**16. Pediatric uveitis**

20 Complementary Pediatrics

as cutaneous or eyelid marginal vesicles, or ulcers on the bulbar conjunctiva that can be rarely accompanied by dendritic epithelial keratitis. Primary ocular HSV infection is a self limited disease that usually resolves spontaneously. Oral antiviral therapy can speed up the

Dendritic ulcers, stromal necrotizing keratitis and disciform keratitis are forms of recurrent ocular infection of HSV. These may also occur in this age group. Topical and oral antiviral

Adenoviruses are the most common viral pathogens that may cause viral keratitis in a child. Pharyngoconjunctival fever (PCF) and epidemic keratoconjunctivitis (EPC) are 2 different clinical pictures that are caused by different serotypes of adenoviruses. PCF is caused by types 3, 4 and 7, while EPC is caused by types 8, 19 and 37. Corneal involvement is much more common and severe in EPC. Keratitis may persist for years in some cases. PCF is the less severe form of the disease. Keratitis is usually mild and self limiting. Mild to moderate fever may accompany PCF. The management of adenoviral keratitis is usually conservative. Topical steroids and cyclosporine may be tried to reduce inflammation. Reduction of transmission risk by avoiding contact with infected patients during the initial 7-10 days of the active disease and by good hygiene is much more important than its management. Ophthalmologists are well experienced about EKC, because unfortunately the eye clinics are usually the most common places to come in contact with the adenovirus and many ophthalmologists are infected once or more with adenoviruses. Many outbreaks occur due

Vernal keratoconjunctivitis is an allergic eye disease that is mainly seen in male children. Symptoms include itching, photophobia, and mucoid discharge. Corneal findings consist of Horner-Trantas dots (degenerated eosinophils and epithelial cells) in the limbal area, punctate epithelial erosions and shield ulcer (an oval noninfectious epithelial ulcer). Corneal findings are generally accompanied by conjunctival ones which are hyperemia, conjunctival edema (chemosis) and papillary hypertrophy. Topical antihistamines and mast-cell stabilizers can be used in the management of vernal conjunctivitis. Severe cases may require

Atopic keratoconjunctivitis is a rare bilateral allergic eye disease that is most commonly diagnosed in young men, but also in children. Clinical picture is similar to vernal keratoconjunctivitis, but more severe. The papillary hypertrophy are less developed compared to vernal keratoconjunctivitis. The history of allergy such as allergic asthma or atopic dermatitis is commonly associated. Keratopathy leading to total corneal neovascularization may occur. Management is similar to vernalis keratoconjunctivitis, but

It is characterized by the ocular triad (conjunctivitis/ episcleritis, iridocyclitis, or keratitis), urethritis and arthritis. It is usually associated with gram-negative bacterial ( Salmonella,

topical corticosteroid or topical immune-modulating agents such as cyclosporine.

therapy can be used in the management of recurrent HSV keratitis.

to improperly disinfected diagnostic instruments (Kanski, 2011).

**15.2 Allergic diseases affecting the cornea** 

the disease is less responsive.

**15.3 Reiter syndrome** 

resolution.

The uvea is a pigmented structure that primarily lies between the retina and the sclera and constitutes the vascular portion of the eye. It comprises the iris, ciliary body and choroid. Uveitis, by strict definition implies an inflammation of the uveal tract. Uveitis is named according to the anatomical location of inflammation in the uvea. Anterior uveitis may be subdivided into: Iritis and iridocyclitis. Iritis is primarily the inflammation of the iris tissue. On the other hand, iridocyclitis involves both the iris and the pars plicata of the ciliary body. Intermediate uveitis is defined as inflammation predominantly involving the pars plana, the peripheral retina and the vitreous. Posterior uveitis involves the fundus posterior to the vitreous base. Panuveitis implies involvement of the entire uveal tract without a predominant site of inflammation (Kanski, 2011).

Pediatric uveitis may be categorized into 4 types of uveitis based on the anatomical location of the inflammatory process. These are anterior (non-granulomatous and granulomatous), intermediate and posterior uveitis. Etiologic factors associated with these uvetis in children are as follows.

Anterior non-granulomatous uveitis: Idiopathic, HLA-B27 associated, juvenile rheumatoid arthritis (JRA), ankylosing spondylitis, Reiter's disease, psoriasis, inflammatory bowel disease, nephritis, systemic lupus erythematosus, Herpes Simplex virus (HSV), Lyme disease, leukemia, drug-induced.

