Marfan's syndrome is another name of arachnodactyly and was firstly noted by Marfan in 1896. Salle found that ocular signs are also accompanied by this syndrome. Since then, many cases of this syndrome have been reported even in this country. The etiology of this syndrome is not exactly classified but is thought as an hereditorical and general developemental abnormalities of mesodermal origin. Authors report here a case of this syndrome in which ectopia lentis, lens opacity, organization of vitreous and iridodonesis are present.
Arachnodactyly ; Cataract ; Ectopia Lentis ; Marfan Syndrome ; Mesoderm

Ectopia lentis, Synonymously with Congenital dislocated lens, is defined as a dislocation of the ocular lens, of greater or less degree, based on a developmental anomaly. It occurs in three conditions, as an isolated anomaly, in association with ocular anomaly, and as a part of a systemic syndrome with other mesodermal, particularly skeletal anomalies. Among these, simple ectopia lentis is a hereditary autosomal dominant disorders without skeletal, connective tissue or metabolic symptomatology. Except for the dislocation of the lens, these eyes are in other respects grossly normal. Regarding its management, there is a continuing debate proponents of active surgical intervention to prevent future problems to those who will not remove the lens and advocates of conservative treatment because lens surgery can be difficult and has a high rate of intraoperative and post operative complication. Recently, the authours have experienced three patients with simple ectopia lentis, which occurred in one family with autosomal dominant inheritance and achieved satisfactory result surgically. Thus these cases are presented with brief review of literatures.
Connective Tissue ; Dislocations ; Ectopia Lentis* ; Humans ; Mesoderm ; Wills

Aniridia is a very rare disorder the main features of which are congenitally partial or nearly complete absence of the iris and foveal hypoplasia. The authors obseved aniridia in two generations, mother and one daughter. They have cataract, ectopia lentis, nystagmus and foveal hypoplasia as well as aniridia. The lens of daughter was aspirated in both eyes consecutively. Her final best corrected visual acuity was 0.3 with +sph. 13.00D in each eyes.
Aniridia* ; Cataract ; Ectopia Lentis ; Family Characteristics ; Humans ; Iris ; Mothers ; Nuclear Family ; Visual Acuity

Aniridia, a condition which shows only an extremely rudimentary iris, is a re latively rare congenital anomaly. It is almost bilateral and occurs as an autosomal dominant trait. The authors experienced one patient who had congenital aniridia combined with cataract, ectopia lentis, lens coloboma, corneal opacity and nystagmus in both eye. Intracapsular cataract extraction was performed with satisfactory result. The authors report this case with the review of literatures.
Aniridia* ; Cataract ; Cataract Extraction ; Coloboma ; Corneal Opacity ; Ectopia Lentis ; Humans ; Iris

PURPOSE: To evaluate the safety and efficacy of transscleral intraocular lens (IOL) fixation while preserving the anterior vitreous face in treating ectopia lentis of Marfan syndrome. METHODS: This study included six patients (12 eyes) who had undergone surgical intervention for ectopia lentis with or without lenticular opacity. We compared the best-corrected visual acuity (BCVA) before and after the surgery and evaluated perioperative complications. RESULTS: The mean age at the time of surgery was 18.2+/-10.7 years. The mean follow-up period was 11.2+/-7.1 months. Mean BCVA scores changed from 0.96+/-0.37 (LogMar Value) to 0.14+/-0.17 (LogMar Value). All patients showed more than two lines of improvement in visual acuity. In two eyes, pupillary capture was found. Medically controllable intraocular pressure elevation was found in three eyes. CONCLUSIONS: This study suggests that transscleral IOL fixation with a preserved anterior vitreous face can be a safe and effective technique in treating the ectopia lentis of Marfan syndrome.
Ectopia Lentis ; Eye ; Follow-Up Studies ; Humans ; Intraocular Pressure ; Lenses, Intraocular ; Marfan Syndrome ; Visual Acuity

PURPOSE: To evaluate the safety and efficacy of transscleral intraocular lens (IOL) fixation while preserving the anterior vitreous face in treating ectopia lentis of Marfan syndrome. METHODS: This study included six patients (12 eyes) who had undergone surgical intervention for ectopia lentis with or without lenticular opacity. We compared the best-corrected visual acuity (BCVA) before and after the surgery and evaluated perioperative complications. RESULTS: The mean age at the time of surgery was 18.2+/-10.7 years. The mean follow-up period was 11.2+/-7.1 months. Mean BCVA scores changed from 0.96+/-0.37 (LogMar Value) to 0.14+/-0.17 (LogMar Value). All patients showed more than two lines of improvement in visual acuity. In two eyes, pupillary capture was found. Medically controllable intraocular pressure elevation was found in three eyes. CONCLUSIONS: This study suggests that transscleral IOL fixation with a preserved anterior vitreous face can be a safe and effective technique in treating the ectopia lentis of Marfan syndrome.
Ectopia Lentis ; Eye ; Follow-Up Studies ; Humans ; Intraocular Pressure ; Lenses, Intraocular ; Marfan Syndrome ; Visual Acuity

