Atopic Dermatitis Associated Retinal Detachment and Retinal Vasculitis: A Case Report.

Atopic dermatitis is usually associated with various ocular complications. We report a 21-year-old Chinese male who presented to our ophthalmology clinic with bilateral retinal detachment and cataracts. The patient had a clear medical history of atopic dermatitis, which had been diagnosed eight years earlier and had been treated with loratadine and pimecrolimus. Cataract surgery was performed for both eyes, combined with scleral buckling for the right eye and pars plana vitrectomy for the left eye. During postoperative follow-up, fundus fluorescein angiography showed retinal vasculitis in both eyes and macular edema in the left eye, which coincided with an exacerbation of atopic dermatitis. Macular edema improved after four months of regular dupilumab treatment in the dermatology department. The ocular condition remained stable three years postoperatively.

Retinal Vasculitis as an Ocular Manifestation in Atopic Dermatitis-Associated Retinal Detachment: A Case Report.

Atopic dermatitis is usually associated with various ocular complications. We report a 21-year-old Chinese male who presented to our ophthalmology clinic with bilateral retinal detachment and cataracts. The patient had a clear medical history of atopic dermatitis, which had been diagnosed eight years earlier and had been treated with loratadine and pimecrolimus. Cataract surgery was performed in both eyes, combined with scleral buckling in the right eye and pars plana vitrectomy in the left eye. During postoperative follow-up, fundus fluorescein angiography indicated retinal vasculitis in both eyes and macular edema in the left eye, which coincided with an exacerbation of atopic dermatitis. Macular edema improved after four months of regular dupilumab treatment in the dermatology department. The ocular condition remained stable three years postoperatively.

Iridociliary Body Metastasis of Atypical Carcinoid: Case Management with Intravitreal Anti-Vascular Endothelial Growth Factor Injections.

We report a rare case involving a 52-year-old female diagnosed with an atypical bronchial carcinoid tumor with metastases to the mediastinum, hilar lymph nodes, breast, and pancreas. In additional, the patient had metastases to the iris and ciliary body, resulting in progressive vision loss in her left eye. Treatment was successful by intravitreal injections of anti-vascular endothelial growth factor.

Development of Diagnostic Recommendations for Vitreoretinal Lymphoma.

PURPOSE: To develop diagnostic recommendations for diffuse large B-cell vitreoretinal lymphoma (VRL) in Chinese patients. METHODS: Retrospective observational case series. Seventy-three eyes of 40 VRL patients and 8 control patients were analyzed. Eighteen patients from Beijing Tongren Hospital and 46 patients from literature were involved as validations. RESULTS: Diagnostic methods included (1) typical clinical manifestations; (2) vitreous cytology; (3) immunohistochemical examination of vitreous or choroid/retina; (4) aqueous humor or vitreous cytokine; (5) vitreous cell gene rearrangement; (6) vitreous flow cytometry. If patients meet (1)+(2)+(3), or if they meet (1), and two of (4), (5), (6) are positive, they can be diagnosed as VRL. The sensitivity and specificity values for accurate diagnosis were 0.975 and 1.00. One hundred percent eyes from Beijing Tongren Hospital and 92.7% eyes from literature can be diagnosed. CONCLUSION: We developed diagnostic recommendations for diffuse large B-cell VRL through vitreous cytology combined with multiple auxiliary examinations.

Long-term efficacy and safety of sirolimus for retinal astrocytic hamartoma associated with tuberous sclerosis complex.

Mammalian target of rapamycin (mTOR) inhibitors (sirolimus or everolimus) have been demonstrated effective in reducing the size of tuberous sclerosis complex (TSC)-associated retinal astrocytic hamartoma (RAH) in short term. To investigate the long-term efficacy and safety of sirolimus on TSC-associated RAH, 13 TSC-associated RAH patients (59 RAH lesions) who received sirolimus therapy for at least 2 years were retrospectively enrolled in this study. Changes in the maximal thickness (MT) of RAH on optical coherence tomography and the longest base diameter (LBD) of RAH on color fundus photography were assessed. The results showed that for a mean follow-up of 39 months, sirolimus was associated with a mean reduction of 14.6% in MT and 6.8% in LBD of RAHs. The main impacts of sirolimus occurred within the first 6-12 months, with 14.8% reduction in MT and 4.7% reduction in LBD. Mouth ulceration (10 [76.9%]) and acne (9 [69.2%]) were the most common adverse events. These follow-up data support the long-term use of sirolimus in TSC-associated RAH patients, and persistent use of sirolimus possibly prevents tumor regrowth.

