Perimacular retinal folds from childhood head trauma
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《英国医生杂志》
1 Department of Pathology, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA, 2 Department of Paediatrics, Wake Forest University School of Medicine, 3 Department of Ophthalmology, Wake Forest University School of Medicine
Correspondence to: P E Lantz plantz@wfubmc.edu
Introduction
A forensic autopsy showed no direct trauma to the orbits or eyes. There were prominent bilateral scalp contusions with soft tissue and intramuscular haemorrhage, symmetrical parietal skull fractures with coronal sutural diastasis, and a lacerated dura mater with extrusion of brain and blood. In addition to bilateral subdural and subarachnoid haemorrhages, a thin epidural haematoma partially covered the frontoparietal, calvarial lamina interna. The brain showed bilateral cortical contusions, severe cerebral oedema, and diffuse anoxic-ischemic injury. Postmortem ocular examination showed haemorrhages of the optic nerve sheaths with subdural haemorrhage greater than subarachnoid haemorrhage. Both eyes had extensive retinal haemorrhages with perimacular retinal folds (fig 2). Retinoschisis and peripapillary intrascleral haemorrhages were evident, and the retinal haemorrhages extended from the posterior pole to the ora serrata affecting the preretinal, intraretinal, and subretinal layers.
Fig 2 Transilluminated retinal image of right eye at autopsy showing circinate, elevated, perimacular retinal fold and extensive retinal haemorrhages
When investigators went to the house to recover the television before the family returned home, it was still on the carpeted floor. The 480 mm screen television with built in videocassette recorder weighed 19.5 kg. The homemade television stand measured 762 mm (height)x635 mm (width)x508 mm (depth) and had a bottom drawer that held videotapes. A greasy smudged area on the glass of the television corresponded with the impact site on the child's head.
A re-enactment in which a 11.4 kg weight (similar to the child's weight at autopsy of 11.8 kg) was placed on the partially opened drawer caused the television and television stand to readily topple forward. According to investigators, the family home was 7.8 km from the workplace and about 6 km from the local hospital. Based on the distance and estimated driving times plus workplace time clock records, the father was home with the children about 20 minutes when the incident happened. The day after the incident, while in foster care, the 3 year old sibling corroborated the father's account. Despite all this evidence, the paediatric ophthalmologist repeated that perimacular retinal folds coincident with retinal haemorrhages were considered specific for shaken baby syndrome secondary to retinal traction exerted by the oscillating vitreous.
Search for published evidence
We found 42 articles and book chapters discussing perimacular retinal folds in childhood abusive head trauma. Seventeen mentioned the presence of retinal folds in non-accidental head injury but did not comment on specificity or formative mechanism. A table on bmj.com gives details of the remaining articles. All but two of the articles are non-comparative clinical or autopsy case series, case reports, review articles, or book chapters.
The two studies that included controls both showed bias in selection of controls and contained no cases with perimacular retinal folds but discussed the postulated causal mechanism.8 9 In the prospective controlled study, the authors reported on 79 children younger than 3 years who had sustained head injuries.8 The manner of injury in one case was indeterminate. Three children, including one who died, had non-accidental head injury diagnosed, all of whom had retinal haemorrhages; 72 of the 75 children with non-abusive injuries were managed by observation alone. No perimacular retinal folds were observed; however, the presumed causative mechanism of traumatic retinoschisis and retinal folds was discussed.
The second controlled study was a prospective autopsy study that examined the presence and location of ocular findings in 169 childhood deaths.9 Ocular haemorrhages (retinal, peripheral retinal, optic nerve sheath and intrascleral) were more likely in craniocerebral trauma than in non-head injuries and natural diseases. Although case selection was purportedly random, the study contained a disproportionately high number of deaths from child abuse compared with natural and non-abusive causes. Case selection depended on the pathologist's willingness to participate in the study, and we were told by one of the authors that pathologists were more willing to participate when they believed that the deaths were abusive or suspicious (M Gilliland, personal communication, 2002). Perimacular retinal folds were not noted, but the authors concluded that acceleration-deceleration injury to the retina accounts for peripheral retinal haemorrhages and retinal folds.
