INTRODUCTION — The ophthalmologic aspects of abusive head trauma (AHT) will be reviewed here. The pathogenesis of retinal hemorrhages in AHT, other aspects of AHT, and the management of suspected child abuse are discussed separately:
DEFINITION — AHT in young children refers to repeated acceleration-deceleration abusive injury with or without blunt head impact [1-5]. Characteristic clinical features include retinal hemorrhages (often but not always bilateral, multilayered, and extensive), subdural hematoma, and/or occult fractures (particularly of the ribs and long bone metaphyses). Multiple episodes of trauma may occur before the abuse is detected [6-9]. Early recognition can be lifesaving. (See "Child abuse: Epidemiology, mechanisms, and types of abusive head trauma in infants and children", section on 'Definitions'.)
EPIDEMIOLOGY — Child abuse is an important cause of death in children. Among child abuse fatalities, head injury is the leading cause of death in infancy. Mortality rates in series of cases of AHT range from approximately 15 to 25 percent. The epidemiology and risk factors for physical abuse and AHT are discussed separately. (See "Physical child abuse: Recognition", section on 'Epidemiology' and "Child abuse: Epidemiology, mechanisms, and types of abusive head trauma in infants and children".)
Children with AHT commonly have abnormal eye findings such as hemorrhages of the retina, vitreous, or optic nerve sheath (the latter visible only on autopsy) and less commonly, perimacular retinal folding or retinoschisis (splitting of the retinal layers) . For example, in a systematic review of 20 observational studies with results of eye examination in 973 victims of abuse, intraocular hemorrhage (retinal or vitreous hemorrhage) was seen in approximately 85 percent of children with AHT. Thus, a careful funduscopic examination with indirect ophthalmoscopy by an ophthalmologist is indicated in all children in whom AHT is suspected. (See 'Ophthalmology consultation' below.)
EVALUATION — The diagnosis of AHT often requires a high index of suspicion. An accurate history is rarely provided at the time of presentation. The signs and symptoms can vary from mild and nonspecific to severe and immediately life-threatening. AHT should be suspected in infants who present with unexplained vomiting, poor feeding, lethargy, irritability, seizures, impaired consciousness, or apnea. (See "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children", section on 'Clinical features'.)
Nonophthalmologic aspects of the physical examination of the infant with inflicted head injury are discussed separately and often involve abnormal findings on examination of the skin, chest, and abdomen. (See "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children", section on 'Physical examination'.)
Initial eye examination — The ophthalmologic examination is essential when evaluating young children whose injuries are suspicious for child abuse . When present, retinal hemorrhages are often unaccompanied by external eye findings. However, poorly reactive pupils or a gaze preference may be noted. Periorbital ecchymoses, subconjunctival hemorrhage (especially in infants younger than 12 months and outside of the newborn period [11,12]), hyphema, and orbital fractures may indicate direct trauma to the face or orbit, whereas funduscopic findings such as retinal hemorrhages may indicate occult intracranial injury. Late signs of severe direct injury on funduscopy may include optic atrophy, retinal detachment, retinal scars, and choroidal rupture [13,14]. (See "Orbital fractures" and "Traumatic hyphema: Clinical features and diagnosis".)
The ability to detect retinal hemorrhages and other abnormalities of the fundus depends upon the technique that is used to examine the retina:
●Dilated examination – Pupillary constriction, which may result from the administration of barbiturates for sedation or narcotics for pain control, can limit the yield of the retinal examination, even with an indirect ophthalmoscope. Pharmacologic dilation (table 1) or pathologic dilation of the pupils facilitates detection of retinal hemorrhages and other abnormalities, as well as retinal photography. These benefits of pharmacologic dilation usually outweigh the loss of ability to monitor pupillary reaction for several hours. Dilating one eye at a time may be an alternative in situations where the ability to monitor pupillary reaction is necessary (eg, patients with signs of severe increased intracranial pressure) . Even when the pupils cannot be pharmacologically dilated, an initial undilated examination by an ophthalmologist is worthwhile and should be obtained as soon as possible after presentation; it may provide a partial view of the fundus and identification of retinal hemorrhage patterns that may change quickly, due to the rapid clearance of intraretinal hemorrhages .
●Direct ophthalmoscopy – The handheld direct ophthalmoscope provides a magnified but therefore limited view of the fundus: little more than 1 disc diameter can be visualized (picture 1). Extensive retinal hemorrhages in the posterior pole of the fundus in children with severe neurologic injury (and fixed dilated pupils) may be identified, but peripheral findings, details of the pattern of retinal hemorrhage, and more subtle findings in children with less severe retinal hemorrhages may be easily missed .
A specific type of handheld direct ophthalmoscope (the Pan Optic) increases the field of vision to 2 to 5 disc diameters and may increase the ability to detect retinal abnormalities with direct ophthalmoscopy; however, the view is two-dimensional and not as wide as with an indirect ophthalmoscope. A formal study of the use of the Pan Optic scope for evaluating retinal findings in this context has not been reported. In addition, cell phone attachments enabling viewing and photography of the retina have become commercially available; these devices offer a similar view to the Pan Optic scope.
