ObjectiveTo analyze and discuss the clinical features and management of pediatric retinal detachment (RD) associated with morning glory syndrome (MGS). MethodsThe clinical data of 49 patients (51 eyes) with RD associated with MGS were retrospectively analyzed, including 27 males (27 eyes) and 22 females (24 eyes). The age at first diagnosis ranged from 1 week after birth and 13 years old (4.2±3.2) years. There were 33 eyes of exudative RD, 3 eyes of rhegmatogenous RD and 15 eyes of RD with undetermined cause. Twenty eyes of 20 patients had other congenital ocular abnormalities, including persistent hyperplastic primary vitreous, microphthalmia, choroidal coloboma, iris coloboma. Besides retinal detachment, other complications were found, including cataract, secondary glaucoma,corneal leukoma or edema, strabismus and nystagmus. Twenty-two cases (22 eyes) received treatment. Five cases of mild exudative retinal detachment took oral methazolamide tablets. Three eyes with mild and restricted retinal detachments got retinal laser photocoagulation around the optic disc. Fourteen cases of 14 eyes underwent surgery including vitrectomy, lensectomy, and phacoemulsification. Follow-up after treatment were three months or more, with an average of (20.3±11.8) months. The visual acuity, retinal reattachment and intraocular pressure and other complications after treatment were followed up. ResultsFive patients of exudative retinal detachment were remained stable by taking methazolamide tablets. Two of the 3 eyes remained stable after laser therapy; however, the remaining one eye was not controlled after relaser. Cataract and glaucoma were resolved by lensectomy in 7 eyes and phaco-emulsification surgery in 1 eye. Retina was re-attached in 6 eyes after vitrectomy. Among those treated, 10 eyes had records of visual acuity. Visual acuity was improved in 1 eye after laser therapy and remained stable in 9 eyes. There were no drugs and surgery-related complications. ConclusionsRetinal detachment was the main complication of MGS. The high incidence and poor prognosis call for the need of close follow-up and timely treatment, including medicine, laser and surgery.
Objective To observe the clinical manifestations and the effect factors of thyroid-associated ophthalmopath (TAO) with optic neuropathy. Methods The clinical data of 62 cases (120 eyes) of TAO with optic neuropathy diagnosed in 1994-2001 were retrospectively analyzed. Results The incidence of TAO with optic neuropathy was 18.3% in all the simultaneous TAO inpatients, and was more frequently found in the male than in the female ones. The incidence of TAO with optic neuropathy was correlated with orbitono-increase, ocular myopathy, hypertension, hyperlipemia, diabetes, and cardiopathy (P＜0.0000), but not with exophthalmos, intraocular pressure, and the disease course of TAO (Pgt;0.05). In 62 patients with the methylprednisolone pulse therapy, the visual acuity improved in 33 (29.0%), improved while the treatment but decreased after stop treating in 29 (24.2%), and no changes in 58 (46.8%). In 63 eyes undergone orbital decompression with methylprednisolone pulse therapy, 59 (93.7%) had better visual acuity. Conclusion The incidence of TAO with optic neuropathy was correlated with orbitono-increase, ocular myopathy, hypertension, hyperlipemia, diabetes, and cardiopathy. Met hylprednisolone pulse therapy and orbital decompression are the effective measures for TAO with optic neuropathy. (Chin J Ocul Fundus Dis，2004，20：142-144)