To know the causes and visual prognosis of ectopia lentis in children, medical records of 41 patients (82 eyes) were retrospectively analysed.The patients were divided into systemic disease-associated, simple, and familial types according to the causes and directions of lens deviation were compared.Surgical methods, pre-and post-operative corrected vision, and complications were investigated. The mean age at diagnosis was 45/12 years old. The decreased vision (87%) and the presence of family history (7.3%) werethe chief complaints on the first examination. According to the causes, 19patients had Marfan syndrome, 14 simple type, and 8 familial type respectively. The directions of lens deviation in Marfan syndrome were different from in simple and familial types. Surgical management have been per-formed in twenty-six patients (44 eyes) on the average 6years of age, and all but one showed an improvement of postoperative corrected vision. One eye presented a dyscoria as a complication. These results are the first report in Korea about the causes and the visual prognosis of ectopia lentis in children and may give us a reference for further management of these patients.
Child* ; Diagnosis ; Ectopia Lentis* ; Humans ; Korea ; Marfan Syndrome ; Medical Records ; Prognosis ; Retrospective Studies

PURPOSE: We evaluated the clinical results of phacoemulsifacation using microhook iris retractor for the management of ectopia lentis in Marfan syndrome. METHODS: Seventeen eyes of 9 patients with Marfan syndrome were included. All the patients had undergone phacoemulsifacation with microhook iris retractor capsule stabilization between May 1997 and January 2000. RESULTS: Mean patient age was 11.6 +/-8.2 years old. Superior nasal(38%) and superior temporal(38%) were the most common direction of lens dislocation. Fifteen eyes demonstrated improvement in best corrected visual acuity by 2 lines or more following average follow-up period of 22.9 +/-9.2 months. As postoperative complications retinal detachment developed in 3 eyes. CONCLUSIONS: Phacoemulsifacation using microhook iris retractor for capsule stabilization appears to be a good technique for the management of ectopia lentis in Marfan syndrome.
Ectopia Lentis* ; Follow-Up Studies ; Humans ; Iris* ; Lens Subluxation ; Marfan Syndrome* ; Phacoemulsification* ; Postoperative Complications ; Retinal Detachment ; Visual Acuity

Homocystinuria is an inborn error on the pathway of the methionine metabolism. It is mainly caused by a cystathionine B-synthase deficiency in the brain or liver. Homocystinuria is biochemically characterized by: 1) an increase of the homocystine and methionine concentration in the plasma; and 2) a decrease of the cystine with an increased excretion of homocystine in the urine. The clinical manifestations of this autosomal recessive disorder include: ectopoia lentis, skeletal abnormalities, high incidence of thromboembolism and high frequency of mental retardation. We have been experiencing a case of a 10 year old female patient who has suffered from homocystinuria. She has undergone mental retardation, poor vision caused by ocular complications and Marfanoid feautures.
Brain ; Child ; Cystathionine ; Cystine ; Ectopia Lentis* ; Female ; Homocystine ; Homocystinuria* ; Humans ; Incidence ; Intellectual Disability ; Liver ; Metabolism ; Methionine ; Plasma ; Thromboembolism

PURPOSE: To analyze the clinical features and the long term visual results of children with ectopia lentis after lensectomy. METHODS: Enrolled in this study were 79 eyes of 43 patients who were operated on and followed up for at least 3 years. We performed a retrospective analysis of the patient records. RESULTS: The mean age at diagnosis was 4.6 years, mean age at surgery was 5.7 years and average follow-up was 7.1 years. Of the 43 patients with bilateral ectopia lentis, 7 underwent monocular surgery. The mean age at diagnosis was higher and mean postoperative BCVA was significantly lower in the monocular surgery group than in the binocular surgery group. All eyes showed reduced hyperopia for 5 years postoperatively, but the reduction rate of the monocular operated eyes was significantly lower than that of the binocular operated eyes (P<0.05). No complication occurred during surgery and 1 eye (1.3%) with Marfan syndrome showed retinal detachment 9 years after surgery. CONCLUSIONS: Surgical treatment of ectopia lentis was safe and showed good visual outcomes after 7.1 years of follow-up. Nevertheless, consideration must be given to the possibility of amblyopia due to the difference of the degree of dislocation between the two eyes.
Amblyopia ; Child* ; Diagnosis ; Dislocations ; Ectopia Lentis* ; Follow-Up Studies ; Humans ; Hyperopia ; Marfan Syndrome ; Retinal Detachment ; Retrospective Studies ; Telescopes