Review Article
Terson's syndrome, the current concepts and management strategies: A review of literature

https://doi.org/10.1016/j.clineuro.2021.107008Get rights and content

Highlights

  • Terson's Syndrome now describes intraocular haemorrhage secondary to an acutely raised intracranial pressure.

  • Aneurysmal subarachnoid haemorrhage is commonly associated with Terson’s Syndrome.

  • If left untreated, Terson’s Syndrome may cause visual deterioration and sometimes permanent visual loss.

  • Conservative management is feasible in most patients. Early identification and vitrectomy can save vision in certain cases.

Abstract

Terson's Syndrome describes intraocular hemorrhage secondary to an acutely raised intracranial pressure (ICP). Although Terson’s Syndrome is common amongst patients with subarachnoid hemorrhage (SAH), it is underdiagnosed and often overlooked. This review discusses the current understanding of the etiopathogenesis, clinical features, and management of Terson's Syndrome and highlights the visual and prognostic implications to stress the importance of timely diagnosis and management.

The origin of intraocular hemorrhage in Terson’s Syndrome has been debated. A recognized theory suggests that an acutely raised ICP induces effusion of cerebrospinal fluid into the optic nerve sheath which dilates the retrobulbar aspect of the sheath in the orbit. Dilatation mechanically compresses the central retinal vein and retinochoroidal veins resulting in venous hypertension and rupture of thin retinal vessels. A commonly reported clinical feature is decreased visual acuity and blurred vision. These may be accompanied by symptoms of increased ICP including loss of consciousness and headache. Diagnosis is established using evidence from the clinical presentation, ophthalmoscopy, and, when required, imaging including B-mode ultrasound, CT, MRI, and fluorescein angiography. Terson's Syndrome is managed conservatively by observation for mild cases and with vitrectomy for bilateral cases and for patients whose hemorrhage has not spontaneously resolved after an observational period.

Terson's Syndrome can be used as a prognostic indicator of morbidity and mortality in underlying pathology like SAH. Fundoscopy of patients with SAH, acutely raised ICP or visual disturbance with unknown etiology can help establish a timely Terson’s Syndrome diagnosis. This may avoid the risk of permanent visual impairment.

Introduction

Intraretinal hemorrhage with subarachnoid hemorrhage (SAH) was first described by Moritz Litten in 1881 and named after Albert Terson in 1900, who described the Syndrome of vitreous hemorrhage in association with SAH in a patient [1]. The definition of Terson's Syndrome has since evolved after histopathological research revealed that intraocular hemorrhage in Terson's Syndrome could also be in the retinal, sub-retinal, and sub-hyaloid spaces and after identification of other etiologies (Fig. 1A). This warranted expansion to the historical definition; at present, Terson's Syndrome generally reflects an intraocular hemorrhage secondary to an acutely raised intracranial pressure (ICP), often associated with intracranial hemorrhage or traumatic brain injury (TBI) [2], [3], [4], [5], [6]. The presence of Terson's Syndrome in the setting of SAH is a poor prognostic sign. It is associated with worse clinical outcomes and higher mortality rate compared to a SAH presentation devoid of ocular manifestations [7], [8], [9], [10], [11].

Terson's Syndrome is often an overlooked complication of SAH and is underdiagnosed given ophthalmologic examination is not routinely performed and some patients are unable to convey their visual complaints due to their critical condition [12], [13], [14], [15]. However, given the prognostic implications on mortality and morbidity, identification and management of Terson's Syndrome can help preserve vision and circumvent complications associated with the disease.

The purpose of this review is to discuss the etiopathogenesis, clinical features, and management of Terson's Syndrome and to highlight the visual implications and importance of timely diagnosis and management.

Section snippets

Etiology

Terson's Syndrome is generally caused by or linked to conditions that trigger an acute increase in ICP [2], [4], [5], [16]. Both aneurysmal and non-aneurysmal origins have been described. The reported overall incidence of TS from all causes ranges between 5% and 42% [8], [10], [17], [18]. This broad variation in incidence can be explained by varying sample sizes of each study, varying definitions, and different diagnostic modalities used.

Clinical presentation

Patients show variable clinical presentations depending on the extent of intraocular bleeding and preceding neurological state [6].

Assesment and diagnosis

The diagnosis of Terson's Syndrome is made using clinical presentation and Fundoscopy. Imaging techniques may be useful in confirming and establishing the diagnosis. Fundoscopy using an indirect ophthalmoscope (Fig. 2) is the gold standard for diagnosis of vitreous or sub-hyaloid hemorrhage in Terson's Syndrome [58]. Direct ophthalmoscopy can be used in an emergency setting. Given the prognostic implications of Terson’s Syndrome on morbidity and mortality, ophthalmologic examination is

Management

The available treatment options for Terson's Syndrome include conservative ophthalmic treatment and surgery. The management algorithm for Terson's Syndrome is presented in Fig. 4.

Conclusion

Terson's Syndrome describes an intraocular hemorrhage secondary to an acutely raised ICP. Aneurysmal and non-aneurysmal etiologies have been identified. Diagnosis using clues from clinical presentation, fundoscopy, and imaging is vital as it can help preserve vision and avoid complications. Ophthalmologic examination is advised for all patients with SAH on hospital admission and those with acutely raised ICP or visual disturbance with unknown etiology. A period of observation should be

Funding statement

This study was not funded by any grant funding or industry support.

Disclosures

None.

Declaration of Competing Interest

None.

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      Citation Excerpt :

      The main monitoring parameters are visual acuity and the evolution of the intraocular hemorrhage, but above all, the early detection of the appearance of other elements compromising the visual prognosis. Monitoring is then clinical but also radiological by ocular ultrasound or even other explorations such as OCT. [2,14] If the hemorrhage does not resolve or does not progress well, a pars plana vitrectomy is indicated, especially for intravitreal hemorrhage [5,14]. However, a surgical approach is not possible in the presence of intraretinal hemorrhage [15].

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