Supporting evidence
Statements in the medical literature that perimacular retinal folds are diagnostic of shaken baby syndrome are not supported by objective scientific evidence. Non-comparative observational reports and unsystematic narrative review articles contain insufficient evidence to provide unbiased support for or against diagnostic specificity, and inferences about associations, causal or otherwise, cannot be determined. Clinical and autopsy evidence of ocular lesions must therefore be considered alongside other physical findings and a thorough investigation before concluding whether a head injury is caused by abuse. The child in our case had ocular haemorrhages (peripheral retinal, optic nerve sheath and intrascleral) and retinoschisis, which again some people consider specific for child abuse. Unfortunately, the evidence for these assumptions has similar problems to that for perimacular retinal folds. An evidence based analyis of indexed medical publications on shaken baby syndrome from 1966-1998 uncovered a weak scientific evidence base.11 Selection bias, inappropriate controls, and the lack of precise criteria for case definition were identified as important problems with the data. Many studies committed a fallacy of assumption, selecting cases by the presence of the clinical findings that were sought as diagnostically valid. Unsystematic reviews and consensus statements often mingled opinion with facts and added no original supporting evidence.
Perimacular retinal folds are associated with increased neurological morbidity and mortality in infants and children with abusive head injuries.6 The reported incidence of perimacular retinal folds in shaken baby syndrome varies from 6% in a consecutive clinical case series to 50% in a sequential autopsy case series.5 12 Clinical and autopsy studies with appropriately matched controls are needed to determine the causal mechanism of perimacular retinal folds and their specificity for abusive head injury. Until good evidence is available, we urge caution in interpreting eye findings out of context.
Details of the included studies are on bmj.com
Editorials by Geddes and Plunkett and Harding et al
Contributors: PEL conceived the idea, collected the articles, and wrote the initial draft. All authors contributed to the review process, writing, and final editing of the paper. PEL is the guarantor.
Competing interests: None declared.
References
Gaynon MW, Koh K, Marmor MF, Frankel LR. Retinal folds in the shaken baby syndrome. Am J Ophthalmol 1988;106: 423-5.
Massicotte SJ, Folberg R, Torczynski E, Gilliland MG, Luckenbach MW. Vitreoretinal traction and perimacular retinal folds in the eyes of deliberately traumatized children. Ophthalmology 1991;98: 1124-7.
Elner SG, Elner VM, Arnall M Albert DM. Ocular and associated findings in suspected child abuse. A necropsy study. Arch Ophtlamol 1990;108: 1094-101.
Han DP, Wilkinson WS. Late ophthalmic manifestations of the shaken baby syndrome. J Paediatr Ophthalmol Strabismus 1990;27: 299-303.
Marshall DH, Brownstein S, Dorey MW, Addison DJ, Carpenter B. The spectrum of postmortem ocular findings in victims of shaken baby syndrome. Can J Ophthalmol 2001;36: 377-83.
Mills M. Funduscopic lesions associated with mortality in shaken baby syndrome. J Am Assoc Pediatr Ophthalmol Strabismus 1998;2: 67-71.
Munger CE, Peiffer RL, Bouldin TW, Kylstra JA, Thompson RL. Ocular and associated neuropathologic observations in suspected whiplash shaken infant syndrome: a retrospective study of 12 cases. Am J Forensic Med Pathol 1993;14: 193-200.
Buys YM, Levin AV, Enzenauer RW, Elder JE, Letourneau MA, Humphreys RP, et al. Retinal findings after head trauma in infants and young children. Ophthalmology 1992;99: 1718-23.
Gilliland MG, Luckenbach MW, Chenier TC. Systemic and ocular findings in 169 prospectively studied child deaths: retinal haemorrhages usually mean child abuse. Forensic Sci Int 1994;68: 117-32.
Greenwald MJ, Weiss A, Oesterle CS, Friendly DS. Traumatic retinoschisis in battered babies. Ophthalmology 1986;93: 618-25.
Donohoe M. Evidence-based medicine and shaken baby syndrome. Part 1: literature review, 1966-1998. Am J Forensic Med Pathol 2003;24: 239-42.
