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Qi Zhang - One of the best experts on this subject based on the ideXlab platform.

  • peripheral retinal nonperfusion in pediatric patients with Morning Glory Syndrome
    Ophthalmic Surgery and Lasers, 2018
    Co-Authors: Kaiqin She, Qi Zhang, Ping Fei, Jie Peng, Jiao Lyu, Qiujing Huang, Peiquan Zhao
    Abstract:

    Background and objective To report the association of Morning Glory Syndrome (MGS) with peripheral retinal nonperfusion in pediatric patients with MGS. Patients and methods The authors retrospectively analyzed the records of pediatric patients with MGS using fundus fluorescein angiography. The peripheral retinal vascular architecture was recorded and graded according to the severity of peripheral retinal nonperfusion. Results Eighty-six eyes of 74 patients were enrolled. Seventy-three of 86 eyes (84.88%) had peripheral retinal nonperfusion, in which mild severity was found in 31 of 86 eyes (36.05%), moderate in 17 of 86 eyes (19.77%), severe in 18 of 86 eyes (20.93%), and extreme in seven of 86 eyes (8.14%). Secondary complications of nonperfusion included leakage in six of 73 eyes (8.22%), fibrovascular proliferation in two of 73 eyes (2.74%), and tractional retinal detachment in one of 73 eyes (1.34%). Conclusion There is a high prevalence of peripheral retinal nonperfusion in pediatric MGS eyes, with secondary complications in some, suggesting that more attention should be paid to the peripheral retina in MGS. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:674-679.].

  • the status and progress of Morning Glory Syndrome
    Chinese Journal of Ocular Fundus Diseases, 2017
    Co-Authors: Kaiqin She, Qi Zhang
    Abstract:

    Morning Glory Syndrome (MGS) is a congenital optic disc anomaly. The characteristic ophthalmoscopic findings consist of a generally enlarged, funnel-shaped and excavated optic disc, surrounded by an elevated annulus of chorioretinal pigment disturbance, with a central glial tuft, multiple narrow branches of retina vessels radiating from the disc. There are peripheral non-perfusion retinal areas in most cases. The pathogenesis of MGS remains unclear. MGS might be associated with many ocular and systemic abnormalities, involving facial, central nervous, cerebrovascular and endocrine systems. Persistent hyperplastic primary vitreous and retinal detachments (RD) are the most common ocular complications of MGS. The mechanism RD in MGS is unclear. Vitrectomy with long-acting gas or silicone tamponade and photocoagulation around the breaks or the enlarged disc might be efficient for rhegmatogenous RD of MGS. Early diagnosis is crucial for recognition and treatment of the ocular and systemic complications, and maintenance of the visual function. Key words: optic nerve disease/congenital; retinal detachment/treatment; photocoagulation; vitrectomy; association; treatment; review

  • fundus fluorescein angiography of pediatric Morning Glory Syndrome patients
    Chinese Journal of Ocular Fundus Diseases, 2015
    Co-Authors: Jie Peng, Qi Zhang
    Abstract:

