Sudden death due to an unrecognized cardiac hydatid cyst: three medicolegal autopsy cases

Echinococcosis is a human infection caused by the larval stage of Echinococcocus granulosus. The most common sites of infection are the liver and the lungs. Cardiac hydatid cysts are very rare, even in regions where hydatic cysts are endemic (the Mediterranean, South America, Africa, and Australia). It has been reported that cardiac involvement is seen in about 0.5-3% of human echinococcosis cases. Three cases of cardiac hydatid disease that caused sudden death and which were histopathologically diagnosed are reported. Cardiac echinococcosis is rare, but due to its insidious presentation and affinity to cause sudden death, it is important that it be identified in the histopathological examination.     

Sudden death from saccular laryngeal cyst

Laryngeal cysts are benign, uncommon lesions of the larynx that have been reported on rare occasions to cause sudden death in infants and adults by acute airways obstruction. In this report, we document the sudden death of a 36-year-old woman from a previously undiagnosed, asymptomatic laryngeal saccular cyst that presented with acute, and consequent fatal, airway obstruction. Difficulty during intubation, both in theater and in emergency settings, is a frequent presenting problem. This can have significant medicolegal implications in determining possible negligence. The diagnosis, classification, and management of such cysts, and their importance to both the forensic pathologist and clinicians are discussed.     

Diagnostic dilemma of sudden deaths due to acute hemorrhagic pancreatitis

Sudden death due to acute pancreatitis has been rarely determined. A review of 3305 autopsies performed between 1991 and 2001 at the Council of Forensic Medicine found 12 cases (0.36%) with sudden death due to acute hemorrhagic pancreatitis without symptoms. A history of chronic alcohol ingestion was obtained from family in four cases (33%), and no stones were found in the bile ducts or in the gall bladders. During the autopsies, hemorrhage and edema were localized on the head of the pancreas in three cases and the whole pancreas in nine cases. The most common extrapancreatic pathology was found in the lung including pulmonary edema, alveolar hemorrhage, pleural effusion, and pulmonary congestion. There was no correlation between pulmonary and pancreatic damage. It is suggested that the forensic pathologists who are dealing with sudden unexpected death must not ignore the examination of pancreatic and extrapancreatic regions to avoid missing acute pancreatitis.     

Pericardial Cyst: Cause of Sudden Cardiac Death?

Cardiovascular disease is the leading cause of sudden death in the world. The etiology of sudden cardiac death involves a wide range of diseases, but seldom pericardial cysts. A pericardial cyst is an uncommon cyst usually located in the middle mediastinum and rarely in the posterior part. They are usually harmless and asymptomatic. Here, we present a case of a 63‐year‐old woman who presented with dyspnea and hoarseness, but died suddenly after a CT scan was attempted. The detailed forensic pathologic and histologic examination revealed a pericardial cyst located in the posterior mediastinum. Toxicology and biochemistry tests, including tryptase, found no competing cause of death.     

Brain arteriovenous malformation and its implication in forensic pathology

Despite the fact that brain arteriovenous malformations (BAVMs) are a possible cause of sudden and unexplained death, very few papers have discussed their importance in the forensic context. BAVMs consist of tangled masses of tortuous arteries and veins devoid of intervening capillaries that frequently extend from brain parenchyma into the subarachnoid space. Apart from BAVMs, three major groups of vascular malformations of the brain are known: cavernous hemangioma, venous angioma, and capillary telangiectasia. BAVMs and cavernous hemangioma often cause hemorrhages, while venous angioma and capillary telangiectasia are typically asymptomatic. Presented here is the case of a 14-year-old girl who died from a ruptured BAVM. The present case is a reminder that the forensic pathologist should be able to recognize BAVMs and to differentiate it from other types of vascular malformations. Although rare, it is a cause of sudden death not to be overlooked, especially in children.     

