Shaken baby syndrome

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Shaken baby syndrome
Classification and external resources
ICD-9 995.55
MedlinePlus 000004
eMedicine topic list

Shaken baby syndrome (SBS) is a triad of medical findings: subdural hematoma, retinal hemorrhage, and cerebral edema from which some physicians, consistent with current medical understanding, infer child abuse caused by intentional shaking. In a majority of cases there is no visible sign of external trauma.

SBS is often fatal and can cause severe brain damage, resulting in lifelong disability. Estimated death rates (mortality) among infants with SBS range from 15% to 38%; the median is 20%–25%. Up to half of deaths related to child abuse are reportedly due to shaken baby syndrome.[1][not in citation given] Nonfatal consequences of SBS include varying degrees of visual impairment (including blindness), motor impairment (e.g. cerebral palsy) and cognitive impairments.[citation needed]

Signs and symptoms[edit]

Subdural hematoma (arrow), bleeding between the dura mater and the brain, commonly occurs in SBS.

Characteristic injuries associated with SBS include retinal hemorrhages, multiple fractures of the long bones, and subdural hematomas (bleeding in the brain).[2] These signs have evolved through the years as the accepted and recognized signs of child abuse and the shaken baby syndrome. Medical professionals strongly suspect shaking as the cause of injuries when a baby or small child presents with retinal hemorrhage, fractures, soft tissue injuries or subdural hematoma, that cannot be explained by accidental trauma or other medical conditions. About three quarters of cases involve retinal hemorrhaging.[3] Additional effects of SBS are diffuse axonal injury, oxygen deprivation and swelling of the brain,[3] which can raise intracranial pressure and damage delicate brain tissue. A recent study found the prevalence of retinal hemorrhages in abusive head trauma was 85%, but only 5.3% in nonabusive pediatric head trauma.[4] Victims of SBS may display irritability, failure to thrive, alterations in eating patterns, lethargy, vomiting, seizures, bulging or tense fontanels (the soft spots on an infant's head), increased size of the head, altered breathing, and dilated pupils.[5]

Fractures of the vertebrae, long bones, and ribs may also be associated with SBS.[6] Dr. John Caffey reported in 1972 that metaphyseal avulsions (small fragments of bone had been torn off where the periosteum covering the bone and the cortical bone are tightly bound together) and "bones on both the proximal and distal sides of a single joint are affected, especially at the knee".[7]

Mechanism[edit]

Rotational injury is especially damaging and likely to occur in shaking trauma.[8] The type of injuries caused by shaking injury are usually not caused by falls and impacts from normal play, which are mostly linear forces.[8] It has been suggested that the mechanism of ocular abnormalities is related to vitreoretinal traction, with movement of the vitreous contributing to development of the characteristic retinal hemorrhages. These ocular findings correlate well with intracranial abnormalities.[9]

Diagnosis[edit]

Hydrocephalus, an accumulation of cerebrospinal fluid, can accompany SBS.[3] CT scanning is one technique used to diagnose the condition.

SBS may be misdiagnosed and underdiagnosed, and caregivers may lie or be unaware of the mechanism of injury.[10][not in citation given] Commonly, there are no externally visible signs of the condition,[10][not in citation given]. Examination by an experienced ophthalmologist is often critical in diagnosing shaken baby syndrome, as particular forms of ocular bleeding are quite characteristic.[11] Magnetic resonance imaging may also depict retinal hemorrhages;[12] this may occasionally be useful if an ophthalmologist examination is delayed or unavailable.[citation needed] No alternative condition mimics all of the symptoms of SBS exactly, but those that must be ruled out include hydrocephalus, sudden infant death syndrome (SIDS), seizure disorders, and infectious or congenital diseases like meningitis and metabolic disorders.[13][8] CT scanning and magnetic resonance imaging are used to diagnose the condition.[10][not in citation given] Conditions that may accompany SBS include bone fractures, injury to the cervical spine (in the neck), hemorrhaging of the retina (in the eye), cerebral hemorrhage or atrophy, hydrocephalus, and papilledema (swelling of the optic disc).[3]

