Arrhythmogenesis and diagnosis of cardiac sarcoidosis. An immunohistochemical study in a sudden cardiac death

We present an uncommon case of sudden cardiac death in a 34-year-old white woman. She was found lifeless at home by her parents. Three months before death she was recovered at the Emergency Room for chest pain, palpitation and loss of consciousness. Subsequent cardiological evaluation with ECG showed sinusal rhythm, QRS deviation to the left, QS aspect, asymmetric and rounded T waves and slight length of QT. During hospitalization she presented some episodes of supraventricular paroxysmal tachycardia and non-sustained ventricular tachycardia. No echocardiography alterations were found. An anti-arrhythmic treatment was prescribed. Autopsy revealed some fibrotic scarring in the myocardium of left ventricle. The histological examination of the heart revealed diffuse and extensive fibrosis with non-caseating sarcoid granulomas. The lungs, kidneys and lymph node also showed the same non-caseating granulomas. The diagnosis of sarcoidosis with massive and extensive cardiac involvement was established as cause of death.     

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 death due to primary diffuse leptomeningeal gliomatosis

Tumors of the central nervous system are an unusual cause of sudden death. This report describes the sudden death of a presumed healthy 28-year-old woman from primary diffuse leptomeningeal gliomatosis. She presented to an emergency room with headache and vomiting, subsequently became unresponsive and was pronounced dead 14 h later. Autopsy revealed a diffuse extensive infiltrate of well-differentiated astrocytoma in the leptomeninges of the brain and spinal cord without an underlying parenchymal tumor. Primary diffuse leptomeningeal gliomatosis is a rare tumor that arises within the leptomeninges from small neuroglial heterotopic rests that undergo neoplastic transformation. Grossly. this tumor can mimic leptomeningeal carcinomatosis, pachymeningitis, tuberculosis, sarcoidosis, and fungal infections. However, the histologic features of primary diffuse leptomeningeal gliomatosis should allow it to be readily distinguished from grossly similar conditions. The mechanism of death in this case is most likely tumor obstruction of cerebrospinal fluid outflow resulting in the usual complications seen with increased intracranial pressure. Although this tumor is aggressive and is associated with a rapidly progressive fatal course, it has not been previously associated with sudden death.     

Acute poisoning involving the pyrrolidinophenone-type designer drug 4'-methyl-alpha-pyrrolidinohexanophenone (MPHP)

A 36-year-old woman consulted the medical emergency unit of a private health center for abdominal pain and gastroenteritis of 5 days duration. Acute right pyelonephritis was diagnosed. Five hours after admission she became unconscious in a state of clinical shock. She was transferred to an intensive care unit but resuscitation attempts were unsuccessful and she died 3 h later. Three days after death, she was buried in the family vault. Five days after the burial, her husband lodged a complaint with the public prosecutor because he had not received a clear explanation from the physicians concerning the cause of his wife's death. After analysis of the medical records of the deceased by two forensic pathologists, a medicolegal autopsy was ordered by the public prosecutor. The corpse was exhumed and autopsy performed 9 days after death. Massive hemoperitoneum was diagnosed with a macroscopically ruptured subcapsular hematoma. Pathological study confirmed acute right pyelonephritis and demonstrated the precise cause of the hemorrhage: rupture of the hepatic artery at the hilar part, following infectious arteritis which was probably secondary to the acute pyelonephritis. To the best of our knowledge, this is the first published report of such a case.     

Sudden Unexpected Death Due to Graves' Disease During Physical Altercation

We report a case of a 30‐year‐old woman who suddenly collapsed after having a physical altercation with her husband. Despite immediate resuscitation, she died on arrival at the hospital. The victim's parents requested an autopsy because they believed that their daughter was killed by her husband. Postmortem examination revealed that the victim had a diffusely enlarged thyroid gland and cardiomegaly with left ventricular hypertrophy. There was no evidence of significant trauma on the body. Further postmortem thyroid function tests and review of her medical history indicated that her death was due to Graves' disease. To the best of our knowledge, this is the first case reported of sudden death due to cardiac arrhythmia from Graves' disease induced by physical and emotional stress associated with the criminal activity of another person. The autopsy findings are described. In addition, the literature is reviewed and the significance of postmortem evaluation of thyroid hormones in the cases of sudden death is discussed.     

