Ординатура / Офтальмология / Английские материалы / Pediatric Opthalmology_Mukherjee_2005

1.Unequivocal evidence of HIV I infection confirmed by ELISA and Western blot test.

2.CD4 cells count less than 200 cells/mm3.

3.Evidence of systemic infection by at least one opportunistic organism.

4.Presence of Kaposi sarcoma.

Management

AIDS is uniformly fatal in spite of treatment. It is a blinding disease in 60% of patients but it is fully preventable disease. Once the disease sets-in, management is by multiple HIV drugs given systemically. Ocular management is paliative. Malignancy do not require separate treatment however concurrent anti cancer drugs may be required. Ocular treatment for anterior uveitis is by local steroids and cycloplegic. Posterior segment treatment consists of systemic anti HIV drugs and surgical treatment for retinal detachment with uniformly poor prognosis.

Connective tissue and uveitis in children

Connective tissue disease involving joints are common causes of both acute and chronic anterior uveitis in children most of them are positive for HLA B-27, antinuclear antibodies and negative for rheumatoid factor. Some of the characteristic features of arthritic uveitis are—They are generally acute, unilateral that may be recurrent in the same eye or may alternate between the two eyes, posterior synechia. Hypopyon is common.

Common joint disorders associated with uveitis in children are :

Juvenile rheumatoid arthritis (JRA), Stills disease, juvenile spondylitis, juvenile Reiters syndrome, arthritis with bowel disease.

Juvenile rheumatoid arthritis24, 29, 42, 43

It is the commonest form of arthritis seen in children between 2 to 16 years of age, peak being 2 to 4 years. It is more frequent is girls. Ratio of girls to boys is roughly 3:2. Antinuclear antibodies is positive in 75% of cases while 95% of patients have positive HLA B-27, it is sero negative.

According to clinical features in first three months the disease has been divided into three sub groups.

1.Predominantly systemic involvement with minimum uveal involvement. It manifests with intermittent fever, lymphadenopathy, hepato spleenomegaly and maculo papular rash. There may be pericarditis.

2.Polyarticular involvement with moderate uveal involvement.

This type affects four or more than five joints during first to three months. Knee joint is mostly involved followed by wrist and ankle. Arthritis may last for many years resulting in permanent joint deformity. The condition is generally symmetrical.

3.Pauci articular involvement with more uveal involvement - About 5% of children with JRA come in this group. Number of joints involved in first three months is four or less, involvement is asymmetric, knee happens to be most commonly involved.

Twenty percent of children develop uveitis. Arthritis usually precedes ocular involvement by several years, occasionally reverse may be true.

Uveitis in juvenile rheumatoid arthritis

Uveitis in ninety percent of cases is mostly anterior uveitis, pan uveal involvement is extremely rare so is involvement of vitreous and choroids.

It is bilateral in 70%-80% of cases and recurrent. It is non granulomatous in nature. There are two groups of patients who have uveitis.

1.Silent white uveitis seen mostly in girls between 4 to 16 years of age. The onset is so insidious without any discomfort that it is called silent uveitis. The condition may be diagnosed not due to redness, lacrimation or photophobia but due to diminished vision or on routine slit lamp examination that shows one to two plus cells + fine KPS and flare.

2.The pausi articular involvement as idiopathic anterior uveitis in adolescent. The ocular manifests may continue despite treatment and apparent cure of joint involvement leading to frequent complications of band keratopathy, cataract secondary glaucoma and macular edema. Somehow hypotony is also a common feature.

The complications are potential threat to vision, hence the child should be under surveillance for at least 6 to 7 years following development of arthritis. Children with iridocyclitis in pausi articular disease should be examined every three months and cases of iris with positive ANA more frequently. Examination should include recording of acuity of vision, measurement of intraocular tension both for glaucoma and hypotony. Child should undergo assessment of errors of refraction and examination of fundus and detailed slit lamp biomicroscopy.

