OVERVIEW: What every practitioner needs to know
Are you sure your patient has an ocular sign of child physical abuse? What are the typical findings for this disease?
Any injury to the eye can be the result of child physical abuse. Therefore, child abuse should always be considered in the differential diagnosis of an eye injury. It has been estimated that 4%-6% of all cases of child physical abuse present first to the ophthalmologist. Certain ocular signs indicate that trauma has occurred even when there is no history of trauma. These signs include:
Retinal bruising (also known as commotio retinae or Berlin edema)
Optic nerve avulsion
Vitreous base detachment
Orbital bone fracture
Some findings usually indicate trauma but may have uncommon medical causes. These include:
Tears of the pupil margin
Trauma should also be considered in the presence of:
Unilateral ectopia lentis
Unilateral optic atrophy
These latter findings are usually more likely.
Medical child abuse can result in an injury to the eye through covert means such as the instillation of noxious chemicals that cause corneal scarring or pupillary abnormalities or as a result of systemic ingestions with neurologic manifestations such as nystagmus and strabismus.
When examining a child with an ocular injury, be sure to examine the entire body for other signs of child physical abuse.
Perhaps the most common manifestation of physical child abuse is the hemorrhagic retinopathy of abusive head trauma characterized by repetitive acceleration-deceleration forces with or without blunt head impact (shaken baby syndrome). In 85% of cases, retinal hemorrhages are too numerous to count, multilayered (preretinal, intraretinal, and subretinal), and extend throughout the entire retinal surface to the peripheral retina (ora serrata). In approximately one third of cases, there may also be a traumatic splitting of the retina (retinoschisis) with or without a surrounding circumlinear fold, hypopigmented line, or hemorrhagic line. Blood accumulates within the “cyst” formed by the splitting of retinal layers.
Other ocular manifestations of abusive head trauma include cortical visual loss from brain injury and optic atrophy. Less commonly, more severe disruption of the intraocular contents may be observed (e.g., retinal detachment).
What other disease/condition shares some of these symptoms?
Accidental injury can cause all the same physical injuries to the eyeball, orbit, and surrounding tissues as child physical abuse. Spontaneous hyphema may be caused by abnormal blood vessels on the surface of the iris (iris neovascularization), leukemia, juvenile xanthogranulomatosis, and other uncommon conditions. Pericular ecchymosis can be caused by a bruise to the forehead that tracks down under the skin. Unilateral bilateral periocular ecchymosis can be a manifestation of neuroblastoma or leukemia of the orbit.
Abnormalities of the pupil structure can be caused by inflammation inside the eye (iritis). Although subconjunctival hemorrhage is rare in childhood, it may be the result of normal birth pertussis. Children with thrombocytopenia may also have conjunctival petechiae or small subconjunctival hemorrhage after straining or crying.
A small number of intraretinal and preretinal hemorrhages confined to the posterior pole (peripapillary and macular retina) have a very broad differential diagnosis beyond the scope of this chapter. Severe multilayered retinal hemorrhages too numerous to count covering the retinal surface and traumatic retinoschisis have a much shorter differential diagnosis. These findings have been observed in:
Fatal head crush injury
Fatal motor vehicle accidents
Extremely severe falls (e.g., 11 meters onto concrete).
Short falls do not appear to cause severe hemorrhagic retinopathy or retinoschisis. Normal birth can result in severe hemorrhagic retinopathy without retinoschisis. Leukemia can result in severe hemorrhagic retinopathy with blood under the internal limiting membrane of the retina, which has an appearance similar to retinoschisis. These patients usually also have leukemic infiltrates of the retina. Severe retinal hemorrhage has also been reported in fatal purulent meningitis and infantile spontaneously ruptured aneurysm. Fortunately, these conditions are easy to differentiate from child physical abuse by history, radiologic findings, and laboratory findings.
What caused this disease to develop at this time?
Injuries of the eyeball, orbit, and surrounding tissues are usually a manifestation of blunt head impact. Such impact can also result in optic atrophy (even without direct impact to the eyeball) or brain injury. Multiple lines of research indicate that the main mechanism for the development of retinal hemorrhage in abusive head trauma is through vitreoretinal traction, which occurs as the child is submitted to repeated acceleration-deceleration forces.
