Author Correction: Determinants of Quantitative Optical Coherence Tomography Angiography Metrics in Patients with Diabetes.

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Determinants of Quantitative Optical Coherence Tomography Angiography Metrics in Patients with Diabetes.

Early microvascular damage in diabetes (e.g. capillary nonperfusion and ischemia) can now be assessed and quantified with optical coherence tomography-angiography (OCT-A). The morphology of vascular tissue is indeed affected by different factors; however, there is a paucity of data examining whether OCT-A metrics are influenced by ocular, systemic and demographic variables in subjects with diabetes. We conducted an observational cross-sectional study and included 434 eyes from 286 patients with diabetes. Foveal avascular zone (FAZ) area, FAZ circularity, total and parafoveal vessel density (VD), fractal dimension (FD), and vessel diameter index (VDI) from the superficial capillary plexus OCT-angiogram were measured by a customized automated image analysis program. We found that diabetic retinopathy (DR) severity was associated with increased FAZ area, decreased FAZ circularity, lower VD, lower FD, and increased VDI. Enlarged FAZ area was correlated with shorter axial length and thinner central subfield macular thickness. Decreased FAZ circularity was correlated with a reduction in visual function. Decreased VD was correlated with thinner macular ganglion-cell inner plexiform layer. Increased VDI was correlated with higher fasting glucose level. We concluded that the effects of ocular and systemic factors in diabetics should be taken into consideration when assessing microvascular alterations via OCT-A.

A Multicenter Study on the Long-term Outcomes of Half-dose Photodynamic Therapy in Chronic Central Serous Chorioretinopathy.

PURPOSE: To investigate long-term efficacy and prognostic factors of half-dose photodynamic therapy (PDT) in chronic central serous chorioretinopathy (CSCR). DESIGN: Retrospective multicenter interventional case series. METHODS: Patients with chronic CSCR undergoing half-dose PDT between 2005 and 2011 were reviewed. Main outcome measures included resolution of serous retinal detachment (SRD) with single PDT, change in best-corrected visual acuities (BCVAs), and recurrence rate of CSCR at 36 months after PDT. Prognostic factors of visual outcome and recurrence of CSCR after PDT were identified with multivariate regression analysis. RESULTS: A total of 136 eyes of 123 patients were followed up for 57.7 ± 16.2 months. At 36 months after PDT, 132 eyes (97.1%) achieved complete resolution of SRD with single PDT and 4 eyes (2.9%) had CSCR recurrence. The mean logMAR BCVA improved from 0.36 ± 0.29 (Snellen equivalent 20/46; range: 0.1-1.2) at baseline to 0.15 ± 0.23 at 36 months (Snellen equivalent 20/28; range: 0.1-1.5; P < .001) and 0.16 ± 0.24 (Snellen equivalent: 20/29; range: 0.1-1.5; P < .001) at final follow-up. Forty-four eyes (32.4%) had ≥3 lines of BCVA gain while 5 eyes (3.7%) had ≥3 lines of BCVA loss at 36 months after PDT. Nine eyes (6.6%) developed CSCR recurrence at final follow-up. Baseline BCVA was significantly associated with the BCVA (P = .009) and the improvement in BCVA (P < .001) at final follow-up. History of bilateral CSCR was significantly associated with CSCR recurrence at final follow-up (P = .036; odds ratio = 15.84, 95% confidence interval = 1.20-208.32). Eight eyes (5.9%) had complications related to PDT. CONCLUSIONS: Chronic CSCR patients treated with half-dose PDT can achieve long-term stable visual acuity and resolution of SRD. Patients with chronic CSCR are recommended to undergo half-dose PDT before they have significant visual deterioration. Patients with bilateral CSCR are more likely to develop CSCR recurrence after half-dose PDT.

Use of intravitreal dexamethasone implant in patients with cataract and macular edema undergoing phacoemulsification.

