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Romanian Neurosurgery (2018) XXXII 3: 409 - 417 | 409

DOI: 10.2478/romneu-2018-0052

Shaken baby syndrome. Case report

Tatiana Iov2, Sofia David1, Simona Damian1, A. Knieling1,


Mădălina Maria Diac1, D. Tabian2, Diana Bulgaru-Iliescu1
1
“Grigore T. Popa” University of Medicine and Pharmacy of Iași, Iasi Forensic Institute
2
Iasi Forensic Institute, ROMANIA

Abstract: Recognition of abuse and the treatment of child victims are recent concepts in
the history of mankind. Increasing the awareness of the need to treat and prevent such
abuse is a characteristic of modern society. The beaten child syndrome was described by
Ambroise Tardieu in 1860, and Shaken Baby Syndrome (SBS) was clearly illustrated in
medical literature a century later by Caffey in 1972. The definition of SBS is based on the
association of major intracranial lesions with minimal external lesions and the diagnosis
is still difficult to establish. The authors describe a reduced number of 7 cases of pediatric
patients addressed for forensic expertise and where suspicion of SBS has arisen. The
lesion mechanisms involved in the production of this syndrome are still controversial
and are sources of frequent debates in legal medicine. These uncertainties can make legal
punishment inoperable. The therapeutic management of these children in neurosurgery
is not subject to international consensus, and discrepancies between different clinics
impede a comparative cohort assessment. However, SBS is a major public health problem
due to severe neurological injuries caused to child victims during brain development.
Key words: SBS, cerebral injuries

Literature review examination of the eyes and bones can, in most


The symptoms of the Shaken Baby cases, identify the Shaken Baby Syndrome.
Syndrome are difficult to identify, and the The Shaken Baby Syndrome (SBS) is also
family usually avoid describing the cranial called head trauma due to child abuse, shaking
trauma suffered by the child. Nonspecific impact syndrome, or shaken child syndrome
symptoms like vomiting, crying and irritability (8).
are often confused with gastric or intestinal The syndrome of the shaken child was
disorders. Violent shaking is often repeated, defined by Dr. Caffey, a radiologist, in 1972 (1)
but the one with the highest intensity is the one as a form of physical abuse to a 1-2 years old
that causes the death. The macroscopic and child. The most at risk are babies aged between
microscopic examination of the brain, the 0 months and 2 years. The head of a baby is
 
 
 
 
 
 
410 | Iov et al - Shaken baby syndrome

relatively heavy compared to his body, and the West of New York has reduced the baby's
neck muscles are so weak that they cannot syndrome rate by 55% and it is currently
hold the baby's head very well (7). Shaking the known as a national model. After the birth of
baby can cause: blindness, brain damage, the child, all parents were shown in the
cerebral palsy, apoplexy attack, epilepsy, loss hospital a video recording about SBS
of hearing, learning difficulties, and later prevention that includes simple information
behavioral problems (9). on how to act if the children cry and then they
Does this syndrome occur only in children were asked to sign a commitment. This project
aged 1-2 years? No, in the United States, such has been replicated in several countries due to
cases have been reported in children up to 7 its resounding success. New York state’s laws
years old (2) and each year, about 1300 now require all hospitals to provide this
children are seriously injured or die from program to all new parents. In recent years,
cranial trauma due shaking. (3) some US states have passed a series of laws on
Shaken child syndrome is a major cause of the prevention of SBS (4).
death, accounting for about 25-30% of all SBS
children. Around 60-75% of the surviving Etiology
children have mild or severe retardation, SBS is produced by vigorously shaking the
paralysis, including tetraplegia, cerebral child's body through his arms, legs, chest or
paralysis, convulsive disorders and blindness. shoulders. The child's head will thus perform
The less serious cases of SBS are difficult to repeated flexion-extension movements. It is a
diagnose, especially in infants. A characteristic non-impact traumatic association, produced
of the shaken child's syndrome is the absence by an indirect whiplash mechanism, with
of external traumatic signs, but the irritability, repeated and accelerated decelerations, with
the slowed down rhythm of the growth, the snap back and forth head movements. The
slowing down of the deglutition reflex, most dangerous are the rotational movements,
lethargy, vomiting, depression, increase of which cause uneven and asymmetrical
head size, intracranial hypertension, changes landslides of the cerebral hemispheres relative
in the respiratory system and dilated pupils are to each other in relation to the skull. However,
some of the signs. Other associated lesions are there are authors who do not recognize the
retinal bleeding, long bone fractures and veracity of this mechanism and who consider
subdual hematomas. (4, 5) Brain injuries of a that such injuries cannot be produced by
traumatic nature, in children, without an simply shaking the baby’s body, in the absence
external head injury, strongly advocate the of an even minor impact between the cephalic
shaking mechanism. (2, 6) extremity and a hard body/ surface. Obviously,
In California, almost all SBS victims who there is also SBS with head impact, but in these
survive are transferred to maternal care after cases, the lesions are somewhat specific to
diagnosis. In the United States, there is also a classical mechanical trauma mechanisms.
child-shatter prevention project that in the Nevertheless, most of the literature in this
 
