Eugene S. Lit

Retinal manifestations of morning glory disc syndrome.

Morning glory disc syndrome refers to an uncommon, specific optic nerve appearance of an excavated disc defect, named for its similarity in appearance to the flower. There have been case reports of various associations with systemic defects, such as hypertelorism, cleft lip and palate, renal anomalies, and encephaloceles. Descriptions of ocular anomalies associated with morning glory syndrome also abound, including strabismus, cataract, nystagmus, ciliary body cyst, lens coloboma, eyelid hemangioma, aniridia, and drusen. In addition, retinal diseases, including detachments, abnormal vascular changes, and subretinal neovascular membrane formation, are variably associated. In this chapter, we try to clarify some of the nomenclature, histopathology, and pathogenesis of these retinal manifestations. The first descriptions of morning glory disc syndrome can be traced back to the early 1900s, but it was not until 1970 that the term was coined by Kindler. There is some variation about how morning glory disc syndrome (Figure 1) is distinguished from the other congenital excavated optic disc defects of optic disc pit (Fig. 1C) and coloboma, (Fig. 1B) with some authors using the terms optic disc coloboma and morning glory disc syndrome interchangeably. Most references, however, refer back to the descriptions of Kindler in defining morning glory disc syndrome as a funnel-shaped, excavated nerve-head defect surrounded by an elevated annulus of chorioretinal pigment disturbance and subretinal fibroglial tissue. It includes multiple narrow branches of retinal vessels at the edge of the morning glory disc syndrome, which appear abnormally straightened as they course over the peripapillary retina. In addition, there usually is a central tuft of white, fiberlike tissue overlying the disc, although there seems to be some disagreement whether these are necessary for use of the term morning glory disc syndrome. The term coloboma, Greek