Sudden Unexpected Death In Children Research Articles

Understanding the role of uncertainty and anxiety in police decision‐making during the investigation of sudden unexpected deaths in children

AbstractExisting policing policy offers a rational approach to decision‐making with minimal reference to human judgement within child death investigations. This study adopted a mixed methodology to capture decisional processes of 26 serving detective inspectors and detective sergeants whilst responding to an immersive scenario which simulated the first hours of a Sudden and Unexpected Death in Children (SUDC) investigation. Participants were presented with four decision points, during which they were asked to choose from decision options while reflecting upon personal and situational factors influencing that decision. Thematic analysis of responses found that investigators disclosed ‘anxiety’ and ‘uncertainty’ throughout their decision‐making during the scenario. In addition, there was no evidence of investigators utilising the models formally advocated within policing literature to make their decisions and assessments. As a result, it is suggested that further improvements are made regarding investigative decision‐making models within operational policing where ‘anxiety’ and ‘uncertainty’ is heightened.

Safe sleep after 1 year of age.

Free AccessSleep Medicine PearlsSafe sleep after 1 year of age Eliot S. Katz, MD, Carolyn D’Ambrosio, MD Eliot S. Katz, MD Address correspondence to: Eliot S. Katz, MD, Division of Respiratory Diseases, Boston Children’s Hospital, 300 Longwood Ave., Boston, MA, 02115; Tel: (617) 355-1900; Email: E-mail Address: [email protected] Division of Respiratory Diseases, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts; Search for more papers by this author , Carolyn D’Ambrosio, MD Department of Pulmonology, Brigham and Women’s Hospital; Harvard Medical School, Boston, Massachusetts Search for more papers by this author Published Online:February 1, 2022https://doi.org/10.5664/jcsm.9660SectionsPDF ShareShare onFacebookTwitterLinkedInRedditEmail ToolsAdd to favoritesDownload CitationsTrack Citations AboutINTRODUCTIONAn 18-month-old toddler with end-stage renal disease was noted to have severe nocturnal hypoxemia episodes during a febrile illness. On physical examination, he was lean and mildly hypotonic but with normal awake gas exchange, no evidence of adenotonsillar enlargement, and no craniofacial abnormalities. Diagnostic polysomnography was performed on a standard infant mattress with a fitted sheet and blanket underneath him. At 01:00, the infant aroused from sleeping in the prone position and rotated his head to the midline (Figure 1). His face became embedded face-down in the linen and he begin to rebreathe carbon dioxide. During the next 6 minutes, there was a rapid increase in his end-tidal CO2 of 10 torr, and a decrease in his oxygen saturation to 88%. After 6 minutes, he aroused again and turned his head to the side, resulting in a rapid normalization of gas exchange.Figure 1: Facial entrapment during overnight polysomnogram.Ten-minute tracing from an overnight polysomnogram in an 18-month-old with truncal hypotonia. During prone sleep, an arousal results in a head movement to the face-down position, resulting in rebreathing of carbon dioxide as evidenced by an increase in the baseline and peak end-tidal carbon dioxide by 10 torr, and a decrease in the oxygen saturation by 10%. After 6 minutes, the entrapment is terminated with an arousal and head turn to the side. EtCO2, end-tidal carbon dioxide; RLEG, Right Leg; NAP, Nasal air pressure; ORL/NS, oro-nasal thermistor; ABD, Abdomen; CAPNO, capnogram; PWAVE, plethysmograph waveform; SaO2’ oxygen saturation; CO2, carbon dioxide.Download FigureQUESTION: What is the most appropriate safe sleep advice for toddlers?ANSWER: Beyond the first year of life, safe sleep advice generally focuses on injuries related to climbing or falling out of bed. Beds should be placed as close to the ground as possible. Nothing should be placed in the crib making it easier to climb out or get tangled, including window-blind cords, curtains, or electrical cords. Care should be taken to ensure that toddlers can’t reach small items that may pose a choking hazard. In addition, as this case demonstrates, toddlers with hypotonia may also potentially experience injury related to the prone positioning, especially in a sleep environment with soft bedding.DISCUSSIONThis case highlights the risk posed by prone positioning and soft bedding in medically complex children after infancy. Sudden entrapment of the face can occur when hypotonic toddlers sleep on a standard firm infant mattress with loose linen, resulting in a rapid elevation in end-tidal CO2 and hypoxemia triggering an arousal response.1 In this case, the arousal resulted in a successful head turn that unobstructed the airway, but it is conceivable that an event like this could have been fatal. Most toddlers who die unexpectedly are indeed found in the prone position, face-down,2 and most cases of sudden unexplained death in epilepsy (SUDEP) are also found prone.3 An elevated CO2 arousal threshold