Treatment[edit]

Treatment involves monitoring of intracranial pressure (the pressure within the skull), draining of fluid from the cerebral ventricles, and, if an intracranial hematoma is present, draining of the hematoma.[3]

Prognosis[edit]

Prognosis depends on severity and can range from total recovery to severe disability to death when the injury is severe.[3] One third of these patients die, one third survives with a major neurological condition, and only one third survives in good condition. The most frequent neurological impairments are learning disabilities, seizure disorders, speech disabilities, hydrocephalus, cerebral palsy, and visual disorders.[8]

Epidemiology[edit]

Small children are at particularly high risk for the abuse that causes SBS given the large difference in size between the small child and an adult.[10][not in citation given] SBS usually occurs in children under the age of two but may occur in those up to age five.[10][not in citation given]

Risk factors[edit]

Caregivers that are at risk for becoming abusive often have unrealistic expectations of the child and may display "role reversal", expecting the child to fulfill the needs of the caregiver.[10][not in citation given] Substance abuse and emotional stress, resulting for example from financial troubles, are other risk factors for aggression and impulsiveness in caregivers.[10][not in citation given] Both males and females can inflict SBS, but the abusers are more often male.[10][not in citation given] Although it had been previously speculated that SBS was an isolated event, a history of prior child abuse is a common finding in cases of SBS.[10][not in citation given] In an estimated 33–40% of cases, evidence of prior head injuries, such as old intracranial bleeds, is present.[10][not in citation given]

History[edit]

In 1946, the concept of SBS and the term "whiplash shaken infant syndrome" was introduced by Dr. John Caffey, a pediatric radiologist.[14] The term described a set of symptoms found with little or no external evidence of head trauma, including retinal hemorrhages and intracranial hemorrhages with subdural or subarachnoid bleeding or both.[7] In 1971, Dr. Norman Guthkelch proposed that whiplash injury caused subdural hemorrhage in infants by tearing the veins in the subdural space.[15] Development of computed tomography and magnetic resonance imaging techniques in the 1970s and 1980s advanced the ability to diagnose the syndrome.[10][not in citation given]

Concerns raised by SBS pioneer[edit]

Dr. Norman Guthkelch stated in 2011,"I don't think that the famous triad, however well some people think it's defined, can ever be so well-defined that you can say that and nothing else cause it — that meaning shaking." He is also concerned by the number of cases similar to one he reviewed in Arizona that he concluded that child likely died of natural causes not SBS stating, "I think I used the expression in my report, 'I wouldn't hang a cat on the evidence of shaking, as presented.' "[16] In 2012, Guthkelch went further, publishing an article "after 40 years of consideration," which is harshly critical of shaken baby prosecutions based solely on the triad of injuries.[17]

Legal issues[edit]

In July 2005, the Court of Appeals in the United Kingdom heard four appeals of SBS convictions: one case was dropped, the sentence was reduced for one, and two convictions were upheld.[18] The court found that the classic triad of retinal hemorrhage, subdural hematoma, and acute encephalopathy are not 100% diagnostic of SBS and that clinical history is also important. In the Court's ruling, they upheld the clinical concept of SBS but dismissed one case and reduced another from murder to manslaughter.[18] In their words: "Whilst a strong pointer to NAHI [non-accidental head injury] on its own we do not think it possible to find that it must automatically and necessarily lead to a diagnosis of NAHI. All the circumstances, including the clinical picture, must be taken into account."[19]

The court invalidated the "unified hypothesis", proposed by British physician J. F. Geddes and colleagues, as an alternative mechanism for the subdural and retinal hemorrhage found in suspected cases of SBS.[18] The unified hypothesis proposed that the hemorrhage was not caused by shearing of subdural and retinal veins but rather by cerebral hypoxia, increased intracranial pressure, and increased pressure in the brain's blood vessels.[18] The court reported that "the unified hypothesis [could] no longer be regarded as a credible or alternative cause of the triad of injuries": subdural haemorrhage, retinal bleeding and encephalopathy due to hypoxemia (low blood oxygen) found in suspected SBS.[18]