    Objective To observe the fundus fluorescein angiography (FFA) manifestations of pediatric Morning Glory Syndrome (MGS) patients. Methods Fourteen eyes diagnosed as MGS of 14 patients were studied. Among the 14 cases, there were 7 male and 7 female patients. At the time of FFA, the mean age of the patients was (38.75±33.91) months old, ranging from 5.5 to 128.0 months. Among the 14 eyes, four (28.57%) were associated with persistent hyperplastic primary vitreous; four (28.57%) were associated with retinal detachment with no retinal breaks, and one (7.14%) was associated with peripapillary subretinal exudation. All patients underwent peripapillary laser photocoagulation under general anesthesia first and then FFA with the third generation of wide-angle digital retinal imaging system. The arm-retinal circulation time (A-RCT), numbers of blood vessels on the edges of optic disc of the MGS eyes and the contralateral healthy eyes, retinal vascular morphology, the peripheral avascular area, neovascularization, retinal detachment and other abnormalities were documented. The horizontal and vertical diameters of the optic disc of the affected eyes and the contralateral healthy eyes were measured. To compare the A-RCT, 16 children with normal FFA were selected as control group. Results The diameters of the vertical and horizontal axis of the affected eyes were as (2.56±0.58) and (2.73±0.60) times of the contralateral healthy eyes respectively. The average A-RCT of the affected eyes and eyes of the control group were (13.25±4.10) and (9.34±2.20) s respectively. The affected eyes had significantly prolonged A-RCT. At early stage, the optic disc and peripapillary areas showed hypo-fluorescence, while the irregular retinochoroidal atrophy area outside of the optic disk manifested as hyper-fluorescence ring. At late stage, optic disc showed hyper-fluorescence. Numbers of blood vessels on the edge of the optic disc of the affected eyes and contralateral healthy eyes were 30.27±4.86 and 15.83±1.95 respectively, the affected eyes had much more vessels than the contralateral healthy eyes. All affected eyes had peripheral retinal non-perfusion areas. Conclusion FFA examination showed prolonged A-RCT and peripheral retinal non-perfusion areas in the affected MGS eyes. Key words: Optic nerve diseases/ diagnosis; Optic disk/abnormalities; Fluorescein angiography

  • clinical analysis and management of pediatric retinal detachment associated with Morning Glory Syndrome
    Chinese Journal of Ocular Fundus Diseases, 2014
    Co-Authors: Ping Fei, Qi Zhang
    Abstract:

    Objective To analyze and discuss the clinical features and management of pediatric retinal detachment (RD) associated with Morning Glory Syndrome (MGS).Methods The 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.Results Five patients of exudative retinal detachment were remainedstable 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.Conclusions Retinal 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. Key words: Optic nerve diseases/congenital;  Retinal detachment/therapy;  Laser coagulation;  Vitrectomy

  • clinical characteristics and treatment of 22 eyes of Morning Glory Syndrome associated with persistent hyperplastic primary vitreous
    British Journal of Ophthalmology, 2013
    Co-Authors: Ping Fei, Qi Zhang, Peiquan Zhao
    Abstract:

    Purpose To describe the clinical manifestations and treatment outcomes in a retrospective case series of Morning Glory Syndrome (MGS) associated with persistent hyperplastic primary vitreous (PHPV). Methods The medical records of 85 eyes/74 patients referred for ophthalmology consultation diagnosed as MGS in our clinic were reviewed retrospectively. All patients underwent thorough ophthalmological examinations. 22 eyes of 19 patients diagnosed as having MGS associated with PHPV were included, accounting for 25.88% of all the MGS eyes. Clinical manifestations and management of these patients were documented. Results 15 patients (78.95%) were younger than 1 year old at the first diagnosis. Six eyes were associated with microphthalmia. 19 of 22 eyes (86.36%) had complications, including cataract (10 eyes), secondary glaucoma (8 eyes), corneal leucoma or oedema (8 eyes), retinal detachment (8 eyes), strabismus (3 eyes) and nystagmus (2 eyes). Treatment methods varied depending on the severity of the complications. Nine eyes with secondary glaucoma or cataract got lensectomy; three eyes underwent combined vitrectomy and lensectomy. Eight patients underwent cranial MRI/MR angiography or CT examination. Widened cerebral fissures of bilateral temporal lobes, abnormal dilated branch of middle cerebral artery in the left hemisphere and abnormal signal in the grey matter of frontal and occipital lobes were revealed respectively in three patients. Conclusions Our study revealed the coexistence of PHPV in a significant percentage of patients with MGS, suggesting a potential common genetic link. Compared with MGS and PHPV alone, the combination of the two conditions manifested with higher incidence and more severe complications in younger patients. Close follow-up was recommended. Lensectomy and vitrectomy were beneficial in the management of the complications.

Peiquan Zhao - One of the best experts on this subject based on the ideXlab platform.