Objective To observe the characteristics of changes of 24hour ambulatory blood pressure and heart rate of 50 patients with anterior ischemic optic neuropathy (AION). Methods Fifty patients with AION and the persons without in the control group, which had the same number, gender and age as the patients with AION, underwent 24-hour ambulatory blood pressure and heart rate measurement. Results Both groups had no difference in mean blood pressure and heart rate during the daytime (t=1.25,0.93; P＞0.05), higher than those in the nighttime (t=3.63,3.16; P＜0.05). Mean blood pressure and heart rate of AION group at night were lower than those of the control group (t=3.82,1.77; P＜0.01,0.05), especially diastolic pressure of AION group was lower than that of the control group from 2 am to 7 am (P＜0.01), as well as the heart rate from 2 am to 5 am (P＜0.05 or P＜0.01). The curves of blood pressure of AION group showed more gradual and fluctuant rising, while those of the control group showed sharper and less fluctuant rising. Conclusion According to the curves of blood pressure rising, the patients with AION may have some defects in auto-regulatory mechanism of blood pressure. The low spots of blood pressure and heart rate in early morning, which might be a critical point leading to AION. (Chin J Ocul Fundus Dis, 2002, 18: 259-261)
Objective To investigate the spectrum of mitochondrial DNA (mtDNA) mutations in Chinese patients with Leber′s hereditary optic neuropathy (LHON). Methods The primary mtDNA mutations (G3460A、G11778A and T14484 C) of 140 patients with LHON were detected by mutation-specific priming polymerase chain reaction (MSP-PCR), heteroduplex-single strand conformation polymorphism polymerase chain reaction (HA-SSCP), restriction fragment length polymorphisms (RFLP) and measurement of DNA sequence. The transmissibility of the patients′ stirps was analyzed.Results In the 140 patients with LHON, G11778A mtDNA primary mutation was found in 130 (92.9%), including 113 males and 17 females; G3460A mutation was found in 2 (1.4%) including 1 male and 1 female; G14484A mutation was found in 8 (5.7% ) including 6 males and 2 females.Conclusion In Chinese patients with LHON, the incidence of G11778A mtDNA mutation is higher than that of G3460A and T14484C. (Chin J Ocul Fundus Dis，2003，19：269-332)
Infiltrative optic neuropathy (ION) is characterized by the infiltration of tumor cells or inflammatory cells in the optic nerve and its sheath. ION is rare in clinic practice, and ION caused by direct infiltration or metastasis of malignant tumors is easily misdiagnosed as optic perineuritis or optic neuritis, which means delayed proper treatment and makes patients risking possible side effects of steroid therapy. Currently, ophthalmologists are lack of sufficient knowledge about ION, which contributes to the high rate of misdiagnosis and missed diagnosis of ION in clinical practice. The diagnosis and treatment of ION have not yet formed systematic standardized guidelines. Therefore, Neuro-ophthalmology Group of Ophthalmology Branch of Chinese Medical Association organized experts to propose consenus of opinions on definition, diagnosis and treatment of ION, which helps to guide clinical diagnosis and treatment of ION, as well as basic researches about ION.
Ras homolog family (Rho)/ Rho-associated coiled-coil kinase (ROCK) signaling pathway widely exists in human and mammal cells, which is closely related to inhibition of repair after optic nerve damage. The expression level of Rho/ROCK signaling pathway-related proteins is up-regulated in glaucoma, and related with the death of retinal ganglionic cell (RGC) and the axon activity. ROCK inhibitors can protect the surviving RGC and promote axon extension with a dose-dependent manner. ROCK inhibitors also can inhibit glial scar formation, lower intraocular pressure and inhibit inflammatory response to some degrees. Rho/ROCK signaling pathway correlates with the optic nerve disease progression, and ROCK inhibitors hope to become a new therapeutic drug.
