ObjectiveTo investigate the clinical characteristics and surgical management of the familial exudative vitreoretinopathy (FEVR)-associated rhegmatogenous retinal detachment (RRD). MethodsRetrospective noncomparative case series. Thirty-three eye of 32 patients were diagnosed FEVR-associated RRD by Fluorescein fundus angiography. There were 26 males and 6 females. The male to female ratio is 4.3:1 with an average age of 19.35±8.83 years. The detection of best corrected visual acuity (BCVA), refraction status, fundus photograph and fluorescein fundus angiography(FFA)were underwent in all patients. FEVR was confirmedby FFA and positive family history. The BCVA, refraction status, morphology of retinal detachment, location, size and shape of retinal hole, presence and grade of proliferative vitreoretinopathy (PVR), and subretinal proliferation were recorded. ResultsAs for the refractive status, the scope of refraction was +2.0 D to-13.0 D andthe BCVA were range from light perception to 0.7. Atrophy holes which located at the temporal half were responsible for retinal detachment in all cases. Besides, horseshoe tears were noted in 6 eyes (18.18%), while macular tears were noted in 2 eyes of RRD (6.06%). The PVR greater than stage C2 was noticed in 10 eyes (30.30%), while subretinal proliferation was presented in 23 eyes (69.70%). ConclusionsMale predominance, juvenile onset and associated with moderate to high myopia are the main characteristics in FEVR-associated RRD. Atrophy holes at the temporal half and the subretinal proliferations were most commonly in FEVR-associated RRD. Detailed fuduns and FFA examination of the fellow eye should be undergone to avoid misdiagnosis.
Objective To observe the efficacy of vitrectomy for vitreous hemorrhage in patients with polypoidal choroidal vasculopathy (PCV). Methods Fourteen patients (14 eyes) of PCV with vitreous hemorrhage diagnosed by routine ophthalmologic examination, A and/or B mode ultrasound, fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were enrolled in this study. The patients included eight males (eight eyes) and six females (six eyes), with the mean age of (58.7plusmn;6.0) years. All patients received vitrectomy with silicone oil and C3F8 gas tamponade. There were eight eyes received photodynamic therapy (PDT) after surgery. The retinal reattachment, visual acuity, pathological lesion degree and complications were comparatively analyzed. Results Among 14 eyes, six eyes (42.9%) recovered, seven eyes (50.0%) improved, and one eye (7.1%) aggravated. Ten eyes achieved retinal reattachment after surgery, while four eyes developed retinal detachment after the first surgery. The retina remained attached in these three eyes after silicon oil tamponade, C3F8 gas tamponade and scleral buckling, respectively; but one eye maintained silicon oil without special treatment. Thirteen eyes (92.9%) achieved retinal reattachment finally. Five eyes of them occurred hyphema one to seven days after surgery, but hyphema was absorbed and intraocular pressure was stable after douche of anterior chamber and pharmacotherapy. The vision improved with more than two lines in one eyes, improved with one to two lines in one eye, unchanged in 10 eyes, and decreased in two eyes. Of eight eyes who underwent PDT, abnormal vessels regressed in five eyes, abnormal vessels remained in three eyes. Conclusions Vitrectomy can remove cloudy refracting media for PCV with vitreous hemorrhage. The combined treatment of vitrectomy and PDT can improve or stabilize visual function,is an effective therapy for the PCV with vitreous hemorrhage.
