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Calcific constrictive pericarditis - A case report
ABSTRACTS
Reference
1. Pichon B, Hill R, Laurent F, et al. Development of a real-time quadruplex PCR assay for simultaneous detection of nuc, Panton–Valentine leucocidin (PVL), mecA and homologue mecALGA251. J Antimicrob Chemother 2012; 67: 2338–41.
A CASE OF DELAYED DIAGNOSIS OF BROWN SNAKE ENVENOMATION Leah Clifton and Jane Vuletic Newcastle Department of Forensic Medicine, Newcastle, NSW, Australia The true global incidence of snake envenomation is unknown, however there is an estimated minimum of 421,000 snake envenomations each year and up to 20,000 deaths worldwide. In Australasia, the annual incidence of snake envenomation is thought to be 4–5 per 100,000, with an average mortality of 2–4 per 100,000 each year. In Australia the Common or Eastern Brown Snake (Pseudonaja textilis) is the most frequently reported culprit of snake envenomations and is the leading cause of snakebite related deaths. The mechanism of death described classically is early cardiovascular collapse or arrest due to suspected massive thrombosis, venom-induced coagulopathy with secondary haemorrhage or multi-organ failure. This poster discusses the case of a 59-year-old woman with a delayed diagnosis of brown snake envenomation who developed complete venom-induced consumption coagulopathy and a catastrophic intracranial haemorrhage. This death was the first snakebite related death in the Hunter Valley region of New South Wales in over a decade and highlighted the need for increased vigilance and clinical awareness of the possibility of a snake bite, and prompted a review of methods of detection of snake envenomation in the post-mortem setting.
SUDDEN DEATH FROM AN EPENDYMOMA A. Devadass1, S. Kirkwood1 and D. Kenwright2 1Department of Anatomical Pathology, Medlab Central, Palmerston North, and 2Department of Anatomical Pathology, Capital and Coast DHB, Wellington, New Zealand Aims: We present the first reported case of sudden death in a man with an ependymoma of the third ventricle causing acute obstructive hydrocephalus. Methods: A 61-year-old male with a history of significant tremors, fits and a recent viral illness was found dead at home a day after he was last seen alive. His medications included augmentin, roxithromycin, paracetamol, diazepam and prednisone. Autopsy showed a slightly oedematous brain weighing 1840 g. On sectioning the brain, copious amounts of cerebrospinal fluid (CSF) extruded. The third and lateral ventricles were significantly dilated. His fourth ventricle was not dilated. An 18 mm exophytic soft tan mass occupying the third ventricle was found. Apart from mild coronary atherosclerosis, there were no other significant findings. Results: Histological examination showed a lesion composed of cells with uniform oval nuclei and scant cytoplasm. Perivascular pseudorosettes were present. The periventricular cerebral tissue showed evidence of oedema. Discussion: This is the first reported case of sudden death occurring in the setting of a third ventricular ependymoma. The features which support this cause include a significant mass lesion in the
S89
third ventricle, significantly enlarged lateral ventricles, periventricular cerebral oedema and a normal sized fourth ventricle.
LEFT VENTRICULAR PREDOMINANT ARVC: DIAGNOSIS IN THE AUTOPSY SETTING AND SUBSEQUENT FAMILY FOLLOW UP Linda Iles1, Natalie Morgan1 and Mary Sheppard2 1Victorian Institute of Forensic Medicine, Melbourne, Vic, Australia, and 2Department of Histopathology, Royal Brompton Hospital and Harefield NHS Foundation Trust, London, United Kingdom We present a series of seven cases of sudden death in previously asymptomatic individuals in whom a pathological diagnosis of left ventricular predominant arrhythmogenic right ventricular cardiomyopathy (ARVC) was made following autopsy. We document the results of subsequent follow up of available first degree relatives, including family history, the results of genetic testing (where available) and the results of clinical cardiological follow up of surviving family members. The recognition of ARVC where left ventricular pathology predominates is important in facilitating the diagnosis of other at risk family members.
