically, we tested the hypothesis of foveal remapping in human achromatopsia. For this purpose, we applied two independent functional magnetic resonance imaging (fMRI)-based mapping approaches, i.e. conventional phase-encoded eccentricity and population receptive field mapping, to separate data sets. Results: Both fMRI approaches produced the same result in the group comparison of achromatopsia versus healthy controls: sizable remapping of the representation of the central visual field in the primary visual cortex was not apparent. Conclusions: Remapping of the cortical representation of the central visual field is not a general feature in achromatopsia. It is concluded that plasticity of the human primary visual cortex is less pronounced than previously assumed. A pretherapeutic imaging workup is proposed to optimize interventions.
  • Use of Hand-Held Optical Coherence Tomography during Retinopathy of Prematurity (ROP) Screening demonstrates an increased Outer Retina from early Postmenstrual Age in Preterm Infants with ROP

    Anwar, Samira; Patel, Aarti; Tyradellis, Straton (2023-10-23)
    Purpose: To identify structural markers of active retinopathy of prematurity (ROP) in foveal and parafoveal retinal layers using hand-held optical coherence tomography (HH-OCT). Methods: We acquired HH-OCT images (n=278) from a prospective mixed cross-sectional longitudinal observational study of 87 participants (23-36 weeks gestational age (GA); n=30 with ROP, n=57 without ROP) between 31 to 44 weeks postmenstrual age (PMA) excluding treated ROP and features of cystoid macular edema (CME). Six retinal layer thicknesses from the fovea to the parafovea were analysed at five locations up to 1000 µm temporally and nasally. Results: The mean outer retinal thickness (OUTRETL) during active ROP increased at the fovea and parafovea from PMA 33 to 39 weeks ( p <0.001) while the parafoveal inner nuclear layer (INL) and retinal nerve fiber layer (RNFL) reduced ( p <0.001). OUTRETL at the fovea from 33 to 39 weeks PMA was consistently thicker in infants with ROP across all levels of prematurity (GA). Conclusions: Increased foveal and parafoveal outer retina measured using HH-OCT shows potential as a marker for ROP screening.
  • Treatment of age-related macular degeneration with aflibercept using a treat, extend and fixed protocol; A 4-year study of treatment outcomes, durability, safety and quality of life (An extension to the MATE randomised controlled trial)

    Empeslidis, Theo (30/09/2023)
    Purpose: Data are limited pertaining to the long-term benefits of aflibercept treatment for neovascular age-related macular degeneration (nAMD). The aim of this study was to provide outcomes, safety, durability and quality-of-life data with aflibercept using a modified treat, extend and fixed regime over 4 years. Methods: Prospective, multicentre, single cohort observational study of treatment-naÃve nAMD participants treated with aflibercept as 2-year extension of the MATE-trial that compared early and late Treat-and-Extend for 2 years. Refracted ETDRS best corrected visual acuity (BCVA), central retinal thickness (CRT), treatment interval and adverse events were assessed. Quality-of-life was measured using the Macular Disease Dependent Quality of Life (MacDQoL) and Macular Disease Treatment Satisfaction Questionnaires (MacTSQ). Results: Twenty-six of 40 participants completing the MATE-trial were enrolled with 20 completing the total 4-year study. Mean BCVA was 60.7 at Month 0 and 64.8 ETDRS letters at Month 48 while CRT decreased from 423.7 ?m to 292.2 ?m. Five participants discontinued treatment due to inactivity. The mean number of treatments and visits for the remaining participants was 27 and 30.0, respectively, with treatment intervals extended to 12 weeks in four participants at Month 48. Both AMD-specific QoL and treatment satisfaction remained stable between Months 0 and 48 and mean BCVA significantly correlated with AMD-specific QoL scores at Months 12, 24 and 48. Conclusions: Results suggest that BCVA can be maintained over 48 months when following a treat-extend-and-fix regimen of aflibercept with intervals out to 12 weeks, while maintaining AMD-specific QoL and treatment satisfaction.
  • Incidence and management of early postoperative complications in lamellar corneal transplantation

