Retinitis pigmentosa is a slow degenerative, hereditary disease of the retina that involves the rods and cones. It typically appears as a recessive trait due to consanguinity of the parents. Patients experience night blindness in childhood, tunnel vision or central visual loss in middle age, and complete blindness in advanced age. Physical examination shows black spots resembling bone corpuscles across the retina, extremely attenuated retinal blood vessels, and pale optic discs, indicating optic nerve atrophy. There is no specific treatment currently available, but cataract surgery and rehabilitation services can help manage complications.

1. RETINITIS
PIGMENTOSA
by Muhd Ariff B Mahdzub

2. • It is slow degenerative, hereditary disease of
the retina, involving the rods and cones.
• It appears as a recessive trait and usually
occurs due to consanguinity of the parents.

3. On History taking
• Age: it begins in childhood and causes
blindness in middle and advanced age.
• Side: It is a bilateral affection usually.
• The course is slow, chronic and progressive.

4. Cont.
• Patients complain of night blindness (early
sign),
• tunnel vision (peripheral visual loss) or
• central visual loss and photopsia (seeing
flashes of light). *advance case

5. On Physical examination
Fundus examination
• The retina is full with black
spots (pigments) which
resemble bone corpuscles
with a spidery outline.
• The retinal blood vessels—
Both arteries and veins,
become extremely
attenuated
• The optic disc—It shows
features of consecutive
optic atrophy, i.e. pale,
yellow wavy appearance

6. gradual increase in density of the pigment with
anterior and posterior spread, (advance case)
-Kanski page:647

7. • Peripheral pigmentation may become severe,
with marked arteriolar narrowing and disc
pallor
• -Kanski page:647

8. cont
• Dark adaptation is increased due to rods
dysfunction.
• The electroretinogram (ERG) and electro-
oculogram (EOG) are markedly subnormal or
completely extinguished early in the disease.

9. Complications
• Posterior subcapsular cataract (common in all
forms of RP),
• open-angle glaucoma (3%),
• keratoconus (uncommon)
• posterior vitreous detachment
Kanski page:646

10. Treatment
• No specific treatment is yet commercially
available
• Cataract surgery is generally benefical
• Regular follow-up to detect treatable
complications
• rehabilitation and social service support
• Smoking should be avoided.
Kanski page:646