Glaucoma in children

Glaucoma is a pathology rarely seen in childhood. Children's glaucoma combines a large group of various diseases. Most forms of pediatric glaucoma are the result of developmental defects in the anterior part of the eye and the structures of the anterior chamber angle.

Regardless of the pathophysiological mechanism, most forms of the disease have similar clinical symptoms, usually significantly different from glaucoma in adults.

[1], [2], [3], [4]

Symptoms of glaucoma

Enlargement of the eyeball

Children's sclera and cornea are less rigid, more elastic and tensile than adults. High intraocular pressure in many cases entails stretching of the eyeball and thinning of the outer shell of the eye. These changes rarely occur with glaucoma, which developed after 2 years of age.

[5], [6]

Changes in the cornea

Epithelium and stroma of the cornea easily tolerate an increase in its diameter, and descemet membrane and endothelium - much worse. As the stretching of the cornea progresses in the descemet sheath, breaks (Haab striae) occur concentrically or linearly. As a result of such changes, corneal edema may appear. Most of the symptoms of infantile glaucoma (glaucoma of early childhood) is a consequence of corneal edema.

[7], [8]

Photophobia and lacrimation

Apparently photophobia accompanies the opacity and increase in the diameter of the cornea. Severe lacrimation in some cases mimics the obstruction of the nasolacrimal canal.

Excavation of the optic disc

In infantile glaucoma, as well as in older patients, excision of the optic nerve disk occurs. However, in childhood, excavation can be reversible, the degree of excavation of the optic nerve in children is not an accurate prognostic sign.

[9], [10], [11],

Changes in refraction and strabismus

The stretching of the cornea and sclera leads to significant refractive disorders. Timely correction of these disorders is important for the prevention of amblyopia. Strabismus, especially in cases of asymmetric glaucoma, also provokes the appearance of amblyopia. If the refraction is shifted towards myopia, the accompanying glaucoma should be excluded from a child with aphakia.

[12], [13], [14], [15]

Primary congenital glaucoma

Primary congenital glaucoma (trabeculogenesis: primary infantile glaucoma) is the most common form of childhood glaucoma, occurring with a frequency of 1 case per 10,000 newborns. The disease is usually bilateral, but asymmetric and even unilateral forms occur. In the US and England, boys are more likely than girls, but in Japan there is a reverse situation. In Europe and North America, inheritance is manifested as polygenic or multifactorial. In the Middle East, it is inherited by an autosomal recessive type.

With gonioscopy, a number of features are distinguished.

1. Anomalies of the attachment of the iris:
   • flat attachment of the iris to the trabecular area in front or back from the scleral spur.
   • concave attachment of the iris where the surface of its root is shown above the trabecular network, adjacent to the ciliary body and the scleral spur.
2. The absence or rudiment of the scleral spur.
3. Very wide, stretched angle of the anterior chamber.
4. Absence of visible pathology of the helmet canal.
5. Occasionally, pathological changes in the iris are detected.
6. Amorphous tissues with vessels passing from the iris to the Schwalbe ring (Barkan membrane). Goniotomy or trabeculotomy are generally accepted primary surgical interventions aimed at reducing intraocular pressure.

The syndrome of Axenfeld-Rieger (Axenfeld-Rieger)

In describing the pathologically densified and shifted forward Schwalbe rings, the term "posterior embryotaxone" is used. The Aksen-Feld-Rieger syndrome is accompanied by:

• iridocorneal fissures;
• high attachment of the iris to the trabeculae, with the cover of the scleral spur;
• defects of the iris include thinning of its stroma, atrophy, ectopia of the pupil and ectropion of the choroid.

Glaucoma occurs in about 60% of people with this disease.

Rigger's syndrome

When the systemic pathology is accompanied by the aforementioned changes in the eyeball, the term "Rieger syndrome" is used. General pathology in this syndrome includes:

• hypoplasia of the midline of the face;
• a telecanthus with a broad, flat root of the nose;
• anomaly of teeth - absence of maxillary incisors, microdentism, anodontia;
• umbilical hernia;
• congenital heart defects;
• conductive hearing loss;
• mental retardation;
• hypoplasia of the cerebellum.

Despite the fact that the heredity of this disease has been established in an autosomal dominant type, a genetic defect has not yet been determined. There are reports of anomalies of 4, 6, 11 and 18 chromosomes in Rigger syndrome.

