Moncef Khairallah, MD - todnet.org · CME sequelae: lamellar or full-thickness macular hole...
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Moncef Khairallah, MD
Department of Ophthalmology,
Fattouma Bourguiba University Hospital
Faculty of Medicine, University of Monastir
Monastir, Tunisia
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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
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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
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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
Intermediate uveitis
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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
Intermediate uveitis
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
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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
Intermediate uveitis
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CLINICAL FINDINGS
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
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• Snowbank: frank white
exudation over the pars plana
and anterior retina
location: inferior retina, may
extend to involve the whole
periphery
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
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• Idiopathic intermediate uveitis
with snowballs and/or snowbank
(SUN criteria)
Pars planitis
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
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• Retinal vasculitis:
peripheral sheathing,
vascular leakage,
occlusion
• Cystoid macular edema:
the most common
complication of IU
• Optic disc swelling
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
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• FA is useful in the evaluation of:
macular edema, optic disc edema, retinal
vasculitis and its complications
(ischemia, neovascularization)
FLUORESCEIN ANGIOGRAPHY
Intermediate uveitis
Moncef Khairallah, Tunisia
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• Useful in patients with dense vitritis preventing fundus
examination:
- Evaluation of vitreous involvement
- Exclusion of a focus of retinochoroiditis
- Exclusion of a RD
ULTRASONOGRAPHY
Intermediate uveitis
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ULTRASOUND BIOMICROSCOPY
Useful in patients with poor
dilation or opaque media
Allows clear visualization of
pars plana condensations (snow-
bank)
Intermediate uveitis
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OCT provides detailed information about:
-Macular edema
-Subretinal fluid, epiretinal membrane, VRT, macular hole
- Sequential OCT examination is also useful on follow-up
OCT
Intermediate uveitis
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
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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)
Intermediate uveitis
Moncef Khairallah, Tunisia
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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
Intermediate uveitis
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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
Intermediate uveitis
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
DIAGNOSTIC APPROACH TO IU
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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
DIAGNOSTIC APPROACH TO IU
Intermediate uveitis
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
DIAGNOSTIC APPROACH TO IU
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If IU is associated with any systemic disease,
treatment of the underlying disease is mandatory
Specific treatment: for treatable infectious
diseases
MANAGEMENT
Intermediate uveitis
Moncef Khairallah, Tunisia
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
Courtesy, Carlos Pavesio
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
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IU resistant to cortisteroid therapy, side effects:
Immunosuppressive therapy:
Cyclosporine A
Methotrexate
Azathioprine
Other drugs
Biologic agents
MANAGEMENT
Intermediate uveitis
Moncef Khairallah, Tunisia
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Vitrectomy:
Required for the treatment of certain
complications (vitreous opacification, RD,
epiretinal membrane)
Its effects on inflammation and CME remain a
controversial issue
MANAGEMENT
Intermediate uveitis
Moncef Khairallah, Tunisia
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Peripheral laser photocoagulation, cryotherapy:
extensive peripheral retinal ischemia with or
without retinal new vessels
MANAGEMENT
Intermediate uveitis
Moncef Khairallah, Tunisia
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
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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
Intermediate uveitis
Moncef Khairallah, Tunisia
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Thank you for your attention