Cystoid macular edema (CME) and other macular diseases were largely unknown before the invention of the direct ophthalmoscope by Hermann von Helmholtz in 1851. For example, in 1846 Appolinaire Bouchardat reported patients with diabetes and visual loss without cataract or corneal findings, which suggested undiagnosed posterior segment disease. Then in 1856, using the ophthalmoscope, Eduard Jaeger reported “yellowish spots and extravasations which permeate the whole thickness of the retina.” In 1872, Eduard Nettleship reported “oedema or cystoid disease of the retina”.
As ophthalmoscopes and fundus cameras improved, increasingly detailed descriptions of CME were reported by investigators including Karl Hruby, Paul Chandler, John Nicholls, and A. Edward Maumenee. In 1952, S. Rodman Irvine gave the seventh Francis I. Proctor Lecture, “A Newly Defined Vitreous Syndrome Following Cataract Surgery,” to the University of California San Francisco Medical School and the text was published in 1953 (1). Irvine described “prolapse of the vitreous into the anterior chamber with late rupture of the hyaloid face following uncomplicated intracapsular cataract surgery” which could progress to “development of postoperative macular changes” and “macular degeneration.”
The next major advance in the history of the diagnosis of CME was fluorescein angiography, first described by Harold Novotny and David Alvis in 1961 (2). In 1966, J. Donald Gass and Edward Norton reported 44 patients with “macular edema and papilledema following cataract extraction” imaged with fluorescein angiography (3). In this series, Gass and Norton used the terms “cystoid edema of macula,” “cystic macular changes,” “cystoid edema and degeneration of the macula,” “cystoid swelling of the retina,” “cystoid elevation of the macular area,” and “cystoid macular edema.” In a separate paper in 1966, Gass and Norton described the characteristic fluorescein angiographic appearance of CME: “… a remarkable and diagnostic geometric dark stellate figure develops centrally on the background of fluorescein staining” (Figure 1) (4).
Gass and Norton hypothesized that the pathogenesis of CME “involves leakage of serous exudate from the capillary network within the macular portion of the retina and/or the optic nerve head”, in contrast to Irvine’s observation that vitreous prolapse was the primary mechanism. The eponymous “Irvine-Gass syndrome” first appeared in a PubMed-indexed journal in 1971 (5).
The next major advance was optical coherence tomography (OCT), first reported by David Huang, Carmen Puliafito, and colleagues in 1991 (8). OCT became the most common modality to detect CME because, in comparison with fluorescein angiography, OCT is faster, safer, more comfortable for the patient, and able to provide quantitative measurements of retinal thickness, which are useful to gauge the severity of disease and to monitor treatment response (Figure 2).
In the next chapter of The Ophthalmologist’s Time Machine, we will review the history of treatments for CME.
References
- 1. SR Irvine, “A newly defined vitreous syndrome following cataract surgery,” Am J Ophthalmol., 36, 599 (1953).
- 2. HR Novotny, DL Alvis, “A method of photographing fluorescence in circulating blood in the human retina,” Circulation, 24, 82 (1961).
- 3. JD Gass, EW Norton, “Fluorescein studies of patients with macular edema and papilledema following cataract extraction,” Trans Am Ophthalmol Soc., 64, 232 (1966).
- 4. JD Gass, EW Norton, “Cystoid macular edema and papilledema following cataract extraction. A fluorescein fundoscopic and angiographic study,” Arch Ophthalmol., 76, 646 (1966).
- 5. A Jutte, L Lemke, [Edema of the macula and papilla after cataract extraction (Irvine-Gass syndrome)], Klin Monbl Augenheilkd 158, 826 (1971).
- 6. D Huang et al., “Optical coherence tomography,” Science, 254, 1178 (1991).
