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Ординатура / Офтальмология / Английские материалы / Shields Textbook of Glaucoma, 6th edition_Allingham, Damji, Freedman_2010

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for retinal detachment. An alternative involves avoiding pilocarpine and using a transilluminator light held up to the opposite eye to provide pupillary constriction by the consensual light reflex (73). Whether a prophylactic laser iridectomy can prevent the development of glaucomatous optic neuropathy in patients with pigment dispersion or prevent progression in patients with pigmentary glaucoma awaits the results of long-term, multicenter trials.

When the glaucoma can no longer be controlled medically, argon or selective laser trabeculoplasty is usually indicated. Patients with pigmentary glaucoma respond well initially to the laser treatment, although the IOP control tends to decline with time and the surgery is less effective in patients who are older (e.g., patients in their 50s compared with those in their 30s) or who have had the glaucoma for a longer period of time (e.g., 10 years vs. 2 or 3 years) (74, 75 and 76). In general, these patients do well with minimal laser energy per spot (e.g., starting with 300 mW per spot if using argon laser trabeculoplasty, and 0.4 mJ per spot with selective laser trabeculoplasty). Low-energy settings are particularly important in patients who have had pigmentary glaucoma for a prolonged period and have advanced glaucomatous nerve damage to minimize the risk of a sustained postoperative IOP spike (77). Incisional Surgery

When medical therapy and laser trabeculoplasty have failed to adequately control the IOP, glaucoma filtering surgery is usually indicated. A higher percentage of patients with pigmentary glaucoma than of those with COAG require surgery, and men appear to require it at an earlier age than women do (10, 11). Success rates are similar to that with other forms of open-angle glaucoma at comparable age levels. Physical Activity

Exercise may increase pigmentary dispersion and elevate the IOP, which can be a concern in this population of young, active individuals (28). One approach to dealing with this question is to measure the IOP (and observe the amount of pigment in the anterior chamber) before and 30 minutes after the patient's typical exercise routine. If a significant pressure rise is observed, the use of pilocarpine, 0.5%, during exercise may be beneficial.

IRIDOSCHISIS

General Features

Iridoschisis is an uncommon condition, and in contrast to the young age of onset in pigmentary glaucoma, it usually appears in the sixth or seventh decade of life, although it may be seen in younger individuals. A case has been reported in a child with associated microphthalmos (78) and in a 30-year- old person with keratoconus (79). The hallmark is a bilateral separation of the layers of iris stroma, typically in the inferior quadrants. The disorder is complicated by glaucoma in approximately one-half of patients. Corneal edema is also an occasional sequela. Most cases are not associated with other ocular disorders, although concomitant conditions may be seen, including angle-closure glaucoma, anglerecession glaucoma, and syphilitic interstitial keratitis (in addition to those described earlier) (80, 81 and 82).

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Figure 17.8 A: Slitlamp view of a patient with iridoschisis. Note the regions of thinning, or “ shredding,” of the iris stroma, most noticeable at the 10 o'clock position. B: Gonioscopic image of an eye with iridoschisis. Note strands of iris extending toward the cornea. (Photographs courtesy of Joseph A. Halabis, OD.)

Clinicopathologic Features

Slitlamp biomicroscopic examination typically reveals sheets or strands of iris stroma that have partially separated from the rest of the iris (Fig. 17.8A), especially in the inferior quadrants. In some cases, the loose tissue may touch the corneal endothelium with adjacent edema of the cornea. By gonioscopy (Fig. 17.8B), the strands of iris tissue may obscure visualization of the anterior chamber angle. Histopathologic studies of involved iris revealed marked atrophy of the iris stroma with scant or absent collagen fibrils in the area of separation, although there was no evidence of vascular or neural alterations (83). Specular microscopy of the corneal endothelium has revealed a marked decrease in cell density and a high degree of polymegathism in the area directly over the iridoschisis (84). Histopathology of a corneal button, removed because of bullous keratopathy, showed degeneration and focal loss of endothelial cells, patchy posterior banding (110 nm) of Descemet membrane with irregular connective tissue, and stromal and epithelial edema (83).

Mechanisms of Glaucoma

Some patients with iridoschisis and glaucoma have angle closure (60, 83), and it is presumed that a pupillary block mechanism is present, because an iridotomy results in deepening of the anterior chamber (83). Iridoschisis may be an unusual manifestation of iris stromal atrophy resulting from the intermittent or acute IOP evaluation of pupillary block glaucoma (80). In other patients, the angle is open, in which case the meshwork is apparently obstructed by release of pigment from the iris or by the shredded iris stroma (83).

Differential Diagnosis

The main conditions that must be distinguished from iridoschisis are other causes of iris stromal dissolution, such as the iridocorneal endothelial syndrome and Axenfeld-Rieger syndrome, both of which differ from iridoschisis by a much younger age of onset. Trauma can lead to disruption of the iris, creating clinical findings that resemble iridoschisis, which may explain the relationship to anglerecession glaucoma (81). In one patient with iridoschisis, strands of iris floating in the anterior chamber after a trabeculectomy were mistaken for a fungal infection (85).

