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.

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.

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.

[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.

[Recovery of photoreceptor inner/outer segment junction in spectral domain optical coherence tomography after surgery in idiopathic macular hole].

OBJECTIVE: To evaluate the findings of spectral domain optical coherence tomography (SD-OCT) in patients with idiopathic macular hole (IMH). METHODS: It was a retrospective case series study. Pre- and post-operative data from patients with IMH during 14 months were analyzed retrospectively. The main outcome data included logarithm of the minimal angle of resolution (logMAR) and SD-OCT findings, including the size of the IMH and the disruption of the boundary line between inner segments (IS) and outer segments (OS) of the photoreceptors. The data were analyzed using the statistical software SPSS14.0. The correlation among various data was analyzed by Bivariate correlation. The pre-and post-operative data were compared by paired-samples t-tests. RESULTS: Thirty two eyes of 32 patients were studied. The mean logMAR was 1.16 ± 0.46 (ranged from 0.40 to 2.00), the mean diameter of macular hole was (859.7 ± 292.0) µm (ranged from 332 to 1568 µm). The mean diameter of IS/OS disruption was (1965.1 ± 584.1) µm (ranged from 867 to 3444 µm). In eyes after surgery, the mean logMAR was 0.89 ± 0.46 (ranged from 0.30 to 2.00), the mean diameter of the IS/OS disruption was (1350.4 ± 642.6) µm (ranged from 153 to 2546 µm), both were decreased significantly as compare with the preoperative data (t = 3.384, 6.360; P < 0.05). Preoperative diameter of macular hole (r = 0.583) and IS/OS disruption (r = 0.416) were correlated significantly with postoperative logMAR (P < 0.05). CONCLUSIONS: Preoperative diameter of macular hole and IS/OS disruption play the main role in vision prognosis. The IS/OS disruption is decreased significantly after the operation.

[Features of vitreomacular traction syndrome assessed with three-dimensional spectral-domain optical coherence tomography].

OBJECTIVE: To describe features of vitreomacular traction syndrome (VMT) on three-dimensional (3D) spectral-domain optical coherence tomography (SD OCT). METHODS: Eighteen eyes of 18 patients with VMT were diagnosed by SD OCT. SD OCT images were obtained and retrospectively analyzed. These VMT cases were defined as focal type or broad type by the region of the vitreous attachment to the macula. The relationship between the logMAR best corrected visual acuity (BCVA) and the retinal thickness of macular fovea was evaluated by linear regression analysis. RESULTS: SD OCT provided detailed 3D images of VMT and revealed information about the extent of vitreomacular traction. Focal VMT was seen in 14 eyes. Broad VMT was seen in four eyes. Eight cases had concurrent epiretinal membrane in the areas surrounding the fovea. Two cases had concurrent full thickness macular hole and two cases had concurrent lamellar macular hole. LogMAR BCVA correlated with the retinal thickness of macular fovea (r = 0.616, P = 0.007, linear regression analysis). CONCLUSIONS: SD OCT allows good visualization of the architectural morphology of the vitreous and retina in vitreomacular traction syndrome. It is useful and irreplaceable for the diagnosis and follow-up of VMT.

[Correlation between photoreceptor layer and visual acuity in eyes with persistent cystoid macular edema secondary to branch retinal vein occlusion].

OBJECTIVE: To study the correlation of visual acuity and photoreceptor in eyes with persistent cystoid macular edema associated with branch retinal vein occlusion (BRVO). METHODS: It was a retrospective case series study.Medical records of 41 patients (41 eyes) with persistent cystoid macular edema secondary to BRVO diagnosed in Department of Ophthalmology, Peking Union Medical College Hospital from January 2008 to January 2010 were studied retrospectively. On the images obtained by spectral-domain OCT, the thickness of foveal neurosensory retina and photoreceptor layer that was beneath the foveal cystoid spaces, and the transverse length and height of foveal cystoid spaces were measured. The status of the junction between inner and outer segments of the photoreceptors (IS-OS) in the fovea was also assessed. The correlations between the parameters above and visual acuity were analyzed. RESULTS: The average foveal neurosensory thickness was (484.71 ± 131.86) µm, the photoreceptor layer thickness was (92.04 ± 45.53) µm and the size of foveal cavity was 772.59 µm × 362.17 µm. By the Spearman's rank correlation analysis, among the parameters above, only photoreceptor layer thickness showed correlation with logMAR visual acuity (r = -0.373, P = 0.016), while other parameters showed no correlation with logMAR visual acuity. Cases were classified as three groups according to the status of IS-OS layer: continuous group (8 cases), discontinuous group (19 cases) and interrupted group (14 cases). LogMAR visual acuity in these eyes with an interrupted IS-OS line in the fovea was significantly worse than that in eyes with a continuous or discontinuous IS-OS line (P = 0.030 and 0.003, respectively). There was no statistical difference between continuous and discontinuous group (P = 0.750). CONCLUSIONS: In patients with persistent cystoid macular edema associated with BRVO, the thickness of the foveal photoreceptor layer are positively associated with visual acuity, and the eyes without IS-OS layer have worse visual acuity than eyes with IS-OS layer.