Objective To investigate the clinical characteristic of ocular fundus complications in systemic lupus erythematosus (SLE). Methods In 25 cases of SLE with the ocular fundus complications, the ocular fundus, the other ocular tissues, general lesions，and antinuclear antibody (ANA ), anti-double-stranded DNA（anti-dsDNA）, complement 3 (C3), complement 4 (C4)and erythrocyte sedimentation rate(ESR) were analyzed retrospectively. Results In the 25 cases, “classic” SLE retinopathy in 15 (25 eyes), retinal vein occlusion (RVO) in 9 (12 eyes), RVO combined with retinal arter y occlusion in 1 (2 eyes), exudative retinal detachment in 1 (2 eyes), vitreous hemorrhage combined with neovascular glaucoma in 1 (1 eye), and optic discedem a except RVO in 3 (6 eyes) were found. Nine cases accompanied with other ocular signs and 21 with general lesions. Positive ANA and anti-dsDNA and elevated ESR in all of the patients, decreasing C3 in 19, and C4in 17 were found.Conclusions SLE can cause serious ocular fundus complications accompanied with other ocular signs. Regular ophthalmic examination should be performed on the patients with SLE to detect and treat the ocular complications promptly. (Chin J Ocul Fundus Dis，2004，20：206-208)
Objective To observe the prognosis of visual acuity (VA) of patients with different classification of polypoidal choroidal vasculopathy (PCV). Methods Sixty-seven PCV patients (68 eyes) diagnosed by fundus photography, fundus fluorescein angiography, indocyanine green angiography (ICGA) and ocular coherence tomography were enrolled in this retrospective study. The patients were classified into static, exudative, small hemorrhage and large hemorrhage according to activity and pathological characteristics of lesions. The patients were classified into aciniform, single or several single, combined branching choroidal vascular network (BVN) according to morphological characteristics and combination with BVN of lesions on ICGA. The patients also were classified into macular, vascular arcade, peripapillary and mixing zone according to distribution of lesions. The VA of all the types were observed. Results There were 16, 19, 19, 14 eyes in the type of static, exudative, small hemorrhage and large hemorrhage PCV, which with logMAR VA of 0.34plusmn;0.52, 0.70plusmn;0.98, 0.60plusmn;0.50, 0.91plusmn;0.75 respectively. The VA of static PCV patients was better than that in exudative, small hemorrhage and large hemorrhage PCV patients (q=4.75, 4.26, 5.13; P＜0.05). There was no significant difference of VA between exudative and small hemorrhage PCV patients (q=0.98, P＞0.05). There were 22, 38 and eight eyes in the type of aciniform, single or several single, combined BVN PCV, which with logMAR VA of 0.52plusmn;0.55, 0.59plusmn;0.43, 0.80plusmn;0.95 respectively. The VA of combined BVN PCV patients was worse than that in aciniform and single or several single PCV patients (q=3.81, 3.02;P＜0.05). There were 34, 13, 8 and 13 eyes in the type of macular, vascular arcade, peripapillary and mixing zone PCV, which with logMAR VA of 0.78plusmn;0.43, 0.57plusmn;0.37, 0.38plusmn;0.27, 0.74plusmn;0.41 respectively. The VA of macular PCV patients was less than that in vascular arcade and peripapillary PCV patients (q=4.61,5.11;P＜0.05). There was no significant difference of VA between macula and mixing zone PCV patients (q=0.73,P＞0.05). Conclusions The VA of PCV patients is variable.It is related to activity and pathological characteristics of lesions, morphological characteristics and combination with BVN of lesions on ICGA, and distribution of lesions.
Purpose Observation on changes of choroidal circulation in 21 cases (42 eyes) of retinal dystrophies. Methods Fundus fluorescein angiography (ICGA) were used for comparartive analyses of the retinal and choroidal blood circulation of 21 cases (42 eyes) of retinal dystrophic diseases. Results The changes of choroidal circulation,i.e.slow perfusion or filling defects were observed in four kinds of retinal dystrophies. Conclusion ICGA may assist in providing valuable informations on choroidal circulation of retinal dystrophic diseases. (Chin J Ocul Fundus Dis,1998,14:88-91)
Objective To evaluate the treatment of surgery and high-dose corticosteroid relevant factors to prognosis in traumatic optic neuropathy. Methods Forty patients(40 eyes) with traumatic optic neuropathy were enrolled.Optic nerve decompression using transcranial approaches,sinus endoscopy and orbital-ethmoidal sinus rout were performed in 14 patients.Eleven patients were treated with high-dose corticosteroids (5 cases with 1 mg/kg dexamethasone,6 cases with 30 mg/kg methylprednisolone) and 15 patients received nonspecific management chose by themselves.The outcomes of visual acuity in short term and final stage were compared between surgery,high-dose corticosteroid and nonspecific treatment.Multiple variable analysis was done to determine the factors affecting the outcome of visual acuity. Results No light perception were found in 19 cases (19 out of 44 cases,47.5%),whereas visual acuity was light perception to 0.02 in 12 cases (30.0%) and 0.05 or better in 9 cases (22.5%).The odds ratio of high-dose corticosteroid to nonspecific therapy was 2.96 (P=0.0125).The final visual acuity in patients treated with high-dose corticosteroid were better than other two groups (P=0.005,P=0.023,respectively).The short term (within 3 days) effective rate was higher in corticosteroid therapy group than operated group (P=0.024).No light perception following optic nerve trauma appeared to be more danger as 2.14 folds (P=0.0349) than those with light perception or better in term of final visual acuity outcome. Conclusions High-dose corticosteroid may be benefit to traumatic optic neuropathy.The treatment in traumatic optic neuropathy using optic nerve decompression needs to be determined.No light perception at initial is an important risk factor in the outcome. (Chin J Ocul Fundus Dis,2000,16:75-77)