The vitreous of infants is firmly attached to the retina, particularly in the macula where retinoschisis occurs and at the area of the ora serrata where one sees peripheral hemorrhages in victims of shaken baby syndrome. There also appears to be injury to the tissues of the orbit, presumably from the globe translating within the orbit during these repeated acceleration-deceleration (“shaking”) events. Other factors such as hypoxia, increased intracranial pressure, and increased intrathoracic pressure may also play a smaller role but by themselves do not cause severe hemorrhagic retinopathy.
What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?
Any hemorrhagic manifestation, including retinal hemorrhages, should evoke an evaluation for the possibility of coagulopathy and include at the least, a complete blood count with differential, prothrombin time/partial thromboplastin time/international normalized ratio, and testing for von Willebrand disease, with additional tests as indicated. The role of thrombophilia, which affects 5% of the North American population, is not well known, but testing may be considered.
Would imaging studies be helpful? If so, which ones?
When child physical abuse is expected based on the presence of an eye injury in children younger than 2 years of age, consider a radiologic skeletal survey. Neuroimaging may also be indicated, as the injury does indicate that trauma was applied to the head. Magnetic resonance imaging of the brain is better than computed tomography in revealing the common neurotrauma seen in shaken baby syndrome.
Confirming the diagnosis
Although there are unknown specific algorithms for clinical decision making with regard specifically to the ocular signs of child physical abuse, full-body examination, appropriate radiologic investigation, and laboratory testing as indicated above would be routine. A protocol for ocular examination in the setting of suspected abuse of head injury had been published by Levin and Christian.
If you are able to confirm that the patient has ocular manifestations of child physical abuse, what treatment should be initiated?
The treatment of injuries of the periocular tissues, eyeball, and orbit as a result of child physical abuse does not differ from the treatment of these same injuries when they result from accidental trauma, although reporting of suspected child abuse is mandated throughout North America. Consultation with a child abuse pediatrician/team is advised. Careful description and photodocumentation of the injuries in encouraged.
There is no specific treatment for retinal hemorrhage. Most hemorrhage will resolve without visual compromise. Hemorrhage in the fovea or subretinal hemorrhage under the macula can result in fibrosis and pigmentary alteration with long-term visual loss or amblyopia. Traumatic retinoschisis likewise usually does not result in visual compromise but can result in long-term retinal fibrosis and retinal distortion with secondary visual abnormalities.
Surgery to remove the blood in a retinoschisis cyst has not been reported to yield satisfactory outcomes. Vitreous hemorrhage that extends out of a retinoschisis cavity may require surgical removal if spontaneous resolution does not occur. Most importantly, continued follow-up is required to detect visual loss and treat ambloypia that may result from blood or scarring in the visual axis as a result of abusive head injury. Likewise, visual injury due to cortical damage or optic nerve atrophy may be a later manifestation after the acute presentation and require low-vision intervention as part of the long-term rehabilitation program.
What are the adverse effects associated with each treatment option?
The adverse effects of treatment for ocular trauma is beyond the scope of this chapter. In almost every instance, the risks of treatment are far outweighed by the benefits. With regard to retinoschisis, surgical risks of retinal surgical intervention appear to be greater than the risk of observation and long-term monitoring with patching treatment for amblyopia.
What are the possible outcomes of the ocular manifestations of child physical abuse?
Prognostic information for each type of ocular trauma should be tailored to the particular findings present. Victims of abusive head injury should be given a guarded prognosis, as visual compromise resulting from the optic atrophy and cortical visual impairment may not become appreciated until some time after the initial injury. Vitreous hemorrhage after retinoschisis may also be delayed. Sequential examination is critical to monitor for amblyopia and late complications.
What causes this disease and how frequent is it?
The causes and epidemiology of child physical abuse are covered elsewhere in this program. There are no particular data regarding the ocular manifestations of child physical abuse other than those described above. In 85% of shaken baby syndrome cases, retinal hemorrhages are present. In approximately two thirds of patients with retinal hemorrhages, the hemorrhages are too numerous to count, are multilayered (preretinal, intraretinal, and subretinal), and extend throughout the entire retinal surface to the peripheral retina (ora serrata).
Although there are no known genetic factors in the incidence of the ocular signs of child physical abuse, we do know that overreporting of child physical abuse and a greater consideration of this diagnosis is found in children of visible minority from lower socioeconomic classes. Likewise, underreporting and less consideration of the diagnosis is given to white patients of higher socioeconomic class.