A POST-MORTEM CASE OF SUBPHRENIC AND PERINEPHRIC ABSCESSES IN ASSOCIATION WITH ACUTE PANCREATITIS Raha Madadi Ghahan and Allan David Cala Newcastle Department of Forensic Medicine, John Hunter Hospital, Newcastle, NSW, Australia Introduction: The sub-phrenic space is defined as the area below the diaphragm and above the transverse colon. Most sub-phrenic abscesses are secondary to direct contamination of this space after abdominal surgery or trauma. Pancreatitis is a rare cause of subphrenic abscess with only few cases having been reported in the literature. Case presentation: We present a post-mortem case of a 60-yearold Caucasian man with an extensive history of alcohol use who was found dead by police. Autopsy examination revealed a large sub-phrenic abscess extending to right peri-nephric fat and the right adrenal gland. The shrunken pancreas was covered with pus. Extensive chalky fat necrosis was present in the abdominal cavity. The histological examination of the pancreas showed features of acute suppurative pancreatitis with abscess formation on the background of chronic pancreatitis. Gram stain showed numerous Gram negative cocci which were sub-typed as Klebsiella pneumonia and Strep aglacia in microbiological examination of abdominal swabs. Conclusion: Sub-phrenic abscess has a high mortality rate of about 30%. Early diagnosis and appropriate imaging are helpful tools to decrease the mortality.
CALCIFIC CONSTRICTIVE PERICARDITIS – A CASE REPORT Gretchen Pomare and Paul Morrow Department of Forensic Pathology, LabPlus, Auckland City Hospital, Auckland, New Zealand
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
S90
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Calcific constrictive pericarditis is a restrictive cardiomyopathy due to the formation of a non-compliant shell around the heart, usually secondary to an underlying inflammatory process resulting in pericardial fibrosis and calcification. We report the case of a 57-year-old Samoan male who died suddenly while exerting himself. At the time of autopsy, there was a vague history of heart surgery and an old scar was present over his anterior chest. The pericardium showed a large band of calcification with obliteration of the pericardial space. An old healed infarct was seen in the cerebellum. Review of historical medical notes revealed a prolonged hospital admission 38 years prior for pericardial effusions and constrictive pericarditis requiring pericardiectomy. Tests for tuberculosis were negative and histology of the pericardium showed non-specific inflammation and fibrosis. Post-operatively, the patient had a cardiac arrest. After discharge, the patient remained asymptomatic until his sudden death many years later. Calcific constrictive pericarditis has decreased in incidence in line with the decreased incidence of tuberculosis. Other causes include cardiovascular surgery, radiation therapy, fungal disease and chronic renal failure. However, more that half of cases are idiopathic with no identifiable cause found. LHERMITTE–DUCLOS DISEASE AND COWDEN SYNDROME – CASE REPORT Loui Rassam and Tim Lyons Department of Forensic Medicine, Newcastle, NSW, Australia Introduction: Dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos Disease) is a rare lesion that unilaterally enlarges the cerebellum, but maintains its cortical foliar architecture. Cowden syndrome (CS) is a multi-system hamartomatous overgrowth of tissues of all three embryonic origins. About 80% of patients with CS have an identifiable germ line mutation in the phosphatase and tensin homolog (PTEN) gene. Case report: We report a case of Lhermitte–Duclos disease as part of Cowden syndrome diagnosed at post-mortem examination of a 42-year-old woman who died of bilateral bronchopneumonia. As a result of this diagnosis, her family was referred for genetic counselling and testing for PTEN gene was recommended. Aim and discussion: Cowden syndrome patients are at increased risk of developing breast, thyroid, and endometrial cancers. Genetic testing for PTEN gene is widely available. Recognition of this syndrome at post-mortem examination offers family members the chance of PTEN testing, regular screening and early detection of cancer. MASSIVE BILATERAL ADRENAL HAEMORRHAGE IN THE SETTING OF SEPTICAEMIA Kyra Thompson1 and Beng Ong2 1Pathology Queensland, Queensland Health, Brisbane, and 2Forensic and Scientific Services, Queensland Health, Brisbane, Qld, Australia Bilateral adrenal haemorrhage is an uncommon finding in the adult autopsy. Causes include stressful illnesses, haemorrhagic diatheses and thromboembolic disease. We present an example of massive bilateral adrenal haemorrhage seen radiographically and at autopsy in a 76-year-old woman with sepsis.
