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Case Study
OF Retinitis Pigmentosa
By 3rd year Student
Shagufta Kadri (SY003)
From Aditya Jyot Institute of Optometry
Retinitis Pigmentosa
• Definition: The Primary Pigmentary retinal dystrophy
is a hereditary disorder predominantly affecting the
rods more than cones.
Retinitis Pigmentosa
• Inheritance: Most common mode is autosomal
recessive, followed by autosomal dominant, X linked
recessive is the least common.
• Incidence: 5 person per 1000
• Age: Appears in childhood and progresses slowly
• Race: No race is prone to it
• Sex: Males are commonly affected than female 3:2
• Laterality: Bilateral
Clinical features
• Ocular association: Myopia, POAG,
Micropthalmos, Keratoconous, Posterior
Subcapsular cataract vitreous detachment.
• Systemic association: Laurence-Moon Biedl
syndrome, Cockayne’s syndrome, Refsum’s
syndrome.
Clinical features
• Atypical RP:
RP punctate albescens
Sector RP
Pericentral RP
RP with exudative vasculopathy
Clinical features
• Fundus changes: The classical clinical triad of
RP are as follow
• Retinal arterioles are attenuated
• Optic disc become pale and waxy
• Retinal pigmentary changes: It show
perivascular ‘bone spicule’
• Other changes include Choroidal sclerosis,
CM, Atrophic or cellophane maculopathy.
Clinical features
Visual symptoms:
Bilateral involvement
Tunnel vision
Nyctalopia
Clinical features
• Visual field changes: It shows annular or ring
shaped scotoma which expands both peripherally
and centrally
• Electro physiological changes: ERG is subnormal or
abolished
• EOG shows absence of light
• Colour Vision: Normal
Case Presentation
Demographic data
• Name: XYZ
• Age: 27/F
• Add: Mumbai
• Occupation: Therapist
• Education: Studied in normal school
• Financial status: Middle class
• Marital status: Single
History
History
• H/O ocular disease: RP in (BE) since 10
years
• H/O Trauma: NiL
• H/O ocular medication: NiL
• H/O Glasses: Since 6 months
• Complaint: C/O cannot see far for distance
since childhood
History
• Systemic History: NiL
• Any Medication: NiL
• Allergic to medication: Not aware of
any.
• Accompanied by: Mother and Brother
History
• Family History: Positive H/O consangnuity.
1)Brother
Normal
2)Patient
RP
3)Sister
RP
4)Sister
RP
5)Brother
Normal
Task Related problem
• Distance Vn task problem: Patient cannot
see TV and Bus No.
• Intermediate Vn task problem:Difficulty in
scanning utensils while cooking.
• Near Vn task problem: Patient can’t read
normal print books.
Task Related Problem
• Mobility: Patient can’t go alone in new
place.
• Glare Problem: Delayed dark adaptation.
• Difficulty in Night
Quantitative Examination
Quantitative Examination
• PGP: (RE): -11.00DS/-1.50DC x 160
(LE): -14.00DS
• Vn(aided): (Distance)
• RE: 1.16 LogMAR
• LE: 1.28 Log MAR NI with PH
• Near Vn: RE: N36
• LE: N24 @12cm
Refraction
• Objective:
• Retino: (RE): -8.00/-0.75X180
• (LE): -11.00DS
• Subjective: Acceptance
• RE: -11.00DS/-1.50DCX 160 1.16LogMAR (Vn)
• LE: -14.00DS 1.28LogMAR (Vn)
Slit Lamp Examination
(BE)
Eyelid: Flat
Conjuctiva: Normal
Cornea: Clear
Lens: Clear
Ant Chamber: Normal
Fundus: pale optic disc,macula healthy,Bone
spicule pigmentary changes in periphery
Qualitative Examination
Qualitative Examination
• Contrast sensitivity: (Pelli Robson)
• RE: 0.15
• LE: 0.15 @1mt
• BE: 0.15
• Amsler chart: NA
• Colour Vision: Basic Colour discrimination
present
Qualitative Examination
• Visual Field: Confrontation
• RE LE
Confrontation test
• Area not seen
•
• Area seen
• Confrontation test is done at one arm distance
with patient’s corresponding eyes.