Apnea caused by retrobulbar anesthesia: A case report.

BACKGROUND: Apnea caused by retrobulbar anesthesia is a very rare but severe complication during ophthalmic surgery. CASE SUMMARY: We report a rare case of apnea caused by retrobulbar anesthesia, and emergency resuscitation was used. A 74-year-old female patient was diagnosed with rhegmatogenous retinal detachment in the right eye and planned to undergo vitrectomy under retrobulbar anesthesia. After the retrobulbar anesthesia in her right eye, she became unconscious and apneic. It was suggested that she had developed brainstem anesthesia. Assisted ventilation was initiated. Atropine 0.5 mg, epinephrine 1 mg, ephedrine 30 mg, and lipid emulsion were given. Five minutes later, her consciousness and breathing gradually returned, but with uncertain light perception in her right eye. Alprostadil 20 µg was given, and after 2 h her visual acuity resumed to the preoperative level. CONCLUSION: Brainstem anesthesia is a serious complication secondary to retrobulbar anesthesia. Medical staff should pay attention to the identification of brainstem anesthesia and be familiar with the emergency treatment for this complication.

Altered amplitude of low-frequency fluctuations and default mode network connectivity in high myopia: a resting-state fMRI study.

AIM: To analyze changes in amplitude of low-frequency fluctuations (ALFFs) and default mode network (DMN) connectivity in the brain, using resting-state functional magnetic resonance imaging (rs-fMRI), in high myopia (HM) patients. METHODS: Eleven patients with HM (HM group) and 15 age- and sex-matched non-HM controls (non-HM group) were recruited. ALFFs were calculated and compared between HM group and non-HM group. Independent component analysis (ICA) was conducted to identify DMN, and comparisons between DMNs of two groups were performed. Region-of-interest (ROI)-based analysis was performed to explore functional connectivity (FC) between DMN regions. RESULTS: Significantly increased ALFFs in left inferior temporal gyrus (ITG), bilateral rectus gyrus (REC), bilateral middle temporal gyrus (MTG), left superior temporal gyrus (STG), and left angular gyrus (ANG) were detected in HM group compared with non-HM group (all P<0.01). HM group showed increased FC in the posterior cingulate gyrus (PCC)/precuneus (preCUN) and decreased FC in the left medial prefrontal cortex (mPFG) within DMN compared with non-HM group (all P<0.01). Compared with non-HM group, HM group showed higher FC between mPFG and bilateral middle frontal gyrus (MFG), ANG, and MTG (all P<0.01). In addition, HM patients showed higher FC between PCC/(preCUN) and the right cerebellum, superior frontal gyrus (SFG), left preCUN, superior frontal gyrus (SFG), and medial orbital of the superior frontal gyrus (ORB supmed; all P<0.01). CONCLUSION: HM patients show different ALFFs and DMNs compared with non-HM subjects, which may imply the cognitive alterations related to HM.

Reliability of optic disc edema area in estimating the severity of papilledema in patients with POEMS syndrome.

BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome involving multisystem. Optic disc edema (ODE) is the most common ocular manifestation in patients with POEMS syndrome and serves as an independent prognostic factor. However, parameters previously used to estimate its severity were inconvenient and costly. This study was designed to bring forward a novel and practical parameter, optic disc edema area, to evaluate ODE in patients with this disease and applied it to assess effectiveness of lenalidomide combined with dexamethasone in respect of ODE. RESULTS: Forty-one treatment-naive patients with POEMS syndrome were enrolled in this single-center prospective study and treated with lenalidomide combined with dexamethasone. They received ocular examination to determine optic disc edema (ODE) area and other optic manifestations. Meanwhile, serum VEGF was measured before and after treatment. Among 41 enrolled patients, 38 received complete ocular examinations, and 25 of which had ODE at initial visit. Binocular mean ODE area of patients with ODE was significantly related to ODE grade (r = 0.620, p = 0.003) and peripapillary retinal thickness (r = 0.760, p < 0.001) before treatment. Serum VEGF was significantly higher in patients with ODE than their counterparts (p = 0.025) and positively correlated with binocular mean ODE area (r = 0.460, p = 0.036). After treatment, ODE area, along with serum VEGF, decreased markedly (p < 0.001). CONCLUSION: ODE area was a reliable index to evaluate ODE severity and could precisely reflect ODE improvement through systemic treatment. Additionally, it was related to serum VEGF, a key factor in disease pathogenesis, suggesting its potential as an indicator of the overall severity of this disease. TRIAL REGISTRATION: Clinicaltrials, NCT01816620. Registered March 222,013.

