Retinitis Pigmentosa 
An inherited disorder of pigment epithelium and receptor cells (recessive, dominant or x-linked inheritance)

Clinical Findings
- Night blindness, constricted visual fields
- Often good central vision, but constricted fields
- Retinal pigmentation – bone spicule
- Optic atrophy and retinal artery attenuation
- Electroretinogram abnormal from an early age- before there is any other evidence of abnormality erg information

 
Management
No therapy is available.

Retinitis pigmentosa information



Associated disorders

- Refsum’s Syndrome – R.P., deafness, elevated blood phytamic acid
- Bardet Biedl Syndrome – R.P., & obesity metal retardation, hypogonadism,
- Ushers syndrome – R.P. & deafness

Central Retinal Degenerations
Stargardts Muscular Dystrophy – decreased vision, pigmentary macular change (“beaten- bronze” appearance) with or without yellow fleck deposits. Recessively inherited.

Vitelliform Macular Dystrophy
– an “egg yolk appearance” at the macula.  Dominantly inherited.

Retinopathy Of Prematurity 
Infants born prematurely have an incompletely vascularized retina.  The exposure of this immature retina to high oxygen concentrations may result in abnormal vascular development.

In the acute phase an arterio-venous mesenchymal shunt is present in the peripheral retina at the junction of the vascularized and nonvascularised zones.  This may be visible ophthalmoscopically as an elevated ridge.

If the condition progresses intra vitreal fibrovascular proliferation may occur progressing even to an acute exudative retinal detachment.

Chronic changes secondary to contracture of this fibrovascular tissue may result in retinal folds, dragging of the optic disc, and even traction retinal detachment.  This retinal detachment and fibrous tissue amt be seen as a white retrolental opacity – retrolental fibroplasia.

Infants at risk require ophthalmic examination .  Those at risk are – less than 1500 gms at birth, severely ill babies, especially babies who required supplemental oxygen.

Supplemental oxygen levels should be restricted to a that level which is adequate to maintain cerebral and other vital functions.  Arterial oxygen levels must be monitored.  But even with the greatest care retrolental fibroplasia still occurs.