Moncef Khairallah, MD

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1 Moncef Khairallah, MD Department of Ophthalmology, Fattouma Bourguiba University Hospital Faculty of Medicine, University of Monastir Monastir, Tunisia

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

3 EPIDEMIOLOGY IU: accounts for % 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%

4 CLINICAL FEATURES Symptoms 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)

5 CLINICAL FINDINGS 1. Quiet AC or associated anterior uveitis 2. Vitreous inflammatory features 3. Fundus changes, but absence of foci of retinal or choroidal inflammation

6 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

7 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

8 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

9 CLINICAL FINDINGS Snowbank: frank white exudation over the pars plana and anterior retina location: inferior retina, may extend to involve the whole periphery

10 Idiopathic intermediate uveitis with snowballs and/or snowbank (SUN criteria) Intermediate uveitis CLINICAL FINDINGS Pars planitis

11 CLINICAL FINDINGS Retinal vasculitis: peripheral sheathing, vascular leakage, occlusion Cystoid macular edema: the most common complication of IU Optic disc swelling

12 FLUORESCEIN ANGIOGRAPHY FA is useful in the evaluation of: macular edema, optic disc edema, retinal vasculitis and its complications (ischemia, neovascularization)

13 ULTRASONOGRAPHY Useful in patients with dense vitritis preventing fundus examination: - Evaluation of vitreous involvement - Exclusion of a focus of retinochoroiditis - Exclusion of a RD

14 ULTRASOUND BIOMICROSCOPY Useful in patients with poor dilation or opaque media Allows clear visualization of pars plana condensations (snowbank)

15 OCT OCT provides detailed information about: -Macular edema -Subretinal fluid, epiretinal membrane, VRT, macular hole - Sequential OCT examination is also useful on follow-up

16 CLINICAL COURSE AND PROGNOSIS 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

17 COMPLICATIONS Cystoid macular edema (CME) 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

18 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

19 DIAGNOSTIC APPROACH TO IU 1 st 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

20 2 nd 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 DIAGNOSTIC APPROACH TO IU

21 DIAGNOSTIC APPROACH TO IU 3 rd 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

22 DIAGNOSTIC APPROACH TO IU IU and multiple sclerosis 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

23 MANAGEMENT If IU is associated with any systemic disease, treatment of the underlying disease is mandatory Specific treatment: for treatable infectious diseases

24 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

25 Corticosteroids: MANAGEMENT MODALITIES 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

26 First-line therapy: Corticosteroids Unilateral IU: periocular injection of triamcinolone acetonide (Kenalog*, Kenacort*): -20 or 40 mg - Subtenon or orbital floor MANAGEMENT - Good response in most patients - Can be repeated if necessary Courtesy, Carlos Pavesio

27 MANAGEMENT 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)

28 MANAGEMENT IU resistant to cortisteroid therapy, side effects: Immunosuppressive therapy: Cyclosporine A Methotrexate Azathioprine Other drugs Biologic agents

29 MANAGEMENT Vitrectomy: Required for the treatment of certain complications (vitreous opacification, RD, epiretinal membrane) Its effects on inflammation and CME remain a controversial issue

30 MANAGEMENT Peripheral laser photocoagulation, cryotherapy: extensive peripheral retinal ischemia with or without retinal new vessels

31 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

32 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

33 Thank you for your attention

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