Decreased Alertness in a 10-Month-Old Girl

A 10-month-old girl is transferred from an outside urgent care center to the emergency department (ED) for dehydration and a change in her level of alertness. According to her mother's boyfriend, she had been crying in her crib before she was noted to throw her arms in the air for approximately 5 minutes and then breathe irregularly. She vomited once and continued to cry inconsolably before she arrived at the urgent care center. An abdominal radiograph was performed at the urgent care center and interpreted as consistent with possible constipation, but it was otherwise unremarkable. She has had multiple episodes of nonbloody, nonbilious emesis on the way to the ED. There is no recent history of diarrhea or change in bowel habits.

The patient was recently diagnosed with an upper respiratory infection and has received several doses of acetaminophen over the last few days. Her symptoms have included tactile fevers, runny nose, and raspy cough. Before this occurrence, her breathing had been normal and without increased effort. She had been feeding well earlier today. She is currently taking no prescription medications and has no reported allergies. Her immunizations are not up-to-date; she only received immunizations at 2 months of age. She lives at home with her mother, her mother's boyfriend, and her 2-year-old brother.

The physical examination is most notable for her general appearance; she appears somnolent, is lying motionless, is not making eye contact or engaging in other social interactions, and is not crying. She vomits several times during the evaluation. Her vital signs include an axillary temperature of 99.1°F (37.3°C), a heart rate of 90 bpm, a blood pressure of 101/56 mm Hg, a respiratory rate of 12 breaths/min, and an oxygen saturation of 100% while breathing room air. Her pupils are equal and 5 mm in diameter; they are measured at 3 mm in response to light. The anterior fontanel is flat. A small, 5-mm, purple ecchymosis overlying the left maxilla is noted. Two lacerations on the inferior mucosal surface of the upper lip are identified that line up with the upper incisors. The lungs are clear to auscultation and the heart sounds are normal. The abdomen feels soft and appears nondistended and nontender. Bowel sounds are present. The stool is guaiac-negative. Capillary refill of the fingernails is less than 2 seconds. The neurologic examination is notable for diffuse hypotonia.

In the ED, she is given a bolus of 200 mL of normal saline with 5% dextrose and continued on maintenance fluid. The initial laboratory results are significant for leukocytosis and anemia, with a white blood cell (WBC) count of 18.4 × 10³/µL (18.4 × 10⁹/L), with 72% neutrophils (0.72), a hemoglobin of 9.7 g/dL (97 g/L), and a hematocrit of 27.1% (0.271). Her serum glucose is normal at 108 mg/dL (5.99 mmol/L), and the electrolytes are likewise within normal limits. An ophthalmologic examination (see Figure 1) and computed tomography (CT) scanning of the head (see Figure 2) are performed. A skeletal survey is ordered as well.

Questions answered correctly will be highlighted.

What is the diagnosis?

Hint: The findings of the retinal examination are very characteristic of this diagnosis.

Meningitis

Abusive head trauma

Cysticercosis

Discussion

The initial CT scan of the head obtained in the ED (see Figure 2) shows bilateral subdural hematomas outlining bilateral cerebral convexities, with hyperdense blood noted in the left frontal lobe. The patient was started on fosphenytoin for seizure prophylaxis. The ophthalmologic examination revealed bilateral retinal hemorrhages extending into the periphery (see Figure 1). Intraretinal hemorrhages of the macula were noted in the left eye, with possible choroidal rupture. On careful questioning, the caretaker repeatedly denied any history of trauma. A skeletal survey was performed that revealed a healing right radial neck fracture. The medical record from the urgent care center was obtained; this included a clinical note dated 4 months prior to patient's presentation to the ED, which described a visit for repeated emesis and irritability. A bruise on the chin was noted on that visit, and the explanation given was a fall inside the patient's crib that occurred 4 days prior to that visit. A report to the Department of Children and Families was found to have been made for missed well-child visits. The level of suspicion for suspected abusive head trauma (AHT) and child abuse was extremely high. It is important for emergency clinicians to be mindful of the diagnosis of AHT, but there are other rare causes that can mimic abuse.

