Ascending Aortic Rupture through a Penetrating Atherosclerotic Ulcer: A Rare Cause of Sudden Unexpected Death

Spontaneous rupture of the aorta through an atherosclerotic lesion without preexisting aortic aneurysm, dissection, or history of trauma is very rare. Without prompt aortic repair, all cases result in sudden death with a definitive diagnosis made only intraoperatively or during autopsy. The phenomenon has been uniformly found in individuals with hypertension. The author reports a sudden unexpected death caused by spontaneous rupture of the ascending aorta in a 57‐year‐old man with a history of hypertension. The ascending aortic wall showed a longitudinal intimal tear measuring approximately 1 cm in length and rupture of the ascending aorta through an atherosclerotic ulcer, leading to massive hemopericardium and eventual death. Chronic hypertension and a penetrating atherosclerotic ulcer of the ascending aorta were the apparent underlying etiologies of the aortic rupture in the present case. This case illustrates not only the association between a rupture and a penetrating atherosclerotic ulcer with a silent death, but also raises awareness of possible such deaths.     

Acute Aortic Dissection Diagnosed after Embalming: Macroscopic and Microscopic Findings

A 58‐year‐old man died suddenly in Madagascar and poisoning was suspected. The body was embalmed after death and the general state of preservation was good. We found a major aortic dissection with a large false lumen from the aortic root to the common iliac arteries and a hemopericardium with formalinized blood clot. The intimal tear was on the ascending aorta, and an intramural hemorrhage was noted at the right coronary artery, attesting to a retrograde dissection. Microscopic studies confirmed aortic dissection with extensive intramural hemorrhage and also confirmed the retrograde dissection to the right coronary artery with a reduction of 90% of the true lumen. Classically, aortic dissection occurs in individuals with hypertension and individuals with genetic disorders of collagen formation. The diagnosis is often first established at the postmortem examination. Aortic dissection is therefore dealt with largely in necropsy studies. The usual cause of death is rupture into the pericardial sac. One case of bloodless dissection has been reported but the sudden death was explained by acute myocardial ischemia secondary to dissection of the left coronary artery. In our case, we found major hemopericardium and also intramural hemorrhage at the right coronary artery. We were able to make the diagnosis of aortic dissection and exclude the suspicion of homicide 15 days after death and after embalming.     

Marfan syndrome. A contribution to forensic medicine cases

A 19-year-old school boy suffered from fluctuating uncharacteristic chest pain in the last 20 h before his death. He died unexpectedly within a few minutes of a hemopericardium, which resulted from an aneurysmal rupture of the ascending aorta. The patient's past history as well as the autopsy and ultrastructural findings led to the diagnosis of Marfan's syndrome with alterations of the cardiovascular skeletal system but no ophthalmological involvement ("oligosymptomatic" form of Marfan's syndrome). Appraisal of the cause of death is made more difficult by the fact that medical treatment was undertaken on the same day, whereby subtotal liver crushing was established that had resulted from attempts at resuscitation.     

Giant cell myocarditis with cardiac tamponade: a rare combination

Giant cell myocarditis (GCM) is a rare but fatal disease of idiopathic origin. It results in focal necrosis of myocardium. This is a case report of middle aged Malaysian Indian female who died due to cardiac tamponade due to rupture myocardium and tear in the root of aorta. On naked eye examination, it simply resembled as recent as well as old fibrotic areas of myocardial infarction. She was clinically diagnosed as a case of obstructive cardiomyopathy with atrioventricular block, and was on pace maker. There was subendocardial fibrosis and left ventricular transmural infarction in the left ventricle. On histopathology, this was diagnosed as GCM, there were widespread areas of inflammatory cellular infiltration within the myocardium with multinucleated giant cells and granulomas interspersed with lymphocytes. Microscopic field showed up to 10 multinucleated giant cells. In this case, there were focal areas at multiple locations and caused uneven thickness in the left ventricle wall. Idiopathic GCM is very rare and causation of hemopericardium is the unique feature of this case. In this case the direct link of GCM with aortitis and rupture of left ventricle wall resulting in hemopericardium is shown. This case is documented through macroscopic as well as microscopic photographs in H&E, Ziel-Nelson, and GMS staining.     