during sleep may be observed in children with neurological impairments, such as Prader-Willi syndrome, that may further contribute to their risk of sudden death.4In 1992, the American Academy of Pediatrics advised against prone sleeping for infants.5 Supine positioning (“back to sleep”) for infants during sleep is associated with a lower risk of the sudden infant death syndrome (SIDS).6 In 2016, the American Academy of Pediatrics made further “safe to sleep” recommendations for infants, including using a firm sleep surface, breastfeeding, having infants sleep in the parents’ room on a separate surface, keeping soft objects and loose bedding away from the infant’s sleep area, and consideration of a pacifier during sleep.7 Other recommendations included avoidance of the following; smoke exposure, alcohol/illicit drug use before or after birth, overheating or head covering, commercial sleep positioners, and cardiorespiratory monitors to reduce the risk of SIDS.7 The Committee further advised that “once an infant can roll from supine to prone and from prone to supine, the infant can be allowed to remain in the sleep position that he or she assumes.” However, the American Academy of Pediatrics’ policy makes no recommendations for safe sleep in the many toddlers with medical conditions resulting in decreased tone that may also place them at risk for sleep-related suffocation.Medically complex children have more severe gastroesophageal reflux which may be exacerbated in the supine position. Prone and left lateral positioning may result in less gastroesophageal reflux in premature infants.8 Nevertheless, in weighing the risks of prone positioning, the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition advises against positional therapy (head elevation, lateral/prone positioning) to treat gastroesophageal reflux in sleeping infants, but does recommend to consider positioning therapy in children.9 However, as the present case demonstrates, the prone position also poses a risk for older medically complex children, who may need enhanced monitoring and supervision during sleep to rapidly respond to life threatening situations. Indeed, a case-control study of SUDEP found that direct supervision and monitoring devices were protective.10SLEEP MEDICINE PEARLSThe current safe sleep guidelines for infants in the first year of life are essential and should be extended to older children with hypotonia of any etiology.Sleep laboratories should be vigilant in their prestudy evaluation to identify children at risk for sleeping prone and with soft bedding in order to provide enhanced monitoring and supervision.DISCLOSURE STATEMENTAll authors have seen and approved the manuscript. Work for this study was performed at Boston Children’s Hospital, Boston, MA. The authors report no conflicts of interest.REFERENCES1. Lijowska AS, Reed NW, Chiodini BA, Thach BT. Sequential arousal and airway-defensive behavior of infants in asphyxial sleep environments. J Appl Physiol 1985. 1997;83(1):219–228 . Google Scholar2. Krous HF, Chadwick AE, Crandall L, Nadeau-Manning JM. Sudden unexpected death in childhood: a report of 50 cases. Pediatr Dev Pathol. 2005;8(3):307–319 . CrossrefGoogle Scholar3. Kloster R, Engelskjøn T. Sudden unexpected death in epilepsy (SUDEP): a clinical perspective and a search for risk factors. J Neurol Neurosurg Psychiatry. 1999;67(4):439–444 . CrossrefGoogle Scholar4. Livingston FR, Arens R, Bailey SL, Keens TG, Ward SL. Hypercapnic arousal responses in Prader-Willi syndrome. Chest. 1995;108(6):1627–1631 . CrossrefGoogle Scholar5. Kattwinkel J, Brooks J, Myerberg D. Positioning and SIDS. Pediatrics. 1992;89(6 Pt 1):1120–1126. Google Scholar6. Malloy MH, Freeman DH. Age at death, season, and day of death as indicators of the effect of the back to sleep program on sudden infant death syndrome in the United States, 1992-1999. Arch Pediatr Adolesc Med. 2004;158(4):359–365 . CrossrefGoogle Scholar7. Moon RY; Task Force on Sudden Infant Death Syndrome. SIDS and other sleep-related infant deaths: updated 2016 recommendations for a safe infant sleeping environment. Pediatrics. 2016;138(5):e20162938 . CrossrefGoogle Scholar8. Corvaglia L, Rotatori R, Ferlini M, Aceti A, Ancora G, Faldella G. The effect of body positioning on gastroesophageal reflux in premature infants: evaluation by combined impedance and pH monitoring. J Pediatr. 2007;151(6):591–596, 596.e1 . CrossrefGoogle Scholar9. Rosen R, Vandenplas Y, Singendonk M, et al.. Pediatric Gastroesophageal Reflux Clinical Practice Guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66(3):516–554 . Google Scholar10. Langan Y, Nashef L, Sander JW. Case-control study of SUDEP. Neurology. 2005;64(7):1131–1133 . CrossrefGoogle Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 18 • Issue 2 • February 1, 2022ISSN (print): 1550-9389ISSN (online): 1550-9397Frequency: Monthly Metrics History Submitted for publicationMay 29, 2021Submitted in final revised formSeptember 4, 2021Accepted for publicationSeptember 7, 2021Published onlineFebruary 1, 2022 Information© 2022 American Academy of Sleep MedicinePDF download

Sudden unexpected death in children: myth or reality?

