Guidance for the Clinician in Rendering Pediatric Care Clinical Report—The Eye Examination in the Evaluation of Child Abuse

Alex V. Levin, MD, MHSc and Cindy W. Christian, MD, abstract COMMITTEE ON CHILD ABUSE AND NEGLECT, SECTION ON OPHTHALMOLOGY Retinal hemorrhage is an important indicator of possible abusive head trauma, but it is also found in a number of other conditions. Distin- KEY WORDS eye examination, child abuse, abusive head trauma, retinal guishing the type, number, and pattern of retinal hemorrhages may be hemorrhage helpful in establishing a differential diagnosis. Identification of ocular ABBREVIATIONS abnormalities requires a full retinal examination by an ophthalmolo- ALTE—apparent life-threatening event gist using indirect ophthalmoscopy through a pupil that has been phar- AHT—abusive head trauma macologically dilated. At autopsy, removal of the eyes and orbital tis- This document is copyrighted and is property of the American Academy of Pediatrics and its Board of Directors. All authors sues may also reveal abnormalities not discovered before death. In have filed conflict of interest statements with the American previously well young children who experience unexpected apparent Academy of Pediatrics. Any conflicts have been resolved through life-threatening events with no obvious cause, children with head a process approved by the Board of Directors. The American trauma that results in significant intracranial hemorrhage and brain Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of injury, victims of abusive head trauma, and children with unexplained this publication. death, premortem clinical eye examination and postmortem examination The guidance in this report does not indicate an exclusive of the eyes and orbits may be helpful in detecting abnormalities that can course of treatment or serve as a standard of medical care. help establish the underlying etiology. Pediatrics 2010;126:376–380 Variations, taking into account individual circumstances, may be appropriate.

BACKGROUND When a previously well child experiences an apparent life-threatening event (ALTE) or unexpected death without obvious cause, pediatricians and other physicians must attempt to identify the etiology. In the case of an ALTE, one should consider diagnoses such as gastroesophageal reflux, seizures, other central nervous system disease, metabolic dis- ease, breath-holding, and abusive head trauma (AHT). Retinal examina- tions have been used with limited success for screening ALTE victims www.pediatrics.org/cgi/doi/10.1542/peds.2010-1397 for possible AHT.1,2 Victims of AHT present to medical care with a wide range of symptoms, from mild irritability and vomiting to unexplained doi:10.1542/peds.2010-1397 coma or seizures.3 It has been estimated that approximately 4% to 6% All clinical reports from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed, 4 of abused children present first to an ophthalmologist, and the most revised, or retired at or before that time. common ocular manifestation of abuse is retinal hemorrhage. Some PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). children present with a false history of trauma, and others present Copyright © 2010 by the American Academy of Pediatrics with only the symptoms that resulted from their abuse. Unsuspecting physicians misdiagnose the condition of up to one-third of symptom- atic victims, depending on their age, severity of symptoms, and family composition.5 When a child dies unexpectedly, considerations include previously undiagnosed or new systemic disease, sudden infant death syndrome, and covert abusive injury. Retinal hemorrhages have been recognized as a key indicator of abu- sive head injury for more than 30 years, particularly in association with severe repetitive acceleration-deceleration forces with or without blunt head impact, in children younger than 5 years.6,7 Because retinal