Trial
• Distance: Reverse Galilean telescope.
• Full Field telescope.
• Near: Stand Magnifier 3X 4X
• Hand aheld Magnifier 4X 5X
• Patient is comfortable with Stand magnifier 4X
Functional Adaptive devices
• Retinitis Pigmentosa patients are required to
use broad conical beam search light for
mobility.
• Reading stand for heavier books.
• Use foldable cane with light.
• Use large print books.
Rehabilitation Centre
• Low Vision Rehabilitation centre
Rehabilitation centre
• A low vision centre is incomplete without
Rehabilitation centre.
• Mobility and orientation training: To teach
orientation and mobility and equip one with
techniques to maintain independent lifestyle.
• Patient & Family Counselling/Psychological
consultation:
• The psychiatrist should counsel how to face about
the hardest challenge that is adapting to life with
impaired vision
Rehabilitation centre
• Vocational counseling: To help the patient
refer for his disability certification and to further
guide him regarding employment and placements.
• Funding issues: Patient should get help from
NGO or government in finding funding.
• Optical Aids: To be trained how to use
prescribed devices for near and distance.
• Features and Magnification of device should be
explained on how to use them.
Management
• Final Prescription:
• RE: -11.00DS/-1.50DCX 160 1.16LogMAR
(Vn)
• LE: -14.00DS 1.28LogMAR (Vn)
• Telescope:
• Near: (BE) Stand Magnifier with 4X N18
Management
• Vitamin A and lutien may slow the progress
• Genetic Counselling for no consanguinous
marriage
• Final: Follow up after 6 months
• Continue same glasses
Low vision case Study Of Retintis Pigmentosa

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Low vision case Study Of Retintis Pigmentosa

  • 1. Case Study OF Retinitis Pigmentosa By 3rd year Student Shagufta Kadri (SY003) From Aditya Jyot Institute of Optometry
  • 2. Retinitis Pigmentosa • Definition: The Primary Pigmentary retinal dystrophy is a hereditary disorder predominantly affecting the rods more than cones.
  • 3. Retinitis Pigmentosa • Inheritance: Most common mode is autosomal recessive, followed by autosomal dominant, X linked recessive is the least common. • Incidence: 5 person per 1000 • Age: Appears in childhood and progresses slowly • Race: No race is prone to it • Sex: Males are commonly affected than female 3:2 • Laterality: Bilateral
  • 4. Clinical features • Ocular association: Myopia, POAG, Micropthalmos, Keratoconous, Posterior Subcapsular cataract vitreous detachment. • Systemic association: Laurence-Moon Biedl syndrome, Cockayne’s syndrome, Refsum’s syndrome.
  • 5. Clinical features • Atypical RP: RP punctate albescens Sector RP Pericentral RP RP with exudative vasculopathy
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  • 8. Clinical features • Fundus changes: The classical clinical triad of RP are as follow • Retinal arterioles are attenuated • Optic disc become pale and waxy • Retinal pigmentary changes: It show perivascular ‘bone spicule’ • Other changes include Choroidal sclerosis, CM, Atrophic or cellophane maculopathy.
