Operative treatment of ectopia lentis in marfan's syndrome

Suresh Kumar

Users Online: 7835

ARTICLE

Year : 1960 | Volume : 8 | Issue : 4 | Page : 87-91

Operative treatment of ectopia lentis in marfan's syndrome

Suresh Kumar
Department of Ophthalmology, G. S. V. M. Medical College, Kanpur, India

Date of Web Publication

5-May-2008

Correspondence Address:
Suresh Kumar
Department of Ophthalmology, G. S. V. M. Medical College, Kanpur
India

Source of Support: None, Conflict of Interest: None

Check

How to cite this article:
Kumar S. Operative treatment of ectopia lentis in marfan's syndrome. Indian J Ophthalmol 1960;8:87-91

How to cite this URL:
Kumar S. Operative treatment of ectopia lentis in marfan's syndrome. Indian J Ophthalmol [serial online] 1960 [cited 2023 Dec 8];8:87-91. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1960/8/4/87/40672

Table 2

Click here to view

Table 1

Click here to view

Marfan's Syndrome is a rare hereditary disease of a dominant trait with a high familial incidence. The characteristics are : tall, slim built stature ectopia lentis, arachnodactyly with long hands and feet, congenital heart defects, high arched palate, spinal deformities, generalised muscular under-development and mental retardation. All or some of these characters may be found in a typical case.

The subluxation of the crystalline lens is an important feature of the disease. It is usually bilateral and dates from birth. The cause of dislocation is a failure in development of the suspensory ligament which is found to L under-developed r absent over a sector of its circumference. The more developed portion of the ligament, which is commonly the tipper, pulls upon the lens dislocating it usually up and out, thus leaving the pupil divided into two regions-one phakic and the other aphakic. Freed from its ligamentous attachment, the lens, besides getting dislocated assumes a more spherical form, becomes tremulous and gets tilted. This introduces a grave visual defect in the phakic portion of the pupil, the nature of which is of high myopia with a moderate degree of astigmatism. The chief and only complaint of the patient is one of defective vision and this leads him to seek the advice of the ophthalmic surgeon first. In some instances the defect can be remedied by prescribing suitable glasses for either the phakic or aphakic portion of the pupil; but in others, inspite of the serious hazards involved, more heroic measures such as extraction of the lens has to be attempted.

The aim of this paper is to present four cases those of two pairs of brothers, of typical Marfan's Syndrome and in two of them the simple expedient of an optical iridectomy performed on both the eyes produced excellent visual results, with none of the risks and complications that accompany more serious operations like extraction.

Case Histories

The case histories of the four cases are tabulated for better visualization. [Table - 1] All the four cases presented the general features of Marfan's Syndrome and arachnodactyly with some slight variations.

In all of them there was iridodonesis with deep and irregular depths of anterior chambers depending on the degree and direction of the subluxation of the lens and the tension was normal.

In case 3, with an undilated pupil, only the phakic portion was used for visionary whereas with a dilated pupil the aphakic area gave better vision.

Old records of his eye examination which the patient was carrying revealed that he was first examined by an ophthalmic surgeon in August 1934. and was diagnosed to be a case of congenital binocular subluxation of the lens. He had been prescribed bifocal glasses. Vision in R. E. with + 6.0 sph. in both upper and lower segments was 6/24 and J12. In the L. E. he was advised --6.o sph. for distance with 6/24 vision and for near- 3.0 sph. with J4 vision. The patient said the glasses were very comfortable and had served him usefully for the last 24 years.

Operation

Both eyes of cases 1 and 2 were operated upon in March 1055. Case 3, MM. L. M. was advised operation in the right eye but he preferred to try glasses for some more time. Case 4, A. L. M. was not advised operation a chances of visual improvement were remote. In the 4 eyes operated the operation performed was an Optical Iridectomy in lower nasal quadrant, the aim being to excise only that much of iris tissue as would leave a sufficiently large aphakic area for subsequent correction with suitable glasses.

