Hospital for Sick Children's, The: Atlas of Pediatric Ophthalmology & Strabismus, 1st Edition

Ocular Manifestations of Systemic Disease


Child Abuse

Alex V. Levin

Child abuse is often categorized into four subgroups: physical abuse, sexual abuse, child neglect, and emotional abuse. All types of child abuse may have ocular manifestations. It is estimated that 4% to 6% of all child abuse cases will first present to an ophthalmologist. In most developed countries, ophthalmologists would be considered mandated reporters of suspected child abuse. The suspicion need not be proven by the physician. Child protective agencies, the judicial system, and the police are entities responsible for determining whether the suspicion is validated and, if so, determining who the perpetrator is. The ophthalmologist's responsibility is to report the ocular manifestations and the degree of certainty to which these findings support a diagnosis of abuse.

Inflicted eye injuries may be isolated, part of the battered child syndrome, or secondary to shaken baby syndrome. Long-term outcomes in abused children include visual loss due to amblyopia, injury, and the sequelae of brain injury (e.g., cortical visual impairment, visual field loss). Children who are victims of sexual abuse may experience the ocular effects of sexually transmitted disease (e.g., cytomegalovirus due to HIV infection [Chapter 19: Infectious Diseases, Figs. 19.9, 19.10 and 19.11]). Noncompliance and neglect are frequent problems in the ophthalmic care of children. Ophthalmologists will often see parents missing appointments made for their child and failing to comply with prescribed therapy such as patching without any apparent reason for doing so, such as financial or child care obstacles. Nonorganic failure to thrive, also known as psychosocial dwarfism, is a more dramatic and systemic manifestation of child neglect. Lastly, emotional abuse may have its ocular manifestation in what children are allowed to see. In today's society, children are increasingly and inappropriately exposed to graphic if not real images of sexual activity, drug abuse, and violence. Emotional abuse also entails severe verbal belittlement and harsh discipline.

It may be difficult to draw distinct lines along the continuums that describe the many behaviors referred to as child abuse. In some cultures, actions such as spanking, coining, and even shaking may be acceptable practices. Marriage and sexual intercourse with young adolescents are accepted in some cultures as well. Child care practices and means of discipline vary widely around the world. However, as ophthalmologists, we must uphold a standard that protects children from harm and rely on public systems and personal advocacy aimed at following through on our suspicions in any individual case that a child may indeed be at risk for, if not already a victim of, child abuse.



Figure 12.1 Shaken Baby Syndrome—Brain Injury

Shaken baby syndrome is a form of physical abuse in which the perpetrator submits a child to violent acceleration–deceleration forces with or without impact of the head. Characteristic brain injuries include subdural hemorrhage (arrows). This child's imaging suggests blood of two separate ages. The hemorrhage size is compressing the brain, causing a shift of the midline and obliteration of the ventricles. There is also diffuse severe cerebral edema with loss of gray-white differentiation. Other brain injuries in shaken baby syndrome include subarachnoid hemorrhage and, less commonly, parenchymal hemorrhage or contusion and brain laceration.


Figure 12.2 Shaken Baby Syndrome—Brain Injury

Approximately 25% to 35% of the victims of shaken baby syndrome die and another third survive with long-term neurologic sequelae including visual loss or blindness, cerebral palsy, and mental retardation. This image demonstrates the cerebral atrophy that may occur. The most common cause of visual loss following inflicted childhood neurotrauma is cortical visual impairment due to coup or contrecoup contusion of the occipital lobes or infarction of the posterior cerebral circulation as a result of severe cerebral edema.


Figure 12.3 Shaken Baby Syndrome—Rib Fractures

This child has numerous rib fractures, some of which are circled. In the “classic” form of shaken baby syndrome, the perpetrator grasps the child by the thorax and shakes him or her violently. There are usually no external signs of injury. Crying of an infant is the most common inciting event, and victims are usually less than 1 year old (although child victims as old as 5 years have been reported). Posterior and posterior–lateral rib fractures are the most common thorax injury. Other fractures occur when the child is grasped by a limb and shaken. This may result in stripping of the periosteum with subperiosteal hemorrhage and typical metaphyseal fractures, termed chip, corner, or bucket handle fractures of the long bones.


Figure 12.4 Shaken Baby Syndrome—Mild Retinal Hemorrhages

Retinal hemorrhages are the characteristic ocular manifestation of shaken baby syndrome and perhaps the most common manifestation of physical abuse that an ophthalmologist will encounter. One must carefully describe the number, type, pattern, laterality, and distribution of the hemorrhages to best document and interpret the finding. Use of the generic term “retinal hemorrhages” is discouraged. This child shows a nonspecific hemorrhagic retinopathy characterized by a small number of intraretinal (nerve fiber layer [flame hemorrhage, f] more than deeper [dot/blot] hemorrhage, d) and preretinal, p hemorrhages confined to the peripapillary region. Although shaken baby syndrome may be the most common cause of this picture in an otherwise previously well infant, there are numerous other causes that should be considered.




Figure 12.5 Shaken Baby Syndrome—Moderate Retinal Hemorrhages

As compared to Fig. 12.4, this child has a larger number of intraretinal and preretinal hemorrhages, which extend beyond the view of this photograph throughout the macula and almost to the midperiphery. Although this is more likely a victim of shaken baby syndrome, such patterns can also be seen after severe life-threatening accidental injury (e.g., motor vehicle accident), normal birth, and leukemia, and perhaps in rare cases as a result of other systemic diseases which are usually readily apparent. Note that this victim of shaken baby syndrome does not have papilledema. Papilledema occurs in less than 10% of cases.


Figure 12.6 Shaken Baby Syndrome—Severe Retinal Hemorrhages

This victim of shaken baby syndrome has a severe hemorrhagic retinopathy, with too numerous to count preretinal, intraretinal, and subretinal hemorrhages. Other than birth, there is virtually no other reported explanation for these findings, with the exception perhaps of severe fatal head crush injury. Birth flame hemorrhages resolve by 1 week (usually 2 to 3 days) and dot/blot hemorrhages by 4 to 6 weeks. Retinal hemorrhages of shaken baby syndrome can't be dated. Many of the hemorrhages pictured here have white centers. This is a very nonspecific finding that can be seen with retinal hemorrhage from any cause. Note also the venous engorgement. Venous obstruction due to increased intracranial pressure plays a role in only a minority of cases of shaken baby syndrome. Vitreoretinal interface shearing and perhaps shaking injury to orbital tissue appear to play the prominent pathogenic role.


Figure 12.7 Shaken Baby Syndrome—Peripheral Retinal Hemorrhages

Hemorrhage to the ora serrata is a sign seen in approximately two thirds of shaken baby syndrome cases. The presence or absence of signs of head impact does not influence the type or severity of hemorrhagic retinopathy. The attachment of the vitreous to this peripheral region of the retina (P) is likely the key element as the severe repetitive acceleration–deceleration forces would result in more damage to this area via vitreoretinal traction. Note also the relative sparing of the midperipheral retina (*), an area where there is less vitreoretinal adhesion. Hemorrhage in the peripheral retina is statistically more likely to indicate inflicted versus accidental injury.




Figure 12.8 Shaken Baby Syndrome—Asymmetric Retinal Hemorrhages

The retinal hemorrhages of shaken baby syndrome may be unilateral or asymmetric, as shown here. The cause for this well-recognized phenomenon is not known but may be due to the differential forces experienced by the two orbits and globes. In studies large enough to do proper statistical analysis, there does not appear to be a relationship between the sideness of the intracranial hemorrhage and the sideness of the hemorrhagic retinopathy. Likewise, Terson syndrome (intracranial plus intraocular hemorrhage usually due to trauma or subarachnoid hemorrhage in adults) is very uncommon in children.


Figure 12.9 Shaken Baby Syndrome—Purtscher Retinopathy

Note the intraretinal white patches in the macula of this child. Purtscher retinopathy is due to severe compression of the chest. Given the presence of rib fractures in some victims (Fig. 12.3), it is not surprising that Purtscher retinopathy would occasionally be seen. However, increased intrathoracic pressure is not a major component to the pathophysiology of retinal hemorrhage in child abuse. Cardiopulmonary resuscitation (CPR) rarely causes retinal hemorrhage, and if it does, only a few intra- or preretinal hemorrhages in the peripapillary area would be expected. Purtscher retinopathy has never been seen as a result of the chest compressions of CPR in animal models or humans.


Figure 12.10 Shaken Baby Syndrome—Traumatic Retinoschisis

Due to the tight adherence of the pediatric vitreous to the macula, the severe acceleration–deceleration forces of shaken baby syndrome, with or without head impact, can result in shearing of the retina such that a space is created between any layers, usually the internal limiting membrane, within which blood can accumulate. There is characteristically a curvilinear line at the edge of the elevated schisis cavity that is hemorrhagic or hypopigmented due to traumatic disruption of the retinal pigmented epithelium. Macular retinoschisis has not been reported in a child from any other condition, except perhaps severe fatal head crush injury. Hemorrhage can leave the cavity to create vitreous hemorrhage.




Figure 12.11 Shaken Baby Syndrome—Paramacular Folds

Seen best when the dome of the central schisis (Fig. 12.10) cavity collapses, ante- or postmortem, a curvilinear hemorrhagic or more often hypopigmented/white (arrows) raised fold of retina demarcates the outer edge of the traumatic retinoschisis. Blood vessels can be seen coursing up and over the fold. Hemorrhage is still present within the retina and under the internal limiting membrane within the base of the cavity. Histologic preparations may show vitreous still attached to the apex of the folds.


Figure 12.12 Shaken Baby Syndrome—Retinoschisis

When hemorrhage occurs over a blood vessel, it can become trapped under the internal limiting membrane, which is tightly adherent to the vessels. This creates a lesion that resembles the traumatic retinoschisis of shaken baby syndrome but is far less specific. Virtually any cause of hemorrhage due to a ruptured vessel can result in this finding. Note the absence of retinal folds or demarcation lines at the edge of the cavity. This child is a victim of shaken baby syndrome who also had sickle cell retinopathy (Chapter 18: Hematology,Figs. 18.1, 18.2, 18.3 and 18.4).


Figure 12.13 Shaken Baby Syndrome—Optic Atrophy

The second most common cause of visual loss following shaken baby syndrome is optic atrophy. Recent research suggests that this atrophy is due to direct optic nerve injury as a result of the severe acceleration–deceleration forces on orbital tissues. Even optic nerve sheath laceration has been observed. Retinal hemorrhage and traumatic retinoschisis (Figs. 12.10 and 12.11) rarely result in visual loss. However, as shown here, retinal scarring and pigmentary changes may occasionally occur. The optic nerve atrophy may take weeks to develop. Ophthalmic follow-up is recommended in all cases of shaken baby syndrome.




Figure 12.14 Physical Abuse

This child was the victim of a belt beating by his father. The child squirmed and the belt buckle “accidentally” struck the eye, resulting in hyphema (left image) and commotio retina (right image). The use of implements to discipline a child is considered abuse. Such behaviors set up a situation in which the caretaker is more likely to lose control of the situation. It is this loss of control, especially in periods of stress, that characterizes child abuse. Virtually any ocular injury can be the result of abuse. Some ocular findings, such as sclerocorneal laceration or avulsion of the vitreous base, are always an indicator of trauma. Findings such as unilateral cataract, optic atrophy, and cornea scarring with no other explanation might also raise the possibility of prior covert inflicted injury.


Figure 12.15 Physical Abuse

Periocular and facial injury is a not uncommon manifestation of child physical abuse. This child demonstrates both acute and chronic injuries. The head and neck are the most often injured body areas as a result of abuse. However, accidental facial injury is far more common than abuse. Forehead injury can cause bilateral periocular ecchymosis, which can mimic child abuse. Therefore, a diagnosis of abuse must also take into consideration a full ocular examination pattern of injuries and a good history. In child abuse, the history typically is inconsistent with the ocular findings or changes repeatedly with different inquiries.




Figure 12.16 Physical Abuse—Pattern Injury

The ophthalmologist should examine the child when suspicions of abuse are raised by the ocular findings if there is not another pediatric professional involved. This child demonstrates characteristic loop-shaped bruises on his back due to a beating with a belt. One can also see circular areas representing the belt buckle. An endless assortment of implements have been used on children ranging from cigarettes and cigarette lighters to straps and chords, all of which may leave a pattern of bruising that clearly could not be accidental in nature. Hand prints from a spanking or slap may also be seen.


Figure 12.17 Physical Abuse—Burns

Burning, either by forced submersion, the use of hot implements, or, as in this case, the throwing of hot water on the child, is another form of physical abuse. The sparing of the eyelid skin in this photograph indicates that the child had time to forcibly close his eyes. It is important, however, to also rule out the presence of injury to the ocular surface. Other signs of physical abuse may also be present. Burns may leave significant scarring, thus simultaneously representing both physical and emotional abuse.


Figure 12.18 Munchausen Syndrome by Proxy (Factitious Disorder by Proxy)

This child is a victim of covert suffocation by his mother, the most common form of Munchausen syndrome by proxy, a form of child abuse in which a parent, almost always the mother, engages in behaviors that result in the appearance of an illness in her otherwise well child. The perpetrator may falsify historical information, manipulate laboratory test results (e.g., spitting in a blood culture tube), or covertly cause direct physical injury. Other reported ocular signs include pupillary abnormalities due to covert poisoning or topical medication instillation, corneal scarring due to instillation of noxious chemicals, or orbital cellulitis due to the periocular injection of foreign materials. The child is usually preverbal. The perpetrator often has a history of working within a health care setting or some other familiarity with medical issues and may appear to be a very loving and cooperative caretaker. Early suspicion and recognition of this disorder can prevent physicians from engaging in unnecessary diagnostic investigations.


Figure 12.19 Sexual Abuse—Pubic Lice

Sexual abuse is a chronic secretive act in which the perpetrator is usually known to the child and can manipulate the child through coercion or threat to engage in a wide variety of acts ranging from inappropriate fondling to vaginal or anal penetration. It may be years before the child discloses the abuse. The diagnosis is made by trained child abuse professionals skilled in proper examination and interviewing techniques. The suspicion of sexual abuse may be raised when an ophthalmologist sees ocular manifestations of sexually transmitted disease in a nonneonatal prepubertal child. However, some of these infections may be transmitted nonsexually, such as the pubic lice seen here, herpes simplex, and molluscum. There is evidence that chlamydia and gonorrhea may be nonsexually transmitted to the eye, unlike the genitals or throat. It is advised that the ophthalmologist to obtain professional consultation for a full diagnostic evaluation before reporting prematurely.