Cytomegalovirus Uveitis: Taiwan expert consensus.

Cytomegalovirus (CMV) uveitis, a type of herpetic uveitis, is a major cause of infectious uveitis. Anterior and posterior CMV uveitis have diverse clinical presentations and treatment modalities. Based on expert consensus in Taiwan, this article provides suggestions regarding clinical manifestations, diagnosis, and treatment strategies for CMV uveitis based on clinical practice experience in Taiwan. CMV uveitis may have a distinct clinical presentation. Polymerase chain reaction (PCR) is an essential diagnostic tool to confirm a diagnosis. Antiviral therapy is the mainstay of treatment. Different agents, routes, and other supplemental treatments have been summarized and discussed in this article. Early diagnosis and appropriate treatment of CMV uveitis are crucial to avoid irreversible complications and vision loss. This consensus provides practical guidelines for ophthalmologists in Taiwan.

Mutation survey in Taiwanese patients with Stickler syndrome.

PURPOSE: The purpose of this study was to identify gene mutation and phenotype correlations in a cohort of Taiwanese patients with Stickler syndrome. MATERIALS AND METHODS: Patients clinically diagnosed with Stickler syndrome or suspected Stickler syndrome were enrolled. DNA was extracted from venous blood samples. For the targeted next-generation sequencing (NGS) approach, specific primers were designed for all COL2A1, COL11A1, COL11A2, COL9A1, and COL9A2 exons and flanking intron sequences. RESULTS: Twenty-three patients from 12 families were enrolled in this study. The myopia power in these 23 cases (35 eyes) ranged from -4.625 to -25.625 D, with a median of -10.00 D. Four patients had retinal detachment. Fourteen patients had a cleft palate. These 23 patients and 13 healthy controls were enrolled in the NGS study. Three families had significant single nucleotide variants (SNVs) in COL2A1. The mutation rates in this survey were 25% (3/12 families) and 35% (8/23 cases). The SNV of family #1, located at exon 27, c.1753G >T, p. Gly585Val, was novel and has not yet been reported in the ClinVar database. Families #10 and #11 had the same SNV, located in exon 33, c.2101C >T, p. Arg701X. Both variants were classified as likely pathogenic according to the American College of Medical Genetics and Genomics guidelines. CONCLUSION: Genetic mutations in COL2A1 were found in 25% of Taiwanese families with Stickler syndrome. One novel variant was identified using NGS, which expanded the COL2A1 mutation spectrum. Molecular genetic analysis is helpful to confirm the clinical diagnosis of patients with suspected Stickler syndrome.

Case Report: Serial changes of ocular complications related to immune checkpoint inhibitors pembrolizumab and nivolumab.

Background: To demonstrate the rare ocular side effects in a patient receiving pembrolizumab and nivolumab for metastatic ovarian cancer. Case presentation: A 37-year-old woman with recurrent metastatic ovarian cancer presented with blurred vision and photophobia after receiving pembrolizumab. Ocular findings were bilateral anterior chamber reactions, iris cysts, and macular flecks. Optical coherence tomography (OCT) indicated retinal pigment epithelium (RPE) and ellipsoid-band disruption. Her symptoms subsided with topical steroids but fundal appearance persisted despite cessation of immunotherapies. Similar episodes attacked again with multiple exudative subretinal fluid (SRF) developed after she received pembrolizumab and nivolumab. Steroids could cease anterior chamber reactions while SRF only subsided after discontinuation of immunotherapy. Extensive RPE and ellipsoid-band disruption remained without vision improvements. Conclusions: We report a rare case of uveitis and retinopathy after immunotherapies with sequent pembrolizumab and nivolumab. A serial change of the maculopathy is demonstrated. Possible ocular toxicities during the treatment course should be considered, and the benefits of continuing the immunotherapy must be weighed against the risks.

Analysis of tear film spatial instability for pediatric myopia under treatment.

In Taiwan, the prevalence of myopia in children between 6 and 18 years old is over 80%, and high myopia accounts for over 20%, which turned out to be in the leading place worldwide. Orthokeratology and low-dose atropine are proven treatments to reduce myopia progression, though the potential corneal disturbances remain an issue in young populations. The alteration of the tear film is widely discussed but there is no consensus to date, so we aim to investigate the tear film spatial instability in children with myopia control using atropine or orthokeratology. Thirty-eight treatment-naïve participants and 126 myopic children under treatments were enrolled. The ocular surface homeostasis, spatial distribution of tear break-up, and high-order aberrations (HOAs) of the corneal surface were assessed. We found out that myopic children treated with either atropine or orthokeratology showed ocular surface homeostasis similar to that in treatment-naïve children. Nevertheless, children treated with orthokeratology presented higher HOAs (p < 0.00001) and a tendency of the first tear break-up zone at the inner half of the cornea (p = 0.04). This unique spatial instability of the tear film associated with myopia treatment might provide a more focused way of monitoring the pediatric tear film instability.