Management

Eyes with an angle-closure mechanism of glaucoma should be treated with a laser iridotomy, or with conventional surgical iridectomy if corneal edema prevents laser surgery. The open-angle form of glaucoma can be controlled medically in some patients by using an approach similar to that for COAG,

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but other patients require glaucoma filtering surgery. PLATEAU IRIS

One mechanism leading to angle-closure glaucoma appears to result from an abnormal anatomic configuration of the anterior chamber angle without pupillary block (86, 87). It is far less common than pupillary block glaucoma and is usually only recognized after a peripheral iridotomy for a presumed pupillary block mechanism has failed. Consequently, two variations of plateau iris have been described (88).

Plateau Iris Configuration

This diagnosis is made preoperatively on the basis of the gonioscopic findings of a closed anterior chamber angle but a flat iris plane (as opposed to the forward bowing of peripheral iris with the pupillary block mechanism) and a more normal central anterior chamber depth. Relative pupillary block plays a significant role in this situation, and most of these cases are cured by peripheral iridotomy. Recently, plateau iris configuration (with an angle that is appositionally closed after iridotomy) was described in association with long anterior zonules (89).

Plateau Iris Syndrome

Plateau iris syndrome constitutes a small percentage of eyes with the plateau iris configuration and represents the true plateau iris mechanism. The peripheral iris is anteriorly displaced (Fig. 17.9A) so that, as the pupil is dilated, it bunches up and closes the anterior chamber angle despite a patent P.281

iridotomy. This was traditionally believed to be caused at least partly by an anterior insertion of the iris, although studies with ultrasonographic biomicroscopy suggest that an anterior position of the ciliary processes prevents the peripheral iris from falling posteriorly after an iridotomy (Fig. 17.9B) (90, 91 and 92). Progressive anterior dislocation of the ciliary body with bolstering of the peripheral iris and formation of peripheral anterior synechiae (i.e., chronic angle closure) has been described in a case of plateau iris with long-term follow-up with use of ultrasonographic biomicroscopy (93).

Figure 17.9 A: Schematic showing height of plateau and relationship to angle structures. When the height of the plateau is such that dilation of the pupil will result in occlusion of the trabecular meshwork (c and d), then angle closure is possible despite a patent peripheral iridotomy. B: Ultrasonographic biomicroscopy shows plateau iris with the ciliary body touching the posterior iris.

Clinically, it is important to suspect this syndrome if the peripheral anterior chamber is shallow (i.e., van Herick technique, less than one fourth of the corneal thickness) despite a patent peripheral iridotomy or if there is a “high” plateau iris configuration on gonioscopy or an attack of angle-closure glaucoma despite a patent peripheral iridotomy. Additional tests that may help confirm the diagnosis include ultrasonographic biomicroscopy and provoking angle closure with phenylephrine (2.5% or 5%), ensuring that the pupil dilates to more than 7 mm and that the IOP increases after dilation. These cases are usually treated with dilute pilocarpine at bedtime or with peripheral iridoplasty (see Chapter 12). Pseudoplateau Iris Due to Cysts of the Iris or Ciliary Body

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Since the advent of ultrasonographic biomicroscopy, it has become evident that cysts of the iris or ciliary body epithelium can mimic the plateau iris syndrome (94). Clinically, this condition can be termed pseudoplateau iris and may be difficult to distinguish from true plateau iris. In a review of patients with plateau and pseudoplateau iris (with diagnosis based on results of ultrasound biomicroscopic examination), patients with pseudoplateau iris had a greater degree of trabecular meshwork pigmentation, had fewer clock-hours of gonioscopic angle closure, and were more likely to be male and have a bumpy peripheral iris appearance (visualized by using a narrow slit beam [Fig. 17.10A]), compared with patients who had plateau iris. Spherical equivalent did not differ significantly between the two groups (95).

It is important to confirm the diagnosis and the extent of cysts by performing ultrasound biomicroscopic examination (Fig. 17.10B,C), because secondary cysts can result from traumatic implantation of epithelium, from metastatic or parasitic lesions, or after the long-term use of miotics (96). If significant angle closure is not present, the prognosis is generally good (97, 98). In the case of significant angle closure, treatment may necessitate puncture of the cysts with a needle or with an Nd:YAG laser. Iridoplasty can also be helpful (99).

Swelling of the Ciliary Body

Any disorder giving rise to the swelling of the ciliary body or forward rotation of the ciliary body can create a plateau-like configuration of the iris.

