Malignancies in Immunoglobulin G4-related ophthalmic disease.

PURPOSE: Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the patterns of affecting organs have different risks of malignancies. We attempt to determine the association of malignancies with IgG4-related ophthalmic disease (IgG4-ROD). DESIGN: Retrospective cohort study. METHODS: Review of medical records, orbital images and histopathology reports in a territory-wide cohort of biopsy proven IgG4-ROD patients from 2005-2019. FINDINGS: Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n = 108), orbital mass (n = 30), infiltrated orbital fat (n = 10), conjunctiva (n = 2) or extraocular muscles (n = 3), 13% (16/122) developed malignancies over 73 ± 48months' follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs = 10.0, 95%CI = 4.5-17.6) while that of extra-orbital malignancies was similar. The SIRs was highest within the first year (SIR = 46.7, 95%CI = 18.5-87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients at IgG4-ROD diagnosis (64.9 ± 7.1, 68.3 ± 8.5 versus 55.2 ± 15.0 years, P < 0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%) were associated with OAL (all P < 0.05). Pre-treatment serum IgG4 level or extra-orbital IgG4-RD involvement was similar among patients with or without malignancies. CONCLUSION: In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.

Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study.

BACKGROUND: Oral corticosteroid remains the first-line treatment of IgG4-related ophthalmic disease, but steroid-dependence is common and serious. Factors associated with steroid dependence and relapse have to be further explored. STUDY POPULATION: A city-wide, biopsy-proven, Chinese cohort. METHODS: Retrospective, masked review of medical records, orbital images and histopathology reports. RESULTS: There were 101 patients with at least 24-month follow-up. Up to 82% (82/101) received oral corticosteroid as first-line treatments, and 7 of them received also concomitant steroid-sparing agents (SSA)/biological agents as primary treatment. There was 61% (50/82) of patients required long-term corticosteroid (alone=23, with SSA=27) after 1.9±0.7 (range 1-5) relapses. When compared with the 21% (17/82) of patients who tapered corticosteroid successfully for 24 months, steroid dependence was associated with elevated baseline serum IgG4 level (94% vs 65%, p<0.01) and Mikulicz syndrome (46% vs 18%, p<0.05). Up to 13% (11/82) of patients tolerated residual disease after tapering off corticosteroid. There was 17% (17/101) of patients did not require any medications after biopsies. They were more likely to have debulking surgeries (71% vs 40%, p<0.05), discrete orbital lesions (65% vs 26%, p<0.05), normal baseline serum IgG4 level (24% vs 6%, p<0.05) and no Mikulicz syndrome (94% vs 61%, p<0.05). CONCLUSION: In this cohort, 60% of patients required long-term maintenance oral corticosteroid. Elevated pretreatment serum IgG4 level and Mikulicz syndrome were associated with steroid dependence. Debulking surgery is an alternative for a subgroup of patients with discrete orbital lesions, normal baseline IgG4 level and no Mikulicz syndrome.

Radiological Determinants of Complicated Immunoglobulin G4-Related Ophthalmic Disease: A Territory-Wide Cohort Study.

PURPOSE: To evaluate the presenting radiological features of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). METHODS: A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. RESULTS: A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. CONCLUSIONS: In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.

14-year case series of eyelid sebaceous gland carcinoma in Chinese patients and review of management.

AIMS: Sebaceous gland carcinoma (SGC) of the eyelid is a rare but potentially deadly cancer. The purpose of this study was to determine whether the American Joint Committee on Cancer (AJCC) 7th Edition T category for SGC correlated with metastasis and survival in the Chinese population. METHODS: This was a retrospective, single-centre cohort study. Patients with surgically resected eyelid SGC between January 2001 and May 2015 at the Hong Kong Eye Hospital were reviewed. Tumours were staged using the AJCC criteria. The main outcome measures included local recurrence, metastasis and death. Disease-free survival (DFS) was measured from the completion of treatment; overall survival was measured from the date of initial diagnosis. RESULTS: The study included 22 Chinese patients with a mean age of 65.4 years. The majority presented as a nodular lesion (91%) with 12 eyes (54.5%) initially misdiagnosed and a mean presentation time of 1 year. It was found that those with AJCC stage T2b or higher were significantly associated with lymph node metastasis (P=0.002) when compared with those with stage T2a. Older age at diagnosis (P=0.035) and no misdiagnosis (P=0.025) were associated with shorter DFS. Those with stage 3a or higher were associated with shorter DFS (P=0.007) and overall survival (P=0.024). CONCLUSION: Similar to previous reports, in this Chinese cohort, AJCC staging for SGC correlated with lymph node metastasis, DFS and overall survival. Those with stage 2b or higher on presentation will need closer surveillance for lymph node metastasis and may benefit from sentinel lymph node biopsy.

