Traumatic Retinoschisis Research Articles

Pediatric abusive head trauma: visual outcomes, evoked potentials, diffusion tensor imaging, and relationships to retinal hemorrhages

PurposeFunction and anatomy of the visual system were evaluated in children with abusive head trauma (AHT). The relationships between retinal hemorrhages at presentation were examined with outcome measures.MethodsRetrospective review of data in children with AHT for 1) visual acuity at last follow-up, 2) visual evoked potentials (VEP) after recovery, 3) diffusion metrics of white matter tracts and grey matter within the occipital lobe on diffusion tensor imaging (DTI), and 4) patterns of retinal hemorrhages at presentation. Visual acuity was converted into logarithm of minimum angle of resolution (logMAR) after correction for age. VEPs were also scored by objective signal-to-noise ratio (SNR).ResultsOf 202 AHT victims reviewed, 45 met inclusion criteria. Median logMAR was reduced to 0.8 (approximately 20/125 Snellen equivalent), with 27% having no measurable vision. Thirty-two percent of subjects had no detectable VEP signal. VEPs were significantly reduced in subjects initially presenting with traumatic retinoschisis or hemorrhages involving the macula (p < 0.01). DTI tract volumes were decreased in AHT subjects compared to controls (p < 0.001). DTI metrics were most affected in AHT victims showing macular abnormalities on follow-up ocular examination. However, DTI metrics were not correlated with visual acuity or VEPS. There was large inter-subject variability within each grouping.DiscussionMechanisms causing traumatic retinoschisis, or traumatic abnormalities of the macula, are associated with significant long-term visual pathway dysfunction. AHT associated abnormalities of the macula, and visual cortical pathways were more fully captured by VEPs than visual acuity or DTI metrics.

OPTICAL COHERENCE TOMOGRAPHY AND VISUAL OUTCOMES IN PEDIATRIC ABUSIVE HEAD TRAUMA.

Compare follow-up optical coherence tomography with visual function in children with abusive head trauma (shaken baby syndrome). Retrospective follow-up studies of three children who were victims of abusive head trauma within the first year of life. Optical coherence tomography showed disrupted retinal layering, thick detached internal limiting membrane, focal posterior vitreous separation, and multilayered tractional retinoschisis. Significant vision loss occurred in three of four eyes with a history of traumatic retinoschisis. Normal visual acuity and low-normal visual-evoked potentials were measured in a child with foveal distortion, reduced global nerve fiber layer thickness, detached internal limiting membrane, and history of vitreous hemorrhage. Significant abnormalities of retinal anatomy can be detected on optical coherence tomography years after abusive head trauma. Optical coherence tomographies and assessment of visual pathways help to elucidate causes of visual dysfunction in children with abusive head trauma.

Retinal and visual function in infants with non-accidental trauma and retinal hemorrhages.

To investigate retinal function and visual outcomes in infants with retinal hemorrhages due to non-accidental trauma (NAT). This is a retrospective review of full-field or multifocal electroretinogram (ERG) recordings, visual acuity in log minimum angle of resolution (logMAR), clinical status, and neuroimaging. Multifocal ERGs from the central 40° were compared to corresponding fundus imaging. Visual acuity was measured by Teller cards at follow-up. ERGs were compared to controls recorded under anesthesia. Sixteen children met inclusion criteria (14 recorded during the acute phase and 2 during long-term follow-up). During the acute phase, ERGs (n = 4 full field; n = 10 multifocal ERG) showed abnormal amplitude, latency, or both in at least one eye. Ten subjects had significantly reduced responses in both eyes, 3 of which had an ERG dominated by a negative waveform (absent b-wave or P1). The remaining six subjects had responses in one eye that were near normal (≥ 50% of controls). ERGs were sometimes abnormal in local areas without hemorrhage. ERGs could be preserved in local areas adjacent to traumatic retinoschisis. Two subjects with reduced visual acuity had belated ERGs: One had an abnormal macular ERG and the other had a normal macular ERG implying cortical visual impairment. At follow-up, 10 of 14 subjects had significant visual acuity loss (≥ 0.7 age-corrected logMAR); four subjects had mild vision loss (≤ 0.5 age-corrected logMAR). Visual acuity outcome was not reliably associated with the fundus appearance in the acute phase. All subjects with a negative ERG waveform had severe vision loss on follow-up. Retinal dysfunction was common during the acute phase of NAT. A near normal appearing fundus did not imply normal retinal function, and ERG abnormality did not always predict a poor visual acuity outcome. However, a negative ERG waveform was associated severe visual acuity loss. Potential artifacts of retinal hemorrhages and anesthesia could not fully account for multifocal ERG abnormalities. Retinal function can be preserved in areas adjacent to traumatic retinoschisis.

