PERCEIVED SHAKEN BABY SYNDROME -BY A COLLEAGUE

[Alison J Stevens]

Inflicted Brain Injuries: Don’t Discard Differential Diagnosis

Michael D. Innis, M.B.B.S.

The great enemy of the truth is very often not the lie: deliberate, contrived and dishonest; but the myth: persistent, persuasive, and unrealistic.

--John F. Kennedy

There are, in fact, two things: science and opinion. The former brings knowledge, the latter ignorance.

--Hippocrates of Cos

The concept of shaken baby syndrome is an unfortunate example of a theory being adopted by consensus rather than being supported by science and clinical observation.

The proposed causative mechanism, shaking, is often contaminated by incidents involving actual head trauma. Flaws in the biomechanical theory underlying the concept, [1] and flaws
in the “confessional” literature used to support the concept have been reviewed
by others. [2]

In recent years, the concept of shaken baby syndrome has taken on increasingly pejorative labels, such as “abusive head injury,” and now “inflicted brain injury.” One group of authors,
Maguire et al.,[3] claim that their systematic review, the largest of its kind,
offers for the first time a valid “statistical probability” of inflicted brain
injury when certain key features are present.

One of the “key features” upon which Maguire et al. base their opinion, retinal hemorrhages, has long been known to be associated with raised intracranial pressure from any cause, [4] as
in Terson’s syndrome [5] and following vitamin C or vitamin K deficiency [6-10].
Relying solely on this “key feature” can have disastrous consequences for the child’s
caregivers.

Under these circumstances, inappropriate accusations of child abuse could be appropriately avoided by doing the recognized, accepted, and pertinent laboratory tests for deficiency
of vitamins C or K. [6, 9] It is likewise pertinent to ask in how many cases, in
the “largest review of its kind,” was the modified prothrombin time known as
the PIVKA test (proteins induced by vitamin K antagonism or absence) performed?
And, how often was serum level of vitamin C estimated?

In light of what is now known about the effects of nutritional deficiencies, the diagnosis of inflicted brain injury should not be accepted unless pertinent nutritional disorders have
specifically been excluded.

In a recent case, the Dublin city coroner, ignoring the opinions of specialists involved in the case, recorded the cause of death in an infant as “natural causes,” saying: “there is no
evidence of cerebral trauma or ‘shaken baby syndrome,’ despite the radiological
and clinical findings of subdural hemorrhage and retinal hemorrhages.” [11]

Despite pronouncements about “rotational cranial injuries” in shaken baby syndrome, [12] these conclusions are based on opinion and consensus, not science.

Apnea is also rated high on their list of statistical markers of inflicted brain injury, and Maguire et al. [3] claim that it is a distinguishing feature. As evidence for this opinion,
they cite 2003 article by one of their group, A.M. Kemp [13] and an article by
Geddes et al., [14] in which it is assumed, without proof, that the injuries
associated with apnea were inflicted. Kemp et al. conclude that “at this point
in time we do not know the minimum forces necessary to cause NAHI
[non-accidental head injury].” [3]

These authors disregard the fact that apnea is a feature of the condition known as an apparent life threatening event (ALTE)] which can be caused by prematurity, gastroesophageal reflux, cardiac arrhthymia,
laryngomalacia, tracheomalacia, infection, metabolic disorders, and seizure, and
other conditions. [15]

Apparent Life Threatening Event (ALTE) was defined by the 1986 National Institutes of Health Consensus Development Conference on Infantile Apnea and Home Monitoring as follows:

[ALTE is] an episode that is frightening to the observer and is characterized by some combination of apnea (central or occasionally obstructive), color change
(usually cyanotic or pallid but occasionally erythematous or plethoric), marked
change in muscle tone (usually marked limpness), choking or gagging. In some
cases, the observer fears that the infant has died. ALTE is not so much a
specific diagnosis as a description of an event.

In 2003, Geddes et al. reported that apnea associated with an ALTE resulted in severe cerebral hypoxia, brain swelling, and intracranial hemorrhage. [16] Maguire et al. do
not mention this article by Geddes, in which she stated: “We emphasize, that
the literature to support a diagnosis of shaken baby syndrome/inflicted head
injury is based on imprecise and ill-defined criteria, biased selection,
circular reasoning, inappropriate controls, and conclusions that overstep the
data. If it is the questioning of the criteria that is worrisome, we will
continue to do so and to cause worry.” Maguire et al. did not mention
that Geddes changed hier view between 2001 and 2003. In fact, ALTE is
associated with all of the signs and symptoms hitherto attributed to shaken
baby syndrome, [10] which Maguire, Kemp, and their coauthors now refer to as
inflicted brain injury.

