Ординатура / Офтальмология / Английские материалы / Pediatric Opthalmology_Mukherjee_2005

and young adults. There are two types i.e. (1) erythema multiform minor (2) Erythema multi forme major (Stevens Johnson syndrome) The latter is not only bilateral sight threatening, it can often be fatal. Etiological factors that can culminate into this troublesome disease are :

1.Drugs. Many drugs that are routinely used for day to day management of various symptoms can cause Stevens Johnson syndrome. Top of the list is taken by sulpha drugs including acetazolamide others are penicillin’s, phenylbutazone barbiturates, salicylates.

2.Infection. (i) Recurrent infection by herpes simplex type I and II. (ii) Respiratory infection with mycoplasma pneumonae.

3.Idiopathic.

The disease has three stages :

1. Prodromal 2. Vesciculo bullus stage 3. Complication.

The pro dromal stage consist of malaise, sore throat, fever, joint pain and swelling.

In second stage. The muco cutaneous involvement consists of erythematous macules on dorsal hand and feet, exterior surface of forearm and legs. The lesion soon becomes papular, which become bullus. Any part of the mucous membrane may be involved. The lesions are generally symmetrical. This stage lasts for seven to fifteen days. Healing starts within a few days and heals completely. Recurrence has been reported if the child is exposed to same precipitating factors.

The ocular lesions

Lesions of the eyes seen in 50% of cases. It is bilateral, generally symmetrical, starts as muco purulent conjunctivitis that may become purulent. There may be vescicle formation. Due to vasculitis part of the conjunctiva gets infarcted. There may be formation of pseudo membrane or true membrane. Shedding of these membranes leave raw areas in the conjunctiva. When two such raw areas come in contact with each other adhesion develop. That result in formation of symblepharon. Shrinking of conjunctiva leads to trichiasis, entropion, obliteration of lacrimal ducts, keratanisation.

Complications. Erythema multi form minor may pass off without any complication. However Steven’s Johnson has far reaching vision threatening complication like xerosis of conjunctiva and cornea, keratanisation of cornea, vascularisation and opacification of cornea.

Management. There is no effective treatment of ocular involvement. Local steroid may minimise vasculitis and prevent conjunctival infraction which in turn reduces formation of pseudo membrane. Attempts should be made to prevent adhesion between two raw surfaces by instilling bland lotion systemic steroid started in early stage are life saving and minimise scarring.

Conjunctivitis of unknown causes in children :

1. Folliculosis. This is not a true conjunctivitis. It is a benign, bilateral, non-infective follicular hypertrophy in children. The follicles are small, seen mostly in lower fornix, tarsal conjunctiva is not much involved. Cornea is never involved. There is hardly any discharge. There is no itching. It is to be differentiated from other causes of follicles and papillae formation

like chronic follicular conjunctivitis, trachoma and spring catarrh. The condition does not require any treatment except reassurance to the parents. Chronic follicular conjunctivitis is an infective conjunctivitis of under nourished children. It is a bilateral condition. The follicles are numerous both upper and lower tarsal conjunctiva are involved. There may be minimal discharge.

2.Iatrogenic conjunctivitis (Drug induced). It is a non specific conjunctivitis following prolonged administration of host of commonly used local drops that include - pilocarpine, atropine, home atropine, idoxuridine, neomycin, gentamycin sulpha cetamide. It is generally associated with dermatitis of the lid margin and excoriation of the outer angle. There are some follicles which may be associated with itching and confused with non drug induced allergic conjunctivitis. Treatment consists of stopping the offending drug and replacement by nontoxic drug. Weak solution of steroid. In spite of treatment follicles may persist for few days to weeks.

3.Ligneous conjunctivitis. This is rare type of conjunctivitis seen in children may be seen soon after birth or develop any time even in adults. It is more common in girls, is generally bilateral but asymmetrical. Most important feature is formation of membrane on the tarsal conjunctiva that gives it a hard feeling. There may be formation of granuloma on the tarsal conjunctiva. Exact cause of this condition is not known. However chronic infective process and hypersensitivity are two important factors. There is no satisfactory treatment. Initially the condition may be confused with membranous or pseudo membranous conjunctivitis and StevensJohnson syndrome.

Granulomas of conjunctiva

Conjunctival granuloma are seen in chronically inflamed conjunctiva. Common cause in children are :

Rhinosporidiosis52, 53 (Oculo sporidiosis)

Rhinosporidiosis is caused by fungus Rhinosporidium Seebri. Ocular involvement is seen mostly in children in endemic areas. The disease has wide distribution mostly in tropics with moderately to heavy annual rainfall and high humidity. Almost all mucous membranes of the body are known to be involved including conjunctiva and lacrimal sac; lesions of skin and bone have also been reported. The spores of the organism get implanted in the mucous membrane and develop into sporangia, a mature sporangium may contain as many as 16,000 spores.

