Pre-Operative Ocular Findings and Long-Term Follow-Up in a Large Cohort of Non-Syndromic Unicoronal Craniosynostosis.

(1) Background: Non-syndromic unicoronal craniosynostosis (UCS) is associated with a high prevalence of ocular anomalies. Currently, the etiology of this association remains obscure, however, it is presumed to be primarily attributed to their orbital malformations and/or secondary to craniofacial surgery. We assessed pre-operative ophthalmological examinations of non-syndromic UCS patients and compared them with their postoperative outcomes and long-term follow-up. (2) Methods: A retrospective case series was conducted on medical records of patients with non-syndromic UCS at Sophia Children's Hospital, Rotterdam. Ophthalmologic examinations were collected at different time periods: T1 (first visit), T2 (<1 year after cranioplasty), and T3 (long-term follow-up at last visit). The McNemar's test was used for statistical analysis. (3) Results: A total of 101 patients were included, for whom examinations were available at T1 and T3. Patients had a mean age of 2.8 years (±2.7) and 9.5 (±4.9) at T1 and T3, respectively. At T1, 52 patients (51.5%) were diagnosed with strabismus, and 61 patients (60.4%) at T3. Vertical strabismus increased significantly from 23 patients (22.8%) at T1 to 36 patients (35.6%) at T3 (p = 0.011). Followed by astigmatism, which increased significantly from 38 (37.6%) at T1 to 59 (58.4%) patients at T3 (p = 0.001). T1 was available in 20 patients prior to fronto-orbital advancement (FOA), therefore, a sub-analysis was conducted on these patients, which was followed shortly after FOA at T2. Prior to FOA, strabismus was present in 11 patients (55.0%) and in 12 patients (60.0%) at T2. After FOA, strabismus worsened in two patients. (4) Conclusions: This study showed the high prevalence of ocular anomalies in patients with non-syndromic UCS before and after cranioplasty and at long-term follow-up. The findings of this study show that ophthalmic and orthoptic examinations are an important part of the optimal treatment of patients with non-syndromic UCS.

Torticollis in Non-Syndromic Unicoronal Craniosynostosis Is Predominantly Ocular Related.

(1) Background: Patients with unicoronal craniosynostosis (UCS) often show torticollis which can result from either an ocular cause or contraction of the sternocleidomastoid muscle. For clinicians, it is crucial to know the prevalence of ocular torticollis (OT) to ensure appropriate referral for treatment. Furthermore, associated ophthalmic features with OT in these patients are scarcely described. The aim of this study was to determine the prevalence of OT in non-syndromic UCS patients and investigate its associated ophthalmic features. (2) Methods: In this descriptive cross-sectional study medical records of non-syndromic UCS patients treated between 1994-2022 in one tertiary care hospital in The Netherlands were retrospectively reviewed. Collected data included: diagnosis and type of torticollis, binocular single vision (BSV), strabismus, ocular motility, alphabetical patterns, refractive error, and amblyopia. Patients were classified as OT, based on their ophthalmic and/or orthoptic diagnosis. Prevalence was determined with the 95% CI using the Clopper-Pearson exact test. Associations between OT and the ophthalmic features were determined using Chi-square or Fishers' exact test and its effect size was calculated using Cramer's V. (3) Results: In total, 146 patients were included, of whom 57 had torticollis. An ocular cause for the torticollis was found in 54 patients. The prevalence of OT was 37% (n = 146; 95% CI [0.292-0.454]). Significant associations were found between OT and strabismus (p < 0.001), ocular motility abnormalities (p < 0.001), alphabetical patterns (p < 0.001), and amblyopia (p = 0.002). BSV (p = 0.277) and refractive error (p = 1.0) were not significantly associated with OT. However, in OT the BSV was relatively poor (42.1%) and more frequently absent (26.3%) compared to the non-torticollis group (7% poor and 16.3% absent). In both groups, excyclotorsion was predominantly present (62.3%). (4) Conclusions: In 95% of cases, torticollis in UCS patients is ocular-related. Overall, one in three patients with UCS have OT. This study emphasizes the importance of a timely referral of all patients with UCS with torticollis to an orthoptist and/or ophthalmologist, specialized in diagnosing and treatment of OT, before considering physiotherapy.

Effect of Midface Surgery on Ocular Outcomes in Patients with Orbital and Midface Malformations.

