Nadeem Atwan MBBS¹

¹ – St. George’s University of London

Introduction

Until recently, Terson’s syndrome (TS) was defined as the development of vitreous hemorrhage occurring in tandem with a subarachnoid hemorrhage (SAH). However, the definition has been expanded to include SAH, increased intracranial pressure (ICP), traumatic brain injury, and any form of intracerebral hemorrhage (1). More than 90% of vitreous hemorrhage cases are caused by either direct trauma to the head and orbit or by diabetes; less than 6% are caused by Terson’s (2). TS affects men and women equally and has been reported in infants as young as 7 months of age (3,4).

This article will serve as a brief summary on the proposed pathophysiology, diagnosis, clinical implication, and management of TS.

Pathophysiology

There are two proposed mechanisms of the pathophysiology of TS. The first relates to the original definition of the syndrome, but this does not by any means imply that it is of less credibility. When the blood-brain barrier is disrupted and a subarachnoid hemorrhage ensues, a direct leak of blood can occur through the optic nerve into the vitreous cavity. This is possible due to the anatomical continuity between the optic nerve and cerebrospinal-fluid space (CSF) of the cranium. This anatomical continuity serves the surgical basis to optic nerve sheath fenestration as a treatment option for idiopathic intracranial hypertension (IIH).

The second suggested pathophysiology actually pertains to cases of increased ICP regardless of the presence of an intracranial bleed or SAH. It states that the increased ICP creates a pressure gradient that will cause an influx of CSF into the optic nerve sheath, this in turn will compress the central retinal vein which ultimately leads to hemorrhage from tiny retinal capillaries (5).

Diagnosis

As the gold standard to assess for any signs of an intracranial bleed or raised ICP is a CT scan of the head, TS can be diagnosed by diligently inspecting the posterior segment of the globe. Another method of diagnosis would be direct examination as is done using an indirect ophthalmoscope or during comprehensive slit lamp assessment.

Clinical Implication

The importance and clinical relevance of TS is profound. Firstly, the positive predictive value of vitreous hemorrhage with SAH is relatively high, 83.3%; however, the poor negative predictive value of anywhere between 10 and 40% is rather a weak tool to rule out intracranial hemorrhage (6).

The second, and likely most important, element is the prognostic implication of diagnosing TS. A mortality rate of 90% was reported in patients with SAH TS vs a mortality rate of 10% in patients with SAH without vitreous hemorrhage (7). There are differing results among other studies, but the data clearly indicates that the presence of vitreous hemorrhage on a background of SAH significantly increases the risk of death.

Management

As a diagnosis, the treatment plan for vitreous hemorrhage depends on many features such as cause (e.g., proliferative diabetic retinopathy), amount, timing, and prior events in the same eye. Minor vitreous hemorrhages can resolve spontaneously.

Interestingly, the literature data suggests that >95% of patients with vitreous hemorrhage in TS who undergo par-plana vitrectomy had rapid and significant visual improvement (8). This substantially favorable outcome is achieved when pars-plana vitrectomy is performed within 3 months post vitreous hemorrhagic events in TS. Delays beyond 90 days seem to reduce the success rate of restoring visual improvement; average around 90 days was 20/30 vision but delays up to 6 months reduced visual acuity correction to about 20/40 and 20/50 on average (8).

Conclusion

TS is increasingly becoming a more important differential diagnosis among radiologists and ophthalmologists. A key history in the ophthalmic clinic is vital, as incidental visualization of a vitreous hemorrhage may require consultation with medics, radiologists, and stroke teams if a intracranial hemorrhage is suspected. If TS is diagnosed, it is also imperative to consider the definitive treatment, pars-plana vitrectomy, in the critical time window to maximize visual acuity return to the patient.

References

1. Czorlich P, Skevas C, Knospe V, Vettorazzi E, Richard G, Wagenfeld L, Westphal M, Regelsberger J. Terson syndrome in subarachnoid hemorrhage, intracerebral hemorrhage, and traumatic brain injury. Neurosurg Rev. 2015 Jan;38(1):129-36; discussion 136. doi: 10.1007/s10143-014-0564-4. Epub 2014 Aug 31. PMID: 25173620.

2. Conart JB, Berrod JP. Hémorragies du vitré non traumatiques [Non-traumatic vitreous hemorrhage]. J Fr Ophtalmol. 2016 Feb;39(2):219-25. French. doi: 10.1016/j.jfo.2015.11.001. Epub 2016 Jan 27. PMID: 26826742.

3. He T, Wu LN, Chen B, Xing YQ. [Incidence and relative factors of Terson syndrome in patients with aneurysmal subarachnoid hemorrhage]. Zhonghua Yan Ke Za Zhi. 2011 Dec;47(12):1096-101. Chinese. PMID: 22336119.

4. Bhardwaj G, Jacobs MB, Moran KT, Tan K. Terson syndrome with ipsilateral severe hemorrhagic retinopathy in a 7-month-old child. J AAPOS. 2010 Oct;14(5):441-3. doi: 10.1016/j.jaapos.2010.06.009. Epub 2010 Sep 25. PMID: 20869894.

5. Gress DR, Wintermark M, Gean AD. A case of Terson syndrome and its mechanism of bleeding. J Neuroradiol. 2013 Oct;40(4):312-4. doi: 10.1016/j.neurad.2013.07.003. Epub 2013 Oct 4. PMID: 24095292.

6. Joswig H, Epprecht L, Valmaggia C, Leschka S, Hildebrandt G, Fournier JY, Stienen MN. Terson syndrome in aneurysmal subarachnoid hemorrhage-its relation to intracranial pressure, admission factors, and clinical outcome. Acta Neurochir (Wien). 2016 Jun;158(6):1027-36. doi: 10.1007/s00701-016-2766-8. Epub 2016 Apr 1. PMID: 27038169.

7. Pfausler B, Belcl R, Metzler R, Mohsenipour I, Schmutzhard E. Terson’s syndrome in spontaneous subarachnoid hemorrhage: a prospective study in 60 consecutive patients. J Neurosurg. 1996 Sep;85(3):392-4. doi: 10.3171/jns.1996.85.3.0392. PMID: 8751622.

8. Garweg JG, Koerner F. Outcome indicators for vitrectomy in Terson syndrome. Acta Ophthalmol. 2009 Mar;87(2):222-6. doi: 10.1111/j.1755-3768.2008.01200.x. Epub 2008 Jun 3. PMID: 18537934.