Ординатура / Офтальмология / Английские материалы / Pediatric Opthalmology_Mukherjee_2005

Cutting of consumption of sweets like candies, chocolates and supplementation of food with multi vitamin orally helps in reduction of recurrence. Children should be instructed to keep the hands clean and nails trimmed.

2. Chalazion. In contrast to stye, chalazion is a chronic granulomatous inflammation of meibomian glands. Otherwise chalazion share same epidimiology as stye i.e. it is a disorder of children and young adults and it is equally common in both sexes, any or all lids may be involved separately or simultaneously. There may be repeated crops of chalazia. It is more common with uncorrected errors of refraction. It differs from stye in following points:

It is chronic granuloma, neither painful nor tender, preauricular glands are not enlarged.

The chalazion is heralded by changes in the duct of the meibomian gland which are obstructed due to infection spreading from the lid margin. This results in stagnation of oily meibomian discharge in the acini of the glands. This pent up lipid acts as an irritant that result in a granuloma with giant cell without caceasion. The chalazion is well circumscribed and smooth without a true capsule of a cyst.

The skin over the chalazion can be moved. On evertion of the lid the conjunctiva over the chalazion shows a bluish coloration.

There may be multiple chalazia in the same lid or in all the lids. Chalazion is a selflimiting inflammation. Small chalazion may resolve without any treatment or simply by frequent hot fomentation. If it does not resolve by it self-following changes may be seen:.

(i) The growth increases in size and the central core may liquefy forming a tarsal cyst that may cause pseudo ptosis and induce astigmatism.

(ii) It bursts on

(a) Most commonly on the conjunctival surface, giving rise to a sessile papillomatous granuloma.

(b) It may less frequently rupture on the skin surface.

(c) Rarely it may protrude through the opening of meibomian orifice. Chalazia in children never undergo neoplastic change. Though meibomian cell carcinoma after 40 years may initially present as chalazia.

(iii) The chalazion gets secondarily infected and becomes severely painful. It is called Hordeolum internum. Sometimes chalazion may be associated with stye as well.

Management of chalazia21,22

Many of the chalazia resolve without treatment. However if a chalazion persists it requires surgical drainage and curettage of the granulomatous tissue followed by cauterisation of the wall of the growth by carbolic. In children chalazia should be incised under general anaesthesia.

Intra lesion injection of steroid: 10mg of triamcinolone acetonide is injected in the chalazion under local anaesthesia from conjunctival surface. A patient may require two to three such shots.

Hordeolum internum requires more energetic treatment that consists of dry hot fomentation, systemic analgesic and anti-inflammatory drugs, systemic broad-spec- trum antibiotic. No attempt should be made to drain the acute chalazion unless pain and

inflammation has subsided.

Prevention is similar to that of stye i.e. hygiene of lids, face and hands, correction of errors of refraction, long term local application of broad spectrum antibiotic on the lid margin.

Differential diagnosis of burst chalazion consist of : Foreign body granuloma.

Papilloma.

Haemangioma.

Rhinosporidiosis of tarsal conjunctiva.

C. Cutaneous and subcutaneous inflammation of the lids

All infective processes that involve the skin of the face and forehead can affect the lid. They can be viral, bacterial and fungal.

1.Viral infection of the lids are :

(a) Chicken pox (b) Measles

(c) Molluscum (d) Herpes zoster

(e) Herpes simplex

All the above conditions cause eruptions on the lid, may involve the conjunctiva, as well as cornea. Herpes zoster is classically unilateral others are bilateral all the above conditions have or may have systemic involvement.

(a) Chicken pox (Varicella)16,18 is very common exanthematous disorder of the lid caused by varicella zoster virus, it starts as hyperaemia with vesicle formation, which subside within 10 to 12 days leaving no scar, the vesicles can be seen on the lid margin as well, may develop in conjunctiva simulating phlycten. There may be ulceration or vesicles on the conjunctiva, may produce superficial keratitis pseudo dendretic keratitis and disciformkeraitis. Frank ulcer is not uncommon. If there is involvement of nasolacrimal duct it may lead to chronic dacryocystitis4.

