Ординатура / Офтальмология / Английские материалы / Clinical Pathways in Glaucoma_Zimmerman, Kooner_2001
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180 Pigmentary Glaucoma
Figure 9-1. Differential diagnosis of patient with pigmentary glaucoma.
Cardillo Piccolino and associates37 later described two brothers with pigmentary glaucoma who presented with bilateral degeneration of the peripapillary RPE, one of whom developed recurrent serous RPE detachments and neovascular choroidal membranes. Weseley and co-workers54 reported an incidence of lattice degeneration of the peripheral retina of 16.0% in 119 pigment dispersion patients. Scuderi and co-workers55 found lattice degeneration to be present in 33.3% of 24 patients with pigment dispersion. The authors accounted
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Table 9-4. Retinal Abnormalities Associated with Pigmentary Glaucoma
Degeneration of the retinal pigment epithelium
Lattice degeneration
Increased risk of rhegmatogenous retinal detachment
for the higher incidence of lattice degeneration in their series compared to that of Weseley et al14 by noting that the mean age of their study group was approximately 10 years higher than Weseley’s and that their patients were less myopic and thus less likely to exhibit lattice degeneration, which is more common in patients with myopia of 3.00 diopter (D) or less.56 Weakness of the peripheral retina was also noted by Brachet and Chermet,57 who reported 19 cases of rhegmatogenous retinal detachment in pigmentary dispersion patients. Finally, Scheie and Cameron8 found an incidence of retinal detachment of 6.6% in pigment dispersion patients and 7.6% in pigmentary glaucoma patients. This is much higher than the expected annual incidence of phakic, nontraumatic retinal detachment in the general population, which has been reported to be 0.005% to 0.01%. Furthermore, the majority of patients with retinal detachments in Scheie and Cameron’s series were only moderately myopic, suggesting that the increased risk for retinal detachment in pigment dispersion syndrome is substantial.
Treatment and Management
What Is the Treatment for Pigmentary Glaucoma?
The treatment for pigmentary glaucoma is similar to that for POAG (Table 9–5). Often with mild IOP elevation and optic disc damage, a topical beta-blocker is sufficient to lower IOP and prevent further visual field loss. When IOP remains elevated or evidence of visual field progression develops, other aqueous suppressants, such as 2-agonists or carbonic anhydrase inhibitors, may be added.
Table 9-5. Treatment of Pigmentary Glaucoma
Aqueous suppressants
Beta-blockers2-agonists
Carbonic anhydrase inhibitors
Increased aqueous outflow
Prostaglandin F2
Miotics
Pilocarpine
Carbachol
Dapiprazole
Argon laser trabeculoplasty
Laser iridotomy
Filtration surgery
182 Pigmentary Glaucoma
Because the conventional aqueous outflow pathway is compromised in pigmentary glaucoma, drugs that increase outflow through the uveoscleral pathway, such as prostaglandin F2 , may have added beneficial effect.
In some patients, the addition of a miotic, such as pilocarpine or carbachol, may be necessary. Along with increasing aqueous outflow, miotics may help prevent progression of the disease by flattening the iris and preventing further iridozonular contact and pigment dispersion.28 Unfortunately, miotic therapy is often poorly tolerated in young myopic patients because of accommodative spasm and increased myopia. In these patients, sustained-release forms of the drug, such as pilocarpine gel or Ocuserts, can sometimes be used more effectively. Miotics should be used cautiously in these patients and only after a dilated retinal exam, because of the association of peripheral retinal pathology and pigmentary glaucoma.57 Because of their ability to produce miosis and iris flattening without ciliary spasm and induced myopia, -adrenergic antagonists, such as thymoxamine, may someday play an important role in the treatment of pigmentary glaucoma. Mastropasqua and associates58 have shown that long-term therapy with dapiprazole, an -adrenergic antagonist, results in significantly increased outflow facility in pigmentary glaucoma patients. The disadvantages of dapiprazole include high cost of the medication and short shelf-life after reconstituting.
For those patients who are not adequately controlled with medical therapy, argon laser trabeculoplasty (ALT) can sometimes be used successfully, especially in younger patients, although its effect is usually short-lived. Lunde59 reported that an initial drop of 10.6 mm Hg following ALT in 13 eyes of 10 patients with pigmentary glaucoma was followed by an increase in IOP to higher than baseline levels after 9 months. Lehto60 reported an initial IOP-low- ering effect of 53% in pigmentary glaucoma patients, which decreased to only 14% after 3 months. When performing ALT in pigmentary glaucoma patients, lower energy levels should be used because of the increased pigmentation and energy absorption of the trabecular meshwork.