●Indirect ophthalmoscopy – The indirect ophthalmoscope, most often a head-mounted unit used with a hand-held lens, provides a wide-angle binocular (three-dimensional) view that simultaneously includes nearly all of the areas in the posterior pole of the fundus (picture 1). By directing the view toward different parts of the retina, much or all of the periphery can be seen. In an uncooperative child who is moving his or her eyes, the fundus image may flash past the viewer. The larger field of view provided by the indirect ophthalmoscope increases the likelihood that important features such as the optic nerve head, hemorrhages, and the pattern of retinal findings will come into view.
●Postmortem examination – Postmortem examination of the eyes can reveal signs of possible child abuse and should be performed in cases of suspected abuse or unexplained death in infants and young children . Postmortem ocular examination supplements pre-mortem findings and should be performed even if the eyes were examined clinically before death. However, postmortem ophthalmoscopy is limited by corneal opacification.
Pathologic examination of the eyes and orbital tissues, which necessitates removal at autopsy, is also recommended in these patients to assure that all abnormalities are identified, including optic nerve sheath hemorrhage [18,19]. Pathological examination is best performed by an ocular pathologist.
Retinal findings, such as retinal hemorrhages, may be detected by any physician who is familiar with such hemorrhages and has experience with funduscopy [15,20-22]. Clinicians other than ophthalmologists may also dilate the pupils to improve their view (table 1).
However, detection of intraocular abnormalities is optimized by ophthalmology consultation, which permits better evaluation of the entire retina with an indirect ophthalmoscope and dilation of the child's pupils. For example, in a retrospective series of 123 children admitted for subdural hematomas caused by abuse, physicians other than ophthalmologists failed to detect retinal hemorrhages in 29 percent of patients . In another series, no attempt was made by clinicians other than ophthalmologists to visualize the retina in 36 percent of patients with AHT; among the children who were examined, hemorrhages were missed by non-ophthalmologists in 13 percent of the cases . Furthermore, examination by a non-ophthalmologist may not sufficiently identify the patterns and details of retinal hemorrhages, which may have diagnostic implications. Thus, consultation with an ophthalmologist should occur for all children under five years of age with suspected AHT regardless of initial findings on funduscopic examination by a non-ophthalmologist.
Ophthalmology consultation — Consultation with an ophthalmologist for a complete, dilated indirect ophthalmoscopic examination is recommended in all children younger than five years of age in whom AHT is suspected (eg, intracranial hemorrhage, increased intracranial pressure, or unexplained coma) . The American Academy of Pediatrics recommends that this examination should take place within 24 to 72 hours. We suggest that, whenever possible, examinations should be conducted within 24 to 36 hours because there is potential for some intraretinal hemorrhages to resolve and to be missed by 48 hours [16,17]. The detection and initial description of retinal hemorrhages may be a crucial step in the identification of abuse and appropriate direction of additional evaluation . (See "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)
Consultation with an ophthalmologist is recommended whether or not retinal hemorrhages are detected by other clinicians. The ophthalmologist may be able to detect additional hemorrhages and characterize them in a fashion that permits better assessment of the likelihood that child abuse occurred [15,17]. The ophthalmologist also can provide a more knowledgeable opinion regarding the etiology of the retinal findings and better photographic documentation (if available) of the hemorrhages for legal purposes  (see 'Differential diagnosis' below). Consultation should not be delayed if pharmacologic pupillary dilation is not yet permitted, as an initial undilated examination still may allow a helpful fundus view.
Occasionally, intracranial abnormalities, including subdural hemorrhage, subarachnoid hemorrhage, and cerebral edema, may be missed on initial computed tomography (CT) scans of the head or may not be present [25-29]. In such cases, the detection of retinal hemorrhages is a strong indicator that AHT may still have occurred and prompt additional neuroimaging such as magnetic resonance imaging. For patients with no findings of intracranial hemorrhage or increased intracranial pressure on CT, eye examination by an ophthalmologist may still be warranted if suspicion for AHT remains high (eg, persistent altered mental status and/or a clear history of shaking or abusive head injury). Otherwise, the likelihood that an eye examination will demonstrate retinal hemorrhages is low in children with normal neuroimaging and no evidence of head trauma. For example, among 78 infants with isolated long-bone fractures and dilated fundus examinations, none had retinal hemorrhages . Similarly, in a study of 190 children evaluated for AHT, all 15 children with retinal hemorrhages had positive neuroimaging, and none of 85 children with negative neuroimaging had retinal hemorrhage . In larger studies, a small proportion of children (0.6 to 3.2 percent) with normal neuroimaging have been found to have retinal hemorrhage, but severe retinal findings considered highly specific for AHT were seen in <1 percent of all patients [32,33].
OPHTHALMOLOGIC FINDINGS — The grossly visible ophthalmoscopic findings of AHT may include retinal hemorrhages, retinal folds, retinoschisis (ie, splitting of the retina), vitreous hemorrhage, papilledema, and/or optic atrophy. Additional vitreoretinal findings, such as vitreoretinal traction, vitreous separation, and subclinical retinoschisis, may also be seen with specialized retinal imaging called optical coherence tomography [34-36].
Retinal hemorrhages — Retinal hemorrhages have been reported in 47 to 100 percent of cases of AHT, with a frequency of approximately 85 percent in most case series [10,15,21,24,37-45]. Retinal hemorrhage is most commonly found on autopsy and is least common among children with normal neurologic outcome.