An Exceptional Case of Acute Respiratory Failure Caused by Intra‐Thoracic Gastric Perforation Secondary to Overeating

An 18‐year‐old female patient arrived at the emergency department complaining of abdominal pain and fullness after a heavy meal. Physical examination revealed she was filthy and cover in feces, and she experienced severe abdominal distension. She died in ED and a diagnostic autopsy examination was requested. At external examination, the pathologist observed a significant dilation of the anal sphincter and suspected sexual assault, thus alerting the Judicial Authority who assigned the case to our department for a forensic autopsy. During the autopsy, we observed anal orifice expansion without signs of violence; food was found in the pleural cavity. The stomach was hyper‐distended and perforated at three different points as well as the diaphragm. The patient was suffering from anorexia nervosa with episodes of overeating followed by manual voiding of her feces from the anal cavity (thus explaining the anal dilatation). The forensic pathologists closed the case as an accidental death.     

Sudden death during prolonged religious fasting

Sickle cell trait (SCT) is estimated to occur in 6% to 10% of Africans, and the United Kingdom (UK) prevalence rate has been estimated at 3.2%. Although sudden death in sickle cell disease is well known, its occurrence in SCT is rare and requires extremes of physiological stress. We present a case of a 29-year-old black woman who died suddenly during a period of religious fasting. Her medical history was unremarkable, and there was no family history of sickle cell disease. At postmortem, she was found to be dehydrated, and macroscopically, the main abnormal findings were congested lungs and a small spleen. Histological examination revealed extensive vascular congestion with red blood cell sickling in both lungs, the liver, and the spleen. Electrophoresis on a postmortem blood sample confirmed the clinical suspicion that the patient was a carrier of SCT. The case highlights a novel scenario of SCT associated sudden death. We discuss the potential pathophysiological mechanisms that may have led to the patient's demise. We also remind pathologists to consider this diagnosis as potentially contributing to the cause of death in apparently fit young people of ethnic origin during episodes of physiological stress.     

Asphyxia by tracheobronchial thrombus

Asphyxia secondary to airway obstruction has numerous underlying causes, both acute and chronic. Causes of chronic airway obstruction, such as neoplasms and tracheal scarring, are often clinically apparent well prior to asphyxia. Causes of acute airway obstruction may not be as obvious to clinicians or investigators. These include infections, anaphylactic reactions, status asthmaticus, inhalational injuries, and aspirations, which may result in acute obstruction and sudden death.We report the deaths of 2 individuals, a 43-year-old female and a 78-year-old female, both with adenocarcinoma. The 43-year-old was hospitalized with a stage III, poorly differentiated infiltrating ductal carcinoma of the breast metastatic to the lymph nodes. She was intubated to treat poor respiratory function and acidosis. A bronchoalveolar lavage was consistent with alveolar hemorrhage; no organisms were identified. Blood and "clot" were in her endotracheal tube, so the endotracheal tube was replaced. She became comatose and life support was withdrawn. At autopsy, a large red-gray thrombus obstructed the trachea and extended into the right bronchus. Microscopically, the entire clot was composed of fibrin, red blood cells, and some mucus. Findings of acute respiratory distress syndrome with hyaline membranes were identified. The cause of death was listed as acute respiratory distress syndrome with tracheobronchial thrombus. Experiencing a decline in mental status, the 78-year-old had metastatic adenocarcinoma of unknown primary. She developed sudden respiratory distress and an airway obstruction was discovered. After failure to relieve the obstruction, she decompensated and died. At autopsy, a large, red-gray thrombus obstructed the distal trachea and both bronchi. Microscopically, the thrombus was composed of fibrin, platelets, and red blood cells. The cause of death was asphyxia secondary to airway obstruction by thrombus. We present these 2 unusual cases of asphyxia and review of the literature focusing on asphyxia and the etiology of airway thrombi.     