Pathology (2014), 46(S1)
SYSTEMIC SCLEROSIS (SCLERODERMA): AN INSIGHT INTO A RARE CONDITION AND CAUSE OF DEATH Y. L. Tang1 and M. Wang2 1Department of Pathology, Tan Tock Seng Hospital, and 2Forensic Medicine Division, Applied Sciences Group, Health Sciences Authority, Singapore Systemic sclerosis is a debilitating and rare autoimmune disease with only approximately 200 cases in Singapore (from 2008– 2012). Cardiovascular and pulmonary involvement remain the leading causes of mortality in these patients. We present a 46-year-old Chinese male who was found dead in his home. He had presented to the emergency department with generalised weakness, dysphagia and skin thickening prior to his demise, prompting a presumptive diagnosis of scleroderma. He also had anaemia and mild transaminitis. Significant autopsy findings included thickened skin and extensive adhesions in the pleural cavity. Histological sections showed extensive subendocardial fibrosis with vasculopathy affecting the intramural myocardial vessels as well as pulmonary arterial hypertension. Fibrosis involving the wall of the oesophagus and the dermis was also present. An interesting finding was that of extensive hyalinisation of the lymph nodes in the deceased. Our findings concur with those described previously in literature and the likely cause of mortality in our case was due to extensive cardiac and pulmonary involvement. Most of the mortalities occurring from this condition are not subjected to the coroner’s jurisdiction. However, the rare case that presents at the autopsy suite does provide an educational insight to the pathology behind the condition. INCREASING BLOOD ALCOHOL DETECTION AND BLOOD ALCOHOL LEVELS IN SUICIDAL HANGING FROM 2007 TO 2011 R. Tse, R. Lai and A. D. Cala Newcastle Department of Forensic Medicine, John Hunter Hospital, Newcastle, NSW, Australia Aim: It is well known that alcohol intoxication can be a risk factor for suicidal behaviour. This study was undertaken to investigate whether there have been recent changes or trends in relation to blood alcohol levels (BAL) in cases of suicidal hanging, the most frequent suicide method in Australia. Methods: A 5-year retrospective study (2007–2011) was conducted of all non-decomposed suicidal hanging cases at the Newcastle Department of Forensic Medicine, NSW, Australia. Results: A total of 247 suicidal hanging cases (M:F ¼ 8.5:1; mean age 41.3 years) were identified. In 2007, there were 36.73% of cases with alcohol detected (mean BAL of 0.048 g/100 mL) of which 22.45% had a BAL of > 0.1 g/100 mL. This increased to 56.14% of cases with alcohol detected (mean BAL of 0.070 g/100 mL) and 35.09% with a BAL of > 0.1 g/100 mL in 2011. Discussion: There has been a noticeable increase in alcohol intoxication in cases of suicidal hanging during the study period. More people, mainly males, consumed more alcohol prior to suicide in 2011 in comparison with 2007. The reasons for the increase are complex and multiple. Further investigation is warranted to investigate whether this trend is observed nationwide and the reasons for this change.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
Reference
1. Pichon B, Hill R, Laurent F, et al. Development of a real-time quadruplex PCR assay for simultaneous detection of nuc, Panton–Valentine leucocidin (PVL), mecA and homologue mecALGA251. J Antimicrob Chemother 2012; 67: 2338–41.
A CASE OF DELAYED DIAGNOSIS OF BROWN SNAKE ENVENOMATION Leah Clifton and Jane Vuletic Newcastle Department of Forensic Medicine, Newcastle, NSW, Australia The true global incidence of snake envenomation is unknown, however there is an estimated minimum of 421,000 snake envenomations each year and up to 20,000 deaths worldwide. In Australasia, the annual incidence of snake envenomation is thought to be 4–5 per 100,000, with an average mortality of 2–4 per 100,000 each year. In Australia the Common or Eastern Brown Snake (Pseudonaja textilis) is the most frequently reported culprit of snake envenomations and is the leading cause of snakebite related deaths. The mechanism of death described classically is early cardiovascular collapse or arrest due to suspected massive thrombosis, venom-induced coagulopathy with secondary haemorrhage or multi-organ failure. This poster discusses the case of a 59-year-old woman with a delayed diagnosis of brown snake envenomation who developed complete venom-induced consumption coagulopathy and a catastrophic intracranial haemorrhage. This death was the first snakebite related death in the Hunter Valley region of New South Wales in over a decade and highlighted the need for increased vigilance and clinical awareness of the possibility of a snake bite, and prompted a review of methods of detection of snake envenomation in the post-mortem setting.
SUDDEN DEATH FROM AN EPENDYMOMA A. Devadass1, S. Kirkwood1 and D. Kenwright2 1Department of Anatomical Pathology, Medlab Central, Palmerston North, and 2Department of Anatomical Pathology, Capital and Coast DHB, Wellington, New Zealand Aims: We present the first reported case of sudden death in a man with an ependymoma of the third ventricle causing acute obstructive hydrocephalus. Methods: A 61-year-old male with a history of significant tremors, fits and a recent viral illness was found dead at home a day after he was last seen alive. His medications included augmentin, roxithromycin, paracetamol, diazepam and prednisone. Autopsy showed a slightly oedematous brain weighing 1840 g. On sectioning the brain, copious amounts of cerebrospinal fluid (CSF) extruded. The third and lateral ventricles were significantly dilated. His fourth ventricle was not dilated. An 18 mm exophytic soft tan mass occupying the third ventricle was found. Apart from mild coronary atherosclerosis, there were no other significant findings. Results: Histological examination showed a lesion composed of cells with uniform oval nuclei and scant cytoplasm. Perivascular pseudorosettes were present. The periventricular cerebral tissue showed evidence of oedema. Discussion: This is the first reported case of sudden death occurring in the setting of a third ventricular ependymoma. The features which support this cause include a significant mass lesion in the
S89
third ventricle, significantly enlarged lateral ventricles, periventricular cerebral oedema and a normal sized fourth ventricle.