    Romano, Davide (2023-04-27)
    Purpose: To provide a comprehensive review of the incidence, risk factors, and management of early complications after deep anterior lamellar keratoplasty (DALK), Descemet stripping automated keratoplasty (DSAEK), and Descemet membrane endothelial keratoplasty (DMEK). Methods: A literature review of complications, that can occur from the time of the transplant up to 1 month after the transplant procedure, was conducted. Case reports and case series were included in the review. Results: Complications in the earliest postoperative days following anterior and posterior lamellar keratoplasty have shown to affect graft survival. These complications include, but are not limited to, double anterior chamber, sclerokeratitis endothelial graft detachment, acute glaucoma, fluid misdirection syndrome, donor-transmitted and recurrent infection, and Uretts-Zavalia syndrome. Conclusion: It is essential for surgeons and clinicians to not only be aware of these complications but also know how to manage them to minimize their impact on long-term transplant survival and visual outcomes.
  • The MATE trial: a multicentre, mixed-methodology, pilot, randomised controlled trial in neovascular age-related macular degeneration

    Empeslidis, Theo (2023-04-20)
    Background/objectives: In healthcare research investigating complex interventions, gaps in understanding of processes can be filled by using qualitative methods alongside a quantitative approach. The aim of this mixed-methods pilot trial was to provide feasibility evidence comparing two treatment regimens for neovascular age-related macular degeneration (nAMD) to inform a future large-scale randomised controlled trial (RCT). Subjects/methods: Forty-four treatment-naïve nAMD patients were followed over 24 months and randomised to one of two treatment regimens: standard care (SC) or treat and extend (T&E). The primary objective evaluated feasibility of the MATE trial via evaluations of screening logs for recruitment rates, nonparticipation and screen fails, whilst qualitative in-depth interviews with key study staff evaluated the recruitment phase and running of the trial. The secondary objective assessed changes in visual acuity and central retinal thickness (CRT) between the two treatment arms. Results: The overall recruitment rate was 3.07 participants per month with a 40.8% non-participation rate, 18.51% screen-failure rate and 15% withdrawal/non-completion rate. Key themes in the recruitment phase included human factors, protocol-related issues, recruitment processes and challenges. Both treatment regimens showed a trend towards a visual acuity gain at month 12 which was not maintained at month 24, whilst CRT reduced similarly in both regimens over the same time period. These were achieved with one less treatment following a T&E regimen. Conclusion: This mixed-methodology, pilot RCT achieved its pre-defined recruitment, nonparticipation and screen failure rates, thus deeming it a success. With some minor protocol amendments, progression to a large-scale RCT will be achievable.
  • Lacrimal gland biopsies-results from a tertiary centre in the UK

    Awad, Mary; Burns, Joyce; De Silva, Ian; Sampath, Raghavan (2022-12-21)
    Purpose: To report the histopathological results of lacrimal gland biopsies over a 21-year period in a tertiary referral centre in the United Kingdom. To the best of our knowledge, this represents the largest series to be published in the United Kingdom. Methods: A retrospective observational review was carried out for patients who underwent lacrimal gland biopsies in a tertiary referral centre at the University Hospitals of Leicester, United Kingdom between the years of 2000 and 2021. Results: Lacrimal gland biopsies were performed on 248 patients during the specified 21-year period. They comprised 157 (63.3%) females and 91 (36.7%) males. The mean age at presentation was 50.8 years (range 15-94 years). The majority of patients were Caucasian (69.4%, n = 172) followed by Asians (25.0%, n = 62), African/Afro-Caribbean (4.8%, n = 12) and other ethnicities (0.8%, n = 2). The most common histopathological diagnosis was chronic inflammation dacryoadenitis (69.0%, n = 171) followed by lymphomas (15.3%, n = 38). Conclusion: Our study shows that chronic inflammation accounts for the majority of histopathological diagnosis followed by lymphoproliferative disorders.
  • Ocular-surface regeneration therapies for eye disorders: The state of the art

    Romano, Davide (2023-06-15)
    The ocular surface is a complex structure that includes cornea, conjunctiva, limbus, and tear film, and is critical for maintaining visual function. When the ocular-surface integrity is altered by a disease, conventional therapies usually rely on topical drops or tissue replacement with more invasive procedures, such as corneal transplants. However, in the last years, regeneration therapies have emerged as a promising approach to repair the damaged ocular surface by stimulating cell proliferation and restoring the eye homeostasis and function. This article reviews the different strategies employed in ocular-surface regeneration, including cell-based therapies, growth-factor-based therapies, and tissue-engineering approaches. Dry eye and neurotrophic keratopathy diseases can be treated with nerve-growth factors to stimulate the limbal stem-cell proliferation and the corneal nerve regeneration, whereas conjunctival autograft or amniotic membrane are used in subjects with corneal limbus dysfunction, such as limbal stem-cell deficiency or pterygium. Further, new therapies are available for patients with corneal endothelium diseases to promote the expansion and migration of cells without the need of corneal keratoplasty. Finally, gene therapy is a promising new frontier of regeneration medicine that can modify the gene expression and, potentially, restore the corneal transparency by reducing fibrosis and neovascularization, as well as by stimulating stem-cell proliferation and tissue regeneration.
  • Managing post-keratoplasty astigmatism: High-tech vs. low-tech imaging techniques for guiding suture manipulation