Determination of Tear Lipid Film Thickness Based on a Reflected Placido Disk Tear Film Analyzer.

This study aims at determining the thickness of the tear lipid layer (LL) observed from a placido-disc-based tear film analyzer. We prospectively collected reflections of placido-disk LL images using a tear film analyzer (Keratograph® 5M, Oculus) from subjects with dry eye symptoms. The LL thickness (LLT) over the inferior half of the cornea was estimated with the use of interference color analysis and the preprocessing of images with and without ring segmentation were obtained and analyzed. Moreover, LLTs before and after 1 h of applying topical ointment (Duratears, Alcon) were compared to validate the estimation of LLT. Our results suggested that the tear LLT can be assessed using a placido-disk-based tear film analyzer and interference color analysis. We verified a high correlation between non-segmented and segmented LL images and estimated LLT increase after applying ointment. In addition, we concluded that LLT can be evaluated by direct interference analysis without segmentation preprocessing.

Defective angles of localized retinal nerve fiber layer reflect the severity of visual field defect- a cross-sectional analysis.

BACKGROUND: In order to detect glaucomatous optic nerve damages early on and evaluate the severity of glaucoma, a previously developed analytic method based on photographic retinal nerve fiber layer (RNFL) angle defect was proposed. However, the correlation between these defective angles and the severity of visual field defect has not been verified. This study aimed to confirm the correlation described above. METHODS: We reviewed a total of 227 glaucomatous eyes (38 enrolled, 189 excluded) during an interval of 5 years. The angles of all eyes were measured on RNFL photograph, of which angle α is the angular width between the macula center and the proximity of RNFL defect, and angle ß (+c) is the sum of angular width(s) of localized RNFL defect. The severity of visual field defect was determined by mean deviation (MD), pattern standard deviation (PSD), and visual field index (VFI). Correlation analysis was performed on angle α and angle ß (+c) with the presence of central scotoma and visual field defect parameters, respectively. RESULTS: Angle ß (+c) showed significant correlation with MD (P = 0.007), PSD (P = 0.02), VFI (P = 0.03), and average RNFL thickness (P = 0.03). No correlation was found between angle α and the presence of central scotoma. CONCLUSIONS: In conclusion, measuring the angular width of localized RNFL defect is a viable method for determining the severity of visual field defect.

To treat or not to treat: a clinical series of retinal arterial macroaneurysms: A single-center retrospective study.

Retinal arterial macroaneurysms (RAMs) develop as outpouchings of the arterial wall that is weakened by arteriosclerosis. The traditional treatment of RAMs comprises observation, focal laser photocoagulation, or surgery. Recently, intravitreal injection of anti-vascular endothelial growth factor (VEGF) drugs has been announced as an effective therapy for fovea-threatening RAMs and quickly improve visual acuity and central retinal thickness (CRT).In the retrospective series, medical charts and ocular images of 24 patients diagnosed as having RAM between May 2011 and November 2018 in our facility were reviewed to delineate clinical manifestations and visual prognosis in RAM patients receiving different treatment modalities. Twenty-four patients (25 eyes; 11 men and 13 women) were enrolled, and one eye with comorbidity of branch retinal vein occlusion was excluded. The mean age of the patients was 69.00 ±â€Š13.45 years. Fourteen patients (58.33%) had a history of hypertension, and 17 patients (70.83%) were aged > 60 years. Furthermore, patients with fovea-threatening RAMs presented with either hypertension or were aged > 60 years.Eyes with fovea involvement (n = 18) were analyzed and separated into two groups according to their treatment modalities: those receiving anti-VEGF intravitreal injections (n = 13) and observation only (n = 5). The baseline visual acuity revealed no significant difference in the two groups. In patients receiving anti-VEGF intravitreal injections, a significantly better visual acuity was detected after anti-VEGF intravitreal injections than the baseline visual acuity (logMAR, 0.78 ±â€Š0.51 vs 1.52 ±â€Š0.48, P < .001), and CRT significantly improved (505.50 ±â€Š159.26 µm vs 243.60 ±â€Š60.17 µm, P = .001). Patients receiving anti-VEGF intravitreal injections also revealed better final visual acuity than those in the observation group (logMAR, 0.78 ±â€Š0.51 vs 1.34 ±â€Š0.48, P = .04).A systematic work-up for hypertension and arteriosclerotic disease could be considered the recommended procedure once RAM has been diagnosed. With better final visual acuity, significant visual improvements, and fast reduction of CRT observed in patients with fovea-threatening RAMs receiving anti-VEGF intravitreal injections, intravitreal anti-VEGF was considered an effective therapy for complicated RAM. During the follow-up period, the majority of RAM eyes had good maintenance of visual function even with foveal complications.

