REFERAT

TERSON’S SYNDROME

Dokter pembimbing

dr. Gilbert W. Simanjuntak, Sp.M

Disusun oleh

Andreas Octaviano Rainaldy

0861050149

KEPANITERAAN KLINIK ILMU KESEHATAN MATA

PERIODE 29 JUNI – 04 AGUSTUS 2012

FAKULTAS KEDOKTERAN

UNIVERSITAS KRISTEN INDONESIA

JAKARTA

TERSON'S SYNDROME

Signs and Symptoms

Sindrom Terson yang pertama kali dijelaskan oleh dokter mata dari Perancis Albert Terson di awal 1900s.1, 2 Sindrom Terson didefinisikan sebagai perdarahan intraokular terkait dengan perdarahan intrakranial akut.1-5 Pada saat ini, hubungan antara perdarahan vitreous dengan perdarahan subarachnoid akut juga sudah dapat dijelaskan.1,2 Kunci untuk mendiagnostik Terson Syndrome adalah terjadi secara bilateral, perdarahan segmen posterior yang terdapat pada beberapa intraretinal, lokasi preretinal atau intravitreal. Penyebab utama terjadinya Terson Sindrom bersifat spontan atau bisa juga akibat perdarahan intrakranial (biasanya perdarahan subarachnoid).1 Perdarahan subarachnoid dari aneurisma otak, khususnya aneurisma dari arteri komunikans anterior, telah digambarkan sebagai penyebab yang paling umum.

Intraocular hemorrhage is seen in approximately 20% of patients with acute intracranial bleeding.1 Significant vitreous hemorrhage occurs in a smaller percentage of these patients.1,4 Although the intraocular bleeding may consist of subretinal and deep intraretinal hemorrhage, they may also lie superficially, being just under the internal limiting membrane or su bhyaloid. Significant vitreous hemorrhage will occur if the blood breaks through the internal limiting membrane or the posterior hyaloid face and moves into the vitreous gel.1,3

Visual acuity is often diminished and in some cases is the first sign of trouble. 3 The amount of acute vision loss is related to the extent of the bleeding.3,4 Late complications include epiretinal membrane formation and, rarely, tractional or rhegmatogenous retinal detachments.5 It is important to note that some patients suffering from ruptured intracranial aneurysms may exhibit vision loss secondary to the associated vitreous hemorrhage while not demonstrating any headache, neurological deficits or signs of meningeal irritation.2 Terson's syndrome seems to be an anomaly of adults, with at least one study documenting that the maximal incidence of intraretinal hemorrhage in children with non-abuse intracranial hemorrhage (intracranial hemorrhage not associated with shaken baby syndrome) is 8%.4

Pathophysiology

The etiopathology of Terson's syndrome has been controversial since its inception.1 Early

Superior and nasal subretinal hemorrhage with disc edema.

investigators believed that the resulting intraocular hemorrhage emanated from the direct dissection of subarachnoid hemorrhage down the optic nerve sheath.1 However, poor anatomical communication between the subarachnoid space of the optic nerve and the vitreous humor makes this mechanism unlikely. Currently, sudden spiking of intracranial pressure occurring at the time of an intracranial bleed is recognized as the primary event that precipitates the intraocular bleeding.1 Frequently, the amount of ocular hemorrhage correlates directly with the rapidity and magnitude of intracranial pressure elevation. Why this type of increased intracranial pressure translates into intraocular bleeding remains unclear.1 Regillo postulates that increased orbital venous pressure, which translates directly through the cavernous sinus, or compression of both the ophthalmic veins and adjacent retinochoroidal anastamoses secondary to rapid effusion of cerebrospinal fluid or blood into the optic nerve sheath could explain the phenomenon.1 Either way, it appears that an acute obstruction of the retinal venous circulation results in the rupture of superficial retinal vessels.1

Pathogenesis of vitreous haemorrhage

The pathogenesis of vitreous haemorrhage has been much debated.6,7,8,9 Increased intracranial pressure may force blood into the subarachnoid space and along the optic nerve sheath into the pre-retinal space, or the sudden rise in intracranial pressure may lead to a decrease in venous return to the cavernous sinus or obstruct the retinochoroidal anastomoses and central retinal vein, culminating in venous stasis and haemorrhage. It is now generally accepted that vitreous haemorrhage results from ocular blood.7,8,9

Management

Since patients with this entity have suffered some catastrophic intracranial event and have already presented to a general or emergency physician with other significant systemic symptoms and signs, the diagnosis of Terson's syndrome is generally straightforward.1,5 Some suggest that the presence of Terson's syndrome may be associated with a higher rate of mortality.1 In suspected cases that do not have an established traumatic source of intracranial hemorrhage, emergency neuroimaging with either tomography or magnetic resonance imaging is indicated, as a ruptured intracranial aneurysm is the likely cause.1

Clinical Pearls

In Terson's syndrome, in general when the blood clears, visual acuity returns. When the vision remains decreased, it is typically secondary to persistent vitreous hemorrhage or retinal pigment epithelial disturbances and/or epiretinal membrane formation.

Even in cases without these significant complications, occasionally some degree of visual acuity loss may persist indefinitely because the subretinal hemorrhage or the injurious

event itself disrupted the optic nerve, retinal pigment epithelium or the retinal tissue. In these cases, electrodiagnostic testing may be useful in providing definitive diagnosis.

In cases of acute painful CN III palsy, always examine within the eye for retinal or vitreous hemorrhage due to the high incidence of intracranial aneurysm causing CN III palsy. However, do this through an undilated pupil, as pupil dilation is contraindicated in CN III palsy due to the necessity of following the pupillary responses.

 References

1. Regillo CD Distant Trauma with Posterior Segment Effects. In : Yanoff M, Duker JS Ophthalmology. Philadelphia, PA : Mosby 1999; 8(44):1 6.

2. Wietholter S, Steube D, Stotz HP Terson syndrome: a frequently missed ophthalmologic complication in subarachnoid hemorrhage. Zentralbl Neurochir 1998; 59(3):166-70.

3. Murthy S, Salas D, Hirekataur S, et al. Terson's syndrome presenting as an ophthalmic emergency. Acta Ophthalmol Scand 2002; 80(6):665-6.

4. Schloff S, Mullaney PB, Armstrong, D.C., et al. Retinal findings in children with intracranial hemorrhage. Ophthalmology 2002; 109(8):1472-6.

5. Sadeh AD, Lazar M, Loewenstein A. Macular ring in a patient with Terson's syndrome. Acta Ophthalmol Scand 1999; 77(5):599-600.

6. Riddoch G, Goulden C. On the relationship between subarachnoid and intraocular haemorrhage. Br J Ophthalmol 1925;9:209–33.

7. Castren JA. Pathogenesis and treatment of Terson syndrome. Acta Ophthalmol 1963;41:430–4.

8. Ballantyne AJ. The ocular manifestations of spontaneous subarachnoid haemorrhage. Br J Ophthalmol 1943;27:383–414.

9. Ogawa T, Kitaoaka T, Dake Y, et al. Terson syndrome: a case report suggesting the mechanism of vitreous hemorrhage. Ophthalmology 2001;108:1654–6.