Difference between revisions of "Terson Syndrome"

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{{Article
 
{{Article
 
|Authors=Tahira.Mathen
 
|Authors=Tahira.Mathen
|Category=Retina/Vitreous
+
|Category=Ocular Trauma, Retina/Vitreous
|Assigned editor=Theodore.Leng
+
|Assigned editor=James.Auran,Neelakshi.E.Bhagatne
|Reviewer=Theodore.Leng
+
|Reviewer=Neelakshi.E.Bhagatne
|Date reviewed=November 30, 2014
+
|Date reviewed=February 15, 2021
 
|Article status=Up to Date
 
|Article status=Up to Date
 
}}
 
}}
 
 
{{Infobox disease
 
{{Infobox disease
 
| Name = {{PAGENAME}}
 
| Name = {{PAGENAME}}
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= Disease =
 
= Disease =
[[File:AA0 54317-2.jpg|thumb|394x394px|Fig. 1. Mutliple intraretinal and preretinal hemorrhages in a patient with Terson syndrome.<ref>AAO One Network Images. one.aao.org/images/terson-syndrome</ref>]]
+
[[File:AA0 54317-2.jpg|thumb|750x750px|Fig. 1. Mutliple intraretinal and preretinal hemorrhages in a patient with Terson syndrome.<ref>AAO One Network Images. one.aao.org/images/terson-syndrome</ref>|centre]]
Vitreous hemorrhage associated with subarachnoid hemorrhage (SAH) was first described by German ophthalmologist Moritz  
+
Vitreous hemorrhage associated with subarachnoid hemorrhage (SAH) was first described by German ophthalmologist Moritz Litten in 1881 and then in 1900 by French ophthalmologist Albert Terson <ref name=":0">Czorlich P, Skevas C, Knospe V, et
 
 
Litten in 1881 and then in 1900 by French ophthalmologist Albert Terson <ref name=":0">Czorlich P, Skevas C, Knospe V, et
 
 
al. Terson syndrome in subarachnoid hemorrhage,
 
al. Terson syndrome in subarachnoid hemorrhage,
 
intracerebral hemorrhage, and traumatic brain injury. Neurosurg
 
intracerebral hemorrhage, and traumatic brain injury. Neurosurg
Line 28: Line 25:
 
a prospective interdisciplinary study. Ophthalmology.
 
a prospective interdisciplinary study. Ophthalmology.
 
2014 Aug;121(8):1628-33.
 
2014 Aug;121(8):1628-33.
</ref>.
+
</ref>.  
  
Terson syndrome is now recognized as intraocular hemorrhage associated with SAH, intracerebral hemorrhage, or traumatic brain injury <ref name=":0" />. Hemorrhage may be present in the vitreous, sub-hyaloid, or intraretina/sub-internal limiting membrane.
+
Terson syndrome is now recognized as intraocular hemorrhage associated with SAH, intracerebral hemorrhage, or traumatic brain injury <ref name=":0" />. Hemorrhage may be present in the vitreous, sub-hyaloid,subretinal space, or beneath the internal limiting membrane.
  
 
= Epidemiology =
 
= Epidemiology =
Line 45: Line 42:
 
syndrome and its implications for macular surgery. Dev Ophthalmol 1997; 29: pp.
 
syndrome and its implications for macular surgery. Dev Ophthalmol 1997; 29: pp.
 
44-54.
 
44-54.
</ref><ref name=":0" />, 9.1% of intracerebral hemorrhages and 3.1% of traumatic brain injury <ref name=":0" />. 5.5% of vitreous hemorrhages not caused by diabetes or trauma are caused by Terson syndrome <ref>Verbraeken H, Van Egmond J. Non-diabetic and non-oculotraumatic vitreous haemorrhage treated by pars
+
</ref><ref name=":0" />, 9.1% of intracerebral hemorrhages and 3.1% of traumatic brain injury <ref name=":0" />.   5.5% of vitreous hemorrhages not caused by diabetes or trauma are caused by Terson syndrome <ref>Verbraeken H, Van Egmond J. Non-diabetic and non-oculotraumatic vitreous haemorrhage treated by pars
 
plana vitrectomy. Bull Soc Belge Ophtalmol.
 
plana vitrectomy. Bull Soc Belge Ophtalmol.
 
