Year : 1977 | Volume
: 25 | Issue : 4 | Page : 50--51
Bilateral peripapillary staphyloma (ectasia)
Daljit Singh, Arun Verma
Department of Ophthalmology, Medical College, Amritsar, India
Department of Ophthalmology, Medical College, Amritsar
|How to cite this article:|
Singh D, Verma A. Bilateral peripapillary staphyloma (ectasia).Indian J Ophthalmol 1977;25:50-51
|How to cite this URL:|
Singh D, Verma A. Bilateral peripapillary staphyloma (ectasia). Indian J Ophthalmol [serial online] 1977 [cited 2013 May 21 ];25:50-51
Available from: http://www.ijo.in/text.asp?1977/25/4/50/34617
Peripapillary ectasia is a deep excavation involving the posterior pole of the globe so that a normal nerve head with its normal arrangement of vessels is visible at the bottom of a pit in the surrounding tunics.
This condition is amongst the rarest congenital anomalies of the eye. Zimmermann  in 1897 described this anomaly in a living dog. The first human case was described by Stock and von Szily  in 1906. Since then a total of 16 cases have been described. Out of these only one case showed bilateral ectasias  .
Three of reported cases showed an intermittent and marked contraction of the walls of the ectasia (contractile peripapillary staphyloma) ,,.
Reported here is the first Indian case showing non-contractile bilateral peripapillary staphyloma.
S.S., 19 years male came with the complaints of defective vision in both the eyes since early childhood. Both fundi showed almost similar abnormalities.
On each side the disc was vertically oval, the length being about 2.5 times the breadth, surrounded by a thin strip of choroidal atrophy on the nasal side, above and below. On the temporal side there was a big crescent almost the size of the disc. White looking choroidal vessels were very prominent on the nasal side of the disc.
The choroidal vessels lateral to the crescent appeared normal. The disc appeared to be lying at the bottom of a pit which had very steep margins on the nasal side. Here the depth of the pit was about 10 D. On the temporal side there was only a slight depression which could be detected by an optical section in conjunction with Hruby lens or a Goldmann lens. The steep nasal margin of the pit appeared grey in colour. The retinal vessels crossing the ridge made a very sharp turn before flattening out on the retina. With careful optical section of the retina the limits of the depression could be traced for about 3 DD on the nasal side, 1.5 DD above and below and about 4.5 DD on the temporal side. The lateral part of the depression merged imperceptibly into the surrounding retina. The limits of the depression were also shown by the bending of the retinal vessels. The rest of the fundus was normal.
The fundus picture of the right eye [Figure 1] was taken with the help of Carl Zeiss Jena slit-lamp (not fundus camera). The improvised technique of photography will be described in a separate paper. The picture shows the disc and the surrounding retina. The grey ridge of the sclera represents the sharp nasal edge of the ectasia and is marked with arrows.
The peripapillary ectasia described above did not show any movement by breath-holding, Valsalva manouver or by throwing strong light into the same or the fellow eye.
Perimetery showed some enlargement of the blind spot commensurate with the atrophic areas around the disc. The grey ridge did not make any scotoma.
Uncorrected vision in both the eyes was 6/60 Corrected vision using-3.0 D sph. with-1.25 D cyl. axis 180 degrees, was 6/12 in each eye.
There was no systemic abnormality. Examination of other members of the family did not show any abnormality.
Peripapillary staphyloma needs to be differentiated from:
(a) Coloboma of the optic disc, in which the defect is within the abnormal nerve head itself.
(b) Myopic conus, in which the defect is usually temporal to the disc and progressive.
The depth of the pit has been measured in only six of the reported cases, and ranged from 8 to 20 D. The depth of the pit in our case was about 10 D.
Most of the reported cases had very poor vision. Hancock's  patient improved upto 6/18. The case reported by Caldwell et al  had normal vision in both the affected eyes. In our patient the vision in both the eyes improved to 6/12.
Histological study has not been done in any human case. Zimmermann  who found this condition in a dog performed histological studies. He found the thin scleral ectasia to be lined with remnants of retina and choroid. The optic nerve was atrophic with increase in the interstitial connective tissue. The nerve fibre layer was absent temporally but present elsewhere.
Francois et al  performed elaborate biometric studies on their patient of unilateral peripapillary ectasia with poor vision. All the measurements were within normal limits.
The development of peripapillary ectasia is probably due to the failure in the condensation of the mesoderm which consolidates to form the sclera, a process normally delayed at the posterior pole of the globe  .
The paper describes a rare case of bilateral peripapillary staphyloma. The staphyloma was non-contractile and was about 10 D in depth. The vision was poor but improved to 6,112 with glasses.
|1||Caldwell, J.B.H., Lears, M.L. and Gilman, M., 1971, Amer. J. Opthal., 71,423.|
|2|| Duke Elder, 1964, System of Ophthalmology, 3 part 2, p. 540, Henry Kimpton, London.|
|3||Francois, J., Goes, F. and Yobbagyi, P. 1967, Ophthalmologica, 154, 446.|
|4|| Hancock, L, 1907, Quoted by Wise et al.|
|5|| Kral, K. and Svarc, D., 1971, Amer. J. Ophthal., 71, 1090.|
|6|| Sugar, H.S. and Beckman, H., 1969, Amer. J. Ophthal., 68, 895|
|7|| Stock, W. and von Szily, A., 1906, Quoted by Wise et al.|
|8|| Wise, J.B., Maclenn, A.L. and Gass, J.D.N., 1966, Arch. Ophthal., 75, 426.|
|9|| Zimmermann, 1897, Quoted by Wise et al.|