On January 31, 2008, the Wisconsin Court of Appeals granted Audrey A. Edmunds a new trial based on "competing credible medical opinions in determining whether there is a reasonable doubt as to Edmunds's guilt." Specifically, the appeals court found that "Edmunds presented evidence that was not discovered until after her conviction, in the form of expert medical testimony, that a significant and legitimate debate in the medical community has developed in the past ten years over whether infants can be fatally injured through shaking alone, whether an infant may suffer head trauma and yet experience a significant lucid interval prior to death, and whether other causes may mimic the symptoms traditionally viewed as indicating shaken baby or shaken impact syndrome."[20]

The terms non-accidental head injury or inflicted traumatic brain injury have been suggested instead of "SBS".[21]

Controversies and alternative hypotheses[edit]

Vitamin C deficiency[edit]

Some authors have suggested that certain cases of suspected shaken baby syndrome may result from vitamin C deficiency.[22][23][24][25] This contested hypothesis is based upon a speculated marginal, near scorbutic condition or lack of essential nutrient(s) repletion and a potential elevated histamine level. However symptoms consistent with increased histamine levels, such as low blood pressure and allergic symptoms, are not commonly associated with scurvy as clinically significant vitamin C deficiency. A literature review of this hypothesis in the journal Pediatrics International "From the available information in the literature, concluded that there was no convincing evidence to conclude that vitamin C deficiency can be considered to be a cause of shaken baby syndrome."[26]

The proponents of such hypotheses often question the adequacy of nutrient tissue levels, especially vitamin C,[27][28] for those children currently or recently ill, bacterial infections, those with higher individual requirements, those suffering from environmental challenges (e.g. allergies), and perhaps transient vaccination-related stresses.[29] Given that patients presenting with suspected SBS would constitute only the most severely affected group of children with nutritionally deficiencies, one would expect a larger number of individuals seen with milder symptoms. At the time of this writing, infantile scurvy in the United States is practically nonexistent.[30] No cases of scurvy mimicking SBS or Sudden Infant Death Syndrome have been reported, and scurvy typically occurs later in infancy, rarely causes death or intracranial bleeding, and is accompanied by other changes of the bones and skin and invariably an unusually deficient dietary history.[31][32]

Gestational problems[edit]

Gestational problems affecting both mother and fetus, the birthing process, prematurity and nutritional deficits can accelerate skeletal and hemorrhagic pathologies that can also mimic SBS, even before birth.[33][34][35][36] Because the age of suspected SBS victims is usually older than those patients suffering from birth-related problems, the distinction is usually not problematic.[citation needed]

Diffuse injury[edit]

A 2001 study reported that the predominant histological abnormality in cases of inflicted head injury in the very young is diffuse hypoxic brain damage, not diffuse axonal injury (DAI),[37] and suggested two possible explanations: either the unmyelinated axon of the immature cerebral hemispheres is relatively resistant to traumatic damage, or in shaking-type injuries the brain is not exposed to the forces necessary to produce DAI.[38]

Force[edit]

There has been controversy regarding the amount of force required to produce the brain damage seen in shaken baby syndrome. A biomechanical experiment in 2005 demonstrated that "forceful shaking can severely injure or kill an infant, this is because the cervical spine would be severely injured and not because subdural hematomas would be caused by high head rotational accelerations... an infant head subjected to the levels of rotational velocity and acceleration called for in the SBS literature, would experience forces on the infant neck far exceeding the limits for structural failure of the cervical spine. Furthermore, shaking cervical spine injury can occur at much lower levels of head velocity and acceleration than those reported for SBS."[39][40] A detailed explanation of the calculations was provided in a letter to the editor published in Forensic Science International in February 2006.[41]

