Ocular Tics and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS).

Little is known about ocular tics in Pediatric Autoimmune Neuropsychiatric Disorders associated with Streptococcal infections (PANDAS). In this retrospective study, we examined the clinical records of children with motor tics referred to the Ophthalmology Unit, Azienda Ospedaliero-Universitaria di Sassari, Italy, in 2010-2019. The presence of ocular tics was investigated. Data about antistreptolysin O (ASO) and anti-DNase B antibody titers, erythrocyte sedimentation rate (ESR), plasma C-reactive protein (CRP), and antibiotic use were recorded. Forty children (thirty-four boys and six girls; mean age: 7.65 ± 2.5 years) with motor tics were identified; thirty-three (82.5%) showed ocular tics. Children with ocular tics had significantly higher titers of anti-DNase B antibodies (p = 0.04) and CRP (p = 0.016) than those with extraocular tics. A diagnosis of PANDAS was made in 24 (60%) children. PANDAS children with oculomotor tics had significantly higher titers of anti-DNase B antibodies (p = 0.05) than those with extraocular tics. Oral antibiotics were given to 25/33 (76%) children with ocular tics and 21/24 (87.5%) with PANDAS. All treated patients showed marked improvement/complete resolution of symptoms. Results suggest that higher titers of anti-DNase B antibodies may be implicated in the pathogenesis of ocular tics in PANDAS. Oral antibiotics may be beneficial in improving ocular tics. Further research is necessary to confirm our findings.

Real-time monitoring of riboflavin concentration using different clinically available ophthalmic formulations for epi-off and epi-on corneal cross-linking.

PURPOSE: To assess the feasibility of theranostics to determine the riboflavin concentration in the cornea using clinically available ophthalmic formulations during epithelium-off (epi-off) and transepithelial (epi-on) corneal cross-linking procedures. METHODS: Thirty-two eye bank human donor corneas were equally randomized in eight groups; groups 1 to 3 and groups 4 to 8 underwent epi-off and epi-on delivery of riboflavin respectively. Riboflavin ophthalmic solutions were applied onto the cornea according to the manufacturers' instructions. The amount of riboflavin into the cornea was estimated, at preset time intervals during imbibition time, using theranostic UV-A device (C4V CHROMO4VIS, Regensight srl, Italy) and expressed as riboflavin score (d.u.). Measurements of corneal riboflavin concentration (expressed as µg/cm3) were also performed by spectroscopy absorbance technique (AvaLight-DH-S-BAL, Avantes) for external validation of theranostic measurements. RESULTS: At the end of imbibition time in epi-off delivery protocols, the average riboflavin score ranged from 0.77 ± 0.38 (the average corneal riboflavin concentration was 213 ± 190 µg/cm3) to 1.79 ± 0.07 (554 ± 103 µg/cm3). In epi-on delivery protocols, the average riboflavin score ranged from 0.17 ± 0.01 to 0.67 ± 0.19 (corneal riboflavin concentration ranged from 6 ± 5 µg/cm3 to 122 ± 39 µg/cm3) at the end of imbibition time. A statistically significant linear correlation (P ≤ 0.05) was found between the theranostic and spectrophotometry measurements in all groups. CONCLUSIONS: Real-time theranostic imaging provided an accurate strategy for assessing permeation of riboflavin into the human cornea during the imbibition phase of corneal cross-linking, regardless of delivery protocol. A large variability in corneal riboflavin concentration exists between clinically available ophthalmic formulations both in epi-off and epi-on delivery protocols.

Subthreshold Micropulse Laser for Long-Lasting Submacular Fluid after Rhegmatogeous Retinal Detachment Surgery.

Purpose: To assess the safety and efficacy of subthreshold micropulse laser (SML) photo-stimulation in the management of persistent subfoveal fluid (PSF) after surgery for rhegmatogenous retinal detachment (RRD). Methods: In this pilot study, 11 eyes of 11 patients (8 men, 3 women) with long-lasting (12-18 months) PSF after surgery for RRD were evaluated before and after photostimulation with subthreshold micropulse yellow laser. Ophthalmic examination included best-corrected visual acuity (BCVA), Amsler grid test, ophthalmoscopy, autofluorescence (AF), and optical coherence tomography (OCT) with measurement of central point foveal thickness (CPFT). Primary outcome was subfoveal fluid resolution and secondary outcome was BCVA improvement. Results: The mean CPFT and BCVA were, respectively, 436.8 ± 28.8 µm and 0.25 ± 0.1 µm decimal equivalent (DE) before photostimulation and 278 ± 54.4 µm and 0.57 ± 0.2 µm DE after photostimulation, a statistically significant difference (P < 0.001). Nine (81.8%) eyes showed improved BCVA, disappearance of macular detachment on ophthalmoscopy, reduced retinal pigment epithelium distress on AF, and restored macular profile with no neuroretinal alterations on OCT scans. Conclusion: Although PSF after RRD surgery is often a self-limiting disease, our results suggest that SML photostimulation may be effective and safe in patients with clinically significant long-lasting PSF. Larger case-control studies are necessary to confirm these results.

