ANDREA C. TONGUE, MD
Lake Oswego, OR
Child abuse has occurred throughout recorded civilization and has received considerable attention in the medical literature. In 1860, Ambroise Tardieu,1 a French physician, published the results of autopsies performed on 32 children who died of injuries, mainly inflicted by their parents. Caffey, 2 in 1946, suggested that multiple fractures in long bones in infants with chronic subdural hematomas were secondary to trauma. Two of these children also had retinal hemorrhages. Caffey recognized that only trauma could explain the spectrum of injuries, yet he failed to implicate intentional trauma and stated that the injuries could be the result of forgotten or relatively minor traumatic episodes. Seven years later, Silverman3 emphasized that children with multiple unexplained fractures may actually be the victims of trauma intentionally inflicted by their caretakers. In 1961, Kempe organized a multidisciplinary symposium,4 “Battered Child Syndrome,” which was instrumental in alerting the media, professionals, and the public to the problem of child abuse. This symposium was largely responsible for the legislation that protects children. By 1966, all states, except one, had laws mandating that physicians report suspected child abuse.
The first article in the ophthalmologic literature describing ocular injuries resulting from child abuse appeared in 1964.5 It was followed by a number of articles dealing with ocular abnormalities in children suffering from probable abuse. Findings most commonly described were periorbital bruising or edema and retinal hemorrhages. However, these were rarely concomitant findings. Retinal hemorrhages occurred predominantly in children with signs or symptoms of central nervous system (CNS) injuries. In 1974 Caffey6 reported the prominent feature of the shaken baby syndrome: the paucity of external or radiologic signs of injury to the head or neck in infants or young toddlers who had intracranial and retinal hemorrhages. Ophthalmologists may be asked to examine possible victims of abuse to determine whether retinal hemorrhages exist; they may be asked whether the existence of the retinal hemorrhages indicates deliberate or accidental trauma. Although it is possible that certain types of retinal hemorrhages and retinoschisis are a sign of shaken baby syndrome,7 to date there is no evidence that clearly establishes that retinal hemorrhages, be they intraretinal, subretinal, or subhyaloid, are indicative of nonaccidental trauma. Evidence does exist, however, that retinal hemorrhages in all layers of the retina occur in experimental8,9 as well as clinical situations that are not related to child abuse. They are seen in newborns, in some infant eyes after cataract surgery, in infants undergoing extracorporeal membrane oxygenation therapy, in infants with subdural or subarachnoid hemorrhages secondary to accidental trauma, and in infants with bleeding dyscrasias and hemoglobinopathies. Central nervous system trauma (and associated retinal hemorrhages) from abuse occurs predominantly in children younger than 2 years of age and occurs in this age group with much greater frequency than accidental CNS trauma. It is difficult to obtain an age-matched control group of children who present with CNS injuries from accidental trauma to ascertain whether the same type of retinal pathology occurs in this group. Age-matched controls are critical in order to rule out factors in the vascular system, hemodynamics, and immaturity of ocular and brain tissue that may predispose infants to retinal hemorrhages with greater frequency than older children.
In this respect, it is interesting to note that Lange’s fold occurs only in children’s eyes. This fold is a fixation artifact, seen at the ora serrata in post mortem fixed eyes, and according to Yanoff and Fine9 may be caused by greater adhesions of the inner retinal surface to the vitreous base than the adhesions of the retina to the retinal pigment epithelium in this age group. Could this also be a factor contributing to the formation of retinal folds as described by Massicotte et al11 in this month’s issue of Ophthalmology? They describe circumferential retinal folds in the posterior pole of the eyes of three infants, one of whom was presumed to have been shaken only, the other two showing the additional evidence of direct head trauma. Histologically, the folds were associated with vitreous traction, and the authors hypothesize that vitreous traction secondary to shaking is the cause of the folds. They are, however, careful to point out that controlled studies are needed to establish that these folds do not occur in accidental trauma. Of interest in this respect is an isolated case report by Wolter12 describing a circumferential retinal fold in one eye obtained from an adult who died in a motor vehicle accident. The fold was located anteriorly near the ora serrata, and was associated with peripheral epiretinal acellular membranous changes. Of Massicotte et al’s cases, two had peripheral vitreous base hemorrhages and one had hemorrhages posterior to the folds. Clinically, in my experience, the folds have always been associated with adjacent massive hemorrhages. Could massive retinal hemorrhages in an eye that has plasticity of movement of the retina in relation to the pigment epithelium play a role in the formation of the folds?