The BBC has made a documentary in 3 parts that covers arguments for and against the diagnosis of SBS.[42][43][44]

See also[edit]

  • Baby Shaker - An application removed by Apple that allowed the user to shake their phone until an image of a cartoon baby on the screen died.
  • Charles Randal Smith - Canadian Pathologist who conducted flawed autopsies and whose expert testimony in Shaken Baby Syndrome cases resulted in several wrongful convictions.[45]
  • Louise Woodward case - English au pair convicted of involuntary manslaughter in the 1997 death of Matthew Eappen.
  • Shaken Adult Syndrome

Further reading[edit]

  • Journal Issue: Western Journal of Emergency Medicine, 12(2)

Title: "Challenging the Pathophysiologic Connection between Subdural Hematoma, Retinal Hemorrhage and Shaken Baby Syndrome" Publication Date:

2011 | Publication Info:[46] Western Journal of Emergency Medicine, Department of Emergency Medicine (UCI), UC Irvine

  • US National Library of Medicine, National Institutes of Health

Title: "The evidence base for shaken baby syndrome - We need to question the diagnostic criteria" [47]

External links[edit]

  • [2] The Next Innocence Project: Shaken Baby Syndrome and the Criminal Courts. Deborah Tuerkheimer. Washington University Law Review. Volume 87 Number 1,2009
  • National Child Abuse Defense & Resource Center.org - National Child Abuse Defense & Resource Center
  • [3] British Medical Journal 2004:328:719-720 (27 March)
  • [4] Imaging of the Central Nervous System in Suspected or Alleged Nonaccidental Injury, Including the Mimics.Barnes, Patrick D. MD; Krasnokutsky, Michael MD. Topics in Magnetic Resonance Imaging: February 2007 - Volume 18 - Issue 1 - pp 53–74
  • National Center on Shaken Baby Syndrome National Center on Shaken Baby Syndrome (NCSBS)
  • [5] Shaken Baby Syndrome: A Questionable Scientific Syndrome and a Dangerous Legal Concept. Utah Law Review
  • [6] Madison magazine. article: Oh baby by Melanie Radzicki McManus
  • [7] State's motion to dismiss prosecution in shaken baby syndrome case.
  • Keller KA, Barnes PD (November 2008). "Rickets vs. abuse: a national and international epidemic". Pediatric Radiology 38 (11): 1210–6. doi:10.1007/s00247-008-1001-z. PMID 18810424. 
  • [8] Centre for Forensic Science and Medicine SeminarChild Abuse - Nonaccidental Injury (NAI) presenter(s) Patrick D. Barnes Power Point Presentation. Lecture notes and references.
  • [9] Wrongful Diagnosis of Child Abuse - A Master Theory. Journal of the Royal Society of Medicine. VOLUME 98, NUMBER 6, 2005
  • [10] Diagnosis Murder CBC documentary investigates the topic and re-examines the crucial question: Is Shaken Baby Syndrome conclusive evidence of murder or is it what it is an unproven scientific hypothesis believed to be a scientific fact, 2012

Footnotes[edit]

