Dr. Jayant Kumar,K17257, Dr. Ashish Ahuja, Dr. Naresh Babu K, Dr.Kim Ramasamy
Jayant Kumar 1, Ashish Ahuja 2, Naresh Babu 3, Ramasamy Kim 4
1,2,3,4Department of retina and vitreous, Aravind Eye Hospital, Madurai
Abstract
Introduction: Morning glory syndrome (MGS) has important ocular and systemic associations.
AIM: To document the clinical profile of patients presenting with MGS in our institute and build an approach to its management.
Materials and methods:
It was a retrospective observational study of the 18 eyes of 16 patients with MGS over the past eight years.
Results :
All the presenting features of the18 eyes were noted, some being newly reported . The associations ranged from retinal detachment in 7 eyes to others like microphthalmos, optic disc pit, optic disc coloboma, choroidal coloboma, pulsatile optic disc, choroidal neovascular membrane, altered shape of the globe while systemic associations were were periventricular leucomalacia, delayed milestones with lipodermoid and asthma.
Discussion:
Morning glory syndrome has a variety of ocular and systemic associations.
Conclusion:
The correct diagnosis and evaluation of patients with MGS can prove sight saving, as well as life saving for them.
Keywords: Morning glory syndrome, retinal detachment, pulsatile optic disc
Introduction:
Morning glory syndrome is a rare congenital optic disc anomaly in which the optic disc head is enlarged and excavated with overlying tuft of glial tissue and has increased number of blood vessels arising from its periphery. The nomenclature of this disease was first given by Kindler in 1970 due to its resemblance with a morning glory flower. The syndrome deserves significant clinical attention not only for its rarity, but also for some immensely important ocular and systemic associations which can be sight threatening as well as life threatening if not detected on time. Retinal detachment is one such sight threatening ocular association which has been reported in the range of 26 to 38 % in patients with MGS. Midline craniofacial defects and intracranial vascular anomalies are the commonly known important non-ocular associations of the syndrome. A knowledge of the various ocular and non-ocular associations of the syndrome are a pre-requisite for proper management of these patients.
Aims and Objectives
To document the clinical profile of patients presenting with MGS in a tertiary eye care centre in southern India and build an approach to its management.
Materials and Methods
It was a retrospective observational study. All patients diagnosed with Morning glory syndrome at our institute over the past 8 years were considered for inclusion in our study. The cases where the details of the clinical picture were missing or which did not have a supportive evidence in the form of an appropriate investigation like Ultrasonography B Scan, fundus photograph or Optical coherence tomography were excluded from the study.
Results
18 eyes of 16 patients were found to have clinically documented MGS in the medical records extensively searched. Out of those 16 patients, 14 were unilateral while two were bilateral. The right and the left eye were involved in seven cases each, out of the 14 unilateral cases. Nine of the patients were male and seven were female. The presenting complaints in most of the patients was diminution of vision since birth or early childhood while in some, it was the deviation of eyes or the presence of small eyes which was the chief complaint. Seven patients had strabismus with esotropia being the dominant form, occurring in 5 patients, while exotropia was present in two patients. The visual acuity ranged from PL+ to as good as 6/24 in different patients, depending on the severity and associated complications of the disease. Refraction was done in four patients in which one had compound myopic astigmatism and three others had compound hypermetropic astigmatism. Retinal detachment (RD) was an association in five eyes. Three of them presented with spontaneously settled RD while the remaining two showed detached retina on clinical examination which were managed surgically. One of the cases with detached retina also had subretinal bands. Amongst the other relatively common ocular associations, microphthalmos was present in two eyes, optic disc pit in two eyes and optic disc coloboma was present in three eyes. One eye had choroidal coloboma, one had pulsatile optic disc with an associated temporal avascular zone in the temporal periphery and one each had choroidal neovascular membrane, posterior subcapsular cataract, disc pallor, peripapillary atrophy and relative afferent papillary defect. Also one eye had serous detachment at the fovea while two eyes had serous detachment along with cystic changes at the fovea. The MRI scan of one patient revealed altered shape of the globe along with tortuous course of the optic nerve.
As far as the systemic associations are concerned, one patient had periventricular leucomalacia which was confirmed on MRI, one patient had delayed milestones with lipodermoid and one patient had asthma.
Discussion:
Morning glory syndrome is a rare congenital optic disc anomaly characterized by funnel shaped excavated optic disc surrounded by chorioretinal pigmentary disturbance.
Retinal detachment may develop in upto one third of the patients with morning glory syndrome.
Amblyopia may be a contributing factor to poor vision in unilateral cases. Patients are often referred for leucocoria or strabismus.
The syndrome may be associated with other ophthalmic abnormalities such as cysts of the optic nerve sheath, congenital cataract, microophthalmos , aniridia, strabismus,nystagmus, retinal detachment. Other systemic anomalies like cleft lip and palate, hypertelorism , renal abnormalities ,cardiac defects, cerebrovascular anomalies. In our study, we found more of ocular associations than systemic, which might point to the fact that systemic findings are less likely in south Indian population.
As far as the associated retinal detachment is concerned, the origin of the subretinal fluid is from either from the vitreous or from CSF and tractional forces may have a role. Spontaneous resolution of the retinal detachment may sometimes occur. Retinal detachment in morning glory syndrome is difficult to repair because the break could not be identified in most cases.Some patients require multiple surgeries to achieve retinal reattachment and the visual outcome is poor. The same was found in our study were we did not get very favourable outcomes in the operated cases.
Conclusion:
Morning glory syndrome, even though rare, should be kept in mind as one of the rare differential diagnosis of retinal detachment especially in paediatric patients. Also such patients should be carefully evaluated for other important ocular and systemic associations. A timely diagnosis and intervention can prove sight saving as well as life saving for these patients.
References
1) Anahid safari et al, morning glory syndrome associated with multiple sclerosis, Iran journal of neurology 2014;13(3):177-180.
2) S Chang et al, Retinal detachment associated with optic disc colobomas and morning glory syndrome, Eye(2012) 26,494-500.
3) G N Dutton, congenital disorders of the optic nerve: excavations and hypoplasia , Eye(2004)18,1038-1048.