Prevalence of Ocular Manifestations and Visual Outcomes in Patients With Herpes Zoster Ophthalmicus.

PURPOSE: To investigate the prevalence of ocular manifestations and visual outcomes in patients with herpes zoster ophthalmicus (HZO). METHODS: Consecutive cases diagnosed with HZO who attended 2 hospitals between July 1, 2011, and June 30, 2015, were retrospectively reviewed. Patient demographics, clinical presentations, and management were reviewed. The logistic regression model was used to estimate the odds ratio of visual loss with ocular manifestations. RESULTS: A total of 259 patients were included. Of these, 110 (42.5%) patients were <60 years old and 149 patients (57.5%) were ≥60 years old. None of the patients had received zoster vaccination before presentation. Ocular manifestations were present in 170 (65.6%) patients with no difference between both age groups (P = 0.101). Conjunctivitis was the most common ocular manifestation, followed by anterior uveitis and keratitis. After resolution of HZO, 58.7% of patients had a visual acuity of 6/12 or worse. Epithelial keratitis and stromal keratitis were independent risk factors for visual loss after resolution of HZO (P = 0.003 and P = 0.004, respectively). The corresponding odds ratio was 6.59 [95% confidence interval (CI): 1.87-23.19] and 7.55 (95% CI: 1.88-30.30), respectively. The number of ocular manifestations was also associated with an increased risk of visual loss with an odds ratio of 1.49 (95% CI: 1.01-2.20; P = 0.043). CONCLUSIONS: A substantial proportion of patients with HZO were <60 years old in this study. The absence of zoster vaccination across the study cohort was noteworthy. Keratitis was the main reason for poor visual outcome in these patients.

Change in Tear Film Lipid Layer Thickness, Corneal Thickness, Volume and Topography after Superficial Cauterization for Conjunctivochalasis.

We evaluated the change in tear film lipid layer thickness, corneal thickness, volume and topography after superficial cauterization of symptomatic conjunctivochalasis. Bilateral superficial conjunctival cauterization was performed in 36 eyes of 18 patients with symptomatic conjunctivochalasis. The mean age of patients (12 males, 6 females) was 68.6 ± 10.9 years (range: 44-83 years). Preoperatively, 28 eyes (77.8%) had grade 1 conjunctivochalasis, and 8 eyes (22.2%) had grade 2 conjunctivochalasis. At 1 month postoperatively, the severity of conjunctivochalasis decreased significantly (p < 0.001) and 29 eyes (80.6%) had grade 0 conjunctivochalasis whereas 7 eyes (19.4%) had grade 1 conjunctivochalasis. The mean Ocular Surface Disease Index score decreased from 31.5 ± 15.2 preoperatively to 21.5 ± 14.2 at the end of 1 month postoperatively (p = 0.001). There was a statistically significant increase in mean tear film lipid layer thickness 1 month after the surgery (49.6 ± 16.1 nm vs 62.6 ± 21.6 nm; p < 0.001). The central corneal thickness, thinnest corneal thickness and corneal volume decreased significantly postoperatively (p < 0.001). Our study showed that superficial conjunctival cauterization is an effective technique for management of conjunctivochalasis in the short term. An increase in tear film lipid layer thickness along with a decrease in corneal thickness and volume were observed after surgical correction of conjunctivochalasis.

Adenoviral keratoconjunctivitis.

Viral conjunctivitis caused by adenovirus is the most common infectious conjunctivitis. Adenoviruses are highly contagious pathogens. The modes of transmission are mainly through hand to eye contact, ocular secretions, respiratory droplets, and contact with ophthalmic care providers and their medical instruments. The most frequent manifestation of ocular adenoviral infection is epidemic keratoconjunctivitis, followed by pharyngoconjunctival fever. Epidemic keratoconjunctivitis is also the most severe form and presents with watery discharge, hyperemia, cheosis, and ipsilateral lymphadenopathy. Pharyngoconjunctival fever is characterized by abrupt onset of high fever, pharyngitis, bilateral conjunctivitis, and periauricular lymph node enlargement. Isolated follicular conjunctivitis without corneal or systemic involvement also occurs. The rate of clinical accuracy in diagnosing viral conjunctivitis is less than 50%. Rapid diagnostic tests now being used decrease unnecessary antibiotic use. Treatment for viral conjunctivitis is mostly supportive. The majority of cases are self-limited, and no treatment is necessary in uncomplicated cases.

