Eye findings associated with abusive head. trauma (AHT) Mark Jacobs Staff Specialist Ophthalmologist Sydney Children s Hospital

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1 Eye findings associated with abusive head Mark Jacobs Staff Specialist Ophthalmologist Sydney Children s Hospital Gaurav Bhardwaj Ophthalmology Fellow trauma (AHT)

2 Retinal haemorrhages in AHT Retinal anatomy Pathology of retinal haemorrhages Pathogenesis of retinal haemorrhages in AHT Review of the literature Differential diagnosis Eye findings of AHT in Sydney Standards for the ophthalmic examination Minimum requirements for a written report

3 Anatomy and Pathology Posterior pole Mid-periphery Peripheral

4 Multilayered structure of the retina

5 Choroidal Sub-retinal ELM Deep intra-retinal Superficial intra-retinal ILM Pre-retinal PHM Vitreous Sub-ILM

6 Description of haemorrhages Layer: vitreous, preretinal, intraretinal etc Type: flame, dot-blot etc Number: zero up to too numerous-to-count Distribution: post pole to far periphery Schisis/folds

7 Preretinal and subretinal haemorrhage

8 Retinoschisis and retinal folds Splitting and intraretinal blood-filled cyst Lueder et al, Arch Ophthalmol 2008 Kivlin et al Arch Ophthal 2008

9 Proposed pathogenesis of retinal haemorrhages in AHT Mechanical trauma Raised ICP Raised intrathoracic pressure Hypoxia

10 Mechanical trauma Repetitive acceleration-deceleration Vitreous shaking

11 Raised intracranial pressure

12 Raised intracranial pressure Pathophysiology other factors Increased intravascular pressure Terson syndrome = intracranial blood and retinal haemorrhages Rare in children

13 Increased intrathoracic pressure Pathophysiology other factors Increased intravascular pressure Purtscher retinopathy = chest compression and retinal haemorrhages Rare in children

14 RH BRAIN (MENINGES) CHEST NECK (BRAINSTEM) Compression Whiplash Intracranial haemorrhage Intrathoracic pressure Accelerationdeceleration Hypoventilationhypoxia Terson s Intracranial pressure Purtscher s Vitreo-retinal traction Retinal venous pressure Concussion

15 Review of the literature

16 Intraocular haemorrhages (IOH) in Abusive head trauma (AHT) Sensitivity 75% Specificity 94%

17 Extent of IOH in AHT 100 % I O H Clinical Autopsy 0 Vitreous Preretinal Intraretinal Subretinal Choroidal

18 Location of IOH 100 % I O H Posterior Pole Midperiphery Peripheral

19 Retinoschisis Intraretinal blood filled cyst Sensitivity 14% Specificity Not 100% Crush head injury Severe accidental head injury

20 Perimacular retinal folds Sensitivity 8% Specificity Not 100% Crush head injury Severe accidental head injury Lueder et al, Arch Ophthalmol

21 FALL Retinal folds and retinoschisis Children < 3 AHT CRUSH

22 AHT Postmortem eye findings Retinal haemorrhages- approaches 100% in postmortem studies Choroidal haemorrhage 30 50% of autopsy cases Intrascleral haemorrhages Optic nerve sheath haemorrhages Haemorrhages in extraocular muscles

23 AHT ophthalmic outcomes Blindness in 15 to 28% of cases Additional 15% experience other forms of visual impairment field loss, colour vision impairment, abnormal binocularity Can have no retinal damage and permanent visual loss due to cortical injury Injury to the occipital cortex is the most common cause of visual loss Some will have optic atrophy

24 Differential Diagnosis of retinal haemorrhages in infants Traumatic Non-traumatic

25 Differential Diagnosis Traumatic Common Birth Abusive head trauma Uncommon Crush head injury Severe accidental head injury Blunt or penetrating eye injury

26 80 70 Differential Diagnoses - Birth Normal Forceps Vacuum STUDY (n) %RH Normal Forceps Vacuum C-section Hughes (53) %RH Emerson ( Besio (234) Jain (2016) Mean

27 Differential diagnoses Accidental head injury all severity Prospective controlled studies: Prospective case series Duhaime (1992) Johnson DL (1993) Buys (1992) Elder JE (1991) Ewing-Cobbs (1998) Alario AJ (1992) Keenan (2004) Trenchs V (2008) Pierre-Kahn (2003) Bechtel (2004) Vinchon (2005) Total ~ 400 patients Total 419 patients 8% 2%

28 Differential diagnoses Non traumatic Abnormal blood vessels Abnormal blood constituents Infections Raised intracranial pressure Raised intrathoracic pressure

29 Differential diagnoses Abnormal blood vessels ROP Coat s disease Vasculitis

30 Differential diagnoses Abnormal blood constituents Anaemia Leukaemia Thrombocytopaenia

31 Differential diagnoses Infections CNS meningitis, encephalitis Intraocular malaria, CMV, toxoplasmosis Systemic sepsis

32 Differential diagnoses Raised intracranial pressure Terson s syndrome <8% (aneursym, AVM)

33 Differential diagnoses Raised intrathoracic pressure Purtscher retinopathy Valsalva retinopathy

34 Mimics RH + ICH ± fractures ±bruising Glutaric aciduria Methylmalonic aciduria Osteogenesis imperfecta type 1 Menke s disease Platelet function defect Protein C deficiency Hypofibrinogenaemia Haemorrhagic disease of the newborn Fibromuscular dysplasia Spinal cord AVM