Complications

While operating upon the L.E. of M.Q.K., vitreous presented in the wound immediately after the incision had been completed with the von Graefe's knife. After snipping with scissors the vitreous fell back and the iridectorny was completed. In subsequent operations this complication was prevented by instilling miotics prior to surgery.

Results

The eyes were refracted six weeks after surgery and from the table given below it will be observed there was marked visual improvement in all the 4 eyes. Both eyes of M.Q.K. had a visual acuity of 6/18 for distance and J2 for reading. Results were even more encouraging in the case of M. F. K. in whom a vision 6/12 for distance and J2 for near was achieved.

When examined one year later, the vision was maintained in both eyes of M. F. K. and also in the right eye of M. Q. K. But the left of M. Q. K. had become soft with complete absence of vision. This could not be attributed to vitreous loss during operation as he gave a history of having an attack of ''sore eyes" with consequent ulceration and panophthalmitis in the L.E., his right one being spared.

Discussion

The problem of the partially dislocated lens in cases of Marfan's syndrome is a ticklish one. In some instances visual improvement follows correction of the phakic or aphakic portion of the pupil with suitable glasses. In others operative intervention is indicated and justifiably undertaken depending upon the ingenuity and skill of the ophthalmic surgeon. But by and large the results are very poor indeed.

Operations such as iridotesis, sphincterectomy and iridotomy have been tried by D'Oeuch (1881), Pere (1912), Howell (1934), Rengelhan (1931), and others. Couching has been advocated by Elliot (1918), and needling by Decker (1934) Jackson (1918), Strickler (1918), Lambert (1919), and Parson (1920). All these procedures have proved unsatisfactory and at best are only palliative.

Clarke (1939) analysed the records of 31 patients with congenital dislocation of the lens treated at the Vanderbilt Clinic. In his series operations were performed on 21 eyes. The operations done were needling 3 eyes, preliminary iridectomy without extraction 3 eyes, extraction with iridectomy 2 eyes, extraction without iridectomy r eye and extraction after iridectomy 10 eyes. In the 3 eyes in which iridectomy alone was performed, in one no improvement in vision took place probably due to amblyopia. In the second case there was slight loss of vitreous with good recovery. In the third although there was no loss of vitreous, hypotony and retinal detachment followed. According to Clarke (1939) , the best procedure in cases requiring surgical intervention was preliminary iridectomy followed by a loop extraction of the. lens. This was performed upon To eyes but vitreous loss took place during preliminary iridectomy in 50`' of the cases and in 70 there was further vitreous loss at the time of lens extraction. These results only go to prove the inadvisability of such extraction.

According to Ralph Llyod (1948) who has reviewed 24 cases of typical or atypical Xlarfan's Syndrome, any operation is hazardous, because of the faulty construction and lowered vitality of the eyes of these patients and that the type of operation is not significant. In his series 12 operations were performed on 7 eyes of 9 patients, and complete success was achieved in only one of them and that too where the lenses were removed from the anterior chambers.

Spaeth (1944) and also Cowan have suggested iridectomy to expose a lens free area in cases of ectopia lentis. The results they have obtained compel serious consideration of this procedure. According to Spaeth, aphakic vision from an iridectomy is certain; also an iridectomy. is a less serious operation. Needling has a rather high percentage of complications and attempted intracapsular extraction is always accompanied by serious complications.

The primary aim of undertaking operation in the 2 cases reported in this paper, was the improvement of visual function. In both the cases marked visual improvement followed correction of the aphakic region with strong convex lenses. Hence it was decided to permanently enlarge the aphakic area of the pupil by performing an optical iridectomy in the lower nasal quadrant. This site was chosen for its easy approach, safety from surgical complications and enlargement of the pupil in this direction which provided the best aphakic area. To perform iridectomy in the upper part so as to place the pupil more nearly over the centre of the lens, was thought undesirable because improvement in vision with convex glasses was unlikely, owing to opalescence, tremulousness and high astigmatism of the crystalline lens. Other procedures such as discission and extraction of the lens were also considered. The immediate dangers of needling are small but it was not adopted because repeated attempts are necessary to produce results of doubtful value. Loop extraction of the lens is best with technical difficulties with chances of serious surgical complications such as vitreous loss during operation and the development of retinal detachment secondry glaucoma and chronic iridocyclitis post-operatively. The safety and utility of optical iridectomy, are vindicated by the very good results of our cases.^[27]

