From the Department of Ophthalmology (ERLC, JP), Women’s and Children’s Hospital, North Adelaide; and the Department of Ophthalmology (TS, SL, JP), Flinders Eye Centre, Flinders Medical Centre, Bedford Park, South Australia, Australia.
The authors have no financial or proprietary interest in the materials presented herein.
The authors thank Associate Professor Peter Marshall, Director of Neonatology, Flinders Medical Centre, for his views on this report.
Address correspondence to Edward Rickie L. Chu, MBBS, Department of Ophthalmology, Women’s and Children’s Hospital, North Adelaide, South Australia 5006, Australia.
Terson syndrome is the presence of intraocular hemorrhage (subretinal, intraretinal, subhyaloid, or vitreous hemorrhage) associated with severe brain injury or intracranial hemorrhage (including subarachnoid and subdural hemorrhage).1–6 This case report presents a 2-month-old male infant who had acquired Terson syndrome from a substantial right middle cerebral artery territory infarct associated with intrapa-renchymal hemorrhage within the infarct, subdural hygroma over the right cerebral hemisphere, and an ex vacuo midline shift to the right. One month later, he was noted to have bilateral intraocular hemorrhage. The features, severity, and timing of the pathology are suggestive of Terson syndrome that has occurred during the peripartum period.
A 2-month-old male infant was transferred to the Women’s and Children’s Hospital due to a reduced red reflex in his left eye. The patient was in a footling breach presentation and was delivered at 38 weeks by cesarean section. Postnatally, he developed bronchopulmonary dysplasia, respiratory distress syndrome, persistent pulmonary hypertension of the newborn, and hypoglycemia. Other relevant history included a large infarct in the right middle cerebral artery territory associated with intraparen-chymal hemorrhage within the infarct, subdural hy-groma located over the right cerebral hemisphere, and ex vacuo midline shift to the right. This infarct was discovered following a cranial ultrasound investigating the etiology of clonic seizures. The infarct and its associated features were confirmed with magnetic resonance imaging of the head done at 23 days of age (Fig. 1).
Figure 1. Magnetic Resonance Imaging of the Head at 23 Days Old Showing an Extensive Right Middle Cerebral Artery Territory Infarct, Intraparenchymal Hemorrhage Within the Infarct, Subdural Hygroma over the Right Cerebral Hemisphere, and an Ex Vacuo Midline Shift to the Right. (A) FLAIR Axial, (B) T2 Axial, and (C) T2 Coronal.
On examination, the red reflex was unilaterally absent in the left eye. Examination of the eyes under anesthesia was performed. Anterior segments were unremarkable and intraocular pressures were normal. Posterior segment examination of the right eye revealed moderate vitreous hemorrhage and areas of preretinal hemorrhage, whereas the left fundus was not seen due to a dense vitreous hemorrhage (Fig. 2A). The latter was confirmed with an ultrasound (Fig. 2B).
Figure 2. (A) Dense Vitreous Hemorrhage in the Left Eye on Wide-Field Contact Digital Fundus Camera. (B) Ultrasound of the Same Eye Showing the Vitreous Hemorrhage.
The infant’s coagulation profile that included activated partial thromboplastin time, prothrom-bin time, fibrinogen, antithrombin III, protein C, and protein S was unremarkable. Furthermore, the infant’s mother was tested for factor V Leiden and prothrombin and both were undetectable. Homo-cysteine, protein S, and von Willebrand factor activity–ristocetin cofactor test were all within the normal range; however, antithrombin III and protein C levels were mildly elevated. To our knowledge, apart from the above tests, further investigations or follow-up regarding hypercoagulability were not conducted in both the infant and the mother.
The patient was referred to the Flinders Eye Centre vitreoretinal unit and underwent vitrectomy of the left eye. The operation was uneventful and the patient was subsequently discharged home 2 days later.