1999;272:83-9.
 
1999;272:83-9.
Line 63: Line 60:
  
 
= Pathogenesis =
 
= Pathogenesis =
[[File:Screen Shot 2014-11-23 at 7.45.23 PM.png|thumb|398x398px|Fig. 2. Early (A) and late (B) frames of a fluorescein angiogram showing leakage from the disc margin<ref>Ogawa T, Kitaoka T, Dake Y, Amemiya T. Terson syndrome: a case report suggesting the mechanism of
+
[[File:Screen Shot 2014-11-23 at 7.45.23 PM.png|thumb|622x622px|Fig. 2. Early (A) and late (B) frames of a fluorescein angiogram showing leakage from the disc margin<ref>Ogawa T, Kitaoka T, Dake Y, Amemiya T. Terson syndrome: a case report suggesting the mechanism of
 
vitreous hemorrhage. Ophthalmology. 2001
 
vitreous hemorrhage. Ophthalmology. 2001
 
Sep;108(9):1654-6.
 
Sep;108(9):1654-6.
</ref>.]]
+
</ref>.|centre]]
There are several possible pathophysiologic mechanisms for Terson syndrome. Subarachnoid blood may be directly transmitted forward through the optic nerve sheath <ref name=":1" /><ref name=":0" />. More commonly, a sudden increase in intracranial pressure leads to rapid effusion of CSF into the optic nerve sheath. Then dilation of the retrobulbar optic nerve mechanically compresses the central retinal vein and 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 <ref>Gress DR, Wintermark M, Gean AD. A case of Terson syndrome and its mechanism of bleeding. J
+
There are several possible pathophysiologic mechanisms for Terson syndrome. Subarachnoid blood may be directly transmitted forward through the optic nerve sheath <ref name=":1" /><ref name=":0" />. More commonly, a sudden increase in intracranial pressure leads to rapid effusion of CSF into the optic nerve sheath. The increased pressure wave in the retrobulbar optic nerve sheath mechanically compresses the central retinal vein and venous hypertension results in rupture of thin retinal capillaries. This mechanism is consistent with the fact that Terson syndrome can be seen in patients without intracranial hemorrhage <ref>Gress DR, Wintermark M, Gean AD. A case of Terson syndrome and its mechanism of bleeding. J
 
Neuroradiol. 2013 Oct;40(4):312-4.
 
Neuroradiol. 2013 Oct;40(4):312-4.
 
</ref>.
 
</ref>.
  
Fluorescein angiography has demonstrated a leakage site at the disc margin in a patient with Terson syndrome with vitreous hemorrhage. This suggests potential damage to the peripapillary retina induced by increased intracranial pressure transmitted through the optic nerve sheath <ref>Ogawa T, Kitaoka T, Dake Y, Amemiya T. Terson syndrome: a case report suggesting the mechanism of
+
Fluorescein angiography demonstrates a leakage site at the disc margin in a patient with Terson syndrome with vitreous hemorrhage. This may suggest potential damage to the peripapillary retina induced by increased intracranial pressure transmitted through the optic nerve sheath <ref>Ogawa T, Kitaoka T, Dake Y, Amemiya T. Terson syndrome: a case report suggesting the mechanism of
 
vitreous hemorrhage. Ophthalmology. 2001
 
vitreous hemorrhage. Ophthalmology. 2001
 
Sep;108(9):1654-6.
 
Sep;108(9):1654-6.
Line 77: Line 74:
 
   
 
   
 
= Etiology =
 
= Etiology =
Terson syndrome has been reported to be caused by, or associated with multiple conditions associated with, a spike in intracranial pressure. These causes include carotid artery occlusion, cortical venous sinus thrombosis <ref>Takkar A, Kesav P, Lal V, Gupta A. Teaching NeuroImages: Terson syndrome in cortical venous sinus
+
Terson syndrome has been reported to be associated with multiple conditions that sustain a spike in intracranial pressure. These causes include carotid artery occlusion, cortical venous sinus thrombosis <ref>Takkar A, Kesav P, Lal V, Gupta A. Teaching NeuroImages: Terson syndrome in cortical venous sinus
 
thrombosis. Neurology. 2013 Aug
 
thrombosis. Neurology. 2013 Aug
 
6;81(6):e40-1.
 