Undiagnosed, untreated acute promyelocytic leukemia presenting as a suspicious sudden death

Acute promyelocytic leukemia (APL) is a subtype of acute myelogenous leukemia frequently associated with clotting abnormalities and severe hemorrhagic diathesis. The disease is associated with a high incidence of early fatal hemorrhage. We report the sudden death of a 40-year-old male without significant medical history in which foul play had been initially suspected. A thorough postmortem investigation performed on the decedent lead to the diagnosis of APL. Cause of death was a cerebellar hematoma. Underlying APL should be considered in the differential diagnosis when unexplained bleeding is encountered in a decedent. This case emphasizes the value of routinely collecting bone marrow during an autopsy to enable accurate testing and diagnosis.     

Further observations on the speed of death in hanging

Given that most fatal hangings are suicidal and occur in locations that have been selected to conceal this activity (thus maximizing the chances of a lethal outcome), there has been very little corroboration of the speed with which unconsciousness and death may occur. A 35-year-old male is reported who committed suicide by hanging immediately after talking to his spouse. Police investigations confirmed her reliability as a witness indicating that lethal anoxia in this case had occurred within a very short time (most likely in less than 1 min) of suspension. The speed with which death may result from hanging not only gives an insight into fatal pathophysiological mechanisms, but also provides useful information for situations where a lethal outcome is to be avoided, or is not intended. For example, individuals at risk of suicide who are being monitored in institutional facilities need to be constantly under direct visual surveillance as significant hypoxia can be rapidly induced, parents and caregivers with infants and children in potentially unsafe sleeping environments need to realize how swiftly death or irreversible anoxic brain damage may occur from neck compression, and those who engage in recreational asphyxia should be informed just how quickly a fatal outcome may ensue.     

Sudden death owing to right atrial hemangioma

Primary cardiac tumors are rare, with an autopsy incidence of 0.03%. Seventy-nine percent to 85% of primary cardiac tumors are benign, and of the benign tumors, cardiac hemangiomas account for 5-10% of cases. Most cardiac hemangiomas are asymptomatic and are discovered incidentally at autopsy, or by echocardiography, computerized tomography, or magnetic resonance imaging. We report a case of sudden death owing to cardiac hemangioma in a 22-year-old woman who collapsed while shopping and became unresponsive. The autopsy revealed a hemorrhagic mass on the surface of the right atrium which was infiltrating and replacing the wall of the right atrium; histopathological examination confirmed the tumor was a cavernous hemangioma. Fewer than 20 cases of right atrial cavernous hemangiomas have been reported in English literature, emphasizing the rarity of our case.     

The role of environmental factors in the causation of sudden death in infants: two cases of sudden unexpected death in two unrelated infants who were cared for by the same babysitter

We report two cases of sudden unexpected death in two unrelated African American female infants, 2 months and 4 months old. Both infants were attended to by the same babysitter in the same apartment and died 39 days apart in the same bed and in the same bedroom. The autopsy of the first infant revealed sudden unexplained death in an infant. Toxicologic analysis for carbon monoxide (CO) was not performed because it was not suspected. When the second infant died, investigation into the ambient air quality within the apartment revealed high levels of CO emanating from a poorly ventilated and defective hot water heater, which was located across a hallway from the bedroom where the two babies died. CO saturation levels in the postmortem blood samples of the two babies were elevated and were similar (13% and 14%). Nicotine and cotinine were not detected in the blood sample of the two infants. Cherry-red livor mortis was absent. Acute CO intoxication was determined to be the underlying cause of these two unexpected deaths. These two cases underscore the need to integrate ambient air analysis and postmortem CO analysis as routine components of the comprehensive death investigation of infants who die suddenly and unexpectedly.     

