Terson Syndrome

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 by Tahira Mathen, MD on November 1, 2014.


Disease Entity

Disease

Vitreous hemorrhage associated with subarachnoid hemorrhage was first described by German ophthalmologist Moritz Litten in 1881 and then in 1900 by French ophthalmologist Albert Terson (Czorlich, Litten, Terson).

Terson syndrome is now recognized as intraocular hemorrhage associated with subarachnoid hemorrhage, intracerebral hemorrhage, or traumatic brain injury (Czorlich). Hemorrhage may be present in the vitreous, sub-hyaloid, or intraretina/sub-internal limiting membrane.

Epidemiology

Terson syndrome occurs in 8-19.3% of subarachnoid hemorrhages (Iulano, Michaelewska, Morris, Czorlich), 9.1% of intracerebral hemorrhages, and 3.1% of traumatic brain injury (Czorlich). 5.5% of vitreous hemorrhages not caused by diabetes or trauma are caused by Terson syndrome (Verbraeken).

Terson syndrome usually occurs in adults but has been reports in children as young as 7 months (Bhardwaj, Kapoor). It can be unilateral or bilateral (Ritland).

Pathogenesis

Subarachnoid blood may be transmitted forward through the optic nerve sheath. (Iuliano, Czorlich). A sudden increase in intracranial pressure leads to rapid effusion of CSF into the optic nerve sheath. Dilation of the retrobulbar optic nerve mechanically compresses the central retinal vein. Venous hypertension results in rupture of thin retinal vessels. This mechanism is consistent with the fact that Terson syndrome can be seen in patients without intracranial hemorrhage (Gress).

Fluorescein angiography has demonstrated a leakage site at the disc margin in a patient with TS with vitreous hemorrhage. This suggests damage to peripapillary retina induced by increased intracranial pressure transmitted through the optic nerve sheath (Ogawa).

Etiology

Terson syndrome has been reported to be caused by or associated with multiple conditions associated with a spike in intracranial pressure including carotid artery occlusion, cortical venous sinus thrombosis (Takkar), moyamoya disease (Kim, Arakwawa), epidural saline injection (Naseri), intraarterial angiography, lumbosacral myelomeningocele, iaotregnic during endoscopic third ventriculostomy (Hoving).

Relationship to aneurysm site

There is conflicting data on aneurysm site in SAH and Terson syndrome. Fountas found that anterior circulation aneurysms are more likely to be associated with Terson syndrome (Fountas) and two other studies found that anterior communicating artery aneurysms in particular are associated with a higher rate of TS (Fahmy, Garfinkle). Other studies have shown no correlation between site of aneurysm and TS (Czorlich) or a negative correlation between presence of anterior communicating artery aneurysm and TS (Stienen). There is also no relationship between the location of the aneurysm and which eye is affected by Terson syndrome (Czorlich, Fahmy, Fountas, Pfausler, Sung).

Clinical Presentation

TS can present with dome-shaped hemorrhages in the macula (Friedman). A macular “double ring” sign. The inner ring is cause sub-ILM hemorrhage and the outer ring is caused by sub-hyaloid hemorrhage (Srinivasan).

Although intraocular hemorrhages most frequently develop in the first hour after SAH (Manschot), Terson syndrome can have a delayed onset, with reports of intraocular hemorrhage occurring up to 47 days after SAH (Vanderlinder, Czorlich).

Prognostic factors

Low Glasgow coma scale, high Hunt and Hesse grade, and high Fisher grade are associated with a higher incidence of Terson syndrome (Czorlich).

Neurological outcomes and mortality rate are worse in patients with SAH and Terson syndrome than patients with SAH alone (Czorlich, Fountas, Manschot, Pfausler, Shaw&Landers, Vanderlinden, Stienen). In a study by Pfausler, mortality was 90% in pts with SAH and TS and 10% in those with SAH without TS (Pfausler). In a study by Gutierrez Diaz, mortality was 50% when TS present, and 20% when absent (Gutierrez Diaz).

Diagnosis

Funduscopic exam is the gold standard for diagnosis of Terson syndrome. Loss of red reflex is seen in 20% of eyes with Terson syndrome (Czorlich).

Diagnosis may be delayed due to inability to dilate pupils due to need for neurologic monitoring. Patients may also have cognitive impairment that prevents them from verbalizing visual complaints or complying with visual testing (Ashrafi). Median time from visual symptoms to referral to an ophthalmologist was 5.2 months for unilateral cases and 4.9 months for bilateral cases in a series of 17 patients with TS (Gnanaraj).

Swallow investigated the use of orbital CT to indentify intraocular hemorrhage in patients with Terson syndrome. Retinal crescentic hyperdensities and retinal nodularity were seen in CT in two-thirds of patients with Terson (Swallow, Kim).

Treatment

Intraocular hemorrhage frequently resolves spontaneously. Kapoor). Vision loss is usually reversible but permanent impairment of vision can occur (Roux, Kapoor). Half of vitreous hemorrhage had not resolved after 19 months (Shaw and Landers 1975).

Proliferative vitreoretinopathy, preretinal fibrosis (van Rens), retinal detachment (9%), retinal folds/perimacular folds(20%) (Sharma)(Keithahn), and epiretinal membrane (after Terson syndrome have been reported (Mena, Velikay, Kim, Garweg). 2 patients have also had macular holes which were found intraoperatively during PPV for VH (Rubowitz). Ghost cell glaucoma (4%) (Gnanaraj).

Epiretinal membrane is the most common sequelae of TS (Schultz). Epiretinal membranes have an incidence of 15-78% in eyes with Terson syndrome (Rubowitz, Yokoi, Sharma, Ritland, Schultz). Vitreous blood has been shown to cause ERMs in animal models, possibly by inducing glial proliferation and disruption of the ILM (Rubowitz, Sharma, Augsten).

There is no consensus on optimal timing for vitrectomy in Terson syndrome. IOH can be observed for up to 3 months before considering pars plana vitrectomy (czorlich, fountas, augsten). If bilateral or in a young child at risk of amblyopia may proceed to surgery sooner (Garweg, Czorlich). Augsten recommend operating on withing 4-8 weeks after the injury in patients with bilateral TS (Augsten). In a study of 36 eyes with TS, eyes that were operated on within 90 days of occurrence of VH had better final VA than eyes that were operated on after 90 days (Garweg). Patients younger than 45 had better final VA than patients older than 45 (Garweg). Retinal detachment secondary to PVR developed in 4/36 patients between 6 and 27 months after PPV (Garweg).

In a study of 7 eyes of 6 patients that underwent pars plana vitrectomy for Terson syndrome, median VA went from HM to 20/25 and no complications were observed (czorlich). S/p PPV, Rapid visual improvement in 96% and better than 20/30 vision in 81% (Kuhn). 93% had final VA of 20/40 or better in a series of 15 eyes s/p PPV vfor VH (Sharma). (ILM peeling has also been used (Abdelkader)

There was no difference in final visual acuity between patients who were conservatively managed and thoser who underwent PPV. However, visual recovery was more rapid in the vitrectomy group despite these patients having denser vitreous hemorrhage (Schultz).

Intravitreal tpa and gas have been used for recalcitrant Terson syndrome (Seracarbasse, Kapoor).

1 patient with Terson syndrome and premacular subhyaloid hemorrhage was treated with Nd-YAG to puncture the posterior hyaloids face and allow drainage of blood intot he vitreous. VA improved from 20/400 before treatment to 20/20 1 month after treatment (Ulbig).

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References

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