Objective To analyze the onset, clinical manifestation, causation, complications of pediatric uveitis. Methods One hundred and two patients with uveitis under 16 year-old were retrospectively studied. They visited the clinic in Peking University First Hospital from November 1979 to December 2008. Their age ranged from 2.5 to 16 years old, with a mean of 11.9 years. Routine exam was carried out, including visual acuity, slit lamp, fundus, and laboratory workup. The diagnosis and classification were made by the anatomic location according to the standard of The International Uveitis Working Group. The data of disease history, age of onset, manifestation, recurrence, causation, systemic diseases, complications, and lab examination were analyzed.Results A total of 102 patients (170 eyes) with pediatric uveitis were included in this study, 68 patients (66.6%) were bilateral cases. Anterior uveitis represented in 38 patients (37.3%), intermediate uveitis in 19 (18.6%), posterior uveitis in 10 (9.8%), and panuveitis in 35 (34.3%). The disease duration was from five days to 2.4 years, with a mean of 3.6 months. The follow-up time was two weeks to more than ten years. The first three causes of pediatric uveitis were juvenile idiopathic arthritis, Vogt-Koyanagi-Harada disease, and Behccedil;etprime;s disease. 36 patients were found with complications, and among them 19 had complicated cataract, seven had secondary glaucoma, five had corneal band dystrophy, 12 had iris synechia (both anterior and posterior), one had retinal detachment, two had eye atrophy, and one patient with juvenile idiopathic arthritis had bilateral femoral head necrosis because of the use of steroid and hip joint was replaced. There were ten children suffering more than two complications. Conclusions Pediatric uveitis is a possible blindness disease with variety of etiology and manifestations,and tends to cause complications. Early and special attention must be taken to avoid serious consequences.
Objective To observe the ocular features and analyze the cause of misdiagnosis of intraocular nonHodgkin's lymphoma (IONHL). Methods A retrospective study was performed in 6 patients and related literatures were reviewed. The age of those patients was 46 to 68 yrs with an average of 562 yrs. Four cases were female, two cases were male. Five patients were unilateral and one case was bilateral case. All patients were investigated by Goldmann tonometer, fundus fluorescein angiography (FFA) and ophthalmic Bscan. Pathological samples come from enucleation and vitrectomy, and were analyzed histologically and immunohistochemically. Results Ophthalmological signs of IONHL included decrease visual acuity, red eye, aqueous flare; keratic precipitates (Kp), vitreous opacity, retinal detachment and multiple whiteyellow retinal lesions of different sizes and fuzzy boundaries. IONHL patients were always misdiagnosed as diverse and nonspecific symptoms and signs, two cases were misdiagnosed as retinitis or chorioiditis, one case as intermediate uveitis, one case as secondary glaucoma in uveitis. The duration from onset of symptom and sign to a final diagnosis was from 6 months to 24 months with an average of 13.3 months. Conclusions The clinical manifestation of IONHL was diverse and non-specific，and this disease was unknown to lots of doctors and easily be misdiagnosed.
Objective To observe the hereditary types and clinical characteristics of 137 patients with retinitis pigmentosa (RP) in Ningxia. Methods One hundred and thirty-seven patients with RP who diagnosed by the examinations of visual acuity, optometry, direct or indirect ophthalmoscope, visual field, optical coherence tomography (OCT) and electroretinogram were enrolled. The hereditary types and clinical characteristics were analyzed according to the family history and the Results of ophthalmologic examinations. Results One hundred and thirty-seven patients included 29 autosomal dominant RP (ADRP) patients from 8 families (7.4%), 16 autosomal recessive RP (ARRP) patients from 15 families (13.9%), 10 X-linked RP (XLRP) from 3 families (2.8%), and 82 simplex RP (SRP) patients (75.9%). There were 15 consanguineous marriage families out of 26 families with RP history (57.7%). The patients were classified as typical RP (102 patients, 74.5%) and atypical RP (35 patients, 25.5%). All the ADRP and XLRP patients showed typical clinical features of RP. Ten (62.5%) of ARRP patients and 53 (64.6%) of SRP patients had typical features of RP. Six (37.5%) of ARRP patients and 29 (35.4%) of SRP patients had atypical features of RP. Among atypical RP patients, 17 (48.6%) patients were nonpigmented RP which including 3 patients were misdiagnosed as amblyopia during childhood. The logarithm of minimal angle of resolution (logMAR) best corrected visual acuity (BCVA) of ADRP patients was 1.04plusmn;0.51 at the age older than 51 years, while the BCVA of ARRP and XLRP patients were 0.92plusmn;0.61 and 1.70plusmn;0.02 respectively at 21 to 30 years of age. One hundred and twentythree (89.8%) patients suffered from varying degrees of myopia. OCT showed that the average thickness of macular fovea in ADRP patients was (185.73plusmn;1.23) mu;m at the age older than 51 years, while in ARRP and XLRP patients were (173.21plusmn;0.98) and (170.49plusmn;1.15) mu;m respectively at 21 to 30 years of age. Conclusions ADRP and XLRP are typical RP. All atypical RP are ARRP and SRP. Non-pigmented RP are mainly seen in atypical RP which often misdiagnosed as amblyopia during childhood. The photoreceptors in macula are damaged in the early stage and the decline of visual acuity occurred at 21 to 30 years of age in patients with ARRP and XLRP. The ADRP patients has late slower decline of visual acuity and retain some visual acuity at the age older than 51 years.