6;81(6):e40-1.
Line 90: Line 87:
 
injection. Neurology.
 
injection. Neurology.
 
2001 Jul 24;57(2):364.
 
2001 Jul 24;57(2):364.
</ref>, intraarterial angiography, lumbosacral myelomeningocele and iaotrogenic bleeing during endoscopic third ventriculostomy <ref>Hoving EW, Rahmani M, Los LI, Renardel de Lavalette
+
</ref>, intraarterial angiography, lumbosacral myelomeningocele and iaotrogenic bleeding during endoscopic third ventriculostomy <ref>Hoving EW, Rahmani M, Los LI, Renardel de Lavalette
 
VW. Bilateral retinal hemorrhage after endoscopic third ventriculostomy:
 
VW. Bilateral retinal hemorrhage after endoscopic third ventriculostomy:
 
iatrogenic Terson syndrome. J Neurosurg. 2009
 
iatrogenic Terson syndrome. J Neurosurg. 2009
Line 116: Line 113:
  
 
= Clinical presentation =
 
= Clinical presentation =
[[File:AA0 3389.jpg|thumb|397x397px|Fig. 3. Macular preretinal hemorrhage in a patient with Terson syndrome. <ref>AAO One Network Images. one.aao.org/images/preretinal-hemorrhage-2</ref>]]
+
[[File:AA0 3389.jpg|thumb|700x700px|Fig. 3. Macular preretinal hemorrhage in a patient with Terson syndrome. <ref>AAO One Network Images. one.aao.org/images/preretinal-hemorrhage-2</ref>|centre]]
 
Terson syndrome can present with dome-shaped hemorrhages in the macula <ref>Friedman
 
Terson syndrome can present with dome-shaped hemorrhages in the macula <ref>Friedman
 
S.M., and Margo C.E. Bilateral subinternal limiting membrane hemorrhage with
 
S.M., and Margo C.E. Bilateral subinternal limiting membrane hemorrhage with
Line 140: Line 137:
 
HE Jr & Landers MB III (1975): Vitreous hemorrhage after intracranial
 
HE Jr & Landers MB III (1975): Vitreous hemorrhage after intracranial
 
hemorrhage. Am J Ophthalmol 80: 207–213.
 
hemorrhage. Am J Ophthalmol 80: 207–213.
</ref>. In a study by Pfausler, mortality was 90% in pts with SAH and Terson syndrome and 10% in those with SAH without Terson syndrome <ref name=":6" />. In a study by Gutierrez Diaz, mortality was 50% when Terson syndrome was present, and 20% when absent <ref>Gutierrez Diaz A, Jimenez Carmena J, Ruano Martin F, Diaz Lopez P, Muñoz Casado MJ. Intraocular hemorrhage in sudden increased
+
</ref>. In a study by Pfausler, mortality was 90% in patients with SAH and Terson syndrome and 10% in those with SAH without Terson syndrome <ref name=":6" />. In a study by Gutierrez Diaz, mortality was 50% when Terson syndrome was present, and 20% when absent <ref>Gutierrez Diaz A, Jimenez Carmena J, Ruano Martin F, Diaz Lopez P, Muñoz Casado MJ. Intraocular hemorrhage in sudden increased
 
intracranial pressure (Terson syndrome). Ophthalmologica.
 
intracranial pressure (Terson syndrome). Ophthalmologica.
 
1979;179(3):173-6.
 
1979;179(3):173-6.
Line 146: Line 143:
  
 
= Diagnosis =
 
= Diagnosis =
[[File:Screen Shot 2014-11-23 at 7.47.59 PM.png|thumb|340x340px|Fig. 4. B-scan showing vitreous hemorrhage in a patient with Terson syndrome<ref>Ogawa T, Kitaoka T, Dake Y, Amemiya T. Terson syndrome: a case report
+
[[File:Screen Shot 2014-11-23 at 7.47.59 PM.png|thumb|411x411px|Fig. 4. B-scan showing vitreous hemorrhage in a patient with Terson syndrome<ref>Ogawa T, Kitaoka T, Dake Y, Amemiya T. Terson syndrome: a case report
 
suggesting the mechanism of vitreous hemorrhage.
 
suggesting the mechanism of vitreous hemorrhage.
 
Ophthalmology. 2001 Sep;108(9):1654-6.
 