376 FROM THE AMERICAN ACADEMY OF PEDIATRICS Downloaded from www.aappublications.org/news by guest on September 27, 2021 FROM THE AMERICAN ACADEMY OF PEDIATRICS hemorrhage rarely results in visual The range of retinal hemorrhage find- provides a wide and stereoscopic field compromise, there may be no external ings in AHT and accidental trauma is of view and enables the ophthalmolo- indicators of eye injury unless the vi- broad, and the severity of the find- gist to examine the anterior aspects of sion is significantly impaired by dam- ings can be informative of etiology. the retina to the ora serrata, which is age to the optic nerve or occipital cor- For example, retinal hemorrhage— not possible using a direct ophthalmo- tex or there is retinal detachment, predominantly intraretinal, in small scope even if the pupils are dilated. Eye significant vitreous hemorrhage, or numbers, and confined to the poste- examination for this purpose should other severe disruption of the intraoc- rior pole of the retina—can be seen be performed by an ophthalmologist. ular contents. Both eyes need to have after significant accidental head in- Attention must be paid to special fea- significant visual compromise for a jury or in AHT.6,9 More dramatic retinal tures, such as the presence of trau- child to become visually symptomatic. hemorrhages—multilayered, too nu- matic macular retinoschisis, because Therefore, one cannot rely on ocular merous to count, and extending to the these features may have particular di- signs or symptoms to determine which edge of the retina (ora serrata)—can agnostic significance for abuse. children might benefit from ophthal- be seen after AHT, normal birth, fatal Autopsy is a unique opportunity for ex- mologic consultation and postmortem extreme accidental injury (such as mo- amination not only of the eye and its eye removal. Likewise, retinal exami- tor vehicle accidents), and perhaps af- contents but also of the orbital tissues, nations should not be limited to pa- ter fatal head crush injury.6,10–13 Ocular which may yield findings helpful in dif- tients who are victims of suspected fundus examination can also reveal ferential diagnosis. This is particularly AHT. Searching for retinal hemor- findings of systemic illness that may true when a child dies before clinical rhages as diagnostic criteria for AHT shed light on the etiology of a child’s ophthalmologic consultation can be only in infants with suspected abuse symptoms, such as a cherry-red spot obtained. Even when premortem oph- creates a selection bias. in metabolic disease, retinal vascular thalmoscopy is performed, postmor- Although health care professionals abnormalities in Menkes disease, pap- tem examination is necessary for view- other than ophthalmologists may be illedema, and retinal manifestations of ing the orbital tissues. When possible, skilled at detecting the absence or leukemia or bacterial endocarditis. examination by a trained ocular pa- presence of retinal hemorrhage,8 a full There are no known retinal ocular find- thologist or ophthalmologist with ex- view of the retina and characterization ings in sudden infant death syndrome, perience in interpreting ocular pathol- of the number, types, and patterns of although routine ocular examination ogy is ideal. Postmortem eye and the hemorrhages requires consulta- has not been common practice in orbital tissue examination is another 6,14 tion by an opthalmologist using indi- these cases. means of documenting retinal hemor- rect ophthalmoscopy, preferably with Excluding retinal hemorrhages that rhage and retinoschisis but may also a dilated pupil. Even when there may are associated with vaginal delivery, reveal hemosiderin deposition from be a concern about transiently oblit- AHT is the leading cause of retinal hem- previous events and orbital findings, erating pupillary reactivity in the orrhages in infants. The association of such as hemorrhage into the fat, mus- face of a need to monitor neurologic retinal hemorrhage and AHT has been cles, and cranial nerve sheaths as well status acutely, techniques such as demonstrated repeatedly in clinical as intradural hemorrhage, all of which dilation of 1 eye at a time, use of studies.15–17 Although retinal hemor- may have diagnostic significance in short-acting mydriatics, and use of a rhages in AHT can be unilateral or bi- identifying abused children.21 lens that affords some view through lateral and vary in degree, the severity One obstacle to postmortem examina- an undilated pupil can be employed of retinal hemorrhage often parallels tion of the eyes and orbits has been a to allow indirect ophthalmoscopy, the severity of brain injury.18,19 Because societal distaste or resistance that, in preferably within the first 24 hours intraretinal hemorrhages may resolve some cases, has led to fear among pa- and ideally within 72 hours after the quickly, a retinal examination is not a thologists of legal repercussion. This child’s acute presentation. Even if substitute for brain imaging when may reflect a cultural or emotional ob- the need for eye examination is real- screening physically abused infants jection specifically to eye removal. ized after 72 hours, ophthalmologic who have no neurologic symptoms There might be a misconception that consultation may still be useful to for AHT.20 eye or orbital removal will alter the identify persistent abnormalities The ophthalmologist is in a unique po- appearance of the body postmortem such as hemorrhages, retinoschisis, sition to detail the hemorrhagic reti- at a funeral viewing when this is not, and papilledema. nopathy. The indirect ophthalmoscope in fact, the case. Techniques allow

