Добавил:

Sekretar kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.

Вуз:

Ростовский Государственный Медицинский Университет

Предмет:

Медицина общая

Файл:

Handbook of Glaucoma_Azuara-Blanco, Costa, Wilson_2001

.pdf

Скачиваний:

Добавлен:

28.03.2026

Размер:

10.54 Mб

Скачать

☆

<<< < Предыдущая 1 2 3 4 5 6 7 8 9 10 11 12 13 1415 / 2515 16 17 18 19 20 21 22 23 24 25 > Следующая >>>

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

Gonioscopy should be done if corneal clarity allows. A direct goniolens (e.g. infant Koeppe lens) and an operating microscope are ideal. In a normal infant, the iris inserts flatly into the ciliary body, and the normal trabecular meshwork appears as a smooth homogeneous membrane. In primary congenital glaucoma, the iris insertion is anterior, forming a scalloped line.

Ophthalmoscopy and assessment of the optic nerve are essential to diagnose and monitor glaucoma in children. Examination by direct ophthalmoscopy is facilitated by the use of a Koeppe lens or a contact lens used for vitrectomy surgery. Normal infants have a smaller C/D ratio than adults (most commonly C/D ratio < 0.3). In children with glaucoma the optic cup is larger and typically concentric, with a preferential loss in the vertical poles in advanced cases. Lowering the IOP in children with glaucoma is commonly associated with reversal of optic disc cupping.

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page 150...

Page 150

Figure 10.2 Haab’s striae in congenital glaucoma. Note typical pattern of rail-like refractile material at the level of Descemet’s membrane with a horizontal orientation.

Figure 10.3 Haab’s striae are easily seen with pupil dilated, using the red reflex.

2.When glaucoma appears in older children (up to 3 years of age), progressive enlargement of the globe and myopia may be indicative of uncontrolled glaucoma. After 3 years of age, the eye no longer expands in response to raised IOP.

3.Juvenile glaucoma refers to all forms of open-angle glaucoma diagnosed between the ages of 3 years and adulthood.

Treatment

Congenital glaucoma is managed surgically. Medical therapy is used before surgery or when repeated surgical procedures have failed. Success of surgical treatments is related to the severity and duration

131 of 235

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

of the glaucoma. The worst prognosis occurs in infants with raised pressures and cloudy corneas at birth. The best prognosis is seen in infants with isolated trabeculodysgenesis operated between the second and eighth months of life.

Goniotomy and trabeculotomy (see Chapter 13) are very effective operations in primary congenital glaucoma due to trabeculodysgenesis and they produce

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page_151...

Page 151

similar results. Success rates range from 60% to 90%, although in one-third to one-half of the eyes the procedure must be repeated. Success rates decrease in eyes with iris and/or corneal anomalies. Goniotomy has the advantage of preserving the conjunctiva for possible future filtering procedures. However, goniotomy cannot be done in cases of corneal clouding, and trabeculotomy is preferred to trabeculectomy.

If goniotomy and trabeculotomy are unsuccessful, trabeculectomy with mild mitomycin-C is recommended. Trabeculectomy, with or without mitomycin-C, can be combined with trabeculotomy. Because of the complications of late bleb leak and endophthalmitis, aqueous shunts are an alternative to trabeculectomy in the young. Shunts are always used before cyclodestructive procedures, which are the final option.

Even when the IOP is well controlled, a significant number of children never achieve good vision. The most common cause of poor vision is amblyopia related to anisometropia. Therefore, cycloplegic refraction is essential to prevent amblyopia. Occasionally, the corneal opacity may be permanent and penetrating keratoplasty may be indicated.

Glaucoma associated with developmental disorders

Aniridia

Aniridia is a hereditary bilateral congenital disorder characterized by the partial or total absence of iris (Figure 10.4). It is transmitted as an autosomal dominant trait in two-thirds of cases. A third of cases are sporadic and can be associated with Wilms’ tumor (in 20% of patients). An autosomal recessive mode of inheritance has also been described.

Figure 10.4 Infant with aniridia and subluxated lens.

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page 152...

Page 152

Other common ocular abnormalities include limbal stem cell deficiency and corneal pannus,

132 of 235

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

cataracts (very common after the third decade of life), ectopia lentis, and foveal hypoplasia with reduced vision and nystagmus. Glaucoma occurs in 50–75% of patients with aniridia, usually in late childhood, and is caused by progressive angle closure by the iris remnants. Possible systemic abnormalities include genitourinary abnormalities and mental retardation.

Medical treatment is the initial line of therapy. Although goniotomy and trabeculotomy can be considered in young children, filtering surgery is usually the best surgical approach.

Axenfeld–Rieger syndrome

Axenfeld–Rieger syndrome is a bilateral developmental disorder, with an autosomal dominant inheritance, a high incidence of associated glaucoma (more than 50%) and systemic developmental defects. There is no differentiation by sex, and most cases are diagnosed during childhood.

Three clinical variations have been described:

‘Axenfeld’s anomaly’ consists of a thickened and anteriorly displaced Schwalbe’s line, or posterior embryotoxon (seen on slit-lamp examination as a white line on the posterior cornea near the limbus, Figure 10.5) with abundant anterior strands of uveal tissue attached to Schwalbe’s line (Figure 10.6). These strands bridge the anterior chamber angle from the peripheral iris to the prominent ridge, and beyond these strands the trabecular meshwork is visible. The number and size of iris strands is highly variable, and is not related to the presence of glaucoma. An isolated posterior embryotoxon occurs in 10% of the general population and is not associated with glaucoma or systemic anomalies.