Treatment. There is no known method to immunise the child against chicken pox, there is no specific systemic treatment. The child should be observed for possibility of pneumonia or encephalitis. Antiviral drugs have not proved to be effective. Immunoglobulins have favourable result when started with in three days of onset of skin rashes.

Ocular management. Consists of relief of photophobia and watering due to corneal involvement. Antibiotics are used to prevent secondary bacterial infection. The child should get full dose of cycloplegia, if needed atropine may be used.

(b) Measles (Rubeola, Morbilli)23,24. Measles is very common among non-immunised children. It mainly affects the respiratory tract and conjunctiva. The lid it is involved as part of generalised hyperaemic rashes that become papules, these are slightly raised and red. They may be separate or merge with each other. Conjunctivial involvement is universal; it starts as

catarrhal conjunctivitis that may be superimposed by secondary infection. Koplic spots develop on the conjunctiva, there may be sub conjunctival haemorrhage. corneal involvment is common causing blepharospasm. There are various stages of epithelial keratopathy, frank corneal ulcer develop in malnourished children. Blindness due to measles can be as high as 1% of all children affected mostly due to bacterial infection, malnutrition and hypovitaminosis A. There may be edema of the lid, cellulitis lid, multiple styes and blepharitis.

Management

Treatment is symptomatic. All parents should be encouraged to get children immunised against measles. It is one of major cause of depletion of vitamin A and not only vision threatening but potentially fatal.

1. Compulsory immunisation is essential not only to save the eye but also life of the

child.

2.Administration of additional does of water-soluble vitamin A 50,000 I.U.daily for four days orally.

3.Bland lotion to prevent sticking of eyelids.

4.Broad spectrum antibiotic drops for conjunctivitis

5.Cycloplegie if cornea is involved.

(c) Molluscum contagiosum. This is a chronic self-limited virus disease of skin caused by poxvirus. The lesions may appear any where on the body as dome shaped, translucent vesicles, which are umblicated, pain less, not associated with fever or any other systemic manifestation. They are not as numerous as eruptions of chicken pox, or small pox, number may vary from few to few dozens all over the body. May be seen on the lid or lid margin or both. Some times they are seen on conjunctiva or cornea. Corneal involvement causes redness, watering and photophobia due to pseudo dendritic ulcer, pannus formation and sub epithelial infiltration. When the lesion is near the puncta it may block the puncta causing epiphora. Keratitis and follicular conjunctivitis are toxic in nature.

Treatment. The disease is self-limiting but may last for weeks. It is contagious. There is no known immunisation, anti viral drugs are of not of much use. They are best treated by puncturing each lesion either by needle or electrocautery, the interior of the punctured lesion is curetted by tincture iodine or weak solution of carbolic. The lesion can be treated with cryo as well. Corneal involvement may require broad spectrum anti biotic drops and cycloplegia.

(d) Herpeszoster Ophthalmicus18,19,20. Herpeszoster ophthalmicus is an acute eruptive condition of the lid caused by zoster varicella virus that causes chicken pox otherwise. It is morphologically similar to herpes simplex virus but differs in antigen. Herpes zoster ophthalmicus can occur at any age, however it is less common in children. Children tolerate pain of herpes zoster better than adults, may be the pain is less sever in children. Children are not known to suffer from secondary zoster that is seen in immuno compromised adults.

Herpeszoster ophthalmicus may be mild or may be very severe. It is known to affect all parts of the eye and adnexa except the lens.

In herpes zoster ophthalmicus the infection lies dormant in posterior root ganglion of

trigeminal nerve following systemic infection either in the form of chicken pox or zoster itself and then travels down the first division of the fifth nerve to reach the eye and adnexa.

