moncef khairallah, md - todnet.org · cme sequelae: lamellar or full-thickness macular hole...

Moncef Khairallah, MD

Department of Ophthalmology,

Fattouma Bourguiba University Hospital

Faculty of Medicine, University of Monastir

Monastir, Tunisia

IU: anatomic form of uveitis involving the

pars plana, peripheral retina, and vitreous

Previously termed posterior cyclitis,

chronic cyclitis, peripheral uveitis, basal

uveoretinitis, hyalitis,

pars planitis: subtype of IU

Diagnosis of IU based on typical clinical

features

Close follow-up and appropriate

management of IU and its complications

(CME)

INTRODUCTION

Moncef Khairallah, Tunisia

Bloch-Michel E, Nussenblat RB. International Uveitis Study Group Recommendations for the evaluation of intraocular inflammatory disease. Am J Ophthalmol 1987;103:234-235 Standardization of Uveitis Nomenclature (SUN) for Reporting Clinical Data. Results of the First International Workshop. Am J Ophthalmol 2005;140:509-516

IU: accounts for 10-15 % of all uveitis types

Most affected patients are young adults (>50%) or children

(25%)

Idiopathic IU is the most common uveitic entity in childhood

in our experience

No clear gender or race predilection

IU is idiopathic > 80%

Bilaterality (symmetric or asymmetric): 70 to 90%

EPIDEMIOLOGY

Intermediate uveitis


Patients may be asymptomatic

Or minimal symptoms: floaters, blurred vision, no pain or photophobia

Insidious onset

More severe cases: severe visual loss, red an painful eye (associated anterior uveitis)

Symptoms

CLINICAL FEATURES



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1. Quiet AC or associated anterior uveitis

2. Vitreous inflammatory features

3. Fundus changes, but absence of foci of retinal or choroidal

inflammation

CLINICAL FINDINGS



Anterior uveitis: absent, mild or moderate, rarely

severe, non granulomatous or granulomatous

A dilated, depressed peripheral fundus examination

is mandatory in order not to miss the diagnosis of IU



CLINICAL FINDINGS

Vitritis: is the most consistent

and characteristic sign of IU

The degree of vitreous

inflammation may range from

mild (+) to severe (++++)

Severe vitritis may obscure the

view of retina: impossible to

exclude the diagnosis of

posterior uveitis

Posterior vitreous detachment:

common

Vitreous cells

Vitreous haze



CLINICAL FINDINGS

Snowballs: globular,

yellowish-white foci of

vitreous inflammatory cells

mostly found in the inferior

periphery

Snowballs should be differentiated from

pearl-like precipitates: Behçet disease



CLINICAL FINDINGS

• Snowbank: frank white

exudation over the pars plana

and anterior retina

location: inferior retina, may

extend to involve the whole

periphery



CLINICAL FINDINGS

• Idiopathic intermediate uveitis

with snowballs and/or snowbank

(SUN criteria)

Pars planitis



CLINICAL FINDINGS

• Retinal vasculitis:

peripheral sheathing,

vascular leakage,

occlusion

• Cystoid macular edema:

the most common

complication of IU

• Optic disc swelling



CLINICAL FINDINGS

• FA is useful in the evaluation of:

macular edema, optic disc edema, retinal

vasculitis and its complications

(ischemia, neovascularization)

FLUORESCEIN ANGIOGRAPHY



• Useful in patients with dense vitritis preventing fundus

examination:

- Evaluation of vitreous involvement

- Exclusion of a focus of retinochoroiditis

- Exclusion of a RD

ULTRASONOGRAPHY



ULTRASOUND BIOMICROSCOPY

Useful in patients with poor

dilation or opaque media

Allows clear visualization of

pars plana condensations (snow-

bank)



OCT provides detailed information about:

-Macular edema

-Subretinal fluid, epiretinal membrane, VRT, macular hole

- Sequential OCT examination is also useful on follow-up

OCT



IU often considered as a relatively benign form of uveitis

Visual outcome: VA>20/40 in 75% of cases

However, prognosis of IU may not be good

Factors associated with poor visual outcome:

Severe inflammation

Chronicity

Exacerbations

Complications

CLINICAL COURSE AND PROGNOSIS



The most common complication of IU (28-64%)

CME is the leading cause of significant visual loss in IU

CME sequelae: lamellar or full-thickness macular hole

macular, cystoid degeneration, epiretinal membrane, RPE

alterations

Early detection and prompt treatment of CME is

mandatory

COMPLICATIONS

Cystoid macular edema (CME)



Cataract (15-20%)

Secondary glaucoma (4-15%)