Traumatic macular retinoschisis in infants and children

To provide detailed description of pediatric traumatic retinoschisis. The medical records of children with either abusive head trauma and traumatic macular retinoschisis seen at a single center from 1993 to 2006 were reviewed retrospectively. Clinical details were extracted from the record and photographic documentation. Evaluation regarding abuse excluded ophthalmology findings to avoid circular reasoning. Of 134 patients with suspected abusive head trauma, 31 had retinoschisis. Mean age was 9months. Of the 31, 22 (71%) offered a history of injury, and 9 (29%) were found unresponsive without history of injury; 6 were reportedly shaken. All patients had seizures, vomiting, and/or altered responsiveness. All had subdural hemorrhage, with cerebral edema in 17 (55%). In 10 (32%), there were findings of blunt force head injuries; in 4 of these there was no impact history. Retinal hemorrhages were present in all cases. Agreement between sidedness of retinoschisis and subdural hemorrhage was poor. Eleven patients had retinal folds, 3 of which had a hemorrhagic edge to the schisis. Nine patients had extracranial manifestations of abuse. Multidisciplinary team adjudications were as follows: of the 31 cases, 18 were suspicious for abuse, 11 were indeterminate, and 2 were possibly accounted for by accidental severe crush injury. Three children died, and 11 suffered neurological sequelae. Traumatic retinoschisis in children is highly associated with subdural hemorrhage, neurologic symptoms, and poor outcomes. Even with a conservative approach toopinion formulation, traumatic retinoschisis was associated with likely abuse.

A Systematic Review of the Diagnostic Accuracy of Ocular Signs in Pediatric Abusive Head Trauma

To review systematically the diagnostic accuracy of various ocular signs for pediatric abusive head trauma (AHT). Intraocular hemorrhages (IOH), perimacular retinal folds, traumatic retinoschisis and optic nerve sheath hemorrhages have been reported as cardinal signs of AHT. The evidence base supporting the accuracy of this interpretation, however, has not been systematically reviewed. A systematic keyword search of MEDLINE, EMBASE, and Evidence-Based Medicine Reviews was conducted for original studies reporting ocular findings in AHT. Articles were graded using a checklist for systematic reviews of diagnostic accuracy. The initial search yielded 971 articles, of which 55 relevant studies were graded, and 20 studies met inclusion criteria and were included in the review. The overall sensitivity of IOH for AHT was 75% and their specificity was 94%. Intraretinal hemorrhage at the posterior pole was the most common finding, although extensive, bilateral, and multilayered IOH were the most specific for AHT. Optic nerve sheath hemorrhages had a sensitivity and specificity for AHT of 72% and 71%, respectively. Traumatic retinoschisis and perimacular retinal folds were reported in 8% and 14% of AHT, respectively, but were not reported in other conditions. Prospective, consecutive studies confirm that IOH in infants-particularly bilateral, extensive, and multilayered-are highly specific for AHT. Optic nerve sheath hemorrhages are significantly more common in AHT than in other conditions, in autopsy studies. Traumatic retinoschisis and perimacular folds are present in a minority of AHT, but rarely seen in other conditions.

Subretinal hemorrhage masquerading as a hemorrhagic choroidal detachment in a case of nonaccidental trauma

Retinal hemorrhages are the most commonly reported ocular findings in nonaccidental trauma in children. Other reported ocular findings include perimacular folds, traumatic retinoschisis, choroidal hemorrhages, and retinal detachments. We report the clinical and pathologic findings in a case of a 10-month-old boy who sustained nonaccidental trauma and whose clinical presentation was characteristic of a hemorrhagic choroidal detachment. Postmortem examination revealed a large subretinal hemorrhage, with no evidence of choroidal hemorrhage.