Despite many years of study, sudden unexplained death remains a tenuous diagnosis of exclusion. Here, we discuss the current science behind the uncertainties of sudden death, as well as the questions that still remain. Failure in any part of the complex interplay between peripheral sensors and central cardiorespiratory regulation can result in sudden death. Diagnostic testing with electrocardiograms, electroencephalogram, sleep studies, or even genetic studies have increased our ability to identify patients at the highest risk. Advances in the understanding of sudden unexplained death in children may show common pathways leading to sudden death from multiple different diseases. Although rare, the devastating implication prioritizes the importance in educating patients about how to live with the risk of sudden death.

Correction to: Cardiovascular causes of sudden unexpected death in children and adolescents (0-17years) : Anationwide autopsy study in the Netherlands.

Correction to:Neth Heart J 2018 https://doi.org/10.1007/s12471-018-1152-y In the version of the article originally published online, there was an error in the 'Methods and results' section of the Abstract. It is stated that 'In the 10-14year group, hypertrophic cardiomyopathy (n = 1) and ruptured ….

Working with families after a sudden child death

Abstract Every child's death is a tragedy. Many paediatricians feel unprepared to support bereaved families after a sudden child death, and are concerned about what may or may not be permitted with concurrent Sudden Unexpected Death in Childhood (SUDIC) and coronial investigations. After a child death, parents need to be able to say goodbye to their child, they require information both about the cause of death and the process of investigations, parents may also require emotional support. This review describes how to support bereaved families from the time of death and in the weeks and months that follow.

Cardiovascular causes of sudden unexpected death in children and adolescents (0-17years) : Anationwide autopsy study in the Netherlands.

BackgroundLittle is known about the causes of unexpected death in minors (0–17 years). In young adults an important cause is cardiovascular disease, with primary arrhythmogenic disorders, atherosclerotic events, cardiomyopathies and myocarditis as main contributors. The aim of this autopsy study was to determine the contribution of cardiovascular disease to unexpected death in minors.Methods and resultsIn the Netherlands, systematic investigation of all cases of unexplained death in minors was compulsory in a nationwide governmental project during a 15-month period. Autopsies were performed according to a standardised protocol (autopsy rate 85%). A cardiovascular cause of death was found in 13/56 cases (23%). In the group <1 year, the main cardiovascular causes were various congenital defects (n = 3) and myocarditis (n = 2). In the 1–9 year group, no cardiovascular causes were found. In the 10–14 year group, hypertrophic cardiomyopathy (n = 1) and ruptured ascending aortic aneurysm (n = 1) were among the observed cardiovascular causes. In 14/56 (25%) cases autopsy revealed no structural abnormalities that could explain the sudden death, mostly in the group <1 year.ConclusionThis national cohort with a high autopsy rate reveals a high incidence (23%) of cardiovascular diseases as the pathological substrate of sudden unexpected death in children. Another high percentage of minors (25%) showed no structural abnormalities, with the possibility of a genetic arrhythmia. These findings underline the importance of systematic autopsy in sudden death in minors, with implications for cardiogenetic screening of relatives.Electronic supplementary materialThe online version of this article (10.1007/s12471-018-1152-y) contains supplementary material, which is available to authorized users.

Investigating the Genetic Causes of Sudden Unexpected Death in Children Through Targeted Next-Generation Sequencing Analysis.

Inherited arrhythmia syndromes are responsible for a significant portion of autopsy-negative sudden unexpected death (SUD) cases, but molecular autopsy used to identify potentially causal variants is not routinely included in SUD investigations. We collaborated with a medical examiner's office to assist in finding a diagnosis for their autopsy-negative child SUD cases. 191 child SUD cases (<5 years of age) were selected for analyses. Our next generation sequencing panel incorporated 38 inherited arrhythmia syndrome candidate genes and another 33 genes not previously investigated for variants that may underlie SUDY pathophysiology. Overall, we identified 11 potentially causal disease-associated variants in 12 cases, for an overall yield of 6.3%. We also identified 31 variants of uncertain significance in 36 cases and 16 novel variants predicted to be pathogenic in silico in 15 cases. The disease-associated variants were reported to the medical examiner to notify surviving relatives and recommend clinical assessment. We have identified variants that may assist in the diagnosis of at least 6.3% of autopsy-negative child SUD cases and reduce risk of future SUD in surviving relatives. We recommend a cautious approach to variant interpretation. We also suggest inclusion of cardiomyopathy genes as well as other candidate SUD genes in molecular autopsy analyses.

