Ectopia lentis – eyewiki z gas tecate telefono


Ectopia lentis can occur due to trauma, ocular disease, or systemic disease. Trauma is the most common cause. It may be present at birth or occur at any age. Traumatic ectopia lentis is most often the result of a direct blow to the eye, such as from a baseball or golf ball, but can also occur after blunt trauma to the head or orbit [2]. Though b games unblocked trauma is the most common cause of ectopia lentis, if the trauma is minor, one should consider other underlying diseases that could predispose the patient to lens dislocation. [3]

Simple ectopia lentis – simple ectopia lentis can occur as a congenital disorder or as a spontaneous disorder later in life. Both are hereditary and can be inherited in an autosomal dominant or recessive pattern. Mutations resulting in simple ectopia lentis include mutations to the ADAMTSL4 gene located on chromosome 1 (recessive inheritance pattern) and the FBN1 gene located on chromosome 15 (dominant inheritance pattern) [4]. These mutations often result in irregularity and degeneration of the zonular fibers, leading to lens dislocation. Significant overlap exists between the genetics of simple ectopia lentis and the systemic diseases associated with ectopia lentis (e.g. mutations to the FBN1 gene can also result in Marfan syndrome) [5]. In simple ectopia lentis, the lens dislocation is usually electricity generation bilateral and occurs most often in the superotemporal direction [6].

Ectopia lentis et pupillae – ectopia lentis et pupillae is a rare congenital disorder in which there is asymmetric eccentric pupils that are displaced in the opposite direction of a lens dislocation. The pupils are characteristically oval and ectopic, and often dilate poorly [7]. The irides often appear atrophic with marked transillumination of the periphery on slit lamp examination [8]. The lens dislocation is usually bilateral and commonly not symmetrical [6].

Marfan syndrome – Marfan syndrome is the most common cause of heritable ectopia lentis, and ectopia lentis is the most frequent ocular manifestation of Marfan syndrome, occurring in approximately 75% of patients [9]. Marfan syndrome is an autosomal dominant disease resulting from various mutations to the fibrillin-1 gene located on chromosome 15. It is thought gas key bolt carrier that the increased incidence of ectopia lentis with Marfan syndrome is due to altered fibrillin microfibrils leading to incompetent zonular fibers and structural abnormalities of the lens capsule [10]. Lens dislocation in Marfan syndrome is usually bilateral and occurs most often in the superotemporal direction, though other directions are not uncommon [11].

Homocystinuria – Homocystinuria is the second most common cause of heritable ectopia lentis. Homocystinuria is an autosomal recessive metabolic disorder most often caused by a near absence of cystathionine b-synthetase. It is associated with intellectual disability, osteoporosis, chest deformities, and increased risk of thrombotic episodes. Lens dislocation occurs in 90% of patients, and is thought to be due to decreased zonular integrity due to the enzymatic defect. Lens dislocation in homocystinuria is usually bilateral and in 60% of cases occurs in the inferior or nasal direction. [12] [13]

A history of any recent trauma is of principal importance. Traumatic ectopia lentis generally presents with a red, painful eye secondary to trauma. A detailed family history is required for atraumatic and mildly traumatic presentations. One should specifically ask about visual disturbances, cardiovascular disease, or skeletal abnormalities in the patient or patient’s family.

The most significant symptom of ectopia lentis is reduced visual acuity. Common visual disturbances include poor near vision due to loss of accommodation, poor distant vision due to astigmatism or myopia, and monocular diplopia. The degree of visual acuity reduction will vary with the degree of dislocation, the type of dislocation, and any other concurrent ocular defects. Minimal subluxation may lead to little or no loss of visual r gas constant kj acuity, while disruption of the zonules, leading to increased curvature of the lens, may result in lenticular myopia and astigmatism [6].

The ocular examination for ectopia lentis should consist of checking visual acuity, an external ocular exam, a slit lamp exam, retinoscopy and refraction, and a dilated fundus examination. Corneal diameter should be measured, as megalocornea is associated with Marfan syndrome. If astigmatism is present, keratometry may help determine whether the astigmatism is primarily corneal or if it is the result of altered lens location. Intraocular pressure may be elevated due to pupillary block or posttraumatic angle recession. Dilated fundus examination is necessary as retinal detachment is a possible consequence of ectopia lentis [6].

Medical management involves refractive correction as well as co-management of any associated systemic disease with the patient’s internist or pediatrician. As many predisposing diseases are hereditary, genetic counseling may be indicated. Treatment of ectopia lentis itself depends on where and how far the lens moves, as well as any resulting complications. In all cases eur j gastroenterology hepatology impact factor, timely optical correction is important for prevention of amblyopia. If the dislocated lens migrates into the anterior chamber, treatment is initially pharmacological with mydriasis to permit posterior migration of the lens behind the iris. Pupillary block glaucoma should be treated with laser peripheral iridotomy, and elevations in intraocular pressure should be treated medically.

Progressive lens dislocation without major complications has been traditionally managed conservatively; however, some studies suggest that surgical intervention could lead to improved visual acuity [6]. The decision to operate depends on many factors, including visual acuity, lens location, progressive subluxation of the lens, imminent total dislocation, and others.

Save That Kid’s Bag: Treatment of Subluxated Crystalline Lens This award-winning video by Mauricio A. Perez, MD, and Michael E. Snyder, MD, demonstrates treatment of a subluxated crystalline lens in a child. The authors are able to spare the capsular bag using a sceral-fixated modified capsular tension ring. The technique effectively restores visual acuity while electricity gif preserving the posterior segment and avoiding the complications and nuisances of aphakia. They have used the technique primarily in eyes with Marfan’s syndrome and primary ectopia lentis. A 44-year-old woman presented with downward lens dislocation, crowding of the anterior chamber and mydriasis on her left eye. The right eye showed ectopia lentis with gas vs electric oven for baking cakes superior displacement of the lens. The patient had not been diagnosed with Marfan syndrome. To avoid a lens luxation into the anterior or the vitreous chamber, a intracapsular crystalline lens extraction was performed. The lens had a wide coloboma without any zonular support. After the lens extraction, an iris-fixated IOL was implanted.

An individual’s prognosis depends on any underlying ocular or systemic disease, the degree of subluxation, and the management strategy pursued. Large prospective randomized clinical trials are needed to determine the long-term visual outcomes in specific patient populations affected by ectopia lentis. Fortunately, the majority of patients with ectopia lentis achieve a best-corrected visual acuity of 20/40 or better [18] [19] Additional Resources