Ординатура / Офтальмология / Английские материалы / Pediatric Opthalmology_Mukherjee_2005

Angiomatosis of retina is a hereditary hamartomatous disorder of retinal vasculature and optic nerve. It is associated with haemangioblastoma of central nervous system and viscera. In the eye it involves retina mostly and optic nerve head less commonly. It is a autosomal dominant disorder, other parts of the globe orbit or adnexa are not involved.

Ocular Involvement. Retinal involvement starts first, it is generally diagnosed in first decade either due to diminished vision or on routine examination. The angiomas are generally multiple, of different sizes. There are mainly three locations where these angiomas are seen, peripheral, peripapillary and papillary. The angiomas are progressive and keep on increasing in size and number. Even after treatment recurrence in the same quadrant is possible. The peripheral lesion arise as endophytum i.e. they arise from inner retinal layer while peripapillary lesions arise from outer retinal layers and are diffuse. The early angiomas are small but have evidence of arterio venous shunt on fluorescein angiography. Larger angiomas are round elevated bright red mass that have a feeding artery and draining vein both are dilated, tortuous and of same caliber. The disc angiomas are similar but do not develop arterio venous shunt. As the angiomas grow. sub retinal fluid and exudates accumulate round the lesion. The exudate may accumulate under the macula forming a macular star and loss of central vision. The endophytic tumours may bleed leading to vitreous band formation and traction detachment. The optic nerve angioimas when situated in the substance of the optic nerve head may present as disc swelling. Recurrent haemorrhage, rhegmatogenous retinal detachment may lead to secondary glaucoma.

Systemic involvement. Central nervous system involvement occurs after ocular symptoms. Almost all parts of the brainand spinal cord may develop haemangioblastomas. Similar growth can be seen in liver, kidney, and pancreas. All children with suspected angiomatosis retinae should undergo complete systemic examination including ultra sonography C.T. scan and MRI.

Management

Treatment consists of obliterating the retinal angiomas as early as possible. This can be achieved by argon laser, cryo or penetrating diathermy. Argon laser is better for posterior polar lesion while cryo is more effective in peripheral lesions. Argon laser therapy should have large spot, low intensity and long duration; multiple treatment sessions may be required.

D. Tuberous sclerosis (Bournville’s disease)

This is a congenital hamartoma that differs from neurofibromatosis and Sturge Weber syndrome. It does not involve any ocular structure except retina and optic nerve.

It is a fatal condition. The children do not live beyond second decade. It is a hereditary condition all races and both sexes are equally affected. The ocular lesions are bilateral may be a symmetrical the components are :

1.Cutaneous,

2.Cerebral,

3.Ocular,

4.Visceral.

The Cutaneous involvement. The Cutaneous involvement is not present at birth but

becomes visible between two to five years of age as ash-leaf area of depigmented patches on the trunk and limbs best seen by Woods ultra violet lamp. This is followed by so called adenoma sebaceum, that does not involve sebaceous glands, are in fact angiofibromas histologically. Adenoma sebaceum are distributed on the face as wings of butterfly on either side of the mid line beyond naso labial fold and nose they are multiple small redish brown nodules. The nodules increase size at puberty, dilated vessels are common over the nodules.

Central nervous involvement. Central nervous involvement is wide spread in the brain. It may be cerebral; cerebellar or medullary may be seen in ventricles. It may involve the spinal cord. In fact any part of the brain and spinal cord may be involved. They may cause raised intracranial tension, convulsion and mental retardation. The lesions get calcified and are visible on x-ray.

Ocular involvement. Ocular involvement consists of hamartoma of retina that may be single or multiple generally raised and globular or may be flat. Optic nerve changes are not hemartomatous they are due to drusen that is very common with tuberous sclerosis.

Visceral Involvement

Rounded hamartomas can be seen in almost all organs including heart, which may be the cause of death. Fibromas may be seen under or by the side of the nails. There is no known treatment for this condition.

PRE CANCEROUS EPITHELIAL LID TUMOUR IN CHILDREN

Xerodermapigmentosum31,32,35

Xeroderma pigmentosum is relatively rare precancerous disorder of ectodermal and mesodermal structure with prominent extra ocular involvement. Intraocular structures are not involved.

It is an autosomal recessive disease where both the sexes are involved equally. It may affect siblings. The disease is first noticed by second year of life when the child shows signs of intolerance to sunlight. It is due to hypersensitivity to ultraviolet light between 320 nm to 340 nm. The cutaneous signs are : Erythema in all exposed parts of the skin. The skin lesion undergo a chain of changes i.e. erythema bullae formation, development of fine vessels on skin lesions, atrophy of the lid, benign multiple hyperpigmented growth ending in development of various types of diffuse malignant growths that may cause death in adolescence. Various types of malignanies noted are epithelioma, basal cell carcinoma, even malignant melanoma. Ocular structures commonly involved are: lids, conjunctiva and cornea.

