Pars Planitis is a disease of the eye that is characterized by inflammation of the narrowed area (pars plana) between the colored part of the eye (iris) and the choroid. This may lead to blurred vision; dark, floating spots in the vision; and progressive vision loss.

1. PARS PLANITIS
Shah-Noor Hassan FCPS,FRCS(Glasgow)
Vitreo-Retina Consultant
Bangladesh Eye Hospital & Institute

2. History
• Cyclitis- Fuchs in 1908, Duke-Elder 1941
• Peripheral uveitis- Schepens-1950
• Peripheral cyclitis- Brockhurst et.al. - 1960
• Pars planitis- Welch et.al. - 1960
• Chronic cyclitis- Hogan & Kimura in 1961
• Vitritis- Gass et.al. - 1968
• Intermediate uveitis- IUSG- 1987
• SUN working group-2004

3. Nomenclature
• Standardization of Uveitis
Nomenclature working
group classification
• Idiopathic form of
intermediate uveitis
• Includes snowballs and
snowbanking
• If associated with diseases
like Sarcoidosis and Lyme
disease then included in
intermediate uveitis

4. Epidemiology
• 10-25 % of all the uveitis cases
• Children and young adults
• Can occur at any age
• Both sexes are equally affected
• 80% are bilateral
• Less in Chinese and Japanese population

5. Etiology
• Idiopathic
• No known hereditary or environmental factors
• Some isolated cases of familial pars planitis
• Associated with various systemic diseases
• Most common- multiple sclerosis, sarcoidosis

6. Pathogenesis
• Immune mediated response
• But the antigenic stimulus remains speculative
• Davis and colleagues
– Stage 1- immunologically mediated
– Stage 2- Non specific breakdown of intraocular
regulatory mechanisms
(Not necessarily an autoimmune mechanism but
even exogenous viral or bacterial antigens
may be responsible)

7. Pathogenesis
• Escape from regulatory control of Helper T cells
directed against these antigens
• Defective intraocular T cell regulation of B cells
• Decreased helper to suppressor T cell ratios in aqueous
and peripheral blood
• Other mechanisms
– Anterior chamber associated immune deviation
– Auto retinal antibodies
– Related to Demyelination
– HLA-DR15 and HLA-A28 positivity
– Nucleoporin like protien-nup36

8. Pathology
• Peripheral retina and ciliary body
demonstrate condensed vitreous ,
fibroblasts, spindle cells, lymphocytes
and blood vessels
• Prominent lymphocyte cuffing of retinal
veins
• Pars plana exudates
– Loose fibrovascular layer containing
scattered mononuclear inflammatory cells
and a few fibrocyte like cells
– Fibroglial tissue consists of vitreous
collagen, mullers cells and probable
fibrous astrocytes

9. Clinical features
• Floaters and hazy vision
• No pain, photophobia, redness
• First episode is associated with a more
severe and symptomatic iridocyclitis
• Subsequent episodes have a chronic
course…….
• One eye symptomatic other eye may be
asymptomatic and even show signs of
active disease

10. Presentation
• VISION LOSS
• CME, ERM
• PSC
• Vitreous Opacification
• Membranes
• Retinal Detachment
• Vitreous Hemorrhage

11. Presentation
• Cells, flare, KPs in AC, synechiae (Spill
over anterior segment inflammation)
• Snow balls (organized vitreous
inflammatory cells )
• Snow banking (exudates at pars plana)
• May be localised to inferior half

12. Presentation
• Peripheral vasculitis
• CME, Peripapillary retinal edema
• Vitritis, Cyclitis
• Vitreous hemorrhage
• Band shaped keratopathy

13. Effect on macula
• Macular edema (CME) and maculopathy (12-
82 %)
• Most common cause of visual loss
• Incidence increases with duration and severity
of disease

14. Vitreous involvement
• Vitritis
• Snowball formation
• Vitreous membranes and
floaters
• Vitreous hemorrhage

15. Retinal involvement
• Retinal vascular changes
– Tortuosity of arterioles and venules
– Peripheral vascular sheathing
(Periphlebitis-16-36 %)
– Neovascularizations (6.5%)
– Retinal detachment (2.2-51 %)
• Causes of RD
– Vitreous traction due to long standing
inflammation and subsequent hole
formation
– Exudative detachment secondary to
uvietis inflammation