The finding of retinal hemorrhage on ophthalmologic examination significantly increases suspicion for trauma and inflicted injury, but does not necessarily confirm the diagnosis nor does the absence of retinal hemorrhage exclude the diagnosis [10,20,21,46-49]. (See 'Differential diagnosis' below and "Child abuse: Epidemiology, mechanisms, and types of abusive head trauma in infants and children" and "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)
●Description and severity – When describing retinal hemorrhages, it is important to describe the number of hemorrhages, the pattern of distribution, and the types of hemorrhage (figure 1 and figure 2 and figure 3 and figure 4) [15,44]. The types and severity of hemorrhages have diagnostic and prognostic implications [44,45]. The severity of the retinal abnormalities is also correlated with the severity of the intracranial abnormalities .
Retinal hemorrhages in AHT usually are bilateral, although asymmetry and unilaterality are well recognized [15,21,39,42,43,46,50]. In a series of 75 children with AHT, there was no correlation between the laterality of intracranial findings (eg, extra-axial hemorrhage, edema) and the laterality of retinal findings , although a correlation was detected in a smaller series (14 patients) .
In the majority of cases of AHT (approximately two-thirds), the hemorrhages are too numerous to count and extend anteriorly to the ora serrata (figure 5) . However, retinal hemorrhages associated with AHT can be few in number, exclusively intraretinal, and confined to the posterior pole.
In approximately two-thirds of cases of AHT, retinal hemorrhage occurs in multiple layers (eg, preretinal, intraretinal, and/or subretinal) (figure 1) . Less commonly, patients also have vitreous hemorrhage. Dense or extensive preretinal or vitreous hemorrhage may obscure underlying retinal hemorrhage.
Some patients have white-centered hemorrhages that may be caused by fibrin plugs, ischemia, artifactual reflection, or central resolution (picture 2) [21,52]. These white-centered hemorrhages should not be described as Roth spots because doing so falsely implies an infectious origin (ie, endocarditis), which may confuse the child's evaluation. White-centered retinal hemorrhages are a non-specific finding and not diagnostic of septic emboli or any specific etiology.
There is no single recognized standard grading system of retinal hemorrhages . The best description of the hemorrhages is to specify the laterality, number, type(s), and locations of retinal hemorrhages, as well as the presence or absence of macular retinoschisis with or without hemorrhagic macular cyst, retinal folds, and presence of other ocular findings.
Retinal hemorrhages in AHT occur along a spectrum of severity, ranging from mild to severe:
•Mild – Few in number (less than about 10) intraretinal hemorrhages primarily in the posterior pole, usually in a peripapillary (around the optic nerve) and perivascular pattern
•Severe – Too numerous to count, diffusely distributed multi-layered retinal hemorrhages (intraretinal, preretinal, and/or subretinal hemorrhages) in the posterior pole and retinal periphery, with hemorrhages present in the far retinal periphery near the ora serrata. The midperiphery of the retina may be spared. The central macula may be relatively spared as well. There may be macular findings of retinoschisis, hemorrhagic cyst, or circumlinear fold.
The severity of retinal hemorrhages generally correlates with the severity of trauma. Consequently, as the severity of retinal hemorrhages increases from mild towards severe, the suspicion for AHT, in the absence of a clear history of severe non-abusive head trauma, also increases [10,45]. Specific findings that indicate increased severity include:
-Greater number of hemorrhages
-The presence of preretinal or subretinal hemorrhage in addition to intraretinal hemorrhage
-Extension of hemorrhages anteriorly beyond the posterior pole and far into the retinal periphery
-Hemorrhages in the posterior pole, near periphery, and far periphery, with relative sparing of the mid periphery
-Presence of macular retinoschisis and/or circum-macular retinal folds
In order to avoid misclassifying the severity of retinal hemorrhages, funduscopic examination by an ophthalmologist should be conducted within 24 to 36 hours in children with a high suspicion for abusive head trauma.
●Clinical course – Although retinal hemorrhages cannot be precisely dated to determine the timing of the traumatic event, there is useful information related to the pattern of retinal hemorrhages [15,16]:
•Intraretinal hemorrhages resolve quickly (starting within 24 to 48 hours, with most clearing in the first one or two weeks, and isolated hemorrhages persisting slightly longer); thus the presence of too numerous to count intraretinal hemorrhages suggests an injury has occurred acutely, typically in the past few days [16,53].
•Preretinal hemorrhage in AHT is always accompanied by intraretinal hemorrhage and takes longer to clear (on the order of weeks to months). Thus, the presence of preretinal hemorrhage with few or no intraretinal hemorrhages suggests a more chronic injury . The time to resolution also depends, to some extent, upon the hemorrhage size. Generally, blood in a schisis cavity resolves more slowly and vitreous hemorrhage is the slowest to resolve
•Retinal scars and optic atrophy take time to develop and suggest a distant previous injury (one or more months or longer).
Retinal folds and retinoschisis — Circular retinal folds centered on the macula (figure 5) are indicative of severe trauma; they are a distinctive but not pathognomonic finding in AHT (picture 3) [1,21,54-57]. Retinal folds also have been reported after fatal motor vehicle crashes, crush head injuries, and a 30-foot fall with head injury [55,56,58]. (See 'Diagnostic implications of eye findings' below.)
