capsular tension rings (CTR) can also be sewn into the sclera to fixate the capsular bag during phacoemulsification. One of the more popular approaches currently is to place CTR to redistribute zonular stress evenly throughout the capsular bag during phacoemulsification [4, 43, 44]. However, one needs to be careful using CTRs because the CTR can incarcerate cortical material. Incarceration of cortical material will make irrigation and aspiration of lens cortex more difficult and may possibly cause more zonular loss by pulling on cortex trapped between the CTR and the capsular bag. Another pearl is that the CTR should not be placed into a compromised capsular bag as the CTR may exit the capsular bag into the vitreous cavity and then require complex vitreoretinal surgery to remove the CTR and any prolapsed lens material [45].

A CTR is most safely used in a lens capsule with less than three or four hours of the dehiscence and after the removal of the cortex with the posterior capsule intact. However, in this context it may be just as efficacious to use a three-piece IOL placed along the axis of zonular loss. The haptics in a three-piece IOL probably produces as much tension as a CTR to distribute stress evenly among the zonules in an area/axis of compromise. In addition, if the IOL in the capsular bag should ever dislocate into the vitreous cavity, removal of an IOL is much simpler than removal of an IOL with a CTR in the bag.

If significant zonular laxity is present or greater than four or five hours of zonular dehiscence is noted, placement of a three-piece IOL in the sulcus is a safer option than attempting placement of an IOL in the bag. Under these circumstances, the risk of future in-the- bag IOL dislocation into the vitreous cavity is probably quite high, although no good studies elucidate the factors for subsequent in-the-bag IOL dislocations. One should keep in mind that the newer models of anterior chamber IOLs perform remarkably well and can be easily inserted through a widened clear corneal incision. Thus, if concern exists about posterior chamber IOL dislocation in a PXF eye, placement of an anterior chamber IOL can be a very good and safe option.

tion is probably due to the increased leakage seen from iris vessels in pseudoexfoliation eyes [41, 46, 47]. A longer course and slower taper of steroids should be planned for PXF eyes to increase the patient’s comfort and to decrease the risk of cystoid macular edema.

Summary for the Clinician

››Pseudoexfoliation (PXF) eyes have an increased risk of complications associated with cataract surgery due to poor pupil dilation and poor zonular integrity.

››Cataract extraction should be considered at earlier stages in PXF eyes because less zonular stress is induced by the removal of softer nuclei.

››To maximize pupil size during cataract surgery one can use additional mydriatic drops, intracameral lidocaine, viscoelastic, Kuglen hooks, the Beehler hook, or pupil expansion rings.

››A sufficiently large pupil is important for good visualization during cataract surgery and to prevent capsular phimosis that may be associated with late in-the-bag IOL dislocation.

››Cataract surgery techniques such as extra capsular cataract extraction and phacoemulsification using supracapsular prolapse should be used to minimize zonular stress during cataract extraction.

››Other pearls to decrease zonular stress during phacoemulsification include lowering bottle height, minimizing entrance and exit from the eye, chopping techniques, and avoiding standard divide-and-conquer.

››Using a CTR or a three-piece IOL may be helpful in redistributing zonular stress to minimize additional zonular loss.

››PXF eyes have more postoperative inflammation that may require a more prolonged course of anti-inflammatory treatment.

44.4.4  Postoperative Surgical Care

of the Pseudoexfoliation Eye

PXF eyes, even with uncomplicated cataract surgery, tend to have more postoperative cell and flare than non-PXF eyes. The increased postoperative inflamma-

References

 1. Lee, R.K. The molecular pathophysiology of pseudoexfoliation glaucoma. Curr Opin Ophthalmol 2008;19(2): 95–101.

 2. Thorleifsson, G., et al. Common sequence variants in the LOXL1 gene confer susceptibility to exfoliation glaucoma. Science 2007;317(5843):1397–400.

 3. Yang, X., et al. Genetic association of LOXL1 gene variants and exfoliation glaucoma in a Utah cohort. Cell Cycle 2008;7(4): 521–4.