  1. ^ Montelenone JA, Brodeur AE. (1994). Child Maltreatment: A Clinical Guide and Reference. St Louis: GW Medical Publishing. [page needed]
  2. ^ "NINDS Shaken Baby Syndrome information page". National Institute of Neurological Disorders and Stroke. 2014-02-14. 
  3. ^ a b c d e f "Shaken Baby Syndrome". Journal of Forensic Nursing. Retrieved 2011-04-27. 
  4. ^ Medscape
  5. ^ Types of brain injury: Shaken baby syndrome. Brain Injury Association of America. Retrieved on September 24, 2007.
  6. ^ Kempe CH, Silverman FN, Steele BF, Droegemueller W, Silver HK (July 1962). "The battered-child syndrome". JAMA 181: 17–24. doi:10.1001/jama.1962.03050270019004. PMID 14455086. 
  7. ^ a b Caffey J (August 1972). "On the theory and practice of shaking infants. Its potential residual effects of permanent brain damage and mental retardation". American Journal of Diseases of Children 124 (2): 161–9. doi:10.1001/archpedi.1972.02110140011001. PMID 4559532. 
  8. ^ a b c d Oral R (August 2003). "Intentional head trauma in infants: Shaken baby syndrome" (Archived). Virtual Children's Hospital. Archived from the original on 2005-02-14. Retrieved 2006-10-09. 
  9. ^ [1] Post Mortem Findings
  10. ^ a b c d e f g h i j k American Academy of Pediatrics: Committee on Child Abuse and Neglect (July 2001). "Shaken baby syndrome: rotational cranial injuries-technical report". Pediatrics 108 (1): 206–10. doi:10.1542/peds.108.1.206. PMID 11433079. 
  11. ^ "Shaken Baby Syndrome Resources". American Academy of Ophthalmology. 
  12. ^ http://www.ncbi.nlm.nih.gov/pubmed/23568702
  13. ^ Togioka BM, Arnold MA, Bathurst MA et al. (2009). "Retinal hemorrhages and shaken baby syndrome: an evidence-based review". J Emerg Med 37: 98–106. doi:10.1016/j.jemermed.2008.06.022. 
  14. ^ http://pediatrics.aappublications.org/cgi/content/abstract/54/4/396
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  17. ^ http://globalwrong.files.wordpress.com/2013/01/guthkelch-an-preface-to-narang-hous-j-health-law-poly-2012.pdf
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  23. ^ Johnston, C.S. (1996). "Chapter 10) The Antihistamine Action of Ascorbic Acid". Ascorbic Acid; Biochemistry and Biomedical Cell Biology 25. Plenum Press. p. 189. ISBN 978-0-306-45148-5. 
  24. ^ Majno G, Palade GE, Schoefl GI (December 1961). "STUDIES ON INFLAMMATION : II. The Site of Action of Histamine and Serotonin along the Vascular Tree: A Topographic Study". The Journal of Biophysical and Biochemical Cytology 11 (3): 607–26. doi:10.1083/jcb.11.3.607. PMC 2225127. PMID 14468625. 
  25. ^ Gore I, Fujinami T, Shirahama T (October 1965). "Endothelial changes produced by ascorbic acid deficiency in guinea pigs". Archives of Pathology 80 (4): 371–6. PMID 5319838. 
  26. ^ Fung EL, Nelson EA (December 2004). "Could Vitamin C deficiency have a role in shaken baby syndrome?". Pediatrics International 46 (6): 753–5. doi:10.1111/j.1442-200x.2004.01977.x. PMID 15660885. 
  27. ^ Dettman G (March 1978). "Factor "X", sub-clinical scurvy and S.I.D.S. Historical. Part 1". The Australasian Nurses Journal 7 (7): 2–5. PMID 418769. 
  28. ^ Kalokerinos A, Dettman G (July 1976). "Sudden death in infancy syndrome in Western Australia". The Medical Journal of Australia 2 (1): 31–2. PMID 979792. 
  29. ^ Institute of Medicine (IOM) (1991). "Chapter 6) Evidence Concerning Pertussis Vaccines and Other Illnesses and Conditions -- Protracted Inconsolable Crying and Screaming". Adverse Effects of Pertussis and Rubella Vaccines. The National Academies Press. p. 165. ISBN 0-309-04499-5. 