Rupture of a giant splenic artery aneurysm. Report of an autopsy case.

We describe an autopsy case of a 61-year-old woman with von Recklinghausen's disease, who died suddenly following intraperitoneal hemorrhage due to the rupture of a giant splenic artery aneurysm. The aneurysm measured 16 x 13 x 5.5 cm--much larger than those in most previous reports. The pancreatic body, which was pressed by the aneurysm, was widely atrophic. In general, splenic artery aneurysms are more frequent in pregnant women or patients with portal hypertension. The pathogenesis of this aneurysm is presumed to be arterial dysplasia, focal arterial inflammation, or portal hypertension, unlike other aneurysms due to arteriosclerosis or syphilis. Since the patient had not been pregnant and had not had liver cirrhosis or arteriosclerosis, the pathogenic factor could not be determined in this case. The relationship between the genesis of the aneurysm and von Recklinghausen's disease was not clear either.     

Sudden death in an infant caused by rupture of a basilar artery aneurysm.

Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. Seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.     

Traumatic basal subarachnoid hemorrhage. Report of six cases and review of the literature

Basal subarachnoid hemorrhage due to rupture of normal extra- and intracranial arteries, in association with minor trauma to the face and neck and alcohol intoxication, has been well described but often goes unrecognized at autopsy. This results in the incorrect classification of the manner of death as natural. Six cases of subarachnoid hemorrhage due to mild-to-moderate blows to the head or neck are presented. All were men in the age range 28-61 years (mean, 38.8 years). Four had blood alcohol levels of 0.09-0.28 g % at autopsy, and five of six were comatose or dead within 30 min of the initiating trauma. Traumatic ruptures of otherwise normal extra- and intracranial arteries were identified in four cases. The site of rupture was not found in one case, and the final case had rupture of a fibrotic intracranial vertebral artery. Multiple sites of incomplete and complete rupture were found in four cases. Postmortem angiography was used in one case to demonstrate the site of rupture prior to removal of the brain. Postmortem angiography and careful gross and histologic examination of extra- and intracranial cerebral arteries is recommended in all cases of basal subarachnoid hemorrhage where minor trauma to the head or neck has occurred prior to collapse or death, especially if the decedent was intoxicated at the time of the trauma.     

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.     

Sudden death in an infant caused by rupture of a basilar artery aneurysm

Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. Seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.     

Traumatic rupture of an abdominal aortic aneurysm associated with the use of a seatbelt

Injury to the abdominal aorta after blunt trauma continues to be a relatively infrequent occurrence. In this report, we describe a case of traumatic rupture of an abdominal aortic aneurysm associated with inappropriate seatbelt use.     

Cardiac rupture following blunt trauma

A case of cardiac rupture following blunt trauma with no additional injuries is described. This can be a difficult diagnosis because of the presence of additional injuries and the lack of clinical symptoms. Here, there was a rupture of the pericardium, all chambers of the heart and the thoracic aorta.     

Sudden death caused by tension pneumothorax after rupture of a thoracic aortic aneurysm. Case report

A rare case of fatal tension pneumothorax is reported. An aged Japanese man with marked subcutaneous emphysema of the neck was found collapsed in a betting office. He was ascertained to have left tension pneumothorax, based on radiographic examinations carried out before his death. At autopsy, severe pneumomediastinum was observed, and the descending thoracic aorta with a ruptured dissecting aneurysm was closely adhered to the left lung pleura. The hemorrhage spread into the pulmonary parenchyma and finally spouted out from the surface of the lung apex. Because the blood loss itself was not fatal in quantity, it is concluded that the patient died of tension pneumothorax caused by a lung penetration from the rupture of an aortic aneurysm.     

Subarachnoid hemorrhage during sexual activity after sildenafil intake: an accidental association?

We report and describe an autopsy case of a man dead for rupture of cerebral artery aneurysm with subsequent subarachnoid hemorrhage after sexual intercourse. Toxicologic analysis demonstrated that he had consumed sildenafil (Viagra). Although subarachnoid hemorrhage has been reported to be associated with sexual intercourse, it is not among the known adverse effects of sildenafil. However, sildenafil has been found to interact with vascular physiology via multiple mechanisms and in most of the vascular districts of the human body. This case provides an example of a very rare association between this drug and a fatal pathologic event and deserves to be added to the existing clinical knowledge about sildenafil and the pathophysiology of the events involved. This knowledge may be helpful in orienting further investigation into the mechanisms of action of sildenafil and their clinical implications.     