 
 
 
 
 
Romanian Neurosurgery (2018) XXXII 3: 409 - 417 | 411

domain recognizes and supports the existence holding. Under the conditions of a usual grip
of pure SBS without impact. The high of the aggressor's hands on the child's chest, we
sensitivity of children under 1 year old to such may find symmetrical lesions, an oval
movements is due to several factors: ecchymosis on each of hemithorax region,
- the relatively large dimensions and mass anterior (by compression with the thumb) and
of the cephalic extremity comparing to the rest multiple ecchymosis on the posterior thorax,
of the body; paravertebral (compression with the other
- the immaturity of the neck muscles, fingers). However, we should not forget that
which cannot yet support the baby's head, SBS can also be caused by shaking of the upper
allowing broad movements, which are further or lower limbs or shoulders, and can therefore
amplified by inertia, and without defense find mild contusion (ecchymosis) at different
reflections to counteract the amplitude of the levels. It is also possible to find external lesions
movements through muscle contractions; of different age.
- the space between the brain and the dura The internal exam detects some typical
mater is large, allowing the brain to slip more lesions, which grouped, define SBS:
freely in relation to the bone and meningeal - subdural hematoma is found in over 90%
structures; of cases. It occurs by rupture of the bridging
- high water content of the child's brain veins as a result of the violent shaking, causing
compared to an adult’s one; the head to snap back and forth on the neck.
- incomplete myelination of the brain Bilateral and interhemispheric collections are
nerve substance. frequently found. In many cases the volume of
the hematoma is not very high, in contrast to
Pathophysiology and morphopathology the patient's severe clinical condition, which
Because of shaking the baby's body, makes us to believe that in SBS the subdural
considering the morphological characteristics hematoma would not be a cause of death.
of the small child and the rigidity of Although Caffey described chronically
intracranial fibrous structures (dura mater, subdural hematoma as typical, modern
falx cerebri, tentorium cerebelli), and the literature reports the highest frequency for the
relatively fixed structures (cranial nerves, occurrence of acute bleeding. Sometimes
carotid blood vessels, or vertebrobasilar subdual hematomas of different age may
system) the brain will move inside the skull, coexist, indicating repeated trauma. Geddes
with the result of characteristic lesions. mentions the appearance of the intradural
External findings are very poor, except hemorrhage as an effect of bridging vein
when other forms of physical abuse are rupture in SBS. In many cases, the subdural
associated to SBS. However, it is possible to hematoma is accompanied by subarachnoid
identify ecchymotic patches of the fingers of hemorrhages, with elective locations on the
the aggressor on the victim's anterior and convexities of the cerebral hemispheres and
posterior chest, in the positions of hand their internal faces.
 
