Abusive Head Injury or Not? Differential Diagnoses to

Consider Marguerite M. Caré, MD

Cincinnati Children’s Hospital Medical Center

Department of Radiology

Division of Neuroradiology

Objectives

• Discuss controversies and differential diagnoses for abusive head injury

• Review the importance of a multidisciplinary approach to child abuse cases

Abusive Head Injury “Triad”

• Subdural hemorrhage

• Retinal hemorrhages

• Parenchymal injuries – “Encephalopathy”

– Often the most devastating

– May be missed on early imaging

– Dating of injuries

• Skeletal injuries

Abusive Head Injury Outcomes

• Majority of patients have a poor outcome

– Fatalities in ~20%

• Abusive head injury-leading cause of death in child abuse cases

– Morbidities in ~50%

• Cognitive and motor

• Vision

• Language

• Behavioral

• ~20% have no impairment

Makoroff KL and Putnam FW. Outcomes if Infants and Children with Inflicted Traumatic Brian Injury. Developmental Medicine

and Child Neurology 2003, 45: 497-502

CCHMC-Abusive Head Injury

Year Cases Deaths

2007 23 4

2008 22 3

2009 29 5

2010 23 3

2011 20 1

2012 25 4

Large free-standing pediatric medical center with a

long-term child protection and advocacy center

Controversies

• Etiology of subdural collections/hemorrhage

• Hypoxic-ischemic injury and subdural hemorrhage

• Benign enlargement of the subarachnoid spaces

• Intracranial venous thrombosis

Subdural Hemorrhage

• Causes

– Trauma

• Accidental

• Abusive

• Birth

• Post-procedural

– Vascular

– Hematologic

– Metabolic

– Infection

• Etiology

– Bridging vein rupture

• Classic theory

– Vessel rupture in other compartments

• Aneurysm or arteriovenous malformation or fistula

– Subdural membrane

– Intrinsic dural vessels

Mack J, Squier W, and Eastman J. Anatomy and development of the meninges: implications for subdural

collections and CSF circulation. Pediatric Radiology (2009) 39: 200-210.

Squier W and Mack J. The neuropathology of infant subdural hemorrhage. Forensic Sci. Int. (2009)

Proposed Alternative Mechanism of Subdural Hemorrhage

• Dural venous plexus

– Extensive in infants

– Plexus vessels have thin, fenestrated walls

– May play a role in CSF absorption

• Chronic subdurals

– Tentorium, posterior falx, and floor of the posterior cranial fossa

• Leakage from intradural vessels may occur with hypoxia and elevated intravascular pressure resulting in intradural bleeding and thin film subdural hemorrhage

Mack J, Squier W, and Eastman J. Anatomy and development of the meninges: implications for subdural

collections and CSF circulation. Pediatric Radiology (2009) 39: 200-210.

Squier W and Mack J. The neuropathology of infant subdural hemorrhage. Forensic Sci. Int. (2009)

Does hypoxia with raised intravascular pressure cause subdural hemorrhages?

• Some investigations with case studies and models suggest this hypothesis – Mack J, Squier W, and Eastman J. Anatomy and development of the meninges:

implications for subdural collections and CSF circulation. Pediatric Radiology (2009) 39: 200-210.

– Cohen MC, Scheimberg I. Evidence of occurrence of intradural and subdural

hemorrhage in the perinatal and neonatal period in the context of hypoxic ischemic encephalopathy. An observational study from two referral institutions in the United Kingdom. Pediatr Dev Pathol 2009; 12: 169-76.

– Geddes JF, Tasker RC, Hackshaw AK, et al. Dural haemorrhage in non-traumatic

infant deaths: does it explain the bleeding in “shaken baby syndrome”? Neuropathol Appl Neurobiol 2003; 29: 14-22.

– Geddes JF and Talbert DG. Paroxysmal coughing, subdural and retinal bleeding: a computer modeling approach. Neuropathology and Applied Neurobiology (2006),

32; 625-634.

Does hypoxia with raised intravascular pressure cause subdural hemorrhages?

• Some investigations with case studies do not lend support to this hypothesis – Hurley M, Dineen R, Padfield CJH, et al. Is there a causal relationship between the

hypoxia-ischaemia associated with cardiopulmonary arrest and subdural haematomas? An observational study. The British Journal of Radiology, 83

(2010), 736-743.

– Byard RW, Blumbergs P, Rutty G, et al. Lack of evidence of a causal relationship between hypoxic-ischaemic encephalopathy and subdural hemorrhage in fetal life, infancy and early childhood. Pediatr Dev Pathol 2007; 10: 348-50.

– Rafaat KT, Spear RM, Kuelbs C, Parsapour K, and Peterson B. Cranial computed

tomographic findings in a large group of children with drowning: Diagnostic, prognostic, and forensic implications. Pediatr Crti Care Med 2008; Vol. 9, No. 6: 567-572.