Retinal detachment (3-22%) : serous, rhegmatogenous,

tractional, combined

Retinoschisis

Neovascularization (5-15%)

Vitreous hemorrhage (6-28%)

Vasoproliferative tumors

Optic neuritis (7%)

OTHER COMPLICATIONS



DIAGNOSTIC APPROACH TO IU

1st Step: make sure that « IU » is the

definitive diagnosis, by excluding:

A spill-over vitreous inflammation

associated with anterior uveitis

Fuchs syndrome: frequently misdiagnosed

as IU

Posterior uveitis: no focal chorioretinal

inflammatory lesion

A masquerade syndrome:

-Primary ocular lymphoma

-Retinoblastoma

-Endogenous endophthalmitis



2nd Step: Be aware of the differential diagnosis

of IU:

IU is most often idiopathic (> 80%)

Think especially about

sarcoidosis

and Multiple Sclerosis (MS)

Other causes:

Syphilis

Lyme Disease

Enterocolopathy

Whipple’s Disease

Cat-Scratch Disease

Tuberculosis

Toxocariasis

Behçet’s disease




3rd step: The work-up should not be extensive, but limited

and oriented by the :

Clinical findings:

Presence of snowbank: sarcoidosis, tuberculosis, multiple

sclerosis, idiopathic pars planitis

Prominent vitritis: toxocara, Whipple

Prominent vasculitis: Multiple sclerosis, Behçet’s disease

History and systemic symptoms




When should a diagnosis of MS be considered?:

- Young patients, especially women

- A family history of MS, neurological symptoms, visual field defects,

optic disc changes

- IU in the form of pars planitis, presence of prominent peripheral

venous sheathing , peripheral vitreous membranes

Order neurological work-up with MRI

About 20 % of patients with IU will subsequently develop MS or optic

neuritis on follow-up

The 2 diseases share a strong association with the HLA- DR2

IU and multiple sclerosis




If IU is associated with any systemic disease,

treatment of the underlying disease is mandatory

Specific treatment: for treatable infectious

diseases

MANAGEMENT



Mild IU, good VA, No CME, few symptoms no

treatment, but regular follow-up

Indications for treatment:

• Severe vitritis with VA < 20/40

• Cystoid macular edema, even if VA is normal

• Occlusive retinal vasculitis

• Severe snowbanking

• Neovascularization

MANAGEMENT



Corticosteroids:

Local: topical corticosteroids (prescribed only in the

presence of anterior uveitis)

Periocular injections, intravitreal injections, intraocular

implants

Systemic (1 mg/kg/day)

Immunosuppressive agents

Surgical treatment : pars plana vitrectomy, cryotherapy,

peripheral laser photocoagulation, cataract surgery,

glaucoma surgery

MANAGEMENT MODALITIES



First-line therapy: Corticosteroids

Unilateral IU: periocular injection of triamcinolone

acetonide (Kenalog*, Kenacort*):

-20 or 40 mg

- Subtenon or orbital floor

- Good response in most patients

- Can be repeated if necessary

MANAGEMENT



Courtesy, Carlos Pavesio

First-line therapy: Corticosteroids

Bilateral IU: Oral corticosteroids:

- Prednisone or prednisolone

(0.5 to 1 mg/Kg/day, with gradual tapering over

a period of 3-6 months)

MANAGEMENT



IU resistant to cortisteroid therapy, side effects:

Immunosuppressive therapy:

Cyclosporine A

Methotrexate

Azathioprine

Other drugs

Biologic agents

MANAGEMENT



Vitrectomy:

Required for the treatment of certain

complications (vitreous opacification, RD,

epiretinal membrane)

Its effects on inflammation and CME remain a

controversial issue

MANAGEMENT



Peripheral laser photocoagulation, cryotherapy:

extensive peripheral retinal ischemia with or

without retinal new vessels

MANAGEMENT



Cataract surgery:

- Indicated if visually significant catatract

- Inflammation should be controlled for at least 3 months

- Oral prednisone (1 mg/kg): 4-7 days before surgery, with gradual tapering

over a period of 4 to 6 weeks postoperatively

- Surgical technique:

- Phacoemulsification + hydrophobic acrylic intraocular

lens is the techique of choice

- Combined Phaco+vitrectomy if necessary

MANAGEMENT



CONCLUSION

IU accountes for 10-20% of all uveitis cases

Diagnosis of IU is based on strict clinical

features

Most cases of IU are idiopathic (>80%)

Prompt, good control of inflammation and

treatment of complications (CME+++) are

important to improve the visual prognosis



Thank you for your attention

moncef khairallah, md - todnet.org · cme sequelae: lamellar or full-thickness macular hole...

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