ObjectiveTo observe the clinical features and visual prognostic factors of ethambutol-induced optic neuropathy (EON).MethodsA cohort study. Twenty-four inpatients (46 eyes) identified as EON in Neuro-Ophthalmology Department of Chinese PLA General Hospital from January 2014 to December 2017 were enrolled, including 14 males (26 eyes) and 10 females (20 eyes) with a ratio of 1.4/1 male/female. The average age was 42.79±15.12 years and the average weight was 62.46±12.31 kg. The average time duration between oral administration of ethambutol and occurrence of EON was 9.94±16.49 months. The average time of ethambutol duration was 7.06±11.68 months, with an average accumulative dose of 156.7±1 779.0 g and the average daily dose of 15.07±8.95 mg/(kg·d). All patients were tested with visual acuity, fundus photos, colour vision, OCT, visual field, VEP, orbital MRI and the gene of OPA1 and mitochondrial deoxyribonucleic acid (mtDNA). All the patients accepted drug withdrawal immediately after diagnosis, and were given the treatment of systemic nerve nutrition and improvement of microcirculation for 2 weeks. The time of follow-up was more than 12 months. According to whether the visual acuity (VA) in any of eyes was over than 0.1 at the last follow-up, all the patients were divided into two groups: the bad VA group (VA less than or equal to 0.1) and the better VA group (VA over than 0.1) group. The χ2 test and Fisher's exact probabilistic method test were used to compare the counting data between groups, and the Wlincox rank sum test was used to compare the measurement data. Multiple factors of VA outcome between the patients with bad or better va were analyzed by logistic regression.ResultsThirty eyes (65.2%) had VA less than or equal to 0.1 and 5 eyes (10.9%) had VA over than 0.5 at EON onset. The VA of the rest 11 eyes (23.9%) was higher than 0.1 and lower than 0.5. At the last follow-up, 20 eyes (43.5%) had VA less than or equal to 0.1 and 9 eyes (19.6%) had VA over than 0.5, the VA of the rest 17 eyes (36.9%) was higher than 0.1 and lower than 0.5. Fundus examination revealed 7 eyes (15.3%) with optic disc edema. OCT revealed significant loss of the retinal nerve fiber layer (RNFL) in the affected eyes, mainly in the temporal RNFL of the optic disc. All patients had dyschromasia, mainly in distinguishing the color of red and green. The types of visual field defect was as following: central dark spot (52.2%), diffuse visual acuity decreased (30.4%), temporal hemianopsia (17.4%). Orbital MRI revealed that 12/24 (50.0%) patients had T2 lesions with T1 enhancement in 6/24 patients (25.0%). Genetic test showed that 4 patients (16.7%) had gene mutation. Among them, there were 2 patients with OPA1 mutation, 1 with mtDNA 14340 point mutation and 1 with the mtDNA 11778 point mutation. Thirteen patients showed better VA outcomes (over than 0.1) while 11 showed bad VA outcomes after discontinuation of ethambutol. Between the better VA group and the bad VA group, there were statistically significant differences in the daily dose of ethambutol and gene mutation (P=0.031, 0.023). The daily dose was related to visual prognosis of EON while only the daily dose of more than 18 mg/(kg·d) may lead to bad VA outcomes according to the logistic analysis (95% CI 0.007-0.736, OR=0.069, P=0.027).ConclusionsEON may have OPA1 and mtDNA mutation with more bilateral eyes involved and less optic edema, which about 43.5% of the patients showed irreversible visual impact. The daily dose of ethambutol is related to the vision recovery.