• Vast majority of clinical experience and literature suggests that when a young infant presents with hypoxic brain injury, subdural hemorrhages and extensive retinal hemorrhages, abusive injury is the most likely cause

– Remember that each case must be looked at thoroughly and individually

– Abusive injury, as well as other potential diagnoses need to be considered

– Prudent to be familiar with the current literature and controversies

Enlarged Subarachnoid Spaces

• Benign macrocrania

• Benign enlargement of the extra-axial spaces

• External hydrocephalus

• 3-6 months

• Resolves by 2-3 years

• Macrocrania

• Normal development

Subdural Space or Compartment

Do Enlarged Subarachnoid Spaces Contribute to the Development of Subdurals?

• Retrospective review at CCHMC

– 24 month period

– CT and MRI reports of patients < 2 years old

– “macrocrania” and “macrocephaly”

– 279 initial criteria

– Included 168 with enlarged head circumference

– Excluded: • Congenital abnormalities

• Brain volume loss

• Chemotherapy/radiation therapy

• Failure to thrive

• Significant prematurity

Prevalence of Subdural Collections in Children with Macrocrania

168 initial patients

108 large subarachnoid

spaces

4 with thin homogeneous

collections

2 with bilateral complex

collections Concerning for abuse

60 normal subarachnoid

spaces

No subdural collections

Conclusions

• Thin, non-hemorrhagic subdural collections may occur in children with enlarged subarachnoid spaces and macrocrania and may not indicate inflicted injury

• However, every unexpected subdural collection in a young child requires clinical consideration for potential underlying causes

– Primarily abusive head injury

– Particularly in hemorrhagic or complex collections

Greiner M, Richards T, Caré M, and Leach J. Prevalence of Subdural Collections in Children with Macrocrania. AJNR In

Press.

Accidental versus Abusive Injuries

• Accidental injuries are often the most difficult diagnosis to exclude

• History

– Short distance falls

– Proportional to injury

– “killer couch”

Accidental Injuries

• Skull fractures

– Accidental and abusive

• Linear

• Parietal bone

– Complex, crossing sutures, bilateral, and diastatic are concerning

– Healing

• Not accurately dated

• Less distinct and resolved by 6 months

• No follow-up radiographs

Accidental Injuries

• Extra-axial hemorrhage

– Close to site of injury

– More likely epidural

– Subarachnoid and subdural hemorrhage usually in more significant accidental and abusive injury

Accidental Injuries

• Parenchymal injury

– Less common than in abusive head injury

– Contusions and shear injury more often in accidental

– Hypoxic-ischemic injury more common in abusive injuries or significant accidental injury

Birth-Related Hemorrhage

• Vaginal and cesarean

• Asymptomatic

– Thin subdural

– < 3mm

– Posterior location

– Resolve 1 month

• Symptomatic in more traumatic deliveries

Frequency and natural history of subdural hemorrhages in babies and relation to obstetric factors. Whitby, EH, et al.

Lancet 2004, 363(9412) 846-851.

Prevalence and evolution of intracranial hemorrhage in asymptomatic term infants. Rooks, VJ, et al. AJNR 2008, 29;

1082-89.

Hematologic Diagnoses to Consider

• Coagulopathies

– Newborn

– Hemophilia and factor deficiencies

– Acquired

• Disseminated intravascular coagulation (DIC)

• Vascular

• Hemophagocytic lymphohistiocytosis

Neonatal Alloimmune Thrombocytopenia

• Thrombocytopenia in a newborn

• Like hemolytic disease of the newborn

– Human platelet antigen of the fetus in negative mother

• Treat with platelets

• Normal platelets at 2 weeks

Neonatal alloimmune thrombocytopenia. Rayment R, et al. BMJ. 2003 August 9; 327(7410): 331-332

Vitamin K Deficiency Bleeding

• Synthesis of coagulation factors II, VII, IX, and X

• Low newborn vitamin K levels

• Newly born period or late (up to 6 months of age)

– Idiopathic

– Secondary to hepatic or intestinal disease

– Prophylaxis use

• Intracranial hemorrhage

Cekinmez, M, et al. Intracranial Hemorrhages due to late-type vitamin K deficiency bleeding. Childs Nervous System (2008)

24: 821-825

Spinal cord

Intracranial Venous Thrombosis

• Uncommon in the pediatric population

– Canadian Pediatric Ischemic Stroke Registry

• 0.67 per 100, 000

• 43% neonates

• 54% < 1 year old

• 160 patients with sinovenous thrombosis in the first 6 years

• Risk factors

– Usually present

– Neonates

• Dehydration

• Perinatal issues

– Nonneonates

• Head and neck

• Chronic disease

• Hypercoagulable states

DeVerber G, et al. Cerebral sinovenous thrombosis in children. New England Journal of Medicine, 345; 6: 417-423.