Herpes zoster ophthalmicus starts as hyperaemic rash over the fore head and skin of the lid or lid margin. May be associated with mild fever and malaise. The eruptions are initially macular and soon converted into vesicles similar to chicken pox. Generally facial pain precedes appearance of rashes. The vesicles are as a rule always unilateral they do to cross the mid line however associated edema of the forehead, lid and face may spill over the mid line giving a false impression of allergic edema or bacterial cellulitis. Preauricular lymph nodes are enlarged. Like chicken pox the vesicles take ten days to dry and crust. The crust fall in due course leaving shallow scars that may disappear in time. If the lesion was deep it may leave permanent scar on the forehead, lid or lateral side of the nose. Scar of the lid margin may cause notching of lid margin or trichiasis. If the vesicles are present on the tip of the nose it means that nasocillary nerve is also involved. This is called Hutchinson sign. This is almost sure indication of corneal involvement. However cornea may be involved without involvement of nasociliary nerve.

Lesions of herpes zoster may be divided into (i) Involvement of lid and conjunctiva

(ii) Involvement of globe (iii) Neurological lesions

(iv) Post herpetic complications

(i) Lids may show very mild hyperaemia to server vesicular eruption and edema of the lid obliterating interpalpebral fissure.

The vesicle may develop along the distribution of one or all the branches of first division of fifth nerve. Involvement of maxillary division is rare. In initial stages herpes zoster of lid may be confused as allergic edema, insect bite, stye or bacterial cellulitis

Conjunctiva may show vesicles, chemosis and subconjunctival haemorrhage. It is very common to develop secondary bacterial conjunctivitis.

(ii) Involvement of the globe

●Corneal involvement. Involvement of cornea is very common. It may happen without involvement of nasociliary nerve, its severity is not directly proportionate to lid involvement, server corneal involvement may be seen with mild lid involvement. Corneal involvement is varied it may be :-

Epithelial punctate infiltrates that stain better with rosebengal, microdendrites filamentary keratitis

Diminished corneal sensation Numular keratitis

Disciform keratitis

Corneal involvements do not respond much to anti-viral drugs but respond to local steroids. Numular keratitis is known to change density from time to time without treatment.

●Scleral involvement. Scleritis is less common than keratitis it may be missed due to lid edema and conjunctivitis. Scleritis is less common in children.

●Uveitis. Anterior uvea may be involved either as primary inflammatory process of or secondary to keratitis. Forty percent of eyes with herpes zoster develop non-granulomatous iridocyctitis. It may be mild with few kps or may have blood tinged hypopyon due to is ischemic necrosis of iris. atropthic patches on the iris that transilluminate and causes irregular semidilated pupil is also caused due to is ischemic necrosis of iris. Such atrophic patches are not seen in herpes simplex, which is also associated with keratouveitis. There may be chroiditis as well as chorioretinitis.

●Secondary Glaucoma is due to associated trabeculitis and clogging of trabecular meshwork by inflammatory cells. Chronic anterior uveitis and secondary glaucoma may predispose cataract formation.

(iii) Neurological lesions may cause encephalitis and cranial nerve palsy. Commonest being third nerve but any or all the extra ocular muscles may be affected due to involvement of their nerve supply. Optic neuritis is rare complications.

Post herpetic complication

Post herpatic neuralgia is a common feature, fortunately it is milder and short-lived in children. Children tolerate post herpetic neuralgia better than adults.

Neuro tropic keratitis, diminished corneal sensation start from very early phase of corneal involvement, generally recovers within a few months but in some cases it may last for years leading to neuro tropic keratitis requiring tarsorrhaphy.

Delayed lid Changes. There may be hypo pigmentation or hyper pigmentation with scaring of the skin, trichiasis is common, there may be notching of the lid margin. Loss of sensation may last for months.

Other changes. Mucus secreting conjunctivitis, corneal mucus plaque, neurotrophic perforation of cornea, recurrent episcleritis and scleritis, herpes oticus (Ramsay Hunt Syndrome)18 herpes of palate.

Management

Management consists of reducing pain, associated inflammation, control of virimia, prevention of secondary bacterial infection, management of uveokeratitis and its squeals.

Analgesia. Children tolerate pain better, however water soluble non-steroidal anti flamatory drugs helps to over come pain, edema and inflammation.

Antiviral. Antiviral drugs reduce lid inflammation pain and shorten duration of disease but does not affect keratitis. Its role in zoster uveitis is not established. It is claimed to reduce post herpetic neuralgia.

Systemic steroids. Definitely reduce pain inflammation and scaring. However it should be given along with systemic antiviral.