Ocular involvement is said to be primary54 when there is no other evidence of the disease in any part of the body except eye and its adnexa. This is also known as oculosporidiosis. In secondary rhinosporidiosis of the eye the disease spreads from the nasopharynx to the sac.

Ocular structures involved in rhinosporidiosis are - conjunctiva, lacrimal sac and sclera. Combined involvement of sac and conjunctiva is very rare. Conjunctival involvement is generally primary.

Typical conjunctival lesion is a gradually developing painless granuloma in the conjunctiva. Commonly involved are fornices and tarsal conjunctiva. Bulbar conjunctiva is rarely involved. The lesion is more common among boys between 5 to 15 years, who have habit of taking bath in a pool shared by cattle which are invariably infected.

The growths in the tarsal conjunctiva are flat and sessile, those at limbus or bulbar conjunctiva are round. While those arising from fornices have long thin pedicle with finger like projection. All the lesions are bright red in colour, have rough surface dotted with samolina (sujee) like white dots that represent sporangia. The lesions bleed on slight trauma. Generally there is only one granuloma in an eye. Bilateral conjunctival involvement is rare. The size of the growth varies from few millimetres to few centimetres.

Symptoms. Main symptoms are bleeding in the conjunctiva and visible red mass. If the growth hangs over the cornea, it may cause lacrimation. The lesions are non tender and painless. They are fragile, bleed on slight manipulation like everting the lid.

Diagnosis. Diagnosis of conjunctival rhinosporidiosis is simple in endemic areas. Common lesions that form differential diagnosis are burst chalazion, foreign body granuloma, haemangioma, papilloma. However reverse is also true i.e. above conditions may be mistaken as rhinosporidiosis in endemic area.

Diagnosis is best confirmed by histopathology of the excised tissue. Other useful diagnostic method is to examine small piece of tissue dipped in saline under high power that show sporangia. Sometimes free spores are demonstrated in the tears of affected child.

Involvement of lacrimal sac in rhinosporidiosis52,53,54 is more common than conjunctival involvement. The sac can be involved either as primary lesion i.e. without any involvement of nasopharynx or as a secondary spread from nose and throat. Involvement of the sac is in the form of chronic dacryocystitis where the growth arises from the mucosa of the sac and fills the sac to distend it, that presents as a diffuse non tender swelling on the medial side of the eye mostly below the medial palpebral ligament, may spread under the lower lid. The swelling is compressible but non-reducible, does not change in size on coughing or sneezing. The skin over the swelling has orange peel appearance. Temperature over the swelling is not raised. Regurgitation test is negative, complete block of nasolacrimal duct is rare. Erosion of surrounding bone is common in long standing cases. If sac is secondarily infected it presents as cellulitis. This generally happens following syringing. Bilateral involvement is rare. Exact mechanism of sac involvement is not clear specially as a primary lesion. Partial congenital obstruction of nasolacrimal duct, chicken pox or measles are contributory factors in endemic area. A narrow or obstructed nasolacrimal duct due to any of the above contributory factors allows the spore to settle down in the sac where it reaches via puncta and canaliculi. Once the spore reaches the sac it forms a nidus over which the growths develops. (See page 93-94 as well)

Scleral involvement is most rare. It is always associated with a conjunctival growth that hangs over the scleral lesion which is in the form of a staphyloma. The staphyloma is located anterior to the equator. Occasionally it is visible on indirect ophthalmoscope.

Management. There is no known drug that is effective against rhinosporidiosis. Surgery is the treatment of choice. The conjunctival lesions can be removed under topical anaesthesia. If needed short term general anaesthesia may be administered. The growth is

grasped with flat, blunt forceps and pulled away from the conjunctiva or a ligature may be tied round the pedicle. The pedicle is cut with sharp scissors. Even without ligature the bleeding can be stopped by firm pressure. Small growth can be treated with cryo. Surprisingly there is no recurrence in conjunctiva once the growth has been removed surgically even if the child lives in the same environment.

Management of rhinosporidiosis of sac is less satisfactory. It is essentially complete removal by surgery. Unless meticulous care is taken to remove every bit of tissue involved recurrence is the rule.

While removing the sac the nasal growth should also be taken care of simultaneously. The growth of the sac can also be removed by nasal endoscopic surgery.

In scleral involvement the conjunctival growth is removed by usual method. For scleral staphyloma best results are obtained by applying cryo all round the staphyloma. The scleral bulge is indented with scleral explant of suitable size. Intraocular pressure of the eye is kept low either by paracentesis or IV mannitol and maintained low by oral diamox and betablocker drops.

