|Year : 1980 | Volume
| Issue : 1 | Page : 33-35
Ectopia lentis in a family
Ajit Sinha, Azizur Rahman
Department of Ophthalmology Nalanda Medical College and Hospital, Patna, India
Department of Ophthalmology Nalanda Medical College and Hospital, Patna-800007
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sinha A, Rahman A. Ectopia lentis in a family. Indian J Ophthalmol 1980;28:33-5
Congenital subluxation of lens in a family is a rare entity. Hence three cases of bilateral ectopia lentis in a family is being reported.
Ectopia lentis is defined as congenital subluxation of lens. It may be pointed out at the outset that congenital subluxation of lens occurs basically in four forms: (1) Simple ectopia lentis (2) Marfan's syndrome (3) Marchesani's syndrome (4) Etcopia lentis with homocystinuria.
| Case reports|| |
R.P.M., a 40-year Hindu male, farmer by profession was admitted in the eye ward of Nalanda Medical College Hospital, Patna, presenting with the complaints of gradual diminution of vision in right eye more than left eye of about 30 years duration. Family history revealed that a daughter and a son had defective visions. On examination, visual acuity in right eye was Finger counting without glasses and 6/ 18 with+ 14 D Sph through phakic area. In left eye it was 6/36 through aphakic area without glasses which did not improve with glasses. Slit-lamp examination in right eve showed subluxation of lens in up and in direction, Part of the pupillary area was aphakic and part was phakic. Lenticular opacity in right eye was more than in left eye. Vitreous had come anterior to the lens only on temporal side. Suspensory ligaments were deficient on the temporal side. Left eve showed subluxation of lens on nasal side, part of the pupillary area was aphakic and part was phakic. Vitreous was found to be in place, suspensory ligament was visible and intact on the temporal side. Gonioscopy revealed filteration angles of both eyes to be wide (Grade 0). It was slightly narrow in the area towards which the lens had subluxated (Grade II). Fundii were normal. Intraocular tension was within normal limits in both eyes. General examination did not reveal any congenital anomalies of any system. All investigations including qualitative tests of urine by cyanide-nitroprusside test and paper chromatography(8) for homocystine were normal. With the above findings a diagnosis of bilateral simple ectopia lentis was made. Postoperative cataract extraction in right eye was not uneventful. It was complicated by vitreous disturbance and uveitis which was controlled by routine measures.
K.K., a 7-year Hindu girl and 5th living child of R.P.M. (Case 1) presented with the complaint of gradual diminution of vision in both eyes. On examination, VR 6/36, VL 6/36 which did not improve with glasses. Slit-lamp examination in right eye showed subluxation of lens in up and out, and in left eye in down and out positions. In both eyes, part of the pupillary area was aphakic, and part was phakic, lenses were transparent, suspensory ligaments were visible and intact, the vitreous was in position. Gonioscopy of both eyes revealed the filteration angles to be wide (Grade 0). It was slightly narrow in the areas towards which the lens had subluxated (Grade I & II). Fundi were normal. General examination did not reveal any congenital systemic anomalies. Investigations did not reveal any abnormality in blood and routine urine. Homocystine was absent in urine. A diagnosis of bilateral simple ectopia lentis was made.
S.K., a 3½-year Hindu boy and 6th living child of R.P.M. (Case 1) presented with defective vision since birth. On examination, vision could not be recorded as the child was too small to cooperate. He had alternate divergent squint of about 15°. On Slit-lamp examination both eyes showed subluxation of lens in up and out directions [Figure - 1], part of the pupillary area was aphakic and part was phakic, Lenses were transparent. Suspensory ligaments were visible and intact. Vitereous was in position. Gonioscopy and Tonometry could not be done. Fundi were normal. General examination did not reveal any congenital anomalies of any system. Investigations did not reveal any abnormality in blood and routine urine. Homocystine was absent in urine and a diagnosis of bilateral simple ectopia lentis was made.
| Discussion|| |
In the absence of Homocystine in urine and other clinical manifestations of Marfan's and Marchesani's syndrome the cases were labelled as cases of Simple Ectopia Lentis. The latter is a rare condition, its incidence as recorded by Lieberman, Podos and Hartstein (1966) 5.2% and by Nirankari and Chaddah (1967) 18%, among all varieties of subluxation of the lens,. The present reported family of eight had ectopia lentis only among father (40 years) and one daughter (7 years) and a son (3 years). Since simple ectopia lentis is strongly a hereditary condition, the inheritance being usually dominant, a number of individuals affected in a number of generations in one family have been reported by various authors. One of the most interesting pedigree of two generations (such as our series) is that of Adams' in which a mother and seven children were found to be affected. Reports have appeared in the literature in which cases have been reported in 3,4,5 and 6 generations.
The subluxation of the lens in simple ectopia lentis is said to be typically bilateral and symmetrical, a mirror-image in the two eyes and usually in upward direction or up and out. In all our cases we found the lens to be subluxated a symmetrically. This is quite consistent with the findings of Dorsch who also found 3 asymmetrical cases in 73 cases. The downward subluxation is an uncommon phenomenon in ectopia lentis in which it is usually in upward direction, but is a common feature in homocystinuria. The subluxation in left eye in Case 2 was found to be in down and out position. This is quite consistent with the findings of Dorsch! who found only one case in down and out position in 73 cases.
In case 1 the attachment of the vitreous to the posterior capsule of the lens (hyaloideocapsular ligament) had to be cut in order to deliver the lens. This attachment explains the frequent loss of vitreous which accompanies the extraction of such a lens. Cases have been reported where ectopia lentis is also associated with other congenital malformations, but in our series no associated congenital lesion was present.
The ocular complications that may occur in cases of Ectopia Lentis are (1) cataract (2) complete dislocation of the lens (3) anterior uveitis (4) secondary glaucoma and (5) retinal detachment. None of the cases reported developed any of the complications noted above except cataract in Case 1 and alternate divergent squint in Case 3. A subluxated lens may remain transparent indefinitely though occasionally, it turns cataractous(6).
| Summary|| |
Three cases of bilateral simple ectopia lentis found in one family have been presented here for clinical interest with special features of asymmetric subluxation, cataractous lens and vitreo-lental adhesion and alternate divergent squint among different cases.
| References|| |
Duke-Elder, S., 1977, System of Ophthalmology, Vol. 111, Prat II, 710, 712, 1107, Henry Kimpton, London citing Adam, Dorsch.
Lieberman, T.W., Podos, S.M. and Hartstein, J., 1966, Amer. J. Ophthalmol. 61, 252.
Nirankari, M.S., and Chaddah, M.R., 1967, Amer. J. Ophthalmol., 63:1719.
[Figure - 1]