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Shields > SECTION II - The Clinical Forms of Glaucoma >

13 - Childhood Glaucomas: Classification and Examination

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 > 13 - Childhood Glaucomas: Classification and Examination

13

Childhood Glaucomas: Classification and Examination

Childhood glaucomas constitute a rare, heterogeneous group of diseases. Often vision-threatening, these diseases present special challenges in diagnosis and optimal management. Parents and primary care providers usually first recognize the abnormalities that lead to the diagnosis of glaucoma in infants and young children, with devastating consequences when that correct diagnosis is substantially delayed. The clinical presentation of glaucoma varies with the age of onset and the severity of intraocular pressure (IOP) elevation. In addition, detailed ophthalmic examination of young children can be difficult, and management strategies for this rare condition are less familiar than those in adult patients. Pharmacologic, technological, and genetic advances in the diagnosis and treatment of glaucoma engender hope that children with this disease may face brighter visual futures.

CLASSIFICATION OF CHILDHOOD GLAUCOMAS

The glaucomas of childhood have been categorized in various ways, but one simple, commonly used system considers them as either primary or secondary in mechanism. Although this classification system is far from ideal (see Chapter 7), we use this terminology here because our somewhat limited insights today preclude a more meaningful conceptual framework for discussing childhood glaucomas.

The primary glaucomas, often genetic in origin, comprise those in which a developmental abnormality of the anterior chamber angle leads to obstruction of aqueous outflow. Within these primary glaucomas, congenital open-angle glaucoma— often termed primar y congenital glaucoma (PCG)—and juvenile open-angle glaucoma present without consistently associated ocular or systemic developmental anomalies. By contrast, primary glaucomas associated with ocular abnormalities— often called

developmental glaucomas— include primary glaucomas in which a developmental abnormality is responsible for the glaucoma, but in which additional ocular and systemic anomalies are typically present. Unlike the primary childhood glaucomas, secondary childhood glaucomas include glaucomas whose mechanism of outflow obstruction is acquired from other events, such as inflammation or neoplasia, rather than a primary anomaly of the angle. In a yearlong prospective study of all new childhood glaucoma cases in the United Kingdom and the Republic of Ireland, 99 cases were identified: 47 primary and 52 secondary in nature (1).

In this chapter, we consider the pediatric glaucoma patient and details of the examination specific to the child with known or suspected glaucoma. The following chapter, Chapter 14, addresses primary glaucomas of childhood, including PCG and the major developmental glaucomas. Because children are subject to many of the same secondary glaucomas as adults, these topics are discussed together in subsequent chapters of Section II, with special attention given to situations that apply only to children. One exception is the glaucoma occurring after removal of cataracts in infants and young children, as this “aphakic glaucoma” or “pseudophakic glaucoma” may w ell represent the second most commonly encountered single type of childhood glaucoma (after PCG) (1), and is therefore included in Chapter 14. Table 13.1 (2) gives one scheme for considering childhood glaucomas (2). Some pediatric glaucomas may have both primary and secondary causes (e.g., infantile-onset glaucoma in Sturge-Weber syndrome, neurofibromatosis, and aniridia). Many of the primary and developmental glaucomas are genetic in origin (see Chapters 8 and 14). Continuing elucidation of the genetics behind many conditions associated with pediatric glaucoma will no doubt lead to replacement of the current phenotypically driven diagnostic labels, with names and categories based on underlying genetic abnormalities.

SIGNS AND SYMPTOMS OF GLAUCOMA IN CHILDHOOD

The signs and symptoms of glaucoma in children vary tremendously with the age of onset and the degree of IOP elevation. Infants and young children with glaucoma (usually with PCG, but occurring with early-onset glaucoma of any cause) usually present because the family or pediatrician has noticed something abnormal about the eyes or the infant's behavior. Corneal enlargement or opacification (resulting from stretching due to high IOP), or both, often signal glaucoma in the infant; both may progress rapidly over the first 2 years of life if IOP remains elevated (Figs. 13.1 and 13.2). Buphthalmos is a term to describe the abnormal enlargement of an infant's eye secondary to elevated IOP; in extreme cases, these eyes are vulnerable to lens subluxation and rupture with even minor trauma (Fig. 13.3). The classic triad of findings usually ascribed to PCG (see also Chapter 14)—epiphora, photophobia (Fig. 13.4), and blepharospasm (3)—results from corneal e dema, often with associated breaks in the Descemet membrane called Haab striae. Descemet membrane breaks appear to occur only in the first 2 years of life; they leave permanent evidence of early-onset glaucoma and vary with respect to the associated corneal distortion and scarring (Figs. 13.5 and 13.6). Breaks with more vertical orientation may be seen after forceps delivery (Fig. 13.7) (4).