Sudden unexplained death in children

Sudden unexplained death in childhood (SUDC) is the fifth most common category of deaths in toddler-aged children,1Centers for Disease Control and Prevention, National Center for Health StatisticsMultiple Cause of Death 1999–2015. 2016http://wonder.cdc.gov/mcd-icd10.htmlGoogle Scholar yet few paediatricians have heard of it. Unlike sudden infant death syndrome (SIDS) that affects infants aged 1–12 months, children die from SUDC suddenly and unexpectedly on or after their first birthday. In the USA, about 400 children aged 1–18 years die each year with no cause found despite an autopsy and investigation.1Centers for Disease Control and Prevention, National Center for Health StatisticsMultiple Cause of Death 1999–2015. 2016http://wonder.cdc.gov/mcd-icd10.htmlGoogle Scholar, 2Krous HF Chadwick AE Crandall L Nadeau-Manning JM Sudden unexpected death in childhood: a report of 50 cases.Ped Develop Pathol. 2005; 8: 307-319Crossref PubMed Scopus (57) Google Scholar These deaths have evaded the awareness of the public, as well as the public health and medical research communities. Each year, SUDC claims 28 000 potential life-years in the USA and 700 000 potential life-years worldwide,3Centers for Disease Control and Prevention, National Center for Health StatisticsMultiple Cause of Death. 1999–2015http://wonder.cdc.gov/mcd-icd10.htmlGoogle Scholar but education and research efforts to address the phenomenon are essentially non-existent. By comparison, SIDS claims about 1570 lives per year in the USA and has received more than US$500 million from the National Institutes of Health (NIH) and Centers for Disease Control and Prevention (CDC) in the past 20 years.4US National Institutes of HealthEstimates of funding for various research, condition, and disease categories (RCDC). Feb 10, 2016https://report.nih.gov/categorical_spending.aspxGoogle Scholar SUDC has never received targeted funding from either of these institutes. A search of the US National Library of Medicine reveals more than 11 000 research papers regarding SIDS and more than 20 000 for sudden death in adults, but fewer than 30 for SUDC. The funding and research disparity between SIDS and SUDC exceeds 500 to 1, yet the frequency of deaths is 4 to 1. SUDC kills more children aged between 1–18 years than bicycle accidents (97 per year) and fires and burns (280 per year) combined in the USA. Hundreds of millions of dollars and enormous public health resources are allocated to address SIDS, bicycle safety, and fire prevention, and are well justified.4US National Institutes of HealthEstimates of funding for various research, condition, and disease categories (RCDC). Feb 10, 2016https://report.nih.gov/categorical_spending.aspxGoogle Scholar So, why is there a lack of funding to understand and prevent SUDC? No public health official knows the precise incidence of SUDC. Based on US data from the CDC in 2015, we estimate a minimum of 393 deaths, but no specific International Classification of Disease (ICD) code exists for a death that has an unknown cause despite comprehensive investigation.1Centers for Disease Control and Prevention, National Center for Health StatisticsMultiple Cause of Death 1999–2015. 2016http://wonder.cdc.gov/mcd-icd10.htmlGoogle Scholar SIDS has its own code, allowing comparison of trends over time and assessment of public health interventions. In Ireland, the only country to track SUDC cases, the incidence has more than doubled in the past 15 years, while the incidence of SIDS—like in many other countries—declined by more than half.5CM McGarvey, M O'Regan, J Cryan, et al., Sudden unexplained death in childhood (1–4 years) in Ireland: an epidemiological profile and comparison with SIDS. Arch Dis Child; 97: 692–97.Google Scholar SUDC most frequently occurs in boys aged 1–3 years who are born full term as singletons (63–64%).2Krous HF Chadwick AE Crandall L Nadeau-Manning JM Sudden unexpected death in childhood: a report of 50 cases.Ped Develop Pathol. 2005; 8: 307-319Crossref PubMed Scopus (57) Google Scholar, 5CM McGarvey, M O'Regan, J Cryan, et al., Sudden unexplained death in childhood (1–4 years) in Ireland: an epidemiological profile and comparison with SIDS. Arch Dis Child; 97: 692–97.Google Scholar Such deaths usually occur during sleep (>95%) and are unwitnessed (>90%); children are often found unresponsive in the prone position (75%), and with their faces down (50%).6Hesdorffer DC Crandall LA Friedman D Devinsky O Sudden unexplained death in childhood: a comparison of cases with and without a febrile seizure history.Epilepsia. 2015; 56: 1294-1300Crossref PubMed Scopus (38) Google Scholar More than 40% of deaths occur during the winter.6Hesdorffer DC Crandall LA Friedman D Devinsky O Sudden unexplained death in childhood: a comparison of cases with and without a febrile seizure history.Epilepsia. 