Non-accidental Eye Injuries in Pediatric Patients for the Neuro-ophthalmologist

Physical abuse, or non-accidental trauma (NAT), remains a leading cause of injury and death among young children, with abusive head trauma (AHT) being a major contributor. Common associated ocular findings include retinal hemorrhages (RH), subconjunctival hemorrhage, and optic nerve abnormalities, which are critical in diagnosing AHT. This review aims to provide neuro-ophthalmologists with a comprehensive overview of non-accidental eye injuries in pediatric patients. It explores the mechanisms of ocular injuries, common clinical presentations, differential diagnoses, and management strategies, including the use of diagnostic imaging tools like CT, MRI, and optical coherence tomography. It emphasizes the role of neuro-ophthalmologists in identifying and managing AHT-related eye injuries, highlights the importance of a multidisciplinary approach in treatment, and addresses the legal considerations associated with such cases. The findings underscore the need for timely and thorough ophthalmic evaluations to aid in the accurate diagnosis and management of AHT.

1 Title: Non-accidental eye injuries in pediatric patients for the neuro-ophthalmologist Authors: Rachelle Srinivas, DO1; Melanie Truong-Le, DO2 Affiliations: 1. Department of Neurology and Ophthalmology, Michigan State University, East Lansing, MI. 2. Department of Ophthalmology, University of Mississippi Medical Center, Jackson, MS. Corresponding Author: Rachelle Srinivas DO Department of Neurology and Ophthalmology Michigan State University 804 Service Road A217 East Lansing, MI 48824 [e-mail] Word count for entire manuscript: 2564 Word count for abstract: 187 2 Abstract Physical abuse, or non-accidental trauma (NAT), remains a leading cause of injury and death among young children, with abusive head trauma (AHT) being a major contributor. In 2022, over 550,000 children were reported to have experienced abuse or neglect, with more than 80% of infant homicides attributed to such maltreatment. AHT, which affects 21-33.8 per 100,000 children under one year, is often associated with significant ocular and intracranial injuries. Common ocular findings include retinal hemorrhages (RH), subconjunctival hemorrhage, and optic nerve abnormalities, which are critical in diagnosing AHT. This review aims to provide neuro-ophthalmologists with a comprehensive overview of non-accidental eye injuries in pediatric patients. It explores the mechanisms of ocular injuries, common clinical presentations, differential diagnoses, and management strategies, including the use of diagnostic imaging tools like CT, MRI, and optical coherence tomography. The review emphasizes the role of neuro-ophthalmologists in identifying and managing AHT-related eye injuries, highlights the importance of a multidisciplinary approach in treatment, and addresses the legal considerations associated with such cases. The findings underscore the need for timely and thorough ophthalmic evaluations to aid in the accurate diagnosis and management of AHT. 3 Introduction Physical abuse, also referred to as non-accidental trauma (NAT), is a leading cause of injury and death in young children.1,2 According to the National Children’s Alliance, the estimated annual incidence of abuse and neglect was over 550,000 children in 2022, with over 80% of cases of infant homicide attributed to child abuse.1–3 Many cases of child abuse result from abusive head trauma (AHT) with the annual incidence of AHT estimated to affect between 21-33.8 children per 100,000 children under the age of one.4 Ocular injury and intracranial findings are commonly observed in the physical examination of cases of AHT. Ocular injuries can include subconjunctival hemorrhage, periorbital ecchymosis, pupillary involvement, optic disc hemorrhages and edema, retinal detachment and traumatic retinoschisis and retinal hemorrhages (RH), among other findings.5–15 RH are among the most common ocular findings.5,11,12,16 According to Olivia et al, approximately 4-6% of children who have eye signs related to abuse are identified by an ophthalmologist.17 As a tertiary subspecialty, neuro-ophthalmologists are not the first line responders in the majority of cases of acute injuries related to child abuse. However, these patients may continue to experience consequences of injuries that involve optic nerve injuries, anterior visual pathway deficits and future morbidity from evolving consequences of ocular and ophthalmic nature. Purpose of the review The purpose of this review is to provide a comprehensive overview of non-accidental eye injuries seen in pediatric patients, specifically for neuro-ophthalmologists. This review will cover key areas, including the mechanisms of various ocular injuries, commonly observed clinical features and presentations, highlighting optic nerve involvement such as optic nerve hemorrhages, optic nerve edema, optic nerve pallor and visual pathway involvement with any associated visual field pattern defects. We also touch on broadly, the differential diagnosis and recommendations for management, and legal considerations as part of a multidisciplinary team. Epidemiology on non-accidental eye injuries Child abuse is a multifaceted problem with many contributing risk factors. Among them, demographic and social factors such as caregiver stress, income and economic factors, and parental factors have been determined in risk of child abuse.2,18–23 Financial stress is a risk factor for higher rates of child abuse, with low household income and unemployment found to be associated with an increased risk of child maltreatment.23,24 In addition, studies evaluating the association between the economic state and prevalence of child abuse have found a positive correlation between the two.16–18 Berger et al 2011 and Wood et al 2015 found a significant increase in the rate of AHT during a period of economic recession compared with rates of AHT prior to the recession. 