Objective To investigate the clinical characteristics of patients with polypoidal choroidal vasculopathy (PCV) from Central China . Methods This was a retrospective study, and 403 eyes of 362 patients diagnosed as PCV by ocular fundus photography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT) were enrolled. The general clinical data, fundus manifestation and ocular fundus examinations were statistically analyzed. Results Three hundred and sixty-two cases included 249 males (68.8%) and 113 females (31.2%). Age ranged from 45 to 91 years old, and mean age was (64.81plusmn;9.31) years old. Bilateral lesions were observed in 41 patients (11.3%) and unilateral lesions were observed in 321 patients (88.7%)． In these 403 eyes, typical orangered lesions were observed in 162 eyes (40.2%); yellowishwhite exudate could be found in 185 eyes (45.9%); 268 eyes (66.5%) showed variable degrees of subretinal hemorrhage. Drusen was found in 23 eyes (5.7%), pigment proliferation in 20 eyes (5.0%) and fiber vascular scar in 96 eyes (23.8%). The lesions of 386 eyes (95.8%) located in macular region, 53 eyes (13.2%) in peripapillary area. Lesions presented multifoci in 67 eyes (16.6%). Three hundred and four eyes (75.4%) presented typical polypoidal lesions and 152 eyes (37.7%) with abnormal branching choroidal networks. Hemorrhagic retinal pigment epithelial detachments (PED) were found in 200 eyes (49.6%) and serous PED in 96 eyes (23.8%), both existed in 25 eyes（6.2%）. OCT showed 56 eyes (13.9%) presented cystoid dark chamber between the neurosensory retina and 109 eyes (27.0%) with double-layer sign formed by the separation of retinal pigment epithelium and Bruchprime;s membrane (27.0%). Two hundred and seventy-four eyes (68.0%) were found with conelike elevation beneath the RPE layer and 151 eyes (37.6%) with neurosensory detachment. Conclusions In Central China, the majority of PCV patients were male, unilateral. Most PCV lesions were located in the macula. Subretinal hemorrhage, polypoidal lesions and abnormal choroidal vascular networks were common in the PCV patients. Hemorrhagic PED presented a higher ratio than serous PED.
Objective To observe the clinical features of combined central retinal artery and vein occlusion. Methods The clinical data of eight patients of combined central retinal artery and vein occlusion diagnosed by fundus examination and fundus fluorescein angiography (FFA) was analyzed retrospectively, including the causes, fundus manifestations and FFA features. Results 4/8 patients had hypertension and dyslipidemia, 2/8 patients had traumatic retrobulbar hemorrhage, one patient had orbital cellulitis and one patient had systemic lupus erythematosus. All the patients had posterior pole retinal edema, hemorrhage, thin retinal artery, dilated vein, and papilledema. FFA showed delayed arterial filling, and there was no filling of retinal arterial branches until the late stage of FFA. Laminar flow delayed in large retinal veins, and there was no filling or only retrograde filling in retinal vein branches. Large areas with dot-like or patchy weak choroidal fluorescence can be observed in five patients. Conclusions Combined central retinal artery and vein occlusion is rare with complex etiology. The fundus manifestations and FFA features are atypical, but have features of central retinal artery occlusion and central retinal vein occlusion.
Objective To investigate the characteristics of fundus photography and fundus fluorescein angiography (FFA) of IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome and Eales disease. Methods The fundus photography and FFA data of 4 cases (8 eyes) with IRVAN syndrome and 43 cases (68 eyes) with Eales disease were retrospectively analyzed. All patients received ophthalmic routine examinations, including visual acuity, intraocular pressure, slit-lamp microscope and indirect ophthalmoscope. All patients had taken fundus photography and FFA for both eyes, except 4 patients of Eales disease who had vitreous hemorrhage in one eye. All 4 cases(1 male/3 female )with IRVAN syndrome were bilateral and aged 1643 years old( mean age 2700plusmn;1293 years old). 