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Glaucoma

In glaucoma the drainage of aqueous humor (the liquid in the front part of the eye) is inefficient, or the eye produces fluid too quickly to drain effectively.  This causes pressure on many structures of the eye, including the optic nerve.  The damaged optic nerve can result in blindness if not treated.  The most common type of glaucoma is called “primary open angle glaucoma” or “POAG”.  In cases of POAG, the drainage canal for ocular fluid appears to be open.

 A highly nearsighted individual has a greater risk association with POAG, and nearsightedness is more common with EDS. The elongated eyeball, characteristic of nearsightedness, allows a larger optic channel with the optic nerve fiber becoming more susceptible to pressure and injury.19

Glaucoma can be congenital, for example, when the ducts responsible for fluid drainage fail to form completely. Some infants are born with defects in the angle of the eye that slow the normal drainage of aqueous humor, a condition most often correctable with surgery if discovered early enough.  This is often seen in Type VI EDS, in conjunction with an abnormally small cornea (“microcornea”) and the thin, blue sclera.

Individuals who have either Ehlers-Danlos syndrome or Marfan's syndrome, a condition characterized by elongation of the bones, appear to have a higher association with glaucoma.

Treatment for glaucoma (POAG) begins with eye drops and/or pills to lower the pressure.  If the glaucoma is due to a defect in the drainage canal, argon laser surgery is usually indicated to open a few areas for the fluid to drain.  As in any surgical treatment for EDS patients, special care is taken to traumatize the eye as little as possible.20

Symptoms of POAG don’t appear until it may be too late to save the vision in that eye.  Annual eye exams including routine checks of intraocular pressure (with pachymetry or Orbscan for accuracy of diagnosis) and early treatment when warranted are the best ways to thwart glaucoma and its damaging ocular effects.21

Strabismus

Strabismus (crossed eyes or eye drifting outwards, upwards, or downwards) can also be found in EDS patients and non-EDS patients.

Strabismus occurs when the six extra-ocular muscles that control eye movement are not in balance.  Not dissimilar to the loose joints in the EDS patient, one or more of the extra-ocular muscles is looser than the others, resulting in the eye drifting or crossing.

 Extra effort may be needed to keep proper alignment of the eyes, causing eye fatigue.  Multifocal lenses (bifocals or trifocals) can help to balance the muscle activity associated with changing focus from faraway to close up and back to distance.  Prism in prescription glasses can be helpful in directing light to the correct spot on the retina, so that the eyes do not need to work so hard to do so.  Surgical repair of a strabismus may be complicated because sutures may be difficult to place in thinned sclera of EDS, especially in Type VI.  As in any muscle or ligament surgery on the EDS patient, some surgical results may not have lasting effects.22

Macular Degeneration

The macula is the part of the retina that is used for central vision.  In macular degeneration, loss of proper functioning of the macula results in blindness of the central vision (peripheral vision is usually left intact).  It is the leading cause of blindness in those Americans over the age of 55 years, and it affects over 10 million Americans.

The cause of macular degeneration is not yet fully understood, but it does appear that EDS patients are more prone to developing this condition.  Macular degeneration can be divided into two types – atrophic (or the “dry” form) and the more damaging “exudative” (or “wet” form).  Because the macula is physically supported by the collagen of the eye and receives nutrients through the network of blood vessels in the area, it is easy to hypothesize how a collagen and/or vessel abnormality could contribute to macular degeneration.  More research will need to be done, however, to effectively treat or prevent this condition

A major National Eye Institute study, Age Related Eye Disease Study (AREDS), has produced strong evidence that certain nutrients such as beta carotene (vitamin A) and vitamins C and E in conjunction with zinc and Omega – 3 fatty acids may help prevent or slow progression of dry macular degeneration.23

Until recently the only available treatment to seal leaking vessels in the exudative form of macular degeneration was with laser photocoagulation.24  This was followed by Photodynamic Therapy (PDT) with Visudyne® (a drug injected intravenously and used to help direct the laser to the affected area) and is not suitable for all types of lesions.25

