Sudden Unexpected Deaths Due to Intracranial Meningioma: Presentation of Six Fatal Cases,Review of the Literature,and A Discussion of the Mechanisms of Death

Deaths due to meningiomas are routinely diagnosed in clinical practice because this neoplasm tends to present with the typical progression of neurological deficits. On the other hand, sudden unexpected deaths due to meningiomas are rarely described in the literature. The study presents six fatal cases of previously undiagnosed intracranial meningiomas from the Cook County Medical Examiner's Office from 1998 to 2014. The most common explanation of the mechanism of sudden death due to intracranial neoplasms is a rapid increase in intracranial pressure produced by the mass effect of the neoplasm. Other mechanisms of death include acute intracranial and intratumoral hemorrhage, and benign neoplasms that grow in the vicinity of vital centers altering neural discharge in autonomic pathways leading to cardiac suppression or lethal arrhythmia. Forensic pathologists must keep in mind that sudden unexpected death caused by intracranial meningiomas, although extremely rare, may be encountered in the forensic setting.     

Aortic Dissection and Sudden Unexpected Deaths: A Retrospective Study of 31 Forensic Autopsy Cases

Acute aortic dissection (AAD) is the most common cause of sudden unexpected death related to aortic diseases. A retrospective study of 31 sudden unexpected deaths caused by AAD was conducted at Xi'an Jiaotong University Forensic Center from 2001 to 2012. We summarized the forensic characteristics of AAD and assessed the clinically diagnostic accuracy of AAD. The characteristics of sudden unexpected death due to AAD were male predominant (male: female = 6.7:1), relatively young with the mean age of 44, and predominance of type A dissection (77.4%). Cardiac tamponade was the most frequent cause of sudden death (87.1%). Of the 31 cases, 26 (83.9%) patients were not recognized clinically and were misdiagnosed with acute myocardial infarction, coronary artery disease, cholecystitis, acute gastroenteritis, renal/urinary lithiasis, or acute pancreatitis. In summary, AAD can be difficult to recognize, diagnosis is therefore sometimes delayed or missed. The medicolegal death investigation can help physicians have a better understanding of AAD.     

Littre hernia--a rare cause of unexpected death in the elderly

A rare case of sudden and unexpected death is reported in an 87-year-old woman who was found dead at her home. At autopsy, the most striking finding was of a right-sided direct inguinal hernia containing a 20 mm infarcted Meckel diverticulum, with proximal small intestinal obstruction. Significant stenosing atherosclerosis was present in all three major epicardial coronary arteries, but with no histological evidence of acute or chronic ischemic myocardial damage. Death was attributed to small intestinal obstruction due to direct inguinal herniation (and infarction) of a Meckel diverticulum (a Littre hernia), complicating ischemic heart disease. Although Littre hernias are not a reported cause of sudden death in the elderly, congenital gastrointestinal anomalies may rarely play a significant role in terminal episodes well beyond childhood years. The autopsy assessment of sudden death at any age involves detailed examination of the entire length of the intestinal tract.     

Sudden Infant and Early Childhood Death and Sinus of Valsalva Pseudoaneurysms

Two cases of occult pseudoaneurysms of the sinus of Valsalva (SoV) are reported to demonstrate different etiologies and lethal mechanisms. A previously well 9‐month‐old boy who suffered an unexpected fatal cardiorespiratory arrest was found at autopsy to have a ruptured pseudoaneurysm of the left SoV resulting in lethal hemopericardium. A 13.5‐month‐old boy died soon after presenting with a 24‐h history of poor feeding and reduced urine output. At autopsy, destruction of the noncoronary cusp of the aortic valve by bacterial endocarditis was found with extension of the inflammation into a pseudoaneurysm of the SoV with surrounding abscess formation involving the atrioventricular node. Death was most likely due to an arrhythmia. Pseudoaneurysms of the SoV are exceedingly rare but may result in sudden/unexpected death in infancy and early childhood from rupture or involvement of the conduction system or myocardium. Detailed cardiac examination is essential in all unexpected deaths in early life.     

