scholarly journals Spontanious luxation of the lens in Marfan syndrome

Author(s):  
Benchakroun S ◽  
◽  
Taouri N ◽  
Azarkan B ◽  
Tagmouti A ◽  
...  

We report the case of a 22-year-old-man, who presented to the ophthalmic emergencies for decreased visual acuity since 2 days. The clinical examination found visual acuity reduced to counting fingers in the right eye and 3/10 in the left eye. and an anterior lens dislocation in the right eye, while the examination of the left eye after pupillary dilation, revealed lens dislocation in the superonasal direction and absence of zonules from 2 to 5 o’ clock position. The posterior segment examination was normal in both eyes. Otherwise, on general examination we noticed Marfan syndrome characteristic. In our case of the retained diagnosis was Marfan syndrome. The first clinical description was in 1896 by Antoine-Bernard-Jean Marfan [1], which is a rare genetic disease, that touch the conjunctive tissue of many organs, as well the eye that can present many abnormalities as: Ectopia lentis in 50-80%, which is commonly bilateral and many other manifestations also can be seen [2,3].

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Madbouhi K ◽  
◽  
Cherkaoui O ◽  

We report the case of an 18-year-old patient who consults for a loss of visual acuity in the right eye for 1 year. On clinical examination, visual acuity is 20/100. Eye tone is 12 mmhg. Examination of the anterior segment shows the existence of a cataract made up of whitish opacities corresponding to a congenital cerulean cataract (Figure 1). The treatment consisted of a cataract cure by phacoemulsification with good progress. Cerulean cataract is a rare congenital form of bluish tint, made up of whitish opacities in concentric layers with a radial arrangement at their center. Visual acuity is fairly good in childhood but may deteriorate later. In the literature, the incidence of cataracts in Down’s children ranges from 5% to 50%. In previous studies of congenital or infantile cataract, 3-5 % of cases were associated with Down’s syndrome [1].


2015 ◽  
Vol 96 (6) ◽  
pp. 1074-1078
Author(s):  
E E Grishina ◽  
A A Ryabtseva ◽  
T V Belova ◽  
O M Andryukhina

There is a number of literature data on ischemic optic neuropathy development in acute hemorrhage. However, ocular disorders in prolonged chronic hemorrhage and iron-deficiency anemia are not well studied. We present a clinical case of optic nerve and retinal damage in a patient with prolonged chronic gastrointestinal bleeding. 53-year-old patient S. presented with complaints on dramatic sudden loss of vision of his right eye (visual acuity was 0/02 and was not improving with correction). Visual acuity of the left eye was good. Ophthalmoscopy revealed right optic nerve swelling, flame-shaped disc and peripapillary hemorrhages, and multiple soft exudates along blood vessels of the right eye. Optic nerve head of the left eye was pale pink, with well-defined borders. Multiple soft exudates along blood vessels and few flame-shaped hemorrhages were identified as well. Clinical examination revealed iron-deficiency post-hemorrhagic anemia. The diagnosis of anterior ischemic neuropathy of the right eye, ischemic neuroretinopathy of left eye associated with post-hemorrhagic anemia was established. Conservative treatment increased hemoglobin level up to 82 g/l, the red blood cells count - up to 2,88×1012/L, hematocrit was 0.25%, platelet count reached 344×109/L, but the signs of rectal bleeding remained. The patient underwent surgery for hemorrhoids. After the increase of hemoglobin level, visual acuity of the right eye improved to 0.1, the visual acuity of the left eye was 1.0. According to the results of computed peripheral vision test (Humphrey Full Field 120 Point Screening Test), central scotoma and scotomas in the lower half of the field of vision of the right eye remained. In the field of vision of the left eye, the area of absolute arcuate scotoma in the lower-nasal quadrant decreased significantly. Reduced visual acuity was the main complaint of the patient with a longstanding gastrointestinal bleeding. A careful history and thorough clinical examination allowed to establish the cause of the optic nerve and retinal damage, to assign pathogenetically based treatment, which led to an improvement in visual function.