Both peripheral and macular traumatic retinoschisis (ie, splitting of the retina) may occur in AHT (figure 1). Macular retinoschisis can be a subtle finding. If there is hemorrhage in the split layers, the term "hemorrhagic macular cyst" is used; this finding appears as a dense macular hemorrhage within the schisis cavity, sometimes with a meniscal interface between red blood cells and serum.
In many cases of retinoschisis, only the internal limiting membrane and/or nerve fiber layer (figure 6) is pulled away by the vitreous because the vitreous is firmly attached in infants. This finding has been demonstrated by electroretinogram, ultrasonography, and optical coherence tomography. However, deeper schisis and even focal macular detachment can occur . Histologic findings may include attachment of the vitreous at the edges of the lesion, which is visible on optical coherence tomography in live children as well, and depigmentation of the retinal pigmented epithelium underlying the folds .
The schisis cavity may be surrounded by a hypopigmented or hemorrhagic arc or elevated retinal fold (perimacular or paramacular circular folds) (picture 3). The retinal fold may be continuous (360 degrees), discontinuous, or just an arc. It may encompass only the macula or surround the entire posterior pole, including the optic nerve.
It is important that the circumlinear edge abnormalities be recognized clinically, since these findings may distinguish retinoschisis from preretinal (subhyaloid) hemorrhage, particularly when only the internal limiting membrane is elevated. Blood may break through the schisis wall into the preretinal space or vitreous, obscuring the edge changes of a cavity, which may become apparent only after the blood resolves.
The presence of retinal folds typically implies severe injury that often results in death or neurologic sequelae, although some patients have had good visual recovery [1,21,54,57]. (See 'Outcome' below.)
Microanatomic retinal findings — Optical coherence tomography (OCT) is a retinal imaging modality that can be helpful in children with suspected AHT. It provides ultra-high-resolution cross-sectional imaging of the microanatomical structure of the retina, vitreous, and optic nerve head, revealing findings not visible on ophthalmoscopy. Findings in infants with abusive head trauma using OCT have included vitreoretinal traction, vitreous separation from the retina, and subclinical retinoschisis [34-36]. OCT, while safe, painless, and without exposure to ionizing radiation, does require a cooperative or sedated patient and is generally available at pediatric or tertiary ophthalmology referral centers.
Vitreous hemorrhage — Vitreous hemorrhage can obscure fundus detail (figure 1). It can be present at the first examination shortly after injury or occur days later from rupture of the internal limiting membrane over a hemorrhagic cyst (picture 4) .
Papilledema — Papilledema, or optic disc swelling due to raised intracranial pressure, is only present in about 10 percent of AHT cases and usually takes 12 to 24 hours to develop in patients with increased intracranial pressure following head injury. Thus, papilledema noted at the initial presentation may suggest delay in seeking care after AHT has occurred and is an ominous finding that is associated with high mortality [15,21,40].
Optic nerve sheath hemorrhage — Optic nerve sheath hemorrhage is commonly present in patients with AHT and is not always accompanied by retinal hemorrhage [8,21,60,61]. It is also seen in patients with non-abusive head injury . Optic nerve sheath hemorrhage tends to be most prominent anteriorly and may not extend the length of the optic nerve [60,61]. Optic nerve sheath hemorrhages frequently involve multiple layers around the optic nerve but often show a preponderance of hemorrhage in the subdural space [60,62,63]. The extent of the optic nerve sheath hemorrhage does not necessarily correlate with the severity of the intraocular findings or the intracranial hemorrhage [60,61].
Other orbital trauma — Other evidence of acceleration-deceleration-induced orbital trauma found at autopsy includes optic nerve intradural hemorrhage and hemorrhage in the orbital fat, cranial nerve sheaths, or extraocular muscles .
DIAGNOSTIC IMPLICATIONS OF EYE FINDINGS — The diagnosis of AHT is made on the basis of the entire clinical scenario, which typically includes injuries suspicious for abuse and a history that is inconsistent with the severity and/or type of injury [4,37,64]. Thus, eye findings must be placed within the context of all clinical features. (See "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children", section on 'Diagnosis'.)
A systematic review of 20 observational studies comprising ocular findings in 1948 children, including 242 children with non-abusive head injury and 973 children with AHT as determined by perpetrator confession, third-party witness, legal decision, autopsy findings, or multidisciplinary assessment found the following :
●Intraocular hemorrhage (IOH) was present in 44 to 100 percent of children with AHT but only 0 to 10 percent of children with non-abusive head injury. The mean sensitivity of IOH for child abuse was 75 percent and the specificity, based upon the four prospective case-control studies in the review, was 94 percent (range 90 to 100 percent). The specificity for AHT was highest for IOH that was bilateral, preretinal, peripheral, and moderate to severe, ie, extending beyond the posterior pole and involving multiple retinal layers or the vitreous. All IOH in children with non-abusive head injury in the reviewed studies was described as mild, such as confined to the posterior retinal pole and involving a single retinal layer. However, in subsequent reports not included in the systematic review, more widespread and multilayered retinal hemorrhages have rarely occurred from severe, non-abusive injuries (especially severe crush injuries) and non-traumatic critical illness such as sepsis or leukemia with coagulopathy [55,65,66]. Therefore, it is important to recognize the overlap in possible severity of hemorrhages between abusive and accidental head trauma.
