Outcomes of vitreoretinal surgery in retinal detachment associated with morning glory disc anomaly

Purpose:Morning glory disc anomaly (MGDA) is associated with a high prevalence of retinal detachment (RD), sometimes requiring multiple surgeries. The study aimed to establish the outcomes of RD surgery in such eyes and predict factors influencing the outcome, if any.Methods:It was a retrospective observational study of 9 eyes that underwent pars plana vitrectomy (PPV) for RD associated with MGDA. Clinical and surgical details were correlated with outcomes of surgery as noted at the final follow-up.Results:PPV was performed in 9 eyes. Lensectomy was done in 5 of 9 eyes (55.5%) during PPV. Laser photocoagulation around the disc was performed in 55.5% (5/9) of the eyes. Silicone oil tamponade was used in 77.7% (7/9) of eyes and gas tamponade (14% C3F8) was used in 22.3% (2/9) of eyes. Attached retina at final visit was observed in 66% of the eyes (6/9). Two out of 3 eyes that were seen to have preoperative glial tissue at the disc had poor outcome (odds ratio 10, P = 0.16). Five out of 7 (71%) eyes that had silicone oil tamponade, had an attached retina. No identifiable breaks were noted preoperatively in 5 eyes, of which 4 (80%) had an attached retina postoperatively. Vision improved by 1 line in 67% of the eyes that underwent surgery.Conclusion:MGDA related RD presents a unique set of challenges; meticulous PPV with or without lensectomy can help in achieving a successful anatomical and functional outcome in about two-thirds of patients.     

Pathogenesis of retinal detachment associated with morning glory disc

Pars plana vitrectomy was performed on a six-year-old boy with complete retinal detachment associated with a morning glory disc of his left eye. Perfluorodecalin was injected to unfold the retina. During surgery, perfluorodecalin leaked repeatedly under the retina. This case demonstrates that a retinal hole in tissues lying within the optic disc anomaly provides a communication for fluid and perfluorodecalin between the subretinal space and vitreous cavity resulting in a rhegmatogenous retinal detachment in the morning glory syndrome.     

Retinal detachment in the morning glory anomaly

The morning glory anomaly is a rare congenital clinical entity that results from abnormal optic nerve development. In our series of 30 patients, followed for a mean duration of 10.3 years, 11 developed associated retinal detachments. These detachments followed a variable clinical course, including spontaneous reattachment and even redetachment, and may result from an abnormal communication between the subarachnoid space of the optic nerve and the subretinal space, allowing cerebrospinal fluid to accumulate subretinally. The clinical findings (including ultrasonography, computed tomography, and electrophysiologic testing), natural history, complications, and therapeutic indications are discussed.     

Case report: The morning glory syndrome

This is a report of a single case of morning glory syndrome. The morning glory syndrome is a rare congenital optic disc anomaly which has received scant attention in the ophthalmologic literature. The aetiology of the disorder remains controversial, although it would appear to be related to an isolated coloboma of the optic nerve. The association between morning glory syndrome and a variety of ocular (most notably non-rhegmatogenous retinal detachment) and systemic defects makes its recognition and correct management important; this is discussed in some detail.     

Vitrectomy of rhegmatogenous retinal detachment in morning glory syndrome

We report a case of retinal detachment in morning glory syndrome(MGS). We think that identification of the retinal break, removal of the traction force by vitrectomy, the use of long-acting gas as endotamponade, all contributed to the successful treatment of the disease. The pathogenic mechanisms of the disease and the function of B-scan to the diagnosis are included.     

Trisomy 4q with morning glory disc anomaly

The authors describe a case of trisomy 4q with a unilateral morning glory disc anomaly, a previously unreported ocular manifestation. Previous ocular involvements are summarized.     

牵牛花综合征伴裂孔性视网膜脱离的玻璃体手术治疗

我们报道牵牛花综合征伴裂孔性视网膜脱离经玻璃体手术治疗1例。我们认为:发现裂孔、玻璃体手术解除牵拉力以及长效气体的运用是手术成功的关键。文献也包括对牵牛花综合征伴视网膜脱离的病因及B超对此病诊断意义的讨论。     

The morning glory disc anomaly: Contractile movement,classification, and embryogenesis

A case of the morning glory disc anomaly is reported in which alternating contraction and dilation movements were observed and documented with fundus photographs. The movements are attributed to an anomalous communication between the subretinal and subarachnoid spaces that permits flux of fluid to occur between the two compartments, with consequent variation in the degree of retinal elevation within the excavated portion of the lesion. The clinical features of the morning glory disc anomaly, peripapillary staphyloma and optic disc coloboma are presented, and the relationship among these lesions is discussed. The author concludes that the morning glory disc anomaly is a clinical entity distinct from peripapillary staphyloma and optic disc coloboma. It is thought to be due to dysgenesis of the distal optic stalk leading to anomalous persistence of the extension of the cavity of the optic cup into the stalk.     

