Investigation of Sudden Investigation of Sudden and Unexpected - - PowerPoint PPT Presentation

Investigation of Sudden Investigation of Sudden and Unexpected Infant and Unexpected Infant Deaths Deaths

Melanie Newbould Melanie Newbould Paediatric and Perinatal Paediatric and Perinatal Pathologist Pathologist RMCH RMCH

Every year in England Every year in England and Wales and Wales… …

• Around 600 babies die suddenly

Around 600 babies die suddenly and unexpectedly in the first and unexpectedly in the first year of life year of life

• In Lancashire and greater

In Lancashire and greater Manchester we see around 90 Manchester we see around 90-

• 100 each year

100 each year

We have become We have become increasing aware increasing aware… …

• That we simply cannot distinguish

That we simply cannot distinguish deaths due to some natural cause deaths due to some natural cause from those due to asphyxia from those due to asphyxia – – the the asphyxia can be accidental or in asphyxia can be accidental or in some cases deliberate some cases deliberate

• We get occasional cases in which a

We get occasional cases in which a parent confesses to deliberately parent confesses to deliberately smothering the child and we know smothering the child and we know that without the confession we really that without the confession we really would not identify these cases would not identify these cases

So for the past 8 years So for the past 8 years

• We have in this region stopped using

We have in this region stopped using the term the term “ “SIDS SIDS” ” because this implies because this implies a natural disease a natural disease

• We now use the term

We now use the term “ “unascertained unascertained” ” for the cause of for the cause of death and use the term death and use the term “ “Sudden Sudden Unexpected Death in Infancy Unexpected Death in Infancy” ” or

• r

SUDI SUDI to describe the typical to describe the typical presentation presentation

Because we give Because we give “ “Unascertained Unascertained” ” as the cause of death as the cause of death

• It means that there will be an

It means that there will be an inquest inquest

• This is because in order to do

This is because in order to do any investigations such as any investigations such as histology, microbiology, histology, microbiology, metabolic studies, toxicology metabolic studies, toxicology the Coroner must open an the Coroner must open an inquest inquest – – this is the Law this is the Law

At the Inquest At the Inquest… …

• The Coroner takes evidence

The Coroner takes evidence from the pathologist(s) and from the pathologist(s) and sometimes other clinicians sometimes other clinicians

• He/she then gives a decision as

He/she then gives a decision as to whether the death is natural to whether the death is natural

• r not on the balance of
• r not on the balance of

probabilities probabilities

The Kennedy Report The Kennedy Report

• Published in 2004

Published in 2004

• Was set up following the release

Was set up following the release from prison of Sally Clark in Jan from prison of Sally Clark in Jan 2003 2003

• She was convicted of killing her

She was convicted of killing her two infant sons who it was two infant sons who it was claimed had died suddenly and claimed had died suddenly and unexpectedly unexpectedly

The Kennedy Report The Kennedy Report

• Suggested a multi

Suggested a multi-

• agency

agency approach to the investigation of approach to the investigation of SUDI SUDI

• It is suggested that all

It is suggested that all personnel dealing with the death personnel dealing with the death should have some specialised should have some specialised experience experience

It is suggested It is suggested… …

• That the baby is taken

That the baby is taken immediately into A&E immediately into A&E department department

• It is suggested that some

It is suggested that some specimens are taken specimens are taken immediately from the body immediately from the body

Initially Initially

• There should be a strategy for

There should be a strategy for investigation decided between the investigation decided between the paediatric department and the police paediatric department and the police

• As soon as possible the parental

As soon as possible the parental home should be visited home should be visited

• A key part of the Kennedy Report is

A key part of the Kennedy Report is the instigation of a specialised the instigation of a specialised paediatrician who will visit the paediatrician who will visit the parental home in the first 24 hours parental home in the first 24 hours following the death and investigate following the death and investigate such matters as sleeping such matters as sleeping arrangements arrangements

The paediatrician then The paediatrician then… …

• Complies a report for the Coroner

Complies a report for the Coroner and the pathologist and the pathologist

• The Coroner orders the post mortem

The Coroner orders the post mortem examination usually to take place examination usually to take place within 2 within 2-

