Idiopathic Intracranial Hypertension (IIH) is a debilitating disorder characterised by raised intracranial pressure (ICP), papilloedema with the potential risk of permanent visual loss, and headaches ...that are profoundly disabling and reduce the quality of life. The first consensus guidelines have been published on investigation and management of adult IIH and one key area of uncertainty is the utility of dural venous sinus stenting for the management of headache and visual loss. There are an increasing number of series published and to help understand the successes and complications. During a patient physician priority setting, the understanding of the best type of intervention to treat IIH was assigned to the top 10 of most desired research questions for the disease. Ultimately randomised clinical trials (RCTs) in neurovascular stenting for IIH would be instructive, as the literature to date may suffer from publication bias. Due to the increasing incidence of IIH, there is no better time to systematically investigate interventions that may reverse the disease process and achieve remission. In this review we discuss the pathophysiology of IIH in relation to venous sinus stenosis, the role of venous sinus stenting with a review of the relevant literature, the advantages and disadvantages of stenting compared with other surgical interventions, and the future of stenting in the treatment of IIH.
This paper reports a retrospective consecutive case series investigating the efficacy of Mitomycin C-augmented trabeculectomy in the treatment of primary and secondary pediatric glaucoma in a mixed ...etiology, multiethnic cohort of patients.
To evaluate the long-term efficacy and safety of Mitomycin C-augmented trabeculectomy in a mixed, tertiary-referral, pediatric glaucoma cohort.
Retrospective consecutive review of all children (37 eyes) undergoing Mitomycin C-augmented trabeculectomy by a single surgeon between 2008 and 2016. Seventeen eyes (45.9%) had primary congenital glaucoma, and 20 eyes (54.1%) had secondary glaucoma. The median age at surgery was 11 months (range, 2 to 146). The mean follow-up was 69.2±4.7 months (range, 3.5 to 107.9).
Overall, trabeculectomy was successful in 80.6% of eyes at 12 months, 60.5% at 3 years, and 57.5% at 5 years. 45.9% cases (17 eyes) required further laser or surgery for uncontrolled intraocular pressure (IOP) and were therefore deemed as failures. The time to failure ranged from 0.4 to 65.1 months (mean, 22.2±5.1 mo). The proportion of children achieving visual acuity of 1.0 LogMAR equivalent or better increased from 43.2% preoperatively to 63.6% at 1 year and 68% at 5 years. The mean IOP reduced from 24.85±0.88 mm Hg preoperatively to 15.14±0.94 mm Hg at 3 months (39% reduction) and 17.42±1.08 mm Hg at 5 years (30% reduction). IOP-lowering medication requirement reduced from 4.14±0.20 agents preoperatively to 0.84±0.22 at 3 months (80% reduction) and 1.78±0.36 at 5 years (57% reduction). There were no sight-threatening complications such as hyphaema, bleb leak, chronic hypotony, endophthalmitis, retinal detachment, or loss of light perception.
This study provides valuable evidence that Mitomycin C-augmented trabeculectomy is safe and effective as a treatment of primary or secondary pediatric glaucoma, with particularly encouraging results in cases of secondary glaucoma. Trabeculectomy offers the potential for delaying or avoiding glaucoma drainage device surgery in a significant proportion of children.
The identification of somatic
variation is crucial to confirm the heritability of retinoblastoma. We and others have previously shown that, when tumour DNA is unavailable, cell-free DNA (cfDNA) ...derived from aqueous humour (AH) can be used to identify somatic
pathogenic variation. Here we report
pathogenic variant detection, as well as cfDNA concentration in an extended cohort of 75 AH samples from 68 patients. We show cfDNA concentration is highly variable and significantly correlated with the collection point of the AH. Cell-free DNA concentrations above 5 pg/µL enabled the detection of 93% of known or expected
pathogenic variants. In AH samples collected during intravitreal chemotherapy treatment (Tx), the yield of cfDNA above 5 pg/µL and subsequent variant detection was low (≤46%). However, AH collected by an anterior chamber tap after one to three cycles of primary chemotherapy (Dx1+) enabled the detection of 75% of expected pathogenic variants. Further limiting our analysis to Dx1+ samples taken after ≤2 cycles (Dx ≤ 2) provided measurable levels of cfDNA in all cases, and a subsequent variant detection rate of 95%. Early AH sampling is therefore likely to be important in maximising cfDNA concentration and the subsequent detection of somatic
pathogenic variants in retinoblastoma patients undergoing conservative treatment.
Improving understanding of the underlying pathophysiology of giant cell arteritis (GCA) is transforming clinical management by identifying novel avenues for targeted therapies. One key area of ...concern for both clinicians and patients with GCA is glucocorticoid (GC) morbidity. The first randomised controlled trials of targeted treatment to reduce cumulative GC use in GCA have been published, with tocilizumab, an interleukin (IL)-6 receptor inhibitor, now the first ever licensed treatment for GCA. Further potential therapies are emerging owing to our enhanced understanding of the pathophysiology of the disease. Other improvements in the care of our patients are rapid access pathways and imaging techniques, such as ultrasound, which are becoming part of modern rheumatology practice in the UK, Europe and beyond. These have been highlighted in the literature to reduce delay in diagnosis and improve long-term outcomes for those investigated for GCA.
