Inherited retinal degeneration (IRD) may occur in isolation or as part of a multi-systemic condition. Ocular manifestations may be the presenting symptom of a syndromic disease and can include retinitis pigmentosa, cone-rod dystrophy, or maculopathy. Alternatively, patients affected with syndromic disease may already have other systemic manifestations at the time retinal disease is diagnosed. Some of these systemic diseases can cause significant morbidity. Here, we review several of these syndromic IRDs and their underlying genetic causes. Early recognition and referral for systemic evaluation and surveillance may lead to early intervention and an improved outcome. Obtaining a molecular diagnosis can be beneficial in securing a definitive diagnosis, especially in cases with atypical presentations. A genetic diagnosis may also be informative with regard to prognosis and potential therapies. Effective management and rehabilitation for patients with syndromic retinal dystrophy requires a comprehensive genetic-based team approach involving patients, family members, ophthalmologists, primary care physicians, and geneticists [1]. The prognosis and viable treatments may also be revealed by a genetic diagnosis. Syndromic retinal dystrophy patients need comprehensive genetic-based team management and rehabilitation, which includes patients, families, ophthalmologists, primary care doctors, and geneticists [2].

An obstruction in the aqueous humor’s outflow between the anterior and posterior chambers of the eye causes acute angle closure, an emergency ophthalmic disorder that, if untreated, can cause secondary visual neuropathy and an abrupt increase in intraocular pressure. The most frequent primary cause in patients with pre-existing narrow angles, such as those who are long-sighted, is a pupillary block. The usage of frequently prescribed drugs is one such secondary cause of angle closure that has to be made more widely known. To rule out other potential confounding illnesses, particularly those with CNS origins, that appear similarly, a thorough questioning is necessary [1]. All patients presenting with abrupt onset of red eye along with pupillary dilatation, dull pain, and headache should have angle closure excluded. A fundamental eye exam should involve a thorough neurological examination, measurement of intraocular pressure, and evaluation of the anterior segment under a strong light. When there is a strong clinical suspicion of acute angle closure, systemic ocular hypotensive therapy must be administered as away in order to protect the optic nerve and reduce vision loss. To specify definite care, an immediate referral to an ophthalmologist is required [2].

Anterior ischemic optic neuropathy (AION) and posterior ischemic optic neuropathy are the two main kinds of ischemic optic neuropathies (IONs) (PION). AION is made up of both artery-related AION (A-AION: caused by giant cell arteritis) and artery-free AION (NA-AION: due to other causes). PION is divided into three categories: surgical PION, non-arteritic PION (NA-PION), and arteritic PION (A-PION: caused by giant cell arteritis) (a complication of several systemic surgical procedures). These five forms of ION are unique clinical entities from the perspectives of aetiology, pathogenetically, clinically, and treatment. Because A-AION is an ophthalmic emergency that necessitates urgent treatment with high-dose steroid medication to prevent any additional visual loss in one or both eyes, it is imperative that patients aged 50 and older determine whether it is arteritic or not as soon as possible. When systemic corticosteroid medication was administered to NA-AION patients within the first two weeks of start, they had considerably better visual outcomes than untreated patients. Systemic risk factors, in particular nocturnal arterial hypotension, are important contributors to the development of NA-AION; hence, controlling these factors is crucial to the disease's prevention and treatment. When given high-dose systemic steroid therapy in the very early stages of the disease, NA-PION patients' visual acuity and visual fields significantly improved when compared to untreated eyes. In order to stop any additional vision loss in one or both eyes, A-PION, like A-AION, requires prompt treatment with high-dose steroid therapy. Other than taking precautions to stop it from happening in the first place, there is no effective treatment for surgical PION.