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review.

Background: The diagnosis of overlap syndrome involving systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) is not easily established because of its similar clinical presentations and biochemical features to those of lupus hepatitis. The term overlap syndrome is usually used in the context of overlap of autoimmune hepatitis with PSC (primary sclerosing cholangitis) or PBC (primary biliary cholangitis). Few cases of AIH complicated by SLE have been reported in the literature, and the condition is even rarer in childhood. Case presentation: Here we report the case of a 16-year-old girl with SLE who initially presented with autoimmune (cholestatic) hepatitis. According to American Association for the Study of Liver Diseases practice guidelines, the diagnosis was made based on aggregated scores including female (+2); ALP:AST (or ALT) ratio <1.5(+2); elevated serum IgG level(+3); ANA > 1:80 (+3); negative hepatitis viral markers and drug history (+3, +1); average alcohol intake <25 g/day (+2); and histological interface hepatitis features (+3). She then developed a malar rash, ANA positivity, anti-double-stranded DNA (anti-dsDNA) antibodies, and a low complement level. She met 4 of 17 Systemic Lupus International Collaborating Clinics classification criteria (1) for SLE. Our patient responded very well to corticosteroid at an initial dose of methylprednisolone 40 mg Q12H for 4 days tapering to 1 mg/kg/day according to liver function test results and bilirubin level. No relapse occurred during the 3-year follow-up course. Conclusions: Overlapping of SLE and AIH should be suspected when children with SLE have impaired liver function or AIH patients present with a malar or other skin rash. Liver biopsy plays an important role in establishing the differential diagnosis of SLE with liver impairment or overlap with AIH. The prompt diagnosis and adequate further treatment plans can improve disease outcomes.

Tracking the Reflective Light Particles Spreading on the Cornea: An Emerging Assessment for Tear Film Homeostasis.

PURPOSE: To implement an emerging noninvasive approach for assessing the dynamic tear film (TF) homeostasis. METHODS: The video records of dynamic TF from 12 healthy orthokeratology lens wearers were obtained by a clinically available TF analyzer and decomposed as image sequences. The trajectories of TF particles were analyzed by two tracking models, the full-span model (FSM) and the fixed-duration model (FDM). FSM tracked a particle for a complete opening blink cycle, while FDM tracked 1 second of the same cycle. A power-law fitting operation [Formula: see text] was used to extract homeostasis markers based on the tracking model for each subject. RESULTS: Comparing two tracking models (N = 6), only one subject had statistical difference in averaged momentary moving speed (MMS; P = 0.0488), while none had significant difference in averaged momentary moving direction (MMD). However, both models showed good correlations in average MMS (ρ = 0.94, P = 0.0048) and MMD (ρ = 1.00, P < 0.0001) and all extracted homeostasis markers [α, ß, MMS(0.1), and MMS(2.0)]. Assessing interblink reliability in these markers under FDM tracking (N = 12), only one subject in the MMS (0.1) and another subject in the MMS (2.0) were outside 95% limits of agreement, respectively. CONCLUSIONS: FDM is a good alternative to FSM and has tracking properties of higher efficiency and easier implementation. The homeostasis markers under FDM tracking showed a good interblink consistence; therefore this approach will be a promising method for analyzing dynamic TF homeostasis in future practice. TRANSLATIONAL RELEVANCE: FDM analytical architecture can practice the past experimental platform on a TF analyzer to obtain homeostasis markers of TF.

Corrigendum: Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review.

[This corrects the article DOI: 10.3389/fped.2019.00310.].

Progressive large pediatric corneal limbal dermoid management with tissue glue-assisted monolayer amniotic membrane transplantation: A case report.