Abusive head injury, sometimes referred to as "shaken baby syndrome,"^[1] is the most common cause of death resulting from child abuse.^[2] The majority of cases occur in infants less than 1 year old.^[3] Head injury among infants in this age group is often the result of abuse and the mechanism of injury, although much debated, is usually thought to be significant forces generated from angular deceleration with or without impact. AHT in this population represents a significant fraction of young children admitted for head injury.^[4] Approximately 30% of children aged 0-3 years admitted to pediatric hospitals for intracranial injury have been found to meet the criteria for abuse.^[5] Many children with AHT also have a clinical history or findings consistent with prior maltreatment. Crying is thought to be a trigger for many cases of AHT and prevention efforts are directed toward caregiver response to colicky babies and crying infants.

The diagnosis of AHT can be difficult to establish; the clinical presentations may be nonspecific, children are often too young to give history, and witnesses and confessions are rare. The reasons for seeking care in children include seizure, breathing difficulty, apnea, and apparent lifelessness. A history of trauma is often lacking. Approximately 30% of children with AHT may be missed on the initial presentation.^[6] Common misdiagnoses include viral gastroenteritis, sepsis, and accidental head injury. A history of injury mechanism incompatible with an infant's developmental stage or degree of force required to inflict severe injury may raise the suspicion for AHT. Common symptoms at presentation are often the result of acute brain injury (ie, lethargy, decreased level of consciousness, vomiting, apnea, hypotonia, and seizures).

The physical examination findings may include evidence of soft tissue injury, particularly swelling or bruising; however, the absence of bruising or other evidence of trauma neither excludes injury nor abuse.^[7] Funduscopic examination should be performed in any child suspected to have abusive head injury, preferably by an ophthalmologist with sufficient pediatric experience to determine the significance of any identified injury.^[8] Retinal hemorrhages are a hallmark finding in abusive head injury, and they are present in a majority of children who carry the diagnosis.^[9] They may be unilateral or bilateral and involve 1 or more layers. Not all retinal hemorrhages are the same with respect to their significance in predicting an abusive mechanism. The most specific pattern of retinal hemorrhages is numerous hemorrhages involving several layers of the retina and extending to the periphery.^[10] No pattern of hemorrhages, however, is pathognomonic for abuse. The mechanism of retinal hemorrhages is unclear, but the leading theory is that they are caused by vitreous traction on the retina during acceleration/deceleration. Lasting visual impairment in those children who survive AHT is common.^[11]

CT scanning is an essential part of the initial workup of suspected head trauma. CT scanning can also be helpful as a screening neuroimaging study in children with suspected abuse. Even without clinical examination findings of brain injury, a significant number of abused infants will have important findings on neuroimaging.^[12,13] Unilateral, bilateral, or parafalcine subdural hemorrhages are the most common radiologic finding in infants with AHT. Subdural hemorrhages of mixed attenuation have previously been considered as evidence for repeated head injury, with hyperdense components of the hemorrhage associated with injury occurring in the past 48-72 hours and hypodense components representing older injury occurring more than 3 weeks prior to the scan. Hyperacute bleeding or the mixing of blood and cerebrospinal fluid (CSF), however, can produce mixed-density lesions from a single injury.^[14] While the presence of subdural hemorrhage lends supporting evidence to the diagnosis of head trauma, inferences about the timing and mechanism of injury cannot be drawn with certainty from a single noncontrast CT scan.^[15] Magnetic resonance imaging (MRI) can be a useful study for demonstrating parenchymal contusion, axonal shearing, extra-axial hemorrhages, and posterior fossa injuries. Diffusion-weighted imaging and apparent diffusion coefficient mapping are particularly useful in identifying acute hypoxic-ischemic injury.

Additional supportive evidence for child abuse is obtained through a skeletal survey. The presence of previously healed fractures in infants is strongly suggestive of chronic abuse.

While the cause of subdural hematoma in association with retinal hemorrhage will most commonly be abusive head injury, a differential diagnosis for these findings must be considered. Coagulopathies have been associated with retinal and intracranial hemorrhage in infants, including hemophilia, vitamin-K deficiency, and disseminated intravascular coagulopathy. Retinal hemorrhages in these disorders are typically confined to the posterior pole, and the nature of the bleeding problem can be detected by laboratory tests. It is recommended to perform a prothrombin time, activated partial-thromboplastin time, and a platelet count as minimum screening tests.