18,20 In addition to these factors, parental factors have also been found to play a significant role in the risk of child maltreatment. Young parental age at the time of birth, especially under 17 years of age, is a strong risk factor for abuse.2 Additionally, parental education also has implications for the risk of abuse, with low parental education level found to be a predictive factor for maltreatment.2,23 Parental depression and absence of prenatal care have also been identified as a risk factor for maltreatment. 25,26 Parental age is often referenced as maternal age, it is uncertain if studies have compared an age prevalence between maternal versus paternal age and related consequences and is beyond the scope of this review.2,23 Additionally, household and family factors have also been found to have an association with the risk of abuse. Some of these include the number of children and birth order, with the second-born or subsequent 4 infant born to a young parent found to have an association with an increased risk of child abuse. 2,23 The presence of household challenges and high parental stress has also been associated with child maltreatment.25,26 Husa et al compared dynamic changes in household challenges between the pre-birth period and early childhood and found that households that were characterized by low challenges pre-birth that transitioned to high challenges during early childhood had a higher risk for abuse and being reported to child services.26 Pathophysiology and mechanisms of injury The term abusive head trauma (AHT) is used to refer to non-accidental trauma involving skull or intracranial injury in young children. AHT can occur due to violent shaking or blunt force trauma and there can be a broad spectrum of presenting symptoms and outcomes. Shaken baby syndrome (SBS) is a severe form of AHT that can be seen in cases of non-accidental trauma. It generally involves children aged five years of age and younger, with the majority of victims under the age of two. The resulting injuries and symptoms develop due to intense shaking and accelerationdeceleration forces applied to the head and neck. The development of intracranial hemorrhages is thought to result from damage and tearing of the bridging veins.27 It is also suspected that hypoxic-ischemic insult leads to the development of intraparenchymal injury, diffuse cerebral edema, and encephalopathy seen in cases of AHT.28 Similarly, the development of retinal hemorrhages is suspected to occur due to vitreoretinal traction due to intense rotational acceleration-deceleration forces.5,29,30 The classic triad of SBS findings is subdural hemorrhages, retinal hemorrhages, and encephalopathy. 5,27,28 While this combination of findings is associated with high specificity for non-accidental trauma, these findings are not pathognomonic of SBS as there is a broad spectrum of signs and symptoms that can be associated with SBS.28 Among the range of ocular findings associated with AHT, both anterior and posterior ocular findings have been reported.5–10 Fundus findings associated with AHT include retinal hemorrhages, folds, detachment, and retinoschisis.5,6,11–15,30–32 Additionally, macular cysts, optic disc edema and optic atrophy have been identified .5,6,9–12,14,28,31,32 Anterior segment findings including subconjunctival hemorrhages and pupillary involvement are seen.7–10,32,33. Cases of AHT that also include blunt force trauma have also been reported, such as in the case of direct impact injuries caused by use of corporal punishment and battered child syndrome. 34–36 Associated ocular findings include traumatic hyphema and mydriasis, lens subluxation and dislocation, development of cataracts and angle recession.34–37 Clinical presentation and diagnosis Common Ocular Findings Among the ocular findings associated with AHT, retinal hemorrhages (RH) are among the most commonly observed finding.5,38,39 In AHT, RH most commonly affects the posterior pole of the retina and the ora serrata. Involvement of these two areas is thought to result from tight vitreous attachments to the retina at these locations.6,15 The extent of RH can vary to include intraretinal involvement as well as extension to extraretinal involvement including subretinal and preretinal hemorrhages and vitreous hemorrhages. 