Morning glory syndrome

Morning glory syndrome is a congenital optic disc anomaly in which much of die excavated colobomatous optic disc is filled with glial tissue. Ocular complications may include strabismus, reduced visual acuity and retinal detachment and it may have systemic associations as in Aicardi's syndrome. A patient widi monocular morning glory syndrome and reduced visual acuity is reported. The pattern reversal visually evoked potential was reduced and the latency increased in the affected eye.     

Prophylactic juxtapapillary laser photocoagulation in pediatric morning glory syndrome

AIM: To determine the anatomic and visual outcomes of prophylactic juxtapapillary laser photocoagulation treatment alone in the prevention of retinal detachment (RD) in a cohort of pediatric patients diagnosed with morning glory syndrome (MGS). METHODS: A total of 24 eyes of 22 consecutive patients aged 0-15y diagnosed with MGS treated with prophylactic juxtapapillary laser photocoagulation alone were reviewed. Data including demographics, ocular examination, anatomic and visual outcomes, following treatment and complications were collected. RESULTS: Two patients had bilateral laser treatment and 20 had monocular laser treatment. The age at treatment of 13 (59.1%) patients was less than 12mo. The presenting symptoms included strabismus (6/22, 27.3%), decreased vision (2/22, 9.1%), and routine fundus screening (14/22, 63.6%). Fifteen (68.2%) patients underwent cranial magnetic resonance imaging (MRI) examinations, and 3 of those 15 (20.0%) had abnormal findings in the nervous system. Based on preoperative wide-field fundus photography and B-scan echography, all (100.0%) eyes had no obvious RD. On postoperative 1mo and 6mo and the following follow-ups, the anatomic outcomes of all eyes remained stable. The mean follow-up duration was 27.7±17.5mo. No severe complications were found. Preoperative visual acuity acquired from 2 (9.1%) patients ranged from light perception to 20/200. Postoperative acuity acquired from 11 (50.0%) patients ranged from light perception to 20/125. CONCLUSION: The preliminary anatomic and visual outcomes of prophylactic juxtapapillary laser treatment alone in pediatric MGS patients are relatively stable in a short-term follow-up. Further long-term clinical observation will be needed to confirm its efficacy and safety.     

Aniridia,atypical iris defects,optic pit and the morning glory disc anomaly in a family

A two-month-old girl with aniridia was found to have an optic pit in one eye and the morning glory disc anomaly in the other eye. The patient's mother had corectopia and atypical iris defects. The findings are described and the embryology of the various abnormalities briefly reviewed. This is the first report of such an association of findings in a family with aniridia. Failure of normal neuroectodermal development may play a role in the development of optic pits and the morning glory disc anomaly.     

Retinal detachment associated with morning glory syndrome

CASE REPORT: A twenty three year old woman was diagnosed of a morning glory papillary anomaly, then with normal visual acuity (VA). Nine years later, the VA decreased to 0.4, secondary to a serous macular detachment, confirmed by optical coherence tomography (OCT). After treatment with C2F6 gas injection, positioning, and peripapillary laser, the VA improved to 0.7 and the foveolar area reattached. DISCUSSION: The morning glory Syndrome usually has an early diagnosis due to poor visual acuity. Thirty eight percent of the cases have retinal detachment. We show an unusual case of morning glory syndrome with a serous detachment, successfully treated with gas and laser.     

Optical coherence tomographic characteristics in morning glory disc anomaly

CASE REPORT: Optical coherence tomography (OCT) confirms increased optic nerve head dimensions in morning glory anomaly, and provides evidence of increased retinal nerve fibre layer thickness and reduced macular thickness. COMMENTS: This first report of quantitative data from OCT may provide more useful information to corroborate clinical diagnosis of this rare congenital disorder.     