 
 
 
 
 
412 | Iov et al - Shaken baby syndrome

- retinal hemorrhages are present in 85- optic nerves and axonal ruptures. Probably
100% of cases. Pre-retinal, intraretinally, and these lesions are common in SBS, being the
retroretinin hemorrhages, often confusing, are cause of evolution with optic nerve atrophy in
the most common; vitreous hemorrhages are many cases where victims survive, the
rarely encountered (10%). Retinal detachment necroptic exam of a case revealed bilateral
can appear. In many cases (81-85% of cases retro-ocular hemorrhages, but they may have
with retinal hemorrhages), the lesions are been produced by local extension from the
bilateral. It has not been established a relation optic nerve sheaths presenting bleeding
between the location of retinal hemorrhages sleeves.
(right/left) and subdual hematomas. To - parenchymal brain injury. Cerebral
explain these injuries, several theories have contusion appears quite rare due to the
been issued: morphological immaturity of the brain
- optical nerves shearing by the brain that structures, with the lack of myelinization.
slips inside the skull, for this mechanism Contusive hemorrhagic lesions can occur in
advocating the relatively frequent findings of the corpus callosum due to its torsion during
concentric retinal hemorrhages around the movements with the angular accelerator
papilla of optic nerve; component, the basal faces of the frontal and
- hypertension in the retinal venous system temporal lobes, by contact with the rumpus
as a result of cerebral edema, but this is rather surface of the skull base and the upper
the pathophysiological explanation of Terson cerebellum pedunculum by striking the
syndrome; tentorium cerebelli. Meningo-cerebral lesions
- vascular mechanism: Transient stop of are usually accompanied by moderate brain
blood flow due to compression of the vascular edema. Diffuse axonal rupture is a severe and
carotid-ophthalmic system during head typical lesion for SBS, possibly involved more
shaking, or retinal vasospasm; than the subdural hematoma in the infaust
- Retinal hemorrhages as a result of inertial prognosis of the syndrome. These occur as a
movements of the vitreous body during result of the rotating movements of the head,
acceleration / deceleration cycles - Mechanism which create shear forces. Axonal ruptures are
revealed in the small child due to its high largely responsible for the clinical condition of
vitreous consistency and strong adhesion to the victim, with varying levels of mental status,
the vitreoretinal surface; and often even by the patient's death. As a
- vascular ruptures due to the slippage of technique for identifying these lesions in the
the retinal layers and their disruption; histopathology laboratory, 68-kDa
- acute thoracic compression with Valsalva neurofilament immunoprecipitation and beta
effect and retinal hemorrhage due to increased amyloid precursor protein (beta-APP) have
intracerebral venous pressure. already been established, thus visualizing
In the ocular sphere, in the fatal cases, there axonal amputation bulbs in the form of round
are necroptic findings of bleeding around the or oval acidophilus masses. The technique can
 
 
 
 
 
 
Romanian Neurosurgery (2018) XXXII 3: 409 - 417 | 413

also be used to find axonal lesions in the optic bottom of the eye. It must, therefore, enter a
nerves. mandatory protocol to recommend an
- Medullary lesions: subdural hematomas ophthalmologic examination to a child under
and high cervical medullary contusions are 1 year who presents a subdural hematoma,
reported due to shaking of the victim's head. even in the absence of external signs of
Axonal lesions were found. Periadventitial violence. A morphometric analysis of the
hemorrhage in the vertebral artery between C1 retinal bleeding is also useful, as there has been
and C4 was reported by Gleckman in a SBS observed a correlation between the retinal
case. There are injuries that occurs through an hemorrhage and the reality of SBS and visual
indirect mechanism of gripping: rib fractures prognosis.
with typical paravertebral localization,
scapular fractures, vertebral spine apophyses Paraclinical diagnosis
and long bone fractures in exceptional The main investigations that contribute to
situations. the diagnosis of SBS are: Cranio-cerebral
computer tomography (CT), nuclear magnetic
Clinical diagnosis resonance imaging (MRI), high-resolution
Upon reaching the hospital, patients with cranial ultrasound, thoracic radiography (for
SBS typically have nonspecific symptoms, the diagnosis of possible costal fractures),
varying and sometimes progressive degrees of laboratory blood tests (for the differential
consciousness, and respiratory disturbances. diagnosis of hemorrhagic lesions) and lumbar
The Glasgow Coma Scale (GCS) score is one of puncture.
the most important indicators of the severity
of the traumatic brain involvement in SBS. Forensic aspects
Data on the average Glasgow score in patients In case of the survival of the victim, the
with SBS at the time of the arrival at the forensic physician will have to interpret the
emergency unit varies depending on the medical documentation prepared by other
author, in most cases ranging between 9-12 specialists (pediatricians, neurosurgeons,
GCS. Thus, there is a correlation between the ophthalmologists) and in collaboration with
severity of the GCS and prognosis. them, to make a synthesis and establish the
Gillilanda has made a definition of SBS diagnosis of SBS. It should be kept in mind:
based on a clinical diagnostic criterion: - The presence of lesions considered as
echimotic compression marks with fingers specific to the syndrome (subdural hematoma,
and/or ribs fractures, subdural and/or retinal hemorrhage);
leptomeningeal hemorrhage and a history of - The exclusion of other violent or non-
vigorous shaking. According to this author, if violent causes responsible for causing such
at least two criteria are met, the diagnosis of injuries;
SBS can be concluded. For ocular lesions, the
diagnosis is primarily based on an exam of the
 