Recently, it was discovered that there is a protein in the eye which encourages the development of blood vessels.  Given the name "vascular endothelial growth factor" (VEGF), researchers have been working to develop treatments to inhibit VEGF by trapping it or preventing it from binding with elements which will stimulate growth. Chemically synthesized short strands of RNA (nucleic acid) called "aptamers" prevent the binding of VEGF to its receptor.  Presently three types of VEGF inhibitors are in use: Lucentis, Macugen and Avastin. All are given by intraocular injection.26
Posterior Staphyloma

Because of the inherent weakness of the sclera in EDS, these patients are more susceptible to developing posterior staphylomas.  This is usually seen in conjunction with high myopia.  Binocular indirect ophthalmoscopy or fundus photography are both good screening tools for staphylomas.27
Carotid-Cavernous Sinus Fistulas

A carotid-cavernous sinus fistula is the rupture of a blood vessel that subsequently bleeds into a sinus cavity and/or some part of the eye. The blood flow can cause serious structural damage to the eye and is considered a true emergency.  Individuals often report hearing their pulse in their temple and having a frontal headache on one side or the other. Sometimes the eye on that side is proptotic (it seems to be more prominent than the other eye) and it can become very red. 28

 Check for carotid-cavernous sinus fistula by placing a stethoscope over the patient’s temple and listen for a 'whooshing' sound. Carotid-cavernous sinus fistulas are more commonly found in the vascular form of EDS, (Type IV), but all types and the normal population are susceptible as well. 29—Next: Continue to common ocular symptoms associated with Ehlers-Danlos Syndrome

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References

1 P Beighton (1970). Serious ophthalmological complications in the Ehlers-Danlos syndrome.  British Journal of Ophthalmology April 54(4):263-268.

2 Ihme A, Risteli L, Krieg T, Risteli J, Feldmann U, Kruse K, Muller PK (1983).  Biochemical characterization of variants of the Ehlers-Danlos syndrome type VI.  Eur J Clin Invest Aug:13(4):357-62.

3 Heim A, Raghunath M, Meiss L, Heise U, Myllyla R, Kohlschutter A, Steinmann B (1998).  Ehlers-Danlos syndrome type VI (EDSVE): problems of diagnosis and management.  Acta Paediat. 87:708-710.

4 Pasquali M, Still MJ, Vales T, Rosen RI, Evinger JD, Dembure PP, Longo N, Elsas LJ (1997). Abnormal formation of collagen cross-links in skin fibroblasts cultured from patients with Ehlers-Danlos syndrome type VI.  Proc Assoc Am Physicians Jan;109(1):33-41.

5 Curtin BJ, Karlin DB (1970). Axial length measurements and fundus changes of the myopic eye.  Trans Am Ophthalmol Soc 68:312-334.

6 Maumenee IH (1981).  The eye in the marfan’s syndrome.  Trans Am Ophthalmol Soc. 79:684-733).

7 Pemberton J, Freeman M, Schepens C (1966). Familial Retinal Detachment and the Ehlers-Danlos Syndrome.  Archives of Ophthalmology Vol 76(6):817-824.

8 Woodward EG, Morris MT (1990).  Joint hypermobility in keratoconus.  Ophthalmic Physiol Opt.  Oct; 10(4):360-2.

9 Pesudovs K (2004).  Orbscan mapping in Ehlers-Danlos syndrome.  J Cataract Refract Surg 30:1795-1798

10 Segev F, Heon E, Cole W, Wenstrup R, Young F, Slomovic A, Rootman D, Whitaker-Menezes D, Chervoneva I, Birk D (2006).  Structural abnormalities of the cornea and lid resulting from collagen V mutations.  Investigative Ophthalmology and Visual Science; 47:565-573.