Ophthalmology. 2001 Sep;108(9):1654-6.
</ref>.  ]]
+
</ref>.  |centre]]
 
Funduscopic exam is the gold standard for diagnosis of Terson syndrome. Loss of red reflex is seen in 20% of eyes with Terson syndrome<ref name=":0" />.  
 
Funduscopic exam is the gold standard for diagnosis of Terson syndrome. Loss of red reflex is seen in 20% of eyes with Terson syndrome<ref name=":0" />.  
  
B-scan may be used to confirm vitreous hemorrhage when no view to the funds is present (Fig. 4).  
+
B-scan may be used to confirm vitreous hemorrhage when no view to the fundus is present (Fig. 4).  
  
 
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 <ref>Ashrafi AN, Chakrabarti R, Laidlaw J. Terson syndrome: the need for fundoscopy in subarachnoid
 
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 <ref>Ashrafi AN, Chakrabarti R, Laidlaw J. Terson syndrome: the need for fundoscopy in subarachnoid
Line 162: Line 159:
 
</ref>.
 
</ref>.
  
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 syndrome <ref>Swallow CE, Tsuruda JS, Digre KB, Glaser MJ, Davidson
+
Swallow investigated the use of orbital CT to identify intraocular vitreous hemorrhage in patients with Terson syndrome. Retinal crescentic hyperdensities and retinal nodularity were seen in CT in two-thirds of patients with Terson syndrome <ref>Swallow CE, Tsuruda JS, Digre KB, Glaser MJ, Davidson
 
HC, Harnsberger HR. Terson syndrome: CT evaluation in 12
 
HC, Harnsberger HR. Terson syndrome: CT evaluation in 12
 
patients. AJNR Am J Neuroradiol. 1998 Apr;19(4):743-7.
 
patients. AJNR Am J Neuroradiol. 1998 Apr;19(4):743-7.
Line 193: Line 190:
 
detachment with severe proliferative vitreoretinopathy in Terson syndrome.
 
detachment with severe proliferative vitreoretinopathy in Terson syndrome.
 
Ophthalmology 1994; 101: pp. 35-37.
 
Ophthalmology 1994; 101: pp. 35-37.
</ref><ref name=":4" /><ref name=":12" />.  Two patients have also been reported with macular holes, which were found intraoperatively during pars plana vitrectomy for vitreous hemorrhage <ref name=":9" />.  
+
</ref><ref name=":4" /><ref name=":12" />.  Two patients have also been reported with macular holes, which were found intraoperatively during pars plana vitrectomy for vitreous hemorrhage <ref name=":9" />. Dissociated optic nerve fiber layer appearance (DONFL) may be noted after removal of ILM in Terson syndrome with sub-ILM hemorrhage.<ref>Tripathy K. Dissociated optic nerve fiber layer in a case of Terson syndrome [published online ahead of print, 2019 Jun 3]. ''Eur J Ophthalmol''. 2019;1120672119853465. doi:10.1177/1120672119853465</ref>
 
= Treatment and prognosis  =
 
= Treatment and prognosis  =
 
Intraocular hemorrhage frequently resolves spontaneously <ref name=":2" />. Vision loss is usually reversible but permanent impairment of vision can occur <ref name=":2" /><ref>Roux
 
Intraocular hemorrhage frequently resolves spontaneously <ref name=":2" />. Vision loss is usually reversible but permanent impairment of vision can occur <ref name=":2" /><ref>Roux
Line 203: Line 200:
 
PN, Sobol WM, Weingeist TA. Long-term visual outcome in Terson
 
PN, Sobol WM, Weingeist TA. Long-term visual outcome in Terson
 
syndrome. Ophthalmology. 1991
 
syndrome. Ophthalmology. 1991
Dec;98(12):1814-9. </ref>.]]
+
Dec;98(12):1814-9. </ref>.|centre]]
 
There is no consensus on optimal timing for vitrectomy in Terson syndrome. Vitreous hemorrhage can be observed for up to 3 months before considering pars plana vitrectomy <ref name=":0" /><ref name=":5" /><ref name=":11" />. This is supported by a study of 36 eyes with Terson syndrome in which 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 <ref name=":12" />. If the hemorrhage is bilateral or occurs in a young child at risk of amblyopia, one may proceed with surgery sooner <ref name=":0" /><ref name=":12">Garweg JG, Koerner F. Outcome indicators for vitrectomy in Terson syndrome. Acta
 
