Hidden Coronary Artery Ostium and Sudden Death.

A 1-week-old girl died suddenly and unexpectedly. At autopsy the major finding was of a right dominant coronary artery circulation with an inapparent left coronary artery ostium. After careful examination, an anomalous origin of the left coronary artery was found with the ostium located in the non-coronary cusp immediately adjacent to the commissure of the non- and left coronary cusps. The ostium was of small caliber with an obliquely oriented artery (<45°) with no ostial ridges. The artery coursed anteriorly past the left coronary cusp between the aorta and the left atrial appendage to then follow its usual course inferiorly along the anterior aspect of the left ventricle. The reminder of the autopsy was unremarkable. Death was, therefore, attributed to an anomalous and hypoplastic left coronary artery (and ostium) with an acute angle of take-off. Tracing coronary arteries in the very young may be technically difficult due to their small size, thus identifying the location of ostia is important. This may be difficult when the ostium was located close to a commissure.

Cardiac tamponade - a rare cause of sudden death in autoimmune polyglandular syndrome.

A 27-year-old male died suddenly due to cardiac tamponade arising from pericarditis complicating autoimmune polyglandular syndrome (APS) type 2. He had a history of primary Addison disease and autoimmune hypothyroidism which were corroborated at autopsy. In addition a florid fibrinous pericarditis was associated with 287 g of turbid fluid in the pericardial sac. Although pericarditis with tamponade is a potential complication of APS, it has rarely if ever, been reported as a cause of sudden death. Lethal mechanisms may involve both compressive and restrictive effects.

Changing diagnostic patterns in cases of sudden and unexpected natural death in infants and young children: 1994-2018.

AIM: To determine whether there has been a change in the incidence and type of conditions causing sudden and unexpected natural death in infants and young children in recent years. METHODS: A search was undertaken of pathology records at Forensic Science SA in Adelaide, Australia for all cases of sudden and unexpected natural death in children aged less than 10 years at the time of death over two time periods: 1994-1998 and 2014-2018. RESULTS: Overall, 136 cases were identified consisting of 81 boys and 55 girls (M:F = 16:11; age range 0-9 years). No difference was shown in the numbers of sudden unexplained deaths in infants and young children between the two time periods (80 vs. 56; p = 0.18). A trend was shown for a prominent decrease in SIDS cases (55 vs. 12) with an increase in undetermined cases, <1 year (5 vs. 18). However, when the two categories were combined there was no statistical difference between the two periods (60/80 vs. 30/56) (p = 0.26), although a decrease in numbers had occurred. CONCLUSION: Analysis of numbers of fatalities reported from medicolegal institutes should be undertaken with an awareness of the potential effect of diagnostic shift.

The San Diego SIDS definition-20 Years on.

AIM: As it is now 20 years since the San Diego definition of sudden infant death syndrome (SIDS) was proposed, it is timely to examine the impact of this consensus statement. RESULTS: Concerns at the time were expressed that 'death scene' had been replaced by circumstances of death and so it may have been more useful to have a more inclusive statement of 'death scene, including circumstances of death'. The category of unclassified sudden infant deaths (USID) that was proposed has not been widely adopted. More disturbing, however, is the increasing failure to use either the San Diego or earlier definitions in published research, with recent studies showing that almost two-thirds of peer-reviewed SIDS publications (2019-2021) did not quote or reference internationally accepted definitions. This is a decrease of 33% from the 68% of papers that correctly used SIDS definitions in 2011. The definition is therefore not being uniformly applied and in addition, diagnostic shift is occurring, with more pathologists favouring 'undetermined' over a designation of SIDS. CONCLUSIONS: Given these developments, how can we correctly interpret conclusions relating to SIDS research, and can we accurately monitor trends in SIDS mortality? The authors would suggest that unfortunately, at present we cannot with any precision.

An analysis of the use of standard SIDS definitions in the English language literature over a three-year period (2019-2021).

AIM: To evaluate the use of the three standard definitions of sudden infant death syndrome (SIDS) in the contemporary literature. METHODS: A search was undertaken of the US National Library of Medicine 'PubMed' database for all research articles listed under 'sudden infant death syndrome' or 'SIDS', from 2019 to 2021 accessible through the University of Adelaide library. RESULTS: 564 papers had keywords 'sudden infant death syndrome' or 'SIDS'. 300 papers were not included in the study as they were not written in English, SIDS was not the primary focus, publishing was in predatory journals, or they were Letters to the Editor, commentaries, technical reports, reviews or editorials. The remaining 264 papers consisted of 172 papers without a standard definition, including reports without definitions, with idiosyncratic or incorrectly quoted definitions or with incorrect referencing (65%), and 92 with one of the three standard definitions either quoted in full in the text or correctly referenced (35%). CONCLUSION: Nearly two-thirds of peer-reviewed publications on SIDS listed on PubMed over the past three years have not quoted or referenced accepted definitions. Interpreting research conclusions and monitoring trends in SIDS mortality will be extremely difficult if different population subgroups are being targeted by different research groups.