Peripheral iridotomy has been shown to alleviate the reverse papillary block and iris concavity seen in pigment dispersion patients, thus preventing iridozonular contact and further pigment dispersion. Gandolfi and Vecchi61 found a markedly reduced incidence of ocular hypertension in eyes with pigment dispersion syndrome following laser iridotomy. At this point, the role that laser iridotomy may play in the management of pigment dispersion syndrome and pigmentary glaucoma requires further study.
Filtration surgery is necessary in those patients who fail medical and laser therapy. Scheie and Cameron8 found that pigmentary glaucoma patients more frequently required surgical intervention than patients with POAG. Success rates of filtration surgery for these two groups of patients are similar.
Future Considerations
Where Is the Research on Pigmentary Glaucoma Heading?
Much has been learned about pigmentary glaucoma since its original description by Sugar and Barbour4 in the 1940s. Once thought to be a rare disorder, it
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is now known to be fairly common, with a prevalence of 2.45% in a large screening population.16 Future considerations for the diagnosis and treatment of pigment dispersion syndrome and pigmentary glaucoma appear quite promising. The -adrenergic antagonists such as dapiprazole and thymoxamine, which produce miosis and iris flattening with increased aqueous outflow facility and without the induced myopia and ciliary spasm of pilocarpine, are promising. -adrenergic antagonists, however, are not yet available for clinical use in the United States. Peripheral iridotomy, with its ability to alleviate the reverse pupillary block mechanism described by Karickhoff40 may also become more important in the treatment of the disorder, especially early in its course. New methods of patient evaluation, such as the technique for quantification of aqueous melanin granules as described by Küchle and associates48 may help us better assess treatment efficacy. Finally, gene therapy may someday play a role in the disorder as we build on the work of Andersen et al15 and better characterize the genetic basis of pigment dispersion syndrome.
References
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2.Von Hippel E: Zur pathologischen Anatomie des Glaukom. Arch Ophthalmol 1901;52:498.
3.Jess A: Zur Frage des Pigmentglaukoms. Klin Monatsbl 1923;71:175.
4.Sugar HS, Barbour FA: Pigmentary glaucoma: a rare clinical entity. Am J Ophthalmol 1949;32:90–92.
5.Bick MW: Pigmentary glaucoma in females. Arch Ophthalmol 1957;58:483–494.
6.Scheie HG, Fleischauer HW: Idiopathic atrophy of the epithelial layers of the iris and ciliary body. Arch Ophthalmol 1958;59:216–227.
7.Sugar HS: Pigmentary glaucoma: a 25-year review. Am J Ophthalmol 1966;62:499–507.
8.Scheie HG, Cameron JD: Pigment dispersion syndrome: a clinical study. Br J Ophthalmol 1981;65:264–269.
9.Berger A, Ritch R, McDermott J, et al: Pigmentary dispersion, refraction, and glaucoma. Invest Ophthalmol Vis Sci Suppl 1987;28:134.
10.Weber PA, Dingle JB: Pigmentary glaucoma in a black albino. Ann Ophthalmol 1983;15:454–455.
11.Farrar SM, Shields MB, Miller KN, et al: Risk factors for the development and severity of glaucoma in the pigment dispersion syndrome. Am J Ophthalmol 1989;180:223–229.
12.Ritch R: Nonprogressive low-tension glaucoma with pigmentary dispersion. Am J Ophthalmol 1982;94:190–196.
13.Stankovic J: Ein Beitrag zur Kenntnis der vererbung des Pigmentglaucom. Klin Monatsbl Augenheilkd 1961;139:165.
14.Olander KW, Mandelkorn R, Zimmerman T: The pigment dispersion and open-angle glaucoma. Ann Ophthalmol 1982;14:809.
15.Andersen JS, Pralea AM, DelBono EA, et al: A gene responsible for the pigment dispersion syndrome maps to chromosome 7q35-q36. Arch Ophthalmol 1997;115:384–388.
16.Ritch R, Steinberger D, Liebmann JM: Prevalence of pigment dispersion syndrome in a population undergoing glaucoma screening. Am J Ophthalmol 1993;115:707–710.
17.Mapstone R: Pigment release. Br J Ophthalmol 1981;65:258–263.
18.Wilensky JT, Buerk KM, Podos SM: Krukenberg’s spindles. Am J Ophthalmol 1975;79: 220–225.
19.Migliazzo CV, Shaffer RN, Nykin R: Long-term analysis of pigmentary dispersion syndrome and pigmentary glaucoma. Ophthalmology 1986;93:1528–1536.