"Spontaneous" coronary artery dissection. The challenge of detection, the enigma of cause

Sudden death secondary to acute dissection of a coronary artery is a rare, but increasingly recognized, cause of sudden, unexpected death in apparently healthy persons. It has been reported more frequently in women and has been associated with sudden death during the puerperium. It has also been reported that these involved coronary vessels contain increased numbers of eosinophils and often show areas of cystic medial necrosis. In this article, we report a case of sudden death in a 47-year-old white woman due to dissection of the distal segment of her left anterior descending coronary artery. There was marked involvement of the coronary arterial walls with cystic degeneration of the media with accumulation of glycosaminoglycans as demonstrated by Alcian blue staining. There was no eosinophilic infiltrate within the arterial walls. This case is unusual in that this woman's mother and brother both have had aneurysms, which stresses the importance of not only searching carefully for these lesions, but also of obtaining family history in such cases.     

Evaluation of diagnostic tools applied in the examination of sudden unexpected deaths in infancy and early childhood

During the period between 1984 and 1999, 309 cases of sudden unexpected death in infancy and early childhood (0-3 years) were investigated at the Institute of Forensic Medicine in Oslo. In 73 cases, an explainable cause of death was found. In this non-sudden infant death syndrome (SIDS) group, 42 cases were due to disease, 14 to accidents, 7 to neglect/abuse and 10 cases were due to homicide. In 43 cases, there were pathological findings at the autopsy or suspect features in the history and/or circumstances, which were, however, insufficient to explain death ("borderline" SIDS). In the remaining 193 cases, nothing of significance was detected ("pure" SIDS).The purpose of the present study was to evaluate the importance of the different diagnostic tools used in diagnosing non-SIDS and borderline SIDS cases. The definition of SIDS requires a negative history as well as a negative autopsy result. Thus, the following variables were analysed: circumstances, medical history and autopsy, which included a gross pathological investigation, histology, neuropathology, microbiology, radiology and toxicology. In diagnosing deaths due to disease, histology, neuropathology and microbiology were the most important diagnostic tools. In contrast, information about the circumstances of death and the gross pathological findings at autopsy most often revealed the cause of death in accidents and cases of neglect/abuse and homicide.Following the drop in SIDS rate in Norway after 1989, the share of pure SIDS in proportion to the total population of sudden unexpected deaths in infancy and early childhood has decreased. The increasing proportion of non-SIDS and borderline SIDS cases presents a challenge to improve the quality of the investigation in cases of sudden death in infancy and early childhood.     

Association of right coronary artery hypoplasia with sudden death in an eleven-year-old child

Congenital coronary artery abnormalities are a rare but well-documented cause of sudden and unexpected death in the pediatric age group. Most reported cases involve both an aberrant origin and course of the abnormal vessel. A case of unexpected death occurring in an otherwise healthy eleven-year-old boy, who had been previously investigated for sudden collapse, is described. The major finding at autopsy was marked disparity in diameter between the coronary arteries due to diffuse hypoplasia of the right coronary artery. This case demonstrates the importance of meticulous examination of the coronary artery system in cases of sudden death in childhood so that significant reduction in luminal cross section will be adequately documented. The possible role played by this finding in the etiology of sudden death is discussed.     

Neoplastic disease in a medicolegal autopsy material. A retrospective study in northern Sweden

Only a small fraction of sudden unexpected deaths are caused by neoplastic disease and thus subject ot medicolegal autopsy. The medicolegal autopsy forms an opportunity to study not only medically diagnosed and treated neoplasms, but also the natural evolution of untreated disease. In a series of 7,020 consecutive medicolegal autopsies in northern Sweden, we found 171 cases with malignant and/or intracranial neoplasms. In 41 cases, sudden death was caused by previously unknown tumors. The most common mechanisms of death in this group were disseminated cancer, intracranial tumors, pulmonary thromboembolism, hemoptysis, and aspiration of blood, and the most common locations were the bronchi and the lung. In some of these cases, the mechanism was sometimes dramatic, raising a question of violent death or intoxication. In 30 cases, sudden unexpected death was caused by previously known tumors, and also in this group disseminated cancer was the most common cause of death, and the most common locations were the bronchi and the lung. In 22 cases, tumors were found suicidal cases; in 14 of these, the tumor was considered to be a major causative factor to the suicide, while in eight cases the tumor was considered to be an incidental finding. The expected number of cancers in the 1,060 suicides investigated in this series was 27, according to the official cancer prevalence data. Thus, a possible over-representation of suicides among persons with cancer seems doubtful and needs further exploration.     