(1) Background: Orbital and midface malformations occur in multiple craniofacial disorders. Depending on the deformity, surgical corrections include orbital box osteotomy (OBO), Le Fort III (LFIII), monobloc (MB), and facial bipartition (FB). The aim of this study was to determine the effect of these procedures on ocular outcomes. (2) Methods: A retrospective analysis was performed. All patients with craniofacial disorders who had previously undergone midface surgery were included. The Wilcoxon signed ranks test was used for statistical analysis. (3) Results: In total, 63 patients were included: two patients were treated by OBO, 20 by LFIII, 26 by MB, and 15 by FB. Pre-operatively, strabismus was present in 39 patients (61.9%), in whom exotropia was most common (n = 27; 42.9%), followed by esotropia (n = 11; 17.5%). Postoperatively, strabismus significantly worsened (p = 0.035) in the overall population (n = 63). Pre-operative binocular vision (n = 33) was absent in nine patients (27.3%), poor in eight (24.2%), moderate in 15 (45.5%), and good in one (3.0%). Postoperatively, binocular vision significantly improved (p < 0.001). Before surgery, the mean visual acuity (VA) in the better eye was 0.16 LogMAR (Logarithm of the Minimum Angle of Resolution), and 0.31 LogMAR in the worse eye. Furthermore, pre-operative astigmatism was present in 46 patients (73.0%) and hypermetropia in 37 patients (58.7%). No statistical difference was found for VA (n = 51; p = 0.058) postoperatively. (4) Conclusions: Midface surgery has a direct and indirect substantial effect on several ocular outcomes. This study emphasizes the importance of appropriate ophthalmological evaluation in patients with craniofacial disorders undergoing midface surgery.

Efficacy and Safety of Micropulse Transscleral Cyclophotocoagulation.

Background: Early studies have shown that micropulse transscleral cyclophotocoagulation (MP-TSCPC) might be an effective and safe treatment option for lowering intraocular pressure (IOP). These studies were, however, somewhat limited, in particular by their retrospective nature and the length of follow-up. Therefore, we assessed the efficacy and safety of this novel treatment in a large cohort for up to 4 years. Methods: We performed a prospective cohort study, including all patients who were treated with MP-TSCPC since November 2017. The primary outcome was a reduction of IOP and the number of IOP-lowering medications. Results: The mean ± standard deviation baseline IOP and number of IOP-lowering medications were 26.6 ± 10.8 mmHg and 3.3 ± 1.3. IOP was reduced by 8.2 ± 7.9 (31.8% reduction), 6.9 ± 8.7 (28.1% reduction), and 7.1 ± 8.4 (30.2% reduction) mmHg after 6, 12, and 24 months, respectively (p < 0.001). The mean postoperative number of IOP-lowering medications was significantly reduced after 6 months by 0.6 ± 1.5 (p = 0.002) but was not significantly different after 12 or 24 months. Oral acetazolamide was significantly reduced from 28 (29%) eyes before treatment, to 9 (9%) at the last follow-up visit (p < 0.001). No major complications were observed after treatment. Conclusions: MP-TSCPC is a safe and effective treatment option for lowering IOP, but only reduced IOP-lowering medications in the first 6 months after treatment. However, MP-TSCPC is especially effective in getting patients off oral IOP-lowering drugs.

Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis.

BACKGROUND: The aim of this study was to describe the ophthalmic abnormalities and their prevalence in craniosynostosis prior to craniofacial surgery. METHODS: A systematic search was conducted on Medline OVID, Embase, Cochrane, Google Scholar, Web of Science Core Collection. Inclusion criteria were English papers, children aged <18 years with non-syndromic and syndromic craniosynostosis, case reports, case series, and case-control studies. A system of domains was established consisting of an anatomic and functional ophthalmic domain. A meta-analysis of single proportions was carried out using random effects model and pooled mean proportions with 95% confidence intervals (CI) were calculated. RESULTS: Thirty-two papers analyzing 2027 patients were included. Strabismus was the most common anomaly in non-syndromic craniosynostosis: Horizontal strabismus was highest prevalent in unicoronal craniosynostosis (UCS) 19% (95% CI 9-32), followed by vertical strabismus 17% (95% CI 5-33). In syndromic craniosynostosis, horizontal strabismus was most prevalent in Crouzon syndrome 52% (95 CI 26-76), followed by Apert syndrome 50% (95% CI 42-58). Vertical strabismus was most prevalent in Saethre-Chotzen 60% followed by Muenke's syndrome 36%. Furthermore, astigmatism was the second most reported outcome in non-syndromic craniosynostosis and highest prevalent in UCS 35% (95% CI 21-51). In syndromic craniosynostosis, astigmatism was most frequently seen in Crouzon syndrome 43% (95% CI 22-65), followed by Apert syndrome 34% (95% CI 14-58). Moreover, in syndromic craniosynostosis, 5-40% had a decrease in visual acuity (VA) ≤ 0.3 LogMAR in the better eye and 11-65% had a VA ≤ 0.3 LogMAR in at least one eye. DISCUSSION: This review demonstrates the high prevalence of ocular anomalies in non-syndromic and syndromic craniosynostosis. A multidisciplinary and systematic approach is needed for the screening and optimal treatment of these conditions in a timely manner.