●Perimacular retinal folds and retinoschisis were seen in a small proportion of children with AHT and were more commonly seen on autopsy. When present, these findings were highly specific for AHT and were not described in any children with non-abusive head injury or other diseases in this review. However, these findings have rarely been reported after unambiguous severe non-abusive head injury including crush injuries , fatal motor vehicle collisions with severe rotational and deceleration injury , and one fatal 11-meter fall injury, which also produced multiple skull fractures . Thus, when macular retinoschisis or perimacular retinal folds are present in a child without a history of major trauma, the likelihood of AHT is very high.
●Optic nerve sheath hemorrhage was described in 50 percent of 311 children with AHT versus 29 percent of 42 children with non-abusive head injury. Thus, this finding is less specific for the diagnosis of AHT than IOH, perimacular retinal folds, or macular retinoschisis. However, when optic nerve sheath hemorrhage is present without a history of major trauma, AHT is the most likely diagnosis.
In patients with concerning eye findings, additional injuries, including fractures in various stages of healing or without a trauma history on skeletal survey, intraabdominal injury (eg, liver laceration, duodenal hematoma or perforation) and intracranial hemorrhage, especially subdural hematoma, increases the certainty of the diagnosis . Thus, when child abuse is suspected and concerning eye findings are present, additional evaluation (eg, neuroimaging, skeletal survey depending upon the child's age, liver enzyme studies, CT of the abdomen with intravenous contrast) is indicated . (See "Physical child abuse: Diagnostic evaluation and management" and "Orthopedic aspects of child abuse" and "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)
DIFFERENTIAL DIAGNOSIS — A variety of conditions may result in retinal hemorrhages in infants and young children (table 2) [14,15,42-44]. The most common cause of retinal hemorrhage in this age range is trauma, birth-trauma in the first month of life, and, beyond the first month of life, AHT. Even mild retinal hemorrhages are significant in this regard, and it is incorrect to characterize mild hemorrhages as not being characteristic of or consistent with AHT. Increasing retinal hemorrhage severity is associated with increasing likelihood of an abusive injury [10,45,67]. However, many other systemic and ocular conditions may be associated with retinal hemorrhages. Most of these entities have characteristic history, physical examination, and/or laboratory features that distinguish them from AHT; the pattern of retinal hemorrhages is mild to moderate in comparison to the more severe patterns of retinal hemorrhage that can be seen in AHT; and retinal lesions not typically associated with head trauma, such as white patches, yellow exudates, or tan lesions, may be present. As an example, in a prospective case series, 15 percent of critically ill children who did not have AHT had retinal hemorrhages on retinal photography or dilated examination by a pediatric ophthalmologist . Most of these children had fewer than 5 single-layered retinal hemorrhages that were considered mild. The diagnoses in six children with more than 20 multi-layered retinal hemorrhages that were similar to some of the more moderately severe patterns seen in AHT included fatal non-abusive head trauma, severe coagulopathy, and/or sepsis associated with myeloid leukemia.
Having these conditions does not exclude the possibility of child abuse, which must be considered when the retinal hemorrhages have features associated with AHT (ie, numerous, multilayered, and extending to the retinal periphery) and other clinical findings of physical abuse (eg, subdural hematoma, liver laceration, fractures, skin bruising) [14,15,42,47,48,68,69]. (See "Physical child abuse: Recognition", section on 'Red flag physical findings' and "Orthopedic aspects of child abuse" and "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)
In observational studies, neither severe coughing, forceful vomiting (as in hypertrophic pyloric stenosis), nor seizures have been associated with retinal hemorrhages [42,70-73]. Vaccinations do not cause retinal hemorrhages .
Although in some cases neurosurgical intervention for intracranial injury is required prior to ophthalmology consultation, neurosurgery is unlikely itself to produce retinal hemorrhage and is not considered a significant confounding factor in the interpretation of retinal hemorrhage findings in those children .
Perinatal retinal hemorrhages — The incidence of perinatal retinal hemorrhages ranges from 20 to 30 percent among infants examined in the first 24 hours of life, and 10 to 15 percent among infants examined in the first 72 hours of life [15,76,77]. These hemorrhages are possibly related to peripartum prostaglandin release and obstetrical and perinatal hemodynamic changes [15,76,78].
Intraocular hemorrhages can occur with any type of delivery, including cesarean section, but they are particularly common in spontaneous vaginal or vacuum assisted deliveries . Perinatal retinal hemorrhages may sometimes be numerous and extend to the periphery, resembling those seen in AHT. Preretinal hemorrhage also may occur in neonates, but subretinal hemorrhage is rare . Retinoschisis and retinal folds have not been reported with perinatal retinal hemorrhages .
The rate of disappearance of perinatal retinal hemorrhage depends upon the size and type of hemorrhage: intraretinal hemorrhages, which may include both superficial flame-shaped hemorrhages (figure 2) and deeper dot/blot hemorrhages (figure 3) begin to resolve shortly after birth, with most disappearing by two to three weeks, similar to AHT-related retinal hemorrhages [76,79]. Note that while an isolated intraretinal hemorrhage may persist past three weeks of age, numerous intraretinal hemorrhages do not, and this finding after age three weeks cannot be ascribed to birth. Perinatal intra- or sub-foveal, preretinal (figure 4), and vitreous hemorrhage commonly lasts longer.
