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 • Etiology – Trauma – Bridging vein rupture • Accidental • Classic theory • Abusive – Vessel rupture in other • Birth compartments • Post-procedural • Aneurysm or – Vascular arteriovenous malformation or fistula – Hematologic – Subdural membrane – Metabolic – Intrinsic dural vessels – Infection

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 • Leakage from – Extensive in infants intradural vessels may – Plexus vessels have occur with hypoxia and thin, fenestrated walls elevated intravascular – May play a role in CSF pressure resulting in absorption intradural bleeding and • Chronic subdurals thin film subdural – Tentorium, posterior hemorrhage falx, and floor of the posterior cranial fossa

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

4 with thin homogeneous collections 108 large subarachnoid spaces 2 with bilateral 168 initial complex Concerning patients collections for abuse 60 normal No subdural subarachnoid collections spaces 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 Spinal cord 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 Intracranial Venous Thrombosis

• Uncommon in the • Risk factors pediatric population – Usually present – Canadian Pediatric – Neonates Ischemic Stroke • Dehydration Registry • Perinatal issues • 0.67 per 100, 000 – Nonneonates • 43% neonates • Head and neck • 54% < 1 year old • Chronic disease • 160 patients with • Hypercoagulable states sinovenous thrombosis in the first 6 years

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 • Extra-axial hemorrhage – Cortical vein – Subarachnoid – Deep venous – Subpial – Cerebral swelling – Subdural

Does Intracranial Venous Thrombosis Cause Subdural Hemorrhage? Does Intracranial Venous Thrombosis Cause Subdural Hemorrhage?

• DeVerber (NEJM) • McLean (AJNR) – 66/160 cerebral – 36 patients intracranial parenchymal infarcts venous thrombosis • 24 neonates with – 1 day old to 19 years hemorrhagic infarcts – 0/36 subdural – Extra-axial hemorrhage hemorrhage in 14/160 patients – Correspondence with (9%) DeVerber-14% were – In the article, there is young infants with no description of the posterior distribution location or age 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. Abusive Head Injury

Avulsed and thrombosed cortical vein

Absent left cortical veins 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

Initial MR at 14 months Crisis at 2 years 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”