2015; 56: 1294-1300Crossref PubMed Scopus (38) Google Scholar The most common medical history is febrile seizures, reported in about 30% of cases.2Krous HF Chadwick AE Crandall L Nadeau-Manning JM Sudden unexpected death in childhood: a report of 50 cases.Ped Develop Pathol. 2005; 8: 307-319Crossref PubMed Scopus (57) Google Scholar, 6Hesdorffer DC Crandall LA Friedman D Devinsky O Sudden unexplained death in childhood: a comparison of cases with and without a febrile seizure history.Epilepsia. 2015; 56: 1294-1300Crossref PubMed Scopus (38) Google Scholar SUDC cases associated with febrile seizures tend to be reported in older children (median age 24 vs 19 months for SUDC cases without a history of febrile seizures; p=0·03).6Hesdorffer DC Crandall LA Friedman D Devinsky O Sudden unexplained death in childhood: a comparison of cases with and without a febrile seizure history.Epilepsia. 2015; 56: 1294-1300Crossref PubMed Scopus (38) Google Scholar Autopsy results often reveal minor pathological findings, but they do not explain the death. Subtle abnormalities in the hippocampus have been identified in SUDC and SIDS cases.7Hefti MM Cryan JB Haas EA et al.Hippocampal malformation associated with sudden death in early childhood: a neuropathologic study: part 2 of the investigations of The San Diego SUDC Research Project.Forensic Sci Med Pathol. 2016; 12: 14-25Crossref PubMed Scopus (33) Google Scholar, 8Ackerman MJ Andrew TA Baker AM et al.An association of hippocampal malformations and sudden death? We need more data.Forensic Sci Med Pathol. 2016; 12: 229-231Crossref PubMed Scopus (9) Google Scholar In SUDC, these neuropathological findings are most often reported in children with febrile seizures, which can result from interictal epileptiform discharges in hippocampal slices and seizures in animals studies.9Chai X Münzner G Zhao S et al.Epilepsy-induced motility of differentiated neurons.Cereb Cortex. 2014; 24: 2130-2140Crossref PubMed Scopus (34) Google Scholar These neuropathologies are usually seen in the temporal lobes of patients with epilepsy.10Blümcke I Kistner I Clusmann H et al.Towards a clinico-pathological classification of granule cell dispersion in human mesial temporal lobe epilepsies.Acta Neuropathol. 2009; 117: 535-544Crossref PubMed Scopus (116) Google Scholar We suspect that these hippocampal findings result from seizures and interictal epileptiform activity and provide a clue to the mechanism of death in some cases (seizure-induced postictal depression of arousal and respiratory and cardiac function), but these changes are neither the cause of seizures nor proven related to death. In some children, hippocampal or neocortical malformations can cause epilepsy, which increases risk of sudden death. We found that about 10% of children who died from SUDC have pathogenic de novo or inherited mutations in cardiac channel and epilepsy genes (unpublished data). However, in most cases, the cause of death remains unknown. Epidemiological studies on environmental risk factors and systematic genetic studies are needed, as well as studies on how consistently and accurately medical examiners and coroners investigate and identify SUDC. More research is needed to understand SUDC. Medical examiners and coroners need to standardise investigations and reporting of sudden childhood deaths. The pressure to identify a single cause of death with insufficient evidence leads to underestimations of SUDC incidence and buries an opportunity to investigate what we do not understand under a false conclusion. SUDC has also escaped the spotlight because it is outside the domain of any medical specialty to champion its understanding and prevention. SUDC cases are no one's patient—falling into the limbo between paediatricians and medical examiners. These deaths strike seemingly healthy children unpredictably, and prevention strategies do not exist. With a rate of 1·4 in 100 000 toddlers aged 1–4 years, most paediatricians do not encounter a case of SUDC in their career.1Centers for Disease Control and Prevention, National Center for Health StatisticsMultiple Cause of Death 1999–2015. 2016http://wonder.cdc.gov/mcd-icd10.htmlGoogle Scholar Families who experience SUDC are understandably overwhelmed by grief, but they also face paralysing medical uncertainty for their family's safety, and are often simultaneously investigated by law enforcement to ensure a crime has not been committed.11Walsh B Investigating child fatalities: portable guide to investigating child abuse. US Department of Justice, Washington, DC2005https://www.ncjrs.gov/pdffiles1/ojjdp/209764.pdfGoogle Scholar SUDC remains under the radar of public health and research communities. It is time that the medical establishment charts a successful path to understand and prevent sudden death in children. LC reports personal fees from the SUDC Foundation outside the submitted work and holds voluntary positions at the SUDC Foundation as President and Executive Director. OD is a member of the Scientific Advisory Board for the SUDC Foundation and the Principal Investigator for the SUDC Registry and Research Collaborative.