  • peripheral retinal nonperfusion in pediatric patients with Morning Glory Syndrome
    Ophthalmic Surgery and Lasers, 2018
    Co-Authors: Kaiqin She, Qi Zhang, Ping Fei, Jie Peng, Jiao Lyu, Qiujing Huang, Peiquan Zhao
    Abstract:

    Background and objective To report the association of Morning Glory Syndrome (MGS) with peripheral retinal nonperfusion in pediatric patients with MGS. Patients and methods The authors retrospectively analyzed the records of pediatric patients with MGS using fundus fluorescein angiography. The peripheral retinal vascular architecture was recorded and graded according to the severity of peripheral retinal nonperfusion. Results Eighty-six eyes of 74 patients were enrolled. Seventy-three of 86 eyes (84.88%) had peripheral retinal nonperfusion, in which mild severity was found in 31 of 86 eyes (36.05%), moderate in 17 of 86 eyes (19.77%), severe in 18 of 86 eyes (20.93%), and extreme in seven of 86 eyes (8.14%). Secondary complications of nonperfusion included leakage in six of 73 eyes (8.22%), fibrovascular proliferation in two of 73 eyes (2.74%), and tractional retinal detachment in one of 73 eyes (1.34%). Conclusion There is a high prevalence of peripheral retinal nonperfusion in pediatric MGS eyes, with secondary complications in some, suggesting that more attention should be paid to the peripheral retina in MGS. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:674-679.].

  • clinical characteristics and treatment of 22 eyes of Morning Glory Syndrome associated with persistent hyperplastic primary vitreous
    British Journal of Ophthalmology, 2013
    Co-Authors: Ping Fei, Qi Zhang, Peiquan Zhao
    Abstract:

    Purpose To describe the clinical manifestations and treatment outcomes in a retrospective case series of Morning Glory Syndrome (MGS) associated with persistent hyperplastic primary vitreous (PHPV). Methods The medical records of 85 eyes/74 patients referred for ophthalmology consultation diagnosed as MGS in our clinic were reviewed retrospectively. All patients underwent thorough ophthalmological examinations. 22 eyes of 19 patients diagnosed as having MGS associated with PHPV were included, accounting for 25.88% of all the MGS eyes. Clinical manifestations and management of these patients were documented. Results 15 patients (78.95%) were younger than 1 year old at the first diagnosis. Six eyes were associated with microphthalmia. 19 of 22 eyes (86.36%) had complications, including cataract (10 eyes), secondary glaucoma (8 eyes), corneal leucoma or oedema (8 eyes), retinal detachment (8 eyes), strabismus (3 eyes) and nystagmus (2 eyes). Treatment methods varied depending on the severity of the complications. Nine eyes with secondary glaucoma or cataract got lensectomy; three eyes underwent combined vitrectomy and lensectomy. Eight patients underwent cranial MRI/MR angiography or CT examination. Widened cerebral fissures of bilateral temporal lobes, abnormal dilated branch of middle cerebral artery in the left hemisphere and abnormal signal in the grey matter of frontal and occipital lobes were revealed respectively in three patients. Conclusions Our study revealed the coexistence of PHPV in a significant percentage of patients with MGS, suggesting a potential common genetic link. Compared with MGS and PHPV alone, the combination of the two conditions manifested with higher incidence and more severe complications in younger patients. Close follow-up was recommended. Lensectomy and vitrectomy were beneficial in the management of the complications.