Non-inflicted head injury — Retinal hemorrhages have been reported in association with severe non-abusive head injury (as may occur in a motor vehicle crash, crush injury, or fall from great height) [46,48,68,80-83]. However, clinical studies suggest that the rate of retinal hemorrhage in accidental head injuries is low (usually <3 percent). In most series, the incidence of retinal hemorrhages after such injuries is zero, particularly when the mechanism of trauma is a short fall [15,46,48,69,81-86]. Falls from sofas, out of the arms of caregivers, and during play rarely, if ever, cause retinal hemorrhages unless the mechanism of injury is severe (eg, falling off of a moving swing, falling through a stair rail at the top of a flight of stairs) [15,80,87].
When retinal hemorrhages occur in association with non-abusive head injury, the history of trauma usually is clear and consistent, the mechanism of injury severe, other bodily injuries reflect the forces involved, and the infants have no additional unexplained injuries. The retinal hemorrhages commonly are confined to the posterior pole, few in number, and rarely subretinal . However, more severe accidental head trauma may result in more severe retinal hemorrhage, as well as retinoschisis and retinal folds have been rarely reported in cases of extreme unambiguous accidental trauma, such as a fatal car accident [55,56,58,62].
Hematologic conditions — Coagulopathies and other bleeding disorders, including thrombocytopenia, severe anemia, leukemia, factor deficiencies, and vitamin K deficiency, should be considered in the differential diagnosis of intraocular hemorrhage in infants. Bleeding disorders usually are suggested by history, physical examination, and laboratory testing [14,15,43].
Patients with severe bleeding disorders can have intracranial bleeding, but few patients have retinal hemorrhages. In contrast to the retinal hemorrhages associated with AHT, the retinal hemorrhages associated with hematologic abnormalities usually are few in number and confined to the posterior pole [15,88,89]. Leukemia is a possible exception, but children with leukemia generally have additional clinical or laboratory abnormalities to readily suggest the diagnosis. Retinal examination often reveals leukemic infiltrates (picture 5) in addition to the hemorrhages. Lesions with an appearance similar to retinoschisis and folds also have been rarely reported in leukemia . (See "Overview of the clinical presentation and diagnosis of acute lymphoblastic leukemia/lymphoma in children".)
Laboratory evaluation for bleeding disorder should be performed in suspected cases of AHT with hemorrhagic manifestations unless there are obvious indicators of abuse (eg, pattern skin lesions, multiple unexplained fractures). Patients with abnormal laboratory findings may benefit from additional evaluation by a hematologist. (See "Physical child abuse: Diagnostic evaluation and management", section on 'Evaluation for bleeding disorders' and "Approach to the child with bleeding symptoms".)
Elevated coagulation factors may reflect underlying brain pathology, rather than a coagulation disorder [91,92]. Brain injury can result in coagulopathy, which may be mild or severe (eg, disseminated intravascular coagulation). However, retinal hemorrhage in such patients without AHT is quite uncommon. (See "Disseminated intravascular coagulation in infants and children", section on 'Clinical manifestations' and "Evaluation and management of disseminated intravascular coagulation (DIC) in adults".)
Sepsis — Retinal hemorrhages have been described in some critically ill patients with sepsis, though in almost all cases, the patients had fewer than five retinal hemorrhages . The three subjects with more than 20 retinal hemorrhages all had concomitant leukemia, in addition to severe sepsis.
Intracranial hemorrhage — Intraocular hemorrhage (IOH) may occur in association with intracranial hemorrhage (eg, from a ruptured aneurysm or arteriovenous malformation, or epidural hematoma) and subsequent acutely elevated intracranial pressure [93-97]. Although the severity of the retinal hemorrhage is typically limited, ipsilateral severe hemorrhagic retinopathy has been described . Importantly, the pattern of hemorrhage is one primarily of vitreous and preretinal hemorrhage, less so intraretinal hemorrhage, and there are few hemorrhages, even if there is a large preretinal hemorrhage. This pattern contrasts with the patterns seen in AHT, which usually have many more hemorrhages. The combination of intraocular hemorrhage and intracranial hemorrhage has been referred to as Terson syndrome and is uncommon in infants and children [15,99,100]. However, note that the term “Terson syndrome” is best avoided, as it does not imply a specific etiology but rather the coincident presence of intraocular and intracranial hemorrhage. The presence of a vascular anomaly and the pattern of IOH help distinguish this diagnosis from AHT. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Evaluation and diagnosis' and "Brain arteriovenous malformations".)
Raised intracranial pressure — Less acute increases in intracranial pressure are associated with optic disc hemorrhages or superficial intraretinal hemorrhages located in the peripapillary region adjacent to or on a swollen optic nerve head . Raised intracranial pressure does not cause more widespread or multilayered retinal hemorrhage, nor does it cause retinoschisis or retinal folds .
Cerebral venous sinus and cortical vein thrombosis — Retinal hemorrhages can be seen in association with cerebral venous thrombosis . However, these hemorrhages are seen only in the presence of coincident factors and the patterns of retinal hemorrhage observed match the patterns caused by those factors, such as peripapillary superficial intraretinal hemorrhages from raised intracranial pressure and posterior pole intraretinal hemorrhages from sepsis, suggesting that venous thrombosis itself is not directly causative.