Objective To investigate the imaging characteristics of patients with choroidal folds, which including ocular fundus, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT). Methods The clinical data of 62 eyes of 34 patients with choroidal folds were analyzed retrospectively. The patients include 10 patients(20 eyes) of VogtKoyanagiHarada syndrome, 1 patients(2 eyes) of Behcet diseases, 11 patients(21 eyes) of other uveitis, 5 patients (9 eyes) of papolloedema, 2 patients(2 eyes) of choroidal tumor, 2 patients(4 eyes) of, hypotony with macular degeneration, 1 patient(2 eyes) of,Graves diseases, 1 patient (1 eye) of,blunt trauma and 1 patient(1 eye) of uveal effusion syndrome. All patients underwent the examination of direct ophthalmoscope, fundus color photography and FFA, meanwhile, 9 patients (17 eyes) with ICGA examination, 9 patients(18 eyes) with OCT examination. Results Choroidal folds were bright and dark stripes on the fundus, their numbers were variable. They can be arranged radially, horizontally, oblique or concentrically around the macular area, or radiating from optic disk but rarely over equator region. On FFA there were more folds which were subjected to coarse folds and wrinkles. They were obvious at early stage and no leakage at late stage. On ICGA choroidal folds showed normal or hypofluorescence at early stage, and hyperfluorescence or hypofluorescence at late stage. The hyperfluorescence or hypofluorescence bands were corresponding to the hypofluorescence of FFA but not obvious as FFA. On OCT choroidal folds involved choriod and retinal pigment epithelial layer (RPEL). Conclusion Choroidal fold is a bright and dark stripes that involved choriod and RPEL. The angiography showed hypofluorescence bands without leakage. Be familiar with the imaging features of choroidal folds can help to found the choroidal folds and the original diseases.
Objective To observe the characteristics of multiple evanescent white dot syndrome (MEWDS) with modern multimodal imaging modalities. Methods This was a retrospective case study. Eleven patients (11 eyes) diagnosed with MEWDS were enrolled. There were 10 females and 1 male, mean age was 27.6 years (range 15-41 years). The period between disease onset and visiting to the hospital was between 2 to 13 days, the average time was 4.7 days. All the patients underwent examinations of best corrected visual acuity, slit-lamp biomicroscope, indirect ophthalmoscope, fundus color photography, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and spectral domain optical coherence tomography (SD-OCT). The mean follow up duration was 3.2 months. The imaging characteristics were compared. Results Fundus color photography showed foveal orange-red granularity in all eyes. FAF showed strong autofluorescence with a vague boundary. FFA showed a variable number of highly fluorescent fine needle-like dots arranged in a ring in the early stage, and fluorescence remained in the late stage. ICGA showed advanced lesions of vague boundary merged into a large plaque of deep retinal hypofluorescence. SD-OCT showed the hyperreflectant material deposit over the retinal pigment epithelium and extending anteriorly through the interdigitation zone, ellipsoid layer, and toward the external limiting membrane. At the site of extrafoveal lesions, SD-OCT revealed the presence of discontinuities or disruptions centered on the ellipsoid zone to include the interdigitation. Conclusions In MEWDS patients, fundus photography showed foveal orange-red granularity; FFA showed early fluorescent dots distributed in a ring pattern; ICGA showed hypofluorescent lesions in the later stage; SD-OCT showed disruption of the interdigitation zone and ellipsoid zone and accumulations of hyperreflective material that was of variable size and shape; FAF showed strong autofluoresce areas that correlated to spots observed with FFA and ICGA.
Familial exudative vitreoretinopathy (FEVR) is a hereditary retinal vascular dysplasia. So far, 6 genes have been found to be associated with FEVR: Wnt receptor Frizzled Protein 4, Norrie's disease, co-receptor low-density lipoprotein receptor-related protein 5, tetraspanin 12, zinc finger protein 408, and kinesin family members 11 genes. Its clinical manifestations, pathological processes and genetic patterns are diverse, and it shows the relationship between gene polymorphism and clinical manifestation diversity. It is characterized by different symptoms between the same individual, the same family, and the same gene mutation; different clinical stages and gene mutation types of parents or unilateral genetic children; different clinical characteristics and gene mutation patterns of full-term and premature infant; combined with other eye disease and systemic diseases; double gene mutations and single gene mutations have different clinical manifestations and gene mutation characteristics. A comprehensive understanding of the different clinical manifestations and diverse genetics of FEVR can provide better guidance for the treatment of FEVR.
Eye and nervous system are anatomically and physiologically very close. About 40% of the nerve fibers in brain are correlated with visual function. Seven of twelve cranial nerves are straightly correlated with visual afferent or eye movement. Neurological diseases can affect eye in many different ways. Furthermore, systemic disorders can cause secondary neurological lesions or even primary neurological disorders, which sequentially show ocular symptoms a nd signs. Better understanding of the close relationship between eye and brain, together with intimate cooperation between ophthalmologists, neurologists, neuro surgeons and other specialties, are the key point to improve our neuro-ophthalm ology service in China. (Chin J Ocul Fundus Dis,2008,24:82-85)