Objective To analyze the new primary mutation in Chinese people with Leberprime;s hereditary optic neuropathy (LHON). Methods Genomic DNA was collected from 260 suspected LHON patients and 100 normal healthy persons. The mitochondria DNA mutation at nucleotide position (NP) 15257 and the hot spot (14452-14601 bp) of ND6 gene which include the mutations at NP (14482, 14498, 14568, 14596, 14495, and 14459) were screened by using polymerase chain reaction (PCR), heteroduplex-single strand conformation polymorphism (HA-SSCP) and restriction fragment length polymorphism (RFLP) analysis and sequencing. Primary mutation spectrum of Chinese race was analyzed. Results Eight kinds of polymorphism of mitochondria DNA were found in 260 suspected LHON patients and 100 normal healthy persons, including NP 14488C, 14518G, and 14617G which hadnrsquo;t been reported (http://www.mitomap.org/). No mutation at NP 15257, 14482, 14498, 14568, 14596, 14495, and 14459 was found. Conclusion The NP 15257A may not be the primary mutation in Chinese. Because of the race difference, 14452-14601 bp in ND6 gene may not be the hot spot in Chinese patients with LHON, and other hot spots may exist. (Chin J Ocul Fundus Dis, 2006, 22: 82-85)
Optic nerve diseases seriously affect visual function, and early accurate diagnosis and effective follow-up are very important for treatment and prognosis. Optical coherence tomography (OCT) and OCT angiography (OCTA) are non-invasive and high-resolution imaging techniques, which play increasingly important roles in the diagnosis and treatment of optic nerve diseases. OCT can visually display the structure of retinal nerve fiber layer and macular area, accurately measure the thickness of nerve fiber layer and structural parameters of macular area. OCTA can clearly display the changes of microblood flow around optic disc and retinal blood vessels. The combined use of these two technologies will not only help diagnose and monitor optic nerve diseases, but also deepen our understanding of the pathogenesis of optic nerve diseases. In view of the fact that the application of OCT and OCTA in neuro-ophthalmic diseases involving the optic nerve is still in the development stage in the domestic medical community, it is urgent to formulate a guiding document to regulate and promote the application of these two technologies. To this end, based on a systematic literature review and combined with the current clinical practice of OCT and OCTA in China, we formulated the Expert consensus on the clinical application of optical coherence tomography and angiography in optic nerve diseases. This consensus comprehensively expounds the technical principles and main measurement indicators of OCT and OCTA, the specific application, examination specifications and limitations of OCT and OCTA in clinical diagnosis and follow-up of neuroophthalmic diseases involving optic nerve, aiming to improve the application level of OCT and OCTA by doctors, especially neuroophthalmologists, and better play the role of this advanced imaging technology in neuroophthalmology.
Objective To observe the characteristics of indocyanine green angiography and fundus fluorescein angiography (ICGA amp; FFA) in anterior ischemic optic neuropathy ( AION ) , and to investigate the etiology of AION and the value of ICGA and FFA in the diagnosis and study of AION. Methods Simultaneous ICGA and FFA were performed on 32 eyes of 31 AION patients and 38 eyes of 38 non-AION patients. Results The eyes that all or a part of the optic disclies in the watershed zon es of the choroidal blood supply were significantly more in the AION group tha n those in the non-AION group (Plt; 0.005 ) . All the watershed zones through the optic disc were vertical in shape. The fluorescence filling of the inferior and superior choroidal blood vessels near the optic disc were all slower than that of the temporal and nasal choroidal blood vessels. The types of watershed zones in the AION group were highly correspondent to the ischemic areas of the optic disc . In early ICGA , no fluorescence appeared at the optic disc, and in later stage, the ischemic area revealed no fluorescence, and the other area showed mostly asymmetric fluorescence. Conclusion The onset of AION is correlative to the choroidal blood supply around the optic disc. ICGA is more valuable in the diagnosis and study of AION than FFA . (Chin J Ocul Fundus Dis, 2001,17:111-114)
Optical coherence tomography (OCT) can depict mild papilledema and slight changes in the internal segment and external segment; measure thickness of the retinal nerve fiber layer, retinal thickness and macular volume; detect missing axons and damage to the macular ganglion cell complex. Thus, OCT has important application values and widespread prospects in diagnosis and differential diagnosis of glaucoma and nonglaucoma optic neuropathy, optic nerve diseases and macular diseases, outer and inner retinopathy as well as evaluation of curative effects, followup observation, prognosis and mechanisms in neuroophthalmological diseases. Neuro-ophthalmologists should pay more attention to the exploration and application of OCT in the field of neuro-ophthalmology.