Glutaric aciduria — Glutaric aciduria type 1 is an autosomal recessive metabolic disorder that is sometimes associated with subdural hemorrhage after minor head trauma. Preexisting macrocephaly is a hallmark of the condition. Glutaric aciduria eventually results in serious neurological compromise, but affected children may have normal development in early childhood. Approximately 20 percent of patients with glutaric aciduria type 1 have seizures, and 20 to 30 percent have subdural hemorrhages or effusions that may raise the suspicion of AHT. Children with glutaric aciduria may be more prone to subdural hemorrhage after minor accidental trauma because of increased extra-axial fluid spaces. Retinal hemorrhages may also occur, typically just a few pre- or intraretinal hemorrhages that are confined to the posterior pole, though the range of severity of ocular manifestations is not fully described . (See "Organic acidemias: An overview and specific defects", section on 'Clinical manifestations of GA1'.)
In contrast to children with AHT, children with glutaric aciduria do not have bony abnormalities. Glutaric aciduria is diagnosed through urinary screening. Characteristic basal ganglia disease can develop even in the absence of significant changes in glutaric acid concentrations. The neuroimaging findings help to distinguish glutaric aciduria from AHT. (See "Organic acidemias: An overview and specific defects", section on 'Diagnosis of GA1'.)
Chest compression — Rarely cardiopulmonary resuscitation or Purtscher retinopathy has been associated with mild retinal changes in children:
●Cardiopulmonary resuscitation (CPR) – Many infants with severe AHT undergo CPR, including chest compressions and artificial ventilation. However, retinal hemorrhages rarely, if ever, result from CPR [15,42,68,104-107]. In contrast to retinal hemorrhages associated with AHT, CPR-associated retinal hemorrhages are few in number and confined to the posterior pole.
●Purtscher retinopathy – A severe and abrupt increase in intrathoracic pressure may cause intraocular abnormalities, possibly related to complement activation, microemboli, and nerve fiber infarcts (Purtscher retinopathy). Purtscher retinopathy may occur following severe acute compression injuries to the thorax, with characteristic manifestations that include white retinal patches (the predominant feature), few retinal hemorrhages, and retinal edema most commonly surrounding the optic disc. These findings do not resemble the retinal hemorrhages seen in AHT.
Purtscher retinopathy is not a characteristic finding of AHT, though it rarely may be seen (presumably related to the same compression of the thorax that results in rib fractures) . The lack of a single case report of Purtscher retinopathy following CPR provides additional evidence that CPR with chest compression is an unlikely cause of retinal hemorrhage.
Sudden infant death syndrome — Sudden infant death syndrome (SIDS) is not known to be associated with RH. SIDS is a common cause of death in children between 1 and 12 months of age. Children who die from SIDS may share several features with those who are victims of AHT, including the absence of explanatory history and the possibility of a prodromal syndrome such as gastrointestinal complaints. However, by definition, SIDS victims have no evidence of trauma by history, clinical examination, or autopsy. (See "Sudden unexpected infant death including SIDS: Initial management", section on 'Fatal child abuse'.)
Thus, most deaths related to child abuse can be distinguished from SIDS by a complete autopsy, death scene investigation, and a review of the medical history. However, the autopsy cannot distinguish between accidental or deliberate asphyxiation with a soft object and SIDS, although certain factors should raise the suspicion for deliberate asphyxiation and are discussed in detail separately. (See "Sudden unexpected infant death including SIDS: Initial management", section on 'Fatal child abuse'.)
Other considerations — A number of other conditions occasionally may be associated with retinal or vitreous hemorrhages in children and are listed in the table (table 2). However, the frequency of retinal hemorrhages in children with these conditions is extremely low . When retinal hemorrhages do occur in patients with these conditions, they typically are few in number and confined to the posterior pole. As with the conditions described above, these conditions usually have characteristic history, physical examination, and/or laboratory features that distinguish them from AHT. Moreover, many of these conditions have diagnostic eye findings other than retinal hemorrhages, the absence of which precludes those diagnoses. For example, retinopathy of prematurity occurs in premature infants and features areas of avascular retina and pathologic extraretinal neovascularization (see "Retinopathy of prematurity: Pathogenesis, epidemiology, classification, and screening"); Coats disease demonstrates retinal telangiectasias, retinal exudates, and retinal detachment (see "Approach to the child with leukocoria", section on 'Coats disease'); persistent fetal vasculature is characterized by various abnormal structural ocular findings; and infectious retinitis, such as from HSV, CMV, or toxoplasmosis (picture 6), have characteristic acute and post-inflammatory lesions in the retina. Diabetic retinopathy does not occur in children at this age .
Initial management — The management of children with suspected physical abuse is discussed separately. (See "Physical child abuse: Diagnostic evaluation and management", section on 'Management'.)
Ophthalmologic care — Young children who have eye findings of AHT require regular follow-up by an ophthalmologist. Children who have large, dome-shaped retinal hemorrhages require short-term follow-up (during the three to four weeks after injury), because the hemorrhages can break into the vitreous (picture 4). Dense hemorrhages in the vitreous can interfere with vision and may cause amblyopia and large, induced refractive errors in young children . (See "Amblyopia in children: Classification, screening, and evaluation", section on 'Deprivation amblyopia'.)
Vitrectomy may be warranted in patients with dense vitreous hemorrhage, particularly if the electrical response of the retina (electroretinogram) is good . However, even with these interventions, few patients with dense vitreous hemorrhages have been reported to see well after vitrectomy .