Local steroids are required for uveo keratitis, the eyes receiving local steroids should be stained frequently, least secondary bacterial infection gets an upper hand in an already anaesthetic cornea. Bland lotion or local antiviral ointment is prescribed for lid vesicle. Local antiviral drops or ointments are given along with local steroid in uveo keralitis.

(e) Herpes simplex25. Herpes simplex infection is very common infection caused by virus: Herpes hominis. There are two types of herpes simplex i.e. type one that involve the body above the waist and type two that cause lesions below the waist more commonly known as genital herpes, which is a sexually transmitted disease. Herpes simplex can occur as:

Neonatal infection, which is due to type two herpes simplex virus transmitted via birth canal of infected mother. If it is known that the mother has genital herpes simplex it is better to deliver the child by caesarean section. Neo natal herpes simplex may present as few vesicles on the lid or conjunctivitis in the neonate. Neonatal herpes simplex is life threatening condition when server.

Primary ocular infection: commonest age to get ocular infection is between six months to six years. The lids develop cluster of vesicles without predilection for any particular nerve. The vesicles may be bilateral in contrast to zoster, which is unilateral. The vesicles rapidly develop crust and disappear without scaring. It may be associated with acute folicular conjunctivitis. Half the patients develop harpes simplex keratitis in the form of fine or coarse epithelial keratitis most of the corneal lesions heal without scar. Sometimes it may progress to disciform keratits.

Recurrent ocular infection.After primary infection the virus travels to thetrigeminal ganglion and lies dormant unless a triger mechanisation stimulates the virus to reach the target organ after a latent period that varies from person to person. That may be few months to years Recurrent herpes simplex involves cornea and uvea and not the lids. Sever form of herpes simplex requires pediatric consultation for management.

2. Bacterial infection of the skin of the lid

Boil, cellulitis and abscess of lids. Skin of the lid, like any other exposed part of the body, is prone to get infected. It may start in the hair follicle and result as a boil or may be extensive to cause cellulitis. As the skin of the lid is thin, very lax and very vascular, lots of exudates and pus can accumulate under this and ultimately result in abscess formation. Commonest organism is staphylococcus. Haemophilus is an important cause of lid abscess in children. Lid abscess starts as diffuse swelling of the lid. Initially the lid is tense and red. It may obliterate the inter palpebral fissure. Gradually the skin becomes lax and fluctuation develops, pus may point at a dependent part. It is more common in hot seasons, cellulits lid and lid abscess are preseptal hence the globe is neither involved nor proptosis is produced. Differential diagnosis consists of cavernous sinus thrombosis, retrobulbar haemorrhage retro ocular mass, rhabdomyosarcoma, multiple styes, infected chalazion, pre-eruptive stage of herpes zoster.

Treatment consists of

1.Systemic broad-spectrum antibiotic preferably by injection.

2.Hot fomation.

3.Analgesic, anti-inflammatory drugs to reduce pain and inflammation.

4.If pus points or fluctuation develops, pus should be drained by a stab incision.

TUMOURS OF LID IN PAEDIATRIC AGE GROUP26,27,28

Tumours of the lid in all ages could be both congenital as well as acquired; they can be benign or malignant. All lid tumours are difficult to manage satisfactorily. They have been classified as

A.Those involving superficial layers of lid.

B.Those involving deeper layers of lid.

Other classification is according to structure of the lid (in all ages).

A.Epithelial

1.Benign

(a) Papilloma.

(b) Squamous cell hyperplasia. (c) Pseudo cancerous.

(d) Kerato acanthoma. (e) Seborrhic keratosis.

(f) Inverted follicular keratosis.

2.Precancerous

(a) Active keratosis

(b) Intra epithelial epithelioma.

(c) XERODERMA PIGMENTOSUM.

3.Malignant

(a) Basal cell carcinoma (b) Squamous carcinoma

B.Melanocytic

1.Benign

2.Malignant

C.Tumours arising from glands of lid

Meibomian cell carcinoma.