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Table 13.1 Childhood Glaucomas: A Classification Scheme

I. Primary Glaucomas

A. Congenital open-angle glaucomaa

1.Newborn glaucoma (iridotrabeculodysgenesis)

2.Infantile glaucoma (trabeculodysgenesis)

3.Late-recognized

B.Juvenile (open-angle) glaucoma

C.Associated with ocular abnormalities (anterior segment dysgenesis)a 1. Iridodysgenesis

a. Aniridiab

B.Secondary to intraocular neoplasm 1. Retinoblastoma

2. Juvenile xanthogranuloma

3. Leukemia

4. Melanoma

5. Melanocytoma

6. Iris rhabdomyosarcoma

7. Aggressive nevi of the iris

C.Secondary to uveitis

1.Open-angle glaucoma

2.Angle-blockage glaucoma a. Synechial angle closure

b. Iris bombé with pupillary bloc c. Trabecular endothelialization D. Lens-induced glaucoma

1.Subluxation-dislocation and pupillary block a. Marfan syndrome

b. Homocystinuria c. Weill-Marchesani

2.Spherophakia and pupillary block

3.Phacolytic glaucoma

E.After surgery for congenital cataract 1. Lens tissue trabecular obstruction

2. Pupillary block

3. Chronic open-angle glaucoma associated with angle abnormalities

F.Steroid-induced glaucoma

G.Secondary to rubeosis

1.Retinoblastoma

2.Coats disease

3.Medulloepithelioma

4.Familial exudative vitreoretinopathy

5.Chronic retinal detachment

H.Secondary angle-closure glaucoma 1. Retinopathy of prematurity

2. Microphthalmos

3. Nanophthalmos

4. Retinoblastoma

5. Persistent fetal vasculature

6. Congenital pupillary iris-lens membrane

7. Topiramate-induced

8. Central retinal vein occlusion

9. Iris stromal cysts

10. Ciliary body cysts

11. Cystinosis

I.Malignant glaucoma

J.Glaucoma associated with increased episcleral venus or venous pressure 1. Sturge-Weber syndrome (isolated vs. CNS involvement)

2. Cavernous or dural-venous fistula

3. Orbital disease

K.Secondary to maternal rubella

L.Secondary to intraocular infection

1.Acute recurrent toxoplasmosis

2.Acute herpetic iritis

3.Endogenous endophthalmitis

aThese conditions are all considered anterior segment dysgeneses by some authorities, because of their genetic underpinnings and may be further classified as those of neural crest cell origin or non-neural crest cell origin (2).

bGlaucoma associated with these conditions may also be considered secondary in some cases. CNS, central nervous system.

Figure 13.1 Infant with congenital glaucoma, showing buphthalmos and asymmetric enlargement of the corneas, right more than left. Corneal edema has resolved after successful angle surgery.

Additional nonspecific signs of glaucoma in early life include a deep anterior chamber and optic nerve cupping. In the absence of optic atrophy, the optic cup may decrease greatly in size with IOP reduction and will enlarge again if control of IOP is lost (3). Optic atrophy, which may result from chronic or severe IOP elevation, is irreversible.

Figure 13.2 Right eye of a 3-month-old infant presenting with enlarged, cloudy cornea in the setting of newly diagnosed congenital glaucoma. IOP was 35 mm Hg.

Figure 13.3 Severe buphthalmos in the left eye of a 6-monthold infant with congenital glaucoma and bilateral enlarged corneas and high myopia in the setting of Stickler-Marshall syndrome. This blind left eye was exposed and presumed painful and was subsequently enucleated.

Figure 13.4 Extreme photophobia in an infant girl, 6 months of age, with PCG. Bilateral corneal edema and photophobia improved after surgical treatment.

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Figure 13.5 Slitlamp appearance of tears in Descemet membrane, or Haab striae, in a patient with congenital glaucoma.

Figure 13.6 Haab striae in a patient with Axenfeld-Rieger glaucoma, viewed at the slitlamp under high magnification.

Figure 13.7 Forceps-related tears in Descemet membrane. Note the very straight Haab striae, oriented from superotemporal to inferonasal in the cornea of the right eye of this newborn infant boy. Permanent scarring and high astigmatism resulted in amblyopia in this eye.