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  • 10. Clinical features Visual symptoms: Bilateral involvement Tunnel vision Nyctalopia
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  • 12. Clinical features • Visual field changes: It shows annular or ring shaped scotoma which expands both peripherally and centrally • Electro physiological changes: ERG is subnormal or abolished • EOG shows absence of light • Colour Vision: Normal
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  • 15. Demographic data • Name: XYZ • Age: 27/F • Add: Mumbai • Occupation: Therapist • Education: Studied in normal school • Financial status: Middle class • Marital status: Single
  • 17. History • H/O ocular disease: RP in (BE) since 10 years • H/O Trauma: NiL • H/O ocular medication: NiL • H/O Glasses: Since 6 months • Complaint: C/O cannot see far for distance since childhood
  • 18. History • Systemic History: NiL • Any Medication: NiL • Allergic to medication: Not aware of any. • Accompanied by: Mother and Brother
  • 19. History • Family History: Positive H/O consangnuity. 1)Brother Normal 2)Patient RP 3)Sister RP 4)Sister RP 5)Brother Normal
  • 20. Task Related problem • Distance Vn task problem: Patient cannot see TV and Bus No. • Intermediate Vn task problem:Difficulty in scanning utensils while cooking. • Near Vn task problem: Patient can’t read normal print books.
  • 21. Task Related Problem • Mobility: Patient can’t go alone in new place. • Glare Problem: Delayed dark adaptation. • Difficulty in Night
  • 23. Quantitative Examination • PGP: (RE): -11.00DS/-1.50DC x 160 (LE): -14.00DS • Vn(aided): (Distance) • RE: 1.16 LogMAR • LE: 1.28 Log MAR NI with PH • Near Vn: RE: N36 • LE: N24 @12cm
  • 24. Refraction • Objective: • Retino: (RE): -8.00/-0.75X180 • (LE): -11.00DS • Subjective: Acceptance • RE: -11.00DS/-1.50DCX 160 1.16LogMAR (Vn) • LE: -14.00DS 1.28LogMAR (Vn)
  • 25. Slit Lamp Examination (BE) Eyelid: Flat Conjuctiva: Normal Cornea: Clear Lens: Clear Ant Chamber: Normal Fundus: pale optic disc,macula healthy,Bone spicule pigmentary changes in periphery
  • 27. Qualitative Examination • Contrast sensitivity: (Pelli Robson) • RE: 0.15 • LE: 0.15 @1mt • BE: 0.15 • Amsler chart: NA • Colour Vision: Basic Colour discrimination present
  • 28. Qualitative Examination • Visual Field: Confrontation • RE LE
  • 29. Confrontation test • Area not seen • • Area seen • Confrontation test is done at one arm distance with patient’s corresponding eyes.
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  • 33. Trial • Distance: Reverse Galilean telescope. • Full Field telescope. • Near: Stand Magnifier 3X 4X • Hand aheld Magnifier 4X 5X • Patient is comfortable with Stand magnifier 4X
  • 34. Functional Adaptive devices • Retinitis Pigmentosa patients are required to use broad conical beam search light for mobility. • Reading stand for heavier books. • Use foldable cane with light. • Use large print books.
  • 35. Rehabilitation Centre • Low Vision Rehabilitation centre
  • 36. Rehabilitation centre • A low vision centre is incomplete without Rehabilitation centre. • Mobility and orientation training: To teach orientation and mobility and equip one with techniques to maintain independent lifestyle. • Patient & Family Counselling/Psychological consultation: • The psychiatrist should counsel how to face about the hardest challenge that is adapting to life with impaired vision
  • 37. Rehabilitation centre • Vocational counseling: To help the patient refer for his disability certification and to further guide him regarding employment and placements. • Funding issues: Patient should get help from NGO or government in finding funding. • Optical Aids: To be trained how to use prescribed devices for near and distance. • Features and Magnification of device should be explained on how to use them.
  • 38. Management • Final Prescription: • RE: -11.00DS/-1.50DCX 160 1.16LogMAR (Vn) • LE: -14.00DS 1.28LogMAR (Vn) • Telescope: • Near: (BE) Stand Magnifier with 4X N18
  • 39. Management • Vitamin A and lutien may slow the progress • Genetic Counselling for no consanguinous marriage • Final: Follow up after 6 months • Continue same glasses