Forensic considerations in cases of neurofibromatosis--an overview

Neurofibromatosis types 1 and 2 are inherited neurocutaneous disorders characterized by a variety of manifestations that involve the circulatory system, the central and peripheral nervous systems, the skin, and the skeleton. Significant reduction in lifespan occurs in both conditions often related to complications of malignancy and hypertension. Individuals with these conditions may also be the subject of medicolegal autopsy investigation if sudden death occurs. Unexpected lethal events may be associated with intracranial neoplasia and hemorrhage or brainstem compression. Vasculopathy with fibrointimal proliferation may result in critical reduction in blood flow within the coronary or cerebral circulations, and aneurysmal dilatation may be associated with rupture and life-threatening hemorrhage. An autopsy approach to potential cases should include review of the history/hospital record, liaison with a clinical geneticist (to include family follow-up), a full external examination with careful documentation of skin lesions and nodules, measurement of the head circumference in children, photography, possible radiologic examination, a standard internal autopsy examination, documentation of the effects of previous surgery and/or chemo/radiotherapy, examination for specific tumors, specific examination and sampling of vasculature (renal, cerebral, and cardiac), formal neuropathologic examination of brain and spinal cord, possible examination of the eyeballs, examination of the gastrointestinal tract, histology to include tumors, vessels, gut, and bone marrow, toxicological testing for anticonvulsants, and sampling of blood and tissue for possible cytogenetic/molecular evaluation if required.     

Sarcoidosis and mechanisms of unexpected death

Sarcoidosis is a multisystem disease of uncertain etiology characterized by multifocal areas of discrete and confluent granulomatous inflammation that may rarely be responsible for sudden and unexpected death. Two cases are reported to demonstrate disparate pathological features in fatal cases, one involving cardiac sarcoidosis, and the other neurosarcoidosis with hypothalamic infiltration. Sarcoidosis in individuals dying suddenly may be completely unrelated to the death, contributory or causal. Cardiovascular causes of sudden death in sarcoidosis include arrhythmias associated with cardiomyopathy and ischemia, ventricular rupture, and cor pulmonale due to pulmonary hypertension; respiratory causes include hemorrhage and upper airway obstruction; central nervous system causes include arrhythmias from infiltration of autonomic centers, epilepsy, and obstructive hydrocephalus from brainstem involvement; and gastrointestinal deaths may be due to hemorrhage from esophageal varices associated with portal hypertension. The diagnosis relies on the demonstration of typical noncaseating granulomas and the exclusion of other infective and environmental diseases with similar histopathological findings.     

Sudden cardiac death due to giant cell inflammatory processes

Granulomatous inflammation of the myocardium may occur in a number of systemic disease processes including those with infectious etiologies such as fungal, mycobacterial and parasitic infections, as well as hypersensitivity reactions, and rarely autoimmune disorders. In many of these disorders, giant cells are components of the inflammatory infiltrate. Systemic granulomatous processes of unknown pathogenesis, most notably sarcoidosis, may also be associated with involvement of the myocardium. Occasionally, these disorders are associated with sudden death due to pathologic involvement of the heart. In contrast, giant cell myocarditis, also known as idiopathic myocarditis, a rare, frequently fulminant and fatal disorder of unknown etiology, is isolated to the heart and lacks systemic involvement. This disorder is most commonly diagnosed at autopsy. We present two cases in which sudden death resulted from a giant cell inflammatory process affecting the myocardium. Both individuals lacked antemortem diagnoses and collapsed at their respective places of employment. These cases compare and contrast the clinical and pathologic issues involved in the differential diagnoses of the subgroup of sudden cardiac deaths resulting from giant cell inflammatory processes that affect the myocardium, as well as the value of histologic examination and immunohistochemical studies.     

急性坏死性胰腺炎与死后胰腺自溶病理形态学变化

目的比较大鼠急性坏死性胰腺炎（acute necrotizing pancreatitis,ANP）与各种急性死亡大鼠死后48h胰腺的病理形态学变化,探讨两者的鉴别点。方法参照文献制作ANP和电击、机械性窒息（勒死）、急性毒鼠强中毒动物模型,采用半定量评分和图像分析定量分析,观察死后48h胰腺大体和光镜下组织病理变化。结果ANP组炎细胞浸润、脂肪坏死、钙沉积3项与其他组相比有显著意义（P〈0．05）。结论炎细胞浸润、脂肪坏死、钙沉积是ANP在普通光学显微镜下最重要的特异性形态学病理变化,对区别其他急性死亡和死后自溶胰腺有特别重要意义．     

