Posterior Segment
Retinitis Pigmentosa
Also known as: RP, rod-cone dystrophy, rod cone dystrophy, inherited retinal degeneration, inherited retinal dystrophy
Filed as: Soon
Source-cited from the record's own evidence list. Clinical content programme led by Dr Ankit Mathur, PhD, Grad Cert Ocu Thera, B.S. Optom. How the clinical content is governed
Clinical decision support only
Overview
A genetically and phenotypically heterogeneous group of inherited retinal degenerations, commonly rod-cone predominant, with night blindness and progressive peripheral visual-field loss and later central involvement in many patients. RP is not a single uniform disease or a label for every inherited retinal degeneration; early fundus findings may be subtle and absence of family history does not exclude inherited disease.
Recognition
Symptoms: what patients report
- Nyctalopia or delayed dark adaptation, often before acuity loss.
- Progressive peripheral-field restriction and difficulty navigating in dim conditions.
- Photopsia and glare sensitivity may occur.
- Central acuity can remain relatively preserved until later disease or a macular complication.
- Hearing loss or other systemic symptoms may indicate syndromic disease.
Signs: what the examination shows
- Bilateral, usually relatively symmetrical retinal dysfunction; early fundus appearance may be subtle.
- Mid-peripheral pigmentary change, retinal-vessel attenuation and optic-disc pallor may develop.
- Visual field may progress from a mid-peripheral ring scotoma to peripheral constriction and a residual central island.
- OCT may show outer-retinal/ellipsoid-zone loss and associated cystoid macular oedema.
- Cataract may contribute to visual decline.
- Marked asymmetry, inflammation or sudden change suggests a superimposed condition or acquired mimic.
What OptoGuide™ covers for retinitis pigmentosa
- Pattern reasoning: what this combination of findings points to, and its differentiators
- Don't-miss risks and escalation triggers
- Management tiers with linked Australian therapeutics
- Referral urgency, specialty, and letter drafting
Sources
Standard texts: Kanski's Clinical Ophthalmology: A Systematic Approach, The Wills Eye Manual, Oxford Handbook of Ophthalmology, Oxford American Handbook of Ophthalmology, Signs in Ophthalmology: Causes & Differential Diagnosis.