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.     

Sudden death due to ruptured pancreaticoduodenal artery aneurysm

Superior pancreaticoduodenal artery aneurysms are rare; their rupture often leads to sudden death. We report a case of a 59-year-old hypertensive man who died of a massive retroperitoneal hemorrhage following rupture of an aneurysm of the superior pancreaticoduodenal artery. We also discuss this unusual vascular lesion and review the pertinent literature.     

Fatal hemorrhage from ruptured varicose veins of the sigmoid colon in liver cirrhosis

A 41-year-old woman died within a few hours one morning due to massive rectal hemorrhage. At autopsy, complete liver cirrhosis, signs of portal hypertension, liquid blood in the entire colon, and high-grade anemia were detected. The source of bleeding was a ruptured submucosal varix in the sigmoid colon, which was almost invisible even microscopically and had been caused by portal hypertension. In the vicinity, and somewhat further away from the rupture site, numerous, greatly dilated veins with wall sclerosis and intimal thickening, as well as paravascular iron deposits, were found as indications that severe hemorrhages had already occurred earlier. To detect the source of bleeding, meticulous inspection and dissection with hematoxylin and eosin, elastica van Gieson, and iron staining, were necessary.     

Fatal retropleural hematoma from a ruptured abdominal aortic pseudo-aneurysm

This paper has reported a case of sudden unexpected death in a 19-year-old man, shot in the abdomen, who was surgically treated and discharged. He died of massive left retropleural hematoma from a spontaneous rupture of a traumatic pseudo-aneurysm of the abdominal aorta. Diagnosis of the retropleural hemorrhage and the aortic pseudo-aneurysm was possible, though not made antemortem, through chest X-rays and ultrasound studies of the abdomen correlated with the patient's symptoms. Early radiologic signs of the hematoma consisted of obliteration of the aortic silhouette on the anteroposterior view and the left primary sulcus on the lateral film by a convex expanding homogenous density whose medial border blended imperceptibly with the mediastinal shadow. On ultrasound study the pseudo-aneurysm appears as semilucent midline shadow anterior to the vertebral column and related to the aorta, giving the latter an appearance of increased diameter.     

Subaortic Pseudoaneurysm of the Left Ventricle Complicating Staphyloccal Endocarditis*

Abstract: Subaortic pseudoaneurysms are rare but can be a cause of sudden death in young individuals. This case report involves a 20‐year‐old Vietnamese male who died suddenly from rupture of a subaortic pseudoaneurysm with resultant hemopericardium with tamponade. He had a history of bicuspid aortic valve with recent but healed Staphylococcal endocarditis. A review of the literature reveals few similar cases and enlightens the association between aortic bicuspid valve, endocarditis, and subvalvular aortic aneurysm. The pathogenesis as well as recent studies that identified aneurysm predisposing genes in patients with bicuspid aortic valve will be discussed.     

外伤性主动脉破裂死亡30例法医病理学研究

目的　探讨外伤性主动脉破裂法医病理学特点。　方法　对华西医科大学法医学院 1983 -2 0 0 3年 3月所做 3 0例外伤性主动脉破裂病理资料进行回顾性研究。　结果　外伤性主动脉破裂以男性青壮年为主 ( 66% ) ,1983 -1993年以高坠为主 ,1994-2 0 0 3年以车祸为主 ,损伤发生部位以主动脉弓部及起始部为常见 ,且绝大多伤者在 3 0min内死亡。　结论　外伤性主动脉破裂发生 ,应以预防为主。     

Fourteen years' survival with an untreated traumatic rupture of the thoracal aorta

Untreated traumatic rupture of the aorta generally results in sudden death. A case with 14 years' survival after an unrecognized traumatic rupture of the aorta is presented. Chronic traumatic aneurysm of the aorta should be kept in mind in a patient who develops sudden symptoms of arteriosclerotic heart disease months to years after a relevant thoracic trauma.