5,11,12 RH seen in cases of AHT are generally extensive, bilateral and multilayered and have high specificity for AHT.5,6,11,12,30 Furthermore, a positive correlation between the incidence and severity of RH was found associated with the probability of abuse.12 Retinal findings in abusive head trauma (AHT) are critical findings that can help to make the diagnosis. Posterior retinal folds, particularly in the perimacular region, are often associated with subretinal 5 hemorrhages and macular edema.14,15 Retinal detachment is another significant finding in AHT, often seen in conjunction with retinal hemorrhages (RH).31,40 Traumatic retinoschisis, which involves the splitting of the retinal layers, is particularly concerning in cases of severe AHT due to its strong association with permanent vision impairment. Thus underscoring the devastating impact of such trauma on the visual system.31 The combination of perimacular retinal folds, retinoschisis, and an absent visual response has been linked to especially grave outcomes, including fatality, highlighting the severity of the damage inflicted.32 Furthermore, periorbital injuries observed in non-accidental trauma (NAT) can extend beyond the retina to include hemorrhage into the orbital fat and extraocular muscles, as well as periorbital edema and hematoma. These injuries not only indicate the extent of the trauma but also suggest the presence of significant force applied to the head and face, often corroborating the diagnosis of AHT. The constellation of these retinal and periorbital findings provides compelling evidence of the traumatic nature of the injuries, often guiding clinical and forensic evaluations in suspected cases of abuse.13,27 Neuro-Ophthalmic Findings Neuro-ophthalmologists should maintain a vigilant stance when evaluating optic nerve findings in pediatric patients with trauma as some findings in the optic nerve may be significant in the diagnosis of AHT. One key finding is that of optic nerve sheath hemorrhages, which has been observed more commonly in AHT cases compared to accidental head trauma.13These hemorrhages typically affect the immediate retrobulbar region of the optic nerve.13,14,30 Among children with optic nerve sheath hemorrhages, involvement of all layers has been observed, with significantly higher involvement of the dura in AHT victims compared to victims of accidental trauma.13 Though rare, optic disc edema has also been documented in AHT, further complicating the clinical picture and potentially signaling severe injury.5,12 Additionally, the presence of retinal hemorrhages (RH) combined with subsequent optic atrophy is a grave finding, often leading to permanent vision loss.31 The expertise of neuro-ophthalmologists in detecting and interpreting these subtle yet critical signs can benefit in the multidisciplinary setting to accurate diagnose AHT. Associated Neurological Findings In addition to the ocular manifestations of AHT, children with AHT can also have neurological findings consistent with brain injuries. One of the main concerns is intracranial hemorrhages which may include epidural, subdural, subarachnoid, and intraventricular hemorrhages.27 Among these, subdural hemorrhage is strongly associated with AHT.39,41 Beyond hemorrhages, cerebral hypoxia and ischemia may lead to the development of intraparenchymal injury, cerebral edema and encephalopathy seen in victims of AHT. 27,42 The development of cerebrovascular injury relative to AHT is considered a predictive factor to a child’s overall severity of injury.43 Children who survive AHT can face long term if not permanent disabilities, including cognitive, motor, visual, and behavioral deficits, as well as epilepsy and visual impairment.28,41 The most common cause of visual loss in cases of AHT is suspected to be due to damage to the occipital cortex.28,42,44 Diagnostic Imaging and Tools Ophthalmology consultation is standard when abusive head trauma (AHT) is a concern, with a dilated fundus examination (DFE) being the gold standard for detecting retinal hemorrhages (RH). This exam should ideally occur within 24 hours, but within 72 hours is acceptable. When available, the RetCam can be used for detailed imaging of the fundus.16,45 Although no standardized criteria exist for classifying RH 6 in AHT, the traumatic hemorrhagic retinopathy grading system categorizes RH based on grade, spread, and morphology.46 Other imaging tools, such as optical coherence tomography (OCT), help detect small macular hemorrhages and retinal traction.17,47,48 Point-of-care ultrasound is not commonly used but may identify traumatic retinoschisis if the ophthalmological exam is delayed.49 Recent studies have shown that MRI, especially susceptibility-weighted imaging (SWI-MRI), can detect retinal and intracranial hemorrhages with high specificity. This may be useful when a pediatric ophthalmologist is unavailable or a DFE is not possible due to the patient’s hemodynamic status. 