|Year : 1961 | Volume
| Issue : 3 | Page : 60-62
Familial axial cataract
R Abreu, AT Gajria
Department of Ophthalmology, B.Y.L Nair Hospital, Bombay, India
|Date of Web Publication||7-Apr-2008|
Department of Ophthalmology, B.Y.L Nair Hospital, Bombay
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Abreu R, Gajria A T. Familial axial cataract. Indian J Ophthalmol 1961;9:60-2
Berliner describes good many -axial cataracts, like cataracts Pisciformis, Koralliform cataract, Floriform cataract and a number of others. All of them are of a hereditary nature and are rare in occurrence.
We present a series of congenital cataracts observed in three successive generations. Three of the members of the family have axial cataract, the fourth one has peripheral lenticular opacities. Two of these four members had diminished visual acuity. The members of the family examined, were the grandmother, her son, grand-son and grand-daughter.
Case 1 : Mrs. C. T., aged 70 years, presented herself with a history of dimness of distant vision for the last 5 years. There was no history of diabetes, or any other condition likely to give rise to a cataract. On examination with the slit lamp, in both the eyes were noticed minute opaque spots, bluish white in colour, in the axial region of the lens. Distribution of these opacities was irregular but most of them were outside the nuclear zone. Very few opacities were present away from the axial region. No pupillary membrane was present. On top of this, senile cataract with lamellar separation was starting in the lower periphery of the lens in the right eye.
Right eye : The disc was pale and showed shallow glaucomatous type of cupping.
Left eye : There was a nasal shifting of the vessels at the disc. Arteriosclerosis, Grade II could be seen in both the eyes. Her distant vision was partially diminished because of glaucomatous changes. Her visual acuity at present is counting fingers at 4 meters in the right eye and 6/60 in the left. Tension could not be taken.
Case 2: Mr. K. T., the son of the lady, aged 45 years. History : In 1937, lens opacities were detected in each eye on a routine medical check up. Vision was reported defective. In 1939, he was again examined and was told that glasses would not improve his vision on account of cataract. He therefore did not use glasses. At present he is presbyopic. There is no history of diabetes, convulsions, or fever, or any other history to suggest any cause for the juvenile cataracts in his eyes.
Vision : 6/12 in both eyes and j 2 with + 1.75 sphere.
Fundus : Nothing abnormal was detected in both the eyes.
Biomicroscopy : In the right eye remains of pupillary membrane can be seen coming from various directions from the iris, meeting on the surface of the lens at pigmented spots. There is a capsular cataract, white in colour 2 mm above the center of the lens. There is no posterior polar opacity.
There are bluish white opacities all over the lens, more marked in the axial region, and outside the foetal and embryonic nucleus.
In the left eye remains of pupillary membrane are less than those in the right eye. The bluish white opacities in the lens are similar to those in the right eye. There is no capsular or posterior polar opacity.
Case 3: Miss R. T., the daughter of patient 2 aged 14 years. History: Up to the age of 10 years the patient did not have any difficulty in reading from the black board in her school, because the class was small and she used to sit relatively nearer the black board. She did not have any difficulty in reading her books. She had trachoma for which she was treated with terramycin ointment. After that she moved to another school, where she had difficulty in reading from the black board when she sat beyond the second row of benches. She had difficulty in reading her books too. Glasses were tried but without effect. At present she has the same trouble. Again there is no history of convulsions, fever or diabetes.
Vision : 6/60 in both eyes which can be improved to 6/24 in each eye with -3 sphere. She can read only j 5 without glasses.
Fundus : A few myopic changes can be seen.
Biomicroscopy : Right eye remains of pupillary membrane can be seen meeting in a subcapsular opacity.
Bluish white small opacities are seen distributed in the axial region, more in cortex and less in nuclear zone. There were two big opacities in a radiating form in the axial region, one placed anteriorly in cortex and the other posteriorly. Both of them were separated by the nuclear zone containing a few opacities. There are areas of depigmentation in the iris stroma.
Left eye : There is no pupillary membrane. Lenticular opacities are more or less the same as in the right eye. Areas of depigmentation in iris stroma can be seen as in the right eye.
Case 4: Master N. K., aged 15 is the son of patient No. 2.
History : The patient has been using glasses for the last 3 years. There is no history of contributory importance.
Vision : Right eye counts fingers at 6 meters with-0.75 sph.,-1,25 cyl. 60◦ improves to 6/6.
Left eye counts fingers at 6 meters, with-2.5 cyl. at 165◦ improves to 6/12.
Fundus : Nothing abnormal is detected in both fundii.
Biomicroscopy : Discrete small bluish white opacities in the periphery of the lens can be seen in both the eyes. The central part is clear.
| Comments|| |
This is a report on familial cataract with a dominant heridity. We examined members of three successive generations and all were affected. The cataract is stationary in the first two members. From this it appears that it may not progress in the third and fourth cases.
The phenomenon of anticipation has been remarked by several observers in cataract in adults, so that if a cataract which in the first generation has a senile development appears in subsequent generations in earlier adult life, and eventually has a juvenile and ultimately a congenital incidence. We have not been able to find any reference about such anticipation in hereditary and familial lens opacities. Patients 1 and 2 to our knowledge were not examined in young age. Cases 3 and 4 have been examined. Is it likely that hereditary and familial lens opacities appear earlier in succeeding generations ?
This cataract looks like cataract pisciformis which is predominently an axial cataract showing axially located thin white opacities limited to either the posterior adult nuclear zone, or in the juvenile form to the region of the posterior outer fetal nuclear zone.
The capsular or sub-capsular opacities are associated with remains of pupillary membrane. Our observation in this series of cases is in conformity with that.
The last member of the family (Pt. No. 4) appears to have punctate cataract and not cataract pisciformis like the other three members. The slit lamp appearance in case 4, is probably due to mutation.
| Summary|| |
Case histories of four patients, one grandmother, her son and his daughter and son are given with a discussion on anticipation of cataracts in such cases.
| References|| |
Berliner-Biomicroscopy of the eye V0L. II, 1079-1085.
Duke Elder-Text Book of Ophthalmology, Vol. II, p, 1358, 65, 73, VOL III, p. 3194.
We thank Dr. A. M. Gokhale, D.O. (Lond.), D.O. (Bom.), D.O.M.S. (Born.), Head of the Department of Ophthalmology, B, Y. L. Nair Charitable Hospital, Bombay, for his valuable guidance.