Morning Glory Syndrome: A Series of Twenty Cases Including Clinical Findings, Ultrasonography and Computerized Tomography

Abstract

Abstract: : Purpose: To review the clinical and radiological findings of patients with Morning Glory Syndrome (MGS). Methods: A case-series of twenty patients with MGS presenting to Sainte- Justine Hospital. Charts were reviewed for clinical findings, including visual acuity, strabismus and ophthalmic findings. Brain and orbital CT scan s and ultrasounds were also reviewed. Results: 53% (8/15) of patients with snellen visual acuity (VA) recordings had a VA better or equal to 20/400, and 26% (4/15) had a VA better or equal to 20/40. Almost all cases were associated with strabismus (18/20), with esotropia (13/18) predominating. Anisometropia, microphthalmos and other ocular anomalies were very common. In our series, retinal detachment occured in 10% (2/20) of patients and the histopathologic findings of one eviscerated phthisical eye are reviewed. Brain CT scans revealed asymptomatic hygromas (2/20) and focal brain atrophy (1/20), cerebellar vermis hypoplasia in a patient with Joubert syndrome, and an absence of the corpus callosum in a patient with Aicardi's syndrome. Orbital CTs demonstrated typical posterior scleral wall defects, as well as retrobulbar optic nerve thickening, tortuosity and increased signals with constrast. Ultrasonographic measurements of axial length and coloboma depth were also taken. Conclusions: MGS includes a spectrum of severity with the majority of patients having useful vision. Strabismus and anisometropia are common, and patients may respond to amblyopia therapy. Retinal detachment was noted in 10% of patients with average follow-up of ninety-two months. Orbital CT-scans demonstrated intraocular calcifications and posterior scleral ectasia, bothof which are associated with poor visual acuity. B-scan ultrasonography demonstrated a specific new finding: the overhanging glial tissue sign. It is proposed that MGS be classified amongst the neural crest disorders.  

Keywords: optic disc • clinical (human) or epidemiologic studies: nat • imaging methods (CT, FA, ICG, MRI, OCT, RTA, S