Ординатура / Офтальмология / Английские материалы / Pediatric Opthalmology_Mukherjee_2005

Anterior type

The eye is microphthalmic the anterior chamber is shallow, undeveloped angle, iris has large visible blood vessels. Initially the lens is clear with visible retrolental white mass. The ciliary processes are pulled towards the mass and are elongated. The retrolental mass may contract and produce a rent in the posterior capsule leading to cataract formation that may cause swelling of the lens, pushing the iris lens diaphragm forward, narrowing the already faulty angle. The cortical matter may absorb leaving a membranous cataract. The retrolental mass may cause traction detachment.

Posterior type

This is less common than former. Generally cornea is smaller than normal, anterior chamber is either normal or deep, lens is clear and normal in size, there may be coloboma of lens or subluxation of lens. Commonest finding is in the posterior part of the globe resulting in a white opaque membrane or retinal fold extending from optic disc to periphery of the retina, it may extend in the retrolental space resulting in to a white reflex. Posterior variety may be bilateral.

Milder form of this anomaly is Bergmeister papillae and Mitten drof’s dot. Some times remnants may be seen in the mid vitreous at the level of Cloquet’s canal.

Management. There is no known preventive method or medical treatment. Surgical intervention in the form of lensectomy and vitrectomy if done early may have good vision . As the condition is unilateral, amblyopia is common and must be treated vigorously.

COATS DISEASE31,32,33,34

This is a non hereditary non inflammatory disorder of retinal vascularture. The exact pathogenesis of the condition is not known, the most probable cause is congenital malformation of retinal vessels i.e. telengiectasis that is associated with subretial exudation and non rehegmatiogenous retinal detachment. Condition is invariably seen in male children in first decade and in ninety percent cases it is unilateral. In bilateral cases the changes are minimal. In rare instances the condition is detected in infancy. The lesion starts in the retinal periphery as dilated vessels with capillary drop out, rarely there is vascular sheathing. Exudation is an important feature, the exudation is mostly seen in the posterior pole, sub retinal fluid contains cholesterol crystals but no calcium. The natural history of the condition is progression toward formation of white reflex in pupillary area behind the lens. The cause of white reflex in pupillary area is to exudation and retinal detachment. There may be secondary and complicated cataract. Neovascularisation of iris and angle is common feature in poorly managed cases that lead to neovascular glaucoma which may terminate in enucleation. Many blind eyes with glaucoma in Coat’s disease have been removed with mistaken diagnosis of retinoblasoma. The eyes may go into phithisis. In rare instances milder forms may regress spontaneously. There should be no difficulty in diagnosis of a case of Coat’s disease if media is clear. How ever in advanced cases ultra sonography and CT are two most helpful investigation. CT is single most valuable test as it delineates intra ocular morphology.

Differential diagnosis of coat’s disease consists of retinoblastoma, angiomatosis retinae (generally seen in third and four the decade) familial exudative vitreo retinopathy, retinopathy of prematurity and endopahthalmitis.

Management of Coat’s disease consists of early detection exclusion of other causes of white reflex in pupillary area confirmation of diagnosis. Best results are seen if the treatment is initiated before exudation starts. There is no medical treatment. Two modes of treatment that are followed are

1.Cryotherapy and

2.Laser phopcoagulation.

It takes eight to ten months for exudate to disappear after laser photocoagulation. Recurrence due to formation of new telengectasis is know to occur in poorly managed cases or years after initial successful treatment.

LARVAL GRANULOMA OF EYE - TOXOCARIASIS35,36,37 (See page 264)

This chronic systemic disorder is caused by parasite Toxocara canis a roundworm of dogs. There is doubt if toxocariasis can be caused by toxocara catis the round worm of cats. The child gets infected by the ova of the parasite that is passed in the faeces of the infected dog. The children who develop toxocariasis have history of pica. These children come in close contact with puppies that are infected at an early age, consuming food accidentally infected by ova. The commonest age for a child to get infected is Under two years. By two years the child gets symptoms of systemic involvement. The systemic involvement comprises of low grade fever, hepatosplenomegaly, pneumonitis convulsion and cutaneous erythema. The systemic involvement is known as Viscereal larval migrans which does not cause ocular involvement. Ocular involvement without intestinal involvement is most improbable. Visceral larval migrans causes leucocytosis, and eosinophilia Elisa test is positive in titre of 1:8. Positive Elisa helps to differential ocular toxocaria from retinalblastoma.

