|Year : 2018 | Volume
| Issue : 2 | Page : 128-132
Retinal detachment in morning glory disc anomaly: Prevalence and outcome of treatment
Ashish A Ahuja1, Olukorede O Adenuga2, Jayant Kumar1, Naresh B Kannan1, Kim Ramasamy1
1 Retina Department, Aravind Eye Hospitals and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India
2 Retina Department, Aravind Eye Hospitals and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India; Department of Ophthalmology, Jos University Teaching Hospital, Jos, Plateau State, Nigeria
|Date of Web Publication||13-Feb-2019|
Dr. Olukorede O Adenuga
Department of Ophthalmology, Jos University Teaching Hospital, Jos, Plateau State, Nigeria
Source of Support: None, Conflict of Interest: None
Purpose: To determine the prevalence of retinal detachment (RD) in eyes with morning glory disc anomaly (MGDA) and the outcome of treatment. Materials and Methods: Retrospective study of cases of MGDA seen over a 5-year period at a tertiary eye hospital in South India. All the patients had a thorough ophthalmological and systemic examination. Prevalence of RD was determined and outcome of surgery in treated eyes analyzed. Results: Fifteen patients with a mean age of 11.1 years were diagnosed with MGDA during the study period. Two patients had bilateral anomaly. RD was present in four (23.5%) eyes, and three (17.6%) eyes had features of spontaneously settled RD. Three eyes with RD had vitrectomy with successful anatomical reattachment in two eyes and good visual outcome in one eye. Conclusion: RD is a common association of MGDA. Late presentation is frequent, and visual outcome limited following surgical intervention.
Keywords: Morning glory disc anomaly, retinal detachment, vitrectomy
|How to cite this article:|
Ahuja AA, Adenuga OO, Kumar J, Kannan NB, Ramasamy K. Retinal detachment in morning glory disc anomaly: Prevalence and outcome of treatment. Niger J Ophthalmol 2018;26:128-32
| Introduction|| |
Morning glory disc anomaly (MGDA) is a congenital, funnel-shaped excavation of the posterior fundus that incorporates the optic disc, with a white tuft of glial tissue overlying the central portion of the disc and increased number of blood vessels arising from the periphery of the disc. The term reflects the morphological similarity to the flower of the morning glory plant. Its incidence is rare, and no gender predisposition has been found.
Ocular associations found in affected eyes with MGDA may include congenital cataract, persistent hyaloid remnants, lid hemangioma, preretinal gliosis, and posterior lenticonus., Retinal detachment (RD) may also develop in up to one-third of patients and occasionally resolves spontaneously. MGDA may be part of other systemic abnormalities and syndromes, although it does not appear to be a specific genetic disorder. Most established is the finding of a transphenoidal basal encephalocele and midfacial malformations. MGDA is typically unilateral, though bilateral lesions have been reported.
Diagnosis is usually made early as a result of refractive errors, poor visual acuity (VA), and/or comitant strabismus. MGDA is nonprogressive and does not require treatment. However, in view of its high risk for developing RD and its association with some systemic abnormalities, accurate diagnosis and monitoring are essential. The aim of this study was to determine the prevalence of RD in cases of MGDA seen in our institution and the outcome of surgical treatment.
| Materials and Methods|| |
This is a retrospective study involving eyes with MGDA seen in the retina clinic of a tertiary eye hospital in South India over a period of 5 years from January 2011 to December 2015. The study adhered to the tenets of the declaration of Helsinki, and ethical clearance was obtained from the Institutional Review Board of the hospital. Cases were identified from the electronic medical records and the case files retrieved. The following data were then obtained: age, sex, duration of symptoms, laterality, and systemic symptoms. All the patients had a comprehensive ocular and systemic examination. Ocular findings including VA, best corrected VA, anterior segment, and posterior segment findings were documented. Fundus photographs, optical coherence tomography (OCT) scans (Spectralis HRA + OCT by Heidelberg Engineering, Heidelberg, Germany), B scan ultrasonograms, and magnetic resonance/computer tomography scans were also reviewed and were available. Treatment modality as well as visual and anatomical outcomes for eyes with RD was recorded.
| Results|| |
MGDA [Figure 1] was seen in 17 eyes of 15 patients during the study period with two patients (cases 11 and 15) having a bilateral anomaly [Table 1]. There were eight males and seven females giving a male:female ratio of 1.1:1. The mean age of the patients was 11.1 years (SD 10.8, range 0.25–46 years). The presenting complaints included poor vision since birth or early childhood, deviation of the eyes, and presence of a small eye. The VA ranged from light perception to 6/24, and RD [[Figure 2] and [Figure 3]] was an association in four (23.5%) eyes. Two of these eyes had a total RD with features of proliferative vitreoretinopathy in one. Three eyes (17.6%) had retinal pigmentary changes suggestive of spontaneously settled RD. Other ocular associations included strabismus, pulsatile disc, choroidal neovascular membrane, and posterior subcapsular cataract. Systemic associations encountered included periventricular leukomalacia and asthma in a patient each. There was a history of developmental delay in one patient. The mean age of the patients with RD was 11.8 years, whereas those without RD had a mean age of 11.0 years.