Suicide patterns in children and adolescents: a review from a pediatric institution in England.

Suicide is a catastrophic event to both families and communities yet it is potentially preventable. This study aims to determine incidence and patterns of suicide in children and young adolescents in our region, raise awareness of this entity as a potentially preventable cause of death in this age group, and identify its possible associated risk factors. We retrospectively reviewed suicide cases presenting as sudden unexpected death in children and adolescents that underwent coronial post-mortems at our institution. This is the largest pathological review of completed suicide in children and young adolescents within a single institution in the United Kingdom. We identified 23 suicide cases during a 12year period from 2003 to 2015, in which 18 cases (78%) were male and 5 cases (22%) were female. The age range was from 8 to 16years (mean age 12.82 +/- 2.52 SD). With the exception of one case, all of the victims were Caucasian. The majority, 19 cases (81%), were found dead inside their place of residence, 15 of whom were discovered in their own bedrooms. Twenty-one cases (91%) died from neck compression due to hanging; 6 cases (26%) had used the cord of a dressing gown and 5 (22%) opted to use a belt as the ligature. Two cases (9%) that died from multiple-drug toxicity were female. In 7 cases (30.5%) there was evidence of self-harm and in 3 cases (13%) there was a history of previous suicide attempts. Petechial hemorrhages were found at autopsy in more than half of hanging victims and only three cases (14%) displayed dual distribution of post-mortem hypostasis (back and legs). Seven victims (30.5%) left some form of suicide message to family members and friends, 2 of which wrote the message on their arm. Parental separation, conflict with parents, and depression, were common amongst decedents prior to committing suicide. Substance abuse was uncommon in suicide within our cases. Valuable information is available from thorough review of suicide data in children and young adolescents from a single institution. Pathologists and clinicians can play crucial roles in identifying potential risk factors that may contribute to prevent future deaths.

Sudden and unexpected childhood deaths investigated at the Pretoria Medico-Legal Laboratory, South Africa, 2007-2011

Background.  Sudden and unexpected death is well known to occur in infants, and although sudden deaths are less frequent after the first birthday, they still account for a significant proportion of childhood deaths. In 2009, 1.9% of the total deaths in the USA were childhood deaths. In South Africa (SA) this proportion was much higher at 11.85%. According to the law, sudden and unexpected deaths are generally investigated as unnatural deaths. Establishing an exact underlying anatomical cause of death will depend on available resources and can be difficult in a substantial proportion of cases. Methods.  A retrospective descriptive case audit was conducted at the Pretoria Medico-Legal Laboratory (PMLL), SA, from 1 January 2007 through to 31 December 2011. All children aged 1 - 18 years who died suddenly and unexpectedly were included. Results.  Ninety-eight cases were identified, which constituted nearly 1% of total admissions to the PMLL. The majority of the deaths were of children aged 1 - 5 years, and the male/female ratio was 1.04:1. In the largest proportion of cases ( n =28, 28.6%), the medicolegal investigation, including autopsy and ancillary investigations, did not establish an underlying anatomical cause of death. In the cases where a cause of death was established, pneumonia was the most common diagnosis ( n =22, 22.4%). Conclusion.  The fact that the cause of the largest proportion of deaths could not be ascertained emphasises the need for consideration of additional investigative techniques, such as molecular/genetic screening, which have provided an underlying cause of death in a significant number of cases in other countries. There is a lack of published research on the causes and incidence of sudden unexpected deaths in children in SA, and further research in this area is needed.

Sudden and unexpected childhood deaths investigated at the Pretoria Medico-Legal Laboratory, South Africa, 2007 - 2011.

Sudden and unexpected death is well known to occur in infants, and although sudden deaths are less frequent after the first birthday, they still account for a significant proportion of childhood deaths. In 2009, 1.9% of the total deaths in the USA were childhood deaths. In South Africa (SA) this proportion was much higher at 11.85%. According to the law, sudden and unexpected deaths are generally investigated as unnatural deaths. Establishing an exact underlying anatomical cause of death will depend on available resources and can be difficult in a substantial proportion of cases. A retrospective descriptive case audit was conducted at the Pretoria Medico-Legal Laboratory (PMLL), SA, from 1 January 2007 through to 31 December 2011. All children aged 1 - 18 years who died suddenly and unexpectedly were included. Ninety-eight cases were identified, which constituted nearly 1% of total admissions to the PMLL. The majority of the deaths were of children aged 1 - 5 years, and the male/female ratio was 1.04:1. In the largest proportion of cases (n=28, 28.6%), the medicolegal investigation, including autopsy and ancillary investigations, did not establish an underlying anatomical cause of death. In the cases where a cause of death was established, pneumonia was the most common diagnosis (n=22, 22.4%). The fact that the cause of the largest proportion of deaths could not be ascertained emphasises the need for consideration of additional investigative techniques, such as molecular/genetic screening, which have provided an underlying cause of death in a significant number of cases in other countries. There is a lack of published research on the causes and incidence of sudden unexpected deaths in children in SA, and further research in this area is needed.

Sudden Death Due to Undiagnosed Rheumatic Heart Disease in a Child

We report the case of a 5-year-old boy who died from complications of rheumatic heart disease with atypical presentation. He was hospitalized for recent inflammatory and neurological symptoms. He was diagnosed with viral encephalitis. He died the day after he was discharged. The macroscopic autopsy findings were unremarkable. Histology revealed typical rheumatic heart disease. Neuropathology showed cerebral infarction due to an embolic event linked with the rheumatic valvulitis. The cause of death was determined as heart failure due to rheumatic heart disease secondary to an undiagnosed acute rheumatic fever. It is related to an autoimmune response to infection with group A streptococcus. It mainly affects children in developing countries. In our case, viral encephalitis was consistent with the medical history and the proper diagnosis was made on histological analysis. Forensic pathologists should consider this diagnosis facing a sudden unexpected death in childhood, even in industrialized countries.