16. Optic nerve involvement
• Disc edema- 3-38%
• Optic neuritis with or without multiple
sclerosis was seen in 7.4 %

17. Complications
• Glaucoma
– Acute uveitis- 7.6 %
– Chronic – 6.5% at one year, 11.1 at 5 years
• Causes of glaucoma
– Active inflammation
– Steroid usage
– Increasing age
– Number of years since diagnosis

18. Cataract
• 15-50% of eyes
• Posterior or anterior subcapsular
• At times posterior cortical even posterior
polar have been reported
• Incidence increases with duration and
severity of disease
• If treated earlier with immunosuppressive
rather than corticosteroids cataract
formation is less severe

19. Types Of Retinal Detachment
• Exudative RD in 5-17%
• Vitreoretinal traction - in 3-22% TRRD
• Brockhurst and Schepens – 4 types of RRD
Type I:
- Low lying, chronic, associated with demarcation
lines
- Small breaks near ora with exudates
- Benign course

20. Types Of Retinal Detachment
Type II:
- Large dialysis at the posterior edge of the pars plana exudate
- Slowly progressive
- May resolve spontaneously if VR exudation occludes the break
- Seen in pts with a mild chronic inflammatory course
Type III:
- Rapidly progressive
- Large breaks associated with NVVB and circumferential pars
plana exudates.
- Associated with severe chronic uveitis.

21. Pars planitis in children
• More so as an intermediate uveitis
• JIA most common cause (30%)
• 1.8-29% of all uveitis
• Of which 25 % are pars planitis
• Mean age 8.5-10.9 years
• Male preponderence
• Bilateral 84-94 %
• Resolves over several years
• Severe visual loss is uncommon

22. STANDARDIZATION OF UVEITIS
NOMENCLATURE

23. Natural course
Self limited
10 %
Smoldering
59%
Recurrent
31 %

24. Diagnosis
CLINICAL FEATURES
OPHTHALMIC
INVESTIGATIIONS
TO RULE OUT SECONDARY
CAUSES

25. Diagnosis: Clinical
• History
• Clinical findings
• Duration of symptoms, recurrences
• Fever , fatigue or night sweats are typical signs -
Sarcoidosis & TB
• Loss of sensitivity or paresthesias of hands, arms
or legs - Multiple sclerosis
• Dermatitis, Arthritis– Lyme
• Contact with cats – possibility of Bartonella
infection

26. Ophthalmic investigations
• V/A
• SL biomicroscopy
• IOP and
• Fundus examination with scleral depression
• Amsler grid

27. • OCT - Macular oedema
• Fluorescein Angiogram-
Vasculitis ,CNP areas ,
New vessels & CME
• B scan (Hazy media)
• UBM
• Diagnostic vitrectomy
Ophthalmic investigations

28. To rule out secondary causes…
• Complete hemogram
• ELISA for tuberculosis and toxoplasma
• CXR
• Galium Scan and Chest CT
Lab Inv:
- ACE levels- elevated in 60-90% of active sarcoid
patients
- Lysozyme level - Elevated in granulomatous disorders
viz sarcoid, TB, and leprosy
- Elevated antibody titre against Borrelia burgdorferi
• Sarcoidosis
• Tuberculosis.

29. Differential diagnosis
• Non infectious
– Multiple sclerosis (3-27 %)
– Sarcoidosis (23-26%, IU
developing sarcoidosis- 2-
10%)
– Intraocular lymphoma
(PCNSL- 10-20% have
vitreous inflam)
• Infectious conditions
– Tuberculosis
– Syphilis (10.3%)
– Lyme disease
– Toxoplasma
– Toxocariasis
– HTLV-1, EBV, Cat scratch
disease
– Endogenous
endophthalmitis
– ARN, Eales, VKH, Fuch’s

30. MANAGEMENT

31. Four Step Approach (Kaplan et al)

32. Modified 5 step program: S.Foster et al
Topical +/ Periocular corticosteroids
Oral +/ Topical NSAID
After 3rd injection
Systemic C steroids
Inflammation persists or recurs
Peripheral retinal cryopexy /BIOL
Recur following 6th regional steroid injection
PPV/ Immunosupression
Recalcitrant inflammation

33. Addition of systemic steroid or
immunosuppressive agents
Periocular
steroid
Cryo or
peripheral LASER
Vitrectomy

34. Corticosteroids
• Drop in VA due to vitritis, CME, progression of
neovascularization at the vitreous base
• Periocular steroids-
– Long acting Methyl prednisone (40 mg )
– Triamcinolone acetonide (20 mg)
• Complications-
– Glaucoma
– Cataract
– Aponeurotic ptosis
– Enophthalmos
– Orbital scarring

35. Corticosteroids
• IVTA can be given in cases of severe macular
edema
• Complications
Cataract
Glaucoma
Endophthalmitis

36. Oral steroids
• Indicated if the disease activity is not controlled with
periocular steroids
• Prednisolone 1 mg/kg/day tapered once response occurs

37. Immunosuppressive agents
• Antimetabolites : Methotrexate , Azathioprine
• Alkylating Agents : Cyclophosphamide ,
Chlorambucil
• Immunomodulators : Cyclosporine , Tacrolimus
• Complications
– GI upset
– Hepatotoxicity
– Bone marrow suppression

38. Methotrexate
• Folate analogue which inhibits dihydrofolate
reductase
• 7.5-25 mg per week oral/subcutaneous
• Can also lead to pneumonitis
• Effective and safe for chronic anterior and IU
in children

39. Azathioprine
• Purine nucleoside analogue
• Alters purine metabolism
• 50-150 mg per day
• GI upset and hepatotoxicity
Mycophenolate mofetil
• Inhibits purine synthesis
• Prevents replication of T and B lymphocytes
• 1-3 mg per day
• Mycophenolate is faster amongst the 3 in controlling
inflammation

40. Inhibitors of T-cell signaling
• Cyclosporine and Tacrolimus
– Inhibit NF-AT (Nuclear Factor of Activated T-cells )
– Nephrotoxicity and hypertension are important
complications
• Biological response modifiers
– Daclizumab
– Infliximab
– Eternacept
– Interferon alpha

41. Biological response modifiers
• Daclizumab
– Humanized monoclonal ant-IL-2 receptor alpha
antibody
– Suppresses auto reactive T-cells
– 1 mg/kg IV every 2 weeks for 5 doses
– Increase risk of infection

42. Biological response modifiers
• Infliximab
– Binds to TNF and prevents its action on
target tissues
• Eternacept
– Dimeric, fully human, soluble TNF receptor
– Binds tightly and specifically to circulating
and cell-bound TNF
• Adalimumab
– Can be self administered as a subcutaneous
injection
– Fully humanized so less chances of
antibody formation
• Disseminated tuberculosis is one of the
fatal complications

43. Newer steroid implants
• Retisert
– Fluocinolone acetonide implant
– Duration of 30 months
• Ozurdex
– Dexamethasone implant

44. Ablative procedures
• Failed drug therapy
• At times cryotherapy is preferred before
immunosuppressive Rx
• Aim
– To treat neovascularization associated with the
exudates
– To destroy the peripheral vessels which bring in
the inflammatory mediators

45. • Double row ,single freeze
• Apply to pars plana and posterior to it
• CONFLUENT BURNS
• Extend 1 clock hr on either side of all areas
affected by inflammation
• EFFECTS
– Decreases vitritis and improves VA
– Decrease in fluorescein in the treated area
– Induce regression of this NVVB and
consequently stabilize inflammation
Cryo ablation

46. LASER ablation
• LASER photocoagulation works as effective as
cryo
• 3-4 rows of burns are placed at the pars plana
and peripheral retina
• Works on the same mechanism as cryo

47. Vitrectomy
• Vitrectomy for uveitis began in late 1970s
• Aims
– Get rid of inflammatory mediators and immunologically
competent cells
– Clear the media
• Indications
– Refractory uveitis
– Vision loss due to densely opacified vitreous
– Scar tissue pulling on ciliary body causing hypotony
– CME, ERM
– Dense PCO
– TRD

48. MANAGEMENT OF CATARACT:
• Eye - quiet for 3 months
– Preoperative – Start steroids 3 days prior
– Postoperative - slow taper.
• Technique –
– As preferred by surgeon
– Minimal trauma
– Preferably heparin coated IOL

49. What’s new….
• Anti VEGF agents are being evaluated in cases
of uveitis with macular edema
• Lucentis and Avastin have been proved to be
effective in cases of uveitic CME

50. Nevanac in pars planitis
• Case 1: - Short term benefit in cases of
recurrent intermediate uveitis

51. Case 2
• Rapid resolution of vitritis in uncomplicated
case of intermediate uveitis

52. Case 3
• Fresh case of pars planitis with CME
• Nevanac improved the CME

53. Summary
• Examination of pars plana
• Diagnose macular edema
• Rule out secondary causes
• Plan appropriate treatment modility
• Bold use of steroids and immunosuppressive
agents to prevent vision loss due to macular
involvement
• Look out for complications
• Surgical management in resistant cases and to
clear the media

54. Thank you…