 
 
 
 
 
414 | Iov et al - Shaken baby syndrome

- The exclusion of traumatic head injury, - The microscopic examination is


following a very careful external examination; mandatory to confirm the macroscopically-
- A history of violent shaking of the child's observed meninges, cerebral and ocular
body. lesions, to diagnose the microscopic traumatic
- The time of medical care for healing of lesions, to attest their vital lesion character and
SBS is around 45-50 days but, in some cases, it to assess its age.
may exceed 60 days, endangering the life of the - fragments of cerebral substance from all
victim through the existence of the subdural areas with macroscopic lesion will be drawn.
hematoma. For the immunohistochemistry, samples from
- The legal framing of the deed is also given the white substance of both cerebral
by the intentional element, as, in most of cases hemispheres, cerebellum, cerebral trunk,
the perpetrator did not wish to produce these marrow, optic nerves, will be collected.
consequences. Sometimes he/she did not even Usually, staining with hematoxylin-eosin is
know that shaking the child with the intention used, but Perls staining is also useful in
to make him stop crying can cause such assessing the age of a hemorrhagic lesion
serious injuries. because it highlights hemosiderin.
- In the case of healing with sequelae, the - The microscopic examination of the
forensic physician will appreciate the existence cerebral and medullary substance, and of the
of a physical or mental disability, the loss of a cranial nerves after immune marker for the
function (visual, auditory) and the level of beta-amyloid precursor protein (beta-APP) or
functional deficit created because of the for neurofilament (68-kDa neurofilament),
trauma. positive techniques for axonal ruptures, but
In cases of death of the victim, the forensic still hardly accessible in the forensic services in
physician will not see, during an external our country.
examination, any signs or lesion due to - The eyeballs will be examined after
violence, but he will discover serious internal fixation in formalin and sagittal sections will
lesions. As a rule, in all cases where a subdural be practiced, one of them continuing with
hematoma is identified in a baby child, it is longitudinal sectioning of the optic nerve.
mandatory to examine and collect the eyeballs
(through the endocranial orbital approach) for Case report
microscopic examination. It is recommended The emergency sheet revealed that the
to examine the brain after putting it in 10% child was hospitalized for 35 minutes. At the
formalin for 3 weeks, to better identify the hospital admission the child was having first
traumatic brain injuries. The examination of degree coma, left eye = right eye, fixed
the fresh sample and its sectioning are difficult mydriasis, corneal and deglutition reflexes
and predisposes to artifacts due to the low were present, free airways, spontaneous
consistency of the brain in children of this age. breathing, physiological vesicular murmur,
absent rales, cardiac sinusal rhythm, 80 beats
 
 
 
 
 