Comparison of aqueous humor levels of PlGF and VEGF in proliferative diabetic retinopathy before and after intravitreal conbercept injection.

AIMS: The aim of this paper is to observe the change of aqueous humor levels of placenta growth factor (PlGF) and vascular endothelial growth factor (VEGF) in proliferative diabetic retinopathy (PDR) patients before and one week after intravitreal conbercept injection. METHODS: A prospective case series study was conducted in 24 active PDR patients (24 eyes). All the patients had received 0.5 mg of intravitreal conbercept followed by vitrectomy one week later. The aqueous humor was collected before conbercept injection and at the beginning of vitrectomy. RESULTS: Before conbercept injection, the aqueous humor median levels of VEGF-A, VEGF-B and PlGF were457.0pg/mL(IQRfrom392.9to860.6pg/mL), 43.6pg/mL(IQRfrom33.6to81.6pg/mL), 37.5pg/mL(IQRfrom25.0to53.6pg/mL), respectively. One week after conbercept injection, the aqueous humor levels of VEGF-A, VEGF-B and PiGF decreased significantly. The aqueous humor VEGF-A levels in PDR patients with fibrovascular membranes were lower than those without them. There was positive correlation between aqueous humor VEGF-B and PiGF levels (P = 0.007). No significant correlation was found between VEGF-A and PiGF levels. No ocular and systemic adverse events were observed. CONCLUSIONS: The aqueous humor levels of PlGF was correlated with VEGF-B, and levels of VEGF-A, VEGF-B, and PlGF decreased after intravitreal conbercept injection in active PDR patients.

Observation of optic disc neovascularization using OCT angiography in proliferative diabetic retinopathy after intravitreal conbercept injections.

This study reports the short-term efficacy and safety of intravitreal conbercept injections for neovascularization at the disc (NVD) in patients with proliferative diabetic retinopathy (PDR). Conbercept is a recombinant fusion protein with a high affinity for all isoforms of vascular endothelial growth factor (VEGF)-A, placental growth factor and VEGF-B. A prospective case series study was conducted in 15 patients (15 eyes). Patients had complete ocular examinations and received a 0.5 mg intravitreal conbercept injection followed by supplemental pan-retinal photocoagulation (PRP). Optical coherence tomography angiography (OCTA) was performed before and after treatment. Before treatment, the mean NVD area was 1.05 ± 0.33 mm2, and it decreased to 0.56 ± 0.17 mm2 after an interval of 7.5 d (p = 0.000). One eye required vitrectomy during follow-up. Recurrent NVD was observed in 2 eyes, which resolved after repeated injections. The remaining 12 eyes were stable over a mean follow-up period of 8.3 months. The mean area of the NVD in 14 patients without vitrectomy was 0.22 ± 0.11 mm2 (p = 0.000) at the last visit. Intravitreal conbercept injections combined with intensive PRP are an effective and safe treatment for PDR with NVD. Quantitative information on NVD can be obtained with OCTA, which may be clinically useful in evaluating the therapeutic effect.

OCULAR MANIFESTATIONS AND TREATMENT OUTCOMES IN CHINESE PATIENTS WITH POEMS SYNDROME.

PURPOSE: To evaluate the relationship of serum vascular endothelial growth factor (VEGF) levels and ocular manifestations in Chinese patients with POEMS syndrome. METHODS: This is a prospective study. Forty-one treatment-naive patients were enrolled from April 2014 to November 2014. Among the 41 patients, 40 had complete ocular examination, spectral domain optical coherence tomography scan, and serum VEGF measurement before treatment and every 3-month interval after lenalidomide and dexamethasone treatment. RESULTS: Twenty-seven (67.5%) patients had optic disk edema (ODE) at baseline. Retinal manifestations included retinal hemorrhage, subretinal fluid, macular edema, and cotton wool spot. The difference in mean serum VEGF concentrations between patients with and without ODE was significant (P = 0.017). Among patients with ODE, there was a significant positive correlation between mean serum VEGF levels and the binocular mean retinal nerve fiber layer thickness (P = 0.008), as well as mean peripapillary retinal thickness (P = 0.020) before treatment. After 3 months to 17 months treatment, mean serum VEGF concentrations decreased significantly (P < 0.001). Mean retinal nerve fiber layer thickness and mean peripapillary retinal thickness decreased significantly (P < 0.001). The remission rate of ODE was 87.5%, and complete remission rate was 58.3%. CONCLUSION: The ODE is a common manifestation in POEMS syndrome, and raised VEGF might explain the development and mechanism. Systemic treatment could lead to decrease in serum VEGF levels accompanied by regression of ODE.