  30. ^ Lee RV (1983). "Scurvy: a contemporary historical perspective". Connecticut Medicine 47 (10): 629–32, 703–4. PMID 6354581. 
  31. ^ Weinstein M, Babyn Phil, Zlotkin S (2001). "An Orange a Day Keeps the Doctor Away: Scurvy in the Year 2000". Pediatrics 108 (3): e55. doi:10.1542/peds.108.3.e55. PMID 11533373. 
  32. ^ Rajakumar K (2001). "Infantile Scurvy: A Historical Perspective". Pediatrics 108 (4): e76. doi:10.1542/peds.108.4.e76. PMID 11581484. 
  33. ^ Cushing H, Goodrich JT (August 2000). "Reprint of "Concerning Surgical Intervention for the Intracranial Hemorrhages of the New-born" by Harvey Cushing, M.D. 1905". Child's Nervous System 16 (8): 484–92. doi:10.1007/s003810000255. PMID 11007498. 
  34. ^ Williams Obstetrics (1997). "Chapter 20". Diseases and Injuries of the Fetus and Newborn 20. Appleton & Lange, Stamford, CT. pp. 997–998. ISBN 0-8385-9638-X. 
  35. ^ Williams Obstetrics (2005). "Chapter 29". Diseases and Injuries of the Fetus and Newborn 22. McGraw-Hill Companies. pp. 649–691. ISBN 0-07-141315-4. 
  36. ^ Looney CB, Smith JK, Merck LH et al. (February 2007). "Intracranial hemorrhage in asymptomatic neonates: prevalence on MR images and relationship to obstetric and neonatal risk factors". Radiology 242 (2): 535–41. doi:10.1148/radiol.2422060133. PMID 17179400. 
  37. ^ Geddes JF, Hackshaw AK, Vowles GH, Nickols CD, Whitwell HL (July 2001). "Neuropathology of inflicted head injury in children. I. Patterns of brain damage". Brain 124 (7): 1290–8. doi:10.1093/brain/124.7.1290. PMID 11408324. 
  38. ^ Geddes JF, Vowles GH, Hackshaw AK, Nickols CD, Scott IS, Whitwell HL (July 2001). "Neuropathology of inflicted head injury in children. II. Microscopic brain injury in infants". Brain 124 (7): 1299–306. doi:10.1093/brain/124.7.1299. PMID 11408325. 
  39. ^ Margulies S, Prange M, Myers BS et al. (December 2006). "Shaken baby syndrome: a flawed biomechanical analysis". Forensic Science International 164 (2–3): 278–9; author reply 282–3. doi:10.1016/j.forsciint.2005.12.018. PMID 16436323. 
  40. ^ Rangarajan N, Shams T (December 2006). "Re: shaken baby syndrome: a biomechanics analysis of injury mechanisms". Forensic Science International 164 (2–3): 280–1; author reply 282–3. doi:10.1016/j.forsciint.2005.12.017. PMID 16497461. 
  41. ^ Bandak F (December 2006). "Response to the Letter to the Editor". Forensic Science International 157 (1): 282–3. doi:10.1016/j.forsciint.2006.01.001.  which refers to
    Margulies S, Prange M, Myers BS et al. (December 2006). "Shaken baby syndrome: a flawed biomechanical analysis". Forensic Science International 164 (2–3): 278–9; author reply 282–3. doi:10.1016/j.forsciint.2005.12.018. PMID 16436323. 
  42. ^ "Panorama Shaken Babies (BBC 1) (Part 1 of 3)". Panorama. 10 March 2008. BBC. http://news.bbc.co.uk/2/hi/programmes/panorama/7312438.stm. Retrieved 2010-01-04.
  43. ^ "Panorama Shaken Babies (BBC 1) (Part 2 of 3)". Panorama. BBC.
  44. ^ "Panorama Shaken Babies (BBC 1) (Part 3 of 3)". Panorama. BBC.
  45. ^ http://www.nationalpost.com/news/Medical+panel+condemns+disgraced+pathologist+abominable+failures/4503500/story.html
  46. ^ http://escholarship.org/uc/uciem_westjem?volume=12;issue=2
  47. ^ Geddes JF, Plunkett J (March 2004). "The evidence base for shaken baby syndrome". BMJ 328 (7442): 719–20. doi:10.1136/bmj.328.7442.719. PMC 381308. PMID 15044267.