Woman experiences profoundly decreased vision in ICU
On examination, dense khaki-colored material was seen in the vitreous with foci of dark hemorrhage.
A 68-year-old Cantonese-speaking woman was seen for an inpatient consultation by the retina service at the New England Eye Center for profound vision loss in both eyes.
One month before the consultation, she was waiting for the subway when she suddenly lost consciousness. Bystanders rushed her to the emergency department where she was found to be minimally responsive to external stimuli, and she was intubated for airway protection. After a complicated 6-week course in the ICU, she became more responsive and expressed to her family that she could not see out of either eye. Although her ability to provide a history was limited due to intermittent delirium and language issues, she described painless, profound vision loss in both eyes to the degree that she was only able to see her hand immediately in front of her face. She was unclear as to the timing of the vision loss. She had no associated flashes, jaw claudication or temple tenderness.
Before the hospitalization, her medical history included bilateral hearing loss, hypertension, hyperlipidemia and hepatitis C. Her ocular history was remarkable only for bilateral upper blepharoplasty performed approximately 1 year ago. Seven months before hospitalization, her vision was 20/30 in the right eye and 20/40 in the left eye.
Clinical examination revealed an ill-appearing woman with a healing surgical wound over her right frontal scalp and a ventriculoperitoneal shunt. There was no apparent trauma to the orbits, lids or adnexa. Her vision was hand motion in both eyes. Pupils were equal and reactive without a relative afferent pupillary defect. IOP was normal (10 mm Hg in the right eye and 14 mm Hg in the left eye). Ocular motility was full bilaterally. The anterior segments were normal with 2+ nuclear sclerotic cataracts.
Dilated exam revealed dense khaki-colored material and foci of darker hemorrhage completely filling the vitreous cavity, obscuring visualization of any retinal details in each eye.
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Proliferative diabetic retinopathy (PDR), ocular trauma, hemorrhagic posterior vitreous detachment (PVD), retinal break, retinal vein occlusion, age-related macular degeneration/polypoidal choroidal vasculopathy, ruptured retinal microaneurysm and Terson syndrome are all potential causes of bilateral simultaneous vitreous hemorrhage.
PDR is one of the most common causes of vitreous hemorrhage and therefore should be considered, although the patient did not have a history of diabetes. Another potential retinovascular cause includes rupture of a retinal artery macroaneurysm (RAM), and systemic hypertension is a predisposing factor. However, bilateral simultaneous rupture of RAM would be unusual. Other causes of vitreous hemorrhage include hemorrhagic PVDs, retinal breaks and retinal detachment. At age 68, choroidal neovascularization associated with AMD or aneurysmal type 2 neovascularization (PCV) are in the differential. Without a clear view of the retinal anatomy, a B-scan is essential to triage and manage the patient. Dye-based (fluorescein and indocyanine green) angiography can be helpful to identify retinal microaneurysm, CNV or polyps in PCV. Finally, vitreous hemorrhage associated with intracranial hemorrhage, also known as Terson syndrome, is an etiology that is consistent with bilateral occurrence and loss of consciousness.
B-scan ultrasound revealed bilateral dense hyperechoic vitreous debris consistent with hemorrhage (Figure 1). There were no retinal tears or detachment. Fluorescein angiography could not be obtained given the patient’s systemic status and dense vitreous debris preventing visualization. Hemoglobin A1c was normal at 5.8 mg/dL. For the loss of conscious, the patient underwent urgent CT/CTA of the head upon presentation, which showed extensive subarachnoid hemorrhage filling the basal cisterns, sylvian fissures and subarachnoid spaces around the frontal lobes (Figure 2). A bilobed 3 mm by 4 mm aneurysm was discovered at the left A1-A2 junction (Figure 3a). There were no skull fractures.
During her 6 weeks in the hospital before ophthalmology consultation, she had undergone a number of neurosurgical interventions. An external ventricular drain had been placed to evacuate ventricular hemorrhage and alleviate hydrocephalus. The anterior communicating artery aneurysm was coiled (Figure 3b), and the ventriculoperitoneal shunt was placed.