Unilateral situs inversus of optic disc associated with reduced binocularity and stereoacuity resembling monofixation syndrome

Situs inversus of the optic disc is a rare, usually bilateral, congenital embryological abnormality associated with high myopia, optic disc coloboma or tilted optic disc. It is characterized by emergence of the retinal vessels in an anomalous direction with dysversion of the optic disc. In this report we present a 13-year-old boy diagnosed with isolated, unilateral situs inversus of the optic disc associated with reduced binocularity and stereoacuity resembling a monofixation syndrome. The clinicians should be aware of this association and assess the binocularity in patients with unilateral optic disc or macular anomalies. Conversely, patients with reduced binocularity and stereoacuity should be carefully evaluated for macular or optic nerve anomalies, if not associated with strabismus, anisometropia and eccentric fixation. Typical fundus picture, optical coherence tomography and multifocal electro retinogram of the patient would be instructive to a clinician.     

Perthes syndrome associated with bilateral optic disc edema

CASE REPORT: Perthes syndrome, or traumatic asphyxia syndrome, results from a severe crush injury of the thorax. It manifests itself with facial and upper chest petechiae, subconjunctival hemorrhages, cervical cyanosis, and occasionally neurological symptoms. A patient who had been incarcerated under a block of marble presented with severe traumatic asphyxia syndrome complicated by pulmonary contusion, fractures of the right clavicle and the 8th and 9th ribs, minimal right hemothorax, liver contusion, and bilateral optic disc edema. COMMENTS: To our knowledge, we present the first case of Perthes syndrome with bilateral optic disc edema.     

Marfan综合征伴孔源性视网膜脱离

目的：分析Marfan综合征伴孔源性视网膜脱离眼发病特征与治疗原则。方法：回顾并随访了1998年至2001年间上海市第一人民医院进行视网膜脱离手术治疗的10例12眼。结果：平均随访5．2个月，总的视网膜解剖复位率83．3％(10／12)，其中只作巩膜外手术7眼，复位率100％，玻璃体手术5眼，复位率60％。视力提高7眼(58．3％)。结论：仔细采用多种方法检查可以提高术前裂孔检出率。经巩膜环扎加压手术可以有效处理简单视网膜脱离眼，而毋实施玻璃体手术联合晶体摘除术。治疗中需加倍警惕对侧眼病变。     

婴幼儿牵牛花综合征的临床特征分析

目的分析总结婴幼儿牵牛花综合征患者的一般临床特征和眼底表现。方法回顾性分析2006年6月至2010年6月于上海交通大学医学院附属新华医院眼科就诊的牵牛花综合征患儿的临床资料,统计分析患者性别、年龄、眼别及视力等情况。观察指标包括视力检查、眼内压检查、眼前节检查、眼底检查以及屈光度检查等。对病例进行计数并求百分比。结果共有13例（14眼）被诊断为牵牛花综合征。患儿就诊时的年龄为2个月-6岁,平均（30．6＋24．7）个月。其中男性7例（54％）,女性6例（46％）;6只（43％）为左眼,8只（57％）为右眼;除1例患者（8％）为双眼发病外,其余12例患者均为单眼发病。能够进行视力检查的8例患者的最佳矫正视力均低于0．1。所有患者眼底表现均非常典型,似盛开的牵牛花。其中有4例（4眼,28％）合并有永存原始玻璃体增生症,9例（9眼,64％）合并有黄斑异位。1例（2眼,14％）合并有视网膜脱离。结论牵牛花综合征是一种严重影响患者视觉功能的先天缺陷综合征。准确及时的诊断和定期随访有利于保存这类患儿的视功能。     

Delayed presentation of morning glory disc anomaly and transsphenoidal encephalocele: A management dilemma

Background Morning glory disc anomaly (MGDA) is a rare developmental abnormality of the optic disc that is associated with many other neurological and vascular conditions. Most cases are diagnosed in childhood.

Case Report We report a 57-year-old woman who presented to the ophthalmology department for assessment of long-standing poor vision in the left eye and exotropia. Examination showed a left MGDA and bitemporal hemianopsia. These findings prompted magnetic resonance imaging, revealing a transsphenoidal basal meningoencephalocele with herniation of the optic chiasm and inferior hypothalamus into the dural sac. Due to the eloquence of the neurovascular structures it contained, a decision was made not to reduce the meningoencephalocele. Instead, a ventriculoperitoneal shunt was placed. The patient’s ophthalmologic examination remained stable over the following year.

Conclusion While rare, MGDA can be first diagnosed in late adulthood and a thorough evaluation should be completed to assess for midline cranial defects, vascular abnormalities, and other associated abnormalities. Patients presenting late in life with basal encephalocele, herniation of the optic chiasm, and bitemporal hemianopsia present a management dilemma. In this case, a ventriculoperitoneal shunt was placed with the intention of lowering intracranial pressure to prevent further herniation and reduce the risk of cerebrospinal fluid leak.