PURPOSE: To study the safety and efficacy of intravitreal dexamethasone implant in patients with cataract and macular edema undergoing phacoemulsification and intraocular lens (IOL) implantation. METHODS: Twenty-four eyes with macular edema secondary to diabetic retinopathy (diabetic macular edema [DME]) and retinal vein occlusion (RVO) were retrospectively reviewed. These eyes underwent phacoemulsification with IOL implantation and intravitreal dexamethasone implant 0.7 mg at the same setting between September 2012 and September 2013. Demographic data, best-corrected visual acuity (BCVA), central macular thickness (CMT), intraocular pressure (IOP), surgical time, and complications were recorded. RESULTS: Twelve eyes had DME and 12 eyes had RVO (10 central RVO and 2 branch RVO). Median baseline logMAR BCVA was 1.0 (Snellen 20/200) and mean baseline CMT was 530.2 ± 218.9 µm. Median follow-up duration was 13 months. At last follow-up, median visual acuity improved significantly to 0.523 (Snellen 20/66) (p = 0.003) and CMT decreased to 300.7 ± 78.1 µm (p = 0.000). Median surgical time was 23 minutes. There were no intraoperative complications. In 12 eyes, macular edema recurred, requiring further treatment, and median time to recurrences was 21 weeks. One eye had raised IOP after second dexamethasone implant for recurrent macular edema. No major complication such as vitreous hemorrhage, retinal detachment, or endophthalmitis occurred. CONCLUSIONS: Combined cataract surgery with intravitreal dexamethasone implant seems to be safe and effective in treating patients with cataract and macular edema in this small case series. A larger prospective study with longer follow-up is necessary to demonstrate the long-term benefit of this combined procedure.

A case of giant nodular posterior scleritis mimicking choroidal malignancy.

To report a case of giant nodular posterior scleritis mimicking a choroidal tumor. A 42-year-old lady with systemic hypertension presented with a 1-week history of unilateral visual loss, pain and redness in her left eye. Examination showed sectoral anterior episcleritis in her left eye as well as a dome-shaped choroidal mass at the inferior-temporal periphery, associated with retinal hemorrhages and subretinal fluid. Systemic evaluation and imaging of the choroidal mass were performed and could not rule out amelanotic choroidal melanoma. At the same time, she was prescribed a 2-week course of oral nonsteroidal anti-inflammatory drug (NSAID) for her sectoral anterior episcleritis. The choroidal mass was found to have resolved completely right before her scheduled fine needle biopsy. Diagnosis of nodular posterior scleritis and a trial of oral NSAID can be considered in patients presenting with a choroidal mass before any invasive procedure.

Influence of molecular genetics in Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease is a systemic autoimmune disorder against melanocytes. Recent studies have identified multiple genetic factors that might be associated with the pathogenesis of VKH disease. We performed an electronic database search of PubMed, MEDLINE, and EMBASE, and all relevant papers published up to 13 June 2014 were reviewed. A total of 1,031 publications including articles relevant to the genetics of VKH disease and the references of these articles were reviewed. The review identified a number of genetic factors which might be involved in the pathogenesis of VKH disease, some of which may alter the clinical course of VKH disease. Genes which might be involved in the pathogenesis of VKH disease included genes expressing HLA, complement factor H, interleukins, cytotoxic T-lymphocyte antigen 4 (CTLA-4), killer cell immunoglobulin-like receptors (KIR), programmed cell death 1 (PDCD1), protein tyrosine phosphatase non-receptor 22 (PTPN22), osteopontin, tumor necrosis factor alpha-induced protein 3 (TNFAIP3), macrophage migration inhibitory factor (MIF), and other immune response genes. Further studies to explore the correlation among different genotypes and phenotypes of VKH disease will be useful to shed light on the pathogenesis of uveitis in VKH disease and may facilitate the development of new treatment modalities of uveitis in VKH disease.

The roles of genetic factors in uveitis and their clinical significance.

BACKGROUND: Uveitis is a diverse group of intraocular inflammatory disease and is a significant cause of visual loss worldwide. Recent studies have identified various endogenous immune mechanisms and genetic factors that are involved in the pathogenesis of uveitis. This review provides an overview on the role of genetics in the development and clinical course of uveitis. METHODS: PUBMED was used for literature search, and articles published from 1970 to 2012 that evaluated the genetic associations and mechanisms involved in the development and clinical features of uveitis were included. RESULTS: Studies have demonstrated associations between various genetic factors and the development and clinical course of intraocular inflammatory conditions. Genes involved included genes expressing interleukins, chemokines, chemokine receptors, and tumor necrosis factor and genes involved in complement system, oxidation, and other intracellular molecular pathways. CONCLUSION: Multiple genetic factors play important roles in the pathogenesis of uveitis and may influence the clinical course of uveitis. Further studies to investigate the genetic mechanisms of uveitis might identify additional genetic associations and might have the potential for identifying novel therapeutic targets in the treatment of intraocular inflammation.