Kivlin JD, Simons KB, Lazoritz S, Ruttum MS. Shaken baby syndrome. Ophthalmology 2000;107: 1246-54.(P E Lantz, associate prof)
Correspondence to: P E Lantz plantz@wfubmc.edu
Introduction
A forensic autopsy showed no direct trauma to the orbits or eyes. There were prominent bilateral scalp contusions with soft tissue and intramuscular haemorrhage, symmetrical parietal skull fractures with coronal sutural diastasis, and a lacerated dura mater with extrusion of brain and blood. In addition to bilateral subdural and subarachnoid haemorrhages, a thin epidural haematoma partially covered the frontoparietal, calvarial lamina interna. The brain showed bilateral cortical contusions, severe cerebral oedema, and diffuse anoxic-ischemic injury. Postmortem ocular examination showed haemorrhages of the optic nerve sheaths with subdural haemorrhage greater than subarachnoid haemorrhage. Both eyes had extensive retinal haemorrhages with perimacular retinal folds (fig 2). Retinoschisis and peripapillary intrascleral haemorrhages were evident, and the retinal haemorrhages extended from the posterior pole to the ora serrata affecting the preretinal, intraretinal, and subretinal layers.
Fig 2 Transilluminated retinal image of right eye at autopsy showing circinate, elevated, perimacular retinal fold and extensive retinal haemorrhages
When investigators went to the house to recover the television before the family returned home, it was still on the carpeted floor. The 480 mm screen television with built in videocassette recorder weighed 19.5 kg. The homemade television stand measured 762 mm (height)x635 mm (width)x508 mm (depth) and had a bottom drawer that held videotapes. A greasy smudged area on the glass of the television corresponded with the impact site on the child's head.
A re-enactment in which a 11.4 kg weight (similar to the child's weight at autopsy of 11.8 kg) was placed on the partially opened drawer caused the television and television stand to readily topple forward. According to investigators, the family home was 7.8 km from the workplace and about 6 km from the local hospital. Based on the distance and estimated driving times plus workplace time clock records, the father was home with the children about 20 minutes when the incident happened. The day after the incident, while in foster care, the 3 year old sibling corroborated the father's account. Despite all this evidence, the paediatric ophthalmologist repeated that perimacular retinal folds coincident with retinal haemorrhages were considered specific for shaken baby syndrome secondary to retinal traction exerted by the oscillating vitreous.
Search for published evidence
We found 42 articles and book chapters discussing perimacular retinal folds in childhood abusive head trauma. Seventeen mentioned the presence of retinal folds in non-accidental head injury but did not comment on specificity or formative mechanism. A table on bmj.com gives details of the remaining articles. All but two of the articles are non-comparative clinical or autopsy case series, case reports, review articles, or book chapters.
The two studies that included controls both showed bias in selection of controls and contained no cases with perimacular retinal folds but discussed the postulated causal mechanism.8 9 In the prospective controlled study, the authors reported on 79 children younger than 3 years who had sustained head injuries.8 The manner of injury in one case was indeterminate. Three children, including one who died, had non-accidental head injury diagnosed, all of whom had retinal haemorrhages; 72 of the 75 children with non-abusive injuries were managed by observation alone. No perimacular retinal folds were observed; however, the presumed causative mechanism of traumatic retinoschisis and retinal folds was discussed.
The second controlled study was a prospective autopsy study that examined the presence and location of ocular findings in 169 childhood deaths.9 Ocular haemorrhages (retinal, peripheral retinal, optic nerve sheath and intrascleral) were more likely in craniocerebral trauma than in non-head injuries and natural diseases. Although case selection was purportedly random, the study contained a disproportionately high number of deaths from child abuse compared with natural and non-abusive causes. Case selection depended on the pathologist's willingness to participate in the study, and we were told by one of the authors that pathologists were more willing to participate when they believed that the deaths were abusive or suspicious (M Gilliland, personal communication, 2002). Perimacular retinal folds were not noted, but the authors concluded that acceleration-deceleration injury to the retina accounts for peripheral retinal haemorrhages and retinal folds.