RATIONALE: Limbal dermoids are choristomas known as congenital benign tumors found in abnormal locations. Despite the benign nature, enlarging limbal dermoids may cause visual abnormalities by cornea infiltration with fat component, visual axis invasion, gradually induced corneal astigmatism, and finally result in anisometropic amblyopia. Here we report a rare case of progressive, large pediatric corneal limbal dermoid in a newborn, managed with tissue glue-assisted monolayer amniotic membrane transplantation. PATIENT CONCERNS: A 1-day-old male baby (gestational age, 36 ±â€Š6 weeks; birth body weight, 2785 gram) presented to our clinic with a whitish mass on his right eye since birth. DIAGNOSIS: Ocular examination revealed a solid, whitish-yellow, and ovoid mass with central keratinized epithelium over the superior limbus; the lesion covered two-thirds of the cornea with rapid progression in size. The final pathological examination revealed that the lesion is composed of keratotic lining squamous epithelium resembling epidermis, underling dermal fibrotic connective tissue, and mature fat. INTERVENTIONS: The patient underwent deep lamellar excision followed by mitomycin C (MMC) soaking (0.2 mg/mL, 3 minutes) and tissue glue-assisted monolayer amniotic membrane transplantation with the ring conformer at 2 months of age. OUTCOMES: The ring conformer was smoothly removed 2 weeks after the operation. The patient showed a smooth healing process with less pain and rapid corneal re-epithelization. The ocular surface was stable during the follow-up visits, and no complications were detected. Only mild post-operative scarring over the incision wound was observed. LESSONS: Although a combination of excision, lamellar keratoplasty, and multilayer amniotic membrane and limbal stem cell transplantation is advocated for the treatment of grade II and III pediatric corneal limbal dermoids, the procedure used in this study offers an alternative surgical approach. However, because of the large size of the lesion and the young age of the patient, the management of amblyopia with visual rehabilitation and corneal transplantation is still needed in the future.

Meibomian Gland Performance in Glaucomatous Patients With Long-term Instillation of IOP-lowering Medications.

PURPOSE: To investigate the meibomian gland (MG) performance in patients with glaucoma under topical intraocular pressure (IOP)-lowering medications. MATERIALS AND METHODS: This was a cross-sectional case-control study. Patients with glaucoma under different dosages and instillation periods of topical IOP-lowering medications were included. A total of 30 eyes out of 30 healthy participants and 85 eyes out of 85 patients with glaucoma were analyzed. The burden of instilling antiglaucoma agents [burden of antiglaucoma (BAG)] was simply scored for each participant based on the number, formula, frequency, and duration of topical IOP-lowering medications used. All participants completed the MG and tear assessments, including Standard Patient Evaluation of Eye Dryness questionnaire, lipid layer thickness, MG secretion and dropout, Schirmer test, tear break-up time, and blinking patterns. RESULTS: Patients with glaucoma had significantly lower Standard Patient Evaluation of Eye Dryness scores, thinner lipid layer thickness, worse mebium quality, and lower MG secretion compared with healthy participants. Among the patients with glaucoma, MG loss ratio (P=0.006) and meiboscale (P=0.017) were significantly correlated with the BAG score. Compared with the low BAG group (score <80), the high BAG group (score ≥80) had significantly shorter tear break-up time (P=0.047), lower MG density (P=0.032), higher MG loss ratio (P=0.011), and higher meiboscale (P=0.036). CONCLUSIONS: Patients with a higher BAG agents had more unstable tear films and more severe MG dropout. Therefore, MG disease should be particularly observed in patients with glaucoma following a higher BAG regimen.

Idiopathic Acute Transverse Myelitis in Children: A Retrospective Series.

OBJECTIVE: Acute transverse myelitis (ATM) is a focal inflammatory disorder of the spinal cord, resulting in motor, sensory, and autonomic dysfunction. In this study, we delineate the clinical manifestations, neuroimaging characteristics, and outcome-associated risk factors in children with idiopathic ATM. METHODS: We retrospectively reviewed the medical charts and neuroimages in nine children aged younger than 18 years diagnosed with ATM between January 2006 and August 2014. RESULTS: The mean onset age was 5 years and 9 months. Infectious prodromes were observed in six patients. Leg weakness was observed in all patients, autonomic sphincter dysfunction was observed in seven patients, and sensory deficits on admission were observed only in four patients. The diagnosis was delayed in patients younger than 5.5 years compared with older children. The adverse outcomes cannot be predicted by the course of the disease, the laboratory findings, nor the extent of magnetic resonance imaging-detected spinal lesions; however, these outcomes can be predicted by poor early response to corticosteroids and the requirement of additional treatments (p < 0.05). CONCLUSION: The diagnosis of ATM is challenging in young children. Children with ATM who responded early to corticosteroids had more favorable outcomes than those who required additional therapies.