11 McDermott ML, Holladay J, Liu D, Puklin JE, Shin DH, Cowden JW (1998).  Corneal topography in Ehlers-Danlos syndrome.  J Cataract Refract Surg Sep;24(9):1212-5.

12 Hyams S,Kar H, Neumann E (1969). Ocular signs of a systemic connective tissue disorder.  Br J Ophthalmol Jan; 53(1):53-58

13 Sharma Y, Sudan R, Gaur A (2003). Post traumatic subconjunctival dislocation of lens in Ehlers-Danlos syndrome.  Indian J Ophthalmol Jun;51(2):185-6.

14  Gurwood AS, Mastrangelo DL (1997).  Understanding angioid streaks.  J Am Optom Assoc May;68(5):309-24.

15 Grand MG, Isserman MJ, Miller CW (1987). Angioid streaks associated with pseudoxanthoma elasticum in a 13-year-old patient.  Ophthalmology Feb;94(2):197-200.

16 Gomolin JE (1992).  Development of angioid streaks in association with pseudoxanthoma elasticum.  Can J Ophthalmol Feb;27(1):30-1.

17 Seki M, Iwasaki M, Takei K, Maeda T (1989).  A case of Ehlers-Danlos syndrome.  U.S. National Library of Medicine.  27(1):208-19.

18 Choudhury R, Revenco V, Darciuc R (2009).  Ehlers-Danlos syndrome.  BMJ Case Reports 10.1136.

19 Musch DC, Lichter PR, Guire KE, Standardi CL (1999).  The collaborative initial glaucoma treatment study: study design, methods, and baseline characteristics of enrolled patients.  Ophthalmology Apr;106(4):653-62.

20 Higginbotham, E (1998).  Initial treatment for open-angle glaucoma – medical, laser, or surgical?  Arch Ophthalmol; 116:239-240.

21 Lee D, Higginbotham E (2005).  Glaucoma and its treatment: a review.  American Journal of Health-System Pharmacy 62(7):691-699.

22 Meyer E, Ludatscher RM, Zonis S (1988).  Collagen fibril abnormalities in the extraocular muscles in Ehlers-Danlos syndrome.  J Pediatr Ophthalmol Strabismus :25(2):67-72.

23 Sangiovanni JP, Agron E, Meleth AD, Reed GF, Sperduto RD, Clemons TE, Chew EY (2009).  Omega-3 long-chain polyunsaturated fatty acid intake and 12-y incidence of neovascular age-related macular degeneration and central geographic atrophy: AREDS report 30, a prospective cohort study from the Age-Related Eye Disease Study.  Am J Clin Nutr; 90(6):1601-7.

24 Clemons TE, Miltob RC, Klein R, Seddon JM, Ferris FL (2005).  Risk factors for the incidence of advanced age-related macular degeneration in the Age-Related Eye Disease Study (AREDS) report no. 19.  Ophthalmology Apr;112(4):533-9.

25 Bee WL, Lindblad AS, Ferris FL (2003).  Who should receive oral supplement treatment for age-related macular degeneration?  Curr Opin Ophthalmol ;14(3):159-62.

26 Chew EY, Sperduto RD, Milton RC, Clemons TE, Gensler GR, Bressler SB, Klein Rm Dlein B, Ferris F (2009).  Risk of advanced age-related macular degeneration after cataract surgery in the Age-Related Eye Disease Study:AREDS report 25.  Ophthalmology 116(2):297-303.

27 Steidl SM, Pruett RC (1997).  Macular complications associated with posterior staphyloma. Am J Ophthalmol 123(2):181-7.

28 Gupta S, Thakur AS, Bhardwj N, Singh H (2008).  Carotid cavernous sinus fistula presenting with pulsating exophthalmos and secondary glaucoma.  J Indian Med Assoc;106(5):312, 346.

29 Calzolari F, Ravalli L (1997).  Spontaneous carotid-cavernous fistula: correlations between clinical findings and venous drainage.  Radiol Med 93(4):358-66.

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