Tumours of conjunctiva

Tumours of conjunctiva in children are rare. They are generally - Hamartomas, choriostomas, haemangiomas and xeroderma pigmentosa.

Hamartomas that have congenital involvement are : Neuro fibromatosis (von Reckling Hausen’s disease and Enccphalo trigeminal angiomatosis (Sturge Weber Syndrome).

Conjunctival involvement in neuro fibromatosis is less common than in Sturge Weber syndrome. Conjunctiva is never involved alone, it is always associated with lid involvement and many have associated systemic involvement.

Choriostomas. These are fairly common congenital benign tumours. There are two types of choriostoma : 1. Dermoid, 2. Dermolipoma.

Haemangiomas of conjunctiva. These benign tumours are not true tumours. They may develop alone in the conjunctiva or may be seen with haemangioma of lids. (See page 61-62)

Cysts of the conjunctiva

Various types of conjunctival cysts are : traumatic, retention, lymphatic or developmental. They can be parasitic in nature i.e. cysticercosis, echynoccosis, coenurosis.

It is estimated that non traumatic cysts are seen in 43% of eyes mostly in fornices. They vary from pin head size to large enough to fill the fornix. Common causes of cysts in conjunctiva are : trauma, congenital anomaly, parasitic infection and adhesion between two layers of conjunctiva.

Cysts can be grouped into two broad groups i.e. traumatic and non-traumatic.

Traumatic cysts are mostly epithelial implantation cyst due to trapping of epithelium under the mucosa. They are seen following wounds of conjunctiva, squint surgery, retained conjunctival foreign body.

Non-traumatic cysts are : Dermoids, parasitic cyst, retention cysts and lymphatic

cysts.

Retention cysts

These generally develop in glands of Krause following chronic inflammation or trauma. The ducts of the glands are obstructed resulting in formation of cysts. They are generally seen in the upper part of the conjunctiva.

Symptoms depends on size and position of the cyst. The child may not be aware of small cysts. They may be discovered during routine examination of the eye. Parents may become aware of large cysts. There may be multiple translucent cysts. A large cyst may cause astigmatism or may cause a diffuse swelling in the lid.

Management. Small cysts do not require any treatment. Large cysts are removed for cosmetic purpose and to reduce astigmatism. Aim should be to remove the cyst without rupture.

Parasitic conjunctivitis

Parasitic infestation of conjunctiva is far less common than bacterial or viral infection. Parasites range from protozoa to helminth. Rarely arthropods may invade the conjunctiva. Parasitic infection of conjunctiva is generally seen in tropical and sub-tropical regions. Some of the parasitic conjunctivitis are seen only in endemic areas (river blindness). Some of parasitic conjunctivitis are very rare and may not be seen in children.

Common parasitic conjunctivitis seen in children are : Cysticercosis, echinococcosis, coneurosis, thelaziasis, onchocerciasis. (See Chapter Ocular Manifestation of Systemic Diseases)

Cysticercosis56, 59 is a systemic parasitic infection. About forty percent of persons infested by the parasite, have ocular involvement. The ocular involvement can be (1) less vision threatening, extra ocular manifestation in the form of conjunctival or orbital cyst. (2) More severe and blinding intraocular involvement.

Cysticercosis is caused by Cysticercus cellulose the larval stage of pork worm taenia solium.

Humans harbour the parasite in two forms i.e. (1) Definite host form of adult tapeworm or (2) Intermediate host, harbouring larval stage. Pig is the usual and natural intermediate host. Humans get infected by consuming under cooked pork that contains viable cysticersis which mature in human intestine, and become adult worm. Other method of infection is consuming food and water contaminated with ova of the parasite. Latter mode of infection is more common than in former. This is the mode of infection in strictly vegetarians and those persons who shun pork due to religious reasons.

The embryo reaches the eye via ophthalmic artery. Left eye is more frequently effected than right eye. Most of conjunctival lesions are located near the medial canthus. However right eye is not immune. Bilateral and multiple unilateral involvement are rare. Involvement of left eye is so frequent that all conjunctival cystic lesions in the left eye should be considered to be cysticercosis unless proved otherwise.57 The swellings are of variable size generally fixed to episcleral tissue or the sclera. Initially the conjunctiva moves freely over the translucent oval swelling. The cyst is painless. It may migrate along the extra ocular muscle

from behind to be visible on the conjunctiva and extruded56, 60. The cyst may occasionally extrude through the conjunctiva. If the cyst ruptures it causes localised conjunctivitis.

Differential diagnosis consist of other parasitic cyst. Diagnosis is confirmed by histopathology after removal. However increased eosinophils in differential count and presence of ova in stool is highly suggestive.