Myocardial sarcoidosis as a rare cause of sudden cardiac death

Two relatively young women died suddenly due to myocardial sarcoidosis. Necropsy in both cases revealed compact infiltration of the ventricular septum by fibrous tissue. Histologic sections elucidated extensive granulomatous degeneration and giant cells of the Langhans' and foreign body type without central necrosis of the granulomas. Both women had been entirely free of symptoms, but in the second case of a 35-year-old, who had been 6 months pregnant, an ultrasound sonography of the heart had documented a suspicious area in the ventricular septum interpreted as a scar. Further investigations had been postponed until delivery. Isolated myocardial sarcoidosis should be taken into account as one possible cause of sudden death, especially in young people.     

Sudden unexpected infant death due to fibroma of the heart

A 7-month-old previously healthy female infant was found dead in her crib by her mother shortly after having been laid down to sleep following the noontime feeding. Because the child did not suffer from an acute illness and no other evidence pointed to a cause of death, it was initially assumed by the police that she had died of sudden infant death syndrome. At autopsy, however, the cause of death was determined to be cardiac arrhythmia secondary to fibroma of the heart.     

Sudden infant death syndrome: diagnostic practices and investigative policies, 2004

Using a 2004 population-based survey of all US medical examiner and coroner offices, we examined the characteristics of offices accepting an infant death case and calculated the percentage of offices that had death scene investigation or autopsy policies for the investigation of sudden unexpected infant death (SUID). We also calculated the percentage of offices that used and did not use sudden infant death syndrome (SIDS) as a cause of death, and we compared differences in characteristics among those offices.Of medical examiner and coroner offices, 52% did not report an infant death in 2004. Of the 7957 infant deaths reported, 43% occurred in jurisdictions that experienced 1 or 2 infant deaths. Of the offices that used SIDS as a classification, 34% did not have policies for conducting death scene investigations and autopsies for SUID. At least 5% of offices that reported an infant death did not use SIDS as a cause of death classification. These findings have important implications for understanding recent trends in SIDS and SUID. Supporting the implementation of national standards for investigating and certifying infant deaths could provide guidelines for consistent practices in medical examiner and coroner offices.     

The High Court's lost chance in medical negligence: Tabet v Gett (2010) 240 CLR 537

In 2010 the High Court of Australia in Tabet v Gett (2010) 240 CLR 537 determined an appeal in a medical negligence case concerning a six-year-old girl who had presented to a major paediatric hospital with symptoms over several weeks of headaches and vomiting after a recent history of chicken pox. The differential diagnosis was varicella, meningitis or encephalitis and two days later, after she deteriorated neurologically, she received a lumbar puncture. Three days later she suffered a seizure and irreversible brain damage. A CT scan performed at that point showed a brain tumour. As Australia does not have a no-fault system providing compensation to cover the long-term care required for such a condition, the girl (through her parents and lawyers) sued her treating physician. She alleged that, because a cerebral CT scan was not performed when clinically indicated after the diagnosis of meningitis or encephalitis and before the lumbar puncture, she had "lost the chance" to have her brain tumour treated before she sustained permanent brain damage. She succeeded at first instance, but lost on appeal. The High Court also rejected her claim, holding unanimously that there were no policy reasons to allow recovery of damages based on possible (less than 50%) "loss of a chance" of a better medical outcome. The court held that the law of torts in Australia required "all or nothing" proof that physical injury was caused or contributed to by a negligent party. The High Court, however, did not exclude loss of chance as forming the substance of a probable (greater than 50%) claim in medical negligence in some future case. In the meantime, patients injured in Australia as a result of possible medical negligence (particularly in the intractable difficult instances of late diagnosis) must face the injustice of the significant day-to-day care needs of victims being carried by family members and the taxpayer-funded public hospital system. The High Court in Tabet v Gett again provides evidence that, as currently constituted, it remains deaf to the injustice caused by State legislation excessively restricting the access to reasonable compensation by victims of medical negligence.     