 4. Wiggs, J.L. Association between LOXL1 and pseudoexfoliation. Arch Ophthalmol 2008;126(3): 420–1.

 5. Ozaki, M., et al. Association of LOXL1 gene polymorphisms with pseudoexfoliation in the Japanese. Invest Ophthalmol Vis Sci 2008;49:3976–3980.

 6. Hayashi, H., et al. Lysyl oxidase-like 1 polymorphisms and exfoliation syndrome in the Japanese population. Am J Oph­ thalmol 2008;145(3): 582–5.

 7. Fan, B.J., et al. DNA sequence variants in the LOXL1 gene are associated with pseudoexfoliation glaucoma in a U.S. clinic-based population with broad ethnic diversity. BMC Med Genet 2008;9: 5.

 8. Challa, P., et al. Analysis of LOXL1 polymorphisms in a United States population with pseudoexfoliation glaucoma. Mol Vis 2008;14: 146–9.

 9. Fingert, J.H.et al, . LOXL1 mutations are associated with exfoliation syndrome in patients from the midwestern United States. Am J Ophthalmol 2007;144(6): 974–5.

10.Schlotzer-Schrehardt, U., G.O. Naumann. Ocular and systemic pseudoexfoliation syndrome. Am J Ophthalmol 2006; 141(5): 921–37.

11.Kuchle, M., U. Schlotzer-Schrehardt, G.O. Naumann. Occurrence of pseudoexfoliative material in parabulbar structures in pseudoexfoliation syndrome. Acta Ophthalmol (Copenh) 1991;69(1): 124–30.

12.Streeten, B.W., et al. Pseudoexfoliative fibrillopathy in the skin of patients with ocular pseudoexfoliation. Am J Oph­ thalmol 1990;110(5): 490–9.

13.Streeten, B.W., et al. Pseudoexfoliative fibrillopathy in visceral organs of a patient with pseudoexfoliation syndrome. Arch Ophthalmol 1992;110(12):1757–62.

14.Gillies, W.E., A.M. Brooks. Central retinal vein occlusion in pseudoexfoliation of the lens capsule. Clin Experiment Ophthalmol 2002;30(3): 176–87.

15.Shrum, K.R., M.G. Hattenhauer, D. Hodge. Cardiovascular and cerebrovascular mortality associated with ocular pseudoexfoliation. Am J Ophthalmol 2000;129(1): 83–6.

16.Mitchell, P., J.J. Wang, W. Smith. Association of pseudoexfoliation syndrome with increased vascular risk. Am J Oph­ thalmol 1997;124(5): 685–7.

17.Bojic, L., et al. Pseudoexfoliation syndrome and asymptomatic myocardial dysfunction. Graefes Arch Clin Exp Ophthalmol 2005;243(5): 446–9.

18.Citirik, M., et al. A possible link between the pseudoexfoliation syndrome and coronary artery disease. Eye 2007;21(1): 11–5.

19.Schumacher, S., et al. Pseudoexfoliation syndrome and aneurysms of the abdominal aorta. Lancet 2001;357(9253): 359–60.

20.Tarkkanen, A., A. Reunanen, T. Kivela. Frequency of systemic vascular diseases in patients with primary open-angle glaucoma and exfoliation glaucoma. Acta Ophthalmol 2008; 86:598–602.

21.Hietanen, J., et al. Evaluation of the clinical association between exfoliation syndrome and abdominal aortic aneurysm. Acta Ophthalmol Scand 2002;80(6): 617–9.

22.Ringvold, A. Pseudoexfoliation and aortic aneurysms. Lancet 2001;357(9274): 2139–40.

23.Visontai, Z., et al. Increase of carotid artery stiffness and decrease of baroreflex sensitivity in exfoliation syndrome and glaucoma. Br J Ophthalmol 2006;90(5): 563–7.

24.Irkec, M. Exfoliation and carotid stiffness. Br J Ophthalmol 2006;90(5): 529–30.

25.Roedl, J.B., et al. Homocysteine in tear fluid of patients with pseudoexfoliation glaucoma. J Glaucoma 2007;16(2): 234–9.

26.Bleich, S., et al. Elevated homocysteine levels in aqueous humor of patients with pseudoexfoliation glaucoma. Am J Ophthalmol 2004;138(1): 162–4.

