Ординатура / Офтальмология / Английские материалы / Pediatric Opthalmology_Mukherjee_2005

Conditions where steroids are used for long time and most of the time in both the eyes are-vernal catarrh, interstitial keratitis, iridocyclitis due to juvenile rheumatoid arthritis, post operative and post traumatic status, keratoplasty and sympathetic ophthalmitis.

In these cases steroid induced cataract may have to be taken as inevitable therapeutic risk and dealt with accordingly.

Out of all the conditions listed above spring catarrh is the most offending condition that causes steroid induced cataract in children because the parents are not explained the regime to follow who allow the children to use strongest possible steroid for quick relief.

Exact mechanism of production of cataract by steroids is not known. Steroid induced cataract has been reported following use of skin ointment, nasal drops, and inhalers containing steroids of various strength and for a long time.

Most widely accepted theory is that steroids act on the lens epithelium that is responsible for hydration of lens fibers. How ever it does not explain why steroid induced cataracts always start in posterior cortex.

Exact time taken for cataract to develop is not known. It never develops following use of steroid for short duration even in high dose like sub conjuctival injection.

The opacity develops as a posterior sub capsular opacity that is denser in the axis of the lens. It may be in the form of circular dense disc surrounded by lighter areas of opacification. The opacity being near the nodal point causes much visual disturbances for its size.

Common symptoms are : Glare, diminished distant vision, diminished vision in bright light, difficulty in near vision. It is frequent to find associated glaucoma that may contribute to further loss of vision. Hence, it is essential that all children who have been on steroid on long term should under go tests for raised intraocular pressure as well.

Management. As cataract is a common feature of steroid use, steps should be taken to:

1.Reduce the strength and frequency of steroid to a level that gives best therapeutic response without causing cataract.

2.Replace steroid by non steroidal anti-inflammatory drugs when ever possible.

3.Manage spring catarrh by weakest solution of steroid, mastcell stabiliser, astringent, anti histamine etc.

4.Examine both eyes periodically for diminished vision, glaucoma and amblyopia.

5.Surgical intervention when required with P.C.I.O.L and management of amblyopia.

Less common cataracts in children

1. Electric cataract57,58. The child is likely to develop cataract when struck by electric current or lightening of more than 200 volts that passes through the head. The Electric cataract is generally bilateral, how ever it may be denser on the side affected more severely than the other. Generally these children have sever skin and multisystemic involvement that may over shadow the ocular manifestation which becomes apparent only if the child survives and acute systemic effects of electric shock has subsided.

There are various types of opacities in electric cataract. They can be vacuoles, streaks, both in anterior and posterior cortex. Generally the complete lens gets opacified rapidly.

It may some times take months and years to develop cataract. If there are only few vacuoles present in anterior cortex with in first few months chances are that no significant opacities will develop.

Management. Cataract is to be managed as any other cataract following blunt injury, after the acute multisystemic signs and symptoms have subsided.

2. Sunflower Cataract59. This is due to electrical dissociation of retained intra ocular copper that does not combine with the cells. The reaction depends upon copper content of retained foreign body. More the copper in the foreign body, more sever and faster the reaction. The copper is mostly deposited on continuous membrane like lens capsule and corneal periphery59 various manifestations of intra ocular copper foreign body are - Endophthalmitis that is supurative in nature, chronic non granulomatous panuveitis and sun flower cataract.

The sun flower cataracts involve both anterior and posterior cortex and sub capsular area as yellowish dust in the centre of the lens with radiating yellowish petals pointing towards the periphery. Fully developed sun flower cataract is visible with on ordinary oblique illumination. Copper also gets deposited on corneal periphery as Kayser-Fleischer ring. Glaucoma is common. It is rare in children.

Management. It consists of removal of foreign body, vitrectomy, lens extraction and management of glaucoma.

C. Cataract in siderosis59

Iron is one of the commonest forms of intraocular foreign bodies in adults it is rare in children. It is rarely elemental iron, it is generally an alloy of iron. It is always associated with penetrating wound. The elemental iron is converted into its ferrous and ferric compounds. The metal is disseminated through out the tissues of the eye by electrolytic dissociation and gets combined with cellular protein that destroys the cells causing atrophy of the cells. The overall effect of dissemination depends upon ferrous content of the foreign body. Occasionally a small particle may get embedded in the avascular lens through the pupil and remain localised as a small dot of lenticular opacity never progressing to full blown cataract.

The earliest change is deposition of iron in the anterior sub capsular area of lens. The metal is deposited as brown rings resembling rust ring in the mid periphery. The lens subsequently develops larger opacities. The iris also develops brown iron deposits. The retina develops degenerative changes similar to pigmentary degeneration causing diminished night vision, dyschromatopsia and diminished vision. Diagnosis is based on history of penetrating injury on examination an external wound of entry is always present. There may be hole in the iris through which the foreign body has passed. Inter lenticular particle may be visible. It the media is clear the foreign body can be seen with direct and indirect ophthalmoscope. Brown pigment on lens periphery and iris should arouse suspicion of iron as foreign body.