 
Romanian Neurosurgery (2018) XXXII 3: 409 - 417 | 415

per minute heart rate. The craniocerebral CT repeatedly applied "physical corrections" to
scan revealed a parenchymatous hypodensity him, striking him with the hands or hitting
extended on the entire left hemisphere and him with a strap. The last correction was
also on the right frontal lobe paramedian on applied by taking him to his shoulders and
the right anterior carotid artery’s territory, shaking him, then applying several strokes
significant collapse of the left lateral ventricle, with her hand over baby’s head and body, then
subfalcine engagement of the median line hit him with a rubber hose (for household use).
structures, bilateral transtentorial Immediately after this correction, the minor
engagement. felt dizziness, lethargic, unable to keep his
A copy of the consultation sheet showed balance, reason why her grandmother sit him
that the child was hospitalized for 17 hours in the bed. Six hours after the "correction", the
and 44 minutes with the diagnosis of” Massive child, who was sitting on the bed, fell aside, fed
cerebral infarct in the territory of the medium an insufficiently, drank water, and then fell
carotid artery, posterior carotid artery, left asleep. Two days after the "correction", the
anterior carotid artery and right anterior condition of the child got worse and the
carotid artery”. The patient’s reason for ambulance was announced.
presentation: third degree coma, bilateral fixed The body belongs to a male child, 89 cm in
mydriasis. The medical history showed that height, age-appropriate constitution, 2 years
the child developed consciousness disorders and 10 months old, with a known identity.
apparently after a trauma through aggression. Macroscopically examination revealed
Neurological examination: without active multiple scratches: on the right frontal region,
movements, areflexia (hyporeflexia), reactive left eye internal and external angle,
exclusively to pain. Local examination: right mandibular- left side, left laterocervical region;
frontal localized excoriations at the internal multiple ecchymosis with localization on the
and external angle of the left eye and at the left forearm and left hand, at the buttocks, thighs
auricular concha; right laterocervical localized and calves. Internal examination findings
ecchymosis at thighs and buttocks bilateral. were: left hemisphere subdural hematoma 0.4
From the epicrisis of the observation sheet it cm thick; cerebral edema; contusion and
turned out that the evolution was unfavorable cerebral dilaceration at the base of left cerebral
with the installation of a non-reversible hemisphere.
cardiorespiratory arrest at the resuscitation The anatomo-pathological microscopic
maneuvers and exitus. examination establishes the following: focal
Necroptic exam revealed: inflammatory infiltration in leptomeninges,
There was no investigation data at the time diffuse cerebral contusion, important edema
of the necropsy. After a through investigation in perivascular and pericelular spaces.
of social data it appears that the minor was in Due to the absence of high-intensity
the care of his grandmother with his other external cranial marks and the presence of
brothers. It also appears that the grandmother traumatic brain injuries, in the provisional
 
 
 
 
 
 
416 | Iov et al - Shaken baby syndrome

conclusions, the mechanism of production Because this type of trauma is produced as


was established as a mechanism for shattering. a result of lack of education in this respect, we
Later on, the mechanism for brain lesions appreciate that prevention measures are of
(the subdural hematoma, the contusion and paramount importance, with physicians
the cerebral dilaceration) was issued: it was taking the lead in these actions. Consequently,
possible to produce it most probably by even in maternity, doctors and nurses need to
vibrating cerebral substance in the sudden inform the parents of the little boy barely come
mobilization of the child’s body and head by into the world about the adverse consequences
another person, but it cannot be excluded of shaking the child. Massive campaigns over
repeatedly active blows to the head with or by time in civilized countries, supported by the
blunt objects, if the investigation demonstrates media, had the motto: "never shake the child!”.
this mechanism. Guidelines for parents are simple messages
The grandmother was assessed by forensic that aim to summarize the attitude to adopt in
experts and psychiatrists. At the examination difficult times.
of the abuser it was concluded that the Because this type of trauma is produced as
grandmother suffers from major depressive a result of lack of education in this respect, we
disorder with anxious phobic elements and appreciate that prevention measures have
preinvolutive background with reactive onset. importance, doctors has the main role in these
The grandmother’s behavior creates the actions. Therefore, even in maternity, doctors
constitutive elements of the first degree and nurses have to inform the parents of the
murder and has been committed with little boy barely come into the world about the
discernment. adverse consequences of shaking baby.

Conclusions Correspondence
Anton Knieling
The mechanism of producing cranio-
tony_knieling@yahoo.com
cerebral lesions was most likely a hyperflexion
followed by head hyperextension due to
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