Sudden unexpected death in early childhood: general observations in a series of 151 cases: Part 1 of the investigations of the San Diego SUDC Research Project.

PurposeThe purpose of this study was to determine the major subcategories and clinicopathologic features of sudden unexpected death in young children in a large retrospective cohort, and to confirm the association of sudden unexplained death in children (abbreviated by us for unexplained deaths as SUDC) with hippocampal pathology and/or febrile seizures.MethodsWe undertook analysis of a retrospective cohort of 151 cases, of which 80 % (121/151) were subclassified as SUDC, 11 % (16/151) as explained, 7 % (10/151) as undetermined, and 3 % (4/151) as seizure-related.ResultsThere were no significant differences between SUDC and explained cases in postnatal, gestational, or postconceptional age, frequency of preterm birth, gender, race, or organ weights. In contrast, 96.7 % (117/121) of the SUDC group were discovered during a sleep period compared to 53.3 % (8/15) of the explained group (p < 0.001), and 48.8 % (59/121) of the SUDC cases had a personal and/or family history of febrile seizures compared to 6.7 % (1/15) of the explained group (p < 0.001). Of the explained deaths, 56 % (9/16) were subclassified as infection, 31 % (5/16) cardiac, 6 % (1/16) accidental, and 6 % (1/16) metabolic. Two of the three cases specifically tested for cardiac channelopathies at autopsy based upon clinical indications had genetic variants in cardiac genes, one of uncertain significance. Bacterial cultures at autopsy typically revealed organisms interpreted as contaminants. Two of the four seizure-related deaths were witnessed, with two of the brains from these cases showing generalized malformations. Hippocampal anomalies, including a specific combination we termed hippocampal maldevelopment associated with sudden death, were found in almost 50 % (40/83) of the SUDC and undetermined cases in which hippocampal sections were available.ConclusionsThis study highlights the key role for the hippocampus, febrile seizures, and sleep in SUDC pathophysiology. It also demonstrates the role of known predisposing conditions such as cardiac channelopathies and infections in causing sudden unexpected death in childhood, and the need for improved ancillary testing and protective strategies in these cases, even when the cause of death is established at autopsy.

Sudden unexpected death in children with congenital heart defects.

Congenital heart defects (CHDs) are the most common birth defects and are an important cause of death in children. The fear of sudden unexpected death has led to restrictions of physical activity and competitive sports. The aim of the present study was to investigate the rate of sudden unexpected deaths unrelated to surgery in children 2-18 years old with CHDs and, secondarily, to determine whether these deaths were related to cardiac disease, comorbidity, or physical activity. To identify children with CHDs and to determine the number of deaths, data concerning all 9 43 871 live births in Norway in 1994-2009 were retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospital's Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012, and information for the deceased children was retrieved from medical records at Norwegian hospitals. Among 11 272 children with CHDs, we identified 19 (0.2%) children 2-18 years old who experienced sudden unexpected deaths unrelated to cardiac surgery. A cardiac cause of death was identified in seven of these cases. None of the children died during physical activity, whereas two children survived cardiac arrest during sports. Sudden unexpected death was infrequent among children with CHDs who survived 2 years of age. Comorbidity was common among the children who died. This study indicates that sudden unexpected death in children with CHDs rarely occurs during physical activity.

Investigating Unexpected Child Deaths: An Audit of the New Joint Agency Approach

Key Practitioner Messages The joint agency approach should be used to investigate all unexpected child deaths Practitioners should ensure that final case discussions are not unduly delayed so that parents do not have to wait several months for information on the cause of their child's death. All bereaved parents should be given the opportunity to have a follow‐up appointment with the paediatrician responsible for managing sudden unexpected death in children.

Two Unusual Cases of Sudden Death in Children

We report two unusual cases of sudden unexpected death in children. Histopathologic examination showed intimal fibroplasias, a variant of fibromuscular dysplasia of the right coronary artery associated in both cases with fatty infiltration of the right ventricular myocardium. The significance of this particular combination of two lesions known to induce a sudden death in young people is discussed.