35 Confounding factors Raised intrathoracic pressure CPR with chest compressions Kanter (1986), Odom (1997), Gilliland(1993) 2% Convulsions Sandramouli (1997), Tyagi (1998), Mei-Zahav(2002), Ho (2005) 1% Vomiting Herr (2004) 100 patients 0% Cough Goldman (2006) 100 patients 0%

36 Sydney Study Retinal Haemorrhages in young children Comparison of retinal findings in high risk conditions in children < 3 years old Wide field retinal imaging + Indirect ophthalmoscopy Reporting by 2 ophthalmologists, masked as to causation

37 Methods Prospective, consecutive admissions Two centres 1. The Children s Hospital at Westmead 2. Sydney Children s Hospital Duration October 2008 July 2010 (22 months)

38 Aims To objectively assess retinal haemorrhages (with retinal imaging) all children under 3 years with: Head injury Raised ICP Raised intrathoracic pressure Hypoxia

39 Methods All wide field retinal images assessed by 2 independent experts, masked to causation, for Presence of RH Layer involved Distribution in the retina Presence of schisis, or retinal folds

40 Determination of abuse Performed by Paediatrician in Child Protection Unit

41 Results CATEGORY AGE (m) (SD) COHORT HEAD INJURY 13.3 (10.5) 118 RAISED INTRACRANIAL PRESSURE RAISED INTRATHORACIC PRESSURE 9.5 (9.4) (10.3) 28 HYPOXIA 4.7 (4.3) 15 TOTAL 12.0 (10.2) 183

42 HEAD INJURY ABUSIVE INDETERMINATE ACCIDENTAL 11 Mean age : 5.8 months (1 13 months) M:F 2:1 Skull fractures 5 (24%) Intracranial haemorrhage SDH (16) 81% EDH (1) 5% SAH/SDH (1) 5% Total (18) 86% RH 78% Mean age : 12.4 months (3-36 months) M : F - 7:4 Skull fractures 7 (64%) Intracranial haemorrhage SDH (7) 64% EDH (1) 12% Total (8) 72% RH 36% Mean age : 15.0 months (1-36 months) M:F 51 : 35 Skull fractures 58 (67%) Intracranial haemorrhage SDH (19) 22% EDH (14) 16% SAH (10) 12% Combination (4) 5% Total (36) 42% RH 5%

43 Head Injuries- 118 children No 6 4 Accident Indeterminate Abuse

44 Demographics of AHT Overall incidence of AHT with SDH 9.6/100,000 (infants less than one year) Significant risk factors: Aboriginal background (x2=12.1, p<0.0001) Rural location (x2=17.8, p<0.0001)

45 Demographics of AHT Mean maternal age 26.7, paternal age 28.3 Separated parents 43% Previous DOCS involvement 29% Current or previous drug or alcohol misuse 33% Smoking/alcohol during pregnancy 24% One or both parents with mental disorder 43% One set of twins (19%)

46 Analysis of AHT SDH (17) 81%, bilateral SDH (14) 67% No explanation for injury (9) 43% Trivial mechanism (7) 33% Perpetrator confession/admission (4) 19% 2 shaking, 1 forceful jerk, 1 slamming into cot Witnessed abuse (1) 4% Battered over head Other inflicted injuries (12) 57% Rib or long bone fractures Recent presentations to hospital (5) 24% Mortality rate (3) 14%

47

48 Distribution of RH in AHT (%)

49 Analysis of RH in AHT Most important factors in presence of RH SDH SAH Most important factors in presence of schisis/folds Infarction

50 Analysis of RH in AHT Most important factors in severity of RH Infarction Diffusion abnormality on MRI SDH

51 Most specific patterns for AHT Severe, panretinal pattern with multi-layered haemorrhages (100% specific)- present in 62% Macular retinoschisis or perimacular retinal folds (100% specific) - present in 38% (8 cases)

52 Bilateral extensive RH in multiple layers with bilateral perimacular folds

53 Bilateral, confluent RH, in multiple layers, with large pre-retinal hemorrhage and probable macular schisis bilaterally

54 Raised intracranial pressure (non-traumatic) 19 children Mean age = 9.6 (range months) Duration of symptoms 1 day 3weeks RH seen in 3 cases (16%)

55

56 Raised intrathoracic pressure CPR - Drowning, cardiac problems etc 26 Chest crush injury piano crush 1-4WD crush abdomen/lower chest Retinal haemorrhages 3 (11%) All had CPR for > 30 mins

57 Prolonged CPR with hypoxia

58 Hypoxia Apnoeic / near SIDS 12 Choking 3 TOTAL 15 Rate of RH 0

59 Discussion Widespread, bilateral RHs and retinoschisis commonly occur in very young infants where there is high suspicion of abuse Distinguishing features for abuse : Severity of the RH Macular retinoschisis / retinal fold Combination of retinal findings with other features

60 Discussion RH uncommon in raised ICP Terson s syndrome can occur in children Perhaps a sudden rise in ICP is responsible for the RHs in certain accidental HI e.g. head crush

61 Discussion Retinal findings used to update the probability of abuse Mild RH at Posterior pole = non-specific Severe RH extending peripherally = more specific

62 Standards for Ophthalmic examination Child to be seen within 48 hours of injury Dilated retinal examination Ophthalmologist assessment with indirect ophthalmoscopy Detailed description and drawings of eye findings by an ophthalmologist Wide field retinal imaging if available to augment the ophthalmologists findings Optical coherence tomography in the future

63 Minimum requirements for a written report Detailed description of eye findings by an ophthalmologist

64

65 Thank you for your attention

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