20.Richter CU, Richardson TM, Grant WM: Pigmentary dispersion syndrome and pigmentary glaucoma: a prospective study of the natural history. Arch Ophthalmol 1986;104:211–215.
21.Murrell WJ, Shihab Z, Lamberts DW, et al: The corneal endothelium and central corneal thickness in pigmentary dispersion syndrome. Arch Ophthalmol 1986;104:845–846.
22.Lichter PR: Pigmentary glaucoma: current concepts. Trans Am Acad Ophthalmol Otolaryngol 1974;78:309–313.
23.Campbell DG, Schertzer RM: Pigmentary glaucoma. In Ritch R, Shields MB, Krupin T, (eds): The Glaucomas, Vol. 2, 2d Ed. St. Louis: Mosby-Year Book, 1996;975–991.
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24.Calhoun FP Jr: Pigmentary glaucoma and its relation to Krukenberg’s spindles. Am J Ophthalmol 1953;36:1398–1415.
25.Lichter PR, Shaffer RN: Iris processes and glaucoma. Am J Ophthalmol 1970;70:905–911.
26.Shenker HI, Luntz M, Kels B, et al: Exercise-induced increase of intraocular pressure in the pigmentary dispersion syndrome. Am J Ophthalmol 1980;89: 598–600.
27.Epstein DL, Boger WPI, Grant WM: Phenylephrine provocative testing in the pigmentary dispersion syndrome. Am J Ophthalmol 1978;85:43–50.
28.Haynes WL, Johnston AT, Alward WLM: Inhibition of exercise-induced pigment dispersion in a patient with the pigment dispersion syndrome. Am J Ophthalmol 1990;109:599–601.
29.Haynes WL, Alward WLM, Tello C, et al: Incomplete elimination of exercise-induced pigment dispersion by laser iridotomy in pigment dispersion syndrome. Ophthalmic Surg Lasers 1995;26:484–486.
30.Smith DL, Kao SF, Rabbini R, et al: The effects of exercise on intraocular pressure in pigmentary glaucoma patients. Ophthalmic Surg 1989;20:561–567.
31.Smith DL, Rabbani R, Kao SF, et al: Pigmentary glaucoma and exercise. Invest Ophthalmol Vis Sci Suppl 1988;29:28.
32.Marcus DF, Krupin T, Podos SM, et al: The effect of exercise on intraocular pressure. I. Human beings. Invest Ophthalmol Vis Sci 1970;9:749–772.
33.Stewart RH, LeBlanc R, Becker B: Effects of exercise on aqueous dynamics. Am J Ophthalmol 1970;69:245–248.
34.Rodriques MM, Spaeth GL, Weinreb S, et al: Spectrum of trabecular pigmentation in open-angle glaucoma: a clinicopathologic study. Trans Am Acad Ophthalmol Otolaryngol 1976;81:258–276.
35.Kupfer C, Kuwabara T, Kaiser-Kupfer M: The histopathology of pigmentary dispersion syndrome with glaucoma. Am J Ophthalmol 1975;80:857–862.
36.Chew EY, Deutman AF: Pigment dispersion syndrome and pigmented pattern dystrophy of retinal pigment epithelium. Br J Ophthalmol 1983;67:538–541.
37.Cardillo Piccolino F, Calabria G, Polizzi A, et al: Pigmentary retinal dystrophy associated with pigmentary glaucoma. Graefes Arch Clin Exp Ophthalmol 1989;227:335–339.
38.Campbell DG. Pigmentary dispersion and glaucoma: a new theory. Arch Ophthalmol 1979;97:1667–1672.
39.Kampik A, Green WR, Quigley HA, et al: Scanning and transmission electron microscopic of two cases of pigment dispersion syndrome. Am J Ophthalmol 1981;91:573–587.
40.Karickhoff JR: Reverse pupillary block in pigmentary glaucoma: follow up and new developments. Ophthalmic Surg 1993;24:562–563.
41.Potash SD, Tello C, Liebmann J, et al: Ultrasound biomicroscopy in pigment dispersion syndrome. Ophthalmology 1994;101:332–339.
42.Campbell DG: Iridotomy, blinking, and pigmentary glaucoma. Invest Ophthalmol Vis Sci 1993;34 (suppl):993.
43.Liebmann JM, Tello C, Chew SJ, et al: Prevention of blinking alters iris configuration in pigment dispersion syndrome and in normal eyes. Ophthalmology 1995;102:446–455.
44.Pavlin CJ, Harasiewicz K, Foster FS: Posterior iris bowing in pigmentary dispersion syndrome caused by accommodation. Am J Ophthalmol 1994;118:114–116.
