|Year : 1972 | Volume
| Issue : 3 | Page : 109-112
Wandering lens and its management in a case of Marfan's syndrome
MR Jain, RL Agarwal, HR Sharma
Department of Ophthalmology, R. N. T. Medical College and Hospital, Udaipur, India
M R Jain
Department of Ophthalmology, R. N. T. Medical College and Hospital, Udaipur
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jain M R, Agarwal R L, Sharma H R. Wandering lens and its management in a case of Marfan's syndrome. Indian J Ophthalmol 1972;20:109-12
Incomplete bilateral symmetrical subluxation of the lens occurs in 80 per cent of cases of Marfan's syndrome (DUKE ELDER, 1964) but complete luxation into the anterior chamber or posterior chamber is rare (CHOYCE, 1957; FRANCO et al., 1948). Late spontaneous dislocation of the lens in Marfan's has been reported after the age of 20 years by HANDMANN, 1914; WESSELY, 1919; COUTURIER 1922; and Fox 1926). In these cases the lenses were small and spherical.
The case in question had late spontaneous dislocation of the lens in the anterior chamber leading to acute iridocyclitis. After dilatation of the pupil, the lens travelled to the vitreous chamber and revealed its attachment to the ciliary body by 2-3 zonular strands. From here the lens could voluntarily be brought in the pupillary area and could be manoeuvred to lie temporarily in the anterior chamber leading to the classical entity of `Wandering Lens' (DUKE ELDER, 1969). The lens when extracted was found to be small and spherical.
| Case Report|| |
A 22-year old male bus conductor had apparently normal eyes with useful vision till four years back. He never wore glasses. Once after a bus travel, he felt sudden chill in his body associated with severe pain and lacrimation in the left eye. Vision decreased considerably within 4 to 5 days. He consulted an ophthalmologist, who advised him immediate admission for operation. Patient somehow did not follow the advice and gradually became blind with shrinkage of the eye ball in due course of time.
On 4th May, 1970, during his evening stroll, he took a glass of water and suddenly felt a sever chill in the body followed by severe lacrimation and pain in the right eye. Next day he realized that his vision has decreased considerably and he attended the eye out-patient department on 8th May, 1970.
On examination of the right eye, he was found to have slight hazy cornea with marked ciliary congestion. Anterior chamber was deep and contained oil globule like mass (spheroid transparent lens) in its lower part [Figure - 1], Pupil was almost central, slightly irregular, of normal size and showed vitreous with lot of pigments deposited on its anterior surface. Light reflex was sluggish. Vision was finger counting at a distance of 3 meters.
Tension was 16 mm of Hg. by Schiotz tonometer.
Slit lamp examination showed slightly hazy cornea with few punctate pigmented keratic precipitates. Aqueous flare was two plus. Lens was transparent and freely movable. Iris pattern was lost and showed few patches of atrophy. Pupillary area showed degenerated vitreous with lot of pigment particles floating in the vitreous.
Left eye was completely blind and phthisical. No details of the anterior segment could be seen.
General Examination - Skeleton of the patient showed characteristic feature of Marfan's syndrome. Height was 6'-3" and showed arachnodactyly and dolicocephaly with lack of subcutaneous fat. All other systems were normal. [Figure - 3]
Family History - Out of eight issues to his parents, only he is alive. Mother had three abortions and father expired at the age of 45 years. Presence of the syndrome in the dead members of the family could not be elicited.
Investigations- Blood examination including Kahn test and blood sugar were normal. Urine culture revealed no micro-organisms.
| Management|| |
Intensive treatment with mydriatic , cortico-steroids locally, subconjunctivally and parenterally alongwith Hexamethyl-diaminoisopropanol di-iodide (*Entodon. I. V.), Oxyphenbutazone (**Tanderil tables) and Procain penicillin G in oil with 2% Aluminium Monostearate in oil (***PAM. I. M.) injections were given.
After dilatation of the pupil, the lens travelled to the vitreous chamber. Here the lens floated in the vitreous and quite often moved like a flap door to the pupil with its hinged attachment to the lower part of the ciliary body by few zonular strands [Figure - 2]
Within 15 days circumcorneal congestion completely disappeared, cornea became bright and aqueous flare was negative. The usual feature observed was that patient could voluntarily bring the lens in the pupillary area by blinking and obtain vision of 6/36. Fundus reflex improved considerably, revealing normal fundus. Tension was 18 mm of Hg. Schiotz.
After 25 days, i.e., 5th June, 1970, when the eye became absolutely quiet, 1 it was decided to take the lens out.
With atropinised pupil, the patient was laid prone on the bed. After a little manoeuvre, lens came to lie in the anterior chamber. In the same posture, 1 % drops of eserine were instilled frequently for 45 minutes but the pupil did not contract and the moment the patient was made supine, lens travelled back to vitreous chamber.
