Grand Rounds at the New England Eye Center

Infant presents with bilateral retinal hemorrhages

Pupils were 3 mm, round and sluggishly reactive bilaterally without afferent pupillary defects.

A 3-month-old full-term female infant presented to the University of New Mexico Hospital with decreased oral intake, reduced wet diapers and possible seizure activity.

Alison J. Lauter
Alison J. Lauter
Sarah E. Thornton
Sarah E. Thornton

The infant was noted to be healthy until 3 weeks before presentation, when she became inconsolable and colicky. The patient also had 10 days of intermittent vomiting that resolved several days before presentation. According to the mother, 5 days before presenting to the emergency department, the infant fell off the couch and hit her head on the hardwood floor with no loss of consciousness. The night before presentation, the infant was noted to have “difficulty focusing her eyes” and shaking of the right leg. A low-grade fever of less than 100°F was also noted the evening before presentation, for which the child received oral Tylenol. Over the past 24 hours, the mother reported the child had only 4 ounces of fluid intake and three wet diapers. Ophthalmology was consulted by the pediatrics team to evaluate the infant for inability to fix and follow objects.

Bilateral retinal hemorrhages
Bilateral retinal hemorrhages radiating from the optic nerve all the way to the periphery in a 3-month-old infant.

Source: Huan Meng Mills, MD, and Arup Das, MD, PhD

Examination

On examination, the infant was minimally responsive. The patient did not blink her eyes to bright light. Pupils were 3 mm, round and sluggishly reactive bilaterally without afferent pupillary defects. Both eyes were soft to palpation. The external exam was within normal limits bilaterally without any masses or discoloration. The conjunctival membranes were white and quiet. The corneas were clear bilaterally. The anterior chambers were deep and formed without hyphema or hypopyon. The red reflex was intact bilaterally. Dilated fundus exam revealed bilateral pink and healthy optic nerves with sharp margins and absent edema. Retinal hemorrhages were noted in all four quadrants bilaterally (Figure 1). The majority of the hemorrhages were intraretinal with some subretinal and preretinal hemorrhages.

What is your diagnosis?

See answer on next page.

Retinal hemorrhages

The differential diagnosis for retinal hemorrhages in an infant includes coagulopathy, thrombocytopenia, disseminated intravascular coagulation, leukemia, Terson syndrome, bacterial meningitis, cytomegalovirus infection, retinal hemangioma, increased intracranial pressure, accidental head trauma and abusive head trauma. The finding of extensive bilateral retinal hemorrhages in a 3-month-old with minor trauma is highly concerning for nonaccidental causes of injury. The number and extensive nature of the hemorrhages are concerning features that make abuse highly likely. The sluggish pupils and unresponsiveness are also concerning features for abusive head trauma.

Case continued

When child abuse is suspected, clinicians should always emphasize the need for further evaluation and avoid making accusations. If possible, photos should always be obtained to document the hemorrhages. Social work and child protective services should be notified immediately. In any infant suspected of head trauma, brain imaging such as a CT scan or MRI of the head should be ordered emergently. A stat CT scan of the head without contrast was obtained for our patient and revealed bilateral subdural hematomas, cerebral edema and brain parenchymal atrophy (Figure 2). Shortly after admission, an EEG was also performed that revealed status epilepticus and was thought to be due to significant neurological trauma. Neurosurgical intervention was required to drain the subdural hematomas twice during the admission due to elevated intracranial pressure. A full skeletal survey was also conducted on our patient, which did not reveal any other fractures or bodily injuries.

A non-contrast head CT
A non-contrast head CT was obtained and revealed bilateral subdural hematomas, diffuse cerebral edema and brain parenchymal atrophy.

Extensive blood work, including a complete blood count with differential, platelet count, and prothrombin and partial thromboplastin times, was obtained and was within normal limits for our patient. A comprehensive panel with fibrinogen, thrombin time, factors 8, 9, 11 and 13, and von Willebrand testing was also obtained to exclude coagulopathy. Serum AST, ALT and lipase were obtained to rule out traumatic abdominal injury and traumatic pancreatitis. These labs were within normal limits and consistent with the abdominal ultrasound, which was negative for intra-abdominal injuries.