A case of dengue maculopathy with spontaneous recovery.

PURPOSE: To report a case of dengue maculopathy, which resolved spontaneously without treatment. METHODS: A 25-year-old female patient with good past health was admitted to a general hospital in Hong Kong with fever of unknown origin after traveling to Indonesia. Based on the clinical features and a positive real-time polymerase chain reaction for dengue virus type 1, she was diagnosed with dengue fever. The patient developed dengue macu-lopathy mainly affecting the vision of her left eye. Abnormalities on a multifocal electro-retinogram showed bilateral involvement. RESULTS: As there is no proven treatment for dengue maculopathy, the patient opted for observation. Her vision returned to normal within 3 weeks. CONCLUSION: Dengue maculopathy can cause severe visual loss and may resolve without treatment.

Clinical characteristics of intermediate uveitis in Chinese patients.

PURPOSE: To describe the clinical characteristics of Chinese patients with intermediate uveitis (IU). METHODS: Retrospective review of patients with IU with at least 6 months follow-up. RESULTS: Seventy patients were identified and the mean follow-up was 59.7 months. The mean age at presentation was 33.1 years. Fifteen (21.4%) patients had disease onset before the age of 18 years; 91.4% of IU cases were considered idiopathic after investigations. At the last follow-up, 85 (74.6%) eyes retained vision of at least 20/40. Poor visual outcome was significantly associated with poor presenting visual acuity (p = .002), presence of epiretinal membrane or atrophic macular changes (p = .003), persistent cystoid macular edema (p = .015), and increased disease duration (odds ratio = 1.015 per month, p = .002). Pediatric patients were more likely to have bilateral (p = .003) and chronic disease (p < .001). CONCLUSIONS: IU in Chinese patients was mainly idiopathic, with good visual outcomes in most patients after appropriate treatment.

Complement factor H and interleukin gene polymorphisms in patients with non-infectious intermediate and posterior uveitis.

OBJECTIVE: To investigate the associations of complement factor H (CFH), KIAA1109, and interleukin-27 (IL-27) gene polymorphisms in patients with non-infectious intermediate and posterior uveitis. METHODS: The study cohort consisted of a total of 95 Chinese non-infectious uveitis patients, including 38 patients with intermediate uveitis (IU), 38 patients with Vogt-Koyanagi-Harada disease (VKH), and 19 patients with Behçet's disease and 308 healthy controls. The genotypes of CFH-rs800292, KIAA1109-rs4505848, and IL27-rs4788084 were determined using TaqMan single nucleotide polymorphism genotyping assays. RESULTS: The frequency of carriers of G allele for CFH-rs800292 was significantly higher in patients with non-infectious intermediate and posterior uveitis than in controls (GG/AG versus AA; p=0.02). No significant association was found between uveitis and both KIAA1109-rs4505848 and IL27-rs4788084. In stratified analysis by gender, the frequency of carriers with G allele for KIAA1109-rs4505848 was significantly higher in male uveitis patients than in male controls (GG/AG versus AA; p=0.034). There was no significant difference in allelic and genotypic frequencies for CFH-rs800292 and IL27-rs4788084 in either male or female groups. In addition, higher frequency of KIAA1109-rs4505848 G allele was found in Behçet's disease patients compared with controls and IU patients (p=0.01 and p=0.003, respectively). CONCLUSIONS: Our results demonstrated that CFH-rs800292 and KIAA1109-rs4505848 are associated with non-infectious intermediate and posterior uveitis. Moreover, gender susceptibility for uveitis might be involved in the KIAA1109 gene and the KIAA1109-rs4505848 polymorphism might be associated with the development of Behçet's disease.

Review of Clinical Features, Microbiological Spectrum, and Treatment Outcomes of Endogenous Endophthalmitis over an 8-Year Period.