Diagnosis is not difficult with history of joint involvement in child with non granulomatous uveitis, positive ANA and HLA B-27 but negative serology for rheumatoid factor.

Differential diagnosis consists of idiopathic iritis, infective iritis and sarcoid in children.

Management

Management depends upon severity and duration of disease, presence of complications are managed as and when they arises. Severity of the disease can be

1.Mild with +1 cells and flares.

2.Moderate attack lasting more than 4 months with +2 to +4 aqueous cells.

3.Moderately severe lasting more than for four months with acute exacerbation.

4.Severe—attack lasts for years in spite of treatment, respond poorly to treatment, are more prone to develop complications.

Mild to moderate cases can be controlled by cycloplegic and local steroid drops. In initial stages the eye should be atropinised to break the posterior synechia. Once the posterior synechia have been broken the child is put on short acting cycloplegic like home atropine 2%—two to three times a day or cyclopentolate once a day. Children should not be put on atropine for long

times as it may induce atropine induced amblyopia. A school going child under atropine require bifocal for near work. Once the inflammation has been reduced, a short acting cycloplegic like tropicamide at bed time gives sufficient accommodation during day to go to school. For mydriasis induced glare dark glasses are prescribed.

Next line of medicines are steroids in the form of drops or periocular injection. Drops may be required hourly for first few days in acute stages, otherwise instillation four times a day is sufficient. Strength of steroid should be minimum with maximum benefit. Ocular side effects of local steroids should be weighed against its advantages and its dose adjusted accordingly. Periocular steroids are given in children in teens, younger children do not cooperate for periocular injection. Systemic steroids should be avoided under ten years of age due to its systemic side effect. If at all it has to be given it should be prescribed in consultation and under supervision of pediatrician. Older children are given steroid as in adults.

Non steroidal anti-inflammatory drops are generally prescribed as maintenance therapy for long time. So far no ocular side effect has been reported following local administration of NSAID. Systemic non steroidal anti inflammatory drugs are better avoided in children.

Chronic cases not responding to local steroids or intolerant to systemic steroids are put on systemic immune suppressives drugs in consulation with oncologist. Dose of immuno suppressive drugs for uveitis is less than that for malignancy. A constant watch on side effects especially haemotological must be kept. They are also better avoided under ten years of age.

Complications are frequent both due to disease itself and sometimes due to prolonged use of steroids.

Common complications are cataract, secondary glaucoma, band keratopathy, amblyopia and hyptony.

Cataract is managed by surgery for which a quiet eye is a prerequisite. The eye should be white without flare for at least three month before surgery. Surgery should be followed by use of steroid in sufficient dose and frequency for long time. An unnecessary delay in cataract surgery may lead to amblyopia. All surgeries should be done under general anaesthesia with endo tracheal intubation. Thick posterior capsular membrane formation is very common that may require repeated manipulation. Role of intra ocular implant is unsatisfactory, may be avoided as far as possible. Aphakia may be managed either by contact lens, with spectacle reading correction or bifocal. In smaller children contact lens fitting is really difficult though not impossible.

Glaucoma

Possibility of glaucoma should always be kept in mind, it can be due to inflammatory process and its aftermath, steroid induced or both. Secondary post inflammatory glaucoma respond poorly to anti glaucoma drugs. Miotics and prostaglandin analogs are contra indicated. A child can not be put on oral carbonic anhydrase inhibitor for a long time due to systemic side effect. Surgery also does not give satisfactory results, failure is common.

Band keratopathy is managed initially by EDTA otherwise lamellar keratoplasty or excimer photo therapeutic keratectomy may be tried.

Amblyopia is a tumbling block that is produced due to prolonged use of atropine, opacities in media, macular edema, undetected glaucoma, anisometropia following unilateral lens extraction. Failure to use contact lens and unsatisfactory I0L transplant.

Uveitis in ankylosing spondylitis42,45,56

It is a chronic inflammatory disease of unknown origin that involves mostly the axial spine in adults. In children the disease is mostly seen in peripheral joints of lower limb. The disease has positive HLA B 27 in 80 to 90 percent patients. It is sero negative for rheumatoid factor.

