Retinal complications associated with congenital optic disc anomalies determined by swept source optical coherence tomography

Abstract

Optical coherence tomography has evolved over the past 2 decades to be an important ancillary method to evaluate diseases of the anterior and posterior segments of the eye. The more recent development of swept-source optical coherence tomography (SS-OCT) with a wavelength-tunable laser centered at 1050 nm and deeper imaging depth of 2.6 mm has enabled clinicians to evaluate congenital optic disc anomalies including optic disc pits, optic disc colobomas, and morning glory syndrome in more detail. The SS-OCT findings of the posterior precortical vitreous pocket, Cloquet's canal, lamina cribrosa that is torn from the peripapillary sclera, and the retrobulbar subarachnoid space immediately posterior to the highly reflective tissue lining the bottom of the excavation are presented. In addition, abnormal communications between the vitreous cavity and the subretinal and subarachnoid spaces in eyes with congenital optic disc anomalies are also reviewed. The retinal complications associated with congenital optic disc anomalies are treated by vitreous surgery, silicone oil tamponade, and peripapillary laser photocoagulation or scleral buckling. However, the surgical outcomes are limited and not entirely satisfactory. Analyses by SS-OCT of congenital optic disc anomalies should make the treatment correspond better with the pathological findings.

Keywords: coloboma; congenital optic disc anomaly; morning glory syndrome; optic disc pit; optical coherence tomography.

Fig. 1

Fundus photograph and fluorescein angiogram of a 17-year-old woman with optic pit maculopathy. (A) Fundus photograph showing serous retinal detachment connected to an optic pit at the temporal edge of the optic disc; (B) fluorescein angiogram in early phase shows no fluorescein leakage and hypofluorescence at the optic disc pit.

Fig. 2

Intraoperative photograph of the patient shown in Figure 1. White triamcinolone crystals can be seen in the posterior precortical vitreous pocket above the macula and Cloquet's canal above the optic disc.

Fig. 3

Pre- and postoperative optical coherence tomography (OCT) images of the patient shown in Figure 1. (A) Preoperative oblique swept-source OCT (SS-OCT) image showing retinal detachment connected to the optic disc pit as an excavation of the optic disc. At the optic disc pit, a hole-like multilayered appearance at the bottom of the optic disc pit can be seen; (B) vertical SS-OCT images of the optic disc pit showing vitreous strands connecting to the bottom of the optic disc pit; (C) spectral domain OCT does not show precortical vitreous; (D) postoperative horizontal SS-OCT image at 7 months after vitrectomy to create a posterior vitreous detachment without laser photocoagulation around the optic disc pit shows a reduction of retinal detachment and deepened bottom of the optic disc pit without any multilayered structures.

Fig. 4

Preoperative photograph and swept-source optical coherence tomography (SS-OCT) images of a 37-year-old woman with the morning glory syndrome. (A) Preoperative fundus photograph shows excavation of the enlarged optic disc with peripapillary atrophy and localized retinal detachment superior to the vascular arcade. The patient noticed a decrease of vision although the vision did not change from 0.3. Black lines indicate the scan line of the cross section of the SS-OCT images of B and C; (B) vertical SS-OCT image showing macular retinoschisis in the inner plexiform layer and outer plexiform layer; (C) montage image of horizontal sections showing detached epiretinal membrane with vitreous traction (arrow) connected to the peripapillary atrophy and retinal detachment at the excavated optic disc with a hole (arrowhead).

Fig. 5

Preoperative fundus photograph and swept-source optical coherence tomography images of the patient shown in Figure 4. (A) Preoperative fundus photograph showing retinoschisis and localized retinal detachment with epiretinal membrane superior to the vascular arcade; (B) swept-source optical coherence tomography image shows retinoschisis and an outer retinal hole (arrow) in conjunction with an epiretinal membrane (arrowhead).

Fig. 6

Postoperative Optos image and swept-source optical coherence tomography images of the patient shown in Figure 4 at 3 months. (A) Optos ultrawide angle image shows a reduction of retinoschisis superior to the vascular arcade. Vision increased slightly to 0.4; (B) montage image of horizontal section of swept-source optical coherence tomography shows no vitreous stand while the macular retinoschisis and retinal detachment (arrowhead) at the excavated optic disc remains.

Fig. 7

Pre- and postoperative Optos image and swept-source optical coherence tomography (SS-OCT) images of a patient with the morning glory syndrome in a 14-year old boy. (A) Preoperative Optos image showing a total retinal detachment except in the supratemporal quadrant. Black lines indicate the scan lines of cross section of the SS-OCT images of Figures B and C (before initial surgery), and E (after initial surgery); (B) vertical SS-OCT image shows superior and inferior retinal detachments (arrows) and a direct communication between vitreous cavity and possible subarachnoid space; (C) horizontal SS-OCT image shows small retinal break at the edge of the peripapillary atrophy around the excavated optic disc; (D) postoperative Optos image showing retinal reattachment after the second surgery with silicone oil tamponade; (E) oblique SS-OCT image shows macular hole which was revealed after the initial surgery; (F) vertical SS-OCT image shows closure of macular hole after the second surgery with silicone oil tamponade and internal limiting membrane peeling.