D.Vascular

1.CAPILLARY HAEMANGIOMA

2.NAEVUS FLAMMEUS

3.CAVERNOUS HAEMANGIOMA

4.STURGE-WEBER SYNDROME

E.Neural tumours : NEUROFIBROMATOSIS

F.Others

1.LYMPHANGIOMA

2.JUVENILE XATHOGRANULOMA

Condition in capital letters mentioned above are found under 15 years of age and continue to persist in later age group if not treated. All the other tumours are not seen in children. Some of them may be congenital others develop at puberty. Some are self-limiting. Except for neurofibroma and xeroderma pigmentosa none show malignant changes in children.

PHACOMATOSIS29,30

Phakomas are a group of tumours, which are congenital in nature, may be present at birth or manifest later. They have strong hereditary predisposition some of them may be sporadic. The sporadic cases can pass the gene to the next generation, they may be localised to the lid or may involve orbit, or globe, may have intra cranial extension or systemic involvement. All of them are Hamartomas i.e. tumours arising from the tissue components that are normally found at the involved site. They are seen in all races and equal in boys and girls. In fact they are congenital anomalies that result in tumour like malformations.

PHAKOMAS

A.Neurobibromatosis (von Reckling Hausen’s disease)

B.Encephalo facial angiomatosis (Sturge-Weber syndrome)

C.Angio matosis retinae (von Hippel-Lindus disease)

D.Tuberous sclerosis (Bournville’s disease)

E.Arteriovenous malformation of retina and brain (Wybern Mason syndrome)

F.Ataxia talengectasia (Louis Bar Syndrome)

G.Cavernous haemangioma of retina, skin brain (Rendu-Osler-Weber syndrome)

A. Neurofibromatosis

Neurofibromatosis is one of the most common congenital phacomas of the lids. It may be as common as one in every three thousand live births. It can be inherited as autosomal dominant trait. Either of the parents or both may show clinical evidence of disorder or may have subtle changes. Spontaneous mutation may lead to sporadic cases. These sporadic cases may pass the gene to the next generation. Genetically and clinically there are two types of neurofibroma i.e. type I and II commonly known as NF I and NF II.

NF II cause bilateral acoustic neuroma besides usual features of neurofibroma.

The following components may be seen alone or in various combinations, in neurofibromatosis

1.Skin and lids changes

2.Ocular changes other than lid changes

3.Involvement of nervous system

4.Visceral changes.

1. Skin and lid changes. The changes may be present at birth but generally develop by five years of age. They become marked at puberty. The changes may be seen any where on the body as :

(a) Caféaulait spot. These are slightly raised hyper pigmented spots of variable size i.e. pin point to large areas of pigmentation, without sensory loss, they may cross over the midline six hyper pigmented spots or more than five millimetres is significant.

(b) Fibroma molluscum. These are pedunculated mass of various sizes any where on the skin including lid.

(c) Plexiform neuro fibroma. These are most disfiguring growths that are due to involvement of multiple superficial nerves which on palpation feel like a bag of worms. It is most commonly seen in the lid, the whole of the lid is involved and thickened including conjunctiva, the inter palpebral fissure is obliterated initially the lid acquires a S shaped Curve gradually the lid margin develops a convexity down wards, the margin may over hang the lower lid. It may be large enough to be called elephantiasis neuro fibromatosa. There may be hypertrophy of the skin of the face on rare occasion the condition may be bilateral.

2. Ocular involvement. Both neuro ectodermal as well as mesodermal structure are affected. In fact all parts of the eye except the lens may be involved.

(a) Conjunctiva. Palpebral conjunctiva may be involved as part of involvement of the upper lid. The bulbar conjunctiva and lower formix may show small nodules between 1mm to 3mm. These are firm non-tender nodules. Otherwise multiple tortuous nerves may be visible on the conjunctiva.

(b) Cornea. Prominent corneal nerves may be visible on bio microscopy.

(c) Uvea. Commonly involved uveal tissue is iris that may have multiple hyper-pigmented nodules similar to nevi. Chloroid and ciliary body may show localised thickening of the nerve fibers.

(d) Optic nerve. Congenital opaque nerve fibers are more common in persons who have evidence of neurofibromatosis. One fifth of optic gliomas are seen in neurofibroma. Neurofibroma may extend into the chiasma and have positive x-ray finding of pre-chiasmal bony destruction.