About 25% children suffering from ankylosing spondylitis develops anterior uveitis. 50% uveitis with positive HLA B 27 suffer from ankylosing spondylitis.

Ocular manifestations are mainly anterior uveitis that is acute, recurring, unilateral, non granulomatous. About 80% children with uveitis will develop bilateral disease that is rarely simultaneous. An attack of anterior uveitis generally lasts for 4 to 6 weeks. Recurrence may vary between every month to one year. Uveitis may be proceeded, may be simultaneous or may follow diagnosis of spondylitis.

Signs

Consists of circum corneal congestion, fine dusty or small KPs cells and flares are generally in the range of 3+ and 4+. In more severe cases there may be frank hypopyon, posterior synechia results in iris bombe and secondary glaucoma. Cataract, CME and band keratopalthy are less common than in juvenile rheumatoid arthritis. It is more common in adolescent boys than girls.

Diagnosis

Unilateral acute iridocyclitis in second decade in a boy with pain in peripheral joint, positive HLA-B 27 should undergo X-ray of sacro iliac joint for possibility of sacroilitis. Management is with cycloplegic local and periocular steroids.

Reiters syndrome is a rare disease of unknown origin, parts of it may be infective, (urethritis and dysentery) it was originally described as triad of urethritis, conjunctivitis and arthritis. However it is associated more commonly with anterior uveitis, keratitis than conjunctivitis. Conjunectivitis when present is generally bilateral and mucopurulent. Arthritis is poly articular and asymmetrical. uveitis is generally unilateral, non granulomatous.

Treatment comprises of local treatment of mucopurulent conjunctivitis by frequent antibiotic drops. Uveitis is treated with cycloplegic and steroids. Urethritis is non gonococcal and treated with erythromycin. Dysentery is treated by combination of trimethoprim and sulpha methoxazol or ciprofloxacin.

Pars planitis or intermediate uveitis57A,57B

Pars planitis is a common chronic bilateral uveitis that is either not diagnosed till late or misdiagnosed. Its exact etiology is not known. It has variable clinical presentation, hence it has been called by various names since 1908. When Fuch’s called it cyclitis. The Internaational uveitis study group has finally labelled it as intermediate uveitis in 1987.57C Since than it is being referred as such.

Pars planitis is a disease of pars plana of ciliary body adjoining choroid, peripheral retina mostly peripheral retinal vessels. In large series it has been reported to be as common as 10 to 15 percent of all uveitis cases observed. It is a disease of young, adults and children. There are reports that it may run in families. Both boys and girls are affected equally.

To begin with it is almost symptomless, the earliest symptoms that may attract child’s attention is floaters in the affected eye or mild diminished vision. Floaters are generally not taken very seriously by the parents or treating general physician. By the time the next symptoms of diminished vision occurs the disease has progressed far. The vision may be within normal limits initially. Common cause of diminished vision are- Posterior subcapsular cataract and cystoid macular edema.

On slit lamp examination there are cells and flares is aqueous. Posterior synechia are so uncommon in pars palanitis that some authors consider that presence of posterior synechia is evidence strong enough to exclude pars planitis.57D Sometimes there may be few KPs and peripheral corneal edema.

The anterior vitreous shows flares, cells and strands. Examination of peripheral retina with indirect opthalmoscope and scleral indentation shows evidence of peri artrititis and periphlebitis. Most important finding on examination of peripheral retina are snowball opacities and snow banking mostly in the inferior quadrant. There may be new vessel formation near the snow bank that may be source of haemorrhage58. Cystoid macular edema is a late feature and major cause of diminished vision. The course is variable. Some children may have the disease for years with or without loss of vision, others may have choroidal effusion and serous retinal detachment. Hypotony is common. However some patients may have rise of intraocular pressure due to spilling over of the pathology in the angle.

There is no HLA phenotypes. Fundus fluorescein angiography is the diagnostic of CMC and periphlebitis.