There is no consensus on optimal timing for vitrectomy in Terson syndrome. Vitreous hemorrhage can be observed for up to 3 months before considering pars plana vitrectomy <ref name=":0" /><ref name=":5" /><ref name=":11" />. This is supported by a study of 36 eyes with Terson syndrome in which 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 <ref name=":12" />. If the hemorrhage is bilateral or occurs in a young child at risk of amblyopia, one may proceed with surgery sooner <ref name=":0" /><ref name=":12">Garweg JG, Koerner F. Outcome indicators for vitrectomy in Terson syndrome. Acta
 
Ophthalmol. 2009 Mar;87(2):222-6.
 
Ophthalmol. 2009 Mar;87(2):222-6.
Line 216: Line 213:
 
</ref>.  
 
</ref>.  
  
Younger patients (<45 years old) who have PPV for Terson syndrome have better final visual acuity than older patients (>45 years old)<ref name=":12" />.
+
Younger patients (<45 years old) who undergo PPV for Terson syndrome have better final visual acuity than older patients (>45 years old)<ref name=":12" />.
  
 
Studies have shown no difference in final visual acuity between patients who were conservatively managed and those who underwent PPV. However, visual recovery was more rapid in the vitrectomy group despite these patients having denser vitreous hemorrhage <ref name=":10" />.
 
Studies have shown no difference in final visual acuity between patients who were conservatively managed and those who underwent PPV. However, visual recovery was more rapid in the vitrectomy group despite these patients having denser vitreous hemorrhage <ref name=":10" />.
  
Intravitreal tpa and gas have been used for recalcitrant Terson syndrome<ref>Serracarbassa
+
Intravitreal TPA and gas have been used for recalcitrant Terson syndrome<ref>Serracarbassa
 
P.D., Rodrigues L.D., and Rodrigues J.R.: Tissue plasminogen activator and
 
P.D., Rodrigues L.D., and Rodrigues J.R.: Tissue plasminogen activator and
 
intravitreal gas for the treatment of Terson's syndrome: case report. Arq Bras
 
intravitreal gas for the treatment of Terson's syndrome: case report. Arq Bras
Line 226: Line 223:
 
</ref> <ref name=":2" />.
 
</ref> <ref name=":2" />.
  
One patient with Terson syndrome and premacular subhyaloid hemorrhage was treated with Nd-YAG to puncture the posterior hyaloid face and allow drainage of blood into the vitreous. VA improved from 20/400 before treatment to 20/20 one month after treatment <ref>Ulbig MW, Mangouritsas G, Rothbacher HH, Hamilton
+
One patient with Terson syndrome and premacular subhyaloid hemorrhage was treated with Nd-YAG to puncture the posterior hyaloid face to allow drainage of blood into the vitreous. VA improved from 20/400 before treatment to 20/20 one month after treatment <ref>Ulbig MW, Mangouritsas G, Rothbacher HH, Hamilton
 
AM, McHugh JD.
 
AM, McHugh JD.
 
Long-term results after drainage of premacular subhyaloid hemorrhage into
 
Long-term results after drainage of premacular subhyaloid hemorrhage into
Line 232: Line 229:
 
Ophthalmol. 1998 Nov;116(11):1465-9.
 
Ophthalmol. 1998 Nov;116(11):1465-9.
 
</ref>.
 
</ref>.
= Additional Resources =
 
* American Academy of Ophthalmology. ''Retina/Vitreous: Terson syndrome'' [http://store.aao.org/practicing-ophthalmologists-learning-system-2017-2019.html Practicing Ophthalmologists Learning System, 2017 - 2019] San Francisco: American Academy of Ophthalmology, 2017.
 
 
 
= References  =
 
= References  =
 
<references />
 
<references />

Latest revision as of 09:07, February 17, 2021


Terson Syndrome


Disease

Fig. 1. Mutliple intraretinal and preretinal hemorrhages in a patient with Terson syndrome.[1]

Vitreous hemorrhage associated with subarachnoid hemorrhage (SAH) was first described by German ophthalmologist Moritz Litten in 1881 and then in 1900 by French ophthalmologist Albert Terson [2] [3].