Efficacy of the XEN-Implant in Glaucoma and a Meta-Analysis of the Literature.

BACKGROUND: To assess the efficacy of XEN-implant surgery in patients with glaucoma, and to perform a meta-analysis of previously published results and compare these to our data. METHODS: Prospective case-control study, in which all eyes that underwent XEN-implant surgery were included from 2015 onwards. Sub-analyses were performed for eyes that underwent XEN-implant as standalone procedure and as cataract-combined procedure. To compare our results, a systematic review was performed using the Embase, PubMed, Web of Science, and Cochrane database. Meta-analyses were performed by combining data (intraocular pressure (IOP), IOP-lowering medication, and complications) from the retrieved studies. RESULTS: A total of 221 eyes underwent XEN-implant surgery (124 standalone and 97 cataract-combined). The mean ± standard deviation IOP declined from 18.8 ± 6.5 to 13.5 ± 4.3 mmHg at the last follow-up (p < 0.001; 28.9%). Postoperative, no significant differences in IOP or IOP-lowering medication were found between patients with and without combined procedure. Secondary surgeries were performed in 20.8% of eyes, most of them (63.0%) within six months. A meta-analysis of 19 studies retrieved from the systematic review showed a two-years postoperative pooled mean (weighted mean difference) of 14.5 (7.3) mmHg and 1.0 (1.6) for IOP and IOP-lowering medications, respectively (compared to 13.5 (5.3) mmHg and 3.2 (2.4) in the current study). CONCLUSION: XEN-implant surgery was effective and safe in lowering IOP and the number of IOP-lowering medications. There were no differences between standalone and combined procedures.

Plusoptix photoscreener use for paediatric vision screening in Flanders and Iran.

PURPOSE: Photoscreening assesses risk factors for amblyopia, as an alternative to measurement of visual acuity (VA) to detect amblyopia, on the premise that its early correction could prevent development of amblyopia. We studied implementations of Plusoptix photoscreening in existing population-based screening in Flanders and Iran. METHODS: In Flanders, VA is measured at age 3, 4 and 6, photoscreening was added to existing screening at age 1 and 2.5 years in 2013. In Iran, VA is measured at ages 3-6 years, photoscreening was added at ages 3-6 years between 2011 and 2016. Plusoptix use was analysed in the literature for detection of risk factors for amblyopia and amblyopia itself, for ages 0-3 and for 4-6. A questionnaire, containing seven domains: existing vision screening, addition of photoscreening, implementation in screening program, training, attendance, diagnosis and treatment, and costs was distributed. In Iran, screening procedures were observed on site. RESULTS: Implementation of Plusoptix photoscreening was mainly analysed from questionnaires and interviews, its effectiveness from literature data. In Flanders, of 56 759 children photoscreened at age one (81% of children born in 2013), 9.2% had been referred, 13% of these were treated, mostly with glasses, resulting in an increase of 4-year-old children wearing glasses from 4.7% to 6.4%. In Iran, 90% of children aged 3-6 years participated in vision screening in 2016, but only those who failed the vision test were subjected to photoscreening. CONCLUSIONS: In Flanders, the use of Plusoptix photoscreening at ages 1 and 2.5 resulted in an increase of children wearing glasses, but it remains unknown how many cases of amblyopia have been prevented. Studies are needed to determine the relation between size and sort of refractive error and strabismus, and the increased chance to develop amblyopia.