    Romano, Davide (2023-05-14)
    Astigmatism is a visually significant condition that can develop after keratoplasty. The management of post-keratoplasty astigmatism can be performed both when transplant sutures are in place and when they have been removed. Fundamental for astigmatism management is its identification and characterization in terms of type, amount, and direction. Commonly, post-keratoplasty astigmatism is evaluated through corneal tomography or topo-aberrometry; however, many other techniques can be used in case these instruments are not readily available. Here, we describe several low-tech and high-tech techniques used for post-keratoplasty astigmatism detection in order to quickly understand if it contributes to low vision quality and to determine its characteristics. The management of post-keratoplasty astigmatism through suture manipulation is also described.
  • Role of direct supervision in the learning curve of Descemet membrane endothelial keratoplasty surgery

    Romano, Davide (2023-04-25)
    Purpose: The aim of this study was to compare complication rates of Descemet membrane endothelial keratoplasty (DMEK) performed by directly supervised and nondirectly supervised corneal fellows. Methods: This study was a retrospective, comparative case series of DMEK surgeries performed by novice surgeons (less than 15 DMEK cases) with or without direct direct expert supervision. Patients who underwent surgery for Fuchs endothelial dystrophy or pseudophakic bullous keratopathy with a minimum follow-up of 12 weeks were included. Data on patients' demographics, surgical details, surgeon level, intraoperative and postoperative complications, and rate of rebubbling were collected. Results: In this study, 41 nondirectly supervised and 48 directly supervised DMEK surgeries were included. At 6 months, 67.4% of eyes achieved a best-corrected visual acuity of ≤0.3 logMAR with no significant difference between groups (P = 0.95). Intraoperative complications occurred in 22% of cases in the nondirect supervision group and 4.2% in the direct supervision group (P = 0.02). Postoperative complications occurred in 9.8% of cases in the nondirect supervision group and 6.2% of cases in the direct supervision group (P = 0.7). The rebubbling rate was comparable in the 2 groups (34.1% vs. 33.3%, P = 1.0). Five cases (12.2%), all from the nondirect supervision group, required secondary keratoplasty (P = 0.02). The overall complication rate was significantly higher in the nondirect supervision group (31.7% vs. 10.4%, P = 0.03). Conclusions: Functional success can be achieved in directly supervised or nondirectly supervised DMEK surgery. However, nondirectly supervised DMEK surgery may associate with higher rates of complications.
  • Combined aqueous misdirection and persistent choroidal effusions following implantation of a Preserflo MicroShunt

    Malick, Huzaifa
    Aim: To describe a case of aqueous misdirection complicated by subsequent persistent choroidal effusions following implantation of a Preserflo MicroShunt (PMS) device to treat advanced closed angle glaucoma. Methods: A 67-year-old caucasian female with advanced primary angle-closure glaucoma on four medications with an intraocular pressure (IOP) of 26 mm Hg was listed for a PMS insertion with mitomycin C (MMC). Results: Past ocular history was significant for pseudophakia and previous yttrium aluminum garnet (YAG) peripheral iridotomy. Surgery was uneventful but on the first postoperative day, she developed aqueous misdirection complicated by subsequent development of persistent uveal effusions. Conventional treatment strategies including atropine drops, YAG hyaloidotomy and choroidal effusion drainage proved ineffective. A combination of oral steroids and pars plana vitrectomy (PPV) along with an irido-zonulo-hyloidectomy (IZH) proved efficacious. Conclusion: To the best of the author's knowledge, this is the first published case of aqueous misdirection complicated with the presence of significant, unresolving choroidal effusions, highlighting the possibility and sequelae of comorbid pathology in nanophthalmic eyes.
  • Severity of cystoid macular oedema in preterm infants observed using hand-held spectral domain optical coherence tomography improves weekly with postmenstrual age

