Ophthalmologic abnormalities are important in the evaluation of infants suspected of being abused. A common form of child abuse is shaken baby syndrome (SBS), in which an infant is violently shaken, producing rapid, abrupt acceleration and deceleration of the cranium. Retinal hemorrhages are frequently found in children with SBS. A particularly severe form of retinal injury in SBS, traumatic retinoschisis, is characterized by a dome-shaped cavity in the macula with elevated perimacular folds at the periphery of the cavity.1
The etiology of perimacular folds has been the subject of debate. Massicotte et al2 reported persistent attachment of the vitreous to the internal limiting membrane at the apices of these folds and suggested that this finding might constitute evidence of violent shaking. A recent report, however, described similar retinal findings in a 13-month-old infant who sustained a skull fracture and intracranial hemorrhage after a television fell on the infant's head.3 We describe a child with perimacular folds and retinoschisis following a severe crush injury.
A 4-month-old boy was on the floor when a 12-year-old, 63-kg child fell while running backward over an adult who was changing the infant's diaper, transmitting her entire weight through her buttocks directly to the infant's head. The infant was immediately unresponsive. Evaluation at the hospital revealed a large, comminuted, displaced parietal bone fracture; subdural and intraventricular hemorrhage; and brain herniation. Ocular examination revealed no visual responses and fixed, dilated pupils. Fundus examination revealed a large vitreous hemorrhage in the right eye. In the left eye, there was a macular retinoschisis cavity with elevated perimacular folds (Figure 1) and diffuse, 4-quadrant, multilayer retinal hemorrhage. Evaluation by the child protection team revealed no other physical findings or historical information suggestive of abuse. The child was found to be brain dead and he died. Two adults who witnessed the event were interviewed separately shortly after the incident and reported identical details. Forensic investigators determined that the incident was accidental. Histopathologic evaluation of the eyes revealed retinoschisis of the right eye with blood dissecting between the outer nuclear layer and the inner segment and extensive retinal hemorrhage of the left eye with multiple foci of hemorrhage involving all layers of the retina (Figure 2).
Fundus photograph of the left eye demonstrating elevated perimacular fold.
Postmortem photomicrograph of the left eye demonstrating perimacular fold in which the ganglion cells are obscured by severe hemorrhage. Hemorrhage is present in the intraretinal and subretinal layers (hematoxylin-eosin, original magnification ×20).
Retinal findings similar to our patient's have been described in adults with Terson syndrome. Keithahn et al4 postulated that this results from a rapid rise in intracranial pressure being transmitted through the optic nerve, resulting in extravasation of fluid from intraretinal vessels and separation of the internal limiting membrane. We postulate a similar mechanism in our patient, who had a very rapid, massive increase in intracranial pressure, resulting in hemorrhage within the perineural sheaths and separation of the retinal membranes at the outer nuclear layer.
This case demonstrates that a child may develop elevated perimacular folds from a severe head crush injury. The differential diagnosis of this abnormality includes SBS, but confusion with SBS can be eliminated by the corroborated history of severe crush head trauma and supportive physical findings.
Correspondence: Dr Lueder, St Louis Children's Hospital, Room 2S89, Washington University School of Medicine, One Children's Place, St Louis, MO 63110 (email@example.com).
Financial Disclosure: None reported.
Lueder GT, Turner JW, Paschall R. Perimacular Retinal Folds Simulating Nonaccidental Injury in an Infant. Arch Ophthalmol. 2006;124(12):1782-1783. doi:10.1001/archopht.124.12.1782