McLean L, Frasier L, and Hedlund G. Does intracranial venous thrombosis cause subdural hemorrhage in the pediatric

population? AJNR (2012) 33: 1281-84.

Intracranial Venous Thrombosis

• Parenchymal findings

– Focal edema

• Vasogenic and cytotoxic

• May be reversible

– Hemorrhagic regions

• Location depends on involved vessels

– Dural sinus

– Cortical vein

– Deep venous

– Cerebral swelling

• Extra-axial hemorrhage

– Subarachnoid

– Subpial

– Subdural

Does Intracranial Venous Thrombosis Cause Subdural Hemorrhage?

Does Intracranial Venous Thrombosis Cause Subdural Hemorrhage?

• DeVerber (NEJM)

– 66/160 cerebral parenchymal infarcts

• 24 neonates with hemorrhagic infarcts

– Extra-axial hemorrhage in 14/160 patients (9%)

– In the article, there is no description of the location or age

• McLean (AJNR)

– 36 patients intracranial venous thrombosis

– 1 day old to 19 years

– 0/36 subdural hemorrhage

– Correspondence with DeVerber-14% were young infants with posterior distribution SDH

DeVerber G, et al. Cerebral sinovenous thrombosis in children. New England Journal of Medicine, 345; 6: 417-423.

McLean L, Frasier L, and Hedlund G. Does intracranial venous thrombosis cause subdural hemorrhage in the pediatric

population? AJNR (2012) 33: 1281-84.

Some Conclusions

• Isolated subdural hemorrhage is a very rare finding in intracranial venous thrombosis

• McLean (AJNR): – Subdural hemorrhage was not present in isolated, non-traumatic

causes of extensive intracranial venous thrombosis

– Intracranial venous thrombosis is likely not the inciting event for the development of subdural hemorrhage

– Prospective studies are needed demonstrating intracranial venous thrombosis in proven abusive head injury cases

McLean L, Frasier L, and Hedlund G. Does intracranial venous thrombosis cause subdural hemorrhage in the pediatric

population? AJNR (2012) 33: 1281-84.

Absent left cortical veins

Avulsed and thrombosed cortical vein

Abusive Head Injury

Hemophagocytic Lymphohistiocytosis

• Familial-infants

– Secondary-EBV

• Acute illness with prolonged fever

– Hepatic failure

– Neurologic findings

– Bone marrow

• Dismal prognosis without treatment-bone marrow transplant

Infectious Diagnoses to Consider

• Complicated paranasal sinus disease

• Bacterial meningitis

• Herpes encephalitis

– Often hemorrhagic

Infection

• Extra-axial collections

– Purulent

• Epidural-close proximity

• Subdural-close or remote

– “Sympathetic effusions”

• Parenchymal injury

– Cerebritis/abscess

– Ischemia/infarct

• Vascular complications

Complicated Sinus Disease

Meningitis

Cortical vein thrombosis

Meningitis

Metabolic Diagnoses to Consider

• Congenital-inborn errors of metabolism

• Acquired

• Brain volume loss

• Subdural collections or hemorrhage

• Abuse may coexist

Glutaric Aciduria Type I

• Organic aciduria

• Enzyme deficiency – Glutaryl-CoA

dehydrogenase

– Prevents normal amino acid metabolism

• Presentation – Macrocrania

– Acute encephalopathy and seizures

– Motor and developmental delay

Glutaric Aciduria Type I

• Brain imaging

– Abnormal white matter and basal ganglia

– Bilateral temporal cysts or enlarged spaces

• Prominent sylvian fissure

– Volume loss

– Subdural hematomas

Glutaric Aciduria Type I

Crisis at 2 years Initial MR at 14 months

Menkes Syndrome

• Kinky hair syndrome

• X-linked recessive mitochondrial disorder

• Decreased absorption of copper from the gastrointestinal tract

• Early infancy

• Hypotonia, seizures

Menkes Syndrome

• Brain imaging

– Volume loss

– Abnormal white matter signal

– Subdural collections

– Tortuous arteries

Menkes Syndrome

Initial MR at 1 month 18 months

Menkes Syndrome

• Radiographs

– Wormian bones

– Rib fractures

– Metaphyseal injury

• In certain cases of potential abuse, a definitive diagnosis can be made

• Others may be more difficult with nonspecific imaging findings

Child Abuse?

• Multidisciplinary approach – Clinical/Historical

• Reported history

• Presentation

• Physical examination

• Laboratory studies

– Radiology

– Investigation • Social services

• Police/Legal

“Big Picture”

• A suspected child abuse case require a multidisciplinary approach

• Be familiar with current controversies

• Be familiar with and consider alternative diagnoses

• “Big picture”