Differential diagnosis consists of - idiopathic iridocylitis. Absence of joint systems rule out juvenile rheumatoid arthritis associated with uveitis. Peripheral granuloma of toxocara is excluded by negative serology test. Careful examination of retinal periphery, presence of snowball makes it imperative to exclude sarcoid by X-ray chest, gallium scan and raised serum calcium.

Management

Almost eighty percent of children do not require any treatment. Local and peripbulbar steroids are required if vision falls below 6/18. Cycloplegic do not have any added advantage. Children not improving with local peribulbar steroids should be put on systemic steroid for long time with usual precaution. Immuno suppressive drugs may be required. Rarely surgical intervention like lensectomy or vitrectomy is required.

Sympathetic ophthalmia57, 60, 61

Sympathetic ophthalmia is a bilateral potential blinding disorder. It is said to be rare disease in advanced countries and urban areas of developing countries but it’s incidence in under developed countries must be more than reported. It is a bilateral chronic pan uveitis

with acute exacerbation and recurrences. It is most commonly seen following penetrating injury within five mm. of limbus with incarceration of uvea. However, rupture of the globe anywhere with incarceration of any intra ocular structure can cause sympathetic ophthalmia. About 60% of the cases are seen following accidental penetrating wound. However, it has been reported following blunt injury. About 30% are seen following surgical manipulation in which the globe is penetrated. Common surgical procedures are—lens extraction, any glaucoma operation, iris inclusion surgeries for glaucoma were notorious to produce sympathetic ophthalmia, retinal detachment surgeries with drainage of sub retinal fluid. Even laser photo coagulation have been reported to cause sympathetic ophthalmia. It is said that sympathetic ophlhalmia does not develop if the injured eye has developed suppuration. However, it has been reported following perforation of corneal ulcer.

The eye that is injured is called exciting eye and the eye that develops chronic pan uveitis is called sympathizing eye. No race, age or sex is immune. Children are affected more than adults, number of boys developing sympathetic uveitis is more than girls. This is perhaps due to the fact that boys are more prone to injury than girls.

Interval between injury in exciting eye and development of sympathetic uveitis varies. It spans between 5 days to 66 years, 70% of cases develop within three months and 90% within first year.

Etiology of the condition is not well understood, however, there is sufficient evidence to consider auto immunity as prime factor. The reaction is delayed type-four hypersensitivity to uveal antigens located in retinal pigment epithelium and melanocytes. Anti retinal antibodies have been found in some cases24, 66, 61. The immune reaction results in chronic granulomatous lesion that forms a giant cell similar to tuberculosis without caseation. The chorio capillaries are spared. There is thickening of all the parts of uvea, specially the choroid. Small yellowish white spots called Delen Fuch’s nodule are seen scattered in the choroid. They are common in the lower mid periphery, however, they are not diagnosed of sympathetic uveitis.

Clinical features

Clinical features are variable, all eyes with penetrating injury do not develop sympathetic uveitis. Some may develop it within few days, others may not be effected for decades after initial injury. Clinical presentation depends upon inflammatory process which in turn is perhaps genetically predisposed. The disease generally develops in iris but may start anywhere in the uvea and it is bound to become panuveal.

The injured eye has evidence of penetrating injury and its complication that may mask the presence of indolent plastic iridocyclitis.

The early symptoms of uveitis in the sympathizing eye are heralded by photophobia, loss of accommodation, lacrimation and blurring of vision. The eye at this stage may not be congested. First sign of sympathetic uveitis is flare the retrolental space. The anterior chamber may show 2+ to 3+ cells and flare. The cornea has various shapes of KPs. Generally they are large mutton flat in nature but may be fine dusty. The iris is greatly thickened. The pupil is miotic and irregular due to strong posterior synechia. There may be both Koeppe and

Busacca’s nodule present. Onset of inflammation in sympathetising eye generally worsens, the signs and symptoms in the exciting eye.