• 3 days of death

3 days of death

• This will usually be carried out by a

This will usually be carried out by a pathologist with a special interest in pathologist with a special interest in paediatric examinations, sometimes paediatric examinations, sometimes with a forensic pathologist with a forensic pathologist – – who is who is an expert in injury an expert in injury

Unless there is an Unless there is an

• bvious cause
• bvious cause…

…

• The pathologist(s) must give the

The pathologist(s) must give the cause of death as cause of death as “ “Unexplained Unexplained” ”

• r
• r “

“Unascertained Unascertained” ” at this stage at this stage

• The Coroner will then open an

The Coroner will then open an inquest following which the inquest following which the funeral can usually be held (not funeral can usually be held (not if any injuries have been found) if any injuries have been found)

The Post mortem report is The Post mortem report is confidential until the inquest confidential until the inquest

• It must not be disclosed to a third

It must not be disclosed to a third party without Coronial consent (that party without Coronial consent (that includes the paediatrician! includes the paediatrician! – – however however in most cases this is a formality only in most cases this is a formality only

• As soon as possible it is

As soon as possible it is recommended that the paediatrician recommended that the paediatrician goes through the post mortem goes through the post mortem findings with the family findings with the family

The Kennedy report The Kennedy report… …

• Recommends that all professionals

Recommends that all professionals involved meet 2 involved meet 2-

• 3 months after the

3 months after the death (GP, Paediatrician, police, death (GP, Paediatrician, police, social workers etc) and discuss their social workers etc) and discuss their

• findings. The report generated by
• findings. The report generated by

this meeting should then be made this meeting should then be made available to the Coroner before the available to the Coroner before the inquest inquest

• The paediatrician should also write a

The paediatrician should also write a detailed letter to the parents detailed letter to the parents explaining all the findings of this explaining all the findings of this report and offer to meet them report and offer to meet them

At the inquest At the inquest… …

• At the inquest (held when all

At the inquest (held when all statements and results are available) statements and results are available) the Coroner decides the Cause of the Coroner decides the Cause of death and the death certificate is death and the death certificate is issued issued

• The inquest is public

The inquest is public – – the press are the press are usually there usually there – – (there may issues of (there may issues of interest to the public involved). The interest to the public involved). The Kennedy report recommends that it Kennedy report recommends that it is held in private if at all possible is held in private if at all possible

The Kennedy Report The Kennedy Report … …

• Recommends that two terms

Recommends that two terms should be used should be used … …

– – SIDS SIDS – – for those cases that fulfil for those cases that fulfil the criteria exactly the criteria exactly – – SUDI SUDI for other deaths where there for other deaths where there are no post mortem findings and are no post mortem findings and where the police are not where the police are not conducting an investigation conducting an investigation

SIDS SIDS -

• definition

definition

• An infant dying suddenly and

An infant dying suddenly and unexpectedly between the age of one unexpectedly between the age of one month and one year (sometimes two month and one year (sometimes two years) in whom no cause of death is years) in whom no cause of death is found at post mortem and where the found at post mortem and where the clinical circumstances are typical. clinical circumstances are typical. Usually this means an infant found Usually this means an infant found dead in the cot, often in the morning dead in the cot, often in the morning

So what is different about the So what is different about the Kennedy protocol? Kennedy protocol?

• The SUDI paediatrician

The SUDI paediatrician – – this this person has a key role in the person has a key role in the investigation and the various investigation and the various meetings that are suggested meetings that are suggested

• The multi

The multi-

• agency meetings

agency meetings

What investigations are What investigations are suggested? First in A&E suggested? First in A&E

• Blood gases

Blood gases

• Urea and electrolytes

Urea and electrolytes

• Blood sugar

Blood sugar

• Blood cultures

Blood cultures

• Blood and urine samples for metabolic

Blood and urine samples for metabolic investigations investigations

• CSF for culture

CSF for culture – – also for metabolic also for metabolic investigations investigations

• In some cases skin for fibroblast culture

In some cases skin for fibroblast culture and muscle biopsy and muscle biopsy

• Note the Coroner must give consent for these

Note the Coroner must give consent for these

The post mortem The post mortem

• The pathologist(s) should have

The pathologist(s) should have access to the all the information access to the all the information collected by police and SUDI collected by police and SUDI paediatrician including paediatrician including photographs and videos of the photographs and videos of the scene scene

Before starting the PM Before starting the PM

• We get a full skeletal survey.

