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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
Moncef Khairallah, Tunisia
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
Moncef Khairallah, Tunisia
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
Moncef Khairallah, Tunisia
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
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
Moncef Khairallah, Tunisia
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
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
• 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
• Idiopathic intermediate uveitis
with snowballs and/or snowbank
(SUN criteria)
Pars planitis
Intermediate uveitis
Moncef Khairallah, Tunisia
CLINICAL FINDINGS
• 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
• 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
• 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
Moncef Khairallah, Tunisia
ULTRASOUND BIOMICROSCOPY
Useful in patients with poor
dilation or opaque media
Allows clear visualization of
pars plana condensations (snow-
bank)
Intermediate uveitis
Moncef Khairallah, Tunisia
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
Moncef Khairallah, Tunisia
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
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
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
Moncef Khairallah, Tunisia
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
Moncef Khairallah, Tunisia
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
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
Moncef Khairallah, Tunisia
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
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
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
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
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
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
IU resistant to cortisteroid therapy, side effects:
Immunosuppressive therapy:
Cyclosporine A
Methotrexate
Azathioprine
Other drugs
Biologic agents
MANAGEMENT
Intermediate uveitis
Moncef Khairallah, Tunisia
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
Peripheral laser photocoagulation, cryotherapy:
extensive peripheral retinal ischemia with or
without retinal new vessels
MANAGEMENT
Intermediate uveitis
Moncef Khairallah, Tunisia
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
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
Thank you for your attention