PEDIATRICS Volume 126, Number 2, August 2010 377 Downloaded from www.aappublications.org/news by guest on September 27, 2021 for proper removal of the eye and deceleration mechanism that distin- Full ophthalmic examination by an orbits without disfigurement.6 Al- guishes this form of abuse from single- ophthalmologist and/or postmortem though consent is not routinely ob- impact trauma is the critical factor in eye removal and pathologic examina- tained for coroner cases/forensic causing retinal hemorrhage.8 Factors tion can be a critical part of the autopsies, there may be situations or such as hypoxia, anemia, and intracra- diagnostic evaluation of previously jurisdictions in which specific con- nial pressure may play important sec- well children who have experienced sent for eye and orbital tissue re- ondary roles in modulating the ap- an unexplained ALTE or death and moval may be sought. If a substitute pearance of retinal hemorrhages but also for children who have disorders decision-maker refuses this proce- do not, in and of themselves, result in in which there are known associa- dure, it may be necessary to seek le- such a retinopathy.6 Further research tions with ophthalmic findings, par- gal intervention to allow the proce- is needed to better define the role of ticularly AHT. dure to be performed. these and other factors as our under- standing of the pathophysiology and GUIDANCE FOR PEDIATRICIANS STATEMENT OF THE PROBLEM diagnostic specificity of retinal hemor- Ophthalmologic consultation should Although a retinal examination may rhage continues to evolve. be part of the diagnostic evaluation of suggest the etiology for ALTEs and pre- Second, there has been increasing rec- all previously well children younger viously unexplained early childhood ognition of the importance of detailing than 5 years who experience an unex- deaths, premortem clinical ophthal- the appearance of the hemorrhagic plained death or unexplained ALTE or mologic consultation and postmortem retinopathy rather than simply using have a systemic disorder known to removal of the eyes and orbital tissues the term “retinal hemorrhage” generi- have ocular manifestations. are not routine practice in some cen- cally. The differential diagnosis of a ● All infants and young children who ters. Those who fail to conduct these few intraretinal hemorrhages sur- present with significant intracra- procedures, particularly when there is rounding the optic nerve is vastly nial hemorrhage should have an no other explanation for the life- greater than that for “too-numerous- ophthalmologic consultation. Reti- threatening event or death, risk losing to-count” multilayered retinal hemor- nal examination is particularly im- an important opportunity to gain valu- rhages that extend to the ora serrata. portant when there is a suspicion able information. Information gained Third, there continue to be novel re- of AHT, but some retinal hemor- in such an evaluation might lead to ports of causes of retinal hemorrhage rhages may occasionally also be identifying an etiology and, in the case and other ocular findings in young found in infants and children of a surviving child, prevent death by children. These hypothesis-generating with other causes of intracranial preventing further abuse or recogniz- observations allow practitioners to hemorrhage. ing other disease. broaden and at the same time narrow ● Ophthalmologic examination should In recent years, 3 important trends differential diagnosis. For example, include, whenever possible, full indi- have emerged in the understanding of mild posterior pole retinal hemor- rect ophthalmoscopic examination hemorrhagic retinopathy in young rhages have been described in osteo- through a dilated pupil. Descrip- children. First, there has been a genesis imperfecta25 as well as head tions of findings should be detailed continued and dramatic increase in trauma sufficient to cause epidural and include the number, type, ex- the evidence supporting the diag- hemorrhage,9 and more severe hem- tent, and pattern of retinal hemor- nostic specificity of severe hemor- orrhage was observed in 2 cases of fa- rhages, if present. Retinal abnor- rhagic retinopathy as an indicator of tal head crush injury.10,11 Only with malities may be photographed after AHT, in particular, severe repeated more widespread use of ophthalmo- the clinical examination when a acceleration-deceleration with or logic consultation and postmortem oc- camera is available. When indi- without blunt head impact. Extensive ular and orbital examination can such cated, the examination should in- literature worldwide, including clinical entities be discovered and fit into the clude slit-lamp inspection of the an- studies,7 animal models,22 and com- differential diagnostic process appro- terior segment to identify signs of puter modeling,23,24 well beyond the priately. Photodocumentation, when trauma (eg, hyphema). scope of this report, support this the- available, has also proven to be an im- ● Because findings such as retinal ory. It seems that vitreoretinal traction portant ophthalmic procedure for docu- hemorrhage may be transient, it is and orbital injury sustained during menting retinal abnormalities for both desirable that the ophthalmologic the unique repetitive acceleration- clinical and educational purposes. consultation take place preferably