The posterior segment involvement results in the posterior vitreous cells, papillitis, retinal vasculitis and serous retinal detachment, the eyes are generally soft. Extra ocular manifestation are rare, they may be vitiligo, poliosis, alopecia and polycytosis of CSF.

Common complicationa in untreated cases are—Complicated cataract, secondary glaucoma, band keratopathy, retinal detachment, phthises and blindness. Sometimes the exciting eye may have better vision than the sympathetising eye.

Diagnosis is mostly clinical based on

1.History of trauma, that has either not been repaired or repaired without freeing of uvea from the wound. Sometime the injured lens may cause lens related uveitis. This does not involve the other eye and can be managed-well by removal of lens material.

2.Clinical evidence of anterior uveitis or choroiditis.

3.Ultrasonography shows thickening of choroids.

4.Fluorescein angiography may initially show areas of hypo fluorescence due to masking of choroid by Delen Fuch’s nodule followed by hyper fluorescence due to the leak of the dye.

Management

Correct and early diagnosis helps to restore vision better and prevent permanent loss of vision. Management of sympathetic ophthalmia can be divided in the two groups (1) Surgical and (2) Medical.

Surgical treatment is restricted to injured eye that consists of management of (1) Injury and (2) Enucleation of unmanageable injury. All penetrating injuries in children should be repaired under general anaesthesia, under microscope with use of viscoelastic substance. Before embarking on repair of the visible wound possibilities of other sub conjunctival wounds and wounds under the extra ocular muscle should be explored and repaired. The uvea should be dis-engaged from the wound and excised by standard procedure, if necessary. The lens matter should be removed and if needed anterior vitrectomy done. The anterior segment should be reconstructed as far as possible, a sub conjunctival injection of broad spectrum antibiotic with steroid is given along with atropine. The eye is kept under observation for evidence of uveitis.

The other eye is examined regularly. The parents are warned about possibilities of sympathetic ophthalmia and instructed to bring the child for examination if the child develops lacrimation or unexplained blurring of vision.

Enucleation

If the injured eye is not salvaged it is better to enucleate it within the first ten days of injury. The purpose of enucleation is to remove the uvea in toto, evisceration does not serve this purpose hence it is not recommended. Role of enucleation after onset of sympathetic opthalmia is doubtful. Sometimes the exciting eye may have better vision than sympathizing eye.

(ii) Neurological signs and symptoms are headache, neck stiffness, meningism, encephalopathy, myopathy, cranial neuropathy, CSF lymphocytosis.

(iii) Auditory features are tinitus, vertigo and deafness. The auditory symptoms lasts only few months.

(3) Uveitis phase : This can be divided into two groups :

(i) Anterior segment involvement or Vogt Koyanagi form - This consists mostly of granulomatous uveitis that begins with blurring of vision, diminished near vision, photophobia, redness, lacrimation, dull pain. On examination, there is ciliary flush, mild mutton fat KPs, anterior chamber reaction, edema of iris, strong and broad posterior synechia, the iris nodule and atrophy of iris stroma.

(ii) Posterior segment involvement (Harada syndrome) consists of multiple exudative choroidal lesion that results in exudative retinal detachment which is self limiting and resolve spontaneously. There may be papilitis. Involvement of macula results in metamorphopsia. Fluorescein angiography shows multiple area of hypo fluorescence. Area of retinal edema and detachment show hypo fluorescence. Areas of choroidal ischeamia are better shown by indocyanine green.

Exact etiology is not known but it is thought to be a T-cell mediated auto immune reaction to melanocytes resulting into affection of organs that contain melanocytes i.e. skin, uvea etc.

Complications consists of complicated cataract, secondary glaucoma band keratopathy, phthises.

Management is similar to any chronic non-infective granulomatous uveitis. It is generally treated by local (1) Cycloplegia (2) Steroid drops (3) Periocular injection of depot steroids and (4) systemic steroids in divided multiple dose, daily single dose or alternate day single dose. (5) Immunosuppressive agents are effective in recurrence and helps reduce dose of steroids.