Until it is unequivocally proven that retinal folds are secondary to dynamic vitreous traction and shaking and not some other factors, it is imperative that we not equate retinal folds with child abuse, just as we cannot equate the presence of retinal hemorrhage with child abuse. Nonaccidental trauma is still a diagnosis of exclusion. The diagnosis needs to be entertained and vigorously pursued if the facts or explanation do not adequately explain the nature or extent of the injury. Careful documentation of the clinical findings in children who have sustained trauma, accidental or deliberate, is critical in establishing the spectrum of physical findings in these patients. Trauma is the culprit; whether it was accidental or not is known only with absolute certainty by the perpetrator or a direct observer. Physicians can and must make an educated assessment as to the probable cause of the injury: accidental trauma or intentional trauma. They should not be afraid to give their opinion as to the probability that the injury could or could not have resulted from the circumstances leading to it. Reports such as the one published in this issue of Ophthalmology are to be encouraged. It must be emphasized, however, that the ophthalmologic findings cannot be reported in isolation; they need to be documented with all of the other physical findings in these children. Most important, a sufficient number of eyes of children who sustain accidental CNS trauma during infancy need to be examined clinically and histopathologically to ascertain whether certain ocular manifestations are pathognomonic for certain types of trauma. By correlating clinical with histopathologic findings, patterns may be established that may more accurately pinpoint the pathophysiology of the injury.
References
1. [Auguste] Ambroise Tardieu. Cited in Hems M. The “battered child” revisited. JAMA 1984: 251:3295-300.
2. Caffey J. Multiple fractures in the long bones of infants suffering from chronic subdural hematoma. Am J Roentgenol 1946;
56:163-73.
3. Silverman FN. The roentgen manifestations of unrecognized skeletal trauma in infants. AJR Am J Roentgenol 1953; 69:413-27.
4. Kempe CH, Silverman FN, Steele BF, et al. The battered-child syndrome. JAMA 1962; 181:17-24,
5. Kiffney GT, Jr. The eye of the “battered child.” Arch Ophthalmol 1964; 72:231-3.
6. Caffey J. The whiplash shaken infant syndrome: manual shaking by the extremities with whiplash-induced intracranial and intraocular
bleeding, linked with residual permanent brain damage and mental retardation. Pediatrics 1974; 54:396-403.
7. Greenwald MJ, Weiss A. Oesterle CS, Friendly DS. Traumatic retinoschisis in battered babies. Ophthalmology 1986: 93:618-
25.
8. Ommaya AK, Faas F, Yarnell P. Whiplash injury and brain damage an experimental study. JAMA 1968; 204:285-9.
9. Smith DC, Kearns TP, Sayre GP. Preretinal and optic nerve-sheath hemorrhage: pathologic and experimental aspects in subarachnoid
hemorrhage. Trans Am Acad Ophthalmol Otolaryngol 1957; 61:201-11.
10. Yanoff M, Fine B. Ocular Pathology: A Text and Atlas, 2nd ed. Philadelphia: Harper &, Row, 1982.
11. Massicotte SJ, Folberg R, Torczynski E, et al. Vitreoretinal traction and perimacular retinal folds in the eyes of deliberately traumatized
children. Ophthalmology 1991; 98:1124-7.