Systemic lupus erythematosus and antiphospholipid syndrome related retinal vasculitis mimicking ocular cysticercosis: a case report.

Making accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations. The possibility that vasculitis associated with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) can be misdiagnosed as cysticercosis has not been discussed in the literatures.

Spectral domain optical coherence tomography of Vogt-Koyanagi-Harada disease: novel findings and new insights into the pathogenesis.

OBJECTIVE: To provide novel spectral domain optical coherence tomography (SD OCT) findings of Vogt-Koyanagi-Harada (VKH) disease as well as new insights into the pathogenesis of this disease. METHODS: Detailed SD OCT and fluorescein angiography (FA) findings of 18 consecutive VKH patients (11 women and 7 men) from December 2007 to April 2009 who were in acute uveitic stage at presentation were reviewed. All the patients had been followed up for at least 6 months with reevaluation(s) of SD OCT performed in 10 patients. RESULTS: Intraretinal cysts were found to be located in various layers of the outer retina. In addition to the photoreceptor layer, they could also be found between the outer plexiform layer and the outer nuclear layer, or spanning the external limiting membrane. On FA, intraretinal cysts could be hypofluorescent, normofluorescent, or hyperfluorescent. Some intraretinal cysts had a characteristic FA pattern, in which a small round hypofluorescent area was surrounded by a ring of hyperfluorescence (donut-shaped dye pooling). Subretinal fibrinoid deposit appeared in acute uveitic stage in two severe VKH patients and seemed to develop from subretinal exudates and evolved into typical subretinal fibrosis. Gradual transfiguration/migration and progressive proliferation/pigmentation of the subretinal fibrinoid deposit/subretinal fibrosis was observed in one patient. CONCLUSIONS: Intraretinal cysts could form in various layers of the outer retina and may result from extension of choroidal inflammation. Subretinal fibrosis may develop from subretinal exudates in VKH patients and may cause substantial visual impairment.

[Fundus manifestation and treatment of Takayasu's arteritis].

OBJECTIVE: To evaluate the fundus manifestation and treatment of Takayasu's arteritis. METHODS: Retrospective case series was taken to study the clinical data of 15 patients (30 eyes) diagnosed with Takayasu's arteritis at Peking Union Medical College Hospital from 1992 to 2010. Examinations included visual acuity, slit-lamp, fundus, fundus fluorescein angiography (FFA), large vascular ultrasound, digital subtraction angiography (DSA), erythrocyte sedimentation rate (ESR) and reactive protein C (CRP). All patients were given systemic corticosteroids, immunosuppressive agents and anticoagulants. Three patients (4 eyes) received retinal photocoagulation, and 1 patient (1 eye) received vitrectomy combined with silicone oil tamponade. RESULTS: Fifteen cases aged 13 - 49 years [mean (33.6 ± 11.1) years] were enrolled, including 2 male and 13 female patients. Two patients recognized by initial presentation of impaired vision prior to the diagnosis of Takayasu's arteritis. Visual acuity: light perception -0.05, 6 eyes; 0.1 - 0.5, 4 eyes; 0.6 - 1.0, 5 eyes; and above 1.0, 15 eyes. The fundus examination revealed chronic ischemic retinopathy in 16 eyes characterized by microaneurysms, cotton-wool spots and peripapillary arteriovenous anastomosis, and complicated by vitreous hemorrhage, retinal detachment and proliferative retinopathy at the late stage. There was hypertensive retinopathy in 14 eyes with features of narrow retinal artery, arteriovenous crossing signs and retinal hemorrhage. FFA showed prolonged arm-to-retina circulation time and retinal circulation time, microaneurysms and neovasculature. Six cases were classified as brachiocephalic arteritis, and 9 patients belonged to extensive arteritis. ESR increased in 9 patients and CRP elevated in 5 patients. Capillary non-perfusion areas in 4 eyes subsided after retinal photocoagulation, and retina reattached in 1 eye after vitrectomy with visual acuity improved from light perception to count finger. CONCLUSIONS: Fundus manifestation of Takayasu's arteritis is usually characterized by chronic ischemic retinopathy and hypertensive retinopathy, and complications such as vitreous hemorrhage, retinal detachment and proliferative retinopathy occur at advanced stage. The first symptom in some patients can be impaired vision. The prognosis could be improved if ophthalmologists know fundus characteristics of Takayasu's arteritis and give timely and correct diagnosis and treatment.

[Tomographic features of polypoidal choroidal vasculopathy lesions by spectral domain optical coherence tomography].