Instillation of miotics or mydriatics was stopped for 10 days leading to moderate contraction of the pupil. On 15th June, pupil was dilated with frequent instillation of 10% phenylepherine (DROSYN  ) drops and then the patient was laid prone for 15 minutes. Soon the lens came into anterior chamber, but stayed in front of pupillary area due to its zonular attachments. 2 % Pilocarpine and 1% eserine drops constricted the pupil sufficiently within 30 minutes to retain the lens in anterior chamber even when the posture was changed.
Inj. Pethidine 100 mg. with 5 mg. Siquil (Triflupromazine Hydrochloride) was injected by intravenous route and the patient was put on the operation table.
Operation: After proper anaesthesia and akinesia, an ab externo section of approximately 12 mm. was made near the upper outer limbus - and the lens was delivered intracapsularly by wire vectis. surprisingly without any disturbance of vitreous. Subconjunctival injection of dexamethasone, chloromycetin and mydricain was given and both eyes bandaged.
Post-operatively, the patient had uneventful recovery in normal course of time.
Condition on Discharge - Eye was absolutely quiet with normal tension and improvement of vision upto 6/24 with +10.00 D. Sph. Fundus - normal.
Check-up - On 10th July, 1970, the patient was found to have perfectly quiet eye with vision of 6/18 with + 10.00 D. Sph. with + 1.50 D Cyl. 180°. Fundus was normal.
On 24th July, 1970, he felt a little diminution in vision. Fundus examination revealed a shallow retinal detachment in the upper outer quadrant. No hole could be detected. He was advised admission but he did not get admitted and came on 8 th August, 1970, with total detachment. After conservative treatment for about 40 days an operation of scleral buckling with diathermy puncture and coagulation was attempted but as expected the patient's condition did not improve much and he is now having vision of only hand movements.
| Discussion|| |
Typical arachnodactyly with dolicocephaly, lack of subcutaneous fat and spherophakia suggested a diagnosis of Marfan's syndrome. History of death of 7 brothers and sisters of the patient is also in favour of Marfan's since in Marfan's mortality in childhood is high (SORSBY, 1958), though occurrence of severe iridocyclitis in the patient after complete dislocation of the lens associated with history of infantile deaths and abortions also presume the presence of veneral disease like syphlis.
We presume that the patient earlier had partly dislocated spheroid lens, but later on developed spontaneous complete dislocation in the anterior chamber which led to iridocyclitis and consequent blindness in the left eye. He came 4 days after he developed complete dislocation and iridocyclitis in right eye. Intensive treatment relieved him of iridocyclitis within 25 days.
As the presence of a floating lens in the anterior or posterior chamber is a potent source of iridocyclitis, secondary glaucoma and retinal detachment with eventual extinction of vision, extraction of the lens was planned. Since the extraction of the lens from vitreous chamber is much more difficult and dangerous, it is important to trap the lens in anterior chamber. For coaxing and trapping the lens in the anterior chamber, it is advisable to stop the use of atropine or hom-atropine 10 days earlier. Dilate the pupil with 10% phenylepherine drops and make the patient lie prone till the lens comes into the anterior chamber. In the same posture pilocarpine and eserine should be instilled to contract the pupil rapidly and strongly.
Inspite of uneventful intracapsular extraction of the lens and rapid improvement in vision, the patient developed total retinal detachment about 40 days after the operation confirming the important observation made by DUKE ELDER (1969) that `surgical extraction of the dislocated lens is followed more usually by no change or a deterioration in vision than by its improvement and may induce a retinal detachment'. In our case the retinal detachment seems to be due to vitreous attachments to retina which developed due to panuveitis.
As expected, the conservative and surgical treatment of the detachment was fruitless and the patient became almost blind.
| Summary|| |
A typical case of Marfan's syndrome is described. The case had spontaneous dislocation of lens leading to iridocyclitis and hinged wandering lens. After a successful treatment of iridocyclitis, the lens was coaxed into the anterior chamber with the use of phynylepherine drops and was delivered intracapsularly without any disturbance of vitreous. Inspite of uneventful recovery and good postoperative visual gain the patient developed total retinal detachment after about 40 days and resulted in blindness inspite of its medical and surgical treatment.
| References|| |
Choyce, D. P.: Anterior Dislocation of the Lens in Marfan's Syndrome. Brit. J. Ophthal. 41, 527 (1957).
Couturier: (1922) Cited by Duke Elder. Vol. III, Part 2, 1964.
Duke-Elder, W. S.: System of Ophthalmology, Vol. III, Part 2, page 710. Henry Kimpton, London. (1964). System of Ophthalmology, Vol. XI, page 306 & 311. Hentry Kimpton, London. (1969).
Fox: (1926) Cited by Duke Elder, in 3.
Franco et al (1948) Cited by Duke Elder in 3.
Handmann: (1914) Cited by Duke Elder in 3.
Sorsby, A.: Systemic Ophthalmology, 2nd edition, p. 68. Butterworths & Co. Ltd., London. (1958).
Wessely: (1919) : Cited by Duke Elder in 3.
[Figure - 1], [Figure - 2], [Figure - 3]