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Home , Shaken baby syndrome

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 2Iasi 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 (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 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 , shaking trauma suffered by the child. Nonspecific impact syndrome, or shaken child syndrome symptoms like , 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

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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, , the child, all parents were shown in the , apoplexy attack, , 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 , 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 . A characteristic non-impact traumatic association, produced of the shaken child's syndrome is the absence by an indirect 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 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 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 , 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

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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 - 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.

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- 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 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 , 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 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

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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

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- 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

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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 , 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

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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 The mechanism of producing cranio- Anton Knieling [email protected] cerebral lesions was most likely a hyperflexion followed by head hyperextension due to References trauma to the neck that interested the left 1. Caffey J. On the theory and practice of shaking infants. carotid artery; clarification of the mechanism Its potential resitual effects of permanent brain damage was done with neurosurgeon specialists. and mental retardation. Am J Forensic Med Pathology. Neurosurgeon specialists have 1972;22:112-122. hypothesized about the WHIPLASH INJURY 2. Salehi-Had H, Brandt JD, Rosas AJ, Rogers KK. cranio-cerebral lesion mechanism, which can Findings in older children with abusive head injury: does shaken-child syndrome exist. Pedriatics Journal. explain brain contusion lesions associated 2006;117:1039-1044. with cerebral infarction lesions as a result of 3. Keean H et al. A Population-Based Study of Inflicted repeated traumatic lesions of the left carotid Children. JAMA, The Journal of artery at the cervical level. the American Medical Association. Vol. 290. 2006;290(5):621-626.

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4. Dias M et al. Preventing Abusive Head Trauma Among 7. Plăhteanu M, Baciu Gh, Pădure A. Aspecte medico- Infants and Young Children: A Hospital – Based, Parent legale lezionale în traumatologia mecanică. Editura Education Program. : Official Journal of the Performantica. Iaşi. 2004, p.164. American Academy of Pediatrics. 2005;115(4):470-477/ 8. Ciurea AV, Davidescu HB: Traumatologie cranio- 5. Ioan BG. Tanatologie medico-legală – note de curs. cerebrală. Editura Universitară „Carol Davila”. Bucureşti. Editura Junimea. Iaşi. 2007, p 55-56. 2007, p. 287. 6. Grigoriu C. Repere în patologia medico-legală. Editura 9. Bulgaru-Iliescu D. Anomia microsocială – forme şi Junimea. Iaşi. 2007, p.135. consecinţe. Editura Timpul. 2002, p 117-118.