Twelve-Year Outcomes of Pterygium Excision with Conjunctival Autograft versus Intraoperative Mitomycin C in Double-Head Pterygium Surgery.

Purpose. The study aims to compare the long-term outcome of conjunctival autograft (CAU) and mitomycin C (MMC) in double-head pterygium surgery. Methods. This is a follow-up study of a comparative interventional trial. Thirty-nine eyes of the 36 patients with double-head pterygium excision in the original study 12 years ago were recruited for clinical assessment. Seven out of the 36 patients were lost. In the original study, each eye with double-head pterygium was randomized to have pterygium excision with CAU on one "head" (temporal or nasal) and MMC on the other "head." All patients were invited for clinical assessment for conjunctival bed status and the presence of pterygium recurrence in the current study. Results. There was no significant difference between the size, morphology, and type of pterygium among the two treatment groups. The recurrence rate of CAU group and MMC group 12 years after excision was 6.3% and 28.1%, respectively (P = 0.020). Among eyes without recurrence, the conjunctival bed was graded higher in the MMC group than the CAU group (P = 0.024). Conclusion. The use of conjunctival autograft has a significantly lower long-term recurrence rate than mitomycin C in double-head pterygium surgery.

Comparison of outcomes of primary anterior chamber versus secondary scleral-fixated intraocular lens implantation in complicated cataract surgeries.

PURPOSE: To compare the outcome of primary anterior chamber vs secondary scleral-fixated intraocular lens (IOL) implantation in complicated cataract surgeries. DESIGN: Retrospective, comparative study. METHODS: A consecutive series of complicated cataract surgeries with primary anterior chamber (ACIOL) or secondary scleral-fixated IOL implantation from January 1, 2004 to December 31, 2009 was analyzed. Main outcome measures included the postoperative best-corrected visual acuity (BCVA) and postoperative complications. RESULTS: There were 89 eyes in the primary ACIOL group and 74 eyes in the secondary scleral-fixated IOL group. The mean follow-up duration was 64.1 ± 36.7 months. The mean postoperative logarithm of minimal angle of resolution (logMAR) BCVA at 1 year was 0.32 ± 0.54 and 0.34 ± 0.21 in the primary ACIOL group and the secondary scleral-fixated IOL group, respectively (P = .734). The mean latest logMAR BCVA was 0.68 ± 0.54 and 0.61 ± 0.47 in the primary ACIOL group and the secondary scleral-fixated IOL group, respectively (P = .336). The primary ACIOL group had more early postoperative complications (P < .001). No difference in late postoperative complications was observed between the 2 groups (P = .100). Regression analysis showed that primary ACIOL and secondary scleral-fixated IOL implantation had similar latest postoperative logMAR BCVA (P = .927), while the presence of late complications was associated with a worse final visual outcome (P = .000). CONCLUSIONS: This study shows that there are no long-term differences in the visual outcomes and complication profiles after primary ACIOL or secondary scleral-fixated IOL implantation in a complicated cataract operation when capsular support is inadequate.

Application of anterior segment optical coherence tomography to identify eyes with posterior polar cataract at high risk for posterior capsule rupture.

PURPOSE: To use anterior segment optical coherence tomography (AS-OCT) to identify eyes with posterior polar cataract at high risk for posterior capsule rupture (PCR) during cataract extraction. SETTING: Hong Kong Eye Hospital, Hong Kong, China. DESIGN: Retrospective case series. METHODS: Eyes with clinically diagnosed posterior polar cataract that had AS-OCT imaging and phacoemulsification were included. Each eye was graded according to the features of the posterior lens opacity and the underlying capsule. Eyes were categorized as having grade 1 or grade 2 cataract depending on the amount of clearance between the posterior opacity and the capsule (≥ 50% clearance and <50% clearance, respectively). Grade 3 represented the absence of an intact posterior capsule. The incidence of intraoperative PCR was compared with the AS-OCT grading to identify eyes at high risk for PCR. RESULTS: The study assessed 37 eyes, 19 with grade 1 cataract, 13 with grade 2, and 5 with grade 3. Posterior capsule rupture occurred in 8 eyes (21.6%), 1 (5.3%) with grade 1 cataract, 4 (30.8%) with grade 2, and 3 (60.0%) with grade 3. The PCR rate was statistically significantly different between the groups (P = .018). When grade 2 cases and grade 3 cases were combined, AS-OCT had a sensitivity of 87.5% and specificity of 62.1%. CONCLUSIONS: Anterior segment OCT can be used to grade posterior polar cataracts and identify eyes at high risk for PCR, allowing better surgical planning and preoperative counseling.