OBJECTIVE: To analyze the tomographic features of branching vascular networks and polypoidal lesions in patients with polypoidal choroidal vasculopathy (PCV) by spectral domain optical coherence tomography (SD-OCT). METHODS: It was a retrospective case series study. Retrospective review of 63 eyes of 54 patients with PCV identified with indocyanine green angiography (ICGA). Tomographic features of branching vascular networks and polypoidal lesions on SD OCT, ICGA and fluorescein angiography were analyzed. RESULTS: Both branching vascular network and polypoidal lesions were found in 45 eyes on ICGA, branching vascular network only was found in 8 eyes and polypoidal lesions only in 10 eyes. Retinal pigment epithelial detachment (RPED) was found in all 53 eyes with branching vascular networks on SDOCT. Thirty-eight eyes had areas of moderate reflectivity of branching vascular network between the fluctuating retinal pigment epithelium and Bruch membrane. Fifteen eyes had moderate reflectivity of branching vascular network and/or hemorrhage between high doom-like RPED and incomplete Bruch membrane. Solitary polypoidal lesions in 28 patients showed local moderate reflectivity between the RPE and Bruch membrane on SDOCT. Other aggregate polypoidal lesions in 27 patients showed moderate reflectivity between high doom-like RPED and incomplete Bruch membrane. CONCLUSION: The vascular abnormalities of PCV identified with ICGA were visualized on SD-OCT images as areas of moderate reflectivity between RPE and Bruch membrane.

[Relationship between central visual acuity and retinal volume of macular fovea accessed by spectral domain optical coherence tomography in Stargardt disease].

OBJECTIVE: To assess the relationship between central visual acuity and retinal volume of macular fovea in patients with Stargardt disease by spectral-domain optical coherence tomography (SD OCT). METHODS: It was a retrospective case series study. Twenty eyes of 10 patients with Stargardt disease were investigated by three-dimensional spectral-domain optical coherence tomography. SD OCT images were obtained and retrospectively analyzed. The retinal volumes of macular fovea were measured by SD OCT, whose diameters were set as 3 mm and 1 mm separately (volume 3 and volume 1). The retinal thickness of macular fovea (macular thickness) and the width of IS/OS conjunction loss of macular fovea (IS/OS loss) were also measured by SD OCT. We correlated the logMAR BCVA with IS/OS loss, macular thickness, volume 3 and volume 1 by linear regression analysis. RESULTS: LogMAR BCVA was from 0.3 to 1.22. IS/OS loss was from 847 µm to 5306 µm. Macular thickness was from 20 µm to 126 µm. Volume 3 and volume 1 was from 1.06 to 1.76 mm(3) and 0.06 to 0.13 mm(3). LogMAR BCVA correlated with the IS/OS loss (r = 0.695, P < 0.05), macular thickness (r = -0.601, P < 0.05), and volume 3(r = -0.725, P < 0.05). LogMAR BCVA did not correlate with volume 1(r = -0.364, P > 0.05). CONCLUSIONS: SD OCT could demonstrate the retinal structure of Stargardt disease clearly. The retinal volume of macular fovea accessed by SD OCT correlated with the visual acuity of Stargardt disease.

[Prevalence and risk factors of retinal arterial atherosclerosis in urban subjects participating in routine physical examinations].

OBJECTIVE: To determine the prevalence and risk factors of atherosclerotic lesions in retinal arteries of an urban population undergoing routine physical examinations. METHODS: Demographic and clinical information of subjects participating in annual routine physical examinations at Peking Union Medical Center from January to October of 2010 were collected and summarized. Univariate and multivariate Logistic regression analyses were conducted to evaluate the demographic and clinical factors associated with retinal arterial atherosclerosis. RESULTS: Among a total of 17 886 non-diabetic adults evaluated during the study period, retinal arterial atherosclerosis was diagnosed in 1721 (9.6%). There were predominantly grade 1 (88%) and grade 2 (11%) lesions. Prevalence of retinal arterial atherosclerosis increased with age. And traditional risk factors for cardiovascular diseases included overweight or obesity, hypertension, dyslipidemia and elevated fasting serum glucose. In a multivariate Logistic regression model, advanced age, male gender, obesity or overweight, hypertension and dyslipidemia were independently associated with a higher risk of retinal arterial atherosclerosis. Hypertension was prevalent in subjects above 40 years old while overweight or obesity had a high prevalence in all age groups. CONCLUSION: Retinal arterial atherosclerosis is common in this urban population. And aging, overweight/obesity and hypertension are the dominant risk factors. Routine physical examination is valuable for both the prevention and an early diagnosis of this disease.