Sudden, unexpected death due to undiagnosed frontal glioblastoma in a schizophrenic patient

The incidence of sudden death due to undiagnosed primary intracranial tumor is low in forensic autopsy. We report a case of a 48-year-old white male, known to be a schizophrenic patient for several years, and in whom a medico-legal autopsy disclosed a large, previously undiagnosed, bilateral frontal glioblastoma infiltrating the genu of corpus callosum. We emphasize the importance of performing complete autopsy, including a thorough neuropathological examination, in all cases of sudden unexpected death, especially in those cases in which no extracerebral cause of death had been established and whose clinical history was primarily of a psychiatric nature.     

Dissecting coronary artery aneurysm: a report of two cases

Two cases of sudden, unexpected death resulting from coronary artery dissection have been reported. Since sudden and unexpected death falls within medical examiners' jurisdiction, the systematic autopsy examination of such cases offers an opportunity to evaluate this entity as well as other rare causes of natural death. A review of the literature concerning the subject has been presented.     

Sudden and Unexpected Death in Three Cases of Ehlers‐Danlos Syndrome Type IV*

Abstract: Ehlers–Danlos syndrome (EDS) type IV is a connective tissue disorder characterized by the inability to produce sufficient amounts of collagen or a defect in the structure of collagen. The most serious complications include a rupture of a viscus or vascular rupture with or without mural dissection. Death may result from internal hemorrhage. This report describes three cases of sudden and unexpected death caused by EDS type IV. Two cases involved hemothorax as a result of dissection of the subclavian artery and aorta, respectively. The third case represented spontaneous pulmonary rupture and hemorrhage. A detailed family history should be sought, and additional specimens collected to confirm the diagnosis, including skin fibroblasts for collagen testing and blood for DNA testing. The forensic pathologist should consider the possibility of EDS type IV upon discovery of spontaneous visceral or arterial rupture and should alert the family members of this hereditary and potentially fatal condition.     

Mechanisms of unexpected death in tuberous sclerosis

Tuberous sclerosis complex is a protean autosomal dominant disorder characterized by multifocal tissue lesions arising from defects in cellular migration, proliferation, and differentiation. It has an association with sudden death. In the current study, review of all cases of sudden death due to tuberous sclerosis was undertaken at the Forensic Science Centre in Adelaide, Australia from 1991 to 2001, in addition to an analysis of cases from the literature. There were two local cases where unexpected death had occurred in individuals with known tuberous sclerosis, involving a 31-year-old male (epilepsy), and a 24-year-old female (massive hemorrhage into a renal angiomyolipoma). Fatal mechanisms in cases of tuberous sclerosis may be associated with underlying cardiovascular, renal and cerebral abnormalities. Sudden death may be due to cardiac arrhythmia, epilepsy, and intra-tumoral hemorrhage with additional complications including cardiac outflow obstruction, obstructive hydrocephalus, aneurysm rupture, and spontaneous pneumothorax. An awareness of the highly variable tissue manifestations of tuberous sclerosis and the mechanisms that may be responsible for death is necessary to establish correctly the diagnosis in occult cases (possibly with molecular confirmation), and to chart accurately organ changes in individuals with established disease.     

Sudden death due to unsuspected coronary vasculitis.

Coronary artery vasculitis is a well-recognized complication of polyarteritis nodosa and is occasionally seen in other forms of systemic vasculitis. However, involvement of the major epicardial coronary arteries leading to myocardial infarction and death is uncommon. Isolated coronary arteritis is even more rare. We report three cases of sudden death due to myocardial ischemia associated with arteritis of the major coronary arteries. All three decedents were previously healthy young to middle-aged men who had died suddenly after complaints of chest pain and shortness of breath. The autopsy findings and differential diagnoses are presented. Such cases are of particular interest to the medical examiner because of the sudden, unexpected nature of the deaths. An approach to the correct diagnosis is discussed.     

Sudden death associated with a multifocal type II hemangioendothelioma of the liver in a 3-month-old infant

Sudden unexpected death in an infant caused by or associated with neoplasm is rare. We describe a case of a sudden death in an apparently healthy 3-month-old female, in which the autopsy revealed a multiple type II infantile hepatic hemangioendothelioma (IHE). This uncommon tumor has, untreated, a relatively high mortality rate, mainly due cardiac failure resulting from massive arteriovenous shunts, but the association with sudden infant death is very rare.     

