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International Ophthalmology (The International Journal of Clinical. Ophthalmology and Visual Sciences). Medical Treatment of Crystalline Lens Dislocation into ...
International Ophthalmology (The International Journal of Clinical Ophthalmology and Visual Sciences) Medical Treatment of Crystalline Lens Dislocation into the Anterior Chamber in a Patient with Marfan Syndrome --Manuscript Draft-Manuscript Number: Full Title:

Medical Treatment of Crystalline Lens Dislocation into the Anterior Chamber in a Patient with Marfan Syndrome

Article Type:

Case Report/Small Series/Short Article

Keywords:

Marfan syndrome; anterior chamber; lens dislocation; medical treatment,

Corresponding Author:

Manuel Garza-Leon, MD Instituto para Preservación de la Visión and Destellos de Luz non-profit foundation Monterrey, Nuevo Leon MEXICO

Corresponding Author Secondary Information: Corresponding Author's Institution:

Instituto para Preservación de la Visión and Destellos de Luz non-profit foundation

Corresponding Author's Secondary Institution: First Author:

Manuel Garza-Leon, MD

First Author Secondary Information: All Authors:

Manuel Garza-Leon, MD

All Authors Secondary Information: Suggested Reviewers:

Byung Joo Song [email protected] He reported a surgical management of a of crystalline lens dislocation into the anterior chamber with corneal touch and secondary glaucoma Mauro Waiswol [email protected] He is a cataracts specialist with interest in ectopia lentis and reported a Lens subluxation grading system Abhay R Vasavada [email protected] He reported the Management of bilateral anterior dislocation of a lens in a child with Marfan’s syndrome. Minas T Coroneo [email protected] Ophthalmologist interested in Marfan syndrome

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Medical Treatment of Crystalline Lens Dislocation into the Anterior Chamber in a Patient with Marfan Syndrome.

Manuel Garza Leon MD 1

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Instituto para Preservación de la Visión and Destellos de Luz non-profit foundation.

Corresponding Author: Manuel Garza-León MD. Instituto para Preservación de la Visión. Ruperto Martínez # 1317 Pte. Colonia Centro, Monterrey N.L, México. Tel & fax 52+ (81) 8882-4208. email: [email protected]

The author has no financial or proprietary interest in any product discussed in the article and has full control of all primary data.

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Abstract

We report the case of a 32-year-old male patient with spontaneous crystalline lens dislocation into the anterior chamber with corneal touch and increased intraocular pressure. The case was handled in a conservative way: before bringing the patient to supine position, pharmacological pupil dilation with Tropicamide-Phenylephrine was performed (T-P Ofteno, Laboratorios Sophia, Guadalajara, Mexico). One drop every 15 minutes for one hour. Once the posterior displacement of the lens behind the iris was confirmed, the pharmacological dilation was reverted by applying 2% Pilocarpine (Pil Ofteno, Laboratorios Sophia, Guadalajara, Mexico). After confirming the lens luxation into the vitreous cavity was treated with 2% Pilocarpine and 5% sodium chloride solution (Hiperton al 5%, Laboratorios Grin, México D.F, México), every 6 hours.

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Introduction Marfan syndrome is a connective tissue disorder inherited on a dominant autosomal basis, with an incidence of 1:10,000 individuals. The disease is caused by an alteration on fibrillin-1 production. Fibrillin -1 is a 350-kDa acidic glycoprotein whose production is regulated by the fibrillin-1 (FB1) gene located in the 15q15-21 chromosome. [1,2]

This disorder affects the skeletal, pulmonary, ocular and cardiovascular systems; the skin, the intergumentary system and the dura can also be affected. According to Ghent’s classification, the only ophthalmologic major criterion is ectopia lentis. In addition, there are three minor ophthalmologic criteria: abnormally flat cornea, increased axial length of the ocular globe, and iris hypoplasia or ciliary muscle dysfunction.[3]

Ectopia lentis is the predominant ocular abnormality and occurs in 50 to 80% of patients [4]. Other ocular manifestations include axial or lenticular myopia, detached retina, pre-senile cataracts, glaucoma, strabismus, iris abnormalities and flat cornea. [4,6]

Lens luxation in to the vitreous cavity is not frequent [5], and luxation into the anterior chamber is still rarer, with severe complications such as corneal endothelium damage, reverse pupillary block, and acute glaucoma.

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Case Report We present the case of a 32-year-old male patient with confirmed Marfan syndrome diagnosis, who has been using contact lenses for 2 years because a bilateral dislocation of the crystalline lens into the vitreous cavity. The patient presented with a history 10-hour of ocular pain and blurred vision on the left eye. An ophthalmological examination revealed visual acuity of 20/60 with contact lens on the right eye, and CF at 10 feet on the left eye. Intraocular pressure was 13 mmHg on the right eye and 28 mmHg on the left. Ciliary injection and mild central corneal edema were found on the left eye, and a secondary aphakia due to a lens luxation into the vitreous cavity on the right eye. On the left eye, the crystalline lens was luxated into the anterior chamber with corneal touch at the center. The iris was areflectic with a frontal displacement in the temporal section that produced an angular closure from the I to the VII meridian, the nasal section had a Van Herrick's grade 4 anterior chamber angle without crystalline-cornea touch (figure 1). The posterior segment of both eyes had normal characteristics. Given that it was a weekend, and we had no access to OR, we decided to undertake a conservative approach: we put the patient in supine position and proceeded to pharmacologically dilate the pupil with Triptocamide-Phenilephrine drops (T-P Ofteno, Laboratorios Sophia, Guadalajara, Mexico) one (1) drop every 15 minutes for one hour. Once the posterior displacement of the lens behind the iris was confirmed (figure 2), the dilation was pharmacologically reversed by applying 2% Pilocarpine (Pil Ofteno, Laboratorios Sophia, Guadalajara, Mexico), one drop every 15 minutes during 45 minutes. At the end there was 3mm pupil dilation, moderate corneal edema in the central sector and presence of the lens in the vitreous cavity beneath the pupil’s inferior border (figure 3).