Novel retinal finding in a patient with 4q12 deletion.

BACKGROUND: Chromosome 4q deletions are rare disorders phenotypically characterized by several features. The most commonly described ocular abnormalities include unilateral microphthalmia with bilateral colobomata, blue sclerae with pigmented retinal clumps, hypermetropia, and a divergent squint. PURPOSE: To report a case of 4q12 deletion with a singular retinal feature. MATERIALS AND METHODS: Case report. RESULTS: A 20-year-old Caucasian female with a history of poliosis, progressive appearance of small areas of skin depigmentation along trunk and limbs since birth and diagnosis of learning deficit was referred for a complete ocular examination. The genetic counseling showed microdeletion in the 4q12 region. An audiometric test was performed, showing a progressive bilateral neurosensorial hypoacusia. Ocular examination showed the presence of multifocal, tiny, whitish deposits in the posterior pole. Multimodal imaging defined the lesions as small elevations of the retinal pigment epithelium with slight hyper-autofluorescence and staining in the late phase of fluoresceine angiography (FA). Visual acuity was 20/20. The retinal findings did not change during the three-month follow-up. CONCLUSIONS: Although the findings herein reported have never been described before in patients affected by 4q12 mutations, we do not exclude that they could represent a manifestation of the peculiar genetic asset of the patient, related to dysfunction in pigment epithelium/neuroretinal metabolic activity.

MicroRNA Expression in the Aqueous Humor of Patients with Diabetic Macular Edema.

We identified and compared secreted microRNA (miRNA) expression in aqueous humor (AH) and plasma samples among patients with: type 2 diabetes mellitus (T2D) complicated by non-proliferative diabetic retinopathy (DR) associated with diabetic macular edema (DME) (DME group: 12 patients); T2D patients without DR (D group: 8 patients); and non-diabetic patients (CTR group: 10 patients). Individual patient AH samples from five subjects in each group were profiled on TaqMan Low Density MicroRNA Array Cards. Differentially expressed miRNAs identified from profiling were then validated in single assay for all subjects. The miRNAs validated in AH were then evaluated in single assay in plasma. Gene Ontology (GO) analysis was conducted. From AH profiling, 119 mature miRNAs were detected: 86 in the DME group, 113 in the D group and 107 in the CTR group. miRNA underexpression in the DME group was confirmed in single assay for let-7c-5p, miR-200b-3p, miR-199a-3p and miR-365-3p. Of these four, miR-199a-3p and miR-365-3p were downregulated also in the plasma of the DME group. GO highlighted 54 validated target genes of miR-199a-3p, miR-200b-3p and miR-365-3p potentially implied in DME pathogenesis. Although more studies are needed, miR-200b-3p, let-7c-5p, miR-365-3p and miR-199a-3p represent interesting molecules in the study of DME pathogenesis.

Intraconal Retro-Orbital B-Type Antoni Neurinoma Causing Vision Loss.

Schwannoma is a rare benign tumor, originating from Schwann cells of the peripheral nerve sheath. We describe a case of intraconal orbital schwannoma, causing compression to the optic nerve and progressive visual impairment treated with surgical resection. The diagnosis of benign orbital schwannoma was provided by histopathological examination (large spindle cell tumor with AntoniB-type cell pattern) and also with immunohistochemistry (positivity for S-100 protein). The 6 months' follow-up was performed. The patient was asymptomatic during the 6-month follow-up.

Iontophoresis Corneal Cross-linking With Enhanced Fluence and Pulsed UV-A Light: 3-Year Clinical Results.