Discussion

According to the National Institute of Child Health and Human Development, more than 9 million children present to emergency departments seeking treatment for traumatic injuries yearly. Abusive head trauma is the most common cause of death among these injured children. Upward of 89% of infants involved in abusive head trauma sustain retinal hemorrhages. Mortality rates from abusive head trauma range from 15% to 25%. In 1998, Overpeck and colleagues estimated that 80% of infant homicides are thought to be due to child abuse. Younger children, particularly infants younger than 6 months of age, were at highest risk. In addition, other risk factors include a maternal age of younger than 19 years, no prenatal care and fewer than 12 years of education.

Ophthalmologists play a key role in identifying abusive head injury because retinal hemorrhages are a clinical hallmark. Severe repetitive acceleration-deceleration forces coupled with vitreoretinal traction in the presence or absence of blunt trauma in young children are thought to cause retinal hemorrhages. Ideally, an ophthalmologist should perform an indirect dilated fundus exam within 72 hours of presentation. The more extensive the retinal hemorrhage, the more likely the patient is to be a victim of abusive head trauma. The severity of retinal hemorrhages also directly correlates with the severity of head injury. A small number of retinal hemorrhages can be a normal finding after spontaneous vaginal delivery or accidental head injury.

Kivlin and colleagues analyzed 123 children with subdural hematomas secondary to abuse in a retrospective case series. Of these children, 90% had ophthalmologic assessments, and retinal hemorrhages were detected in 83% of examined children. The retinal hemorrhages were noted to be bilateral in 85% of children who were affected. In addition, the researchers noted that poor visual response, poor pupillary response and retinal hemorrhages were strong predictors of infant death. Of infants who survived, approximately 20% had poor vision due to severe neurological impairment. It was also noted that non-ophthalmologists missed 29% of retinal hemorrhages in affected patients.

In conclusion, severe bilateral retinal hemorrhages are highly concerning for abusive head trauma and should prompt an extensive trauma workup. Furthermore, any child who is suspected of having abusive head trauma should receive a full dilated exam by an ophthalmologist within 72 hours of presentation.

A 3-month-old full-term female infant presented to the University of New Mexico Hospital with decreased oral intake, reduced wet diapers and possible seizure activity.

Alison J. Lauter
Alison J. Lauter
Sarah E. Thornton
Sarah E. Thornton

The infant was noted to be healthy until 3 weeks before presentation, when she became inconsolable and colicky. The patient also had 10 days of intermittent vomiting that resolved several days before presentation. According to the mother, 5 days before presenting to the emergency department, the infant fell off the couch and hit her head on the hardwood floor with no loss of consciousness. The night before presentation, the infant was noted to have “difficulty focusing her eyes” and shaking of the right leg. A low-grade fever of less than 100°F was also noted the evening before presentation, for which the child received oral Tylenol. Over the past 24 hours, the mother reported the child had only 4 ounces of fluid intake and three wet diapers. Ophthalmology was consulted by the pediatrics team to evaluate the infant for inability to fix and follow objects.

Bilateral retinal hemorrhages
Bilateral retinal hemorrhages radiating from the optic nerve all the way to the periphery in a 3-month-old infant.

Source: Huan Meng Mills, MD, and Arup Das, MD, PhD

Examination

On examination, the infant was minimally responsive. The patient did not blink her eyes to bright light. Pupils were 3 mm, round and sluggishly reactive bilaterally without afferent pupillary defects. Both eyes were soft to palpation. The external exam was within normal limits bilaterally without any masses or discoloration. The conjunctival membranes were white and quiet. The corneas were clear bilaterally. The anterior chambers were deep and formed without hyphema or hypopyon. The red reflex was intact bilaterally. Dilated fundus exam revealed bilateral pink and healthy optic nerves with sharp margins and absent edema. Retinal hemorrhages were noted in all four quadrants bilaterally (Figure 1). The majority of the hemorrhages were intraretinal with some subretinal and preretinal hemorrhages.

What is your diagnosis?

See answer on next page.