When fractured vertebrae, ribs, skull, and limbs are associated with bruises or missing teeth that parents or caregivers are unable to explain, nutritional deficiencies should be
ruled out before concluding that physical violence was the cause of such
findings.

Even when a child has clinical findings that resemble “bite marks” or “ligature marks on hands and feet,” missing fingernails, or tissue tears that suggest lacerations or avulsive injuries,
the possibility of microscopic polyarteritis should be ruled out by tests for
neutrophilia; lymphopenia; and elevated levels of aspartate aminotransferase (AST),
alanine aminotransferase (ALT), C-reactive protein (CRP), and lactate
dehydrogenase (LDH) before accusing the caregiver of committing a crime.

Referring to the use of orthodox medical evidence, at the re-trial of a woman whose life sentence was quashed after she had already served three years for the alleged murder of a
child in her care, Lord Justice Toulson said, “Today’s orthodoxy may become tomorrow’s
outdated learning.” [17]

Although pattern recognition is important and efficient in making diagnoses in medicine, physicians must always remember that symptoms and findings typically have a differential
diagnosis, and when the differential diagnosis is bypassed, errors can be made,
causing harm to both patient and caregivers.

Michael D. Innis, MBBS, DTM&H, FRCPA, FRCPath, is honorary consultant hematologist, Princess Alexandra Hospital, Brisbane, Queensland, Australia. Contact: 1 White-Dove Court, Wurtulla, Queensland, Australia 4575. Phone +61
(0)7.5493.2826. Fax [phone]. E-mail: [e-mail]

Disclaimer: The views expressed are solely those of the author.

Potential conflict of interest: Dr. Innis has been paid consulting fees in three cases of alleged child abuse. He has given his opinion pro bono in several
other cases.

REFERENCES

1. Uscinski R. The shaken baby syndrome. J Am Phys Surg 2004;9:76-77.

2. Leestma JE. “Shaken Baby Syndrome”: Do confessions by alleged perpetrators validate the concept? J Am Phys Surg 2006;11:14-16.

3. Maguire S, Pickerd N, Farewell D., et al. Which clinical features distinguish inflicted from non-inflict brain injury? A systematic review. [Published online ahead of
print June 15, 2009] Arch Dis Child.
doi:10.1136/adc.2008.150110.

4. Muller, PJ, Deck JHN. Intraocular and optic nerve sheath hemorrhage in cases of sudden intracranial hypertension. J
Neurosurg 1974;41:160-166.

5. Medele RJ, Stummer W, Mueller A, Steiger H, Reulen H. Terson’s syndrome in subarachnoid hemorrhage and severe brain injury accompanied by acutely raised intracranial pressure. J Neurosurg 1998;88:851-854.

6. Clemetson CAB. Child abuse or Barlow’s disease. Med Hypotheses 2002;59(1):52-56.

7. Clemetson CAB Vaccinations, inoculations and ascorbic acid. J Ortho Mol Med 1999;14:137-142.

8. Innis MD. Vaccines, apparent life-threatening events, Barlow’s disease, and questions about “shaken baby syndrome.” J Am Phys Surg 2006;11:17-19.

9. Rutty GN, Smith M, Malia RG. Late form hemorrhagic disease of the newborn. A fatal case report with illustrations of investigations which may assist avoiding the mistaken diagnosis
of child abuse. Am J Forensic Med Path
1999;20(1):48-51

10. Innis MD. Vitamin K deficiency disease. J Orthomol Med 2008;23:15-20.

11. Duncan P. Parents given apology over their baby’s death. Irish Times, Jul 17, 2009.

12. American Academy of Pediatrics. Shaken baby syndrome: rotational cranial injuries. Pediatrics 2001;108(1):206-210.

13. Kemp AM, Stoodley N, Cobley C, et al. Apnoea and brain swelling in non-accidental head injury. Arch Dis Child 2003;88:472-476.

14. Geddes JF, Hackshaw AK, Vowles GH, Nickols CD, Whitwell HL. Neuropathology of inflicted head injury in children. I. Patterns of brain damage. Brain 2001;124(Pt7):1290-1298.

15. McGovern MC, Smith MR. Causes of apparent life threatening events in infants a systematic review. Arch Dis Child.2004;89:1043-1048.

16. Geddes JF, Taskert RC, Hackshaw AK, et al. Dural hemorrhage in non-traumatic infant deaths: does it explain
the bleeding in “shaken baby syndrome”? Neuropathol
App Neurobiol 2003; 29:14-22.

17. Lewis P, Dodd V. Babysitter freed from jail after court orders retrial on murder charge. Guardian, May 2, 2008.NIA