Terson syndrome is now recognized as intraocular hemorrhage associated with SAH, intracerebral hemorrhage, or traumatic brain injury [2]. Hemorrhage may be present in the vitreous, sub-hyaloid,subretinal space, or beneath the internal limiting membrane.

Epidemiology

Terson syndrome occurs in 8-19.3% of SAH [4][5][6][7][2], 9.1% of intracerebral hemorrhages and 3.1% of traumatic brain injury [2]. 5.5% of vitreous hemorrhages not caused by diabetes or trauma are caused by Terson syndrome [8].

Terson syndrome usually occurs in adults, but has been reported in children as young as 7 months [9][10]. It can be unilateral or bilateral [11].

Pathogenesis

Fig. 2. Early (A) and late (B) frames of a fluorescein angiogram showing leakage from the disc margin[12].

There are several possible pathophysiologic mechanisms for Terson syndrome. Subarachnoid blood may be directly transmitted forward through the optic nerve sheath [4][2]. More commonly, a sudden increase in intracranial pressure leads to rapid effusion of CSF into the optic nerve sheath. The increased pressure wave in the retrobulbar optic nerve sheath mechanically compresses the central retinal vein and venous hypertension results in rupture of thin retinal capillaries. This mechanism is consistent with the fact that Terson syndrome can be seen in patients without intracranial hemorrhage [13].

Fluorescein angiography demonstrates a leakage site at the disc margin in a patient with Terson syndrome with vitreous hemorrhage. This may suggest potential damage to the peripapillary retina induced by increased intracranial pressure transmitted through the optic nerve sheath [14].

Etiology

Terson syndrome has been reported to be associated with multiple conditions that sustain a spike in intracranial pressure. These causes include carotid artery occlusion, cortical venous sinus thrombosis [15], moyamoya disease [16][17], epidural saline injection [18], intraarterial angiography, lumbosacral myelomeningocele and iaotrogenic bleeding during endoscopic third ventriculostomy [19].

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 [20] and two other studies found that anterior communicating artery aneurysms in particular are associated with a higher rate of Terson syndrome [21][22]. Other studies have shown no correlation between site of aneurysm and Terson syndrome [2] or a negative correlation between presence of anterior communicating artery aneurysm and Terson syndrome [23]. There is also no relationship between the location of the aneurysm and which eye is affected by Terson syndrome [2][20][24][25].

Clinical presentation

Fig. 3. Macular preretinal hemorrhage in a patient with Terson syndrome. [26]

Terson syndrome can present with dome-shaped hemorrhages in the macula [27]. A macular “double ring” sign may be seen with the inner ring caused sub-ILM hemorrhage and the outer ring caused by sub-hyaloid hemorrhage [28].

Although intraocular hemorrhages most frequently develop in the first hour after SAH [29], Terson syndrome can have a delayed onset, with reports of intraocular hemorrhage occurring up to 47 days after SAH [2][30].

Relationship to neurologic outcomes

Low Glasgow coma scale, high Hunt and Hesse grade, and high Fisher grade are associated with a higher incidence of Terson syndrome [2].

Neurological outcomes and mortality rate are worse in patients with SAH and Terson syndrome than patients with SAH alone [2] [20] [24][23][29][31]. In a study by Pfausler, mortality was 90% in patients with SAH and Terson syndrome and 10% in those with SAH without Terson syndrome [24]. In a study by Gutierrez Diaz, mortality was 50% when Terson syndrome was present, and 20% when absent [32].

Diagnosis

Fig. 4. B-scan showing vitreous hemorrhage in a patient with Terson syndrome[33].

Funduscopic exam is the gold standard for diagnosis of Terson syndrome. Loss of red reflex is seen in 20% of eyes with Terson syndrome[2].

B-scan may be used to confirm vitreous hemorrhage when no view to the fundus is present (Fig. 4).

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 [34]. 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 Terson syndrome [35].

Swallow investigated the use of orbital CT to identify intraocular vitreous hemorrhage in patients with Terson syndrome. Retinal crescentic hyperdensities and retinal nodularity were seen in CT in two-thirds of patients with Terson syndrome [36][16]. Thus CT may be useful to identify possible Terson syndrome prior to an eye exam.