Whole-body post-mortem computed tomography compared with autopsy in the investigation of unexpected death in infants and children

To investigate the contribution of whole-body post-mortem computed tomography (PMCT) in sudden unexpected death in infants and children. Forty-seven cases of sudden unexpected death in children investigated with radiographic skeletal survey, whole-body PMCT and autopsy were enrolled. For imaging interpretation, non-specific post-mortem modifications and abnormal findings related to the presumed cause of death were considered separately. All findings were correlated with autopsy findings. There were 31 boys and 16 girls. Of these, 44 children (93.6 %) were younger than 2 years. The cause of death was found at autopsy in 18 cases (38.3 %), with 4 confirmed as child abuse, 12 as infectious diseases, 1 as metabolic disease and 1 as bowel volvulus. PMCT results were in accordance with autopsy in all but three of these 18 cases. Death remains unexplained in 29 cases (61.7 %) and was correlated with no abnormal findings on PMCT in 27 cases. Major discrepancies between PMCT and autopsy findings concerned pulmonary analysis. Whole-body PMCT may detect relevant findings that can help to explain sudden unexpected death and is essential for detecting non-accidental injuries. We found broad concordance between autopsy and PMCT, except in a few cases of pneumonia. It is a non-invasive technique acceptable to relatives. • Whole-body post-mortem computed tomography (PMCT) is an effective non-invasive method. • Whole-body PMCT is essential for detecting child abuse in unexpected death. • There is concordance on cause of death between PMCT and autopsy. • Whole-body PMCT could improve autopsy through dissection and sampling guidance. • PMCT shows findings that may be relevant when parents reject autopsy.

Sudden unexpected death related to enterovirus myocarditis: histopathology, immunohistochemistry and molecular pathology diagnosis at post-mortem

BackgroundViral myocarditis is a major cause of sudden unexpected death in children and young adults. Until recently, coxsackievirus B3 (CVB3) has been the most commonly implicated virus in myocarditis. At present, no standard diagnosis is generally accepted due to the insensitivity of traditional diagnostic tests. This has prompted health professionals to seek new diagnostic approaches, which resulted in the emergence of new molecular pathological tests and a more detailed immunohistochemical and histopathological analysis. When supplemented with immunohistochemistry and molecular pathology, conventional histopathology may provide important clues regarding myocarditis underlying etiology.MethodsThis study is based on post-mortem samples from sudden unexpected death victims and controls who were investigated prospectively. Immunohistochemical investigations for the detection of the enteroviral capsid protein VP1 and the characterization and quantification of myocardial inflammatory reactions as well as molecular pathological methods for enteroviral genome detection were performed.ResultsOverall, 48 sudden unexpected death victims were enrolled. As for controls, 37 cases of unnatural traffic accident victims were studied. Enterovirus was detected in 6 sudden unexpected death cases (12.5 %). The control samples were completely enterovirus negative. Furthermore, the enteroviral capsid protein VP1 in the myocardium was detected in enterovirus-positive cases revealed by means of reverse transcriptase-polymerase chain reaction (RT-PCR). Unlike control samples, immunohistochemical investigations showed a significant presence of T and B lymphocytes in sudden unexpected death victims.ConclusionsOur findings demonstrate clearly a higher prevalence of viral myocarditis in cases of sudden unexpected death compared to control subjects, suggesting that coxsackie B enterovirus may contribute to myocarditis pathogenesis significantly.

Sudden unexpected death in children with epilepsy: The many faces of fungal pathogenicity

Epilepsy is one of the most prevalent neurological diseases worldwide. The mortality rates are considerably higher in people with epilepsy than would be expected in a healthy population and sudden unexpected death in epilepsy (SUDEP) is the most frequent epilepsy-related category of death. Most children had seizures before the occurrence of the fatal event and autonomic dysfunction has been proposed as mechanisms of sudden death in this population. Is this sense, we raise the question whether is there a possible relationship between SUDEP in children and fungal pathogenicity. Indeed, the role of fungal pathogenicity in the establishment of epilepsy and even in cases of SUDEP has an interesting role in this scenario. Moreover, maternal infections during pregnancy have been associated with an increased risk for several brain disorders, however, this fact is still considered uncertain with respect to epilepsy.Based on this information and considering that maternal-fetal yeast infection is directly associated with an increased risk for epilepsy in childhood and that some patients have medically intractable epilepsy, the chances of these children suffering a fatal event cannot be overlooked by healthcare professionals. Thus, as exact knowledge regarding this association is lacking, some possibilities could be evaluated, and more emphasis on translational research would contribute to further progress to the knowledge of SUDEP.

Sudden death of a child because of an intestinal obstruction caused by a large congenital mesenteric defect

Transmesenteric hernias are internal hernias caused by a congenital defect in the mesentery. They are rare causes of intestinal obstruction, but most commonly affect the small bowel. We report an unexpected death of an infant with a bowel obstruction caused by a congenital mesenteric defect, which was undiagnosed despite visits to three different hospitals. Mesenteric defects are usually 2-3 cm in diameter. At autopsy, we found an oval, 14 × 7 cm congenital defect in the ileal mesentery through which the small bowel had herniated. Diagnosis of such defects remains difficult, even with currently available imaging techniques. Diagnosis is particularly difficult in infants who usually have nonspecific symptoms. Therefore, it is important that sudden unexpected deaths in children undergo full forensic evaluation to establish the precise cause of death. It is also important for forensic physicians to inform clinicians of the risk of such diseases, particularly in emergency situations.