PAGE BREAK

Retinal hemorrhages

The differential diagnosis for retinal hemorrhages in an infant includes coagulopathy, thrombocytopenia, disseminated intravascular coagulation, leukemia, Terson syndrome, bacterial meningitis, cytomegalovirus infection, retinal hemangioma, increased intracranial pressure, accidental head trauma and abusive head trauma. The finding of extensive bilateral retinal hemorrhages in a 3-month-old with minor trauma is highly concerning for nonaccidental causes of injury. The number and extensive nature of the hemorrhages are concerning features that make abuse highly likely. The sluggish pupils and unresponsiveness are also concerning features for abusive head trauma.

Case continued

When child abuse is suspected, clinicians should always emphasize the need for further evaluation and avoid making accusations. If possible, photos should always be obtained to document the hemorrhages. Social work and child protective services should be notified immediately. In any infant suspected of head trauma, brain imaging such as a CT scan or MRI of the head should be ordered emergently. A stat CT scan of the head without contrast was obtained for our patient and revealed bilateral subdural hematomas, cerebral edema and brain parenchymal atrophy (Figure 2). Shortly after admission, an EEG was also performed that revealed status epilepticus and was thought to be due to significant neurological trauma. Neurosurgical intervention was required to drain the subdural hematomas twice during the admission due to elevated intracranial pressure. A full skeletal survey was also conducted on our patient, which did not reveal any other fractures or bodily injuries.

A non-contrast head CT
A non-contrast head CT was obtained and revealed bilateral subdural hematomas, diffuse cerebral edema and brain parenchymal atrophy.

Extensive blood work, including a complete blood count with differential, platelet count, and prothrombin and partial thromboplastin times, was obtained and was within normal limits for our patient. A comprehensive panel with fibrinogen, thrombin time, factors 8, 9, 11 and 13, and von Willebrand testing was also obtained to exclude coagulopathy. Serum AST, ALT and lipase were obtained to rule out traumatic abdominal injury and traumatic pancreatitis. These labs were within normal limits and consistent with the abdominal ultrasound, which was negative for intra-abdominal injuries.

Discussion

According to the National Institute of Child Health and Human Development, more than 9 million children present to emergency departments seeking treatment for traumatic injuries yearly. Abusive head trauma is the most common cause of death among these injured children. Upward of 89% of infants involved in abusive head trauma sustain retinal hemorrhages. Mortality rates from abusive head trauma range from 15% to 25%. In 1998, Overpeck and colleagues estimated that 80% of infant homicides are thought to be due to child abuse. Younger children, particularly infants younger than 6 months of age, were at highest risk. In addition, other risk factors include a maternal age of younger than 19 years, no prenatal care and fewer than 12 years of education.

Ophthalmologists play a key role in identifying abusive head injury because retinal hemorrhages are a clinical hallmark. Severe repetitive acceleration-deceleration forces coupled with vitreoretinal traction in the presence or absence of blunt trauma in young children are thought to cause retinal hemorrhages. Ideally, an ophthalmologist should perform an indirect dilated fundus exam within 72 hours of presentation. The more extensive the retinal hemorrhage, the more likely the patient is to be a victim of abusive head trauma. The severity of retinal hemorrhages also directly correlates with the severity of head injury. A small number of retinal hemorrhages can be a normal finding after spontaneous vaginal delivery or accidental head injury.

PAGE BREAK

Kivlin and colleagues analyzed 123 children with subdural hematomas secondary to abuse in a retrospective case series. Of these children, 90% had ophthalmologic assessments, and retinal hemorrhages were detected in 83% of examined children. The retinal hemorrhages were noted to be bilateral in 85% of children who were affected. In addition, the researchers noted that poor visual response, poor pupillary response and retinal hemorrhages were strong predictors of infant death. Of infants who survived, approximately 20% had poor vision due to severe neurological impairment. It was also noted that non-ophthalmologists missed 29% of retinal hemorrhages in affected patients.

In conclusion, severe bilateral retinal hemorrhages are highly concerning for abusive head trauma and should prompt an extensive trauma workup. Furthermore, any child who is suspected of having abusive head trauma should receive a full dilated exam by an ophthalmologist within 72 hours of presentation.