Aniridia

A rare congenital bilateral anomaly that is observed in various forms: sporadic and inherited in an autosomal dominant type. Glaucoma occurs in 50% of individuals. The pathomorphology of glaucoma has various manifestations. In some cases, the angle of the anterior chamber is free from goniosinechia, in others, the progressive development of synechia can lead to secondary, closed-angle glaucoma.

[16], [17], [18], [19], [20]

Syndromes, including children's glaucoma

Some congenital diseases are accompanied by defects in the anterior segment of the eyeball, including the angle of the anterior chamber, the iris and the lens. In some cases, these disorders cause glaucoma.

Sturgeon-Weber Syndrome (facial angioma)

This syndrome includes the classic triad:

1. crimson telangiectasia of the face;
2. intracranial angiomas;
3. glaucoma.

Glaucoma, almost in all cases unilateral, is diagnosed in approximately 1/3 of patients with Stirge-Weber syndrome. It can manifest itself at an early age, but in many cases it manifests in children of the older age group. The pathophysiology of the disease is diverse and associated with the development of the same disorders as in congenital glaucoma - increased episcleral venous pressure, premature aging of the structures of the anterior chamber angle. In addition, the possibility of formation of choroidal hemangiomas increases the risk of damage to the choroid or hemorrhagic complications during intraocular surgery. The disease occurs sporadically, there are isolated reports of family cases.

Marbling of the skin with congenital telangiectasias

A rare syndrome, in many respects identical to the Stirge-Weber syndrome. There are vascular disorders associated with the lesion of the skin and include marbled skin, episodic cramps, apoplexy and glaucoma.

Neurofibromatosis

Glaucoma can occur with Type I neurofibromatosis. In doing so, it is often combined with the ipsilateral columboma of the iris or eyelid and the plexiform neuroma of the orbit. The etiology of the process is associated with a combination of a number of factors, including both the pathology of the anterior chamber chamber tissue and the angle closure caused by neurofibromatosis.

Rubinstein-Taybi Syndrome

Rare syndrome, characterized by hypertelorism, antimonogloidnym eye incision, ptosis, lengthening eyelashes, wide fingers, increasing toes. In all likelihood, glaucoma occurs as a result of underdevelopment of the anterior chamber angle.

The anomaly of Peters

Peters anomaly is described as a congenital central opacity of the cornea, combined with defects of the stroma, descemet membrane and endothelium. Glaucoma in Peters' anomaly can exist from birth, and can occur after a through keratoplasty.

Juvenile open angle glaucoma

This form of glaucoma, inherited by an autosomal dominant type, is rare. Marked for the markers lq. Clinical examination is poorly informative, with gonioscopy of the pathology of the anterior chamber angle is not determined. Histological examination reveals an isolated pathology of the trabecular network.

Secondary glaucoma

Afakic glaucoma

In patients who have experienced cataract extraction in childhood, this form of glaucoma occurs with a frequency of 20-30%. The disease can begin many years after the operation. Pathogenesis is not completely clear, although in some cases it is associated with the development of pathological changes in the corner of the anterior chamber. Some sources associate aphakic glaucoma with certain types of cataracts, including nuclear cataract and persistent hyperplasia of the vitreous (PGST). An important risk factor is micro-roftalm. The extent to which the surgical intervention is responsible for the occurrence of glaucoma is unknown. The disease is difficult to treat and has an unfavorable prognosis.

[21], [22], [23], [24], [25], [26], [27]

Retinopathy of prematurity

Glaucoma can occur with severe retinopathy of prematurity, characterized by total retinal damage. The mechanism of the disorder is multifactorial, including neovascularization, the closing of the anterior chamber angle and the pupillary block.

The lens pathology and its interaction with the iris diaphragm

Patients with spherofakia (small spherical lens) tend to shift the lens anteriorly and the appearance of glaucoma. The disease can manifest itself both in the case of isolated spherofakia and in the symptom complex of Weil-Markesian syndrome. In homocystinuria, despite the fact that the lenses have a normal size, they are also prone to dislocation forward and the development of glaucoma.

Juvenile xanthogranulemia

Juvenile xanthogranulem is a benign disease commonly found on the skin, and less commonly as an intraocular process that can lead to glaucoma. Glaucoma is usually a consequence of hemorrhage.

