Introduction
The true incidence and prevalence of child abuse is unknown. The reason for this is that in nearly every study trying to establish the incidence and prevalence, researchers use their own definition. Most often this is a ‘broad definition’, such as that of the World Health Organization (WHO): ‘Child abuse, sometimes referred to as child abuse and neglect, includes all forms of physical and emotional ill-treatment, sexual abuse, neglect, and exploitation that results in actual or potential harm to the child’s health, development or dignity. Within this broad definition, five subtypes can be distinguished—physical abuse; sexual abuse; neglect and negligent treatment; emotional abuse; and exploitation’.
A specific form of child abuse which can lead to serious damage, including death, is abusive head trauma (AHT). The group that is most susceptible to AHT are children under the age of 1 year. In two studies, performed in Scotland and the United States, the incidence of severe and fatal AHT was respectively 24.6 and 30 infants per 100,000 children per year [
1]. There have been several studies in which parental shaking as a method of discipline were evaluated; in a Dutch study among parents of 3,259 infants, aged 1–6 months, 5.6% (95% CI 4.2–7.0%) of parents reported having smothered, slapped or shaken their child at least one time because of crying [
2]. An American study showed that shaking occurred in 2.6% of children under the age of 2 years [
3]. In this study, it was estimated that for every 1 child with severe injuries after AHT 150 children suffer AHT without clinical sequela. In a study on nearly 230,000 children in North Carolina it was shown that 29.7 (95% CI 22.9–36.7) children per 100,000 person years suffered from AHT [
1].
The terminology AHT as well as Abuse Head Trauma (AHD) are currently seen as a more appropriate compared to the widely used terminologies of the shaken baby syndrome, the whiplash shaken baby and shaken-impact syndrome. Although these terms all more or less indicate the same clinical entity, AHT and AHD cover all forms of inflicted brain injury irrespective of the underlying mechanisms.
If a child initially survives severe AHT the neurological outcome can be poor. In recent years several children were seen who after documented severe AHT developed multicystic encephalomalacia (MCE).
MCE is an increasingly rare but well documented finding in premature neonates that occurs almost exclusively in premature neonates and is not seen at an older age [
4]. Although initially believed to be a result from a cerebrovascular accident (CVA), it is becoming clearer that the development of brain injury in these children is more complex and consists of destructive and developmental disturbances [
5].
Discussion
Subdural hematomas (SDH) were present in all cases. The radiological signs, together with other information, such as clinical evaluation, parental history and fundoscopy, raised the suspicion of AHT. In view of the clinical evaluation we postulate that in our cases MCE was not caused by perinatal trauma but by AHT.
AHT, which includes both shaking only, shaking-impact and impact only trauma, is highly associated with SDH, but the findings are not pathognomonic [
7]. Severe trauma, e.g. a motor vehicle accident, can mimic the mechanism and clinical findings seen in AHT [
8]. Furthermore coexistent bleeding disorders might make an infant more vulnerable for developing SDH [
7]. Underlying diseases, e.g. Glutaric aciduria type 1, hase to be ruled out before diagnosing AHT [
9,
10].
As stated above, the shaking-impact mechanism in AHT is generally accepted as a cause of SDH. The acceleration-deceleration process of the brain during shaking, described by Caffey, causes rupturing of vulnerable veins, which are bridging between the two membranes [
11]. This causes bleeding into the subdural space. Intracranial hemorrhage is a space-occupying process, in severe cases which can lead to ischemic brain injury. Even in cases where the SDH is not severe enough to cause a space-occupying process ischemic brain injury can be seen, we therefore assume that this is caused by generalized edema of the brain [
12]. Ischemic brain injury can also be caused by cardiac or respiratory failure, this is mostly seen in patients after the perinatal period [
12]. It is suggested that hypoxic ischemic events can cause MCE in patients and that it is mostly seen in infants after severe asphyxia.
As shown above, five children were diagnosed with MCE from our database search from 2001. In all our cases AHT was proven and the absence of MCE on initial imaging indicates that MCE developed as a result of AHT.
MCE is mostly seen in infants after a perinatal period of severe asphyxia and this hypoxic-ischemic event is the generally accepted major cause of MCE. In general, hypoxic brain events have four major causes:
1.
Disturbed oxygen uptake in blood leading to oxygenation of tissue (including the brain). This can for example be seen in cases of carbon-monoxide poisoning.
2.
Disturbance of blood circulation to the brain as a result of a systemic disturbed blood flow. This can be seen in cases of hypovolemic shock.
3.
Disturbance of blood circulation to the brain as a result of compression of the vasculature of the head and neck. This can be seen in cases of strangulation.
4.
Diffuse brain edema leading to a rise in intracranial pressure leading to hypoperfusion of the brain. This can be seen in case of neurotrauma such as MVA and AHT.
MCE has a poor prognosis in neonates, especially when the neonate is persistently hypotonic after birth. Most children die or are severely handicapped [
13,
14].
Neonates with MCE due to perinatal trauma show several other changes, including alterations in the basal ganglia and a white cerebellar sign. In our cases, the same alterations were seen which raises the suspicion that they had a hypoxic-ischemic event not caused by perinatal trauma but by AHT.
Another sign seen in all of our children was retinal hemorrhage. Although there are several causes of retinal hemorrhages, it appears in 85% of the cases with AHT [
15]. In cases of AHT especially bilateral retinal bleeding are seen.
SDH and retinal hemorrhages raise high suspicions of AHT due to infant shaking. One of the most striking aspects of this kind of trauma is the absence of external visible signs of trauma. On radiographic examination, (old) rib fractures and metaphysical fractures of the long bones are strong indicators of inflicted injury [
16].
In conclusion, MCE after AHT is rare, but has been described [
17]. MCE is mostly seen after severe asphyxia during the perinatal period and is thought to be caused by a hypoxic-ischemic event that causes brain damage. After the perinatal period hypoxic-ischemic encephalomalacia is mostly seen after cardiopulmonary arrest [
18]. A hypoxic-ischemic event during AHT may also be the cause of MCE in our experience. In all cases AHT was related with SDH. This can cause an hypoxic-ischemic event, but it has to be severe. In our cases the hypoxic-ischemic events might have caused the development of MCE. Within a week after admission all children developed signs of MCE, as seen on MRI. At autopsy, the brain showed cystic changes in all cases. This confirmed the diagnosis of MCE after AHT.
Our cases were admitted to hospital and developed MCE due to hypoxic-ischemic event related to proven AHT. We have shown that MCE can develop in cases of AHT with a hypoxic-ischemic event from AHT provoking the pathological mechanism behind the development of MCE. It is not clear why some children develop MCE, while others do not.