38,45,50,51 Differential Diagnosis While retinal hemorrhages (RH) are strongly associated with abusive head trauma (AHT), it's important to recognize that other conditions can also present with RH.52 Diagnosing AHT is not a decision made lightly; it requires a thorough and careful assessment of the entire clinical picture for each patient. Severe RH, especially when extensive and involving multiple layers of the retina, are highly correlated with AHT.5,6,11,12,16 However, the pattern of RH must be carefully analyzed, as certain patterns can be seen in other medical conditions such as leukemia, coagulopathies, meningitis, retinal infections, arteriovenous malformations, metabolic disorders like glutaric aciduria type 1 and galactosemia, birth trauma, or even following cardiopulmonary resuscitation (CPR).52–54 The presence of additional retinal findings, such as macular folds, retinoschisis, cotton wool spots, optic disc swelling, lipid deposits, or focal white lesions, can help distinguish trauma-related RH from those caused by other conditions.52,53 Beyond ophthalmologic findings, a comprehensive evaluation that includes a detailed physical examination, skeletal survey, skin examination, and laboratory and radiologic studies is essential.7–9,27,41,55 These specialized assessments are crucial in making a confident and accurate diagnosis of AHT, ensuring that the diagnosis is based on a complete understanding of all the evidence rather than a single finding7–9,27,41,55 Management and Treatment A multidisciplinary management team is key in the diagnosis and management of abusive head trauma (AHT). Ophthalmologists are uniquely positioned to identify signs of possible abuse through detailed ophthalmic evaluations. In suspected AHT cases, it is essential to perform a comprehensive eye examination, including indirect ophthalmoscopy within 72 hours of presentation.16,45 Key findings, such as the location, pattern, and extent of retinal hemorrhages (RH), along with any optic nerve abnormalities like edema or pallor, should be meticulously documented.16,54 When available, widefield retinal photography or fundus photography can provide valuable visual evidence.53,54 Neuroimaging, particularly a head CT followed by MRI, is crucial in the acute assessment of AHT, as these studies can reveal intracranial hemorrhages and other brain injuries.54,56 Additionally, a basic laboratory workup, including blood counts and coagulation studies, is necessary to rule out other conditions.54 The management of RH is generally supportive, with most cases resolving gradually. However, prolonged vitreous hemorrhage may sometimes require surgical intervention, though this remains a debated approach.16,54 For subdural hemorrhages (SDH) in children under two, neurosurgical intervention is considered based on the severity of symptoms and imaging findings.56 Long-term complications of AHT often include neurological deficits, visual impairment, and other ophthalmic issues such as optic atrophy, disc pallor, derivational amblyopia or strabismus. 28,41,54,56–58 The 7 ongoing care of these patients requires close collaboration among neuro-ophthalmologists, neurologists, neurosurgeons, radiologists, child protective services, and social workers to ensure comprehensive management and support for affected children. Documentation and reporting Neuro-ophthalmologists, along with all physicians involved in the evaluation of suspected child abuse, carry a significant legal and ethical responsibility to report any suspected cases of abuse to child protective service agencies for further investigation.54,59 This duty is crucial, as early detection and reporting can prevent further harm to the child. The process for filing a report varies by location, and it is important for neuro-ophthalmologists to be familiar with the specific protocols in their area. Many healthcare institutions provide support through dedicated social work and child protective services staff who can assist neuroophthalmologists in navigating the reporting process. Ensuring that these steps are followed not only fulfills a legal obligation but also upholds the ethical commitment to protect vulnerable patients and advocate for their safety.59 Conclusion In conclusion, this review presents the epidemiology, pathophysiology, clinical presentation highlighting ophthalmic and neuro-ophthalmic findings, diagnosis and management of AHT, a consequence of child abuse. Child abuse is a serious medical issue that remains a leading cause of severe injury and mortality among young children, often resulting in long-term neurological and visual impairments. Neuroophthalmologists can play an important role in the multidisciplinary team tasked with diagnosing and managing cases of abusive head trauma (AHT). Early identification of ocular signs, such as retinal hemorrhages, optic nerve involvement, and other key findings, is crucial in recognizing potential abuse. The ophthalmic examination, particularly of the optic nerve and neuroimaging by neuro-ophthalmologists, may be an added expertise to the diagnostic process. Neuro-ophthalmologists should remain vigilant and well-versed in the ocular findings commonly associated with AHT and the potential long-term complications. When these findings raise suspicion of child abuse, neuro-ophthalmologists have a legal and ethical duty to report them to child protective services agencies to ensure further investigation and protection of the child. Increasing education and awareness of child abuse, along with the common presenting symptoms, including ocular findings, is essential. This knowledge helps reduce the incidence of missed cases and facilitates timely intervention and treatment, ultimately improving outcomes for affected children. 8 References 1. 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