Supporting evidence
Statements in the medical literature that perimacular retinal folds are diagnostic of shaken baby syndrome are not supported by objective scientific evidence. Non-comparative observational reports and unsystematic narrative review articles contain insufficient evidence to provide unbiased support for or against diagnostic specificity, and inferences about associations, causal or otherwise, cannot be determined. Clinical and autopsy evidence of ocular lesions must therefore be considered alongside other physical findings and a thorough investigation before concluding whether a head injury is caused by abuse. The child in our case had ocular haemorrhages (peripheral retinal, optic nerve sheath and intrascleral) and retinoschisis, which again some people consider specific for child abuse. Unfortunately, the evidence for these assumptions has similar problems to that for perimacular retinal folds. An evidence based analyis of indexed medical publications on shaken baby syndrome from 1966-1998 uncovered a weak scientific evidence base.11 Selection bias, inappropriate controls, and the lack of precise criteria for case definition were identified as important problems with the data. Many studies committed a fallacy of assumption, selecting cases by the presence of the clinical findings that were sought as diagnostically valid. Unsystematic reviews and consensus statements often mingled opinion with facts and added no original supporting evidence.
Perimacular retinal folds are associated with increased neurological morbidity and mortality in infants and children with abusive head injuries.6 The reported incidence of perimacular retinal folds in shaken baby syndrome varies from 6% in a consecutive clinical case series to 50% in a sequential autopsy case series.5 12 Clinical and autopsy studies with appropriately matched controls are needed to determine the causal mechanism of perimacular retinal folds and their specificity for abusive head injury. Until good evidence is available, we urge caution in interpreting eye findings out of context.
Details of the included studies are on bmj.com
Editorials by Geddes and Plunkett and Harding et al
Contributors: PEL conceived the idea, collected the articles, and wrote the initial draft. All authors contributed to the review process, writing, and final editing of the paper. PEL is the guarantor.
Competing interests: None declared.
References
Gaynon MW, Koh K, Marmor MF, Frankel LR. Retinal folds in the shaken baby syndrome. Am J Ophthalmol 1988;106: 423-5.
Massicotte SJ, Folberg R, Torczynski E, Gilliland MG, Luckenbach MW. Vitreoretinal traction and perimacular retinal folds in the eyes of deliberately traumatized children. Ophthalmology 1991;98: 1124-7.
Elner SG, Elner VM, Arnall M Albert DM. Ocular and associated findings in suspected child abuse. A necropsy study. Arch Ophtlamol 1990;108: 1094-101.
Han DP, Wilkinson WS. Late ophthalmic manifestations of the shaken baby syndrome. J Paediatr Ophthalmol Strabismus 1990;27: 299-303.
Marshall DH, Brownstein S, Dorey MW, Addison DJ, Carpenter B. The spectrum of postmortem ocular findings in victims of shaken baby syndrome. Can J Ophthalmol 2001;36: 377-83.
Mills M. Funduscopic lesions associated with mortality in shaken baby syndrome. J Am Assoc Pediatr Ophthalmol Strabismus 1998;2: 67-71.
Munger CE, Peiffer RL, Bouldin TW, Kylstra JA, Thompson RL. Ocular and associated neuropathologic observations in suspected whiplash shaken infant syndrome: a retrospective study of 12 cases. Am J Forensic Med Pathol 1993;14: 193-200.
Buys YM, Levin AV, Enzenauer RW, Elder JE, Letourneau MA, Humphreys RP, et al. Retinal findings after head trauma in infants and young children. Ophthalmology 1992;99: 1718-23.
Gilliland MG, Luckenbach MW, Chenier TC. Systemic and ocular findings in 169 prospectively studied child deaths: retinal haemorrhages usually mean child abuse. Forensic Sci Int 1994;68: 117-32.
Greenwald MJ, Weiss A, Oesterle CS, Friendly DS. Traumatic retinoschisis in battered babies. Ophthalmology 1986;93: 618-25.
Donohoe M. Evidence-based medicine and shaken baby syndrome. Part 1: literature review, 1966-1998. Am J Forensic Med Pathol 2003;24: 239-42.
Kivlin JD, Simons KB, Lazoritz S, Ruttum MS. Shaken baby syndrome. Ophthalmology 2000;107: 1246-54.(P E Lantz, associate prof)