BASICS
DESCRIPTION
• Named after the French ophthalmologist Albert Terson who described the condition in 1900
• Originally defined as vitreous hemorrhage in the setting of subarachnoid hemorrhage
• Definition has evolved to include any intraocular hemorrhage associated with intracranial hemorrhage and increased intracranial pressure
EPIDEMIOLOGY
Incidence
Approximately 15% of patients with subarachnoid hemorrhage develop intraocular hemorrhage (1)[A].
RISK FACTORS
• Patients with higher grade intracranial hemorrhage are more likely to have Terson’s syndrome.
• The presence of Terson’s syndrome is associated with higher mortality and morbidity in patients with intracranial hemorrhage (2).
GENERAL PREVENTION
• Modify risk factors associated with intracranial hemorrhage
– Smoking cessation
– Treatment of hypertension
PATHOPHYSIOLOGY
• Mechanism is controversial with several competing theories
– Original theory suggested that intracerebral blood directly communicated with intraocular space via the optic nerve sheath.
– Intracranial bleeding may result in elevated venous pressure that is transmitted to the intraocular vessels.
– Elevated intracranial pressure is transmitted along optic nerve sheath resulting in rupture of the peripapillary vessels.
ETIOLOGY
Intracranial hemorrhage combined with intracranial hypertension leading to intraocular hemorrhage
DIAGNOSIS
HISTORY
• A history of intracranial hemorrhage and increased intracranial pressure is usually present.
• Visual changes are usually present
– Decreased vision
– Restricted peripheral vision
– New-onset floaters
• Patients with impaired neurological status may not be able to provide a history.
PHYSICAL EXAM
• Visual acuity may range from 20/20 to light perception.
• Ocular findings are usually bilateral.
• Ophthalmic manifestations may include:
– Vitreous hemorrhage
– Subhyaloid hemorrhage
– Sub-internal limiting membrane (ILM) hemorrhage
– Retinal hemorrhage
– Peripapillary hemorrhage
DIAGNOSTIC TESTS & INTERPRETATION
Imaging
• Neuroimaging studies in consultation with a Neurosurgical specialist
– CT scan
– MRI scan
– Cerebral angiography
• B-scan ultrasonography is recommended if the ocular hemorrhage obscures visualization of the retina.
Diagnostic Procedures/Other
Cerebrospinal fluid examination may be helpful in equivocal cases.
Pathological Findings
• Histology of vitreous samples show red blood cells with few white blood cells.
• Epiretinal membranes with glial proliferation may be found.
DIFFERENTIAL DIAGNOSIS
• Vitreous hemorrhage due to other causes
• Proliferative diabetic retinopathy
• Sickle cell retinopathy
• Shaken baby syndrome Valsalva retinopathy
TREATMENT
ADDITIONAL TREATMENT
General Measures
• Treatment of the intracranial hemorrhage and control of intracranial pressure is managed by a neurosurgical specialist.
• Intraocular hemorrhage is managed with:
– Observation with elevated head positioning to allow for spontaneous reabsorption
– Avoidance of anticoagulant medications when medically possible
Pediatric Considerations
• The development of amblyopia in children with unilateral or asymmetric visual loss should be monitored closely.
• Early vitrectomy and/or patching of the fellow eye may be necessary to prevent amblyopia.
Issues for Referral
Any patient who presents with ocular findings consistent with Terson’s syndrome should be referred immediately for urgent neurosurgical consultation
SURGERY/OTHER PROCEDURES
• YAG Laser vitreolysis to disrupt the posterior hyaloid face and promote absorption of the hemorrhage
– This may increase the risk of epiretinal membrane formation.
• Pars plana vitrectomy surgery is indicated in the following situations:
– Nonclearing vitreous hemorrhage
– Retinal detachment associated with vitreous hemorrhage
– Thick sub-ILM hemorrhage involving the fovea
– Monocular patients with decreased vision
– Children at risk for amblyopia
IN-PATIENT CONSIDERATIONS
Initial Stabilization
Managed by neurosurgical specialist
Admission Criteria
Determined by neurosurgical specialist
Discharge Criteria
Determined by neurosurgical specialist
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Resolution of the intraocular hemorrhage may take months without surgery
Patient Monitoring
Patients should be examined serially until the intraocular hemorrhage has cleared completely
PATIENT EDUCATION
Amsler grid monitoring may help to identify patients with epiretinal membrane development.
PROGNOSIS
• The visual prognosis in adults is excellent with >80% of patients recovering normal or near-normal vision (3).
• Children have a more guarded visual prognosis because of the risk of amblyopia.
• Overall prognosis is related to the severity of the intracranial hemorrhage and the subsequent neurological sequelae.
COMPLICATIONS
• Risk of amblyopia in children under the age of 9 years
• Epiretinal membrane formation occurs as a late finding in a significant proportion of patients (4).
– Subhyaloid or sub-ILM hemorrhage may stimulate glial cell proliferation.
– May present years following resolution of the hemorrhage
– Macular holes may also occur
REFERENCES
1. McCarron MO, Alberts MJ, McCarron P. A systematic review of Terson’s syndrome: Frequency and prognosis after subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 2004;75:491–493.
2. Fountas KN, Kapsalaki EZ, Lee GP, et al. Terson hemorrhage in patients suffering aneurysmal subarachnoid hemorrhage: Predisposing factors and prognostic significance. J Neurosurg 2008;109:439–444.
3. Kuhn F, Morris R, Witherspoon CD, et al. Terson syndrome. Results of vitrectomy and the significance of vitreous hemorrhage in patients with subarachnoid hemorrhage. Ophthalmology 1998;105:472–477.
4. Schultz PN, Sobol WM, Weingeist TA. Long-term visual outcome in Terson syndrome. Ophthalmology 1991;98:1814–1819.