|Year : 1989 | Volume
| Issue : 4 | Page : 202-204
Bilateral posterior lens dislocation in Marfan's syndrome
Atul Kumar, SP Garg, Lalit Verma, Surinder Kumar
Dr. R.P. Centre for 'Ophthalmic Sciences, AIIMS, Ansari Nagar, New Delhi-110 029, India
S P Garg
Dr. R.P. Centre for 'Ophthalmic Sciences, AIIMS, Ansari Nagar, New Delhi-110 029
Source of Support: None, Conflict of Interest: None
Complete dislocation of the lens is a rare feature of Marfan's Syndrome. We observed four cases of bilateral complete posterior dislocation of the lens in established cases of Marfan's Syndrome over a one year period. An ophthalmologist may encounter infrequently, unilateral posterior dislocation in one eye and ectopia lentis in the other, but bilateral spontaneous posterior dislocation is rare. All our cases had associated vitreous degeneration with vitreous herniating into the anterior chamber. The pathogenesis of complete posterior dislocation in Marfan's Syndrome is discussed.
Keywords: Marfan′s Syndrome, Ectopia lentis, Subluxation, Posterior lens dislocation, Vitreous degeneration
|How to cite this article:|
Kumar A, Garg S P, Verma L, Kumar S. Bilateral posterior lens dislocation in Marfan's syndrome. Indian J Ophthalmol 1989;37:202-4
|How to cite this URL:|
Kumar A, Garg S P, Verma L, Kumar S. Bilateral posterior lens dislocation in Marfan's syndrome. Indian J Ophthalmol [serial online] 1989 [cited 2020 May 25];37:202-4. Available from: http://www.ijo.in/text.asp?1989/37/4/202/26043
| Introduction|| |
Marfan's Syndrome has been reported to occur in 4 to 6 per 100000 of population . Ocular features of this syndrome have been extensively reported . Ectopia lentis, the most common ocular feature, occurs in 70 to 80 % of cases. Isolated reports of spontaneous complete posterior luxation lying over the retina, or producing secondary complications like glaucoma or uveitis exists in literature . However, bilateral posterior dislocation is a rare feature. In this study we report four cases of established Marfan's Syndrome with bilateral spontaneous posterior dislocation seen over a period of one year. The pathogenesis of spontaneous dislocation in Marfan's Syndrome is discussed.
| Case report|| |
Case Report 1 : A 35-year-old female presented with acute painless visual Toss in the left eye of eight days duration. On systemic examination, findings consistent with diagnosis of Marfan's Syndrome were found [Table - 1]. Ocular examination revealed a visual acuity of absent light perception OD, and counting figures at half foot OS. The right eye had extensive rubeosis iridis and a dense pupillary membrane which prevented visualization of posterior segment. The left eye had a small (1.5 mm) pupil, not dilating with mydriatics. Evidence of vitreous degeneration was present in both eyes. Fundus evaluation of the left eye showed a dislocated lens lying in the posterior vitreous with an associated total retinal detachment and a single horse-shoe tear 1'Oclock position.
Ultrasonography of the right eye revealed spikes consistent with a total retinal detachement, with a posteriorly dislocated lens. Intraocular pressure recorded was 38 mm Hg OD and 18mm OS. The patient underwent Successful retinal detachment surgery in the left eye and had visual acuity of 20/200 at 6 months follow up.
Case Report-II: The patient was a tall-statured 40-year old male with long, thin extremities. Arachnodactly, prognathis_raand a high arched palate were also present. On the basis of these findings and the results of other investigations [Table - 1] the patient was diagnosed as a case of Marfan's Syndrome. Ocular examination revealed visuals acuity of hand movements with accurate projection OD and 20/60 OS. Small sized (2.5 mm) pupils reacting sluggishly and resistant to dilatation were present bilaterally. Fluid vitreous, hearniating and touching the posterior corneal surface, was seen in both the eyes. Posterior dislocation of lenses was seen in both the eyes and there was an associated total retinal detachment in the right eye with two horse-shoe tears lying at the 12'Oclock meridian. Applanation intraocular pressure measured 16 mm Hg OU. Surgery for retinal detachment was successful with the patient gaining a vision of 20/70 at six weeks post operative.
