Long-term study of patients with congenital pit of the optic nerve and persistent macular detachment.

During the period 1970-87 we evaluated the changes of the optic disc, peripapillary area, detached macula and visual acuity in 16 cases with congenital pit of the optic nerve and macular detachment. The study revealed in 9 of the 16 cases (56%) an increase of the dimension of the pit or changes in its color, findings which were directly related to the duration of the macular detachment. Chorioretinal scarring, pigment migration, or both, were also noted mainly at the temporal margin of optic disc. In 5/16 cases we found during the follow-up an extension of macular elevation. In altogether 10 out of 16 cases the retinal elevation covered the larger portion of the mid-periphery temporally. In 7/16 cases the final visual acuity remained unchanged, in 9/16 cases deteriorated. The difference, however, in the latter 9 cases between initial and final visual acuity was negligible. During the follow-up period deterioration of the visual fields was also noted.

Cilioretinal arteries in conjunction with a pit of the optic disc.

Cilioretinal arteries were noted to accompany pits of the optic disc in 16 of 25 cases (64%). Fourteen of the 16 cases were found to have 2 cilioretinal arteries (86%). The cilioretinal arteries were directly related to the pit of the disc and emerged either from the bottom or the margin of the pit in a proportion of 86%. This finding reinforces the assumption that the optic disc pit belongs to the same spectrum of congenital anomalies which also includes optic disc coloboma, megalopapilla and morning glory syndrome. A fluorescein angiography study showed hyperfluorescence of the pit in 12/16 cases and in particular in all the cases in which both cilioretinal arteries emerged from the pit. Hypofluorescence was noted in 7/9 cases in which the pit was not associated with cilioretinal arteries. Our findings could support the view that hyperfluorescence of the pit, which is not a constant phenomenon, mostly depends on the presence of cilioretinal arteries emerging from the pit.