(e) Retina is less commonly involved.

(f) Glaucoma is very common with neurofibroma of the lid. Exact mechanism of glaucoma is not well understood. It is termed as associated congenital glaucoma, which has all the features of buphthalmos that may be obscured by pseudo ptoisis. The pathology may be due to presence of neuro fibroma at the angle, fibro vascular closure of angle or due to forward displacement of lens iris diaphragm due to growth of ciliary body. Treatment of glaucoma in neurofibroma is unsatisfactory.

(g) Orbit. Bony faults in the sphenoid may lead to pulsating exophthalmos. Otherwise there may be proptosis due to retro bulbar mass with enlargement of orbit.

3. Nervous system involvement. Generally there are multiple neurofibromatous growths in the brain, meningies, cranial nerves specially bilateral acoustic neuroma. Spinal nerves may be involved with involvement of spinal cord. There may be changes in the bony spine. Even autonomic nervous system may be involved.

4. Visceral involvement. Any of the viscera can be involved in neurofibroma. Pheochromocytoma has been reported more commonly in neurofibroma. There may be gonadal changes.

Management

There is no known treatment to eradicate neurofibromatosis. Treatment is mostly symptomatic minor defects can be treated with plastic reconstruction. If possible amblyopia should

be treated. Glaucoma may be managed medically or surgically.

B. Encephalo trigeminal syndrome (Sturge-Weber syndrome)

This congenital hamartomous anomaly differs form other phacomatoses by not being hereditary. It is seen in all races, both boys and girls are equally affected. It is generally unilateral but in ten percent of cases it can be bilateral. It has following components.

1.Skin and lid involvement.

2.Ocular involvement.

3.Central nervous involvement.

4.Visceral and other involvement.

1.Skin involvement is present at birth as port wine stain. The facial angiomatosis that frequently involves both lids may be localised or have extensive involvement of not only facial skin but also the trunk. The size and shape of the cutaneous angioma does not change with age. Some times only dilated conjunctival vessels can be seen in the epsilateral side. Generally there is hemiatrophy of the face on the affected side. Sturge Weber syndrome does not cause ptosis which is a frequent feature of neurofibroma.

2.Ocular involvement. Besides lid, the globe too has angiomatous malformation mostly in the uvea that result into heterochromia of iris, choroidal haemangioma and associated glaucoma. The choroidal haemangioma is generally single, large and situated in the posterior pole on the temporal side of the disc. The haemanagiomas have diffuse out line, they are raised, have orange hue may be mistaken as amelanotic melanoma of choriod. The hemangioma due to its proximity to macula may cause cystoid macular edema. There may be non rhegmatogenous retinal detachment that may lead to secondary glaucoma in an eye that is already predisposed to glaucoma.

Glaucoma About 30% of eyes in Sturge Weber syndrome develop glaucoma by second year. A clinical feature of glaucoma is similar to congenital buphthalmos. Including enlargement of globe, large cornea, normal or deep A.C. Stretching of the globe may sub luxate the lens. The most widely accepted theory of raised intra ocular tension is increased episcleral pressure. Other probable causes are malformation of angle, peripheral anterior synechea. Sturge Weber syndrome glaucoma can be controlled by medical treatment, however if the medicines fail to reduce pressure below episcleral pressure these patients reqire surgical treatment. Trabeculectomy may help. Common complications of glaucoma surgery are intra operative hyphaema and large chroidal effusion in post-operative period.

3. Central nervous system involvement. Intra cranial haemangioma of the tapeto meninges on the same side as that of facial angioma is a constant feature. The angiomas and underlying cortex develop calcification that shows up as tram track appearance on x-ray of the skull. Intra cranial calcification develops in half of the cases by second year of life. The angioma cause Jacksonian epileptoform attack on the contra lateral side, which is difficult of treat medically. There may be contra lateral hemipersis. Various degree of mental retardation is also known to take place. There may be atrophy of ipsilateral cerebral and cerebellar cortex. Homonymous hemianopia is frequent.

C. Angiomatosis retina