Spotlight Case: Shaken Up
A 13-month-old boy with asthma and no ocular history was brought to the hospital after being found minimally responsive. Per history, the child was laid down for a nap on a futon at 10:30 a.m. that day. Around 11:30 a.m., the mother's boyfriend heard the patient fall from the futon where he was sleeping and found him unconscious with shallow breathing. At that time, an ambulance was called and arrived within eight to ten minutes of contact. Cardiopulmonary resuscitation was initiated. Air transport then transferred the patient to a tertiary care hospital. The patient was admitted, intubated, and underwent extensive full-body imaging. Pupils were noted to be minimally reactive. Ophthalmology was consulted.
A CT of the head showed an epidural hematoma and intraventricular hemorrhage in the lateral and third ventricles. On skeletal survey, no other fractures were identified. Ophthalmologic examination was remarkable for bilateral 4.5 mm pupils, which were nonreactive. There was no resistance to retropulsion. Tactile tensions were soft in both eyes. Portable slit-lamp examination at bedside was unremarkable. Dilated fundus examination was notable for discs obscured by hemorrhage bilaterally, with extensive preretinal, intraretinal, and subretinal hemorrhages without obvious macular retinoschisis (Figure 1).
What’s your diagnosis?
The correct diagnosis is shaken baby syndrome.
The differential diagnosis of shaken baby syndrome includes vascular occlusion and intracranial pressure due to mass lesion or pseudotumor.
Shaken baby syndrome is almost always associated with abuse by a caretaker, frequently a male or nonrelative, who shakes an infant violently to attempt to quiet the crying child. The force administered to the infant creates linear and rotational movement of the brain that result in the brain turning from its central axis. Due to the fact that the head is larger than the body in children, excessive movement of the head from the already immature neck musculature and support causes severe internal damage.
When an infant is shaken violently, the brain is perturbed, and tearing of veins from the cortex to the dural venous sinus occurs. This often gives evidence of shaking when physical external damage such as head trauma is not evident.
When the infant brain is shaken with such force, there is often evidence for subdural hemorrhages, an indicator of accelerated movement-based trauma. These subdural hemorrhages are most frequently accompanied by retinal hemorrhages, also caused by the accelerated movement. The intensity of the hemorrhages can be used as an indicator of the extent of abuse and force of the trauma.
It should be noted that these hemorrhages can occur in situations outside of abuse (ie, motor vehicle accidents, etc.); however, the combination of retinal and subdural hemorrhages comprise the vast majority of cases of shaken baby syndrome. Children diagnosed with shaken baby syndrome may have other signs of abuse, includingbroken bones or cigarette burns.
Immediately after being shaken, an infant may manifest signs of weakness such as vomiting, lethargy, feeding problems, and—in extreme cases—altered mental status or convulsions. These are nonspecific symptoms, so if brought in for medical evaluation, a high index of suspicion for abuse must be maintained.
Retinal hemorrhages are a strong indicator of shaken baby syndrome, as they are rare, even in the most extreme of trauma. Retinal hemorrhages tend to be flame-shaped and occur bilaterally in the majority of cases. Intraretinal hemorrhages and subhyaloid hemorrhages may lead to retinal detachment and hemorrhages of the posterior pole and vitreous. Additionally, intracranial pressure and swelling of the brain have been documented.
The prognosis varies, from complete recovery to death, depending on the extent of the trauma. Even with medical intervention, patients can rapidly decompensate, as in the case of this patient who died within hours of admission. Roughly two-thirds of patients survive; however, half of those who survive have neuropathic and neurological damage.
The American Academy of Ophthalmology (AAO) has published appropriate steps to monitor and manage patients diagnosed with shaken baby syndrome. Neuroimaging is frequently performed. Elevated intracranial pressure may require neurosurgical intervention for draining of fluid and hematomas. Pupillary response documentation and fundus photographs are recommended initially as part of a complete eye examination. Retinal hemorrhages with subdural hemorrhages are usually sufficient to raise suspicion of this diagnosis. While no ophthalmic interventions may be necessary in the acute setting, social workers and protective services should be contacted to ensure the patient does not incur additional harm. Community awareness programs are growing to prevent the high mortality and morbidity associated with this form of abuse.
- Bilateral retinal hemorrhages in an infant should always raise suspicion of shaken baby syndrome.
- Neuroimaging and skeletal surveys may be necessary to evaluate for subdural hemorrhages and broken bones.
- When possible in these cases, it is important to take retinal fundus photos to document the extent of injury.
- If shaken baby syndrome is suspected, evaluation should be coordinated with a pediatrician and social services—this can be life-saving. The infant may require urgent admission to an intensive care unit.
- Retinal surgery for any associated retinal detachment or hemorrhage is usually delayed until the patient is medically stable.
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- Togioka BM, Arnold MA, Bathurst MA, et al. Retinal hemorrhages and shaken baby syndrome: an evidence-based review [published online December 11, 2008]. J Emerg Med. 2009;37(1):98-106. doi:10.1016/j.jemermed.2008.06.022.
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Mr. Menon - None.
Dr. Weaver - None.
Dr. Reddy - None.