12. Wolfer JR. Circular fixed fold of the retina. Am J Ophthalmol 1965; 60:805-11.
Lake Oswego, OR
Child abuse has occurred throughout recorded civilization and has received considerable attention in the medical literature. In 1860, Ambroise Tardieu,1 a French physician, published the results of autopsies performed on 32 children who died of injuries, mainly inflicted by their parents. Caffey, 2 in 1946, suggested that multiple fractures in long bones in infants with chronic subdural hematomas were secondary to trauma. Two of these children also had retinal hemorrhages. Caffey recognized that only trauma could explain the spectrum of injuries, yet he failed to implicate intentional trauma and stated that the injuries could be the result of forgotten or relatively minor traumatic episodes. Seven years later, Silverman3 emphasized that children with multiple unexplained fractures may actually be the victims of trauma intentionally inflicted by their caretakers. In 1961, Kempe organized a multidisciplinary symposium,4 “Battered Child Syndrome,” which was instrumental in alerting the media, professionals, and the public to the problem of child abuse. This symposium was largely responsible for the legislation that protects children. By 1966, all states, except one, had laws mandating that physicians report suspected child abuse.
The first article in the ophthalmologic literature describing ocular injuries resulting from child abuse appeared in 1964.5 It was followed by a number of articles dealing with ocular abnormalities in children suffering from probable abuse. Findings most commonly described were periorbital bruising or edema and retinal hemorrhages. However, these were rarely concomitant findings. Retinal hemorrhages occurred predominantly in children with signs or symptoms of central nervous system (CNS) injuries. In 1974 Caffey6 reported the prominent feature of the shaken baby syndrome: the paucity of external or radiologic signs of injury to the head or neck in infants or young toddlers who had intracranial and retinal hemorrhages. Ophthalmologists may be asked to examine possible victims of abuse to determine whether retinal hemorrhages exist; they may be asked whether the existence of the retinal hemorrhages indicates deliberate or accidental trauma. Although it is possible that certain types of retinal hemorrhages and retinoschisis are a sign of shaken baby syndrome,7 to date there is no evidence that clearly establishes that retinal hemorrhages, be they intraretinal, subretinal, or subhyaloid, are indicative of nonaccidental trauma. Evidence does exist, however, that retinal hemorrhages in all layers of the retina occur in experimental8,9 as well as clinical situations that are not related to child abuse. They are seen in newborns, in some infant eyes after cataract surgery, in infants undergoing extracorporeal membrane oxygenation therapy, in infants with subdural or subarachnoid hemorrhages secondary to accidental trauma, and in infants with bleeding dyscrasias and hemoglobinopathies. Central nervous system trauma (and associated retinal hemorrhages) from abuse occurs predominantly in children younger than 2 years of age and occurs in this age group with much greater frequency than accidental CNS trauma. It is difficult to obtain an age-matched control group of children who present with CNS injuries from accidental trauma to ascertain whether the same type of retinal pathology occurs in this group. Age-matched controls are critical in order to rule out factors in the vascular system, hemodynamics, and immaturity of ocular and brain tissue that may predispose infants to retinal hemorrhages with greater frequency than older children.
In this respect, it is interesting to note that Lange’s fold occurs only in children’s eyes. This fold is a fixation artifact, seen at the ora serrata in post mortem fixed eyes, and according to Yanoff and Fine9 may be caused by greater adhesions of the inner retinal surface to the vitreous base than the adhesions of the retina to the retinal pigment epithelium in this age group. Could this also be a factor contributing to the formation of retinal folds as described by Massicotte et al11 in this month’s issue of Ophthalmology? They describe circumferential retinal folds in the posterior pole of the eyes of three infants, one of whom was presumed to have been shaken only, the other two showing the additional evidence of direct head trauma. Histologically, the folds were associated with vitreous traction, and the authors hypothesize that vitreous traction secondary to shaking is the cause of the folds. They are, however, careful to point out that controlled studies are needed to establish that these folds do not occur in accidental trauma. Of interest in this respect is an isolated case report by Wolter12 describing a circumferential retinal fold in one eye obtained from an adult who died in a motor vehicle accident. The fold was located anteriorly near the ora serrata, and was associated with peripheral epiretinal acellular membranous changes. Of Massicotte et al’s cases, two had peripheral vitreous base hemorrhages and one had hemorrhages posterior to the folds. Clinically, in my experience, the folds have always been associated with adjacent massive hemorrhages. Could massive retinal hemorrhages in an eye that has plasticity of movement of the retina in relation to the pigment epithelium play a role in the formation of the folds?