Ocular toxoariasis In contrast to age of systemic involvement which is two year, average age to develop ocular toxocariasis is 7.5 years. It is generally unilateral. There are three known forms of ocular toxocariasis. They are

A.Chronic endophthalmitis.

B.Posterior pole involvement

C.Peripheral granuloma.

Chronic endophthalmitis is most common and devastating. It is seen between 2 to 9 years of age. The ocular involvement is typically a combination of chronic uveitis with vitrities resulting in to loss of vision, leucokoria and strabismus. A combination uvea and vitrities leads to posterior cyclitic membrane, retinal detachment and complication cataract. Visual prognosis is very poor. These eyes are generally enucleated as they become painful blind eyes. The lingering fear of reinoblasoma is perhaps the most important motive to remove such eyes. Histopathology may reveal dead larva.

Posterior pole involvement This occurs in slightly older, children i.e. between 6 to 14 years and is unilateral leading to loss of vision. The granulomn is situated between the

macula and the disc or may be seen over the macula. It is a raised yellowish mass wider than optic nerve head. The mass may be surrounded by retinal edema, hard exudates or stress lines, retinal vessels may dip in the mass.

Peripheral granuloma : This is least vision threatening seen in children as well as adult upto third and fourth decade is again unilateral produces least inflammatory signs. Generally vision is unaffected however it may produce cystoid macular edema or retinal detachment. The lesion is a hemi spherical mass anterior to equator with vitreous band.

Management

The management comprises of prevention of infection, treatment of systemic involvement and treatment of ocular involvement.

Prevention

All domestic dogs and cat should be dewormed from time to time. Domesticated puppies should be toilet trained. This reduces chances of contamination of food. Pica in the child should be managed in consultation with pediatrician. Deworming of children has doubtful role.

Treatment of visceral larval migrans is systemic administration of anti helmenthic drugs like thiabendazole, albendazol, Dead worm may cause systemic allergic reaction.

Treatment of ocular involvement:

There is no medical treatment that can treat larval granuloma. Curiously by the time ocular involvement takes place systemic signs and symptoms subside with normal WBC count, however Elisa remains positive. Local treatment comprises management of uvetitis with cycloplegic and local steroid mostly peri-ocular depot injection.

Surgical treatment Parsplana vitrectomy removes larval antigen cuts posterior cyclitic membrane and releases traction vitreo retinal bands that may cause traction or rhegmatogenous retinal detachment. Retinal detachment in treated by sclera buckling. Over all visual results are poor.

INTRA OCULAR GROWTH OTHER THAN RETINOBLASTOMA THAT CAUSE WHITE REFLEX IN PUPILLARY AREA

They can be

A.Malignant

1.Meduloepithelioma

2.Leukaemia

3.Glioneuroma

B.Benign (Hamartomas)

1.Astrocytoma

2.Haemangiomas

(a) Capillary hemangioma (b) Cavernous hemangioma

C.Phacomas

A. Malignant

1.Meduloepithilioma See page 284.

2.Leukemia40

Ocular signs are never presenting features of leukaemia. Intraocular involvement is late. It is generally seen as recurrence after initial remission and always have poor prognosis. All the intra ocular structures that have blood vessels in them i.e. uvea, retina and optic disc may be involved. The vitreous may get seeded by leukaemia infiltration. A pseudohypopyon may also develop. Infiltration of optic nerve is always associated with vision loss which is not recovered following apparent cure by radiation or chemotherapy. Infiltration of iris and ciliary body may be mistaken as anterior uveitis specially with pseudohypopyon. There may be localised or diffuse infiltration of choroid. As most of the children with leukaemia are immune compromised either due to malignancy or chemophathapy or both it is difficult to state if the uveal lesion is due to malignant infiltration or due to opportunistic organism. The controversy can be settled by fine needle biopsy of the lesion.

The fundus lesions of the retina may be due to

1.Direct infiltration of retina or optic nerve or

2.Due to associated anaemia and thrombo cytopenia.

The hemato logical retinal lesions are superficial linear or flame shaped haemorrhages. Superficial haemorrhage with white centres are due to anaemia and thrombo cytopenia.

Other lesions may be non rhegmatogenous retinal detachment, retinal pigment epithelial detachment.

