Was it always so?
“The authority of those who teach is often an obstacle to those who want to learn.” – Marcis Tullius Cicero, 101–43 BC
At what age did you first take a real, deep interest in discovering ‘stuff’? You know what I’m talking about – not just picking up the beach stone and appreciating the softness of its surface – no. You took the beach stone and asked someone, “Why is this so soft? What is it doing here? Where did it come from? Is it made of sand, too?”
Curious minds are always asking questions. You, reader, could be a little bit annoying when you were younger, couldn’t you? Even so, I would bet that you weren’t as annoying as those fools that try and stand in the way of curious minds. The enforcers of right and wrong. The binary-minded, unoriginal Yes Men. We don’t want the right answers here! We want fearlessly wrong answers to the right questions, time and time again until we get something new! Well that’s not entirely true – you’re all doctors, so try not to get it too wrong, but still, new!
Here, feast on something new (in the order they appear):
NEURO-OPHTHALMOLOGY
Could idebenone by the solution for treating dominant optic atrophy?
Dominant optic atrophy (DOA) is a disease of the retinal ganglion cells, with no current treatment options. In most cases, DOA is caused by a mutation in the OPA1 gene. The aim of this study was to evaluate the effect of idebenone treatment in patients with OPA1 DOA. Sixteen patients with OPA1-DOA were treated with 900mg idebenone daily for one year. The primary endpoint was the best recovery / least deterioration of visual acuity. Secondary endpoints were the changes of visual acuity, colour vision, contrast sensitivity, visual field, peripapillary retinal nerve fibre layer thickness (pRNFLT), and visual-related quality of life. For the primary endpoint, a significant increase was observed for the right eye (p=0.0027), the left eye (p=0.0111) and for the better-seeing eye (p=0.0152). For visual fields, a significant improvement was observed for the left eye between baseline and nine months (p=0.0038). Regarding pRNFLT, a significant decrease was found for the left eye between baseline and three months (p=0.0413) and between baseline and six months (p=0.0448). In the visual function questionnaire, a significant improvement was observed in the subscale general vision (p=0.0156) and in the composite score (p=0.0256). In conclusion, best recovery of visual acuity improved, although the amount of improvement was minimal. Furthermore, a maintenance of visual function after 12 months of idebenone intake could be observed as well as a significant improvement in vision-related quality of life. Further research with a control group is required to determine whether this effect is due to idebenone treatment, the placebo effect, or is explainable by the natural progression of DOA. The authors recommend a randomised, placebo-controlled, and double-blind study over a longer time period to explore further evidence. – Claire Howard
Idebenone treatment in patients with OPA1-dominant optic atrophy: a prospective phase 2 trial. Valentin K, Georgi T, Riedl R, et al. NEURO-OPHTHALMOLOGY 2023;47(5-6):237–47. Taylor & Francis Group
Longitudinal findings in children with neurofibromatosis type 1
This retrospective chart review evaluates the ophthalmologic findings of children with neurofibromatosis type 1 (NF1). Optic pathway gliomas (OPGs) are the most common tumours found in children with NF1. Findings are compared between eyes with and without optic pathway gliomas (OPGs). Data compared included demographic characteristics, clinical manifestations of disease, and ophthalmologic findings including visual acuity, intraocular pressure, cup-to-disc ratio, visual field testing, and optical coherence tomography findings. Ophthalmologic findings were examined for the cohort for initial and final appointments. The study included 119 participants with 238 total eyes. The most common clinical manifestations of NF1 in this cohort were café au lait macules (98%), axillary or inguinal freckling (91%), Lisch nodules (66%), and cutaneous neurofibromas (57%). Thirty-seven participants had imaging that allowed evaluation for choroidal abnormalities, of which 28 (76%) had choroidal lesions. Twenty-seven participants (23%) had OPGs, and 44 eyes were affected. On initial assessment, eyes with OPGs had worse visual acuity. On final examination, eyes with OPGs were more likely to have a worse visual acuity and a thinner generalised retinal nerve fibre layer (RNFL) thickness, inferior RNFL thickness, and temporal RNFL thickness. This study provides longitudinal follow-up of children affected by NF1 with and without OPGs. – Claire Howard
