Ординатура / Офтальмология / Английские материалы / Atlas of Glaucoma, Second Edition_Choplin, Lundy_2007

examination under anesthesia. Diameters are measured in the vertical and horizontal meridians with calipers.

goniolens such as the Koeppe lens with magnification and a light source (see above). A direct ophthalmoscope may be used, but three-dimensional anatomy is not appreciated with this method and is especially important when evaluating the optic nerve head. Examination and documentation of the disc is of paramount importance for the diagnosis and management of developmental glaucomas. Disc asymmetry and enlarged (greater than 0.3) cup are rare in normal infants and children (Figure 14.52). Reversal of cupping with normalization of intraocular pressure is typically seen in infants and young children due to the distensibility of the scleral canal and lamina cribrosa (Figure 14.53). On the other hand, disc hemorrhage and venous occlusion with elevated pressure is unusual in this age group (Figures 14.54, 14.55).

DIFFERENTIAL DIAGNOSIS

Epiphora, blepharospasm, and photophobia in the infant or young child comprise the classical triad of congenital glaucoma (Figures 14.56, 14.57). Together or isolated, they are a sign of irritation due to the corneal edema secondary to elevated intraocular pressure and rupture of Descemet’s membrane. However, they are not pathognomonic and other cases must be considered, as summarized in Table 14.1.

Elevated intraocular pressure can be due to topical steroid use, traumatic angle recession (Figure 14.58), or traumatic globe perforation with pupillary block. Acquired optic atrophy can be seen in retinitis pigmentosa or after trauma, as in the battered child syndrome (Figure 14.59).

Figure 14.40 Birth trauma and breaks in Descemet’s membrane. This child underwent a difficult forceps delivery. Note the vertical orientation of the corneal defect.

Figure 14.42 Direct goniolenses. (a) Koeppe lens. (b) Layden lens.

Figure 14.41 Shallow anterior chamber secondary to occluded pupil with complete pupillary block. This child had suffered a perforating injury in the past.

GENETICS OF DEVELOPMENTAL GLAUCOMAS

The most common forms of congenital glaucomas are characterized by genetic heterogenicity. Recessive forms are more evident in communities where marriages within the family are common. Abnormalities that involve a number of different chromosomes have been described and suggest that many different genes may be responsible for developmental glaucomas. Some of the genes involved in glaucomas associated with other anomalies, such as aniridia, iridodysgenesis, Peter’s anomaly and Rieger’s syndrome, have been identified.

Shwalbe's line

Figure 14.44 Anatomic landmarks on gonioscopy. Note the difference between peripheral iris processes and peripheral anterior synechiae.

Figure 14.45 Primary congenital glaucoma, gonioscopic view. The iris insertion is posterior and flat. The ciliary body band is covered by iris processes and is barely visible. Grayish pigment is visible just anterior to Schwalbe’s line.

Figure 14.46 Primary congenital glaucoma, gonioscopic view. The iris insertion is anterior and flat. The tissue of the iris base extends to cover the scleral spur and most of the trabecular meshwork.

Figure 14.47 Primary congenital glaucoma, gonioscopic view. Pigmented granules of iris-like tissue appear to be enmeshed in a grayish membrane covering the trabecular meshwork.

Figure 14.54 Disc hemorrhage in a case of childhood glaucoma.

Figure 14.55 Branch retinal vein occlusion in a case of advanced juvenile glaucoma.

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INTRODUCTION

At the present time, reduction of intraocular pressure IOP is the only proven effective therapy for reducing the risk of glaucoma development or its progression. Several large, randomized, multicenter clinical trials supported by the National Eye Institute, discussed in Chapter 9, have clearly demonstrated the benefit of reducing IOP. These trials, which include the Advanced Glaucoma Intervention Study (AGIS), the Collaborative Initial Glaucoma Study (CIGTS), the Ocular Hypertension Treatment Study (OHTS), and the Early Manifest

Glaucoma Trial (EMGT), suggest that chronic differences of just a few millimeters of mercury may be significant in stabilizing glaucoma (Table 15.1). As in other disciplines of medicine, the decision on when to initiate treatment and the selection of therapy is based on the individual patient. In addition to carefully assessing the expense, inconvenience, and potential ocular and systemic side-effects of treatment, the patient’s overall health and life expectancy are also considered. These factors will help to determine how aggressively an individual patient should be treated, since the goal of therapy is the preservation of useful vision for the patient’s lifetime.