Update on shaken baby syndrome: ophthalmology

Shaken baby syndrome is a common problem with a high morbidity and mortality. Ophthalmologists help manage this condition and therefore must keep abreast of current advances. Clinical updates include the discovery that retinal folds and traumatic retinoschisis can very rarely occur after crush head injury, but remain specific for shaken baby syndrome in other scenarios. Pathology updates include new studies on orbital histology and woodpecker anatomy that suggest the retinal and optic nerve hemorrhages in shaken baby syndrome are caused by shaking itself rather than secondary to intracranial pathology. Regarding this shaking injury, some primary prevention strategies have proven surprisingly effective. In the near future, serum biomarkers may be used as a screening tool for inflicted neurotrauma. Animal models such as the neonatal pig and computer models using finite element analysis are promising experimental techniques for studying shaken baby syndrome. Finally, child abuse recently became an accredited subspecialty of pediatrics, which will lead to further advances in patient care, education, research and prevention. Ophthalmologists play a key role in the diagnosis of shaken baby syndrome. In addition, they are in a unique position to study ophthalmic aspects of the syndrome, which in turn furthers the overall understanding of this devastating condition.

Ocular manifestations of crush head injury in children

To review the ocular manifestations of crush head injuries in children. Retrospective clinical and pathological reviews. Group 1: A total of 16 children admitted with crush head injuries from television tip over. Group 2: Nine autopsy findings in crush head injury. Group 1: A total of 11 children had fundus examination: three by neurosurgeons, eight by ophthalmologists. Scattered posterior pole preretinal and blot haemorrhages extending to mid equator region observed in one child. No evidence of traumatic retinoschisis or retinal folds in any patient. Group 2: All with multiple skull fractures and six with subdural haemorrhage. Posterior pole retinal haemorrhages in four children, extending to the ora serrata in one after motor vehicle accident. No child had retinal folds. Subinternal limiting membrane haemorrhages in three children. Optic nerve sheath haemorrhage in three children. Intraretinal and preretinal haemorrhages, predominantly in the posterior pole, can occur in crush injury to the paediatric head. Haemorrhage under the internal limiting membrane or extending to the ora serrata were only seen in situations where crush injury was part of a fatal trauma scenario related to motor vehicles. Retinal folds and the typical macular retinoschisis associated with abusive head injury were not observed.

Ocular and Associated Systemic Findings in Suspected Child Abuse

We reviewed complete ocular and systemic necropsy findings of 10 consecutive children who died of suspected child abuse. All 10 children had evidence at necropsy of blunt head trauma, although external signs of blunt trauma occasionally were covert. Ocular injuries were observed in 7 of the 10 cases and when present always included retinal, vitreous, and subdural optic nerve hemorrhages. In 5 cases, intrascleral hemorrhage from the circle of Zinn occurred at the sclera-optic nerve junction. In 4 cases, traumatic retinoschisis or tractional retinal folds were present. Anterior segment findings were uniformly consistent with blunt trauma. Hemosiderin, indicating old hemorrhage, was present in 3 cases. Intracranial hemorrhage, present in all cases with abnormal ocular findings, was always accompanied by signs of direct head trauma, such as subgaleal hemorrhage, skull fracture, cerebral contusion, or external contusions, which are sometimes subtle or hidden beneath the hair.

Autopsy findings in the eyes of fourteen fatally abused children

The eyes of fourteen fatally abused children and sixteen control cases were examined histopathologically. Ten of the abused children showed intraocular change. The most common ocular changes were subdural hemorrhage of the optic nerve and retinal hemorrhage which involved all the layers of the retina, but most commonly the nerve fiber layer, ganglion cell layer and inner nuclear layer. The presence of blood cavities within the retina partially supported the hypothesis of traumatic retinoschisis. The control cases of non-abused children rarely showed intraocular hemorrhage.

Traumatic Retinoschisis in Battered Babies

Five infants who were victims of physical abuse had extensive bilateral retinal hemorrhages on initial evaluation and subsequently developed signs of permanent retinal damage. None showed external evidence of trauma to the eyes. Vitreous hemorrhage developed after a delay of several days or more in three cases that were followed closely from the time of the traumatic incident. In several eyes, apparent intraretinal blood-filled cavities were seen acutely in the macular region and elsewhere. Late scarring of the macula typically had a cystic or crater-like configuration. Electroretinography showed loss or reduction of the positive 13-wave with preservation of the negative A-wave in every case. We propose that splitting of the retina resulting from the direct mechanical effects of violent shaking was responsible for all of these findings.