43 cases (32 male/11 female) of Eales disease aged 6-59 years old( mean 30.79plusmn;11.46 years old), 29 cases were bilateral and 14 cases were unilateral. Both diseases had retinal vascular whitesheath or white threadlike changes, exudative retinal hemorrhage and vitreous hemorrhage. Results Both arteries and veins of posterior pole of all eyes with IRVAN syndrome were involved and shown multiple retinal macroaneurysms. Other signs of IRVAN syndrome included capillary occlusion and nonperfusion (7/8 eyes, 87.5%),fluorescein leakage and edema of optic disc (5/8 eyes,62.5%), optic atrophy(2/8 eyes,25%), vitreous hemorrhage(1/8 eyes,12.5%), neovascularization of optic disc(2/8 eyes,25%), retinal neovascularization(4/8 eyes,50%) and macular edema(4/8 eyes,50%). The signs of Eales disease included fluorescein leakage of peripheral retinal vein (68/68 eyes, 100%), fluorescein leakage of posterior retinal vein (32/68 eyes, 47.06%), artery involvement (5/68 eyes, 7.35%), peripheral capillary occlusion and nonperfusion (38/68 eyes, 55.88%), fluorescein leakage of optic disc(29/68 eyes, 42.65%), neovascularization of optic disc(4/68 eyes,5.88%), retinal neovascularization(26/68 eyes,38.2%) and macular edema(15/68 eyes,22.06%). Compared IRVAN syndrome with Eales disease, the difference of artery inflammation, vein inflammation, retinal macroaneurysms in posterior area had statistics significance(all P=000,Plt;005), and that of edema of optic disc, retinal vascular nonperfusion area, neovascularization of optic disc, neovascularization elsewhere, and macular edema had no statistics significance(chi;2=0.479，P＞0.05；P=0.131，P＞0.05；chi;2=1.449，P＞0.05；chi;2=0.068，P＞0.05；chi;2=1.676，P＞0.05). Conclusions Both IRVAN syndrome and Eales disease may have vein and artery inflammation in posterior pole of the eye, and may result in neuroretinitis. IRVAN syndrome has much more vein and artery inflammation in posterior pole than Eales disease. Posterior retinal macroaneurysms is the most important sign for the diagnosis and differential diagnosis of IRVAN syndrome and Eales disease.
ObjectiveTo observe the clinical characteristics of retinoblastoma (RB) in Southwest China.MethodsA retrospective clinical study. From January 2010 to December 2017, 66 RB patients diagnosed in Ophthalmology Department of West China Hospital of Sichuan University were included in the study. All the patients underwent ocular B-ultrasound, orbital CT or MRI examination. Ten patients underwent RetCam examination at the same time. Twenty-nine patients were diagnosed by histopathological examination, and 37 patients were diagnosed by clinical symptoms and imaging examination. According to whether the tumor invaded the orbit and optic nerve, it could be divided into extraocular stage and intraocular stage. Intraocular tumors were divided into A-E stages according to the international intraocular RB classification. Treatments were performed according to different stages. The general information, age at diagnosis, course of diseases (the time between onset symptoms and diagnosis), causes of visiting a doctor, classification, treatment methods and eyeball preservation rate were retrospectively analyzed.ResultsPatients all came from Southwest China (56 patients from Sichuan Province, 2 patients from Yunnan Province, 2 patients from Guizhou Province, and 6 patients from Tibet). The permanent residence were identified in 43 patients, including 27 patients (62.8%) from rural areas. There were 38 males (57.6%); 50 unilateral tumors (75.8%) and 16 bilateral tumors (24.2%); 51 first-visiting patients (77.3%) and 15 re-visiting patients (22.7%). The average diagnostic age of first-visiting patients was 20.9±14.4 months, with 23.2±14.7 and 11.2±7.6 months for unilateral and bilateral tumors, respectively. There were 41 patients had definite course and causes, of whom the average course was 90.6±115.2 days. The most common cause was leucocoria in 32 patients (62.7%), followed by redness and swelling in 4 patients (9.8%), and other causes in 5 patients (12.2%). Among the 15 re-visiting patients, the average diagnostic age was 63.6±46.8 months, the average course was 32.8±45.5 months. Recurrence was occurred in 5 patients (33.3%), leucocoria in 4 patients (26.7%), postoperative complication in 3 patients (20.0%), protrusion in 2 patients (13.3%) and redness in 1 (6.7%) patient, respectively. Fifty out of 82 eyes were admitted to hospital, including 37 eyes of first-visiting patients and 13 eyes of re-visiting patients. Among 37 first-visiting eyes, there were 5 eyes (13.5%) in stage A-C, 26 eyes (70.3%) in stage D-E, 6 eyes (16.2%) in extraocular stage. Five eyes in stage A-C were treated with laser photocoagulation and (or) cryotherapy combined with systemic chemotherapy. Four eyes in stage D were treated with intraocular arterial chemotherapy. Nineteen eyes (51.3%) were performed with enucleation, 2 eyes (5.4%) with evisceration and 7 eyes (18.9%) abandoned treatment. Among 13 re-visiting eyes, 6 eyes (46.2%, with 5 eyes of recurrence) had been enucleated before, 4 eyes (30.8%) were in extraocular stage and 3 eyes (23.1%) in stage D-E. Five eyes (38.5%) were treated with evisceration, 4 eyes (30.8%) with enucleation, 1 eye with oculoplastic surgery and 3 eyes (23.1%) abandoned treatment. The rate of eye preservation was 18.0%, 29.0% for intraocular stage and 0% for extraocular stage, respectively.ConclusionRB patients in Southwest China have a longer course between onset symptoms and diagnosis, more advanced classification and lower rate of eye preservation.