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within 24 hours after the patient was conducted, the eyes and orbital COMMITTEE ON CHILD ABUSE AND presents for medical care and ide- tissues should be removed en bloc at NEGLECT, 2009–2010 ally within 72 hours. autopsy per published techniques.26 Carole Jenny, MD, MBA, Chairperson Cindy W. Christian, MD ● A retinal examination is not an ap- When possible, an examination by an James E. Crawford-Jakublak, MD propriate screening test for brain ocular pathologist or ophthalmologist Emalee Flaherty, MD with experience in interpreting ocular Roberta A. Hibbard, MD injury in neurologically asymptom- Rich Kaplan, MD atic potential victims of abuse. Such pathology is preferable. LIAISONS children should undergo brain im- ● Postmortem eye removal may not Harriet MacMillan, MD – American Academy of aging as the appropriate screen. be indicated in children who have Child and Adolescent Psychiatry clearly died from witnessed severe ● When pharmacologic dilation is felt Janet Saul, PhD – Centers for Disease Control accidental head trauma or other- and Prevention to be undesirable, as for children wise readily diagnosed systemic with severe unstable central ner- STAFF medical conditions. Tammy Piazza Hurley vous system injury, timely ophthal- ● Ophthalmologic examination and/or SECTION ON OPHTHALMOLOGY mologic consultation is still needed. EXECUTIVE COMMITTEE, 2009–2010 postmortem eye and orbital tissue An attempt should still be made to Gregg T. Lueder, MD, Chairperson view the retina and optic nerve removal should be performed in all James B. Ruben, MD, Chairperson-Elect through the use of direct ophthal- cases in which a child is alleged to Richard J. Blocker, MD have suffered significant morbidity David B. Granet, MD moscopy, small pupil indirect oph- Daniel J. Karr, MD thalmoscopic techniques, sequen- secondary to a short fall or other Sharon S. Lehman, MD minor trauma disproportionate to Sebastian J. Troia, MD tial pharmacologic dilation, and/or the clinical injury and consistent fast-acting mydriatics (eg, phenyl- LIAISONS with child abuse. ephrine 2.5%). Kyle A. Arnoldi, CO – American Association of Certified Orthoptists ● When a previously well child LEAD AUTHORS Christie L. Morse, MD – American Academy of younger than 5 years dies without Alex, V. Levin, MD, MHSc – Former Section on Ophthalmology Child Abuse and Neglect Executive Michael Xavier Repka, MD – American explanation, regardless of whether Committee Member Association for Pediatric Ophthalmology a premortem retinal examination Cindy W. Christian, MD and Strabismus REFERENCES

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PEDIATRICS Volume 126, Number 2, August 2010 379 Downloaded from www.aappublications.org/news by guest on September 27, 2021 injury in the shaken baby syndrome. Arch Protective ocular mechanisms in wood- 25. Ganesh A, Jenny C, Geyer J, Shouldice M, Ophthalmol. 1989;107(10):1472–1474 peckers. Eye. 2007;21(1):83–89 Levin AV. Retinal hemorrhages in type I 20. Rubin D, Christian CW, Bilaniuk LT, Zazyczny 23. Cirovic S, Bhola RM, Hose DR, Howard IC, osteogenesis imperfecta after minor KA, Durbin D. Occult head injury in high-risk Lawford PV, Parsons MA. Mechanistic hy- trauma. Ophthalmology. 2004;111(7): abused children. Pediatrics. 2003;111(6 pt pothesis for eye injury in infant shaking: an 1428–1431 1):1382–1386 experimental and computational study. Fo- 26. Gilliland MG, Levin AV, Enzenauer RW, et al. 21. Wygnanski-Jaffe T, Levin AV, Shafiq A, et al. rens Sci Med Pathol. 2005;1(1):53–59 Guidelines for postmortem protocol for oc- Postmortem orbital findings in shaken baby 24. Rangarajan N, Kamalakkannan SB, Hasija V, ular investigation of sudden unexplained in- syndrome. Am J Ophthalmol. 2006;142(2): et al. Finite element model of ocular injury fant death and suspected physical child 233–240 in shaken baby syndrome. J AAPOS. 2009; abuse. Am J Forensic Med Pathol. 2007; 22. Wygnanski-Jaffe T, Murphy C, Smith C, et al. 13(4):364–369 28(4):323–329

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