Management consists of 1. Prophylaxis, 2. Surgical removal of the cyst.

Prophylaxis consists of proper cleanliness and food hygiene. Health authorities should see that infected pigs are not slaughtered for consumption. Pork should be well cooked, raw vegetables should be avoided least they are contaminated. Children should be dewormed regularly either by Mebendazol 100 mg BD for three days or albendazol 400 mg single dose at least once a year. Role of antihelminth as cure of conjunctival cyst is not confirmed.

Surgical treatment consists of complete removal of cyst without rupture. Rupture causes severe conjunctivitis. Following successful removal, there is no recurrence.

Other parts of the eye that may be invaded by cysticercosis are lids, orbit, retina, vitreous, and anterior chamber.

Intra ocular involvement results when the embryo reaches the interior of the eye via posterior ciliary artery. The embryo develops in to an expanding cyst in the choroid that lifts the retina to produce exudative retinal detachment. Perforation of the retina by cysticareus results a free floating cyst in the vitreous.

The cyst on rupture produces larval endophthalmitis both large intra vitreal cyst and endophthalmitis cause white reflex in pupillary area. Death of the larvae also causes endophthalmitis severe enough to cause loss of eye.

Echinococcosis62

Echinococcosis is systemic parasitic infection caused by larva of T. Echinococci a helminth found in intestine of dogs. Man and other animals can get infected from affected dogs. Human is an intermediate host who gets infected following ingestion of contaminated food. No part of the body is immune to echinoccosis however, liver and lungs are most frequent sites.

Ocular involvement consist of—Cyst in sub-retinal space, vitreous and anterior chamber. Conjunctival cyst formation is less common than seen in cysticercosis. Orbital cysts cause proptosis, chemosis of conjunctiva and lid. Cyst when present in the conjunctiva may be attached to one of the recti.

Management consists of prevention of infection in children. This consists of regular de-worming of the pet and the child. Avoiding green raw vegetables. Definitive treatment is removal of the cyst surgically without rupture.

Coenurosis

This is systemic and ocular involvement of an intermediate host by larval stage of dog tapeworm multiceps. Humans act rarely as intermediate host which are generally sheep and goats. Children get infected directly by ova of the parasite from soil, vegetable or fur of dogs deposited in the conjunctival sac and develop cyst similar to cysticercosis. Adults get infected by ingestion of contaminated food.

All the parts of the eye can be infected. In children subconjunctival cyst is most common presentation. In adults it can involve sub retinal space, vitreous or orbit. There is no known medical treatment, surgical removal is best option.

Thelaziasis63, 64

Thelaziasis is a nematode infection of ocular tissue mostly in animals. There are about ten species of thelazia that have been reported to infect conjunctiva. Common being thelazia callipaeda and thelazia californiensis. Involvement of human eye is rare. In humans mostly outer eye is involved. Parts involved are conjunctiva, lid and lacrimal passage. The nematode finds its way to conjunctiva through a arthropod most probably a fly that lays eggs in the conjunctiva which hatches there to complete its life cycle and produces conjunctivitis, hyperemia and chemosis of conjunctiva. Unlike other nematodes thelazia does not cause cyst in the ocular tissue. Live worm can be found in the conjunctival sac.

Intra ocular involvement have been reported. Intraocular penetration may result through an abraded cornea or sclera. Live worm have been removed from anterior chamber.63

Management of conjunctival involvement is manual removal of worm from conjunctival sac after it has been immobilised by local anaesthetic agent. Worm from AC should be removed surgically. Associated uveitis is treated by usual local cycloplegic and steroid. When surgical removal is not possible, Diethyl carbamazine 50 mg three times for 14 days may prove effective.

Ocular myiasis

Ocular myiasis or larval conjunctivitis is caused by deposition of eggs of some of the flies. Three species of flies have been identified to cause myiasis.61 Even common housefly can cause ocular myiasis. The flies are attracted to the conjunctival and nasal discharge of the filthy child. The flies lay eggs in the conjunctiva where the eggs hatch as larvae (maggots). It is the larvae that are the cause of conjunctival inflammation. The maggot infestation is seen mostly in emaciated children. There are two types of conjunctival infection by larvae :

1.Larval conjunctivitis. There is redness, itching, burning and lacrimation. On examination small elongated white larvae are seen crawling in the conjunctival sac. There may be associated marginal keratitis.

2.Destructive myiasis. The larvae penetrate deep under the conjunctiva and skin into the orbit. Occasionally the bones may be eaten away and meninges exposed. Unless the larvae are eradicated promptly they may cause death.

Management consists of maintaining proper cleanliness in emaciated child. Use of mosquito net to prevent fly from depositing eggs on the conjunctiva. Attempt should be made to remove the larvae manually as soon as detected.

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