In all cases of suspected intra ocular iron foreign bodies, the X-ray reveals its position unless it is very small CT and USG may show such bodies. MRI is contra indicated in metal foreign bodies.

Management depends on correct diagnosis of retained iron foreign bodies. Very small inter lenticular bodies do not require removal but the eye is kept under observation for possibility of development of siderosis on long run. Large foreign bodies are removed after proper

localisation through vitrectomy. Removal of iron foreign body reduces chance of siderosis. The lens, if opaque is removed by any of the standard procedures.

Cataract secondary to ocular inflammation

Cataract is a common complication of chronic uveitis of long duration. Exact cause of cataract formation is not known. It is multi factorial. The two common offending factors are the toxic effect of inflammation on the lenticular metabolism and Corticosteroids, which form the sheet anchor of the treatment of chronic uveitis. Commonly opacification starts in the posterior capsule. Later involving posterior cortex and ultimately covering whole of the lens. Sometimes broad posterior synechiea which are difficult to break cause anterior sub capsular opacities that are generally stationary.

Management consist of early and proper treatment of uveitis and steroid should be used in its minimum possible strength and duration. No surgical intervention should be undertaken unless the eye is quiet for at least six weak. The surgery should be followed by prolonged instillation of steroids as trauma of surgery results in flare up of uveitis.

Cataract in Lowe’s syndrome

Lowe’s syndrome consists of mental retardation, convulsion, renal stone formation, diabetes, albumen in urine. Ocular manifestation consists of bilateral nuclear cataract that may be microspherophekic. Associated glaucoma may worsen the prognosis.

Cataract in myotonic dystrophy

Myotonic dystrophy is a heredofamilial disorder where muscles have increased tone that is difficult to relax. This increased tone may be associated with progressive weakness of the muscles. The condition is generally seen in adolescents. There is progressive loss of facial expression and ptosis. Lenticular features consist of fine dots beneath the capsule that may have polychromatic lusture. The stelate appearance of such opacities are called Christmas tree cataract.

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B. Structures related to circulation of aqueous. The aqueous is produced from the ciliary processes and poured in posterior chamber from there it passes through the pupil in to the anterior chamber where it circulates and leaves the anterior chamber via angle of anterior chamber.

The structures related to circulation of aqueous are :

1.The aqueous chambers of the eye.

2.The angle of the anterior chamber.

1. The aqueous chambers. The ciliary body, zonules and the lens divide the eyeball in two unequal chambers - A large vitreous chamber and a smaller aqueous chamber.

The aqueous chamber is again divided into two unequal parts by the iris into : (a) A smaller posterior chamber

(b) A larger anterior chamber

(a) The posterior chamber is a space bounded anteriorly by the posterior surface of the iris. Posteriorly it is bound by periphery of anterior lens capsule, anterior plane of zonule and anterior part of the ciliary process.

(b) The anterior chamber is a convex space with convexity forward. The convex surface is bounded by the posterior surface of the cornea. The flat posterior surface is formed by a chink of anterior surface of ciliary body, the iris and the anterior lens capsule. The anterior chamber is lined by endothelium except on the posterior surface formed by lens capsule. The endothelium is absent in crypts of the iris also. The junction of the anterior and posterior surfaces is the angle of the anterior chamber. Being a plano convex space, its depth is maximum at the centre and gradually diminishes on the periphery where the two surfaces meet. Depth of the anterior chamber is genetically determined. It is shallow in new born, attaining adult depth after two years. It is deep in myopia, buphthalmos, megalo cornea, keratoconus, subluxation of lens and aphakia. It is shallow in micro-ophthalmos, micro-cornea, cornea plana, dysgenesis of anterior chamber, narrow angle glaucoma, swollen lens, hypotony and intraocular tumour pushing from behind.

The angle of anterior chamber. The angle formed by the posterior surface with the anterior surface is almost 45°. In normal eye the angle is not blocked even with maximum mydriasis.

Structures related to drainage of aqueous

C. The Trabecular mesh work. At the extreme periphery of the cornea on its inner side of the anterior chamber directly under the limbus is a depression that runs all round the angle of the anterior chamber. This is called scleral sulcus. The posterior lip of the sulcus is sharp and is called the scleral spur. The ciliary body is attached to the scleral spur. A sieve like structure bridges the scleral sulcus and is called trabecular meshwork. The meshwork extends from the scleral spur to periphery of the cornea. The line where the trabecular meshwork is attached to the corneal periphery is called Schwalbe’s line which is the thickened end of Descemet’s membrane. It forms an important landmark in gonioscopy. The trabecular meshwork converts the scleral sulcus into canal of irregular calibre called Schlemm’s canal.

The trabecular meshwork is a collection of tissues that on section looks more or less like a triangle, the apex of which lies at the Schwalbe’s line. The base is formed by scleral spur and ciliary body, the outer wall is formed by canal of Schelemm’s, the inner wall faces the anterior chamber.

The meshwork as per histological picture is divided into8 Corneoscleral meshwork, uveal meshwork, and endothelial meshwork. The uveal meshwork is adjacent to canal of Schlemm hence called juxta canalicular meshwork.