Until it is unequivocally proven that retinal folds are secondary to dynamic vitreous traction and shaking and not some other factors, it is imperative that we not equate retinal folds with child abuse, just as we cannot equate the presence of retinal hemorrhage with child abuse. Nonaccidental trauma is still a diagnosis of exclusion. The diagnosis needs to be entertained and vigorously pursued if the facts or explanation do not adequately explain the nature or extent of the injury. Careful documentation of the clinical findings in children who have sustained trauma, accidental or deliberate, is critical in establishing the spectrum of physical findings in these patients. Trauma is the culprit; whether it was accidental or not is known only with absolute certainty by the perpetrator or a direct observer. Physicians can and must make an educated assessment as to the probable cause of the injury: accidental trauma or intentional trauma. They should not be afraid to give their opinion as to the probability that the injury could or could not have resulted from the circumstances leading to it. Reports such as the one published in this issue of Ophthalmology are to be encouraged. It must be emphasized, however, that the ophthalmologic findings cannot be reported in isolation; they need to be documented with all of the other physical findings in these children. Most important, a sufficient number of eyes of children who sustain accidental CNS trauma during infancy need to be examined clinically and histopathologically to ascertain whether certain ocular manifestations are pathognomonic for certain types of trauma. By correlating clinical with histopathologic findings, patterns may be established that may more accurately pinpoint the pathophysiology of the injury.
References
1. [Auguste] Ambroise Tardieu. Cited in Hems M. The “battered child” revisited. JAMA 1984: 251:3295-300.
2. Caffey J. Multiple fractures in the long bones of infants suffering from chronic subdural hematoma. Am J Roentgenol 1946;
56:163-73.
3. Silverman FN. The roentgen manifestations of unrecognized skeletal trauma in infants. AJR Am J Roentgenol 1953; 69:413-27.
4. Kempe CH, Silverman FN, Steele BF, et al. The battered-child syndrome. JAMA 1962; 181:17-24,
5. Kiffney GT, Jr. The eye of the “battered child.” Arch Ophthalmol 1964; 72:231-3.
6. Caffey J. The whiplash shaken infant syndrome: manual shaking by the extremities with whiplash-induced intracranial and intraocular
bleeding, linked with residual permanent brain damage and mental retardation. Pediatrics 1974; 54:396-403.
7. Greenwald MJ, Weiss A. Oesterle CS, Friendly DS. Traumatic retinoschisis in battered babies. Ophthalmology 1986: 93:618-
25.
8. Ommaya AK, Faas F, Yarnell P. Whiplash injury and brain damage an experimental study. JAMA 1968; 204:285-9.
9. Smith DC, Kearns TP, Sayre GP. Preretinal and optic nerve-sheath hemorrhage: pathologic and experimental aspects in subarachnoid
hemorrhage. Trans Am Acad Ophthalmol Otolaryngol 1957; 61:201-11.
10. Yanoff M, Fine B. Ocular Pathology: A Text and Atlas, 2nd ed. Philadelphia: Harper &, Row, 1982.
11. Massicotte SJ, Folberg R, Torczynski E, et al. Vitreoretinal traction and perimacular retinal folds in the eyes of deliberately traumatized
children. Ophthalmology 1991; 98:1124-7.
12. Wolfer JR. Circular fixed fold of the retina. Am J Ophthalmol 1965; 60:805-11.