Glutaric aciduria type I, a rare metabolic disease, is associated with developmental delay and subdural hemorrhages. Performing an assay for organic acids in the urine can test for this disease. Other causes of intracerebral hemorrhage include cerebral malaria, intracranial aneurysms, galactosemia, and meningitis. Osteogenesis imperfecta is an uncommon connective tissue disorder that frequently results in fractures. Subdural hemorrhage has rarely been described as a complication of this disease.^[22] Because these disorders can closely mimic abusive head trauma, it is important to maintain a nonaccusatory and open-minded posture during the initial evaluation, as parents are understandably sensitive to the possibility that they are being accused of harming their children. Some helpful statements include "I'm concerned that someone may have harmed your child" and "several diseases can explain this pattern of injury, including trauma. We need to check for other signs of these illnesses to make sure your child is safe."

AHT is likely underdiagnosed and underreported, which contributes to the dismal outcomes for children eventually diagnosed with abuse.^[24] In multiple series, the mortality is approximately 20%.^[11,25] The neurologic outcome is also poor, with many survivors having persistent neurologic and behavioral deficits.^[11,26] Having a high suspicion for abusive head injury is critical in the appropriate setting. Clinicians should have a low threshold for performing CT scans of the head on infants coming in with nonspecific findings that could be explained by head injury, when appropriate. While reporting a reasonable suspicion for abuse is mandatory, it is not the job of the healthcare provider to determine the social or legal management of any case. A child protection team, if available, should be consulted with any concerns of abusive injury.

The patient in this case was admitted to the pediatric intensive care unit (ICU). A repeat CT scan of the head was performed 10 hours after presentation because her somnolence failed to improve. It showed an interval increase in subdural blood located above the tentorium. She was then taken emergently to the operating room (OR) for evacuation of the subdural hematomas and placement of bilateral subdural external drains. The subdural pressure was noted to be markedly elevated. Subdural membranes were noted to separate layers of blood in the left frontal lobe. She was again taken to the OR several days later for removal of the subdural drains. She gradually became more alert and playful. The child protection team was contacted in the ED and suspicion for abuse was reported. Further conversations with the mother revealed a history of physical abuse at the hands of her current boyfriend. The Department of Children and Families (the regional department responsible for addressing issues of child safety and potential abuse) assumed immediate temporary custody of the patient and of her older brother. Testing for conditions that mimic the signs of abuse was completed, and no sign of other medical illnesses was found. The patient was discharged 12 days after admission and has had follow-up visits with neurosurgery and ophthalmology. She is currently living with her second foster family and is noted to have persistent developmental delay. Her retinal findings have improved and her foster parents have not noticed any evidence of visual impairment.

Questions answered correctly will be highlighted.

You suspect that a patient you are examining is being abused. Which of the following injuries, if they were present in the patient you are examining, would be pathognomonic for abusive injury?

Bilateral subdural hemorrhages

Rib fractures

Retinal hemorrhages

Bruising in infants

None of the above

During the course of several days, you evaluate a number of children with injuries as reported by their parents. You obtain a history and perform a physical examination on each child, and radiographs are subsequently performed. Which of the following situations should alert you to a strong possibility of abuse/nonaccidental injury?

A spiral tibia fracture in a 3-year-old boy who fell and twisted his leg while jumping on a couch.

A supracondylar fracture in a 6-year-old who fell from the monkey bars onto an outstretched arm.

A posterior rib fracture in a 13-month-old who fell backwards off a single step.

The mechanism of injury must always be considered when evaluating for whether or not there is any suspicion that any given fracture may have occurred by nonaccidental means. Infants and young toddlers typically cannot generate enough force to result in a fracture unless falling from a height. In this case, a fall from 1 step is not sufficient to result in a rib fracture. Certain types of fractures also should raise concern, such as spiral fractures of long bones. The exception to this is a spiral fracture of the tibia, often referred to as a "toddler fracture" since it is particularly seen amongst toddlers. These children are often rambunctious and, although lacking in coordination, they have the physical capacity to climb, jump, and fall while running, all of which may result in an accident that will generate enough energy to cause this type of fracture.

References

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