Anterior granulomatous uveitis: Sarcoidosis, inflammatory bowel disease, syphilis, Herpes simplex virus, tuberculosis, Behcet's disease, multiple sclerosis, fungal disease, Whipple's disease, leprosy.

Intermediate uveitis: JRA, Pars Planitis, Multiple Sclerosis, Lyme disease, Sarcoidosis.

Posterior Uveitis: Toxocariasis, Toxoplasmosis, Leukemia, Tuberculosis, Intraocular Foreign Body, Vogt-Koyanagi Harada Syndrome (VKH), Cytomegalovirus, HSV/VZV, inflammatory bowel disease, syphilis, Behcet's disease, systemic lupus erythematosus, Kawasaki's disease, sarcoidosis, polyarteritis nodosa, Wegener's granulomatosis (Kanski, 2011).

Anterior uveitis is the most common form of uveitis. Features are typically with sudden onset of unilateral pain, photophobia and redness, which may be associated with lacrimation. Occasionally patients may notice mild ocular discomfort a few days before the acute attack when clinical signs are absent. Visual acuity is usually good. The presence of vitreal cells in an active vitritis are the main signs of pars planitis. Posterior uveitis encompasses retinitis, choroiditis and retinal vasculitis. Some lesions may originate primarily in the retina or choroid but often there is involvement of both (Sauberan, 2010).

Special investigations such as skin tests, serology and radiology are indicated in posterior uveitis, granulomatous inflammation, recurrent uveitis and bilateral uveitis.

Treatment of the majority of uveitis involves predominantly the use of anti-inflammatory and immunosuppressive agents. Antibiotic therapy for infectious diseases may be

Pediatric Ophthalmology / Eye and Disorders 23

children should lead the physician to suspect a systemic disease, a syndrome, trauma, or a tumor in the eye. That should be ruled out with ultrasound or CT scan. Other causes of congenital or infantile retinal detachment should also be considered, including Stickler's syndrome, retinopathy of prematurity, persistent hyperplastic primary vitreous (PHPV), also called persistent fetal vasculature (PFV), may also present with a retinal detachment at birth; however, the associated microphthalmos and cataract often makes the diagnosis easier. Stickler's syndrome combines ocular, orthopedic and midfacial anomalies in an autosomal dominant inheritance pattern. Ocular abnormalities include high myopia, empty

Prediction, prophylaxis and timely surgical treatment of retinal detachment may prevent visual loss. Retinal detachment in childhood and adolescence is different from adult cases due to higher rate of complicating predisposing factors such as trauma and high myopia and also due to the delay in the diagnosis that is mostly made after macular involvement. By appropriate surgical treatment the anatomic success rate may be as high as adult cases, however lower functional results are usually achieved because of higher rate of macular involvement (Butler et al., 2001; Topbas et al., 2003). It is important for the pediatrician to refer the children with signs and symptoms of retinal detachment, children with systemic disorders associated with retinal detachment and also children with ocular trauma history

Retinoblastoma (RB) is the most common intraocular malignancy of the childhood, occurring in about in 1/15.000 to 20.000 live births. The tumor develops from the immature

RB occurs in hereditary and nonhereditary forms. It can be unilateral or bilateral. The hereditary form is usually bilateral and multifocal, whereas the nonhereditary form is unilateral and unifocal. The RB gene is recognized to be a recessive suppresser gene located on chromosome 13 at the 13q14 segment and some affected children have other systemic features of the 13 q deletion syndrome. It is accepted that all bilateral and familial cases of RB will manifest a germline mutation, whereas only 10–15% of unilateral sporadic cases will

RB usually diagnosed between 3 months and 3 years of age, but it can be congenital or it can occur in older children. The affected child usually presents with leukocoria and/or strabismus. More advanced tumors can cause painful secondary glaucoma or signs of orbital cellulitis. The diagnosis of RB is best made by slit lamp bio-microscopy and indirect ophthalmoscopy. Ancillary studies are ultrasonography and computed tomography. The

red-reflex examination is the best way to screen for retinoblastoma.

vitreous with membranes and bands.

**17.3 Management** 

to an ophthalmologist.

**18. Retinoblastoma** 

**18.1 Inheritance** 

show germline mutation (Shields, 2006).

**18.2 Symptoms and diagnosis** 

retina.

necessary. Topical steroids are the mainstay treatment for anterior uveitis, while systemic steroids are indicated in most cases of intermediate and posterior uveitis. If the disease is unresponsive to systemic steroids and/or the patient cannot tolerate systemic steroids, other immunosuppressive agents including azathioprine, methotrexate and ciclosporin may be used (Jancevski ,2010).
