Primary prevention

Vitamin A deficiency is extremely rare in high-income countries (e.g., US), but is a serious public health concern among children and pregnant women in, or from, low- and middle-income regions (e.g., Africa, Southeast Asia).[17]​ Common sources of Vitamin A include dairy products, fortified fat spreads, carrots, sweet potatoes, and green vegetables (e.g., spinach, cabbage, broccoli).[18]

Primary prevention of vitamin A deficiency may be relevant in low-income countries, or in migrants from those countries, particularly for children and pregnant women.[11][19][20]​​​[21]​ In at-risk children, the World Health Organization continues to recommend vitamin A supplementation for children ages 6-59 months. One Cochrane review has reported that vitamin A supplementation was associated with a 12% reduction in all-cause mortality, and that it reduced the incidence of night blindness and bitot spots​​.[21]

Despite the increased risk of maternal mortality and poor pregnancy outcomes, UK guidance recommends that pregnant women, or those trying to conceive, avoid vitamin A supplementation and foods rich in vitamin A (e.g., liver or liver products). This is due to the risk of birth defects with high vitamin A intake.[22]​ However, one Cochrane review reported good-quality evidence for prenatal vitamin A supplementation to reduce maternal night blindness for women who live in areas where vitamin A deficiency is common, or who are HIV‐positive.[11]​ This should include those who migrate from these areas.

Secondary prevention

A healthy balanced diet should provide the recommended daily amount of vitamin A. Patients diagnosed with retinitis pigmentosa should consult their ophthalmologist and consider supplemental vitamin A.

Genetic screening is available for many of the inherited chorioretinal dystrophies. This may establish the pattern of inheritance and the risk of transmission to future children. It should be remembered that many genetic defects remain undefined, and that research into inherited chorioretinal dystrophies is ongoing.

All patients should undergo assessment by a visual rehabilitation team and be registered as visually impaired (if required). Even early vision loss may be associated with disability. Vision rehabilitation should focus on improving meaningful outcomes (i.e., reading, daily living activities, safety, community participation, and psychosocial well-being).[51]

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