Summary

Four cases of typical Marfan's Syndrome are presented.
The vision in three of these cases could be improved with strong convex glasses which corrected the aphakic portion of the dilated pupil.
Optical iridectomy aimed at permanently enlarging the aphakic area of the pupil was performed upon 4 eyes.
The results justify the surgery undertaken.
Complication of vitreous loss can be prevented by instilling miotics prior to surgery.
A plea is made for adopting this procedure in suitable cases of Marfan's Syndrome.

References

1.	Bellows, J. G, "Cataract and Anomalies of the Lens,1944 p. 260. C. V.Mosbv Co., st. Louis.
2.	Carroll, F. D. (1945) in discussion on Lloyd, Arch. Ophth. 40, 558.
3.	Clarke, C. C. (1939) Arch. of Ophth. 21, 124.
4.	Consul, B. N.; Kulshesta, O.P., and Kasliwal, R. AI. (1960) J, Ind. Med. Assn., 35, 218.
5.	Das T.; Gulati, G. C.; and Malhotra,R. P. (1953) J. All-Ind. Ophth. Soc. 1, 3.
6.	Decker, P. H. (1934) Pennsylvania M. J. 37, 1008.
7.	D'Oeuch, F. C. (1881) Beitrage zur Kenntisis der Ectopia lentis congenita, Inang. Dissert., Strassburg, Wiesbaden, J.F, Bergmanrr, (1879) Arch. Ophth. 10, 89
8.	Driver, A. H. (1926) Ind. Med. Gaz., 41 , 23.
9.	Duke-Elder, W. S. "Text Book of Ophthalmology", 1938 Vol .2.P 1351, C. V. Mosby Co.. St. Louis.
10.	Elliot, R. H. (1918) The Indian Operation of Couching for Cataract, New York, Paul. B. Hoeber,
11.	Hansraj (1935) Ind. Med. Gaz.70, 81.
12.	Hine_ M. L. (1922-2;) Proc. Rcy. Soc. Med. (Sec. Ophth.). 16, 12.
13.	Horner, W. D., and Noisier, S. (1933) Tr. Sect. Ophth., A. VLA., 126.
14.	Howell, S. C.(1934)J. M. A. Georjia, 23, 186.
15.	Jackson, E., in discussion on Strickler. (Sec 26 below).
16.	Lambert, AV. E.(1919) Arch. Ophth., 48, 84.
17.	Lloyd, Ralph. (1948), Arch. Ophth. 40, 558.
18.	Marfau, A. B. (1896) Bull et mean. Soc. Med. D'hop de Paris 13, 220.
19.	Ormond, A. AV. 31 Williams, If, G. (1924) Guy's Hosp. Rep., 47, 385.
20.	Parson_. J. H . (1920) Proc. Roy Sac., Med. (Sect., Ophth.) 13 , 57.
21.	Pere, J. (1912) Contribution a letude des deplacements congenitaux du cristaliin : etiologie, Pathogenic, anatomic pathologique; complications et traitement, Thesis, Bordeaux, No. 127.
22.	Radox, R. (1942) ''Marlan's Syndrome", Arch. ()phth. 27, 477.
23.	Ringelhan, O.&,Elschnig, A. (1931) Arch. f. Augcnh 104, 325.
24.	Spaeth, E. B. (1944) "Thc Principles and Practice of Ophthalmic Surgery," P. 607, Lea & Febiger, Philadelphia.
25.	Spiece, W. K.(1918)Am.J.Ophth.,1,681.
26.	Strickler, D. A.(1918)Ani. J. Ophth. 2, 526.
27.	Wray, C. (1914) Tr. Op Soc. U Kingdom, 34, 167.