‘Rieger’s anomaly’ designates Axenfeld’s anomaly plus changes in the iris (from mild iris thinning to marked atrophy with polycoria (more than one pupil), corectopia, and ectopia uveae). Many other ocular anomalies have been reported.

Figure 10.5

Axenfeld anomaly with posterior embriotoxon.

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page 153...

Page 153

133 of 235

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

Figure 10.6

Gonioscopic view of numerous iris strands characteristic of Axenfeld–Rieger syndrome.

‘Rieger’s syndrome’ consists of ocular anomalies plus systemic developmental defects, most commonly dental (microdontia, hypodontia) and facial anomalies (maxillary hypoplasia, flattening of midface, receding upper lip and prominent lower lip). Anomalies of the pituitary gland region have been reported in several patients. Other neurological, dermatological and skeletal disorders have been described.

Goniosurgery is not as successful as in eyes with primary congenital glaucoma. Trabeculectomy, whether associated with trabeculotomy or not, with or without mitomycin-C, may be indicated if medical therapy is unsuccessful.

Peters’ anomaly

Peters’ anomaly is a sporadic anomaly present at birth, characterized by a central defect in Descemet’s membrane and corneal endothelium with stromal thinning (posterior keratoconus) and opacification. Iris adhesions typically extend to the borders of the corneal defect. 80% percent of cases are

bilateral.

There are different degrees of adhesions of the lens and the iris to the cornea, and three types of Peters’ anomaly have been differentiated: I. no keratolenticular contact or cataract; II. keratolenticular contact or cataract (Figures 10.7 and 10.8); III. with peripheral anomalies typical of Axenfeld–Rieger syndrome.

About 50% of patients with Peters’ anomaly will develop glaucoma, which usually requires surgical intervention (trabeculectomy). Penetrating keratoplasty is often needed, although the outcome is poor.

Sturge–Weber syndrome

Sturge–Weber syndrome is characterized by a facial hemangioma in the distribution of the trigeminal nerve, which may be associated with meningeal

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page 154...

Page 154

134 of 235

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

Figure 10.7 Peter’s anomaly with central opacity.

Figure 10.8 Slit-lamp view of Peters’ anomaly with lens-corneal adhesions and shallow anterior chamber.

Figure 10.9

Sturge–Weber syndrome. There is extensive facial hemangioma involving the trigeminal nerve.

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page_155...

135 of 235

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

Page 155

Figure 10.10

Choroidal hemangioma in a patient with Sturge–Weber syndrome. Note the ‘tomato catsup’ appearance of the fundus.

hemangiomas that can cause seizures (Figure 10.9). The disease is usually unilateral. Ocular hemangiomas can affect the conjunctiva, episcleral vessels, iris, angle, choroid or retina (Figure 10.10). Glaucoma is common when the hemangioma involves the lid or the conjunctiva, and is explained by two different mechanisms: abnormal development of the trabecular meshwork; and raised episcleral venous pressure.

Medical treatment can be attempted. Goniotomy and trabeculotomy are reasonably successful in infants who typically have a goniodysgenesis, but poor in older children. Infants and young children who do well with goniotomy or trabeculotomy usually require a trabeculectomy at 7 to 10 years of age. Combined trabeculotomy and trabeculectomy is recommended by several authors for children with glaucoma associated with the Sturge–Weber syndrome. However, filtration surgery is frequently complicated by choroidal effusions and there is an increased risk of intraand postoperative suprachoroidal hemorrhage. Prophylactic sclerostomies may be considered at the time of filtering surgery, and intraoperative and postoperative hypotony should be avoided.

Nanophthalmos

This rare anomaly is characterized by a small eye (axial length < 20 mm), reduced corneal diameter, high lens/eye volume ratio, shallow anterior chamber and narrow anterior chamber angle. These eyes are highly hyperopic and develop angle closure glaucoma in the fourth to sixth decades of life. Severe uveal effusions and nonrhegmatogenous retinal detachment are likely after intraocular surgery. These complications are secondary to reduced uveoscleral outflow, which is thought to be related to an abnormally thick sclera.

Laser iridotomy and peripheral iridoplasty are the initial procedures of choice. If filtration surgery is needed, scleral windows (dissecting areas of sclera to two-thirds thickness) and/or full-thickness sclerostomies should be done before

http://www.netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReader.dll?BookID=95266&FileName=Page 156...

Page 156

opening the globe to prevent the accumulation of fluid in the suprachoroidal space.

Glaucoma after congenital cataract surgery

Cataracts in children (Figure 10.11) can be treated by lens aspiration with or without posterior capsulotomy and possible anterior vitrectomy. Intraocular lenses may be inserted in the posterior chamber. Glaucoma after cataract surgery in children is a common and severe complication but the

136 of 235

http://www netlibrary.com.ezproxy.lib.alasu.edu/nlreader/nlReade...

cause of the glaucoma is controversial. It is possible that the trabecular meshwork is damaged by lens remnants and inflammation. Pupillary block and angle closure is an uncommon cause, since the use of vitreous cutting instruments allows a more complete removal of vitreous and lens tissue. Finally, it is suggested that some of these patients may already have an abnormal anterior chamber angle at birth.

Figure 10.11

Bilateral congenital cataract.

Glaucoma in aphakic children is a very challenging problem. Goniosurgery is not useful, and the outcome of trabeculectomy with mitomycin-C is usually poor. Glaucoma drainage devices and cyclodestructive procedures are often needed.