Terson Syndrome

Overview

Terson syndrome is a rare but serious ophthalmologic condition characterized by intraocular hemorrhage, bleeding within the eye, associated with acute intracranial hemorrhage, most commonly subarachnoid hemorrhage (SAH) due to a ruptured cerebral aneurysm. First described by the French ophthalmologist Albert Terson in 1900, the syndrome highlights the close anatomical and physiological relationship between the brain and the eyes. The intraocular bleeding can occur in one or both eyes and may involve the vitreous body, retina, or subhyaloid space. Terson syndrome is a medical emergency because it often signals a life-threatening neurological event.

Causes

The primary cause of Terson syndrome is a sudden increase in intracranial pressure, typically due to:

• Subarachnoid hemorrhage (SAH) – Most commonly from a ruptured cerebral aneurysm.

• Intracerebral hemorrhage

• Traumatic brain injury – Causing sudden intracranial bleeding.

• Severe head trauma

The leading theory suggests that the rapid increase in intracranial pressure causes a secondary rise in pressure within the optic nerve sheath. This increased pressure is transmitted to the retinal and vitreous veins, resulting in venous rupture and intraocular hemorrhage. The bleeding can range from small retinal hemorrhages to dense vitreous hemorrhages that significantly impair vision.

Symptoms

Symptoms of Terson syndrome may vary depending on the extent and location of the intraocular hemorrhage. Since it is often discovered in the context of a neurological emergency, many patients may be unconscious or unable to communicate visual symptoms. In those who are alert, common symptoms include:

• Sudden loss or blurring of vision – Often in one eye, but can be bilateral.

• Visual field defects – Partial vision loss depending on hemorrhage location.

• Floaters or flashes of light – Due to blood in the vitreous body.

• Dark spots or shadows – Known as scotomas, caused by retinal or subhyaloid bleeding.

In unconscious or critically ill patients, diagnosis often relies on fundoscopic examination or imaging rather than patient-reported symptoms.

Diagnosis

Diagnosis of Terson syndrome involves identifying both the neurological event and the associated ocular hemorrhage. Diagnostic steps include:

Neurological Assessment:

• CT or MRI scan of the brain – To confirm subarachnoid hemorrhage, intracranial bleeding, or other neurological causes.

• Lumbar puncture – Sometimes performed to detect subarachnoid hemorrhage if imaging is inconclusive.

Ophthalmologic Evaluation:

• Fundoscopic examination – Performed using an ophthalmoscope to visualize retinal and vitreous hemorrhages.

• Ocular ultrasound (B-scan) – Useful when fundus visualization is obscured by dense vitreous hemorrhage.

• Ocular coherence tomography (OCT) – May help assess subhyaloid or retinal hemorrhages in milder cases.

Timely diagnosis is crucial, as Terson syndrome not only signals a serious intracranial condition but also requires ophthalmologic management to preserve vision.

Treatment

Treatment of Terson syndrome involves two main goals: managing the underlying intracranial pathology and addressing the intraocular hemorrhage.

1. Management of Intracranial Hemorrhage:

• Neurosurgical intervention – May be required in cases of ruptured aneurysm (e.g., clipping or coiling) or hematoma evacuation.

• Blood pressure control and ICU care – To stabilize the patient and prevent further brain injury.

2. Ophthalmologic Management:

• Observation – In mild cases, intraocular hemorrhages may resolve spontaneously over weeks to months.

• Vitrectomy – A surgical procedure to remove blood from the vitreous cavity, typically performed if hemorrhage is dense or persistent and causing significant vision loss.

• Intravitreal medications – Rarely used in this condition but may be considered if there is concurrent retinal pathology.

Ophthalmologic follow-up is essential for monitoring recovery and assessing the need for surgical intervention.

Prognosis

The overall prognosis of Terson syndrome depends heavily on the severity and outcome of the underlying intracranial event. Key factors influencing prognosis include:

• Neurological outcome – Survivors of subarachnoid or intracerebral hemorrhage may recover fully or have long-term deficits depending on the extent of brain injury.

• Ophthalmologic outcome – Visual recovery is generally good in cases where hemorrhage resolves spontaneously or is successfully treated with vitrectomy.

• Bilateral involvement – May significantly impact quality of life if not properly addressed.

Early recognition and appropriate multidisciplinary management greatly improve both survival and visual outcomes. Patients who recover neurologically and undergo timely eye care can often regain good visual function.