Abstract BACKGROUND Optic nerve sheath meningiomas (ONSM) are rare, comprising 1-2% of paediatric CNS meningiomas. Despite slow growth, ONSM often lead to vision loss in the affected eye. Diagnosis ...is challenging due to the tumour’s proximity to the optic nerve, complicating biopsy or complete removal. METHODS We searched our Neuro-Oncology MDT database for patients treated for ONSM between Jan 2018 and Dec 2023. We retrospectively reviewed clinical presentation and diagnostic workup that led to the diagnosis of ONSM. RESULTS 6 children aged 3-15years (mean 9 years) were identified over 5 years. 3 children were treated at GOSH, 2 at UCLH and 1 at BCH. 3/6 patients were male. 2/6 underwent a biopsy. Presenting symptoms included headache, acute visual loss and eye pain. 2/6 patients presented with bilateral vision deterioration. The average time between onset of symptoms and presentation was 8 months (range 3–15 months). Identified causes of delayed diagnosis included delayed presentation related to COVID-19 pandemic (2 patients) and impaired quality of imaging due to dental braces (1 patient). Whilst awaiting diagnosis, 2 patients lost vision completely in their affected eye. Diagnostic workup included: optic disc examination (6 patients), lumbar puncture (2 patients), angiotensin-converting enzyme (sACE) (2 patients), MRI brain and spine (6 patients), CT scan (6 patients), PET-DOTATE scan (3 patients), NF2 genetic testing (4 patients). Two patients completed PBT therapy while 1 is undergoing radiotherapy. One patient underwent an enucleation, while the rest are under surveillance. CONCLUSIONS Paediatric ONSM diagnosis remains challenging despite advanced imaging. Our review highlights the added value of CT and PET-DOTATATE when a biopsy is high-risk. CT reveals calcification in 20-50% ONSM while the role of PET-DOTATATE is related to the expression of somatostatin receptor 2 in meningiomas. Despite optimal treatment, vision loss maybe unavoidable. Early multidisciplinary consultations are advised for enhanced outcomes.
Background: Retinoblastoma (Rb) is the most common intraocular malignancy of childhood. The prognosis in Rb directly relates to the spread of disease beyond the eye, particularly to the central ...nervous system. Therefore, until the recent past, surgically entering an eye with active or recently active Rb was absolutely contraindicated in most centres around the world due to the risk of iatrogenic extraocular spread. Summary: In this review article, we explore the role of intraocular surgery in Rb. We describe the techniques which have been developed in order to safely deliver chemotherapeutic agents into the eye, both intravitreal and intracameral. We discuss the role of vitrectomy and endoresection for treatment of vitreous seeds and active retinal tumours. In the context of previously treated Rb, we outline various surgical interventions performed to improve visual outcomes or to manage the ocular sequelae of the disease. In particular, we review the various techniques used to maximise the safety of cataract, retinal and glaucoma surgery in children with a history of Rb. Key Messages: The role of intraocular surgery in Rb is now well established, particularly in the delivery of chemotherapeutic agents or in the presence of previously treated diseases. The role of intraocular surgery in eyes with active Rb is less well established and remains the subject of much debate. However, it is likely that with further development of safer surgical techniques, the indications and modalities will continue to broaden.
Assessment of disease burden and drug efficacy is achieved preclinically using high resolution micro computed tomography (CT). However, micro-CT is not applicable to clinical human imaging due to ...operating at high dose. In addition, the technology differences between micro-CT and standard clinical CT prevent direct translation of preclinical applications. The current proof-of-concept study presents spectral photon-counting CT as a clinically translatable, molecular imaging tool by assessing contrast uptake in an ex-vivo mouse model of pulmonary tuberculosis (TB). Iodine, a common contrast used in clinical CT imaging, was introduced into a murine model of TB. The excised mouse lungs were imaged using a standard micro-CT subsystem (SuperArgus) and the contrast enhanced TB lesions quantified. The same lungs were imaged using a spectral photoncounting CT system (MARS small-bore scanner). Iodine and soft tissues (water and lipid) were materially separated, and iodine uptake quantified. The volume of the TB infection quantified by spectral CT and micro-CT was found to be 2.96 mm 3 and 2.83 mm 3 , respectively. This proof-of-concept study showed that spectral photon-counting CT could be used as a predictive preclinical imaging tool for the purpose of facilitating drug discovery and development. Also, as this imaging modality is available for human trials, all applications are translatable to human imaging. In conclusion, spectral photon-counting CT could accelerate a deeper understanding of infectious lung diseases using targeted pharmaceuticals and intrinsic markers, and ultimately improve the efficacy of therapies by measuring drug delivery and response to treatment in animal models and later in humans.
In this article, we propose a slice-based interactive segmentation of spectral CT datasets using a bag of features method. The data are acquired from a MARS scanner that divides up the X-ray spectrum ...into multiple energy bins for imaging. In literature, most existing segmentation methods are limited to performing a specific task or tied to a particular imaging modality. Therefore, when applying generalized methods to MARS datasets, the additional energy information acquired from the scanner cannot be sufficiently utilized. We describe a new approach that circumvents this problem by effectively aggregating the data from multiple channels. Our method solves a classification problem to get the solution for segmentation. Starting with a set of labeled pixels, we partition the data using superpixels. Then, a set of local descriptors, extracted from each superpixel, are encoded into a codebook and pooled together to create a global superpixel-level descriptor (bag of features representation). We propose to use the vector of locally aggregated descriptors as our encoding/pooling strategy, as it is efficient to compute and leads to good results with simple linear classifiers. A linear support vector machine is then used to classify the superpixels into different labels. The proposed method was evaluated on multiple MARS datasets. Experimental results show that our method achieved an average of more than 10% increase in the accuracy over other state-of-the-art methods.
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