  • analysis of clinical features in pediatric Morning Glory Syndrome
    Chinese Journal of Optometry & Ophthalmology, 2012
    Co-Authors: Ying Zhu, Peiquan Zhao, L I Jiakai, Yang Dong, Shiyuan Wang
    Abstract:

    Objective To analyze and summarize the clinical features and ocular fundus of pediatric patients with Morning Glory Syndrome.Methods In this retrospective study, the clinical data of pediatric patients with Morning Glory Syndrome, who were treated from June 2006 to June 2010 at the Department of Ophthalmology, Xinhua Hospital, including sex, age, right or left eye and visual acuity,were analyzed.Main outcome measures were best corrected visual acuity (BCVA),intraocular pressure,ocular fundus and dioptroscopy.Results Thirteen patients (14 eyes),7 males (54％) and 6 females (46％),were diagnosed with Morning Glory Syndrome.They were diagnosed at an average age of (30.6±24.7)months (range from 2 months to 6 years of age).The Syndrome presented in 6 left eyes (43％) and 8 right eyes (57％).Only one patient presented bilaterally.Visual acuity measurement was possible in 8 patients and their BCVA were all below 0.1.The ocular fundus of all patients' was exactly like a blooming Morning Glory.Four patients (4 eyes, 28％) had persistent hyperplastic primary vitreous,9 patients (9 eyes,64％) had heterotopia of the macula,while one patient (2 eyes, 14％) had retinal detachment.Conclusion Morning Glory Syndrome is a type of congenital deficiency Syndrome that leads to severe visual impairment.Accurate and prompt diagnosis and regular follow-up is important for maintaining the visual acuity of these patients. Key words: Morning Glory Syndrome;  Abnormal development of disc;  Children

Gary C. Brown - One of the best experts on this subject based on the ideXlab platform.

  • Communication between the subretinal space and the vitreous cavity in the Morning Glory Syndrome
    Graefe's Archive for Clinical and Experimental Ophthalmology, 1995
    Co-Authors: Gustavo E. Coll, Stanley Chang, Thomas E. Flynn, Gary C. Brown
    Abstract:

    • Background: The aim was to describe a pathogenic mechanism for a rhegmatogenous retinal detachment in a 69-year-old man with the Morning Glory Syndrome. • Methods: During vitreous surgery for a retinal detachment, a membrane was removed that covered the optic disc anomaly and produced traction on the peripapillary retina. A retinal hole was found in tissue lying within the optic cup, and the hole was sealed using a autologous plasma —thrombin mixture. Silicone oil was used for retinal tamponade. • Results: A retinal hole in tissue lying within the optic cup provided a fluid pathway between the vitreous cavity and the subretinal space. Following vitrectomy surgery, bubbles of silicone oil passed through the retinal hole into the subretinal space of the macula. • Conclusion: This case demonstrates that a retinal hole in tissue lying within the optic disc anomaly of the Morning Glory Syndrome provides a communication for fluid between the subretinal space and the vitreous cavity, resulting in a rhegmatogenous retinal detachment. Vitreous replacement with silicone oil resulted in the migration of silicone bubbles into the subretinal space.

Lichen Wei - One of the best experts on this subject based on the ideXlab platform.

  • rhegmatogenous retinal detachment in Morning Glory Syndrome pathogenesis and treatment
    International Ophthalmology, 2001
    Co-Authors: Lichen Wei
    Abstract:

    We report a case of Morning Glory Syndrome with retinal detachment. A slit-like retinal break at the edge of the excavated disc anomaly provided a direct communication between the subretinal space and the vitreous cavity. Retinal reattachment and useful vision was achieved after a single procedure of vitrectomy and gas tamponade. We believe that identification of the retinal break, removal of epipapillary fibroglial tissue and its traction force, the avoidance of perfluorocarbon liquid and the appropriate use of long-acting gas as endotamponade, all contributed to the favorable outcome. This is more evidence supporting the rhegmatogenous theory of retinal detachment in Morning Glory Syndrome. A literature review of the clinical presentations and treatments of similar cases is included.

Roberto Gomes Nogueira - One of the best experts on this subject based on the ideXlab platform.