We get a full skeletal survey. This should be carried out by a This should be carried out by a department used to doing it and department used to doing it and reported by a paediatric reported by a paediatric radiologist prior to us starting radiologist prior to us starting the examination the examination

We then do a full post We then do a full post mortem mortem

• That is we look at the body

That is we look at the body externally, we look at the thoracic, externally, we look at the thoracic, abdominal and cranial cavities abdominal and cranial cavities

• If we find a cause of death

If we find a cause of death – – then we then we can take no further investigations can take no further investigations

• But in SUDI deaths it is very unlikely

But in SUDI deaths it is very unlikely that there will be an obvious cause of that there will be an obvious cause of death on naked eye examination death on naked eye examination

Post mortem investigations Post mortem investigations

• Any not taken in A&E

Any not taken in A&E

• Bacteriology

Bacteriology

• Virology

Virology

• Metabolic studies

Metabolic studies – –

– – Urine, Urine, – – blood, bile (for blood, bile (for acyl acyl carnitines carnitines) ) – – Frozen tissues (liver, kidney, Frozen tissues (liver, kidney, myocardium, muscle stained with ORO) myocardium, muscle stained with ORO)

• Toxicology

Toxicology

How often do we find How often do we find something? something?

• Well

Well – – quite often as far as quite often as far as histology is concerned histology is concerned – – in about in about 10 10-

• 20% of our cases each year

20% of our cases each year we find that there is evidence we find that there is evidence that the child had a chest that the child had a chest infection at the time of death infection at the time of death

• This is by far our most common

This is by far our most common finding finding

In the remaining 80 In the remaining 80-

• 90%

90%… …

• There are usually no positive

There are usually no positive findings at post mortem findings at post mortem

What about metabolic What about metabolic disease? disease?

• Well our experience is that there are

Well our experience is that there are not a great many cases presenting as not a great many cases presenting as sudden unexpected infant death in sudden unexpected infant death in this group this group

• Over the past 10 years we have only

Over the past 10 years we have only seen 2 such infants and seen 2 such infants and – – really really-

• these were not in any sense typical

these were not in any sense typical SUDI deaths SUDI deaths

Case1 Case1

• 3 month infant found dead in his cot

3 month infant found dead in his cot in the afternoon in the afternoon – – but in actual fact but in actual fact he had a long history of illness and he had a long history of illness and failure to thrive failure to thrive

• Parents first cousins

Parents first cousins

• History of two previous infant deaths

History of two previous infant deaths – – Reye Reye’ ’s syndrome diagnosed in both s syndrome diagnosed in both cases cases

• Skeletal survey normal

Skeletal survey normal

Case 1 Case 1

• At post mortem

At post mortem – – cardiomegaly cardiomegaly, , hepatomegaly hepatomegaly

• Frozen sections stained for

Frozen sections stained for microscopic fat showed lipid in liver microscopic fat showed lipid in liver – – kidney kidney – – myocardium myocardium-

• muscle. All
• muscle. All

very suggestive of fatty acid very suggestive of fatty acid

• xidation defect
• xidation defect
• In fact DNA studies later one LCHAD

In fact DNA studies later one LCHAD gene showed common mutation. gene showed common mutation.

Case 2 Case 2

• 20 month female

20 month female

• Died suddenly

Died suddenly – – but in fact had been but in fact had been taken to local A&E on several taken to local A&E on several

• ccasions with vague illness
• ccasions with vague illness

(vomiting, lethargy, odd breathing) (vomiting, lethargy, odd breathing)– – especially in week prior to death especially in week prior to death

• Urine sample showed features in

Urine sample showed features in keeping with keeping with methylmalonic methylmalonic acidaemia acidaemia

So my experience is So my experience is that that… …

• Basically deaths due to

Basically deaths due to metabolic causes do not really metabolic causes do not really present like SUDI present like SUDI

• It is of course possible that

It is of course possible that there are metabolic disease that there are metabolic disease that we do not yet recognise we do not yet recognise – – or is

• r is

it? it?