Management. Treatment is essentially directed to systemic involvement, local external beam radiation to eye results in regression but improvement of vision is not always predictable.

3. Glioneuroma. This is very rare tumour, arises from anterior lip of the optic cup, unilateral involves anterior sigment, produces white reflex but does not involve posterior sigments.

B. Benign (Hamartomas)

1.Retinal astrocytoma41,42,43. Retinal astrocytoma is a rare hamartoma of the retina seen in children they are non invasive and non malignant, generally stationary they may be solitary or multicentric. Unilateral lesions are frequent. Commonest lesion is a white superficial, raised lesion little larger than disc. On fluroscein angiography it has slow filling, late staining. It does not require any treatment. The condition is most commonly seen is tuberous sclerosis that has multiple systemic involvements.

2.Retinal haemangiomass41,42,43,44

(a) Retinal capillary haemangioma

These rare vascular tumours that share autosomal dominant trait with retinoblastoma. They are tumours of aduldt hood may become apparent in childhood. They also arise from sensory retina and are multicentric may be bilateral. The similarity with retinoblastoma ends here. The difference are: their colour, afferent and efferent vessels, sub retinal and inter retinal exudale. They never metastasise.

These growths are haemartomas that may be seen on the retina as well as optic nerve head. They may be confined to eye only or may have systemic involvement in twenty five percent cases and is known as vonHippel’s disease In the intra ocular growth may be uniocular

or bilateral may be single growth or there may be more than one growth of different size and duration multiple growths have poorer visual out come.

The single haemangioma of the retina is discovered on routine retinoscopy and fundus examination before retinal detachment sets in. The detached retina may produce a white reflex in pupillary area and draw attention.

The characteristics of retinal capillary haemangioma are : Spherical , raised, bright red growth with afferent vesseles and efferent vessles. The growth generally are one to two disc diameter in size. Both the feeding and draining vessles are dilated and tortous. Larger growths have larger vessels.

There may be inter or sub retinal exudates with macular star formation. These exudates may give a pale reflex during retinoscopy. The exudate cause retinal detachment that may be partial or total. Retinal detachment results in white reflex in pupillary area. Secondary glaucoma and iris neovascularisation are some of the complications.

Diagnosis is confirmed by : Indirect ophthalmoscopy, Bscan, CT. MRI and fundus fluorescing angio graphy.

Fundus fluorescing angio graphy is most important diagnostic procedure in absence of total detachment. Angiography shows rapid filling of the entire vessels and the tumour, hyper fluorescence of the growth, rapid filling of the draining channels. If is leak from the tumour it may colour the vitreous.

Management small peripheral tumours need not be treated but observed periodically for enlargement in size, development of internal and subretinal exudates, retinal detachment, if any of above manifest, the lesion requires treatment. The best mode of treatment is by photo coagulation, peripheral lesion many be treated by cryotherapy. Diathermy has been given up in favour of former two.

There is difference of opinion if

(i) the growth along with its feeding vessels45 should be treated

(ii) only the vessels be obliterated43 and hope that the growth will shrink (iii) the tumour only should be treated42.

The best results are claimed to be by Argon laser with large spot size, longer duration and low intensity. The tumour should not be treated in one session. It should be treated by several sittings spread over months. The retina around the dilated vessels and angioma may require scattered photo coagulation to prevent exudation.

(b) Cavernous hemangioma of the retina41, 44

This is a rare congenital malformation of retinal vasculature , this is a hamartoma, non progressive, non malignant growth may have positive family history generally uniocular and single. Situated behind the equator either in the retina or on the optic nerve head. It is slowly progressive generally symptom less, diagnosed on routine fundus examination in late teens or early third decade.

The retinal growths are inter retinal in nature with slight elevation of the ritual surface. The single growth is in fact a cluster of many smaller thin walled sacular micro aneurysm. The over all size of the growth varies between 2-4 mm in diameter there may be few micro aneurismal

dilatation in the surrounding area. The surface of the retina invariably shows moderate glial proliferation over the growth that may be confused with superficial exudate. There is no arterial dilatation in contrast to capillary haemangioma.

Fluroscein angiograpthy is diagnostic, there is slow filling of the lesion, each saccule accumulates fluorescing individually and remains fluorescent for long time.

As the lesion is non progressive and does not have symptoms it does not require any treatment.

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