PURPOSE: To assess 3-year safety and efficacy of enhanced-fluence pulsed-light iontophoresis cross-linking (EF I-CXL) in patients with progressive keratoconus. METHODS: This prospective interventional pilot study included 24 eyes of 20 patients, with a mean age of 23.9 years (range: 15 to 36 years). Iontophoresis with riboflavin solution was used for stromal imbibition. The treatment energy was optimized at 30% (7 J/cm2) and ultraviolet-A power set at 18 mW/cm2 × 6.28 minutes of pulsed-light on-off exposure, with a total irradiation time of 12.56 minutes. Uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), corneal tomography, and corneal optical coherence tomography (OCT) at baseline and 1, 3, 6, 12, 24, and 3 years postoperatively were evaluated. RESULTS: At 3 years, average UDVA decreased from 0.50 ± 0.10 to 0.36 ± 0.08 logMAR (P < .05), average maximum keratometry decreased from 52.94 ± 1.34 to 51.4 ± 1.49 diopters (D) (Delta: -1.40 ± 0.80 D; P < .05), average coma improved from 0.24 ± 0.05 to 0.12 ± 0.02 µm (P = .001), and symmetry index decreased from 4.22 ± 1.01 to 3.53 ± 0.90 D. Corneal OCT showed demarcation line detection at 285.8 ± 20.2 µm average depth in more than 80% at 1 month postoperatively. CONCLUSIONS: The 3-year results of EF I-CXL showed satisfactory I-CXL functional outcomes, increasing the visibility and the depth of demarcation line closer to epithelium-off standard CXL. [J Refract Surg. 2020;36(5):286-292.].

Longitudinal quantitative assessment of macula during therapy with fingolimod in relapsing-remitting multiple sclerosis.

PURPOSE: Fingolimod is the first oral drug approved for treatment of relapsing-remitting multiple sclerosis (RR-MS), and it has potential macular side effects. Despite the qualitative evidence of macular oedema under treatment, longitudinal quantitative assessment is lacking. To address this issue, we measured macular volume and central foveal thickness in a cohort of MS patients on fingolimod over 12 months of treatment. METHODS: Central foveal thickness (CFT) and total macular volume (TMV) were longitudinally recorded with spectral-domain optical coherence tomography in a cohort of 23 RR-MS patients treated with fingolimod at baseline, 3, 6 and 12 months. OCT parameters were analysed considering previous history of optic neuritis (ON). Comparison of means was performed with variance analysis (ANOVA). RESULTS: Macular oedema occurred in none of the patients. Comparing both groups of patients (with and without previous ON), no statistically significant difference was found during the follow-up both for CFT and TMV (p = 0.99 and p = 0.96, respectively) although a slight early but not significant TMV reduction was detected. CONCLUSIONS: In our cohort, therapy with fingolimod did not cause any change in CFT and TMV in MS patients during a 12-month follow-up independent of previous ON.

Accelerated 15 mW pulsed-light crosslinking to treat progressive keratoconus: Two-year clinical results.

PURPOSE: To assess the clinical and microstructural results of accelerated 15 mW pulsed-light corneal crosslinking (CXL) to treat progressive keratoconus. SETTING: Siena Crosslinking Center, Siena, Italy. DESIGN: Prospective case series. METHODS: After epithelium removal (with Epi-Clear) and 10 minutes stromal soaking with riboflavin 0.1% hydroxypropyl methylcellulose solution, all eyes had 15 mW/cm2 pulsed-light epithelium-off accelerated CXL for 6 minutes of ultraviolet-A (UVA) irradiation (1 second on/1 second off), maintaining a total UVA exposure of 12 minutes at a fluence of 5.4 J/cm2. The 2-year follow-up examination included uncorrected (UDVA) and corrected (CDVA) distance visual acuities, Scheimpflug tomography, in vivo confocal microscopy (IVCM), and spectral-domain optical coherence tomography (SD-OCT). RESULTS: The study comprised 132 eyes of 96 patients (mean age 23.7 years ± 4.3 [SD]) with stage II keratoconus. The change in UDVA and CDVA was statistically significant, from 0.51 ± 0.106 logarithm of the minimum angle of resolution (logMAR) at baseline to 0.309 ± 0.074 logMAR (P = .0001) and 0.271 ± 0.144 logMAR at baseline to 0.135 ± 0.100 logMAR (P = .0023), respectively. Coma values measured by Scheimpflug analysis showed a statistically significant improvement beginning with the first postoperative month (P = .0004). The IVCM scans documented basal epithelial healing occurring 72 hours after treatment associated with the presence of subepithelial nerves. The SD-OCT scans performed in the central 6.0 mm of corneal diameter documented a demarcation line at a mean depth of 280 ± 32 µm. CONCLUSION: The 15 mW/cm2 pulsed-light epithelium-off accelerated CXL was effective and safe, stabilizing keratoconus progression through 2 years of follow-up.

Epithelial cyst of the superior oblique muscle trochlea.

Most of the congenital orbital cysts are choristomas such as dermoid or epidermoid and only in a few cases they are epithelial. Clinically, they manifest as cystic movable formations mostly localized in the upper temporal quadrant of the orbit. We describe here the case of a 49-year-old man with an orbital cyst localized in the upper-nasal quadrant of the orbit and which was showing signs of a gradual enlargement and progression over the past weeks. Computed tomography revealed a cyst of 1.9 × 1.6 cm in size and located within the trochlea of the upper oblique muscle. The cyst was completely extirpated after orbitotomy performed by superciliary approach. Histopathology revealed a cyst with nonkeratinized cuboidal epithelium. Postoperative course was uneventful, without inflammation signs, and after 5 weeks excellent functional and aesthetic effects were achieved with no iatrogenic alteration of the ocular motility.