Purpose. To evaluate the clinical features, microbiological spectrum, and treatment outcomes of endogenous endophthalmitis. Methods. Retrospective review of consecutive cases with infective endogenous endophthalmitis presenting from 2000 to 2007. The main outcome measure was the visual outcome at the latest follow-up visit. Other outcome measures included microbiological investigations, anatomical and clinical outcomes. Results. 22 eyes of 21 patients were included, and the mean follow-up duration was 2.7 years. Eyes with fungal endogenous endophthalmitis were more likely to have visual acuity of finger counting or better at presentation compared with those with bacterial endogenous endophthalmitis (odds ratio = 15.0, P = 0.013). Gram-negative microorganisms accounted for 50% of infections, while fungal and gram-positive organisms accounted for 27.3% and 22.7%, respectively. Despite treatment, the visual outcome was poor in general as 10 (45.5%) eyes had no light perception at the latest follow-up visit and 6 (27.3%) eyes required enucleation or evisceration. Contrary to previous studies, fungal endogenous endophthalmitis did not appear to have better visual outcome compared with bacterial endogenous endophthalmitis. Conclusion. Gram-negative microorganisms were the main causative pathogens of endogenous endophthalmitis in Hong Kong. The visual prognosis of endogenous endophthalmitis is generally poor as almost 50% of eyes were blind despite treatment.

CFH 184G as a genetic risk marker for anterior uveitis in Chinese females.

OBJECTIVE: To investigate the association of three single nucleotide polymorphisms (SNPs) in the complement factor H (CFH), KIAA1109, and interleukin-27 (IL-27) genes in patients with anterior uveitis (AU). METHODS: A case-control study was performed in 98 Chinese AU patients and 308 healthy controls. Three SNPs including CFH-rs800292, KIAA1109-rs4505848, and IL27-rs4788084 were detected using TaqMan SNP Genotyping Assays. Analyses were also stratified according to gender, clinical features and human leukocyte antigen (HLA)-B27 status of the patients. RESULTS: No significant association was found between all three SNPs and AU. However, when stratified by gender, there were significant increases in the frequency of the CFH-rs800292 184G allele and GG homozygosity in female patients compared with control subjects (p=0.003 and p=0.009, respectively). Similar association was not detected in males. No significant association was found between AU and KIAA1109-rs4505848 or IL27-rs4788084 even stratified by gender. There was no significant difference in genotypes of AU patients stratified by various clinical features. Subgroup analyses showed that all three SNPs (rs800292, rs4505848, and rs4788084) were not associated with AU in HLA-B27-positive patients, neither in HLA-B27-negative patients. CONCLUSIONS: Our results showed an association between AU and CFH polymorphism in Chinese female patients but not in males, indicating gender-specific genetic differences in CFH. Gender should be considered in genetic studies of anterior uveitis even extending to other immunologic diseases.

Intravitreal ranibizumab with or without photodynamic therapy for the treatment of symptomatic polypoidal choroidal vasculopathy.

PURPOSE: To evaluate the efficacy of intravitreal ranibizumab with or without verteporfin photodynamic therapy (PDT) in the treatment of symptomatic polypoidal choroidal vasculopathy. METHODS: Twenty-three eyes of 23 patients received 3 monthly intravitreal ranibizumab injections with or without indocyanine green angiography-guided PDT at baseline. All patients had follow-up of ≥12 months. Visual and anatomical outcomes were compared between the two groups and a PDT monotherapy group. RESULTS: Seven eyes had ranibizumab monotherapy, 16 had combined ranibizumab injections and verteporfin PDT, and 12 had PDT monotherapy. At 3 months, the mean logarithm of minimal angle of resolution best-corrected visual acuity improved from 0.92 to 0.74 in the ranibizumab group (P = 0.18), from 0.70 to 0.59 in the combined group (P = 0.037), and from 0.74 to 0.57 in the PDT monotherapy group (P = 0.014). Complete regression of polypoidal lesions in indocyanine green angiography was found in 1 (14.3%) eye in the ranibizumab group, compared with 15 (93.8%) eyes in the combined group (P = 0.001). Additional PDT and ranibizumab injections in eyes with persistent polyps and fluorescein leakage resulted in regression of polyps in all eyes. At 12 months, no significant difference in logarithm of minimal angle of resolution best-corrected visual acuity and visual change was found between eyes initially treated with ranibizumab monotherapy, combined ranibizumab and PDT, or PDT monotherapy (P = 1.00 and P = 0.11, respectively). CONCLUSION: Intravitreal ranibizumab appeared to result in stabilization of vision in patients with symptomatic polypoidal choroidal vasculopathy. However, combined ranibizumab and PDT appeared to be more effective in causing complete regression of the polypoidal lesions in indocyanine green angiography compared with ranibizumab monotherapy.

Ophthalmic manifestations and risk factors for mortality of HIV patients in the post-highly active anti-retroviral therapy era.