Sarcoid uveitis

It is a disease of adults but may be seen in children as well. Sarcoid is a systemic disorder of unknown cause of most probably T-cells mediated immunity that involve almost all organs of the body. All parts of the eye may be affected. Uveitis in sarcoidosis can be acute or chronic both granulomatous and non-granulomatous lesion are possible. Both anterior and posterior segments may be involved. Common posterior segment involvement can be vitritis, vitreous haemorrahage, pars planitis with typical snow bank appearance and candle wax lesion in retina. Diagnosis is confirmed by histopathological examination of skin or conjunctival nodule. Management is systemic steroids, local, cycloplegic, local and periocular steroids.

Fuch’s heterochronic iriodcyclitis

It is commonly presumed that Fuch’s heterochromic iridocyclitis is a disease confined to adults only but it is known to be present in all ages, in all ethnic groups and both sexes. It is a predominantly uniocular disease. Only 10% cases are bilateral. It is generally asymptomatic. Attention may be drawn to uniocular light colour of iris. The iris with light colour is the affected eye. The eye is generally white, on slit lamp examination small non pigmented

keratic precipitates are seen. There may be iris nodules but no posterior synechia are seen, there may be vitreous cells. It has a protracted course with good prognosis. Most of the cases do not require any treatment unless cataract develops. Some cases may require local steroids. Cycloplegies are not required.

Lens induced uveitis

So long as the lens is inside intact capsules it does not produced any reaction either allergy or chemical. Leak of lens material causes two types of reaction.

(I)A chemical reaction due to leak of lens protein through non ruptured lens capsule in hyper mature cataract.

(II)Type two hyper lens induced hyper sensitivity. In children hyper mature lens may result either in traumatic cataract following blunt injury or in unattended congenital cataract. The condition may produce acute or chronic anterior uveitis and occasional glaucoma. Uveitis subsides with complete removal of lens matter. If lens matter is left, it may result into second type of lens induced uveitis i.e. endophthalmitis phacoanaphylactica. Other mode is traumatic rupture of lens either due to accidental penetrating injury or surgical injury resulting in severe uveitis that may involve all the parts. After uveitis subsides in the first eye, the second eyes get effected in similar ways i.e. if the lens ruptures accidentally or during surgery even years after the original incident. This may be confused with sympathetic uveitis. Treatment consists of local cycloplegic, steroid, beta blockers. Complete removal cortical material in the first eye.

Behcets disease42, 66, 67

The disease is seen world over as rare condition, is more common among Japanese. It’s share as causative factor of uveitis in children is very less. Exact etiology of the disease is not known. HLAB-51 is closely associated with it. There are two types of ocular involvement. 1. Mostly anterior segment involvement. 2. Mostly posterior segment involvement with systemic involvement that includes recurrent aphthous ulcer of mouth and genitals, skin lesions, joint pain. There may be sudden development of hyppyon in iridocyclitis. There may be cystoid macular edema, retinal exudates and haemorrhage, cataract and glaucoma. Treatment is unsatisfactory. Local treatment consists of cycloplegic and steroids, systemic steroids are given only in cystoid macular edema for short period. Prolong systemic steroids results in poor visual outcome, some times immuno suppressive may be required.

Anterior segment ischeamia and uveitis

Anterior segment ischemia is mostly an iatrogenic disease following strabismus and retinal surgery. This is more common in adults and incidence rises with age, however, it can occur in children. Basic pathology is cutting blood supply of the anterior uvea by way of disruption of anterior ciliary artery following disinsertion of more than two recti.68 The disorder starts within 24 hours of following surgery with pain, lacrimation, photophobia. It presents as hazy cornea due to edema and folds in Descemet’s membrane, keratic precipitates, aqueous flare and cells. If not attended to it may lead to iris atrophy causing distorted pupil and complicated cataract. Anterior segment ischeamia may also develop in sickle cell haemoglobinopathy. Flourescein angiography of iris shows well demarcated areas of non