So what do I think? So what do I think?

• It is becoming increasingly clear that

It is becoming increasingly clear that SUDI is essentially a condition that is SUDI is essentially a condition that is strongly associated with poor socio strongly associated with poor socio-

• economic conditions

economic conditions

• From 1988 onwards the

From 1988 onwards the “ “back to back to sleep sleep” ” campaign has been campaign has been associated with a decrease in the associated with a decrease in the number of infants dying suddenly and number of infants dying suddenly and unexpectedly unexpectedly

• This has led to a particularly marked

This has led to a particularly marked decrease in such deaths in middle decrease in such deaths in middle class households class households

What factors are What factors are associated with SUDI? associated with SUDI?

• Several studies in different

Several studies in different countries have revealed an countries have revealed an association with poverty ( association with poverty (Eg Eg unwaged households) unwaged households)

• There is also an association

There is also an association with parental smoking with parental smoking

• Co

Co-

• sleeping

sleeping

Co Co-

• sleeping

sleeping

• Up to two thirds of cases in England

Up to two thirds of cases in England and Wales are now associated with and Wales are now associated with infants sleeping in the same bed, infants sleeping in the same bed, chair or sofa as a parent chair or sofa as a parent

• In some of these the position in

In some of these the position in which the child is found does seem which the child is found does seem to suggest that asphyxia must be the to suggest that asphyxia must be the cause (illustrating how important the cause (illustrating how important the history and scene investigations are) history and scene investigations are)

• It is possible that they are all caused

It is possible that they are all caused by asphyxia by asphyxia – – but we just do not but we just do not know know

Co Co-

• sleeping

sleeping

• There seems to be a clearly

There seems to be a clearly increased risk of infant death where increased risk of infant death where A SOFA OR CHAIR IS INVOLVED A SOFA OR CHAIR IS INVOLVED

• But in the case of a conventional

But in the case of a conventional double bed it is more complicated double bed it is more complicated Eg Eg the risk is most marked where the the risk is most marked where the parent smokes parent smokes -

Co Co-

• sleeping

sleeping

• There is some controversy about

There is some controversy about this this

• The medical anthropologists

The medical anthropologists

• ften suggest that co
• ften suggest that co-
• sleeping is

sleeping is natural for the human and has natural for the human and has evolved over the past 5 million evolved over the past 5 million years years

Co Co-

• sleeping

sleeping

• However

However – – it has to be admitted it has to be admitted that for most of that 5 million that for most of that 5 million years (up to years (up to – – say say-

• 15 years ago)

15 years ago) the infant mortality was rather the infant mortality was rather higher than might be considered higher than might be considered acceptable in our society. acceptable in our society.

Co Co-

• sleeping

sleeping

• I think the general point made

I think the general point made by the anthropologists is that it by the anthropologists is that it would be helpful to try to define would be helpful to try to define the factors in co the factors in co-

• sleeping that

sleeping that make it dangerous rather than make it dangerous rather than simply regard it as inappropriate simply regard it as inappropriate – – they often point out that it has they often point out that it has beneficial effects also beneficial effects also – – such as such as the promotion of breast feeding the promotion of breast feeding

Whatever the role Whatever the role played by co played by co-

• sleeping

sleeping

• I think it is clear that environmental

I think it is clear that environmental factors play a major role in SUDI factors play a major role in SUDI

• The number of two

The number of two SUDIs SUDIs in a family in a family is quite low is quite low – – and I think that it is and I think that it is unlikely that more than a few cases unlikely that more than a few cases actually have metabolic causes even actually have metabolic causes even in those cases with more than one in those cases with more than one death death

However However … …

• SUDI is essentially a diagnosis

SUDI is essentially a diagnosis

• f exclusion so we can only
• f exclusion so we can only

really make it if we have really make it if we have excluded as many causes of excluded as many causes of sudden death as we are able to sudden death as we are able to