After confirming the lens luxation into the vitreous cavity was confirmed, treatment with 2% Pilocarpine and 5% sodium chloride solution (Hiperton al 5%, Laboratorios Grin, México D.F, México), every 6 hours was prescribed.

One week later, the corneal edema had disappeared, the intraocular pressure was 12 mmHg, the iris was hypo reflective and the crystalline lens was in the vitreous cavity (figure 4). A non-contact specular

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microscopy was performed (Topcon SP-2000P Specular microscope Topcon Corp. Tokio, Japan), finding 1920 cel/mm3 on the right eye and 1345 cel/mm3 on the left eye.

During the 2-month follow-up, there was no new crystalline lens luxation, and the patient’s cornea remains transparent and without edema.

Discussion There are many causes for anterior crystalline lens dislocation: trauma, inherited disorders, including Marfan Syndrome, homocystinuria, spherophakia, retinitis pigmentosa, pseudoexfoliation syndrome, as well as spontaneous cases.7,8 Among reported complications, we have intraocular pressure increase due to reverse pupillary block, endothelial damage and uveitis.[8-12]

The initial medical treatment’s objective is to reduce intraocular pressure; this can be supported with laser peripheral iridiotomy whenever possible, with the purpose of breaking the pupillary block [8,11]. However, the definitive treatment is generally surgical; Peyman and collaborators [13] suggest a standard vitrectomy and the removed of the crystalline-lens with the vitrophage; more recently, Choi and coauthors10 and Seng and collaborators [14] report an approach with a closed-chamber technique in clear cornea, anterior dry vitrectomy assisted with viscoelastic materials and intracapsular phacoemulsification. And, as Kuan and associates [8], they add lens implantation with scleral fixation. Due to frequently associated high intraocular pressure, surgical treatment shows higher risk of expulsive hemorrhage and endothelial cell loss [10, 14], and reverse pupillary block may develop if miotics are used to keep the crystalline lens in the anterior chamber [9]. We report a conservative approach that reduces the risk of “hot-eye surgery” and allows the physicians to decide which is the best option to correct the aphakia and preserve endothelial cells. In conclusion, the medical procedure described for the treatment of crystalline lens luxation into the anterior chamber has low risk and can be successful.

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References 1. Robinson PN, Arteaga-Solis E, Baldock C, Collod-Béroud G, Booms P, De Paepe A, Dietz HC, Guo G, Handford PA, Judge DP, Kielty CM, Loeys B, Milewicz DM, Ney A, Ramirez F, Reinhardt DP, Tiedemann K, Whiteman P, Godfrey M. The molecular genetics of Marfan syndrome and related disorders. J Med Genet.2006;43:769-787. 2. Sakai LY, Keene DR, Engvall E. Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils. J Cell Biol.1986;103:2499-2509. 3. De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet. 1996;62:417-426. 4. Maumenee IH. The eye in the Marfan syndrome. Trans Am Ophthalmol Soc. 1981;79:684-733. 5. Izquierdo NJ, Traboulsi EI, Enger C, Maumenee IH. Glaucoma in the Marfan syndrome. Trans Am Ophthalmol Soc. 1992;90:111-117. 6. Izquierdo NJ, Traboulsi EI, Enger C, Maumenee IH. Strabismus in the Marfan syndrome. Am J Ophthalmol. 1994;117:632-635. 7. Jarett WH. Dislocation of the lens. A study of 166 hospitalized cases. Arch Ophthalmol 1967;78(3): 289-96. 8. Kwon YA, Bae SH, Sohn YH. Bilateral spontaneous anterior lens dislocation in a retinitis pigmentosa patient. Korean J Ophthalmol. 2007;21:124-6. 9. Jovanoviƒá M, Stefanoviƒá I. Spontaneous dislocation of a transparent lens to the anterior chamber--a case report. Srp Arh Celok Lek. 2010;138(7-8):486-8 10. Choi DY, Kim JG, Song BJ. Surgical management of crystalline lens dislocation into the anterior chamber with corneal touch and secondary glaucoma. J Cataract Refract Surg. 2004;30(3):718-21. 11. Kawashima M, Kawakita T, Shimazaki J. Complete spontaneous crystalline lens dislocation into the anterior chamber with severe corneal endothelial cell loss. Cornea. 2007;26(4):487-9. 12. Vasavada AR, Praveen MR, Desai C. Management of bilateral anterior dislocation of a lens in a child with Marfan's syndrome. J Cataract Refract Surg. 2003;29(3):609-13 13. Peyman GA, Raichand M, Goldberg MF, Ritacca D. Management of subluxated and dislocated lenses with the vitrophage. Br J Ophthalmol 1979; 63:771–778. 14. Seong M, Kim MJ, Tchah H. Argon laser iridotomy as a possible cause of anterior dislocation of a crystalline lens. J Cataract Refract Surg. 2009;35(1):190-2.

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Figure 1. Clinical Picture of left eye (A) with the crystalline lens was luxated into the anterior chamber with central corneal touch and (B) evidence of corneal edema.

Figure 2. Clinical Picture showing the posterior displacement of the lens behind the iris after pharmacological pupil dilatation.

Figure 3. Clinical Picture After the dilation was pharmacologically reversed by applying 2% Pilocarpine there was 3mm pupil dilation, moderate corneal edema in the central sector.

Figure 4. Clinical Picture one week later, the corneal edema had disappeared and the crystalline lens was in the vitreous cavity.

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