Visual potential in children with AHT is often limited by retinal and central nervous system injuries (ie, cortical and optic atrophy). Within these limitations, the correction of refractive errors and treatment of amblyopia can help to maximize visual potential . Maximization of visual potential and visual learning ability can help to optimize overall outcome in survivors of AHT, who often have associated neurologic, cognitive, and psychologic handicaps, as well as visual compromise . For children in whom vision cannot be improved, auditory and tactile learning can be promoted through early intervention programs. (See "Children and youth with special health care needs".)
If the child has normal vision and alignment three months after the injury, then the usual schedule of vision screening can be resumed as for any child. Persistent problems with strabismus and poor vision are followed as indicated by their severity. (See "Evaluation and management of strabismus in children", section on 'Overview of management' and "Vision screening and assessment in infants and children", section on 'Vision screening'.)
OUTCOME — Ophthalmologic findings of AHT that are associated with increased mortality include any one of the following [1,21,113,114]:
●Poor vision on presentation
●Poor pupillary response
●Diffuse retinal hemorrhages or retinal folds
The visual potential in children with AHT is limited by central nervous system injuries and retinal injuries. Visual outcome is good (ie, visual acuity of 20/40 or better) in one-third to one-half of survivors [6,113]. Most intraretinal hemorrhages resolve spontaneously without sequelae . Large vitreous or preretinal hemorrhages may require surgical intervention to prevent visual loss and/or amblyopia. Foveal subretinal hemorrhages are associated with central scotoma. (See 'Ophthalmologic care' above.)
Vision loss is thought to be more often caused by brain injury than retinal injury [21,111], although combined cerebral and ocular factors account for visual loss in some patients [115,116]. Bilateral retinal scarring can occur, both from retinoschisis and from retinal and vitreous hemorrhage [59,110,117].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Child abuse and neglect".)
SUMMARY AND RECOMMENDATIONS
●Evaluation – Prompt consultation with an ophthalmologist for a complete dilated retinal examination should occur in all children younger than five years of age in whom abusive head trauma (AHT) is suspected based upon evidence of traumatic brain injury on neuroimaging, abnormal neurologic findings on physical examination, or clear history of shaking or abusive head injury. Completion of this examination within 24 to 72 hours of presentation is recommended by the American Academy of Pediatrics. However, we suggest that, whenever possible, eye examinations should be conducted within 24 to 36 hours because there is potential for multiple intraretinal hemorrhages to resolve after this time period. (See 'Ophthalmology consultation' above and "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)
Postmortem examination, preferably by an ocular pathologist, should be performed in cases of suspected abuse and unexplained deaths in infants and young children regardless of whether an eye examination was conducted prior to death. (See 'Initial eye examination' above.)
●Ophthalmologic findings – The ophthalmologic findings of AHT may include retinal hemorrhages (figure 1 and picture 2 and picture 4), retinal folds and retinoschisis (picture 3), vitreous hemorrhage, papilledema, optic atrophy, and optic nerve sheath hemorrhage. (See 'Ophthalmologic findings' above.)
The frequency of retinal hemorrhages in children with AHT is approximately 85 percent. The finding of retinal hemorrhages increases the suspicion of inflicted injury but may not confirm the diagnosis, nor does the absence of retinal hemorrhage exclude the diagnosis. However, increasing retinal hemorrhage severity is associated with increased severity of trauma and consequently increased likelihood of abuse. In approximately two-thirds of cases, retinal hemorrhages caused by AHT are bilateral, numerous, multilayered, and present at the ora serrata (figure 5). While retinal hemorrhages cannot be accurately dated to determine the precise timing of the traumatic event, the patterns of hemorrhage seen can provide some indication of injury timing. (See 'Retinal hemorrhages' above.)
●Eye findings and diagnosis – The diagnosis of AHT is made on the basis of the entire clinical scenario, which typically includes injuries suspicious for abuse (eg, diffuse, extensive, multilayered retinal hemorrhages; subdural hematoma; fractures; bruises; intraabdominal injury [eg, liver laceration, duodenal hematoma or perforation]) and a history that is inconsistent with the severity and/or type of injury. (See 'Diagnostic implications of eye findings' above and "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)
●Differential diagnosis – The most common cause of retinal hemorrhage in an infant or young child is trauma, either birth in the first month of life or AHT afterwards. However, a variety of conditions may result in retinal hemorrhages in infants and young children (table 2). Most of these conditions have characteristic history, physical examination, and/or laboratory features that distinguish them from AHT. In addition, when retinal hemorrhages occur in these conditions, they generally are few in number and confined to the posterior pole and are accompanied by other specific ocular or retinal findings. Having these conditions does not preclude abusive injury. (See 'Differential diagnosis' above.)
●Ophthalmologic care and outcome – Young children who have ophthalmologic findings of AHT require regular follow-up by an ophthalmologist. The visual potential in children with AHT may be limited by central nervous system injuries and retinal injuries. Visual outcome is good (ie, visual acuity of 20/40 or better) in one-third to one-half of survivors. (See 'Ophthalmologic care' above and 'Outcome' above.)
ACKNOWLEDGMENT — The editorial staff at UpToDate acknowledge Brian Forbes, MD, PhD, who contributed to earlier versions of this topic review.
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