27.Vessani, R.M., et al. Plasma homocysteine is elevated in patients with exfoliation syndrome. Am J Ophthalmol 2003; 136(1): 41–6.

28.Leibovitch, I., et al. Hyperhomocystinemia in pseudoexfoliation glaucoma. J Glaucoma 2003;12(1): 36–9.

29.Turacli, M.E., et al. Methylenetetrahydrofolate reductase 677 C-T and homocysteine levels in Turkish patients with pseudoexfoliation. Clin Experiment Ophthalmol 2005;33(5): 505–8.

30.Yazdani, S., et al. Sensorineural hearing loss in pseudoexfoliation syndrome. Ophthalmology 2008;115(3): 425–9.

31.Cahill, M., et al. Pseudoexfoliation and sensorineural hearing loss. Eye 2002;16(3): 261–6.

32.Hammer, T., U. Schlotzer-Schrehardt, G.O. Naumann. Unilateral or asymmetric pseudoexfoliation syndrome? An ultrastructural study. Arch Ophthalmol 2001;119(7): 1023–31.

33.Puska, P.M. Unilateral exfoliation syndrome: conversion to bilateral exfoliation and to glaucoma: a prospective 10-year follow-up study. J Glaucoma 2002;11(6): 517–24.

34.Jeng, S.M., et al. The risk of glaucoma in pseudoexfoliation syndrome. J Glaucoma 2007;16(1): 117–21.

35.Grodum, K., A. Heijl, B. Bengtsson. Risk of glaucoma in ocular hypertension with and without pseudoexfoliation. Ophthalmology 2005;112(3): 386–90.

36.Shuba, L., M.T. Nicolela, P.E. Rafuse. Correlation of capsular pseudoexfoliation material and iridocorneal angle pigment with the severity of pseudoexfoliation glaucoma. J Glau­ coma 2007;16(1): 94–7.

37.Pasutto, F., et al. Association of LOXL1 common sequence variants in German and Italian patients with pseudoexfoliation syndrome and pseudoexfoliation glaucoma. Invest Oph­ thalmol Vis Sci 2008;49(4): 1459–63.

38.Conway, R.M., et al. Pseudoexfoliation syndrome: pathological manifestations of relevance to intraocular surgery. Clin Experiment Ophthalmol 2004;32(2): 199–210.

39.Asano, N., U. Schlotzer-Schrehardt, G.O. Naumann. A histopathologic study of iris changes in pseudoexfoliation syndrome. Ophthalmology 1995;102(9): 1279–90.

40.Akman, A., et al. Comparison of various pupil dilatation methods for phacoemulsification in eyes with a small pupil secondary to pseudoexfoliation. Ophthalmology 2004;111(9): 1693–8.

41.Drolsum, L., E. Haaskjold, M. Davanger. Results and complications after extracapsular cataract extraction in eyes with pseudoexfoliation syndrome. Acta Ophthalmol (Copenh) 1993;71(6): 771–6.

42.Drolsum, L., E. Haaskjold, M. Davanger. Pseudoexfoliation syndrome and extracapsular cataract extraction. Acta Ophthalmol (Copenh) 1993;71(6): 765–70.

43.Sun, R. Functions of the capsular tension ring. J Cataract Refract Surg 2007;33(1): 4.

44.Hasanee, K., Ahmed II, Capsular tension rings: update on endocapsular support devices. Ophthalmol Clin North Am 2006;19(4): 507–19.

45.Ahmed II, et al. Surgical repositioning of dislocated capsular tension rings. Ophthalmology 2005;112(10): 1725–33.

46.Kuchle, M., et al. The blood-aqueous barrier in eyes with pseudoexfoliation syndrome. Ophthalmic Res 1995;27(Suppl 1): 136–42.

47.Kuchle, M., et al. Quantitative assessment of aqueous flare and aqueous “cells” in pseudoexfoliation syndrome. Acta Ophthalmol (Copenh) 1992;70(2): 201–8.

Core Messages

››Pigment dispersion glaucoma (PDG) has particular features that affect its clinical management. Outcomes tend to be similar to other types of glaucoma with similar treatments.