BACKGROUND: To evaluate the ophthalmic manifestations and risk factors for mortality in HIV patients in the post-highly active anti-retrovirus therapy (HAART) era. DESIGN: Retrospective study. SAMPLES: 151 patients with HIV infection. METHODS: Review of all HIV patients who have attended the Hong Kong Eye Hospital between 2000 and 2007. MAIN OUTCOME MEASURES: Ocular findings especially opportunistic infections and medical information including mortality during follow up. RESULTS: At presentation, 139 (92.1%) patients were already diagnosed with HIV and 58 (41.7%) had an AIDS indicator condition. Fifty-one (33.8%) patients had HIV-related eye disease on presentation and the leading manifestations were cytomegalovirus (CMV) retinitis and HIV microangiopathy. Low baseline CD4 cell count <100 cells/L was significantly related with HIV-related ophthalmic manifestations and CMV retinitis at presentation (P < 0.013). 105 patients were followed for 6 months or more and the mean follow-up was 4.8 years. There was no significant change in visual acuity compared with baseline (P = 0.13). 20 (19.0%) patients had one eye with final visual acuity of 20/200 or worse and the leading cause for poor vision was CMV retinitis. 11 (10.5%) patients died during the follow-up due to complications of HIV/AIDS. The presence of HIV retinal microangiopathy was significantly associated with mortality (P = 0.005). CONCLUSIONS: CMV retinitis remains the main HIV-related ocular disease in the post-HAART era. HIV retinal microangiopathy might be an important prognostic factor for mortality. Appropriate ophthalmic monitoring is justified to detect for ophthalmic complications in HIV patients regardless of HAART use in order for prompt initiation of treatment.

Photodynamic therapy with or without intravitreal triamcinolone acetonide for symptomatic polypoidal choroidal vasculopathy.

PURPOSE: To evaluate the outcome of verteporfin photodynamic therapy (PDT) with or without intravitreal triamcinolone acetonide (IVTA) for the treatment of symptomatic polypoidal choroidal vasculopathy (PCV). METHODS: Retrospective analysis of PCV patients who underwent PDT with or without IVTA with follow-up of 2 or more years. Changes in best-corrected visual acuity (BCVA), proportion of eyes with moderate visual loss (3 or more lines), and complications were compared between patients with or without IVTA. RESULTS: Twenty-seven eyes of 27 patients were analyzed, with 12 eyes treated by PDT monotherapy and 15 eyes treated by combined PDT with IVTA. The baseline characteristics of both groups were similar. At 1 year, the mean logMAR BCVA for the PDT monotherapy group improved from 0.74 to 0.58 (P = 0.011), whereas the combined PDT and IVTA group improved from 0.84 to 0.74 (P = 0.17). At 2 years, the mean logMAR BCVA for the monotherapy and combined treatment groups were 0.62 and 0.84, respectively, and the changes compared with baseline were not statistically significant. No significant difference was found in the mean logMAR BCVA, the mean line of visual changes, and the mean number of PDT treatments between the 2 groups at 1 and 2 years. One (8.3%) and 4 (26.7%) eyes in the monotherapy and the combined groups lost 3 or more lines at 2 years, respectively. Patients who had combined PDT with IVTA were more likely to develop cataract requiring surgery and ocular hypertension (P = 0.043 and 0.046, respectively). CONCLUSIONS: PDT reduced the risks of visual loss in patients with symptomatic PCV in the short term but the effect might not be sustained after 1 year. The adjunctive use of IVTA during PDT did not appear to result in additional benefit for treating PCV.

Visual outcomes and growth factor changes of two dosages of intravitreal bevacizumab for neovascular age-related macular degeneration: a randomized, controlled trial.

PURPOSE: To evaluate the visual and growth factor changes of two different intravitreal bevacizumab dosages for neovascular age-related macular degeneration. METHODS: Fifty eyes of 50 patients with neovascular age-related macular degeneration were randomized to receive 3 monthly intravitreal injections of 1.25 mg (24 eyes) or 2.5 mg (26 eyes) bevacizumab. Patients were observed for 6 months, and the logarithm of minimal angle of resolution best-corrected visual acuity, central foveal thickness, aqueous vascular endothelial growth factor, and pigment epithelial derived factor levels were assessed. RESULTS: Both groups had significant central foveal thickness reductions at 6 months (P < 0.001). Six (23.1%) eyes in the 2.5-mg group lost 3 or more lines compared with none in the 1.25-mg group (P = 0.023). No significant difference in logarithm of minimal angle of resolution best-corrected visual acuity, central foveal thickness, or growth factors levels was found between the two groups at all visits. Eyes with persistent angiographic leakage at 3 months had significantly higher baseline aqueous vascular endothelial growth factor levels compared with eyes without leakage (P = 0.013). Logistic regression analysis showed that high baseline aqueous vascular endothelial growth factor level was the only significant factor associated with persistent leakage at 3 months (P = 0.040). CONCLUSION: Three monthly intravitreal 1.25-mg bevacizumab injections seemed to result in better visual outcome than 2.5 mg bevacizumab. Baseline aqueous vascular endothelial growth factor level might have a role in predicting angiographic response after bevacizumab injections.