Idiopathic Lipogranulomatous Orbital Inflammation: A Case Report and Literature Review.

A 57-year old woman presented with a 6-month history of a left red eye associated with diplopia. Examination was remarkable for 2 mm left-sided axial proptosis and restriction on left upgaze and abduction associated with diplopia. She had no previous history of trauma or surgery to the face. Magnetic resonance imaging revealed an enhancing infiltrative mass in the left intraconal space. Biopsy of the anterior orbital fat revealed extensive lipogranulomatous inflammation with no abnormalities noted on flow cytometry, culture, or special stains. The patient was managed with a tapering course of oral prednisolone and two 40 mg intraorbital triamcinolone injections resulting in complete resolution of her presenting symptoms. This represents a rare case of idiopathic lipogranulomatous orbital inflammation.

Buried penis and morbid obesity.

Three morbidly obese men aged 69, 49 and 45 years with respective BMIs of 46.3, 49.1 and 59.3 died suddenly from underlying cardiovascular disease. At autopsy all were found to have marked penile shortening typical of an entity known as "buried penis." This condition arises in adulthood most commonly from morbid obesity as the penile shaft becomes enveloped by encroaching suprapubic adipose tissue. It is associated with infective, obstructive and malignant complications. Histology will be required to identify less-common causative conditions or any inflammatory or premalignant/malignant changes.

Methamphetamine use in homicide victims.

Methamphetamine use is increasing in the Australian population. It has a known association with violent and erratic behaviour and with an increased risk of unnatural deaths. To determine whether victims of homicide have significant exposure to methamphetamine 100 randomly selected de-identified homicide cases from 2012 to 2021 were accessed from the Forensic Science SA (FSSA) Pathology Database to determine the type of homicide and whether methamphetamine was detected in the blood. A total of 76 males and 24 females were found (M:F = 3:1). Of these, 30 victims (30%) had methamphetamine detected in blood, consisting of 26 males and 4 females (M:F = 6:1; male age range 18-53 years, average 36.8 years; female age range 28-63 years average 44.8 years). Levels of methamphetamine ranged from 0.02 to 3.3 mg/L with an average of 0.64 mg/L, with the highest numbers of positive cases occurring in victims of gunshot wounds (45.5%), and the lowest in those with lethal blunt force trauma (23.5%). This study has demonstrated that victims of homicide in an Australian population are more likely to have used methamphetamine than members of the general population. The reasons for this remain unclear although involvement in a drug selling environment may be an important determinant.

Cardiac amyloid deposition and the forensic autopsy - A review and analysis.

Although amyloid material in the heart is not infrequently encountered at autopsy it may on occasion be difficult to determine the significance in terms of possible contributions to the terminal mechanisms of death. A review was undertaken of the literature and of autopsy cases at Forensic Science SA over a 20-year-period (2003-2022) for all cases where significant amyloid material had been encountered on microscopy of the heart. Sixteen cases were found consisting of 11 cases where cardiac amyloid was involved in the lethal episode, and five where it was considered an incidental feature. Of the 11 lethal cases, there were three where cardiac amyloidosis was the cause of death, and eight where it was a contributing factor, along with ischaemic heart disease (N = 7) and bronchopneumonia (N = 1). The age range was 47-92 years, average 78.6 years, with a male to female ratio of 10:1. The weights of the hearts ranged from 496 to 1059 g - average 648 g. Of the five cases where it was considered an incidental finding, the causes of death were blunt head trauma (N = 2), small intestinal ischaemia (N = 2) and small intestinal obstruction (N = 1). The weights of the hearts ranged from 299 to 487 g, average 369 g. The most relevant types of amyloidosis in forensic cases tend to be light chain amyloidosis, senile cardiac amyloidosis and familial amyloid cardiomyopathy. Other forms of amyloidosis that affect the heart, which include reactive amyloidosis, haemodialysis-related amyloidosis and isolated atrial amyloidosis, either have minimal or no clinical significance, or are of uncertain significance. While it may be difficult to determine the prognostic significance of amyloid material at autopsy clinicopathological correlation may provide useful supportive information.

Unexpected death involving the spleen - an overview.

The spleen is located in the left upper quadrant of the abdominal cavity and has both immunological and haematological functions. A variety of quite different entities may be associated with sudden death due to splenic involvement. These range from simple traumatic disruption of the parenchyma of a normal spleen with marked intra-abdominal haemorrhage, to conditions such as malaria and infectious mononucleosis where splenomegaly predisposes to rupture. Haematological diseases such as malignancies and haemoglobinopathies may causes sudden death due to haemorrhage or red cell sequestration. Asplenia or polysplenia may be associated with significant congenital heart disease. Hyposplenia, both structural and functional, may also result in rapid demise from sometimes unusual bacterial infections. Vascular abnormalities causing death include entities such as splenic artery aneurysms. Thus, deaths from splenic pathology may be due to localised issues or may be part of more generalised disease. For this reason the autopsy in cases of splenic-associated deaths must be comprehensive and include special testing such as microbiological screening and/or haematologic/genetic evaluation.