Orthokeratology-associated infectious keratitis in a tertiary care eye hospital in Hong Kong.

PURPOSE: To analyze cases of orthokeratology-associated infectious keratitis managed in a tertiary care eye hospital in Hong Kong between 2003 and 2013. DESIGN: Retrospective study. METHODS: Case records of patients with infectious keratitis attributable to orthokeratology contact lenses were analyzed. Data analyzed included clinical features, microbiological evaluation, and treatment outcomes. RESULTS: A total of 23 patients were included (16 female, 7 male, mean age: 15.0 ± 4.2 years; range: 9-23 years). All patients were using overnight orthokeratology for an average of 2.7 ± 2.8 years (range: 3 months - 10 years) before the onset of infection. Clinical features included corneal infiltrate (n = 14, 60.9%) and corneal perineuritis (n = 12, 52.2%). Fifteen eyes (65.2%) had a positive microbiological culture obtained from corneal scrapings. The most commonly isolated organism was Pseudomonas aeruginosa (n = 6), followed by coagulase-negative Staphylococcus (n = 5) and Acanthamoeba (n = 3). Five cases of Pseudomonas aeruginosa and 5 cases of Acanthamoeba were identified from contact lenses or contact lens solution. The mean duration from disease onset to remission was 31.9 ± 34.9 days (range: 6-131 days). All patients responded to medical treatment, and no emergency surgical intervention was needed. The best-corrected logMAR visual acuity improved significantly from 0.62 ± 0.51 (20/83 Snellen) to 0.15 ± 0.20 (20/28 Snellen) (Wilcoxon signed rank test, P < .001). CONCLUSIONS: Orthokeratology-associated infectious keratitis continues to be a serious problem, especially in regions with high prevalence of myopia. Early clinical and microbiological diagnosis and intensive treatment can improve final visual outcomes.

Descemet stripping endothelial keratoplasty: effect of the surgical procedure on corneal optics.

PURPOSE: To determine the changes in corneal optical performance after posterior lamellar corneal transplantation. DESIGN: Retrospective case series. METHODS: The anterior segment in four eyes of four patients who underwent Descemet stripping endothelial keratoplasty (DSEK) with cataract extraction and intraocular lens (IOL) implantation were imaged with the Visante anterior segment optical coherence tomography [OCT] (Carl Zeiss Meditec, Dublin, California, USA). The curvature of the posterior surface of the donor graft was compared with that of the host cornea, and corneal thickness was measured. RESULTS: All eyes had a hyperopic refractive error after surgery. The posterior corneal curvature after surgery was more than that before surgery. Average preoperative keratometry was 43.4 diopters (D), and after surgery, it was 42.8 D using keratometry. However, when the postsurgical corneal power was calculated using the Gaussian optics method, the average value was 40.8 D. CONCLUSIONS: The addition of a donor corneal graft to the posterior surface of decompensated corneas may lessen the effective optical power of the cornea and may have implications for IOL power calculations in these eyes.

Repeatability and reproducibility of pachymetric mapping with Visante anterior segment-optical coherence tomography.

PURPOSE: To determine the repeatability and reproducibility of central and peripheral corneal pachymetry mapping with anterior segment-optical coherence tomography (AS-OCT). METHODS: An observational cross-sectional study involving two groups: 27 healthy eyes and 20 eyes with keratoconus. Each subject underwent scanning sessions with AS-OCT to determine intraobserver repeatability, interobserver reproducibility, and additionally for healthy eyes, intersession reproducibility for different regions of the cornea up to a 10-mm diameter. Main outcome measures were reproducibility and repeatability coefficients, intraclass correlation coefficients, and coefficients of variation of the average central (0-2 mm), pericentral (2-5 mm), transitional (5-7 mm), and peripheral (7-10 mm) corneal thicknesses generated by the Visante AS-OCT (Carl Zeiss Meditec, Inc., Dublin, CA) pachymetric mapping protocol. RESULTS: The coefficients of repeatability were less than 2% in healthy subjects and less than 3% in patients with keratoconus. The reproducibility coefficients were less than 2% and 4% in healthy subjects and patients with keratoconus, respectively. There was no significant difference between scans obtained by different observers or during different visits. The intraclass correlation coefficients were greater than 0.99 and 0.97 in healthy subjects and patients with keratoconus, respectively. CONCLUSIONS: With the pachymetric mapping protocol of Visante AS-OCT, these results suggest that central and peripheral corneal thickness measurements in healthy subjects and in eyes with keratoconus are repeatable and reproducible.