Lids contain various stages of growth ranging from erythema, hyperpigmented nodules to malignant growth. There is atrophy of skin of the lid; ectropion of lower lid is common.

The Conjunctival involvements include nodular growths near limbus, pigmented patches, papillomas dryness of conjunctiva, formation of pterygia and symblepharon. Both eyes are involved, bulbar conjunctiva in the inter palpebral fissure is first to be effected as it is exposed to ultra violet rays more than other parts of the conjunctiva.

Corneal involvement is early and may be first to draw attention of the parents due to

photophobia, lacrimation and redness of the eyes. Corneal involvement may start as early as two years in severe cases, or may be delayed by another two to three years. Whole of the cornea may be involved. Corneal involvement starts as superficial vascularisation of lower part causing intense lacrimation and photophobia. The vessels regress leaving a permanent opacity. Gradually whole of the cornea may become opaque. Other form of corneal involvement is extension of conjunctival neoplasm over the cornea.

Management of xerodermapigmentosum is almost hopeless due to high mortality. There is no way to prevent progress of disease. Treatment is palliative aimed to give maximal relief from sensitivity to ultra violet rays. Parents are instructed to keep the children away from direct sun-rays. All the parts of the body are covered by long sleeved shirts and trousers, cover the feet by fully covered shoes, keep long hair, use sun glasses with side protection. Skin protective lotion containing zinc oxide, titanium oxide and para-amino benzoic acid are smeared over the exposed parts. Malignant growths require separate, specialised treatment.

HAEMANGIOMAS OF THE LID

Haemangiomas of the lid are commonest benign tumours of the lid. They are of three types :

A.Capillary haemangioma

B.Cavernous haemangioma

C.Nevusflammeus

A. Capillary Haemanagioma34,35

Capillary haemangiomas are commonest types of congenital tumours of the lid, they are seen one in every 200 live births. They may be present at birth, as a red spot on the lid and extend on the forehead generally in the mid line the lesions become prominent when the child cries. The growths are more common on upper lid on the medial side than lower lid. They have a typical evolution. They become manifest most commonly after first month and keep on progressing for six months to one year and then start regressing by third year. Only few will remain visible after five years, they appear as strawberries of irregular raised mass that do not have formed capsule on histology. As most of the tumours regress spontaneously, so they do not require any treatment except reassurance to the parents who themselves may had such lesions in their infancy. If the growth is large it may cause mechanical ptosis producing deprivation amblyopia. The growth by itself can cause astigmatism, anisometropia and result in amblyopia.

If the growth is large it may require treatment.

Medical treatment consists of oral prednisone 2mg / kg / day, better given in single dose alternate day, most children show significant regression within ten days, then the dose can be tapered. However in some cases reduction in dose may halt regression. Whenever the child is on systemic steroid its benefit should be weighed against side effects. Injection of depot steroid in the lesion is claimed to be as effective as oral treatment but less toxic.

Other modes of management

Laser. various types of lasers i.e. argon NdYAG and carbon dioxide laser have been tried without uniform result.

Irradiation. Ortho voltage radiation of 200 rads may regress the growth in two weeks. However proper protective shields should be used during radiation to protect the globe and periorbital tissue.

Surgery. Various types of surgical procedures are available but may not be very effective.

B. Cavernous Haemangioma

Cavernous haemangiomas are far less common than capillary haemangiomas in children. They are mostly orbital, single and well capsulated, are more common in females, that manifest at later years of adolescence. They do not have prominent arterial supply which is present in capillary haemangioma. It too has tendency for spontaneous regression. It may get calcified.

C. Nevusflammeus

This is flat cutaneous lesion of the lid that does not blench on pressure. It has a purple blue hue hence the name port wine stain. It may be seen separately or in association with Sturge Weber syndrome. It does not require any treatment except cosmetic in fair skinned children when it is too large.

JUVENILE XANTHOGRANULOMA

This dermatological condition of infancy and childhood is of importance not for its involvement of lid or skin but it causes spontaneous uniocular recurrent hyphaema due to involvement of iris. The skin lesions are multiple yellowish, benign tumours that show spontaneous regression. Intra ocular lesions are seen in iris and ciliary body, as pigmented single lesion or may have a salmon colour. Hyphaema is self-limiting but may cause secondary glaucoma.

THE NEUROLOGICAL DISORDERS OF LID

The two most common neurological disorders of lids in children are

A.Neurological ptosis See Chapter 4, Page 73-76.

