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Review Question - QID 217142

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QID 217142 (Type "217142" in App Search)
A 6-week-old boy is brought to the emergency room by his mother for “seeming like he is not being himself.” The patient’s mother reports that approximately 6 hours ago, he rolled off the bed by himself when she was not paying attention and noticed that he had fallen to the ground. The patient was delivered via spontaneous vaginal delivery at 39 weeks, and his neonatal course and developmental history are unremarkable. The patient did not receive vitamin K at birth due to maternal preference. His mother has a history of von Willebrand disease and is his only caretaker. She works as a greeter in retail and expresses concern that she has difficulty receiving support for child care. The patient’s temperature is 98.6°F (37.0°C), blood pressure is 96/60 mmHg, pulse is 130/min, and respirations are 35/min. On physical examination, the child is lethargic. An ophthalmic exam shows bilateral retinal hemorrhages. He has a bruise over his right arm. A non-contrast computed tomography (CT) scan of the head is obtained and shown in Figure A. Which of the following is the most likely diagnosis?
  • A

Accidental trauma

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Osteogenesis imperfecta

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Shaken baby syndrome

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Vitamin K deficiency bleeding

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von Willebrand disease

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  • A

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This patient with a caregiver who lacks social support, a history of trauma inconsistent with developmental age (6-week-old infants cannot roll over), retinal hemorrhages, and a non-contrast CT scan of the head showing acute-on-chronic subdural hematomas most likely experienced non-accidental trauma (NAT). NAT that consists of blunt force trauma to the head or shaking are referred to as shaken baby syndrome.

Abusive head injury is the most common cause of death in children that experience NAT. Major risk factors for NAT include high caretaker stress and low caretaker educational attainment. Shaking babies leads to acceleration-deceleration injuries that cause shearing of the dural veins and vitreomacular traction. This manifests clinically as subdural hematomas and retinal hemorrhages. In NAT, the history and mechanism of injury will often be inconsistent with developmental age. Other classic findings of NAT include bruising, scalding, and fractures in different stages of healing. Evaluation should include evaluation for an underlying bleeding disorder, non-contrast head CT, skeletal survey, and consultation with a child abuse team. Informing child protective services is mandatory if NAT is suspected. Treatment depends on the injury. Acute subdural hemorrhages may require surgical intervention if midline shift and/or substantial intracranial edema is present.

Paul et al. discuss the epidemiology, risk factors, presentation, diagnosis, and management of non-accidental trauma in pediatric patients. They found that fractures, bruising, abusive head trauma, and ocular findings are common presenting findings in NAT. They recommended that seizure prophylaxis be administered in children with findings of severe traumatic brain injury.

Figure/Illustration A show a non-contrast CT scan of the head in an infant with suspected shaken baby syndrome. Subacute frontal subdural hematomas (red arrow) with an acute crescent-shaped right temporoparietal subdural hematoma (blue arrow) are seen. In addition, a posterior interhemispheric subdural hematoma is indicated with a yellow circle.

Incorrect Answers:
Answer 1: Accidental trauma to the head could cause lethargy and retinal hemorrhages, especially in a patient with multiple risk factors for having a bleeding diathesis. However, subdural hematomas from different times of injury and an injury inconsistent with developmental age (rolling off bed) make NAT more likely.

Answer 2: Osteogenesis imperfecta (OI) can present with multiple fractures in different stages of healing and mimic non-accidental trauma. OI can be distinguished from NAT by implausible mechanisms of injury on history and the presence of retinal hemorrhages in the latter. OI can be diagnosed by genetic testing in a patient with recurrent fractures.

Answer 4: Vitamin K deficiency bleeding (VKDB) can present with bleeding in the intestines or brain within the first 6 months of life. There is an 80-fold increased risk of VKDB in patients who do not receive vitamin K at birth. However, in this patient with an implausible mechanism of trauma, NAT is more likely than NKDB.

Answer 5: von Willebrand disease (VWD) is a bleeding diathesis that is inherited in an autosomal dominant manner in the majority of cases. Patients with von Willebrand disease present with easy mucocutaneous bleeding. Although this patient has a family history of VWD, his implausible mechanism of trauma is more consistent with NAT than VWD; also, retinal hemorrhages would be atypical with VWD.

Bullet Summary:
Abusive head trauma (shaken baby syndrome) presents with a history of injury inconsistent with developmental age, bruises and fractures in different stages of healing, subdural hematomas, and retinal hemorrhages.

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