• So we have to go on doing all

So we have to go on doing all the tests the tests

Case 3 Case 3

A case of A case of “ “out of hospital

• ut of hospital

cardiac arrest cardiac arrest” ”

6 week old boy 6 week old boy

• FTND, 9lb at birth, 2 older

FTND, 9lb at birth, 2 older siblings siblings

• Mother had been a methadone

Mother had been a methadone user in the past user in the past

• Terminal events commenced on

Terminal events commenced on evening of 5 evening of 5th

th April 2003, when

April 2003, when mother went out leaving baby in mother went out leaving baby in care of father care of father

6 week old boy 6 week old boy

• Bottle fed at midnight

Bottle fed at midnight

• 0300hrs

0300hrs – – put to sleep on sofa with put to sleep on sofa with father father

• 0630hrs

0630hrs – – mother returned from her mother returned from her evening out and noted baby and evening out and noted baby and father both asleep father both asleep

• 0640hrs

0640hrs – – baby seemed to be baby seemed to be breathing oddly breathing oddly – – mother called mother called ambulance ambulance – – arrived promptly arrived promptly – – baby baby in state of cardiac arrest on arrival in state of cardiac arrest on arrival

6 week old boy 6 week old boy

• Taken to local A&E

Taken to local A&E – – intubated, ventilated intubated, ventilated

• Had CT scan showing diffuse cerebral

Had CT scan showing diffuse cerebral

• edema
• edema
• Transferred to RMCH PICU during the late

Transferred to RMCH PICU during the late morning morning

• Developed seizures

Developed seizures

• Died 09 04 03 when MRI scan showed

Died 09 04 03 when MRI scan showed severe cerebral oedema with severe cerebral oedema with uncal uncal herniation herniation and coning. Developed and coning. Developed intractable bradycardia intractable bradycardia

• No retinal haemorrhages seen during life

No retinal haemorrhages seen during life

Investigations during terminal Investigations during terminal illness illness

• Admission blood culture

Admission blood culture – – coagulase coagulase negative Staphylococcus negative Staphylococcus

• Toxicology

Toxicology – – urine positive for urine positive for therapeutic drugs only therapeutic drugs only

• DNA

DNA -

• negative for major LCHAD,

negative for major LCHAD, MCAD mutations MCAD mutations

• Normal

Normal acyl acyl carnitines carnitines, ammonia, , ammonia, biotinidase biotinidase

• ECG

ECG – – some abnormalities in keeping some abnormalities in keeping with ischaemia but none that permit with ischaemia but none that permit diagnosis of long QT syndrome diagnosis of long QT syndrome

It is important It is important… …

• That relevant investigations be done

That relevant investigations be done prior to death in the situation of out prior to death in the situation of out

• f hospital cardiac arrest since it is
• f hospital cardiac arrest since it is

likely to be too late to do toxicology, likely to be too late to do toxicology, microbiology etc at post mortem microbiology etc at post mortem

• Is there some way to ensure that

Is there some way to ensure that blood, urine etc is taken as soon as blood, urine etc is taken as soon as possible after admission in this possible after admission in this situation for toxicology? situation for toxicology?

Post mortem Post mortem examination examination

• Normally grown, normally

Normally grown, normally formed boy formed boy

• No external injuries

No external injuries

• Marks of medical intervention

Marks of medical intervention present present

• No congenital abnormalities

No congenital abnormalities

Skeletal survey Skeletal survey

• Multiple Lesions on posterior

Multiple Lesions on posterior aspect of ribs that seemed like aspect of ribs that seemed like fractures fractures

• No other abnormalities

No other abnormalities identified identified

Neuropathology Neuropathology

• Severe hypoxic ischaemic

Severe hypoxic ischaemic

• encephalopathy. NO subdural
• encephalopathy. NO subdural

haemorrhages haemorrhages

• Spinal cord

Spinal cord – – no trauma no trauma

• Eye histology

Eye histology – – multiple retinal multiple retinal haemorrhages haemorrhages – – but the significance but the significance