Fatal Child Abuse Presenting with Multiple Vertebral and Vascular Trauma

This case study involves the unexplained death of a previously healthy 30‐month‐old child. Reportedly, she was found unresponsive by her foster father following a 2‐hour nap. She was transported to the hospital and died in the emergency room of unknown causes. Blood drawn in the ER showed hemoglobin of 4.3 mg/dL. Postmortem skeletal survey, magnetic resonance imaging (MRI) of the brain, and retinal examinations were negative. The medical examiner assumed jurisdiction of the body. The autopsy showed massive retroperitoneal hemorrhage, transections of the abdominal aorta, and inferior vena cava, and complete tears of the anterior longitudinal ligament of the spine at C5/C6 and L1/L2 with diastases of the vertebral bodies at the corresponding intervertebral disk spaces. The case is believed to be extraordinary due to both the extent of injury that does not match the reported history and the mechanism of vertebral and vascular injuries.     

Hyponatremic seizures as a presenting symptom of child abuse

Poisoning is an uncommon manifestation of child abuse. The intentional administration of water to a child as a form of punishment has rarely been reported as the responsible substance among children who have been poisoned. We describe a case of a 5-year-old girl presenting with severe hyponatremia due to acute water intoxication. The patient was brought to the emergency room in status epilepticus. A history was obtained from the child's mother stating that the patient had been playing outside when she collapsed. She had had no known prior illnesses. Laboratory evaluation included a hemoglobin of 10.1 mg%, glucose of 60 mg%, serum sodium of 107 mEq/l, potassium of 3.2 mEq/l and chloride of 71 mEq/l. A CAT scan obtained approximately 1 h after admission revealed generalized cerebral edema. Careful examination of the skin revealed multiple linear ecchymosis of varying ages on the back and thighs and a hand print on the right flank. In addition, the child demonstrated severe failure to thrive with height, weight and bone age compatible with a 2.5-year-old girl. Appropriate therapy for severe hyponatremia was successfully instituted. For the next 12 h she was deeply somnolent, but the following morning was alert and conversant. She stated that she "would be good if she didn't have to drink any more water". The child's mother subsequently admitted that she frequently used water ingestion as a form of punishment. The child stabilized metabolically and demonstrated rapid in-hospital weight gain. She was placed in foster care at discharge and has had no further hyponatremia or seizures.(ABSTRACT TRUNCATED AT 250 WORDS)     

LAP-banding obesity: a case of stomach perforation, peritonitis, and death

We present a case of peritonitis and death due to the misplacement of a laparoscopic adjustable band inserted through, instead of around, the stomach. This represents the first case in the published literature where a LAP-BAND perforated the stomach, followed by peritonitis and death. The morbidly obese female patient with a history of hypertension and arthritis was 47 years old, 5 feet 6 inches tall, weighed 361 pounds, and had a body mass index of 58.3. She underwent a 2-hour, elective, LAP-band insertion operation to achieve weight loss; 27 hours after band insertion, following the conduction of all FDA-mandated Lap-Band postoperative protocol (including a radiologic Gastrogrografin swallow), the patient was discharged with "no evidence of esophageal stasis or obstruction." She remained out of hospital care and in her residence until she called for and was taken by an ambulance to an alternate, local hospital (57 hours after band insertion), when gastric perforation was confirmed via x-ray and CT scans. No open surgery was attempted to repair the damage, and cardiac arrest ensued 7 hours after admission to the second hospital. The patient was pronounced dead 64 hours after LAP-band insertion. This unique case is significant, given that there were no deaths of this kind reported in The LAP-BAND(R) Adjustable Gastric Banding System Summary of Safety and Effectiveness Data by the United States Center for Devices and Radiologic Health, of the Food and Drug Administration, or in searches of the published literature.     