Other clinical manifestations that might help with diagnosis and management
When considering the possibility of an ocular injury as the result of physical child abuse, one should carefully conduct the history and physical examination to look for other indicators of abuse. A changing or inconsistent history or the absence of a history of trauma in the presence of a finding that is clearly traumatic should certainly raise concern. Is the ocular injury a sign of a further eye or brain injury? Complete ocular assessment, often including neuroimaging, is important.
Are additional laboratory studies available; even some that are not widely available?
In particular regarding retinal hemorrhages, some physicians prefer a wider diagnostic evaluation for the possibility of coagulopathy, which might include factor levels, proteins induced by vitamin K deficiency or antagonists (PIVKA), bleeding time (or preferably a platelet function assay (PFA-100) with the PFA-100). Coagulopathy does not cause severe hemorrhagic retinopathy but rather a countable number of intraretinal and preretinal hemorrhages confined to the posterior pole or isolated vitreous hemorrhage. A thrombophilia panel may also be considered. If clinically indicated, appropriate cultures to rule out infection should be considered.
How can ocular signs of child physical abuse be prevented?
Although there are many prevention strategies in place with regard to shaken baby syndrome (e.g., National Center on Shaken Baby Syndrome at http://www.dontshake.org/sbs.php?topNavID=4&subNavID=32&navID=664), there are no specific prevention strategies for the ocular manifestations of child physical abuse.
What is the evidence?
Levin, AV. “Retinal hemorrhage in abusive head injury: state of the art review”. Pediatrics. vol. 126. 2010. pp. 961-70.
Levin, AV, Christian, CW. “Committee on Child Abuse and Neglect and Section on Ophthalmology. The eye examination in the evaluation of child abuse”. Pediatrics. vol. 126. 2010. pp. 376-80.
Ongoing controversies regarding etiology, diagnosis, treatment
There has been some discussion in the literature, but more often in the courtroom, as to the forensic significance and pathophysiology of retinal hemorrhages in infants and young children. Multiple lines of research, including clinical studies on live human victims of abusive head trauma, children with other medical conditions that have overlapping signs and symptoms, postmortem studies, and studies with animals, dummies, and computer modeling, in addition to analysis of perpetrator confessions, inform us clearly that brain and ocular injury as a result of abusive head trauma characterized by repeated acceleration-deceleration trauma with our without blood head impact does indeed occur.
Much debate has centered around inaccurate characterization of diagnostic criteria such as the isolated use of a triad (cerebral edema, subdural hemorrhage, and retinal hemorrhage), which is in fact a fallacy. In every case, the diagnosis is made with other findings and consideration of the history as well as testing to rule out related conditions.
Some debate has focused on the possibility of a role for infantile vaccines, increased intracranial pressure, increased intrathoracic pressure, and hypoxia. There is virtually no evidence that these cause severe hemorrhagic retinopathy. Indeed, there is virtually no evidence that immunizations or hypoxia cause hemorrhage in the eye at all. Increased intracranial pressure is associated with papilledema, which in turn may be associated with peripapillary intraretinal and preretinal hemorrhages but not a severe extensive hemorrhagic retinopathy except in extraordinary and easily diagnosed situations such as infantile aneurysm or severe fatal head crush injury. Even in these circumstances, the role of increased intracranial pressure is not well understood.
The adversarial courtroom system will always promote debates among witnesses, and many hypotheses continue to be generated regarding the relative contributions of numerous medical factors in any given case. Nonetheless, severe extensive multilayered retinal hemorrhage with or without retinoschisis, except as discussed above, appears to be due to the unique mechanisms of injury with vitreoretinal traction and perhaps globe movement within the orbit that are seen in the shaken baby syndrome.
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- OVERVIEW: What every practitioner needs to know
- Are you sure your patient has an ocular sign of child physical abuse? What are the typical findings for this disease?
- What other disease/condition shares some of these symptoms?
- What caused this disease to develop at this time?
- What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?
- Would imaging studies be helpful? If so, which ones?
- Confirming the diagnosis
- If you are able to confirm that the patient has ocular manifestations of child physical abuse, what treatment should be initiated?
- What are the adverse effects associated with each treatment option?
- What are the possible outcomes of the ocular manifestations of child physical abuse?
- What causes this disease and how frequent is it?
- Other clinical manifestations that might help with diagnosis and management
- Are additional laboratory studies available; even some that are not widely available?
- How can ocular signs of child physical abuse be prevented?
- What is the evidence?
- Ongoing controversies regarding etiology, diagnosis, treatment