Complications

Multiple complications have been reported after Terson syndrome. Epiretinal membrane is the most common sequelae of Terson syndrome, with an incidence of 15-78% [37][38][39][40][11]. Vitreous blood may cause ERMs by inducing glial proliferation and disruption of the ILM [37][41][38].

Retinal folds/perimacular folds occur in 20% of patients with Terson syndrome, retinal detachment occurs in 9%, and ghost cell glaucoma occurs in around 4% [37][42][35]. Proliferative vitreoretinopathy and preretinal fibrosis have also been reported after Terson syndrome [43][44][16][45]. Two patients have also been reported with macular holes, which were found intraoperatively during pars plana vitrectomy for vitreous hemorrhage [38]. Dissociated optic nerve fiber layer appearance (DONFL) may be noted after removal of ILM in Terson syndrome with sub-ILM hemorrhage.[46]

Treatment and prognosis

Intraocular hemorrhage frequently resolves spontaneously [10]. Vision loss is usually reversible but permanent impairment of vision can occur [10][47]. It has been reported that about 50% of vitreous hemorrhages do not resolve after 19 months.

Fig. 5. Bilateral Terson syndrome before treatment (top images) and after bilateral pars plana vitrectomy with resolution of hemorrhage (bottom images)[48].

There is no consensus on optimal timing for vitrectomy in Terson syndrome. Vitreous hemorrhage can be observed for up to 3 months before considering pars plana vitrectomy [2][20][41]. This is supported by a study of 36 eyes with Terson syndrome in which 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 [45]. If the hemorrhage is bilateral or occurs in a young child at risk of amblyopia, one may proceed with surgery sooner [2][45]. Augsten recommends operating within 4-8 weeks after the injury in patients with bilateral Terson syndrome [41].

Multiple studies have shown good outcomes after pars plana vitectomy. 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 [2]. In another study on PPV for Terson syndrome, 96% of patients had rapid visual improvement and 81% had better than 20/30 vision [49]. In a series of 15 eyes that underwent PPV for VH, 93% had final VA of 20/40 or better. [37]. ILM peeling has also been described in the surgical management of Terson syndrome [50].

Younger patients (<45 years old) who undergo PPV for Terson syndrome have better final visual acuity than older patients (>45 years old)[45].

Studies have shown no difference in final visual acuity between patients who were conservatively managed and those who underwent PPV. However, visual recovery was more rapid in the vitrectomy group despite these patients having denser vitreous hemorrhage [39].

Intravitreal TPA and gas have been used for recalcitrant Terson syndrome[51] [10].

One patient with Terson syndrome and premacular subhyaloid hemorrhage was treated with Nd-YAG to puncture the posterior hyaloid face to allow drainage of blood into the vitreous. VA improved from 20/400 before treatment to 20/20 one month after treatment [52].

References

  1. AAO One Network Images. one.aao.org/images/terson-syndrome
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 Czorlich P, Skevas C, Knospe V, et al. Terson syndrome in subarachnoid hemorrhage, intracerebral hemorrhage, and traumatic brain injury. Neurosurg Rev. Epub 2014 Aug 31.
  3. Skevas C, Czorlich P,Knospe V, et al. Terson's syndrome--rate and surgical approach in patients with subarachnoid hemorrhage: a prospective interdisciplinary study. Ophthalmology. 2014 Aug;121(8):1628-33.
  4. 4.0 4.1 Iuliano L, Fogliato G, Codenotti M. Intrasurgical imaging of subinternal limiting membrane blood diffusion in terson syndrome. Case Rep Ophthalmol Med. 2014;2014:689793.
  5. Michalewska Z, Michalewski J, Nawrocki J. Possible methods of blood entrance in Terson syndrome. Ophthalmic Surg Lasers Imaging. 2010 Nov-Dec;41 Suppl:S42-9.
  6. Morris R., Kuhn F., and Witherspoon C.D.: Hemorrhagic macular cysts. Ophthalmology 1994; 101: 1.
  7. Morris R., Kuhn F., Witherspoon C.D., et al: Hemorrhagic macular cysts in Terson's syndrome and its implications for macular surgery. Dev Ophthalmol 1997; 29: pp. 44-54.
  8. Verbraeken H, Van Egmond J. Non-diabetic and non-oculotraumatic vitreous haemorrhage treated by pars plana vitrectomy. Bull Soc Belge Ophtalmol. 1999;272:83-9.
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