Sulfa-based compounds may cause an idiosyncratic transient myopia, presumably produced by lens swelling and a forward movement of the lens-iris diaphragm associated with choroidal detachments and ciliary body swelling. Use of oral hydrochlorothiazide, oral acetazolamide, and topiramate, a new sulfaderived antiepileptic medication, have all been reported to precipitate bilateral angle-closure glaucoma, presumably by this mechanism (100, 101 and 102).

Supraciliary effusions and ciliary body thickening also appear to be common after scleral buckling procedures. They can produce conditions conducive to angle closure through a combination of direct anterior iris rotation and induced pupillary block (103).

Other conditions associated with ciliary body swelling include aqueous misdirection (see Chapter 26), acquired

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immunodeficiency syndrome (AIDS) and other inflammatory disorders (see Chapter 22), and idiopathic uveal effusion syndrome.

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Figure 17.10 A: Slitlamp examination shows a “ bump” on the peripheral iris. B: Ultrasonographic biomicroscopy reveals a large cyst in the iridociliary sulcus creating a “bump” in the periphera iris. C: Ultrasonographic biomicroscopy reveals multiple cysts within the iridociliary sulcus, creating a plateaulike configuration to the iris.

KEY POINTS

Pigmentary glaucoma is typically seen in young adults with myopia, with a predilection for men.

Iridozonular and iridociliary contact in these individuals apparently leads to liberation of pigment granules from the iris pigment epithelium.

Clinical findings include transillumination defects in the peripheral iris and deposition of the dispersed pigment on the corneal endothelium, iris stroma, trabecular meshwork, and other anterior ocular structures.

The glaucoma associated with the pigment dispersion is related to the accumulation of pigment in the trabecular meshwork, with subsequent alteration of the trabecular beams, leading to elevated IOP and glaucomatous damage.

Iridoschisis is an uncommon affliction of the elderly, characterized by a separation of layers of iris stroma with occasional associated glaucoma.

Plateau iris is a form of angle-closure glaucoma in which an anterior position of the ciliary processes appears to be responsible for the angle closure.

Pseudoplateau iris is a condition in which primary cysts in the iridociliary sulcus result in a plateau iris-like configuration with subsequent angle-closure glaucoma.

Swelling of the ciliary body with angle closure can occur by various mechanisms, including use of sulfa-based oral medications and scleral buckling.

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18 - Glaucomas Associated with Disorders of the Lens

Authors: Allingham, R. Rand

Title: Shields Textbook of Glaucoma, 6th Edition Copyright ©2011 Lippincott Williams & Wilkins

> Table of Contents > SECTION II - The Clinical Forms of Glaucoma > 18 - Glaucomas Associated with Disorders of the Lens

18

Glaucomas Associated with Disorders of the Lens

Several disorders of the crystalline lens are associated with various forms of glaucoma. In some cases, such as the exfoliation syndrome (see Chapter 15), a cause-and-effect relationship between the lenticular abnormality and the glaucoma is uncertain. In other situations, including some forms of dislocated lenses and cataracts, the glaucoma is more clearly a result of the alteration in the lens.

GLAUCOMAS ASSOCIATED WITH DISLOCATION OF THE LENS Terminology

Several terms have been applied to the clinical situation in which the crystalline lens is displaced from its normal, central position behind the iris. Subluxation of the lens implies an incomplete dislocation in which the lens is still at least partially behind the iris but is tilted or displaced slightly in an anterior or a posterior direction or perpendicular to the optical axis. With complete dislocation, the entire lens may be in the anterior chamber or may have fallen posteriorly into the vitreous cavity. The term ectopia of the lens, or ectopia lentis, is also applied to cases of lens dislocation, but it is nonspecific with regard to the degree of lens displacement.

Subluxation or complete dislocation of the lens may be associated with a number of clinical conditions, all of which can lead to glaucoma by a variety of mechanisms. We first review the more common clinical forms of ectopia lentis and then consider the mechanisms by which these conditions may lead to intraocular pressure (IOP) elevation and how these glaucomas are managed.

Clinical Forms of Ectopia Lentis Traumatic Dislocation

Trauma is the most common cause of a displaced lens (1, 2) (Fig. 18.1). In one series of 166 cases, injury was reported to account for 53% of the total group (2).

Exfoliation Syndrome

The exfoliation syndrome can be associated with spontaneous or traumatic lens subluxation or dislocation (see Chapter 15).

Simple Ectopia Lentis

Dislocation of the lens may occur without associated ocular or systemic abnormalities as a congenital anomaly or as a spontaneous disorder later in life (3). Both forms are typically inherited by an autosomal dominant mode (3). The condition is usually bilateral and symmetrical, with lens dislocation generally upward and outward and occasionally into the anterior chamber. Associated problems include glaucoma and retinal detachment.

Ectopia Lentis et Pupillae

Ectopia lentis et pupillae is a rare, autosomal recessive condition characterized by small, subluxated lenses and by oval or slit-shaped pupils that are displaced, usually in the opposite direction from that of

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