Manganese superoxide dismutase and chemokine genes polymorphisms in chinese patients with anterior uveitis.

PURPOSE: To investigate the association of single-nucleotide polymorphisms (SNPs) in the manganese superoxide dismutase (MnSOD) and two chemokine genes (CCL2 and CCL5) in patients with anterior uveitis (AU). METHODS: Seventy-nine Chinese patients with acute AU were recruited, and genotyping of four SNPs including MnSOD 47, CCL2 -2518, CCL2 -2076, and CCL5 -403 alleles was performed with SNP genotyping assays. The genotype and allele frequencies were compared between patients with AU and 206 healthy control subjects. Analyses were also stratified according to the HLA-B27 status of the patients. RESULTS: There were significant increases in the frequency of the AA homozygosity in the MnSOD 47 SNP (P = 0.049) and in the CCL2 -2518G allele frequency and GG homozygosity in patients with AU compared with control subjects (P = 0.017 and P = 0.024, respectively). No significant association was found between AU with the CCL2 -2076 and CCL5 -403 SNPs. Subgroup analyses showed that the MnSOD 47A polymorphism was significantly associated with AU in HLA-B27-positive patients, but not in HLA-B27-negative patients, whereas the CCL2 -2518G polymorphism was significantly associated with AU in HLA-B27-negative patients, but not in HLA-B27-positive patients. CONCLUSIONS: The 47A polymorphism in the MnSOD gene and the -2518G polymorphism in the CCL2 gene are associated with the development of AU in HLA-B27-positive and -negative Chinese patients, respectively. Further studies to evaluate the interactions of the HLA-B27 status and these SNPs are warranted.

Presence of crystalline lens as a protective factor for the late development of open angle glaucoma after vitrectomy.

PURPOSE: To evaluate the late development of open angle glaucoma (OAG) after vitrectomy and to compare the rate of postvitrectomy OAG development in phakic and pseudophakic eyes. METHODS: Retrospective case series of 101 eyes of 101 patients who had vitrectomy for idiopathic epiretinal membrane or idiopathic macular hole with a follow-up duration of more than 6 months. Eligible patients were assessed for new development of OAG during the follow-up period. Patients were separated into two groups based on the lens status at the end of the vitrectomy for statistical analysis using Kaplan-Meier survival analysis with log-rank test and Cox regression analysis. RESULTS: The mean follow-up duration was 51 months (range, 6-80 months). Of the 101 eyes, 8 (7.9%) eyes developed OAG during the follow-up period. Phakic eyes were less likely to develop OAG after vitrectomy compared with pseudophakic eyes, with 2% and 13%, respectively (log-rank test, P = 0.025). The result remained statistically significant after adjustment for age, refractive error and the use of gas tamponade (adjusted odds ratio = 0.09, P = 0.038). CONCLUSION: The presence of the crystalline lens may be protective against the late development of OAG after vitrectomy.

Solitary superficial angiomyxoma in the eyelid.

PURPOSE: To report the clinicopathologic features of a solitary superficial angiomyxoma arising in the eyelid. DESIGN: Case report. METHODS: A retrospective review of the clinical and pathologic features of a patient with solitary superficial angiomyxoma in the eyelid. RESULTS: A 47-year-old male presented with a right upper lid mass for 6 months. Excisional biopsy was performed, and microscopic examination revealed a tumor comprising loose spindle or stellate-shaped cells in myxoid stroma sprinkled with small numbers of neutrophils. The tumor cells were negative for smooth muscle actin, desmin, S-100 protein, and CD34 on immunostaining. CONCLUSIONS: Ophthalmologists should be aware of superficial angiomyxoma as a rare cutaneous tumor with a tendency for local recurrence. Multiple lesions and occurrence in the external ear can be associated with the Carney's complex.