Objective To compare neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV) in age at diagnosis, gender and disease laterality. Methods One hundred fourteen nAMD patients (114 eyes) and 145 PCV patients (186 eyes) diagnosed by fundus fluorescein angiography (FFA) and indocyanine green angiography were enrolled in this retrospective study. The age at diagnosis, gender and disease laterality of all the patients were collected. Independent sample t-test, chi;2 test and Fisher's exact test were used to compare the age at diagnosis, gender and disease laterality between nAMD and PCV patients. Results The mean age at diagnosis of nAMD group and PCV group were (68.30plusmn;9.86), (65.67plusmn;9.04) years respectively, the difference was statistically significant (t=-2.168, P=0.031). The patients under 70 years old accounts for 50.88% in nAMD group, which is lower than that in PCV group (63.45%), the difference was statistically significant (chi;2 =4.138, P=0.042). The male/female ratio of nAMD group and PCV group were 3.56∶1 and 2.02∶1 respectively, the difference was statistically significant (chi;2 =3.937, P=0.047). Thirty patients (26.32%) and 41 patients (49.46%) were affected unilaterally in nAMD and PCV group, respectively. The difference of bilateral incidence between two groups was not statistically significant (chi;2 =0.123, P=0.726). There were 69 right eyes (47.92%) and 75 left eyes (52.08%) in nAMD group, 92 right eyes (49.46%) and 94 left eyes (50.54%) in PCV group. The difference of disease laterality between two groups was not statistically significant (chi;2 =0.078，P=0.637). Conclusions PCV patients present at younger age than nAMD. nAMD is more prone to affected males than PCV. Nearly a quarter patients are bilateral in nAMD or PCV, there is no difference in bilateral incidence between these two diseases.
Objective To observe the clinical features and outcomes of vitrectomy for diabetic retinopathy (DR) with central retinal vein occlusion (CRVO) in type 2 diabetes mellitus (T2DM). Methods A total of 192 patients (241 eyes) with proliferative DR (PDR) who underwent vitrectomy were enrolled in this study. All the patients were diagnosed as vitreous hemorrhage (VH) because of suddenly decreased vision. There were 93 eyes with tractional retinal detachment (TRD) and six eyes with neovascularization of iris (NVI). The patients were divided into PDR with CRVO group (group A, 41 eyes) and PDR group (group B, 200 eyes) according to the results of fundus examination. All patients received vitrectomy with silicone oil and C3F8 gas tamponade. There were 138 eyes with silicone oil tamponade which including 30 eyes in group A and 108 eyes in group B. The difference of number in silicone oil-filled eyes in two groups was statistically significant (chi;2=5.110,P＜0.05). There were 38 eyes with C3F8 gas tamponade which including six eyes in group A and 32 eyes in group B. There was no difference in C3F8 gas-filled eyes numbers in two groups (chi;2=0.048, P＞0.05). The follow-up ranged from one to 60 months, with the mean of (28.69plusmn;17.28) months. The corrected vision, retinal reattachment, persisting macular edema (ME), neovascular glaucoma (NVG) and repeated VH after surgery were comparatively analyzed. Results Of 241 eyes, there were 41 eyes (17.0%) with CRVO. Before surgery, the differences of corrected vision (Z=-0.138), intraocular pressure (t=0.966), whether there was TRD or not (chi;2=0.412), whether underwent panretinal photocoagulation or not (chi;2=1.416) were not statistically significant (P＞0.05), but the difference of whether NVI were present or not was statistically significant (chi;2=31.724,P＜0.05) between two groups. After surgery, the corrected vision improved in both two groups (Z=2.319, 4.589; P＜0.05). There was no difference of corrected vision after surgery between two groups (Z=0.782,P＞0.05). Postoperative complications occurred in 94 eyes, including 26 eyes in group A and 68 eyes in group B. The differences of incidence of reoperation (chi;2=0.498), retinal reattachment (chi;2=0.818), persisting ME (chi;2=2.722) between two groups after surgery were not statistically significant (P＞0.05). The incidence of repeated VH (chi;2=5.737) and NVG (chi;2=6.604) in group A were higher than those in group B (P＜0.05). Conclusions CRVO is commonly found to coexist with DR in T2DM patients with VH. Combined with CRVO patients are more likely to suffer NVI. Vitrectomy can improve the visual function in PDR with CRVO patients.