Orbital glomangioma.

Glomangioma is a vascular hamartomatous tumor originating from the glomus body, a specialized thermoregulatory organ localized in the dermis and precoccygeal soft tissue. Glomus tumors are characteristically found in the hands and are very rarely reported in the head region. Here we describe a 29-year-old woman presenting with acute proptosis and pain in the left eye. A computed tomography scan of the orbit revealed a well-defined circumscribed mass, displacing the globe and lateral rectus muscle inferotemporally. The tumour was surgically removed through a lateral Kronlein approach. On histology, the lesion consisted of cavernous vascular spaces, partly filled with blood and thrombotic material, and a mixture of capillary-sized vessels and tumor cells; immunostaining for smooth actin showed a strong positivity in the cytoplasm of tumor cells, consistent with a diagnosis of glomangioma. Our case adds to the very few cases of orbital glomangioma reported in the literature and demonstrates that this tumor can be safely removed.

Phacoemulsificator and sterile drapes contamination during cataract surgery: a microbiological study.

PURPOSE: To determine the microbial contamination of the irrigating fluids at the time of phacoemulsification after the use of topical povidone-iodine and antibiotics prophylaxis. METHODS: A total of 119 patients undergoing cataract surgery were enrolled in this prospective study. All patients received 5 mg/mL levofloxacin starting from the day prior to surgery and topical and 5% povidone-iodine drops starting from 30 minutes before the surgery. At the end of each surgery, 2 samples of drainage liquids were sterilely collected from the drainage bags (DBL) and from the peristaltic pump single-cassettes (PCL) of the phacoemulsification machine. Search for aerobic and anaerobic bacteria and fungi was performed. RESULTS: Seventy-five patients (31.5%) revealed a growth of at least one microbial species (53 DBL and 22 PCL, 44.5% vs 18.5%; p<0.001). Sixty-six patients (55.5%) had at least one positive intraoperative solution. Overall, 111 microbial strains were collected: 82 (74%) Gram-positive bacteria, 20 (18%) fungi, and 9 (8%) Gram-negative bacteria. Thirteen staphylococcal isolates from PCL, compared with 52 out of DBL (11% vs 43.7%, p<0.001), fungi were essentially isolated from PCL. No significant correlation was found between microbial isolation and risk factors. No postsurgical infective complication occurred in the follow-up. CONCLUSIONS: Evaluation of intraoperative fluids can provide evidence on sources or vehicles of postsurgical infections. Antibiotic prophylaxis and topical povidone-iodine can significantly contribute to minimize the risk of endophthalmitis.

Spindle cell lipoma of the orbit: a case report of an unusual orbital pathology.

Spindle cell lipoma is a rare and distinct variant of soft tissue tumour characterised by spindle cells in which the fat content may be scarce or absent. Most spindle cell lipomas arise as a subcutaneous mass of the neck, shoulders or back. Rarely, they can also be found in unusual sites, such as the oral cavity, larynx, bronchus, breast, and extremities. Localisation of spindle cell lipoma in the orbit has been described in a few cases. We report here on a case of an orbital mass surgically excised in an adult male and discuss the differential diagnosis with other soft tissue tumours with a lipomatous component, such as lipomatous haemangiopericytoma, a rare variant of haemangiopericytoma.

Pseudophakic eye globe disruption.

PURPOSE: Scleral rupture due to bulb bursting can result from a heavy contusion. Owing to refined surgical techniques and the use of antibiotics and cortisone-based medication, more conservative concepts have followed. In major ruptures, the results were almost always very poor, with atrophy or subatrophy of the eyes. METHODS: A 63-year-old man with major left ocular trauma and intraocular lens dislocation in the subconjunctival area was referred to the authors for clinical and surgical evaluation. RESULTS: Surgery was performed 3 weeks after the trauma to allow for improvement in the patient's condition. The reabsorption of a palpebral-frontal hematoma, which made bulb exploration almost impossible, was fundamental in order to proceed. After 1 year, the best-corrected visual acuity was 0.9. In fact, great improvements in surgical techniques in recent years have allowed us to consider the problem of major rupture in a new way, both technically and from an organizational point of view. CONCLUSIONS: In terms of organization, the concept of urgent surgical procedures must be reevaluated, because besides traumatic damage, incomplete surgical measures may result. This makes all treatment useless, in both barely equipped and highly specialized centers.