|Year : 1980 | Volume
| Issue : 4 | Page : 223-225
N Sen, NT George, VP Munjal, SP Dhir, IS Jain
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160 012, India
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160 012
|How to cite this article:|
Sen N, George N T, Munjal V P, Dhir S P, Jain I S. Congenital aniridia. Indian J Ophthalmol 1980;28:223-5
Congenital Aniridia is uncommon (0.04%). Inspite of the name there is always a circumferential vestige of the iris in the angle,.
It features a galaxy of ocular and systemic manifestations ranging from microcornea, conical cornea, corneal dystrophy, ectopia lentis, cataract and foveal aplasia to obscure associations such as cryptorchism, hypospadias and Wilm's tumor.
We present three cases, one illustrating the futility of filtering surgery in established aniridic glaucoma while the other draws attention to the rare finding of intra retinal lipid deposits and the third links congenital aniridia and buphthalmos to present an unusual clinical combination.
| Case reports|| |
Case No. 1: Miss L, 6F presented with a history of diminished vision in both eyes since birth associated with headache and pain in the eyes.
On examination, visual acuity was counting finger four feet in both eyes with nystagmoid movements. Both eyes showed mild conjunctival and ciliary congestion. The cornea was clear, A.C. irregular with an ectopic cataractous lens at 12 O'clock position in both eyes [Figure - 1]. Fundus examination showed cupping of optic nerve head in both eyes with partial glaucomatous optic atrophy. Gonioscopy showed a small stump of iris adherent all around to posterior corneal surface covering the trabecular region. The intra ocular pressure (I.O.P.) in both eyes was 59 1mm Hg. Scheie's cautery was done in the RE. Subsequently she was operated for glaucoma in the LE. On follow-up the I.O.P. remained high. She was put on Epitrate drops and Diamox. Subsequent examination revealed nonfunctioning bleb in the left eye.
Scheie's cautery was repeated in the left eye on the temporal side. In the postoperative period she had a ciliochoroidal detachment which settled. But the I.O.P. was of 59.1 mm in the left eye and 23.1 mm in the right eye. Scheie's cautery was repeated for the third time in the left eye after a months interval. In the immediate post operative period she again developed a ciliochoroidal detachment which soon settled uneventfully. Later on the I.O.P. was found to be under control in the left eye.
Case 2 :- Miss JK IOF presented with a history of poor vision in both eyes since early child-hood. She had headache on and off since 6 years of age.
The patient has two brothers and one sister. Parents were alive and well. There was no similar history in the family. On examination the visual acuity was counting finger four feet in both eyes. The axes were parallel. There was no nystagmus. The right eye showed conjuctival and ciliary congestion. The cornea was clear. There was total aniridia and an ectopic cataractous lens at 12 0' clock. The left eye showed the same findings [Figure - 2]. The fundi in both eyes showed glaucomatous cupping R>L. The maculae were not discerned.
The peripheral retina showed multiple white, discrete specks, not related to vessels and concentrated just behind the ora serrata.
Gonioscopy showed a closed angle in both eyes. The iris processes were adherent to the posterior corneal surface. The I.O.P. recorded was 50.6 mm in both eyes. Scheie's cautery was done in the right eye. Post operatively the bleb was prominent and the lop low. On the last follow-up the I.O.P. appeared to be controlled. None of the patients family members showed any abnormality. The systemic examination was normal in both patients.
Case 3:- RK-9M gave a history of pain, redness of both eyes and protrusion of left eye since birth with a recent increase since one and a half years. On examination the visual acuity was hand movements close to face in the right eye with perception of light and accurate projection in the left eye. A coarse nystagmus was present.
The right eye showed total aniridia with ectopic lens at 12 0' clock position. The fund us showed a deep cup with some pallor of the disc. The remaining retina was normal.
The left eye was large with a widened palpebral fissure. There was conjuctival and ciliary congestion. A wide ciliary staphyloma was present and the corneal diameter measured 14mm x l4mm Dense corneal edema and corneal infiltrates along with peripheral corneal neovascularisation were prominent. The fundus glow was not visible. The tension in both eyes was 59.1 mm. The patient was not co-operative for gonioscopy. Scheie's cautery was done in the right eye. Postoperatively the tension was well under control. None of the patients' family members showed any abnormality.
| Discussion|| |
Secondary late glaucoma is the rule in congenital aniridia,. The peripheral vestige of iris tissue present in all aniridia patients is largely responsible for the glaucoma as it engulfs the trabecular mesh work by creeping substitution. In their vast experience they have found little to eulogies filtering procedures. Gonioscopy has stood the test of time as an effective procedure.
But even the protagonists of goniotomy sound dubious about its role in established glaucoma. The real panacea then, to this problem of intractable glaucoma is early prophylactic goniotomy.
Filtering surgery in any form seems to have little place in the management as is evident from the 1st case on whom it was done on 3 occasions. A satisfactory explanation for this failure is lacking. Grant et a1 achieved 100% control in six of thirty one patients on whom goniotomy was done as a prophylactic procedure over a seven year follow up. In the wake of our experience we recommend early prophylactic surgery, the choice of which over the bitter years of failure has rested on goniotomy,.
The unusual feature noted in the second case was the presence of numerous, discrete white dots in the peripheral retina. Mention has been made of these deposits as a feature of some cases of sporadic aniridia. These have been shown to be intra retinal lipid deposits by histochemical procedures. The implications of this finding are conjectural.
In the third case, the buphthalmic left eye with its gross ciliary staphyloma and raised tension left no doubt that the process had its inception at birth. The onset and progress of glaucoma in this case is contrary to the acknowledged that secondary glaucoma in aniridia is a late occurrence. Buphthalmos is decidedly rare in congenital aniridia.
All the patients seen in this study were cases of mutational aniridia. Attention is drawn to the frequent association of wilm's Tumour in sporadic Aniridia. None of our patients showed any evidence of Wilm's tumour or other systemic abnormality.
| Summary|| |
Three cases of sporadic aniridia are presented documenting some unusual features.
| References|| |
|1.||Blake, E.M , 1953, Amer. J. Ophthalmol. 36, 907. |
|2.||Mann, Ida, 1957, Developmental abnormalities of the Eye. llnd Edition P. 230, London B.M:A. |
|3.||Duke Elders, S, 1964, System of Ophthalmol. Vol. III p, 66. Henry Kimpton London. |
|4.||Fraumeni, J. 1968, J.A.M.A. R. 206, 825. |
|5.||Grant, M., 1974, Trans. Amer. Soc. 72:207 |
|6.||Barkan, Otto, 1953, Arch. Ophthalmol. 49: 1. |
|7.||Jesberg, D.O., 1962, Arch. Ophthalmol. 68: 331. |
[Figure - 1], [Figure - 2]