Sudden unexpected death due to a previously undiagnosed plasma cell dyscrasia

The plasma cell dyscrasias are a diverse group of disorders characterized by the production of a clonal paraprotein. Sudden death is a recognized complication of the plasma cell dyscrasias, most commonly in individuals with cardiac involvement by amyloidosis. However, the current forensic literature has no reported cases in which sudden death resulted from complications of a plasma cell dyscrasia that was first diagnosed by postmortem histologic examination. We present the case of a woman whose sudden and unexpected death resulted from a seizure. Postmortem examination revealed no evidence of trauma or a grossly identifiable natural disease process that would have accounted for her death. However, microscopic and immunohistologic studies revealed a previously undiagnosed plasma cell dyscrasia, the clonality of which was determined by immunohistochemical studies for immunoglobulin light chains, that was not associated with amyloid deposition. This case elucidates a previously unrecognized cause of sudden unexpected death and illustrates the importance of microscopic studies in selected cases examined in medical examiner/coroner offices.     

Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy

Abstract:  Sickle cell disease (SCD) and sickle cell trait (SCT) can be associated with sudden unexpected death in the pediatric population, usually due to pulmonary complications occurring within the acute chest syndrome (ACS). Musculoskeletal complications can occur and are classically limited to bone infarcts. The occurrence of bone pathology centered upon the epiphyseal growth plate in SCD/SCT is extremely rare, and multiple such injuries in a single patient have not been previously reported. Herein, we describe a case of sudden unexpected death in a 5-year-old child with undiagnosed SCT due to the ACS, with widespread epiphyseal and periosteal bone lesions mimicking multiple inflicted injuries at autopsy. This case highlights the importance of clinicopathological correlation and is the first to describe SCT pathology as a mimic of nonaccidental injury.     

Supine Hypotensive Syndrome as the Probable Cause of Both Maternal and Fetal Death

Abstract: Supine hypotensive syndrome is characterized by severe supine hypotension in late pregnancy, whose clinical presentation ranges from minimal cardiovascular alterations to severe shock, resulting from inferior vena cava compression by gravid uterus. We report a case of a 41‐year‐old 39‐week‐pregnant woman found dead supine. Autopsy revealed the following: cyanosis of the limbs; congestion of the jugular and subclavian veins; abundant abdominal subcutaneous fatty tissue; uterus displacing intestine and diaphragm; collapsed inferior vena cava; both femoral veins dilated and filled with blood; edematous and congested lungs; and placenta 790 g, fetus 3475 g, amniotic fluid 800 cm³. The diagnosis of supine hypotensive syndrome as the probable cause of death is supported by the position of the body and autopsy findings. This syndrome can be considered as the first stage of the physio‐pathological mechanism that led to death in the case presented herein and should be considered by pathologists as a cause of sudden death.     

Sudden and unexpected death from pituitary tumor apoplexy

Pituitary tumor apoplexy refers to a clinical syndrome precipitated by the expansion of a pituitary adenoma by hemorrhage or infarction. Individuals may present with myriad signs, including sudden onset of severe headache, visual changes, altered mental status, cranial nerve palsies, and hormonal dysfunction. This disorder constitutes a medical emergency and warrants an expedited evaluation, diagnosis, and treatment to prevent the potential sequelae of permanent visual loss, endocrine abnormalities, or death. We report a case of sudden death from undiagnosed pituitary tumor apoplexy. The decedent was evaluated by medical personnel on three occasions in the week prior to her death for severe headache, nausea, vomiting, and photophobia. Postmortem examination demonstrated a hemorrhagic infarction of a pituitary adenoma with necrosis and expansion out of the sella turcica. The recognition of and treatment for a patient with pituitary tumor apoplexy requires a rapid multidisciplinary effort. Failure of prompt diagnosis may be fatal and require a medico-legal death investigation for sudden and unexpected death.