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Terson's syndrome is a condition where eye haemorrhages occur due to intracranial bleeding, most often associated with subarachnoid haemorrhage (SAH), commonly from a ruptured cerebral aneurysm. Patients may experience blurred vision, floaters, or complete vision loss due to retinal or vitreous haemorrhage, and neurological symptoms like severe headaches, nausea, seizures, and confusion may also arise. Diagnosis is challenging as the eye bleeding can resemble other conditions, such as diabetic retinopathy or retinal vein occlusion. A fundoscopic exam is the primary diagnostic method, but imaging like CT, MRI, and OCT can aid in confirming the diagnosis. Treatment involves managing intracranial pressure and haemorrhage, with options like vitrectomy or anti-VEGF injections for persistent eye bleeds. The prognosis depends on the severity of both neurological and ocular damage, with early intervention improving recovery chances. However, recurrence risks exist depending on the underlying cause of the haemorrhage. Research continues on improving early diagnosis, surgical approaches, and understanding the genetic and molecular factors influencing the disease.
History of the Disease: An instance of intraretinal hemorrhage coexisting with subarachnoid hemorrhage (SAH) was initially documented by German ophthalmologist Moritz Litten in 1881. Later, in 1900, French ophthalmologist Albert Terson reported a link between SAH—later known as Terson syndrome—and vitreous hemorrhage. This syndrome includes many kinds of intraocular hemorrhages, usually caused by a sudden increase in intracranial pressure (ICP), which can happen in combination with SAH, intracerebral hemorrhage, or traumatic brain injury.
The vitreous, sub-hyaloid, subretinal space, intraretinal regions, or beneath the internal limiting membrane are some of the locations where the hemorrhages may occur in the eye. According to Dr. Hayreh, the rupture of capillaries in the optic nerve, which is most likely brought on by elevated retinal venous pressure from central retinal vein compression, is the source of these retinal hemorrhages. The intricate vascular connections between the brain and the eye are highlighted by the fact that Terson syndrome is most frequently observed after aneurysmal subarachnoid hemorrhages. This emphasizes how crucial it is to diagnose and treat the illness using a thorough, interdisciplinary approach. Terson syndrome occurs in about 13% of SAH patients, especially in more severe instances (shown by a higher Hunt-Hess score), and these cases are linked to a higher risk of death.
Signs and Symptoms: The severity of the vision-related problems that people with Terson syndrome encounter varies greatly. The main symptom, which frequently affects one or both eyes, is an abrupt loss of vision or blurred vision brought on by bleeding in the vitreous cavity, which is the gel-like region that fills most of the eye. The degree of vision impairment in the affected eye can range from slight blurriness to total blindness, depending on the blood volume.
Additional symptoms could include light sensitivity and floaters, which are small dots or forms that appear to move across the visual field. Some individuals may not immediately notice changes in their vision if the bleeding is minor or just affects one eye. However, visual impairments are usually more obvious and can significantly affect daily activities when both eyes are affected.Severe neurological events, especially subarachnoid hemorrhage (bleeding around the brain) or elevated intracranial pressure, are frequently linked to Terson syndrome. Because of this, people may also have symptoms associated with certain brain disorders, such as severe headaches, nausea, vomiting, or stiff necks, which may come before visual problems and act as early warning indicators.
Although there is proof that brain bleeding and eye hemorrhage are related, it is still unknown why some people have Terson syndrome and others do not. More study is necessary to identify at-risk patients since the exact reason why increased brain pressure can sometimes result in ruptured blood vessels in the eye is still unclear. Additionally, even though treating eye hemorrhages can enhance vision, the long-term effects are still unknown, particularly if the diagnosis is delayed. A comprehensive assessment of the brain and eye is necessary to diagnose Terson syndrome. Optical Coherence Tomography (OCT) offers precise cross-sectional images of the retina to verify the existence of hemorrhage, whereas fundoscopy is frequently utilized to find bleeding in the vitreous cavity or on the retina.
An eye ultrasound can assist in visualizing internal bleeding when eye bleeding is suspected but not yet visible. Brain imaging using CT or MRI scans is usually done to look for underlying cerebral hemorrhage or high pressure within the skull because Terson syndrome is frequently associated with serious brain diseases including subarachnoid hemorrhage.
The standard diagnostic criteria and methods
Terson's syndrome is frequently diagnosed by combining neurological and ophthalmological testing. Fundoscopy, which enables direct evaluation of the retina for indications of bleeding, usually in the vitreous or retinal areas, is the main diagnostic technique. Further confirmation of intraocular and cerebral hemorrhages can be obtained using further imaging modalities. Ocular ultrasonography can be used to evaluate the anatomy of the eye when the hemorrhage prevents retinal vision. Terson's syndrome is characterized by hemorrhages beneath the internal limiting membrane, which can be detected by optical coherence tomography (OCT).
Despite being uncommon, fluorescein angiography may be useful in assessing retinal blood flow and detecting issues. To verify whether brain hemorrhages are present, neurological imaging is essential. In order to identify subarachnoid hemorrhage and other forms of cerebral bleeding, a CT scan is frequently the initial step. While MRA or CTA (magnetic resonance or computed tomography angiography) can be useful in identifying vascular abnormalities, such as aneurysms, that may have contributed to the hemorrhage, MRI can offer better detailed imaging of mild brain hemorrhages. An important diagnostic criterion is the temporal correlation between the cerebral hemorrhage and the commencement of ocular bleeding; because of increased intracranial pressure, the eye hemorrhage usually happens soon after the brain bleed.
Other diagnostic assessments include visual acuity tests to measure the impact on vision and lumbar puncture to confirm subarachnoid hemorrhage when imaging results are unclear. This comprehensive approach ensures that Terson’s syndrome is accurately diagnosed and managed.