2013 ◽  
Vol 141 (7-8) ◽  
pp. 516-518
Author(s):  
Aleksandar Gakovic ◽  
Igor Kovacevic ◽  
Jovana Bisevac ◽  
Bojana Radovic ◽  
Katarina Cubrilo ◽  
...  

Introduction. Penetrated injuries are most difficult injuries of the eye. Intraocular foreign body (IOFB) may lodge in any of the structures it encounters, from anterior chamber to the retina and choroid. Notable effects caused by foreign body injury include traumatic cataract, vitreous liquefaction, retinal and subretinal hemorrhages, retinal detachment and development of endophtalmitis. Case Outline. A 49?year?old man sustained injury of the right eye with a piece of metal wire. On admission visual acuity was VOD: 1.0 and lower intraocular tension TOD=6 mmHg (10?22 mmHg). Corneal entry wound was noticed near limb on 11h with a prominating foreign body of 18 mm in length that passed through the iris, lens and vitreous. X?ray findings confirmed existence of a large foreign body extending along the entire length of the globe. IOFB removal was done with anatomic forceps. On postoperative detailed clinical examination we observed retinal rupture in the upper temporal quadrant fitting in the area of the IOFB damaged retina. Laser photocoagulation of retinal tear (laser retinal barrage) was done. Visual acuity on discharge was the same (1.0) and intraocular tension was within normal limits (10 mmHg). Conclusion. Penetrated injury of eye requires detailed examination of all eye structures, beginning from the anterior to posterior segment. Timely diagnosed ruptures of the posterior segment of eye before the development of traumatic cataract, and adequate therapeutic procedures prevent serious complications of IOFB penetrated eye injury such as retinal detachment and permanent reduction of visual acuity.


Author(s):  
Widyandana Doni ◽  
Angela Nurini Agni ◽  
Agus Supartoto

Background: High prevalence of eye disorders in Indonesia requires medical doctors to be skillful and well trained in ophthalmologic examination. Undergraduate medical students usually start their clinical simulation practice and ophthalmology assessment in a safe learning environment. Skill laboratory as the learning facility should be evaluated and improved regularly. This study aimed to evaluate student’s pitfalls in the eyes OSCE station. Methods: Descriptive analytic study involving Objective Structured Clinical Examination (OSCE) eye examination station score from 1st - 4th year undergraduate medical students batch 2010 in Faculty of Medicine, Universitas Gadjah Mada, Indonesia (n=516). All checklists’ scores were analyzed based on particular sub-scales in every examination topic to explore the most pitfalls made by students in eye examination station during OSCE. Results: The order average value of each subscale in OSCE are: Doctor patient interaction (88.42), History taking skills (82.44), Professionalism (76.43), Physical Examination (74.62), Diagnosis (60.68), Management of Pharmacotherapy (54.70). The percentage of failed-students (scores <70) in 1st-4th year OSCE based on topics skills were Year 1: Visual field (5.08%), Visual acuity (14.21%), Anterior Segment (2.54%). 2nd year: IOP by palpation (24.38%), Visual acuity (9.38%), Anterior Segment (29.38%). Year 3:  visual field (4.94%), IOP by palpation (2.47%), Visual acuity (12.35%), Anterior Segment (7.41%), Posterior Segment (22.22%). Year 4: Comprehensive eye exam (17.95%). Conclusions: Students mostly challenged in the skills of diagnosis establishment and pharmacological management. The highest number of failed students in each year OSCE vary from each year. 1st year failed most at visual acuity examination, 2nd year was anterior segment examination and 3rd year was posterior segment examination. Those three skills need to be enhanced systematically. Keywords: OSCE, pitfall pattern, eye examination, undergraduate students, skill laboratory 


Author(s):  
Benchakroun S ◽  
◽  
Taouri N ◽  
Tagmouti A ◽  
Cherkaoui LO ◽  
...  