Sudden death caused by embolization of trophoblast from hydatidiform mole

A 16-year-old pregnant female presented to a hospital emergency room with vaginal bleeding and uterine cramping. She underwent a hysterotomy and curettage and, during the procedure, her pulse dropped from 130 to 30 beats/minute, her pO2 fell to 10 mm of mercury, and she could not be resuscitated. At autopsy, she was found to have massive pulmonary embolization of syncytiotrophoblast from a hydatidiform mole of the uterus. This is the sixth reported case of trophoblastic embolization from a hydatidiform mole ending in death. This fatal termination may occur after a period of respiratory symptoms and may occur regardless of the mode of treatment.     

Fatal angioedema associated with captopril.

A markedly hypertensive, 70-year-old, black man had been on captopril for 2 years when he rapidly developed obstructive angioedema. The initial sign of difficulty in understanding his speech progressed to severe laryngeal and glossal edema over a 3 1/2 h period. His airway became obstructed less than a minute after arrival at the emergency room. Oral intubation was unsuccessful, and a difficult tracheostomy was too late to save the patient. The death was reported to the medical examiner because of its sudden and unusual nature. The risk of angioedema while on angiotensin converting enzyme inhibitor therapy has been noted previously in the clinical literature. Because of the sudden onset and possible confusion with an allergic reaction, this entity is brought to the attention of the forensic medical community.     

Sudden death in a 27-year-old man with Chiari I malformation

We present a case of a witnessed sudden death of a 27-year-old adult man with no antecedent trauma who subsequently was found to have a previously undiagnosed Chiari I malformation. Cases of sudden unprovoked respiratory collapse in children and adults with Chiari I malformation have been well documented, leading to death in some children. There have also been rare examples of sudden death in adults with Chiari I malformation; however, these decedents experienced recent trauma. This is a unique example of a witnessed sudden death of an adult with previously undiagnosed Chiari I malformation in the absence of trauma.     

Alleged lethal sorcery in East Timor

A wide range of cultural and social perspectives exists on the concept of sudden and unexpected death. In countries, without a formal system of death investigation, sudden death is shrouded in mysticism often based on traditional belief systems. This cultural perspective on sudden death is often at variance with medical and forensic concepts and may include explanations such as sorcery, magic, and voodoo. In this case report, the postmortem findings in an alleged victim of lethal 'black magic', known as ema halo by the indigenous people of East Timor, is described. The alleged victim died suddenly in front of witnesses. At autopsy, marked dilation of a bicuspid aortic valve with annuloaortic ectasia and a sinus of Valsalva aneurysm was found after exhumation of the body. The findings mitigated the local belief in witchcraft and established a natural manner of death.     

Bladder rupture after intentional medication overdose

We report the case of a 51-year-old woman who had a medical history of diabetes, depression with past suicide attempts, and suicidal ideation. She was found unresponsive in a motel with multiple bottles of medicines (melatonin, carisoprodol, ativan, and clonazepam) and an unopened bottle of wine. She was transported to the local hospital and treated for benzodiazepine toxicity and aspiration pneumonitis.The decedent gradually became more alert and was extubated 3 days after hospital admission. The decedent was reportedly getting up to use the restroom when she became tachypneic and diaphoretic and complained of generalized body pain. Her condition quickly declined, and she was pronounced deceased. A postmortem examination revealed an acute bladder rupture and soft tissue hemorrhage.A review of the literature reveals that isolated bladder rupture after minimal or no trauma in association with alcohol or drug ingestion is an infrequently reported, but recognized, injury. The diagnosis of bladder rupture should be considered in a patient with lower abdominal pain, even without a history of trauma. A history of voiding or bladder dysfunction should increase the suspicion for this injury. If suspected, a retrograde cystogram should be obtained promptly. Failure to consider and recognize this injury may lead to significant morbidity.