Shaken baby syndrome has several acute ophthalmologic features that can be helpful in confirming the diagnosis of nonaccidental injury in infants with intracranial hemorrhage. Long-term visual sequelae of shaken baby syndrome include retinal folds, pigment disruption, atrophy, and fibrosis; optic nerve atrophy; and occipital infarction. Herein we report the novel complication of neovascularization of the disc following severe retinal injury due to shaking.
A 4-month-old infant was admitted to the hospital for lethargy and new-onset seizures. A computed tomographic scan showed intracranial hemorrhages, diffuse cerebral edema, and almost total infarction of the occipital lobes. An ophthalmologist was consulted to examine for retinal hemorrhages.
Visual behavior could not be evaluated. Both retinas showed massive hemorrhagic retinoschisis cavities elevated into the central vitreous, which encompassed and extended outside of the posterior poles. Of the remaining retina, no vascular details were visible owing to complete saturation with blood. The optic discs could not be identified. The right eye had a small streak of intravitreal hemorrhage over the dome of the schisis cavity.
The child was followed up 5 weeks, 2 months, and 4 months after the original consultation. On the first clinic visit, a dispersed, white, vitreous hemorrhage was seen in the right eye that prevented a view of the retina; partial resorption of hemorrhage and settling of the schisis cavity had occurred in the left eye. At the 2-month follow-up, the right vitreous hemorrhage had not cleared but the left retinal hemorrhage had mostly resorbed, leaving a visible macular pigmentary clump and distorted retinal vasculature. There was no neovascularization. Two months later, there was still no view of the right retina, but florid neovascularization of the left optic disc had developed.
An examination under anesthesia was performed along with fluorescein angioscopy. The neovascularization consisted of large-caliber vessels that extended from the left optic disc directly anteriorly, crossing two thirds of the vitreous cavity; there was frondlike arborization of the anterior half of the vessels (Figure 1). These vessels leaked fluorescein rapidly. There was widespread posterior and midperipheral retinal nonperfusion. Panretinal argon green laser photocoagulation was applied.
Color fundus photograph of the left eye at the time of examination under anesthesia (28-diopter lens). Subretinal pigmentary clumping (closed star) and a partially avulsed retinal vessel (small arrowheads) are identifiable. A robust stalk of neovascularization (large arrowheads) extends from the optic disc (open star) directly anteriorly; the anterior half of the stalk is not visualized owing to photographic defocus.
Vigorous shaking of infants can cause intracranial hemorrhage and intraocular hemorrhage. The intraocular hemorrhage can be subretinal, intraretinal, preretinal, or intravitreal. Retinal schisis cavities, sometimes partly or fully hemorrhagic, can also be seen acutely and are believed to be due to anteroposterior vitreous traction on the internal limiting membrane. Late retinal sequelae of these shaking-related injuries include pigmentary disturbance, retinal fibrosis, and retinal folds.
To our knowledge, this is the first report of the complication of neovascularization of the disc following retinal schisis and hemorrhage from a shaking injury. Neovascularization has been reported with juvenile retinoschisis1- 4 and senile retinoschisis.5 These authors believed that retinal ischemia in the area of the schisis cavity was the mechanism. We believe that our patient also had widespread retinal ischemia on the basis of 2 mechanisms related to anteroposterior vitreous traction on the internal retina: ischemia of superficial retinal layers due to widespread retinoschisis, and ischemia of deeper retina due to disruption of the retinal capillary networks. The time of onset, between 2 and 4 months after injury, is similar to that of the neovascularization seen after ischemic central retinal vein occlusion. The straight anterior direction of the new vessels may be due to the vitreous scaffold having been compressed centrally by the schisis cavities, or alternatively to regrowth of vessels through the Cloquet canal. The tight vitreoretinal adhesions in an infant typically prevent the development of a partial or complete posterior vitreous detachment, which would also prevent growth of neovascularization on the back surface of the hyaloid cortex.
This patient had almost complete clearing of the retinal hemorrhage in the involved eye, and normally would have been seen at less frequent intervals (given the poor cortical prognosis for vision), except that the caregivers had a high level of concern about the vitreous hemorrhage and the status of the retina in the right eye and desired frequent reevaluation. With a longer follow-up interval, it is possible that the optic disc neovascularization could have progressed to rubeosis irides prior to detection. This case suggests that infants with massive retinoschisis and saturated hemorrhage due to shaking injury should be examined at least monthly for several additional visits after clearing of the retinal hemorrhage to monitor for the development of neovascularization.
Corresponding author: Sandra M. Brown, MD, Department of Ophthalmology and Visual Sciences, Texas Tech University Health Sciences Center, Sixth and Flint streets, Lubbock, TX 79430 (e-mail: firstname.lastname@example.org).
Brown SM, Shami M. Optic Disc Neovascularization Following Severe Retinoschisis Due to Shaken Baby Syndrome. Arch Ophthalmol. 1999;117(6):838-839. doi: