Alex V. Levin
All forms of child abuse may have ocular manifestations. It has been estimated that 4% to 6% of cases of child abuse presents first to the ophthalmologist.
PHYSICAL ABUSE
Although any injury to the eye can be the result of child abuse, appropriate attention to history and the careful search for other signs of abusive injury is necessary to differentiate abusive from accidental injury. Some ocular findings are highly suggestive, if not diagnostic, for trauma (Table 587-1). Abuse should be considered even when there is no history of trauma. A misclassification of physical findings as abuse may also occur. For example, a blunt impact to the forehead can result in unior bilateral periocular ecchymosis (“raccoon eyes”) as blood tracts down from the forehead. Bilateral periocular ecchymosis can also be a presenting sign of neuroblastoma with orbital involvement. Thrombocytopenia or strong repetitive Valsalva maneuvers can result in pete-chiae around the eyes or on the conjunctiva.
Table 587-1. Ocular Signs of Physical Abuse
Perhaps the most common reason that an ophthalmologist is involved in child-abuse cases is to search for retinal hemorrhage when there is a concern that an infant has been injured through repetitive acceleration/deceleration mechanisms (ie, abusive head injury, or shaken baby syndrome). Blunt impact, nonabusive injury to the head causes retinal hemorrhage in less than 3% of cases;1 however, if the injury is from a severe motor vehicle accident, the prevalence may rise to 17%.2 Numerous medical conditions can also result in retinal hemorrhage.1 Yet, in abusive head injury with or without impact, the prevalence rises to 85%; two thirds of these hemorrhagic retinopathies are severe and may be hemorrhages in front of (preretinal), within (intraretinal), and under (subretinal) the retina3,4 (Fig. 587-1). The hemorrhages may be too numerous to count and may extend throughout the retina to its edge (ora serrata).
Hemorrhage extending to the ora is statistically correlated with abuse. The hemorrhages may be unilateral or asymmetric between the two eyes.3 With the exception of fatal crush injury to the head5,6 and severe fatal motor vehicle accidents characterized by repetitive acceleration/deceleration, this extensive pattern is not seen with other conditions. Traumatic splitting of the retina with blood accumulating between the layers (traumatic retinoschisis) appears to have particular specificity for abusive injury. This finding has never been reported in other children less than 5 years old, with the exception of a similar although not identical finding in victims of fatal crush injury and severe fatal motor vehicle accident. Thus, it becomes extremely important that one consider the number, type, and pattern of hemorrhages before inferring specificity for abusive injury.
Figure 587-1. Severe hemorrhagic retinopathy in a victim of abusive head injury (shaken baby syndrome). The optic nerve is seen in the center of the photo surrounded by severe preretinal and intraretinal hemorrhage, so much so that barely any normal retina is visible. Hemorrhages of this severity cover virtually the entire retina. Compare to Figure 587-2.
Although some victims of abusive head injury will have no retinal hemorrhages and some will have a sparse number of hemorrhages confined only to the posterior aspects of the retina (the posterior pole; Fig. 587-2), the latter should be considered a nonspecific but possible indicator of abuse. Short falls, cardiopulmonary resuscitation with chest compression, meningitis, thrombocytopenia, glutaric aciduria type 1, and coagulopathy are rare causes of a very nonspecific pattern of mild hemorrhages in the posterior pole.1 Seizures, apnea, isolated hypoxia, and vaccinations do not cause retinal hemorrhages. Less common findings in shaken babies include vitreous hemorrhage, retinal detachment, and total disruption of the ocular contents.
Repeated acceleration/deceleration abusive head injury, with or without impact, can cause blindness as a result of cortical visual impairment. Optic atrophy is the second most common cause of visual loss. This may be due to direct injury to the optic nerve or its blood supply in the orbit, as the globe is also experiencing repetitive acceleration/deceleration.7,8 Postmortem removal of the orbital tissues along with the globe is recommended to uncover orbital hemorrhage and injury that may provide specific evidence of abuse.9 Most researchers and clinicians now believe that the etiology of retinal hemorrhages is secondary to traction on the retina applied by the firmly attached vitreous gel as it is submitted to these forces.3 Some authors have theorized that hemorrhage may be the result of decreased retinal venous outflow due to increased intracranial pressure or increased intrathoracic pressure (as the perpetrator squeezes the child’s chest, at times hard enough to cause rib fractures). However, frank retinal venous obstruction (central retinal vein occlusion) is rarely observed, and the hemorrhages do not follow a vascular pattern. Increased intracranial pressure rarely may cause a small number of retinal hemorrhages on or around the optic nerve, particularly when papilledema is present, but a severe hemorrhagic retinopathy is not observed.
Ophthalmology consultation is essential in a child less than 5 years of age who is felt to be a possible victim of abusive head injury. Only the ophthalmologist can view the entire retina, as this requires pharmacological dilation of the pupils and indirect ophthalmoscopy. Although many pediatricians show considerable skill in identifying the presence or absence of hemorrhage, they are less able to detail the types of hemorrhage, because direct ophthalmoscopy does not visualize the edges of the retina.10 Ophthalmology consultation is necessary to delineate the type of hemorrhages and to recognize patterns that have possible specificity for abuse.11
Figure 587-2. Nonspecific hemorrhagic retinopathy. Although this is also a victim of abusive head injury (shaken baby syndrome), the number and variation in the hemorrhages is less specific and could be due to a variety of medical conditions or severe accidental injury with intracranial injury. Compare to Figure 587-1.
Retinal hemorrhages cannot be dated and may have variable resolution rates. Some can resolve even within the first 24 hours after injury, and there is evidence that hemorrhaging can worsen in the early post-traumatic period.12Where possible, ophthalmology consultation should be conducted within 24 hours of injury. The ophthalmologist should be encouraged to provide detailed descriptions along with drawings or photographs of the hemorrhages.
MEDICAL CHILD ABUSE (MUNCHAUSEN SYNDROME BY PROXY, FACTITIOUS ILLNESS BY PROXY)
The most common ophthalmic manifestations of this form of abuse are subconjunctival hemorrhage due to covert suffocation, or pupillary abnormalities, nystagmus, or other eye movement abnormalities secondary to covert poisoning. Other reported abnormalities include corneal scarring from the covert installation of noxious chemicals, periorbital and orbital cellulitis from covert periocular injections, and papillary abnormalities due to direct installation of pharmaceutical agents. For a more complete description of this syndrome, see Chapter 35.
SEXUAL ABUSE
The presence of sexually transmitted disease in the eye should be considered suspicious for abuse in non-neonatal prepubertal children. Syphilis has many ocular manifestations and is known as “the great imitator” in the eye. This infection is only transmitted sexually. Other infections with ophthalmic manifestations are known to be transmitted both sexually and nonsexually, including HIV. There is some evidence that gonorrhea and chlamydia, although only transmitted to other orifices by sexual contact, may be nonsexually transmitted to the conjunctiva due to its unique position as an external mucous membrane.13 Pubic lice may also be transmitted nonsexually. The presence of gonorrhea, chlamydia, or pubic lice should at least prompt consideration of possible covert sexual abuse and result in a full evaluation, including the culturing of other sites, interview by a trained professional experienced in this area, and examination of the genitalia and anus. If this workup is entirely negative, reporting to child protective services may not be indicated.
Adults in the home should also be tested for the possibility of infection. Herpes simplex and molluscum contagiosum are so frequently transmitted by nonsexual means that such a workup is not indicated. One must also be careful to consider the late presentation of perinatally transmitted infection, in particular chlamydia, which can present even 2 to 4 years after birth. Gonorrhea conjunctivitis in older children may not have the hyperacute purulent presentation commonly seen in neonates. Treatment should still be aggressive, as gonorrhea can result in corneal perforation.
MEDICAL NEGLECT
The failure to follow through with ophthalmic treatment can be the cause of significant visual loss. In particular, when patching therapy has been recommended for treating amblyopia, failure to comply may result in permanent legal blindness. The approach to ophthalmic neglect and noncompliance should be similar to that for any pediatric medical condition. The use of written contracts may be helpful in that child protective services may be more likely to act if a contract signed by the parent has been broken.
EMOTIONAL ABUSE
Perhaps there is a form of abuse in which children are allowed to see things that are inappropriate for their age and development. In one study,14 children witnessed a frightening amount of violence, including murders and stabbings, while in grade school and high school. Children also witness sexual activity, pornography, drug abuse, and domestic violence. Protecting children from these visual images is an important part of maintaining their psychosocial health.