We report a case of a 42-year-old-woman, who presented to the ophthalmic consultation for decreased visual acuity complaints of blurred vision, altered pupillary shape since few months of her right eye. The clinical examination found a reduced visual acuity to counting fingers in the right eye and 20/20 in the left eye. intraocular pressures was 38 mm Hg OD and 14 mm Hg OS. Slit lamp examination of the right eye found: Corneal edema, iris atrophy with a deformation of the iris architecture and pupillary anomalies, with polycoria (Figure 1). The evaluation of the angle by gonioscopy found areas of broad synechiae anterior to Schwalbe’s line (Figure 2). While the examination of the left eye was normal (Figure 1B). The posterior segment examination was normal in both eyes. Specular microscopy confirmed the presence of unilateral endothelial pleomorphism and polymegathism. In our case of the retained diagnosis was iridocorneal endothelial syndrome


2020 ◽  
Vol 2 (3) ◽  
pp. 244-250
Author(s):  
Nurulhuda Md Amin ◽  
Safiyah Jameelah Mohd Yusof ◽  
Nor Fadzillah Abd Jalil ◽  
Raja Norliza Raja Omar ◽  
Mushawiahti Mustapha

Ectopia lentis or crystalline lens subluxation is one of the major criteria to diagnose Marfan syndrome. It may vary from mild lens subluxation to lens dislocation. Herewith is a case report of a 4-year-old autistic boy who had never been diagnosed with Marfan syndrome. He presented to the clinic after his parents noticed he had difficulty focusing on near objects. His bilateral best-corrected visual acuity was 6/60. On examination, there was bilateral lens subluxation superotemporally and lens equator blocking his visual axis. He was sent to the paediatric team and further Marfan workout showed dilated aortic root. He was then diagnosed with Marfan syndrome. He underwent bilateral lens aspiration, anterior vitrectomy, and iris-claw lens implantation. His postoperative bilateral visual acuity on day 1 was 6/30 and his best-corrected visual acuity 3 months after surgery was 6/9 for both eyes. In conclusion, ophthalmologists play an important role in diagnosing and managing Marfan syndrome. Early diagnosis is important to help preserve vision and improve quality of life.


Author(s):  
Nafila Mahida Sukmono ◽  
Ramzi Amin

Introduction Retinal vein occlusion is the largest group of retinal blood vessels after diabetic retinopathy. Occlusion occurring in the retinal vein is divided into central retinal vein occlusion (CRVO) occlusion and branch retinal vein occlusion (BRVO) occlusion. The Beijing Eye Study, reported a higher incidence of BRVO than CRVO, where 10-year incidents for BRVO were 1.6 per 100 subjects, and CRVO was only 0.3% 100 subjects.1 To report a case of Branch Retinal Vein Occlusion with vitreous hemorrhage identified during intraoperative vitrectomy Method: A 49-year-old woman with a history of 15 years of hypertension had right eye vision complaints, increasingly blurred since last 2 months. The right eye visual acuity 2/60 cannot be corrected and left eye 6/30 cannot be corrected. The posterior segment on right eye is difficult to assess. USG B-Scan right eye found vitreous echospike appearance of vitreous bleeding. We manage with vitrectomy and during intraoperative we identified bleeding and ghost vessel in superotemporal area. Bleeding in the superotemporal quadrant is done by photocoagulation laser action. Results: First day postoperative there was increased in visual acuity to 6/60 with a posterior segment that could be assessed, obtained tortous blood vessels, slight bleeding and ghost vessel in the superotemporal area with laser injury. Conclusion: In this case report, patients with BRVO with complications of vitreous hemorrhage performed vitrectomy with additional endolaser in the ischemic area. The result of this action of visual acuity improvement in patient.