• f this is in doubt since these were
• f this is in doubt since these were

not identified on clinical examination not identified on clinical examination shortly after admission shortly after admission

Bone pathology Bone pathology… …

• Lesions involving ribs

Lesions involving ribs not not confirmed to be fractures on confirmed to be fractures on histology histology

• Bone pathologist is not sure

Bone pathologist is not sure what they are what they are – – but could, in any but could, in any case, date from birth case, date from birth

Other investigations Other investigations … …

• Post mortem histology

Post mortem histology – – no no haemosiderin laden macrophages in haemosiderin laden macrophages in lung lung – – focal bronchopneumonia focal bronchopneumonia – – but but this may be terminal this may be terminal

• No other histological features of note

No other histological features of note

• No viruses or bacteria isolated from

No viruses or bacteria isolated from CSF CSF – – PCR negative PCR negative

• No lipid in frozen sections of tissues

No lipid in frozen sections of tissues

So we have So we have… …

• A case of out of hospital collapse in

A case of out of hospital collapse in an infant sleeping on a sofa with his an infant sleeping on a sofa with his father father

• At post mortem signs of brain

At post mortem signs of brain hypoxia hypoxia

• No definite evidence of trauma

No definite evidence of trauma – – at at least none that can be identified as least none that can be identified as definitely post birth definitely post birth

• No natural disease that might have

No natural disease that might have led to the collapse identified led to the collapse identified

“ “Near miss Near miss” ” SIDS SIDS

• We definitely do not use this term

We definitely do not use this term now for the cases of out of hospital now for the cases of out of hospital cardiac arrest cardiac arrest

• In some we find a definite natural

In some we find a definite natural cause but often we don cause but often we don’ ’t when the t when the collapse occurs suddenly without a collapse occurs suddenly without a prodromal prodromal illness illness

• We then use the term

We then use the term “ “unascertained unascertained” ”

Case 4 Case 4

Case 4 Case 4

• Mother aged 16 years, drug abuser,

Mother aged 16 years, drug abuser, unemployed, unsupported unemployed, unsupported

• Slept with child on the settee and, in

Slept with child on the settee and, in the morning found that the baby was the morning found that the baby was lifeless lifeless

• Post mortem carried out by forensic

Post mortem carried out by forensic and paediatric pathologist and paediatric pathologist

Case 4 Case 4

• Baby was clean, well nourished

Baby was clean, well nourished

• X

X-

• ray was normal

ray was normal – – no injuries no injuries

• No injuries at post mortem

No injuries at post mortem

• No congenital abnormalities

No congenital abnormalities

• No evidence of infection

No evidence of infection

• No evidence of metabolic

No evidence of metabolic abnormality abnormality

• Essentially negative post mortem

Essentially negative post mortem

Case 4 Case 4

• So

So “ “unascertained unascertained” ” given as given as cause of death at Inquest cause of death at Inquest

Case 5 Case 5

Case 5 Case 5

• Female infant aged 18 weeks.

Female infant aged 18 weeks. Body weight only 2Kg. Body weight only 2Kg.

• Mother aged 17 years

Mother aged 17 years – – drug drug abuser abuser – – also has 30 month old. also has 30 month old.

• Called an ambulance to say that

Called an ambulance to say that she found her baby dead in her she found her baby dead in her cot at 1630hrs. Last checked cot at 1630hrs. Last checked child at midnight previous night. child at midnight previous night.

Case 5 Case 5

• We found that baby had multiple rib

We found that baby had multiple rib fractures and long bone fractures fractures and long bone fractures

• There were multiple other injuries

There were multiple other injuries – – bruises, burns bruises, burns

• Actually died of bronchopneumonia

Actually died of bronchopneumonia

• Toxicology

Toxicology – – no abnormal substances no abnormal substances

We were in no doubt We were in no doubt… …

• That the cause of death here

That the cause of death here was not wholly natural was not wholly natural

• The baby had been abused and

The baby had been abused and neglected neglected

Case 5 Case 5

• There was a criminal case

There was a criminal case

• Father pleaded guilty to

Father pleaded guilty to manslaugter manslaugter, mother to neglect. , mother to neglect.