A case series of contact lens-associated Fusarium keratitis in Hong Kong.

PURPOSE: To report the clinical features and management outcomes of a cluster of Fusarium keratitis in patients that used the Bausch & Lomb ReNu MostureLoc contact lens solution. METHODS: Retrospective case series. RESULTS: In a 1-year period starting from June 2005, we treated 12 patients with unilateral Fusarium keratitis in our tertiary care center. All patients were contact lens users that used ReNu MostureLoc contact lens solution and had no other specific predisposing conditions. Microbiological examination yielded growth of Fusarium spp. in 7 patients from corneal scrapings at presentation and from 3 patients in subsequent corneal specimens. For 2 other patients, fungi were not detected from corneal scrapings, but Fusarium spp. were isolated from their contact lenses. The infections were treated with topical natamycin and amphotericin B eye drops and with systemic itraconazole in 8 patients. The infection resolved with medical treatment in 8 eyes, a conjunctival flap in 1 eye, and a therapeutic corneal graft in 1 eye. Two eyes required tectonic corneal grafts for perforation. Two of the 3 corneal grafts failed because of graft rejection. Final visual acuities ranged from count fingers to 1.0. CONCLUSIONS: This cluster of Fusarium keratitis seems to be related to the use of the ReNu MoistureLoc contact lens solution. The cure rate with medical therapy was 66%. However, corneal scarring limited visual recovery. This episode highlights the need for clinical vigilance when dealing with corneal infiltrates in contact lens users.

Agreement among 3 methods to measure corneal thickness: ultrasound pachymetry, Orbscan II, and Visante anterior segment optical coherence tomography.

PURPOSE: To assess the agreement of central and paracentral corneal thickness measurements between ultrasound pachymetry (USP), Orbscan II, and Visante anterior segment optical coherence tomography (ASOCT). DESIGN: Observational cross-sectional study. PARTICIPANTS: Seventy eyes of 70 subjects. METHODS: Each subject underwent Orbscan II (using an acoustic equivalent correction factor of 0.89), ASOCT, and USP examination. Bland-Altman plots were used to evaluate agreement between instruments. MAIN OUTCOME MEASURES: Central and paracentral corneal thickness measurements by the 3 methods and agreement, as evaluated by 95% limits of agreement (LOA). RESULTS: The mean measurements of average central corneal thickness by USP, Orbscan II, and ASOCT were 553.5+/-30.26 microm, 553.22+/-25.47 microm, and 538.79+/-26.22 microm, respectively. There was high correlation between instruments: USP with ASOCT (r = 0.936, P<0.001), USP with Orbscan II (r = 0.900, P<0.001) for central corneal thickness measurements, and Orbscan II with ASOCT for average paracentral 2- to 5-mm measurements (r = 0.947, P<0.001). The mean differences (and upper/lower LOA) for central corneal thickness measurements were 0.31+/-13.34 microm (26.44/-25.83) between USP and Orbscan II, 14.74+/-10.84 microm (36.0/-6.51) between USP and ASOCT, and 14.44+/-9.14 microm (32.36/-3.48) between Orbscan II and ASOCT. The average mean difference (and upper/lower LOA) between Orbscan II and ASOCT for paracentral 2- to 5-mm corneal thickness measurements was 10.35+/-8.67 microm (27.35/-6.65). CONCLUSION: Anterior segment optical coherence tomography underestimated corneal thickness compared with that measured with USP. Anterior segment optical coherence tomography had better agreement with the gold standard USP, as compared with Orbscan II. However, important discrepancies among instruments exist. Clinicians should be aware that corneal thickness measurements are influenced by the method of measurement and that, although highly correlated, these instruments should not be used interchangeably for the assessment of corneal thickness.