2022 ◽  
Vol 13 (2) ◽  
pp. 19-24
Author(s):  
Daniel Prado Beraldo ◽  
Fernando Buzatto Mantovan ◽  
Emanuele Moraes Mello

Marfan syndrome (MFS) is a rare autosomal dominant metabolic disorder, caused by mutations in the gene responsible for coding the fibrillin-1 protein. It is characterized by cardiovascular, musculoskeletal and ocular manifestations. The purpose of this report was to present a rare ophthalmologic complication in a patient with MFS. Female, 51 years old, attended the emergency room complaining of severe pain, low visual acuity and hyperemia in the right eye, started two days before. Biomicroscopy: ectopia lentis (luxated) for anterior chamber; Tonometry: 56 mmHg. Hypotensive drugs were prescribed which reduced intraocular pressure (IOP) to 8 mmHg. Referred for emergency surgery (facectomy without IOL implantation) in order to avoid further damage to the cornea and optic nerve. In the post operative period, presented IOP control and improved VA to 20/50. Multidisciplinary follow-up is necessary in patients with MFS, enabling early diagnosis, which can prevent and minimize any complications.


2018 ◽  
Vol 29 (1) ◽  
pp. 38-43 ◽  
Author(s):  
Daniel J Salchow ◽  
Petra Gehle

Purpose: To study ocular manifestations of Marfan syndrome in children and adolescents. Methods: Retrospective comparative cohort study on consecutive patients up to age 17 years, presenting to the interdisciplinary Marfan clinic of Charité-University Medicine Berlin over a period of 4 years. Results: A total of 52 Marfan syndrome patients and 73 controls were enrolled. In Marfan syndrome eyes, the cornea was flatter (mean keratometry, 40.86 ± 2.13 vs 42.55 ± 1.55 diopters in control eyes, p < .001) and corneal astigmatism was greater (1.50 ± 1.22 vs 0.88 ± 0.49 diopters in control eyes, p < .001). The central cornea was thinner in Marfan syndrome eyes (537.35 ± 40.64 vs 552.95 ± 38.57 μm, p = 0.007) and Marfan syndrome eyes were more myopic than control eyes (spherical equivalent, –2.77 ± 4.77 vs −0.64 ± 1.92 diopters, p < .001). Visual acuity was reduced (logMAR 0.11 ± 0.17 vs 0.04 ± 0.26, p = 0.014) and intraocular pressure was lower in Marfan syndrome eyes. Iris transillumination defects were more common in Marfan syndrome eyes (19.6% vs 4.3% in control eyes, odds ratio for Marfan syndrome in the presence of iris transillumination defects = 7.2). Ectopia lentis was only found in Marfan syndrome eyes (25 Marfan syndrome patients, 49% with available data, bilateral in 68%). Conclusion: Iris transillumination defects and ectopia lentis are characteristic ocular findings in children and adolescents with Marfan syndrome. Myopia is more common and corneal curvature, central corneal thickness, and visual acuity are reduced in Marfan syndrome eyes. Children with Marfan syndrome need regular comprehensive eye examinations to identify potential complications.


2021 ◽  
pp. 208-213
Author(s):  
Bhupesh Singh ◽  
Sourabh Sharma ◽  
Suchit Dadia ◽  
Neha Bharti ◽  
Sudhank Bharti

Marfan syndrome (MFS) is known to cause significant refractive error. Treatment options are limited in this condition for correcting refractive error. Clear lens exchange is done in these cases, but complication rates are high. Loss of accommodation is another concern in these young adults. We report toric phakic intraocular lens (pIOL) implantation in improving the uncorrected visual acuity (UCVA) in a known case of MFS with lens coloboma. A 22-year-old female patient with MFS with inferior lens coloboma underwent bilateral toric pIOL implantation in the same sitting. Pre- and post-operative UCVA and best-corrected visual acuity were assessed. Central and peripheral vaulting of the pIOL in relation to the natural lens was also assessed. UCVA improved from 20/500 to 20/20 in the right and 20/550–20/20 in the left eye. Marked central vaulting with partial peripheral vaulting was achieved. There were no post-operative complications. Phakic IOL implantation surgery could be an effective approach to achieve excellent uncorrected refractive outcome in patients with MFS to treat high myopia.


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