Ophthalmologic findings in children with neurofibromatosis type 1. Zimmerman CM, Singh S, Cardakli N, Kraus C. NEURO-OPHTHALMOLOGY 2024;48(1):19–26. Taylor & Francis Group
PAEDIATRIC OPHTHALMOLOGY AND STRABISMOLOGY
Clinical features of basic acquired nonaccommodative esotropia requiring surgery
Basic acquired nonaccommodative esotropia (BANAET) is a concomitant esotropia occurring after the age of six months without significant refractive error or accommodative element. Esotropia is similar for both near and distance and children have prior binocular single vision. The purpose of this study was to review the preoperative clinical features in these patients who have had strabismus surgery over a 10-year period. Two-thousand-and-twelve children were grouped into: (1) managed with one surgery (76.1%), (2) managed with two surgeries (16.3%), and (3) managed with three or more surgeries (7.6%). Mean age at first surgery was 18.9 ±15.6 years. There were 57.1% male and 42.9% female. Most had first surgery at ages 3–6 years. Preop angle of deviation was a mean 36.8 ±17.2PD at distance and 37.3 ±17.4PD at near. Preop spherical equivalent was 0.31 ±1.44D right eye and 0.34 ±1.33D left eye. Mean visual acuity was better in group 1 than groups 2 and 3. Angle of deviation was significantly less for group 1. Amblyopia was present in 18.1% for group 1, 20.2% for group 2, and 26.7% for group 3. Bilateral amblyopia was less for group 1 and amblyopia was most frequent for group 3. Less cylindrical refractive error was in group 1. The authors recommend attention to refractive error in the differential diagnosis of BANAET vs. accommodative esotropia with +2.0D as a cut-off. They consider the prism adaptation test to identify the maximum angle when planning surgery, particularly as there are less favourable clinical features in those needing multiple surgeries. – Fiona Rowe
Basic acquired nonaccommodative esotropia patients managed with surgery; a study of 2102 patients. Akbari MR, Alghurab A, Azizi E, Khorrami-Nejad M. STRABISMUS 2023;31(4):281–9. Taylor & Francis Group
Comparison of motor and sensory outcomes for nonaccommodative esotropia and partial accommodative esotropia
Motor and sensory outcomes of surgery are compared for partial accommodative refractive esotropia (pARET) and acute nonaccommodative comitant esotropia (ANAET) to evaluate factors affecting their surgical success. This was a retrospective study of 38 ANAET (26 males, 12 females) and 33 pARET (13 males, 20 females) cases. Esotropia was noticed at a mean age of 8.55 ±4.65 years (ANAET) and 4.39 ±2.27 years (pARET) and mean age at surgery was 10.62 ±4.99 years and 7.89 ±2.84 years respectively. At surgery, 84% and 97% were <12 years old respectively. Mean duration of esotropia prior to surgery was 39.42 ±35.54 months (ANAET) and 29.95 ±42.45 months (pARET). Precipitating events (e.g. fever, trauma, hypertension) was noted in 30.77% (ANAET) and 0.09% (pARET). Preop amblyopia therapy was given in 23.68% and 51.52%, and vertical deviation in 34.21% and 45.45% respectively. In ANAET postoperatively, combined motor success at near and distance was 78.9% at six months and 81.5% at final follow-up. In pARET postoperatively, combined motor success at near and distance was 70.3% at six months and 73.3% at final follow-up. Greater numbers of ANAET remained ortho during follow-up whilst pARET cases showed a tendency to exo drift. The authors conclude both types of esotropia have good motor success for near and distance deviations and also for sensory outcomes with no significant differences between esotropia types. No demographic or preoperative factors were found to influence surgical outcomes. – Fiona Rowe
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Acquired comitant esotropias – comparison of surgical outcomes of accommodative vs non-accommodative types. Sharma R, Tibrewal S, Majumdar A, et al. STRABISMUS 2023;31(4):293–305. Taylor & Francis Group
PAEDIATRIC OPHTHALMOLOGY AND STRABISMOLOGY
Ahmed and Baerveldt glaucoma drainage implant outcomes
