Morning Glory Disc Anomaly with an ipsilateral Optic Nerve Glioma

Morning glory disc anomaly (MGDA) is a congenital disorder characterized by the funnel shape disc excavation with radiating retinal vessels. MGDA is sometimes associated with midline developmental or vascular malformations. On evaluation for visual function, MGDA and concurrent ipsilateral optic nerve enlargement were found. This case is to report a rare CNS association with MGDA and to alert the importance of systemic evaluation in congenital optic nerve anomalies.

Poster 8 Morning Glory Disc Anomaly with an ipsilateral Optic Nerve Glioma Hyosook Ahn1 Asan Medical center, University of Ulsan, SEOUL, Korea (Republic of) 1 Introduction: Morning glory disc anomaly (MGDA) is a congenital disorder characterized by the funnel shape disc excavation with radiating retinal vessels. MGDA is sometimes associated with midline developmental or vascular malformations. On evaluation for visual function, MGDA and concurrent ipsilateral optic nerve enlargement were found. This case is to report a rare CNS association with MGDA and to alert the importance of systemic evaluation in congenital Optic nerve anomalies. Description of Case(s): 11yrs ago, a 5-year-old girl was referred to my clinic because of strabismus and amblyopia of left eye. At the first visit, VA OD 20/20 OS 20/200 with RAPD +3 OS and left exotropia 15pd were noticed. On fundus exam, left optic disc showed characteristic MGDA. Magnetic resonance imaging studies revealed enlargement of the prechiasmatic ipsilateral optic nerve which consistent with glioma. With long-term follow-up for optic nerve tumor, there is no change in tumor size and visual field. Upon amblyopia treatment, visual acuity was preserved from 20/200 at the initial visit to 20/100 at the last visit. Conclusions, including unique features of the case(s): In conclusion, I recommend that any patient with MGDA take CNS imaging for evaluation of varies midline defect and vascular abnormalities as well as visual functions. Early recognition and management of amblyopia are essential to enhance visual acuity. Especially this patient was followed up to 11 years for concurrent optic nerve glioma. This case is unique to report a rare CNS association with MGDA and long-term follow-up. And it is important to recognize the importance of systemic evaluation and amblyopia management in congenital Optic nerve anomalies. References: 1. Morning glory disc anomaly with an ipsilateral enlargement of the optic nerve pathway D.Thoma, I. Nijs, P.Demarel, I. Casteels Europian J of Pediatric Neurology, 21; 787-798, 2017 2. Morning Glory Disc Anomaly in Association with Ipsilateral Optic nerve Glioma P. Bandopadhayay, L Dagi, N Robison, L Goumnerrova, N Ullrich Arch Ophthalmol vol 130: 1082-1083, 2012. Keywords: Pediatric neuro-ophthalmology, Tumors Financial Disclosures: The authors had no disclosures. Grant Support: None. 64 | North American Neuro-Ophthalmology Society