|Figure 3 B scan ultrasonography showing funnel-shaped excavation of the optic disc with retinal detachment|
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Three (cases 2, 5, and 6) of the four eyes with RD had surgery. This included triamcinolone-assisted vitrectomy, trimming of preretinal glial tissue, internal limiting membrane peeling, retinal photocoagulation along the excavated disc, and internal tamponade with sulfur hexafluoride (SF6) gas in one patient, and silicone oil in two patients. An encircling band was used in combination with vitrectomy in one eye (case 2). Anatomical reattachment was achieved in two (66.7%) eyes. One eye (case 5) developed a re-RD after silicone oil removal and underwent fluid–air exchange with silicone oil tamponade. Retinal reattachment was, however, not successful following resurgery. A good visual outcome was achieved in only one (33.3%) of the three eyes that had surgery. The fourth case of RD was inoperable. All the patients had a minimum follow-up period of 1 year.
| Discussion|| |
RD with retinal schisis is the most severe complication of MGDA that impairs VA. About 24% of eyes in this study had RD at presentation. This lies within 14% reported by Harasymowycz et al. and 37% observed by Haik et al. In MGDA, the optic nerve is located centrally and surrounded by a deeply excavated scleral defect. Fluid sometimes accumulates within and beneath the retina, but the origin of the fluid remains unclear. Anomalous communication of subretinal and subarachnoid or vitreous compartments appears to be the causative factor., The peripapillary retina extends into the anomalous peripapillary scleral defect and has been observed to be thinner, incompletely developed, as well as atrophic. The retina may occasionally detach in this area. The presence of glial tissue within the deeply excavated disc increases the likelihood of subsequent RD. The RD usually involves the peripapillary retina and the posterior pole but occasionally extensive bullous RDs may develop.
Retinal breaks may occur in tissues lying within the optic disc anomaly or at the margin of the excavation providing communication between the vitreous cavity and the subretinal space., Such breaks could be caused by traction from peripapillary fibroglial tissue pulling on the retina. Previously, in most of the RDs associated with MGDA retinal breaks were not found. Low contrast between the white scleral tissue in the optic nerve head defect and the thin retina may be responsible for some breaks located in the optic disc anomaly being missed clinically and intraoperatively. Careful inspection of the optic disc defect during surgery is therefore very important. A missed break may have been responsible for the re-RD in this series. With OCT, however, retinal breaks are now increasingly being identified, particularly with 3D reconstruction., In a series of five eyes with MGDA and RD by Ho et al., slit-like retinal breaks were identified in 80% of the eyes at the margin of the excavation with OCT. Postoperative OCT showed closure of the retinal breaks. In the absence of a retinal break, tissue stretching around the peripapillary conus or an abnormal communication between the cavity of the staphyloma and the subarachnoid space, which allows cerebrospinal fluid to leak into the subretinal space have been suggested as possible causes of RD. In addition, in a recent report by Lytvynchuk et al. on the use of intraoperative spectral domain OCT for analysis of pars plana vitrectomy in a case of MGDA with RD, strong adhesion between posterior condensed vitreous and central retina was found intraoperatively. The authors concluded that tractional forces of the vitreous was the major cause of the RD as no retinal break was found at surgery and that early pars plana vitrectomy in such cases is beneficial as it prevents the future development of retinal breaks and loss of central vision.
Spontaneous resolution of the RD may also occur and should be considered before planning surgery. Seventeen percent of eyes in this present study had retinal pigmentary changes suggestive of spontaneously resolved subretinal fluid. This is lower than 33% reported in the series by Chang et al. Spontaneous resolution is more likely to occur in the absence of a retinal break or when vitreous or glial tissue causing traction on the retina within the defect is absent.,
The retinal complications associated with congenital optic disc anomalies including MGDA are treated by vitreous surgery, silicone oil tamponade, and peripapillary laser photocoagulation or scleral buckling. Subretinal fluid and retinoschisis may take up to 12 months to resolve following successful surgery. Surgical outcomes are, however, limited and not entirely satisfactory. Three of the four eyes with RD in this series had vitrectomy with two eyes achieving anatomical reattachment. In contrast, other authors reported successful reattachment of the retina in 100% of eyes that had surgery., Visual improvement was documented in only one eye following surgery in our study. This is at variance with findings by Chang et al. and Zhang et al. who reported visual improvement in all the eyes operated in their series. Late presentation by patients in this current study may be responsible for the less than optimal results observed. Longstanding central retinal schisis potentially can lead to irreversible atrophic changes of the retinal layers due to persistence of intraretinal and subretinal fluid. In addition, pediatric patients may not be able to complain of decreased vision or loss of vision, and a significant number tend to be discovered incidentally due to chronic RDs.Limitations of this study include its retrospective design and the small cohort of patients reviewed. The retrospective nature may have resulted in some missing data, whereas the small number of cases reviewed is due to the rarity of the condition.
In conclusion, RD is a common association of MGDA with late presentation being a frequent finding. Visual prognosis is guarded, as surgical intervention may not lead to significant improvement in vision, particularly in eyes with longstanding RD.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]