  • absence of mutations in pax6 gene in three cases of Morning Glory Syndrome associated with isolated growth hormone deficiency
    PubMed, 2008
    Co-Authors: Gil Guerrajunior, Roberto Gomes Nogueira, Emerson Salvador De Souza França, Angela Maria Spinolacastro, Adriana Aparecida Sivieromiachon, Sofia Helena Valente De Lemosmarini, Lilia Dsouzali, Priscila Cristina Da Silva, Fernanda Caroline Soardi, Maricilda Palandi De Mello
    Abstract:

    Morning Glory Syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele and hypopituitarism. Clinical signs are varied and often occult. The PAX6 gene is involved in ocular morphogenesis and is expressed in numerous ocular tissues during development especially in the developing central nervous system. The aim of the present study is to evaluate PAX6 in MGS associated with isolated growth hormone deficiency. Three pre-pubertal males (A, B and C) with MGS and short stature due to growth hormone deficiency, treated with recombinant human growth hormone with limited response, were reported. Two of them had basal encephalocele. Coding and non-coding sequences corresponding of PAX6 different transcripts were analyzed by direct sequencing. Nucleotide variations causing putative aminoacid change were not observed. Patient A presented the new IVS2+9G>A transition, whereas patients A and C were heterozygous for known single nucleotide polymorphisms (SNP) within the intron 4. In addition, two SNP heterozygoses were observed for patient C in both intron 9 and 13. Sequencing also revealed several nucleotide variations in patient B. Two heterozygoses for known polymorphisms were identified along with a novel C>A nucleotide change in intron 4. This patient also presented a low number on the TG repeat in intron 9 and a new IVS11+33A>T transversion. Gene regulation and transcription of PAX6 are complex processes; there are two major protein isoforms, PAX6(-5a) and PAX6(+5a), and nine transcripts described. Furthermore, extra transcription regulatory elements have been postulated within PAX6 introns. Considering that neither population distributions on PAX6 polymorphism nor their linkeages with diseases have been reported, a functional effect due to alterations described here cannot be discarded.

  • basal encephalocele associated with Morning Glory Syndrome case report
    Arquivos De Neuro-psiquiatria, 2007
    Co-Authors: Ivanete Minotto, Nitamar Abdala, Adriana Siviero Miachon, Angela Spinola E Castro, Paulo Imamura, Roberto Gomes Nogueira
    Abstract:

    The basal encephaloceles refer to rare entities and they correspond to herniation of brain tissue through defects of skull along the cribiform plate or the sphenoid bone. A rare Morning Glory Syndrome, with characteristic retinal defect has been reported in association with basal encephaloceles. Hypophysis hormonal deficiencies may occur. We accounted for a pituitary dwarfism with delayed diagnosed transsphenoidal encephalocele associated with Morning Glory Syndrome, showing the alterations found in retinography, computed tomography and magnetic resonance imaging.

  • Basal encephalocele associated with Morning Glory Syndrome: case report Encefalocele basal associada a síndrome "Morning Glory": relato de caso
    Academia Brasileira de Neurologia (ABNEURO), 2007
    Co-Authors: Ivanete Minotto, Nitamar Abdala, Adriana Siviero Miachon, Paulo Imamura, Angela Maria Spinola E Castro, Roberto Gomes Nogueira
    Abstract:

    The basal encephaloceles refer to rare entities and they correspond to herniation of brain tissue through defects of skull along the cribiform plate or the sphenoid bone. A rare Morning Glory Syndrome, with characteristic retinal defect has been reported in association with basal encephaloceles. Hypophysis hormonal deficiencies may occur. We accounted for a pituitary dwarfism with delayed diagnosed transsphenoidal encephalocele associated with Morning Glory Syndrome, showing the alterations found in retinography, computed tomography and magnetic resonance imaging.As encefaloceles basais são entidades raras e correspondem a herniações do tecido cerebral através de um defeito do crânio, ao longo da lâmina crivosa etmoidal ou do osso esfenoidal. A rara síndrome Morning Glory, com alterações de fundo de olho características pode apresentar-se associada à encefalocele basal. Deficiências hormonais hipofisárias podem ocorrer. Relatamos caso de nanismo hipofisário com encefalocele transesfenoidal de diagnóstico tardio associada à síndrome de Morning Glory, mostrando as alterações na retinografia, tomografia computadorizada e ressonância magnética