Morning glory syndrome with Moyamoya disease: A rare association with role of imaging

Abstract

Morning glory disc anomaly (MGDA) is a congenital optic nerve anomaly characterized by a funnel-shaped excavation of the posterior globe that incorporates the optic disc. Most cases are isolated and not associated with systemic anomalies. Systemic anomalies include midline cranial facial defects, hypertelorism, agenesis of the corpus callosum, cleft lip and palate, basal encephalocele, congenital forebrain abnormalities, and renal anomalies. We report a case of 4-year-old male child who presented with reduced visual acuity on left eye with poor fixation. The left eye demonstrated 6-diopter esotropia. Examination of fundus revealed features of MGDA. The child was further subjected to magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of brain to rule out other associated anomalies. It demonstrated narrowing at the distal part of internal carotid artery on both sides, left more than right with prominence of lenticulostriate and leptomeningeal vessels. MRI also revealed funnel-shaped excavation of the posterior globe on the left side consistent with MGDA. Ascertaining the accurate diagnosis of MGDA guides appropriate ophthalmic management and should also prompt a search for associated intracranial abnormalities. Although the diagnosis of MGDA is typically made clinically, imaging may feed supplementary value in establishing the diagnosis and reveal the extent and character of associated ocular abnormalities, and cross-sectional imaging permits for evaluation of the globe in the setting of associated opacities of the refractive media, including persistent hyperplastic primary vitreous, which may alleviate the capacity to make this diagnosis on the basis of the fundoscopy examination alone.

Internal carotid artery origin of the anterior cerebral artery: A rare anatomic intracranial arterial variation in a child with morning glory disc anomaly and moyamoya vascular pattern; case report and review of literature

“…Over the past 25 years, several case reports and case series have reported the co-existence of MMD and MGDA. [ 9 10 11 12 13 14 15 ] The aforementioned association has initially described by Hanson et al . [ 16 ] back in 1985, while many additional cases have been reported since.…”

Spotlight on the pediatric eye: a pictorial review of orbital anatomy and congenital orbital pathologies

“…Morning glory disc anomaly (MGDA) is a rare congenital anomaly of the optic nerve with a characteristic fundoscopic appearance of a funnel-shaped excavation of the optic disc with an overlying central glial tuft and abnormal blood vessels. 30 , 31 The deformed optic nerve with radially oriented vessels resembles the petals of a morning glory flower, first described by Kindler in 1970. 30 Most cases of MGDA are isolated, but associations with other ocular and intracranial abnormalities are reported, which can be delineated on imaging.…”

“…30 , 31 The deformed optic nerve with radially oriented vessels resembles the petals of a morning glory flower, first described by Kindler in 1970. 30 Most cases of MGDA are isolated, but associations with other ocular and intracranial abnormalities are reported, which can be delineated on imaging. These include midline facial defects, callosal dysgenesis, Moya Moya disease, retinal detachment, persistent fetal vasculature (PFV), and basal encephalocele.…”

“…These include midline facial defects, callosal dysgenesis, Moya Moya disease, retinal detachment, persistent fetal vasculature (PFV), and basal encephalocele. 30 MGDA is a sporadic condition, and should be differentiated from ocular coloboma, which is a familial condition, due to its genetic implications and for the purpose of family counseling. 31 , 32 On MRI (Figure 6), orbital imaging findings of MGDA are funnel-shaped optic disc with adjacent retinal surface elevation, which is T1WI hyperintense, effacement of the adjacent perioptic nerve subarachnoid space, lack of the usual enhancement with discontinuity of the choroidal–lamina cribrosa, and, less consistently, fatty infiltration of the distal optic nerve sheath, distal optic nerve enhancement, and small size of the globe containing the MGDA.…”

Moyamoya Disease Associated With Morning Glory Disc Anomaly and Other Ophthalmic Findings: A Mini-Review

“…We identified 13 articles of MMD-associated MGDA, including 11 case reports, 1 ophthalmic image report, and 1 retrospective study (5,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). The retrospective study evaluated intracranial vascular anomalies in 20 patients with MGDA and diagnosed four of them with MMD (9).…”