››Patients with Pigment Dispersion syndrome have about a 10% risk of converting to PDG at 5 years and 15% at 15 years.

››Patients with angle recession may respond poorly to standard medical and surgical treatment and final visual outcomes may be influ- encedbyotherocularcomplications.Long-term follow-up is important as a certain percentage of angle recession patients can develop elevated IOP and glaucoma more than 10 years following initial injury.

to PDS may lead to increased intraocular pressure (IOP) and glaucomatous optic neuropathy (GON), which characterize pigment dispersion glaucoma (PDG).

PDS is an autosomal dominant disorder with variable penetrance [2]. It is more common among young myopic males (between 30 and 40 years old) of white ethnicity with a positive family history of PDS. Usually both eyes are involved, although the disease may be asymmetric. The amount of pigment observed during slit lamp examination has not been correlated to the risk of converting to PDG [3].

Clinically the hallmarks of PDG are the Krukenberg spindle (fine pigment granules on the corneal endothelium) (Fig. 45.1) slit like radial midperipheral iris ­transillumination defects (Fig. 45.2), and increased pigmentation of the trabecular meshwork (Fig. 45.3). Other findings include the presence of a pigmented line on the juxtazonular posterior capsule (Scheie Stripe) (Fig. 45.4), pigment on the anterior and posterior lens capsule near the equator (Zentmayer’s ring),

45.1  How Does Glaucoma in Pigment

Dispersion Syndrome Differ Clinically from Other Glaucomas?

Pigment dispersion syndrome (PDS) is a clinical entity characterized by the release of pigment granules throughout the anterior segment which has been attributed to friction between the posterior iris surface and the anterior zonular bundles [1]. Decreased outflow facility due

C. G. Vasconcelos deMoraes ( ) University of Sao Paulo School of Medicine, Rua Alves Guimaraes, Sao Paulo, Brazil e-mail: gustavousp@gmail.com

Fig. 45.1  Krukenberg spindle: fine pigment granules on the corneal endothelium (Courtesy of Robert Ritch, M.D)

Fig. 45.3  Increased trabecular pigmentation in PDS (Courtesy of Robert Ritch, M.D)

and prominent reverse convexity of the peripheral iris. Sampaolesi has also described a more posterior insertion of the iris root observed on gonioscopy [4]. PDG patients show an increased risk of rhegamatogenous retinal detachment (between 4 and 6% in 10 years) when compared to normal myopic individuals [3, 5]; thus, ophthalmologic examination in these patients should include careful­ fundoscopic examination of the peripheral retina. Histological studies have demonstrated the presence of increased pigment granules in Schlemm’s canal of PDG patients [6], which may play a role in the genesis of the disease. Imaging techniques have demonstrated an increased posterior iris

convexity that results in abnormal contact between the iris periphery and the zonular bundles (reverse pupillary block) [2, 7].

PDG patients show higher IOP peaks than PDS individuals with ocular hypertension during 24 h tension curves [8]. In 1956 Becker and Podos found abnormal results in PDS patients during the water drinking test (WDT) and tonography, as well as a high responsiveness to topical steroids [9]. The WDT has been used as a stress test to evaluate how an eye is able to deal with transient IOP elevation. The WDT is performed as follows:(1) water deprivation for at least 4 h before the test, (2) followed by the ingestion of 1 (one) liter of tap water in 5 min, (3) and IOP measurements 15, 30 and 45 min thereafter. The amount of IOP rise observed during the test estimates an eye’s outflow facility reserve. In a study at the University of Sao Paulo’s Glaucoma Center involving untreated PDG and primary open angle glaucoma (POAG) patients with similar baseline IOP, PDG patients showed higher IOP peaks and fluctuation during the modified diurnal tension curve than did POAG patients (unpublished data). These findings are consistent with the results of Becker and Podos described previously.

Patients with PDS have a 10% risk of converting to glaucoma at 5 years and 15% at 15 years. Young myopic males with an initial IOP greater than 21 mmHg are at increased risk of conversion [10]. The Ocular Hypertension Treatment Study (OHTS) group and the European Glaucoma Prevention Study (EGPS) group evaluated the variables associated with conversion