Case Report-III: A 26-year-old female presented with sudden onset of total visual loss in the right eye of four months duration. She had skeletal abnormalities of dolicostenomelia (long, thin extremities), arachnodactly and high-arched palate [Table - 1]. Visual acutiy was projection of light OD and 6/60 OS. There was bilateral posterior dislocation of lenses with a total old retinal detachment and advanced proliferative vitreo-retinopathy (PVR) in the right eye. In view of the old retinal detachment and advanced PVR, no surgery was undertaken.
Case Report-IV: The patient, a 38-year-old female presented with a week long history of marked visual loss in the right eye. The extermities were long, thin with associated hyperflexibility of the joints, arm span (200 cm) was greater than the height of the patient (180 cm) and there was an associated high arched palate [Table - 1]. Ocular examination revealed visual acuity of counting finger 1 ft. OD and 20/30 OS. Posterior segment examination revealed a degenerated liquified vitreous and posterior dislocation of lenses in both the eyes with presence of a total rhegmatogenous retinal detachment in the right eye. The right eye was successfully operated for retinal detachment and corrected vision at 6 weeks follow up was 20/30 OD.
| Discussion|| |
Ocular involvement is one of the commonest feature of Marfan's Syndrome. Of the ocular mainfestations, ectopia lentis occurs in 50-80% of be patens ,,,, Ectopia lentis remains stable in early childhood, and only progresses in the second decade, when it may produce symptoms like pupillary block glaucoma and uveitis. It has been observed that in subluxation, the zonules are broken from the equator of the lens resulting in displacement of the lens from its position, whereas in ectopia lentis, due to zonular laxity the lens gets moved from its position. In Marfan's Syndrome, the zonules can be seen in position on slit-lamp examination and on surgical removal, it is difficult to break the zonules in these cases. Due to these findigs, lenticular displacement in Marfan's has been termed as ectopia lentis and not subluxationof lens.
This hypothesis however, cannot explain complete anterior or posterior dislocation, both of which have been reported in this syndrome. Posterior dislocations though rare, has been reported in 3% patients of Marfan's Syndrome . Anterior dislocation is symptomatic and presents as pupillary block glauoma or secondary uveities. Posterior dislocation may be silent and rarely causes glaucoma or uveitis.
Viterous changes are well described in Marfan's Syndrome . Commonly observed changes are liquefaction of the gel and posterior vitreous detachemt (PVD). Presence of fluid vitreous in the anterior chamber and PVD in all our cases are in agreement with the above description. Posterior dislocation of lens could have been augmented by the disruption of anterior hyaloid face resulting in loss of support.
This study thus highlights two aspects, namely that posterior dislocation of lenses in Marfan's Syndrome seems to be more common than usually described and that cases with posterior dislocation of lenses tend to have more severe vitreous pathology.
| References|| |
Cross HE & Jensen A.D.: Ocular mainfestations in Marfan's syndrome and homocystinuria. Am J Opthalmol. 75:405,1973,
Croxwatto JO, Laobardi, A, Malbream ES: Phacotoxic ' ndopthalmities with Posterior dislocation lens in Marfan's Syndrome Ophthalmologica 193: 23-26, 1986.
Feilder AR: Dislocated lenses: Paediatric Ophthalmolgy, Current aspects, Edited by K. Wybar & D. Taylor. Marcel Dekkel Inc. Publ., Ch. 17, p 169-170, 1983.
Hindle NW, Crawford JS: Dislocation of the lense in Marfan's syndrome Can J Ophthalmol 4:128-134,1969.
Jarret WH Jr. Dislocations of the lense. A study of 166 hospitalised cases. Arch Ophthalmol 78:289.296,1967.
Maumenee IH: The eye in Marfan Sydndrome. Trans Am Ophthalmol Soc 79:684-733 1981.
Nelson LB, Maumence IH: Ectopia Lentis, Surv Ophthalmol 27:143-160,1982.
Pyeritz RE, Mckusick VA The Marfan Syndrome: Diagnosis & Management New Engl J Med, 300: 772-777,1979.
Schepens CL: Retinal Detachment and Allied Disease. Vol. 2, 1983 W.B Saunders Co. Ch. 28, 653, 1983.
[Table - 1]