    Anwar, Samira (2023-03-16)
    Objective: To investigate the relationship between cystoid macular oedema (CMO) measured in preterm infants using hand-held spectral domain optical coherence tomography (HH SD-OCT), with gestational age at birth (GA), birthweight (BW), diagnosis of retinopathy of prematurity (ROP) and the presence or absence of the external limiting membrane (ELM). Methods: We conducted a prospective mixed cross-sectional/longitudinal observational study of 112 participants (23 to 36 weeks GA; n = 25 with, and n = 87 without, CMO). Retinal images were acquired using 344 HH SD-OCT (n = 66 with and n = 278 without, CMO) between 31 to 44 weeks postmenstrual age (PMA). CMO type ('fovea' and 'dome') was measured using thickness, width, area and peak. Results: CMO was observed in 22.9% of preterm infants, and 19.2% of images. The mean values for thickness, width, area and peak of 'dome' CMO were 128.47 µm (SD +/- 34.23), 3624.45 µm (SD +/- 1323.03), 0.49 mm2 (SD +/- 0.28) and 279.81 µm (SD +/- 13.57) respectively. The mean values for thickness, width, area and peak of 'fovea' CMO were 64.37 µm (SD +/- 17.11), 2226.28 µm (SD +/- 1123.82), 0.16 mm2 (SD +/- 0.11) and 95.03 µm (SD +/- 26.99) respectively. Thickness, area width and peak were significantly greater for 'dome CMO compared with 'fovea' CMO (P < 0.0001 for thickness, area and peak; P < 0.01 for width). Area and width significantly decreased with PMA for 'dome' and 'fovea' CMO (p = 0.0028; p < 0.001 respectively). No association was found between the presence of ROP and the detection of CMO or detection of CMO with absence of ELM. Conclusions: HH -OCT in preterm infants demonstrates that the severity of CMO appearance improves each week for both fovea and dome CMO.
  • Virtual keratoconus monitoring clinic in a tertiary university hospital in the United Kingdom

    Molero-Senosiain, Mercedes; Vali, Yahya; Mistry, Prashant; Savant, Vijay (2023-03)
    Purpose: To describe a new pathway for virtual keratoconus (KC) monitoring in the corneal department of a tertiary referral center in the UK during the coronavirus disease 2019 (COVID-19) pandemic. Methods: A virtual outpatient clinic to monitor KC patients (KC PHOTO clinic) was created. All patients from the KC database in our department were included. At each hospital visit, patients' visual acuity and tomography (Pentacam; Oculus, Wetzlar, Germany) were collected by a health-care assistant and an ophthalmic technician, respectively. The results were virtually reviewed by a corneal optometrist to identify stability or progression of KC and discussed with a consultant if needed. Those with progression were contacted by telephone and listed for corneal crosslinking (CXL). Results: From July 2020 until May 2021, 802 patients were invited to attend the virtual KC outpatient clinic. Of them, 536 patients (66.8%) attended and 266 (33.2%) did not attend. After corneal tomography analysis, 351 (65.5%) were stable, 121 (22.6%) showed no definite evidence of progression, and 64 (11.9%) showed progression. Forty-one (64%) patients with progressive KC were listed for CXL and the remaining 23 patients deferred treatment after the pandemic. By converting a face-to-face clinic to a virtual clinic, we were able to increase our capacity by nearly 500 appointments per year. Conclusion: In pandemic times, hospitals have developed novel methods of delivering safe patient care. KC PHOTO is a safe, effective, and innovative method of monitoring KC patients and diagnosing progression. In addition, virtual clinics can increase the clinic capacity tremendously and reduce the need of face-to-face appointments, which is beneficial in pandemic conditions.
  • Home-based screening tools for amblyopia: a systematic review

    Chean, Chung Shen; Kuht, Helen; Thomas, Mervyn; Rufai, Sohaib (2023-02-24)
    Amblyopia is an important public health concern. While home-based screening may present an effective solution, this has not been rigorously assessed in a systematic review. A systematic review was performed using Ovid MEDLINE, PubMed, The Cochrane Library, Embase, Web of Science Core Collection, and Clinicaltrials.gov. All studies reporting the diagnostic accuracy of home-based screening tools for amblyopia among children were included. Studies involving orthoptist or ophthalmologist-led screening and adult subjects were excluded. The main outcome measure was the diagnostic accuracy expressed as sensitivity and specificity. Among 3670 studies identified, 28 were eligible for inclusion in our systematic review. The age range of patients were less than 1 month to 16 years old. 7 studies used internet-based tools, 16 used smartphone/tablet applications, 3 used digital cameras, and 3 used home-based questionnaires and visual acuity tools. All studies included a reference standard except one, which was a longitudinal study. 21 studies had full ophthalmological examination whilst 6 studies had validated visual acuity measurement tools as gold standards. Of the 27 studies which compared against a reference test, only 25 studies reported sensitivity and specificity values. Using the QUADAS-2 tool, 50% of studies were deemed to have applicability concern due to patient selection from tertiary centres and unclear methods for recruitment. There is a need to improve the quality of diagnostic accuracy studies, standardise thresholds for detecting amblyopia, and ensure consistent reporting of results. Further research is needed to evaluate the suitability of these tools for amblyopia screening.
  • Heavy silicone oil tamponade: a multicentre experience