45.Jensen PK, Nissen O, Kessing SV: Exercise and reversed pupillary block in pigmentary glaucoma. Am J Ophthamol 1995;120:110–112.
46.Speakman JS: Pigmentary dispersion. Br J Ophthalmol 1981;65:249–251.
47.Ritch R: A unification hypothesis of pigment dispersion syndrome. Trans Am Ophthalmol Soc 1996;94:381–409.
48.Küchle M, Mardin CY, Nguyen NX, et al: Quantification of aqueous melanin granules in primary pigment dispersion syndrome. Am J Ophthalmol 1998;126:425–431.
49.Richardson TM, Hutchinson BT, Grant WM: The outflow tract in pigmentary glaucoma. A light and electron microscopic study. Arch Ophthalmol 1977;95:1015–1025.
50.Campbell DG, Woods WD, Aiken DG: Studies concerning glaucoma caused by pigment particles in the trabecular meshwork. Invest Ophthalmol Vis Sci 1982;22 (suppl):192.
51.Samples JR, Van Buskirk EM: Pigmentary glaucoma associated with posterior chamber intraocular lenses. Am J Ophthalmol 1985;100:385–388.
52.Chaudhry IM, Moster MR, Augsburger JJ: Iris ring melanoma masquerading as pigmentary glaucoma. Arch Ophthalmol 1997;115:1480–1481.
53.McKinney JK, Alward WLM: Unilateral pigment dispersion and glaucoma caused by angle recession. Arch Ophthalmol 1997;115:1478–1479.
54.Weseley P, Liebmann J, Walsh JB, et al: Lattice degeneration of the retina and the pigment dispersion syndrome. Am J Ophthalmol 1992;114:539–543.
55.Scuderi G, Papale A, Nucci C, et al: Retinal involvement in pigment dispersion syndrome. Intl Ophthalmol 1996;19:375–378.
56.Byer NE: Lattice degeneration of the retina. Surv Ophthalmol 1979;23:213–247.
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57.Brachet A, Chermet TM: Association glaucome pigmentaire et de-collement de retine. Ann Ocul (Paris) 1974;207:451–457.
58.Mastropasqua L, Carpineto P, Ciancaglini M, et al: The usefulness of dapiprazole, an alphaadrenergic blocking agent, in pigmentary glaucoma. Ophthalmic Surg Lasers 1996;27:806–809.
59.Lunde MW: Argon laser trabeculoplasty in pigmentary dispersion syndrome with glaucoma. Am J Ophthalmol 1983;96:721–725.
60.Lehto I: Long-term follow-up of argon laser trabeculoplasty in pigmentary glaucoma. Ophthalmic Surg 1992;23:614–617.
61.Gandolfi SA, Vecchi M: Effect of a YAG laser iridotomy on intraocular pressure in pigment dispersion syndrome. Ophthalmology 1996;103:1693–1695.
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10
Glaucoma Associated
with Lens Disorders
Gustavo E. Gamero
Definition
This chapter discusses glaucoma as a direct or indirect result of some lens abnormality. A careful assessment of this heterogeneous group of diseases will result in the selection of the appropriate management. This discussion does not include cases in which glaucoma and the lens pathology simply coexist and a cause-effect relationship is not present.1, 2
How Is Glaucoma Associated with Lens Disorders Classified?
From a practical viewpoint these entities can be initially approached according to broad clinical features and then subdivided into more specific patterns of disease. We group together entities that have similarities either in their clinical picture or pathogenic mechanism. Accordingly, these glaucomas may be divided into four major categories: (1) glaucoma associated with ectopia lentis,
(2) glaucoma associated with cataract, (3) glaucoma associated with exfoliation syndrome, and (4) glaucoma associated with aphakia and pseudophakia.
Frequent overlapping of clinical features and mechanisms of disease is commonly seen.2 As a result some clinical presentations fit more than one disease category. For example, an eye with phacolytic glaucoma may also have a dislocated lens and angle recession from prior trauma. It is understood that not all possibilities can be outlined separately, and more than one decision-making route may be needed to reach the proper diagnosis. Complex diseases are to be evaluated on a case-by-case basis. By using an approach based on questions and answers, this chapter guides the reader through the appropriate differential diagnosis to select the correct treatment.