[Clinical analysis of intraocular pressure elevation following vitreoretinal surgery].

OBJECTIVE: To study the incidence and risk factors of intraocular pressure (IOP) elevation in patients who underwent vitreoretinal surgery. METHODS: Data for 422 patients (446 eyes) who underwent vitreoretinal surgery from June 2005 to June 2007 were retrospectively analyzed. IOP was measured before surgery and on day 1, 2, 3, 7-14 after surgery with Goldmann applanation tonometer. Ocular hypertension was defined as IOP 25 mmHg or more. All factors were analyzed for association with Ocular hypertension. RESULTS: 232 of the 422 patients were female. 190 were male. The IOP was elevated significantly in 185 eyes (41%) after surgery within 2 weeks. Among them the IOP occurred mostly in day 1 (152 eyes, 82%). The rate of IOP elevation with intraocular tamponade of C3F8 or silicone oil was 53% or 55%, the difference being not statistically significant (P>0.05). The incidence of high IOP in C3F8 injection group and silicone oil injection group were statistically higher than simple pars plana vitrectomy group (P<0.01). The risk factors of ocular hypertension include C3F8 injection (chi2=37.82, P<0.01), silicone oil injection (chi2=27.84, P<0.01), lentectomy (chi2=8.98, P<0.01), scatter endolaser (chi2=7.41, P<0.01), diabetes (chi2=12.12, P<0.01) and increasing patient age (chi2=7.07, P<0.01). CONCLUSION: IOP elevation was a common complication of vitreoretinal surgery. The risk factors of ocular hypertension include C3F8 injection, silicone oil injection, lentectomy, scatter endolaser, diabetes and increasing patient age.

[Clinical characteristics of suspected combined hamartoma of the retina and retinal pigment epithelium].

OBJECTIVE: To analyze the clinical presentations, and explore the diagnosis, and differential diagnosis of combined hamartomas of the retina and retinal pigment epithelium (CHRRPE). METHODS: A retrospective review of five patients with suspected CHRRPE presented to Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, from Nov 2001 to July 2008. The clinical presentations, fundus characteristics, fundus fluorescein angiography (FFA), optic coherence tomography (OCT), as well as B ultrasound scan examinations were analyzed. RESULTS: The five patients were all male, ranging in age from 6 to 18 years (mean 12 years). The lesion was unilateral; there were not any associated diseases. Presenting symptoms included painless decrease in vision, metamorphopsia, and discovery of strabismus. Initial visual acuity ranged from 20/333 to 20/50. Locations of the lesion included on optic disk and adjacent retina, and in the macular area. Clinical characteristics were slight elevation on optic disk or in retina, different degree of hyperpigmentation, retinal vascular tortuosity, as well as epiretinal membrane formation. In arterial phase of FFA, there was hypofluorescence of choroid background, retinal vascular was tortuous and telangiectatic, and there was leakage from vessels within the lesion in venous phase and late phase. OCT showed obscuration of the normal retinal layers, elevated lesion with high reflectivity of the inner retina, and hyporeflective shadowing of the underlying tissue. B ultrasound scan showed slightly elevated solid mass involving the disc or adjacent retina in some patients. CONCLUSIONS: CHRRPE may be confused with other masses of the retina and choroid. Diagnosis of CHRRPE mainly depends on fundus manifestations, FFA and OCT. B ultrasound scan is useful for differential diagnosis.

[Clinical features of silicone oil dependent eyes].

OBJECTIVE: To study the clinical features of silicone oil dependent eyes. METHOD: Retrospective observational case series. Twenty four silicone oil dependent eyes collected from the Peking Union Medical College from Jan.2000 to Dec.2006 were studied. The pathogeny, surgical process and prognosis were analyzed. RESULTS: There were 5.9% silicone oil dependent eyes in eyes underwent vitrectomy and silicone oil tamponade. Silicone oil tamponade was performed again in 17 eyes because of severe proliferative vitreoretinopathy (PVR) or repeated vitreous hemorrhage after silicone oil removal and C3F8 tamponade. Silicone oil replacement was performed in 3 eyes because of severe PVR. Continually low intraocular pressure occurred after silicone oil removal in 4 eyes with 360 degree peripheral retinotomy. Thirteen of 24 patients had visual acuities of finger counting to 0.1, 9 had hand movement, one had light perception and one had no light perception. CONCLUSIONS: The incidence of silicone oil dependent eye is approximately 5.9%. This is often seen in the patients with severe or repeated PVR, or 360 degree peripheral retinotomy. The visual prognosis is poor in most cases.