At the initial consultation, the khaki-colored vitreous opacity was diagnosed as dense dehemoglobinized vitreous hemorrhage. Given the patient’s comorbidities in the absence of any retinal break, the vitreous hemorrhages were initially observed while her neurologic status stabilized. At 1-month follow-up, the vision remained hand motion in the right eye and had dropped to light perception in the left due to persistent dense chronic vitreous hemorrhage. Pars plana vitrectomy was performed in the right eye followed 2 weeks later by pars plana vitrectomy in the left eye. Intraoperative examination of each eye revealed solely dense, dark vitreous hemorrhage, confirming the diagnosis of Terson syndrome.
The clinical association between subarachnoid and vitreous hemorrhage is well established, with the first descriptions dating back to those by Moritz Litten in 1881. Albert Terson expanded the syndrome to include vitreous hemorrhage associated with any intracranial hemorrhage. The definition of Terson syndrome (TS) has since been broadened to include any intraocular hemorrhage associated with any intracranial hemorrhage.
The presentation of TS can vary. Visual acuity at presentation can range from 20/20 to light perception. In addition to vitreous hemorrhage, dome-shaped sub-internal limiting membrane (ILM) hemorrhage, more diffuse subhyaloid hemorrhage or the combination, known as a double-ring sign, can occur.
The incidence of TS in subarachnoid hemorrhage varies by its definition, with estimates ranging from 8% to 40%. TS is less frequently observed in patients with intracerebral hemorrhage (9.1%) and traumatic brain injury (3.1%). With low rates of dilated screening examinations in these patients, the true incidence is likely higher than the clinical incidence. However, numerous studies in the neurosurgical literature show increased morbidity and mortality in patients with TS.
The mechanism of vitreous and retinal hemorrhage is thought to be linked to acute elevation in intracranial pressure. In one study, multivariate analysis found that only intracranial pressure greater than 25 mm H2O was identified as a risk factor for TS. This elevated intracranial pressure is then transmitted to the optic nerve sheath, which likely causes obstruction of venous outflow and subsequent retinal capillary rupture. Blood may break through the ILM and hyaloid initially or in a delayed fashion. Ogawa and colleagues demonstrated the peripapillary origin of the vitreous hemorrhage in one post-vitrectomized eye with TS using fluorescein angiography.
The initial management of vitreous hemorrhage in TS depends on patient characteristics. Some data suggest that there is no difference in the final visual outcome between those who had vitrectomy vs. those who allowed the hemorrhage to resolve spontaneously, but the return of vision is much more rapid when vitrectomy is performed. However, cases of macular hole, epiretinal membrane formation, retinal folds and retinal detachments have been reported and suggest that some patients may benefit from surgical intervention. Further, early vitrectomy is generally preferred in children due to the potential for amblyopia. Similarly, in patients with bilateral vision loss due to hemorrhage or poor vision in the contralateral eye, or if social factors make rapid visual recovery essential, early vitrectomy (less than 3 months) is offered. A series by Garweg and Koerner that reviewed 44 eyes with TS found that those who had undergone vitrectomy within the first 90 days tended to have better visual outcomes. However, even when the patients with delayed surgery were included, 80% had a final visual outcome of 20/40 or greater.
Clinical course continued
At 3 months after vitrectomy in the right eye, visual acuity was 20/70 with a visually significant cataract and epiretinal membrane. At 2 months after vitrectomy in the left eye, visual acuity was 20/30.
- Czorlich P, et al. J Clin Neurosci. 2016;doi:10.1016/j.jocn.2016.04.015.
- Czorlich P, et al. Neurosurg Rev. 2015;doi:10.1007/s10143-014-0564-4.
- Garweg JG, et al. Acta Ophthalmol. 2009;doi:10.1111/j.1755-3768.2008.01200.x.
- Joswig H, et al. Acta Neurochir (Wien). 2016;doi:10.1007/s00701-016-2766-8.
- Ko F, et al. Ophthalmology. 2010;doi:10.1016/j.ophtha.2009.11.028.
- Narayanan R, et al. Ophthalmology. 2017;doi:10.1016/j.ophtha.2016.09.009.
- Ogawa T, et al. Ophthalmology. 2001;doi:10.1016/S0161-6420(01)00673-X.
- Schultz PN, et al. Ophthalmology. 1991;doi:10.1016/S0161-6420(91)32045-1.
- For more information:
- Adam T. Chin, MD, and Caroline R. Baumal, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Adam T. Chin, MD, and Omar Dajani, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.