Our patient had bilateral vitreous hemorrhage associated with an extensive cerebral infarct and in-traparenchymal hemorrhage within the infarct. The above features are consistent with Terson syndrome. Apart from the latter, other causes such as non-accidental injury, birth trauma, and retinopathy of prematurity should also be considered. Although the most common cause of bilateral vitreous hemorrhage in very young children is non-accidental injury,7–9 our patient had been in hospital all his life, making child abuse unlikely. Birth trauma is also a possibility, but he was delivered without labor via lower segment cesarean section. Active or regressed retinopathy of prematurity can also cause vitreous hemorrhage8; however, our patient was a term infant.
Terson syndrome occurs less frequently in children and infants than in adults.7,10 Schloff et al.7 suggested that children may have superior auto-regulation of the retinal vessels and a more limited potential communication between the optic nerve sheath and intracranial space. They also stated that intraretinal hemorrhage in children with intracranial hemorrhage from causes other than non-accidental injury would be expected in less than 8% of the cases. In addition, of the 12% of children who had subarachnoid hemorrhage in their study, none developed intraretinal hemorrhage. Conversely, sub-arachnoid hemorrhage in adults is one of the common causes of Terson syndrome.7
Different hypotheses regarding the mechanism of Terson syndrome have been suggested.1–3,6 The most likely mechanism is cerebrospinal fluid being displaced from the subarachnoid space of the intracranial cavity to the subarachnoid space of the optic nerve sheath, causing dilatation of the latter. This mechanism happens in conditions that elevate intracranial pressure such as intracranial hemorrhage (including subarachnoid or subdural hemorrhage) or severe brain injury.2 Consequently, the central retinal vein (where it traverses the retrobulbar part of the optic nerve sheath) and retinochoroidal “anastomoses” are compressed, causing decreased venous drainage of the eye resulting in venous congestion, rupture of the retinal vessels, and eventually intra-ocular hemorrhage.2–4,6
This case is unique because of the large cerebral pathology that was detected at 23 days of age. However, the exact contributing agent is difficult to ascertain. To be able to obtain such features and severity in the stated time frame, the pathology would have occurred antenatally or in the intrapartum period. The intraocular hemorrhage that was subsequently observed may be the result of cerebral edema and consequent acute elevation of intracranial pressure from the significant cerebral infarct and intrapa-renchymal hemorrhage within the infarct. We also presume that the intraocular hemorrhage may have been present earlier due to the dense appearance of the vitreous hemorrhage and was not noticed until prior to the discharge check of the infant. Younger patients with Terson syndrome have worse visual outcomes and therefore treatment in infants and young children is early vitrectomy to avoid complications such as amblyopia, fibrous proliferation, and traction retinal detachment.4,5,8–10
Terson syndrome is possible in a child who sustained an extensive cerebral infarct with intra-parenchymal hemorrhage within the said infarct associated with intraocular hemorrhage that occurred during the peripartum period.
- Khan SG, Frenkel M. Intravitreal hemorrhage associated with rapid increase in intracranial pressure (Terson’s syndrome). Am J Ophthalmol. 1975;80:37–43.
- Toosi SH, Malton M. Terson’s syndrome: significance of ocular findings. Ann Ophthalmol. 1987;19:7–12.
- Sharma T, Gopal L, Biswas J, et al. Results of vitrectomy in Terson syndrome. Ophthalmic Surg Lasers. 2002;33:195–199.
- Schultz PN, Sobol WM, Weingeist TA. Long-term visual outcome in Terson syndrome. Ophthalmology. 1991;98:1814–1819.
- Kuhn F, Morris R, Witherspoon CD, Mester V. Terson syndrome: results of vitrectomy and the significance of vitreous hemorrhage in patients with subarachnoid hemorrhage. Ophthalmology. 1998;105:472–477. doi:10.1016/S0161-6420(98)93030-5 [CrossRef]
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- Schloff S, Mullaney PB, Armstrong DC, et al. Retinal find-ings in children with intracranial hemorrhage. Ophthalmology. 2002;109:1472–1476. doi:10.1016/S0161-6420(02)01086-2 [CrossRef]
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- Ferrone PJ, de Juan E Jr, . Vitreous hemorrhage in infants. Arch Ophthalmol. 1994;112:1185–1189.