Sudden death due to undiagnosed Wilms' tumor in an adult

Sudden unexpected deaths due to natural causes constitute a large number of cases encountered by the forensic pathologist. In a majority of such cases, heart disease is responsible for sudden death. Rare disease entities resulting in sudden death are occasionally encountered and may not fit the classic epidemiological profile. We present a case of sudden death due to a previously undiagnosed Wilms' tumor (WT) in an adult. The pathology of WT is discussed, as is the topic of sudden death due to previously unrecognized malignancy.     

Unexpected childhood death due to hemolytic uremic syndrome

Two cases of unexpected childhood death due to hemolytic uremic syndrome are reported. A 21-month-old girl who was discovered dead in bed following a short illness was found at autopsy to have overwhelming sepsis resulting from transmural colitis. Escherichia coli serotype 0157A was isolated from the intestine, and renal changes of hemolytic uremic syndrome were found. A 4-year-old girl died suddenly in hospital from intracranial hemorrhage while being treated for hemolytic uremic syndrome-related renal failure. Culture of urine and feces grew verocytotoxin producing E. coli. These cases demonstrate that hemolytic uremic syndrome may be a rare cause of unexpected childhood death and that the diagnosis may not be established prior to autopsy. Postmortem culture of tissues and fluids in cases of suspected sepsis in children may be essential in establishing this diagnosis, because histologic evaluation may be compromised by profound sepsis and tissue putrefaction. Accuracy in diagnosis may have significant public health and medicolegal consequences.     

Spontaneous acute subdural hematoma complicating arachnoid cyst

The overwhelming majority of cases of acute subdural hematoma in the forensic setting occur as a result of head trauma. We report a case of sudden unexpected death in a middle-aged woman with a history of arachnoid cyst who had sudden spontaneous onset of severe headache that was rapidly followed by collapse and death. A postmortem multiple-slice computed tomographic scan showed a large acute subdural hematoma associated with hemorrhage into an arachnoid cyst. Subdural hemorrhage is an uncommon but well-described complication of an arachnoid cyst.     

Sudden death in infancy due to bicuspid aortic valve

Symptoms of bicuspid aortic valve usually occur in the age group of 50-70 years, but rarely, it can also lead to sudden unexpected death in infancy and early childhood. The autopsy of a 2-month-old baby boy, found dead in his cot, revealed the heart weight as 25 g, and the macroscopic examination showed the circumference of the aortic valve consisting of two leaflets as 8 mm. The thickness of the left ventricle, right ventricle, and septum was measured as 8, 7, and 10 mm, respectively. Microscopically, the heart revealed hypertrophic changes of myocytes. Subendocardial areas displayed necrosis of myocytes, and severe and diffuse ischemic changes characterized by loss of myofibers and vacuolization. Interstitial pneumonia was identified in the lungs. Death occurred as a result of a congenital bicuspid aortic valve obstructing the left ventricular outflow tract complicated by lung infection. As there are only a few reported cases in infancy, and congenital bicuspid aortic valve can lead to sudden unexpected death, this case is presented to the forensic community.     

A retrospective analysis of cardiovascular deaths: A 5-year autopsy study of 1045 cases in Antalya,Turkey

This study aims to identify the macroscopic and microscopic changes that occur in the heart in different causes of cardiovascular death and sudden cardiac death in autopsy cases and evaluate the difficulties that a forensic practitioner may encounter during autopsies. All forensic autopsy cases in the Morgue Department of the Council of Forensic Medicine, Antalya Group Administration between January 1, 2015, and December 31, 2019, were examined, retrospectively. The cases were chosen according to inclusion and exclusion criteria, and their autopsy reports were examined in detail. It was determined that 1045 cases met the study criteria, 735 of which were also met the sudden cardiac death criteria. The top three common causes of death were ischemic heart disease (n = 719, 68.8%), left ventricular hypertrophy (n = 105, 10%), and aortic dissection (n = 58, 5.5%). The frequency of myocardial interstitial fibrosis was significantly higher in deaths due to left ventricular hypertrophy than in deaths due to ischemic heart disease and other causes (χ²(2) = 33.365, p < 0.001). Despite detailed autopsy and histopathological examinations, some heart diseases that cause sudden death may still not be detected.