B.Lagophthalmos

B. Lagophthalmos36

Inability to close the lids fully is called lagophthalmos, in an attempt to close the lids the eye ball rolls up due to protective mechanism of Bell’s phenomenon. Lagophthalmos can be due to

1.Neurogenic cause or

2.Mechanical cause.

1. Neurogenic. Neurogenic cause of lagophthalmos is paralysis of motor supply to orbicularis oculi. The lesion is mostly peripheral in the facial canal or its peripheral branches.

Supranuclear lesion of seventh nerve produces total contralateral weakness of lower two third of the face with slight weakness of orbicularis. Weakness of orbicularis in supra nuclear seventh nerve palsy may be so mild as to go unnoticed. It does not require tarsorrhaphy, which is frequently required for peripheral seventh nerve lesion.

Neurogenic lagophthalmos is generally unilateral but can be bilateral, Facial diplegia37 is almost always central in origin, common cause in children are head injury, Mobius syndrome that consists of bilateral sixth and seventh nerve palsy, palatal or lingual palsy, deafness, syndactyly, polydactyly, absent fingers and toes, Vestibular nystagmus that can not be elicited by caloric test.

Rosenthal Malcarson37 syndrome is a rare condition where there is bilateral infra nuclear facial palsy with furrowed tongue. Leprosy is a common cause of bilateral lagophthalmos in adults. It takes minimum 10-15 year for leprotic lagophthalmos to develop hence it is not seen in children.

2. Mechanical causes of lagophthalmos prevent the lids to close over the globe, even when lid closure is achieved the lids gradually separate to expose the anterior part of the globe. The mechanical cause may be in the orbit or in the lids.

The orbital causes are: Proptosis and exophthalmos. Buphthalmos, anterior staphyloma or large ciliary staphylomas do not obstruct closure.

Palpebral causes are Large coloboma of the lids, mostly upper lid, cicatricial ectropion of the lids, they are mostly seen following chemical and thermal burns of the lids.

Symptoms of lagophthalmos are watering, inability to close the eye, redness of the eye, pain, photophobia.

Two mechanisms are involved in watering of the eye in lagophthalmos.

1.Improper closure of the lid, ectropion of the lower lid, early tear film break-up all in combination or alone produce epiphora.

2.Reflex watering is produced due to exposure of the cornea and conjunctiva that result in corneal abrasion and ulceration. which in turn leads to circumciliary congestion. The cause of redness of the eye in is exposure conjunctivitis and circumciliary especially in the lower lid.

Signs of lagophthalmos consist of

(a) Loss of horizontal folds in the fore head. (b) Flattening of the eyebrow.

(c) Widening of the interpalpebral fissure. (d) Weakness of the orbicularis.

(e) Ectropion of the lower lid. Initially the inner margin just fails to remain in contact with the globe later it gets everted. If the evertion is much the tarsal conjunctiva also gets everted and becomes keratanised, dry and ulcerated.

(f) Conjunctival congestion. (g) Exposure keratitis.

Corneal sensation remains normal so long trigeminal is not involved. Once there is loss of sensation the condition becomes grave. The child without corneal sensation does not com-

plain of pain and watering which he would have done in presence of sensation. This may lead to ulceration, perforation and blindness.

Management

Management of lagophthalmos depends on severity and duration.

Lagophthalmos of mild degree and short duration does not require much attention except to keep the cornea protected by bland lubricant or artificial tear. Antibiotic ointments are given during sleep.

Bells palsy is produced secondary to edema of the facial nerve in the facial canal due to uncertain cause is treated by systemic steroids.

In case of corneal ulcer, hypopyon tarsorrhaphy is recommended to save the cornea from perforating.

DISORDERS OF LIDMARGIN IN CHILDREN

Important disorders of lid margin in children consist of:

A.Congenital

1.Coloboma of the lid margin.

2.Ankyloblepharon

3.Trichiasis

4.Distichiasis

5.Entropion

B.Inflammatory

1.Blepharitis

2.Stye

3.Chalazion

C.Post inflammatory and traumatic

1.Entropion

2.Ectropion

3.Trichiasis

Most of the conditions mentioned above except entropion, ectropion and trichiasis has already been discussed.

Entropion. Entropion in children is rare; a congenital entropion may be associated with epicanthic fold. In infants patching of the eye may lead to entropion of short duration that passes off with removal of pad. Cicatricial entropian is seen in children following acid alkali burn or post-inflammatory conditions like Steven’s Johnson syndrome membranous conjunctivitis. Children do not develop trachomatous entropion because it takes minimum 1520 years to develop trachomatous entropion.

Ectropion. Congenital ectropion is rarer than congenital entropion. Ectropion in chil-

dren are mostly traumatic or post inflammatory. The infection in such cases are seen on the skin surface rarely it is seen in facial palsy and in acid in alkali burns.

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