Glaucoma in inflammatory diseases of the eye

Glaucoma can occur due to uveitis. Treatment is aimed at suppressing inflammation. Acute trabecu- litis or blockade with exudate of the trabecular network in some cases also lead to the development of glaucoma.

Injury

Glaucoma associated with blunt trauma of the eyeball is manifested when:

• hyphema (blood blocks the trabecular network);
• recession angle (characteristic later on the beginning of glaucoma).

Classification of glaucoma

None of the existing classifications of pediatric glaucoma is satisfactory. In most classifications glaucoma is divided into primary and secondary. In primary glaucoma, increased intraocular pressure is caused by an inborn violation of outflow of the intraocular fluid. In contrast, secondary glaucoma develops against the backdrop of a pathological process in other parts of the eyeball or systemic disease. This chapter uses an anatomical classification based on the proposed by Hoskins (D. Hoskins)

[28], [29], [30], [31]

Diagnosis of children's glaucoma

Reduction of visual acuity in these patients occurs as a result of damage to the optic nerve, corneal opacity, cataracts and amblyopia. The problems of amblyopia should not be over-exaggerated, it often remains undetected. Against the background of a timely scheduled plsopticheskogo treatment visual acuity can increase.

Many standard methods of adult research can not be used in small children on an outpatient basis. Computerized perimetry, tonometry in out-patient conditions are difficult for preschool children, and scarring and opacification of the cornea complicate the examination of the optic nerve. Often a complete diagnostic examination of a child with glaucoma requires the use of anesthesia.

[32], [33], [34], [35]

Drug treatment of glaucoma

With many forms of pediatric glaucoma, conservative treatment is not effective enough. It is usually prescribed as an attempt to stabilize intraocular pressure before surgery. A combination of oral administration or intravenous administration of acetazolamide with the administration of betaxalol and pilocarpine is common. The doses used are different, but should strictly correspond to the calculation per kg of weight.

[36], [37]

Surgery for glaucoma

To treat children's glaucoma, many methods of surgical interventions have been developed.

Goniotomy

The operation is particularly effective in trabeculogenesis, but requires a transparent cornea. Corneal ruptures can make it difficult to view and in such cases produce trabeculotomy.

Study under anesthesia of young patients with glaucoma

Anesthetics or sedatives

When examining newborns, anesthesia or sedatives should not always be used. Ketamine, suxamethonium and intubation are capable of increasing intraocular pressure. Halothane and many other drugs reduce intraocular pressure.

[38], [39]

Measured parameters

1. Intraocular pressure is measured immediately after the initial anesthesia. In any case, not being accurate and reliable, tonometry under anesthesia can not be the only criterion for assessing the glaucomatous process.
2. The horizontal and vertical diameter of the cornea from limb to limb. Measurement is difficult on significantly enlarged eyes and with an indistinct limb.
3. Investigation of the cornea for the presence of ruptures of the descemet membrane and to assess its overall transparency.
4. Refraction. Progression of myopia may reflect an increase in the size of the eye.
5. Investigation of the size of the eyeball by ultrasound.
6. Study of the optic disc, evaluation of the ratio of the diameter of the excavation and the optic nerve disk, the state of the disk boundaries.

* Preferably, conducting diagnostics, do not take into account the data of previous studies, in order to avoid bias judgments.

Yttrium-aluminum-garnet (YAG) laser goniotomy

Whether this procedure leads to a longer compensation of intraocular pressure in comparison with surgical goniotomy is still unknown.

Trabeculotomy

This is the operation of choice in the treatment of primary congenital glaucoma, when a good view of the anterior chamber angle is impossible

Combination of trabeculotomy-trabeculectomy

This procedure is especially suitable for combining the pathology of the anterior chamber angle with other intraocular abnormalities

[40], [41], [42], [43], [44]

Trabeculectomy

In young patients, filter cushions are rarely formed. The use of 5-fluorouracil (5-FU), mitomycin and local radiation therapy increases the duration of the existence of filter cushions.

Cyclocryotherapy

The destruction of the ciliary body, producing an intraocular fluid, is used in cases when other surgical interventions did not give the expected effect.

[45], [46], [47], [48], [49]

Endolaser

The use of endolaser gives a good effect.

[50], [51], [52], [53]

Implantation of drainage

Currently, a variety of tubular drains are used for implantation. Usually they are used not for primary surgical intervention, but only in cases when the previous operation was ineffective. After the improvement of models of drainage and surgical technique, such serious complications as hypotension decreased.