The canal of Schlemm. This is a venous channel that runs all round the angle of the anterior chamber in the sclera. It has irregular calibre. At most of the places it is a single endothelium lined channel, at places it breaks into tributaries and becomes multi channelled. The tributaries may merge with the main channel at places.

On the outer side the canal-of-Schlemm, is joined to episcleral and conjunctival veins. The episcleral veins drain into anterior ciliary veins, the conjunctival veins drain into palpebral and angular veins.

D. The limbus. The limbus is most important anatomical landmark for glaucoma surgery. It is an area 1 mm wide all round the cornea representing the junction of cornea on one hand, and conjunctiva, episclera and sclera on the other hand. From surgical point of view it has been divided into two circles and two zones in between5. The circles are formed by anterior limbal border, that is insertion of conjunctiva and Tenon’s capsule in the cornea. It overlies the termination of Bowman’s membrane. The posterior limbal border lies approximately over the scleral spur. An imaginary line between the anterior and posterior limbal border is called mid limbal line that lies over the Schwalbe’s line. The mid limbal line is the junction of blue corneal limbus to the scleral limbus.

The space between the anterior limbal border and mid limbal line is called anterior limbal zone. The space beyond the mid limbal line upto posterior limbal line is called posterior limbal zone.

The limbus does not form a ring of uniform width all round the cornea. The anterior limbal zone is narrowest at the horizontal plane of cornea and widest at superior limbus.

The development of anterior chamber. The presence of anterior chamber becomes evident at an early stage of embryo genesis i.e. 20 mm (7 weeks)6. As the lens separates from the surface ectoderm, the surface ectoderm is converted into corneal epithelium. A wave of mesoderm invades the space between the corneal epithelium and the lens to develop stroma of the cornea, endothelium of cornea, iris and iris stroma. The space between the iris mesoderm and the corneal stroma is anlage of the anterior chamber.7 This space remains very narrow as a cleft up to fifth month antenatal and then develops rapidly with the growth of the anterior segment.

The canal of Schlemm develops at about nine weeks6 as a vascular channel at the level of recess of angle and gradually moves anteriorly in relation to the iris.

The angle of the anterior chamber develops two months after the canal of Schlemm.8

The trabecular meshwork develops from vascular mesoderm originating from the margin of the optic cup.6 At birth the canal of Schlemm and the trabeculum both lie at the angle of the anterior chamber.9 The drainage system becomes functional just before birth.

Congenital anomalies of anterior chamber. Majority of glaucomas in children are either congenital or developmental hence it is necessary to know the common congenital anomalies of anterior chamber where most of the lesions lie.

The anterior chamber develops as a chink in the mesoderm between the corneal stroma and iris stroma. It is but natural that congenital anomalies of anterior chamber may involve cornea, iris and angle of anterior chamber in various combinations.

Roughly anomalies of anterior chamber can be divided into following groups :

A.Iridocorneal dysgenesis.

B.Mesodermal dysgenesis (anterior chamber cleavage syndromes)

C.Trabeculodysgenesis

In the first two groups the cornea is frequently involved and glaucoma is a common feature that manifests in childhood.

In trabeculodysgenesis, the trabecular tissue is mostly involved and cornea is either spared or effected minimally. Glaucoma in the form of primary congenital glaucoma is the most important clinical finding.

A. Irido corneal dysgenesis13. This is a rare condition, is most commonly known as essential iris atrophy14 and also as Cogan Reese syndrome or Chandler syndrome due to overlapping signs however some authors divide them as different entities. Endothelial defect being a prominent feature, the term iridocorneal defect is more appropriate.

The exact etiology of the condition is not known15. This is non-hereditary more common in females, it is unilateral mostly, rarely the other eye may be involved. Common age for it to become symptomatic is third and fourth decade. The disease starts in childhood and is detected during routine eye examination. No associated systemic disorders have been reported with the condition. Specular microscopy reveal peculiar cells in the endothelium, the cells are known as iridocorneal endothelial cells (ICE)16,17,18. The ICE cells are pathognomic and are supposed to be the cause of endothelial damage. The ICE cells migrate to the trabecular meshwork and cause mechanical obstructing resulting in glaucoma. The abnormal endothelial cells also cause corneal decompensation resulting in corneal edema. Corneal edema is independent of raised intraocular tension. The progress of the disease is slow, only 50% of eyes require antiglaucoma treatment for raised intraocular pressure that range between moderate and severe.

The ocular findings are :

Peripheral anterior synechea, full thickness hole in the iris, pseudo polycoria, shifting of pupil away from the holes towards the peripheral anterior synechea. The iris is wider in the sector where the holes develop, ectropion of uvea, heterochromia of iris. The iris is atrophic at places, there may be nodules on the iris. A thin transparent membrane is seen over the iris surface. The vision is generally poor due to corneal edema and glaucoma.

The condition should be differentiated from posterior polymorphic corneal dystrophy which may be seen in children, may be associated with glaucoma and hazy cornea and is bilateral.