    Chaudhuri, Ray (2022-12-08)
    Objective: To report multicentred use of the heavy silicone oil Densiron 68 for anatomical reattachment following rhegmatogenous retinal detachment (RRD) repair and its associated complications. Methods and analysis: Patients from seven vitreoretinal units within the UK that underwent RRD repair with Densiron 68 between January 2015 and December 2019 were identified. Primary outcome measures were primary and final reattachment rate, retained Densiron and failure rate. Secondary outcome measures were duration of tamponade, final visual acuity (VA) and complications of heavy silicone oil. Results: 134 eyes of 134 patients were involved in the study. Primary surgical success was achieved in 48.5%, while a final reattachment rate of 73.4% was observed. The mean duration of Densiron 68 tamponade was 139.5 days. Mean final VA was 1.01 (range 0-2.9). 8 eyes (6.0%) required long-term topical steroids for anterior uveitis, whereas none of the eyes required long-term pressure-lowering treatment. Emulsification rate was 10.7% (6 eyes). Conclusion: This is the largest real-world study on Densiron 68 in the UK. Densiron 68 facilitates tamponade of inferior retinal pathology and may be considered as an option for tamponade of inferior retinal pathologies.
  • Electrophysiological and fundoscopic detection of intracranial hypertension in craniosynostosis

    Rufai, Sohaib (2022-01-01)
    Aims: To assess the diagnostic accuracy of fundoscopy and visual evoked potentials (VEPs) in detecting intracranial hypertension (IH) in patients with craniosynostosis undergoing spring-assisted posterior vault expansion (sPVE). Methods: Children with craniosynostosis undergoing sPVE and 48-hour intracranial pressure (ICP) monitoring were included in this single-centre, retrospective, diagnostic accuracy study. Data for ICP, fundoscopy and VEPs were analysed. Primary outcome measures were papilloedema on fundoscopy, VEP assessments and IH, defined as mean ICP > 20 mmHg. Diagnostic indices were calculated for fundoscopy and VEPs against IH. Secondary outcome measures included final visual outcomes. Results: Fundoscopic examinations were available for 35 children and isolated VEPs for 30 children, 22 of whom had at least three serial VEPs. Sensitivity was 32.1% for fundoscopy (95% confidence intervals [CI]: 15.9-52.4) and 58.3% for isolated VEPs (95% CI 36.6-77.9). Specificity for IH was 100% for fundoscopy (95% CI: 59.0-100) and 83.3% for isolated VEPs (95% CI: 35.9-99.6). Where longitudinal deterioration was suspected from some prVEPs but not corroborated by all, sensitivity increased to 70.6% (95% CI: 44.0-89.7), while specificity decreased to 60% (95% CI: 14.7-94.7). Where longitudinal deterioration was clinically significant, sensitivity decreased to 47.1% (23.0-72.2) and specificity increased to 100% (47.8-100). Median final BCVA was 0.24 logMAR (n = 36). UK driving standard BCVA was achieved by 26 patients (72.2%), defined as ≥0.30 logMAR in the better eye. Conclusion: Papilloedema present on fundoscopy reliably indicated IH, but its absence did not exclude IH. VEP testing boosted sensitivity at the expense of specificity, depending on method of analysis.
  • Radiotherapy for nasopharyngeal carcinoma: Effect on the eye 10 years later: A case report

    Taylor, Michael
    A patient who had previously received radiotherapy for a nasopharyngeal carcinoma was rightfully discharged from otorhinolaryngology and oncology once treatment was completed. After 10 years, the patient presented with visual loss in one eye and was found to have radiation retinopathy. This case highlights the importance of recognising the effects that radiation administered to structures near the eye can have on vision. The latency of this case demonstrates the need for routine eye tests in patients who have undergone radiotherapy near the orbit. Prompt recognition and referral to ophthalmologists is necessary for all suspected cases to best manage visual loss.

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