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188 Glaucoma Associated with Lens Disorders
Epidemiology and Importance
This is a very heterogeneous group of diseases that includes distinct hereditary and nonhereditary conditions. As a group they represent a small percentage of the total number of patients with glaucoma, but their impact on the health of the eye can be devastating if treatment is not instituted promptly.1 These patients usually present to ophthalmology clinics with a specific problem. With the exception of exfoliation syndrome, they are not the usual subjects for major population glaucoma studies. In general, the glaucoma associated with hereditary syndromes (e.g., homocystinuria, Weill-Marchesani syndrome) tends to present at an earlier age as the inborn lens abnormality progresses.2 These syndromes do not show a predilection for race or ethnic group and occur as a result of specific chromosomal abnormalities. Other entities, such as glaucomas associated with cataract or exfoliation syndrome, characteristically present in the elderly with variable severity. The presence of cataracts clearly increases with age,3 whereas the presence of exfoliation does show a predilection for certain ethnic backgrounds (see Glaucoma Associated with Exfoliation Syndrome, below).
GLAUCOMA ASSOCIATED WITH ECTOPIA LENTIS
Definition
What Is Glaucoma Associated with Ectopia Lentis?
This type of glaucoma is caused primarily by the abnormal position of the lens and its consequences.3 The various clinical conditions associated with ectopia lentis are listed in Table 10–1. Concurrent factors such as angle abnormalities or vitreous prolapse may play additional roles in the pathogenesis of the glaucoma.4
Table 10–1. Entities Commonly Associated with Ectopia Lentisa
Isolated ectopia lentis
Simple ectopia lentis
Ectopia lentis et pupillae
Systemic disorders
Marfan syndrome*
Homocystinuria*
Weill-Marchesani syndrome*
Hyperlysinemia
Sulfite oxidase deficiency
Ocular disorders
Trauma
Aniridia
Megalocornea
Others**
aEntities only rarely associated with ectopia lentis are not considered in this list.
*These entities make up more than 95% of the cases of nontraumatic, bilateral ectopia lentis. **Less common entities include congenital glaucoma, high myopia, Ehlers-Danlos syndrome, hyperlysinemia, syphilis.
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Epidemiology and Importance
Congenital ectopia lentis is a rare condition, and Marfan syndrome, homocystinuria, and Weill-Marchesani syndrome represent more than 95% of cases. Marfan syndrome has a prevalence of 4 to 6 per 100,000,5 with 60 to 80% developing ectopia lentis. Homocystinuria is even more rare, having a frequency of 0.021% in the mentally retarded,5 with 90% developing ectopia lentis. WeillMarchesani is more rare than Marfan and homocystinuria, and 80 to 90% of these patients develops ectopia lentis. Epidemiologic data on specific acquired conditions such as exfoliation syndrome will be mentioned when the pertinent entities are discussed.
Diagnosis and Differential Diagnosis
What Is the Pathogenesis of Glaucoma in Ectopia Lentis?
The following basic mechanisms of disease apply to most types of ectopia lentis (see Table 10–2). In general open-angle and angle-closure mechanisms can take place. Open-angle glaucoma has been divided in pretrabecular (usually membranes occluding the trabecular meshwork at the angle), trabecular (idiopathic and trabecular blockage by abnormal elements or distortion) and posttrabecular (Schlemm’s canal damage or elevated episcleral venous pressure). Angle closure can be caused by anterior (“pulling”) and posterior (“pushing”) forces, resulting in iris apposition to the cornea.1 Some specific differences will be pointed out when the pertinent entity is discussed.
Open-angle mechanisms consist of structural angle abnormalities and can contribute to the glaucoma in some syndromes.5 Pupillary-block angle closure is the most common mechanism and results from anterior displacement of the lens toward the pupil.2 The clinical presentation varies according to the degree and acuteness of the pupillary block. Therefore, acute, subacute, and chronic angle-closure glaucoma can occur. Vitreous prolapse around the dislocated lens can play an additional role in blocking the pupil and in some cases be the main factor.4 Total dislocation of the lens into the anterior chamber can occlude the pupil anteriorly causing an acute rise in intraocular pressure (IOP) (Tables 10–2 and 10–3). Finally, concurrent processes other than lens dislocation can contribute to the pathogenesis of glaucoma, such as trauma, hemorrhage, neovascularization, and cataract.
How Is Glaucoma in Ectopia Lentis Diagnosed?
The diagnosis of ectopia lentis begins with a good history and physical examination. A complete eye examination must be performed including a slit-lamp evaluation before and after maximal pupillary dilatation. After an initial external examination, a complete systemic workup is done to detect potentially serious conditions associated with ectopia lentis.5 The most noticeable conditions are cardiovascular complications in Marfan syndrome, and thromboembolic disease in homocystinuria (see below). Once a sustained elevation of IOP is documented, the diagnosis of ectopia lentis and glaucoma is made and the differential diagnosis can be outlined.