This study reports the surgical outcomes of both the Ahmed (AGV) and Baerveldt (BGI) glaucoma drainage implants in a cohort of patients with primary congenital glaucoma over a long follow-up in a single tertiary centre. This was a retrospective study of cases <16 years of age over a 10-year period. One-hundred-and-fifty-three eyes of 86 patients were reviewed: 19 AGV and 67 BGI groups. Children were significantly older in the BGI group than AGV: 8.9 ±13.5 months vs. 3.3 ±7.8 months. There was no significant difference between groups for gender, number of glaucoma medications or follow-up period. The number of children with more than one previous glaucoma procedure was significantly higher in the AGV group: 53.3% vs. 21.2%. There was no significant difference between groups for specific postoperative complications but there was significant difference for overall number of complications: 60.8% for AGV vs. 36.4% for BVI. There were also significantly more postoperative revisions and reoperations for AGV: 29.2% vs. 12.1%. The results show both surgical methods are successful in providing adequate intraocular pressure control in the first few postoperative years. However, there are differences in long-term outcomes. Baerveldt had significantly lower mean intraocular pressure, a higher surgical success rate and less complications. The authors note the limitation of their retrospective study design and recommend future prospective clinical trials. – Fiona Rowe
Comparison of the Ahmed and Baerveldt glaucoma drainage implants in the treatment of primary congenital glaucoma. Badawi AH, AlOwaifeer AM, Mofti A, et al. JOURNAL OF PEDIATRIC OPHTHALMOLOGY AND STRABISMUS 2023;60:448–54. SLACK Journals
RETINA VITREOUS AND UVEA
Impact of initial management on relapse in Vogt–Koyanagi–Harada syndrome
In this retrospective, multi-centre, observational study, the impact of the initial management of Vogt-Koyanagi-Harada syndrome (VKHS) was assessed by reviewing the records of all patients diagnosed with VKHS between January 2001 and December 2020 in two French tertiary centres, with patients with less than 6 months of follow-up excluded. Fifty patients were included with a mean age at diagnosis of 41.4 years old and a median duration of follow-up of 29.8 months. Forty-six of the patients received prednisone after methylprednisolone, five patients received at least one associated immunosuppressive therapy (IST) within the first six months and 26 patients received IST during the entire follow-up period. Immunosuppressive therapy was added for steroid-sparing purposes, steroid side-effects or at the physician’s discretion. Treatment delay was set at 26 days using the ROC curve approach. Twenty-eight patients (56%) had at least one relapse during follow-up, at a median of 5.4 months from diagnosis. Multivariate analysis was done, demonstrating a significant association between relapse and delayed treatment (>26 days) (HR=3.69, CI95% 1.30–10.47, p=0.01), whereas there was no association between relapse and the number of corticosteroid pulses at initial management. An analysis showed patients of Afro-Caribbean origin were significantly more likely to relapse (HR=3.18, CI95% 1.26–8.03, p<0.01) and there was a non-significant increase in relapse in patients treated 26 days after symptom onset (HR=2.07, CI95% 0.98–4.38, p=0.05). Overall, this study has shown that early corticosteroid treatment within the first 26 days of symptoms can decrease the relapse rate significantly. However, this study is limited by its retrospective design and small cohort size and further prospective studies are required to investigate the effects of IST as first-line on relapse rate. – Zina Mobarak
Impact of initial management on disease evolution in Vogt–Koyanagi–Harada syndrome: a retrospective cohort of 50 patients. Fauquier A, Barba T, Hot A, et al. OCULAR IMMUNOLOGY AND INFLAMMATION 2024;32(4):402–6. Taylor & Francis Group
DATES FOR YOUR DIARY
And with new research must come new people to talk to about it, as we’ve discussed previously. Know where to find them? I do. In the following dates for your diary. Remember, if nothing takes your fancy here, we’ve got a load more on our website’s events page.
See that last one on the list there, UKEGS? Yes, that’s the one. We’re going to be down in Southampton for that one, so if you’ll be around, why not reach out to us and we’ll see if we can get you into our media coverage of the event? Drop me an email on samuel@pinpoint-scotland.com if you want to talk about – or anything, really – a chinwag is fine.
All the very best to you and yours,