LEFT VENTRICULAR PREDOMINANT ARVC: DIAGNOSIS IN THE AUTOPSY SETTING AND SUBSEQUENT FAMILY FOLLOW UP Linda Iles1, Natalie Morgan1 and Mary Sheppard2 1Victorian Institute of Forensic Medicine, Melbourne, Vic, Australia, and 2Department of Histopathology, Royal Brompton Hospital and Harefield NHS Foundation Trust, London, United Kingdom We present a series of seven cases of sudden death in previously asymptomatic individuals in whom a pathological diagnosis of left ventricular predominant arrhythmogenic right ventricular cardiomyopathy (ARVC) was made following autopsy. We document the results of subsequent follow up of available first degree relatives, including family history, the results of genetic testing (where available) and the results of clinical cardiological follow up of surviving family members. The recognition of ARVC where left ventricular pathology predominates is important in facilitating the diagnosis of other at risk family members.
A POST-MORTEM CASE OF SUBPHRENIC AND PERINEPHRIC ABSCESSES IN ASSOCIATION WITH ACUTE PANCREATITIS Raha Madadi Ghahan and Allan David Cala Newcastle Department of Forensic Medicine, John Hunter Hospital, Newcastle, NSW, Australia Introduction: The sub-phrenic space is defined as the area below the diaphragm and above the transverse colon. Most sub-phrenic abscesses are secondary to direct contamination of this space after abdominal surgery or trauma. Pancreatitis is a rare cause of subphrenic abscess with only few cases having been reported in the literature. Case presentation: We present a post-mortem case of a 60-yearold Caucasian man with an extensive history of alcohol use who was found dead by police. Autopsy examination revealed a large sub-phrenic abscess extending to right peri-nephric fat and the right adrenal gland. The shrunken pancreas was covered with pus. Extensive chalky fat necrosis was present in the abdominal cavity. The histological examination of the pancreas showed features of acute suppurative pancreatitis with abscess formation on the background of chronic pancreatitis. Gram stain showed numerous Gram negative cocci which were sub-typed as Klebsiella pneumonia and Strep aglacia in microbiological examination of abdominal swabs. Conclusion: Sub-phrenic abscess has a high mortality rate of about 30%. Early diagnosis and appropriate imaging are helpful tools to decrease the mortality.
CALCIFIC CONSTRICTIVE PERICARDITIS – A CASE REPORT Gretchen Pomare and Paul Morrow Department of Forensic Pathology, LabPlus, Auckland City Hospital, Auckland, New Zealand
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
S90
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Calcific constrictive pericarditis is a restrictive cardiomyopathy due to the formation of a non-compliant shell around the heart, usually secondary to an underlying inflammatory process resulting in pericardial fibrosis and calcification. We report the case of a 57-year-old Samoan male who died suddenly while exerting himself. At the time of autopsy, there was a vague history of heart surgery and an old scar was present over his anterior chest. The pericardium showed a large band of calcification with obliteration of the pericardial space. An old healed infarct was seen in the cerebellum. Review of historical medical notes revealed a prolonged hospital admission 38 years prior for pericardial effusions and constrictive pericarditis requiring pericardiectomy. Tests for tuberculosis were negative and histology of the pericardium showed non-specific inflammation and fibrosis. Post-operatively, the patient had a cardiac arrest. After discharge, the patient remained asymptomatic until his sudden death many years later. Calcific constrictive pericarditis has decreased in incidence in line with the decreased incidence of tuberculosis. Other causes include cardiovascular surgery, radiation therapy, fungal disease and chronic renal failure. However, more that half of cases are idiopathic with no identifiable cause found. LHERMITTE–DUCLOS DISEASE AND COWDEN SYNDROME – CASE REPORT Loui Rassam and Tim Lyons Department of Forensic Medicine, Newcastle, NSW, Australia Introduction: Dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos Disease) is a rare lesion that unilaterally enlarges the cerebellum, but maintains its cortical foliar architecture. Cowden syndrome (CS) is a multi-system hamartomatous overgrowth of tissues of all three embryonic origins. About 80% of patients with CS have an identifiable germ line mutation in the phosphatase and tensin homolog (PTEN) gene. Case report: We report a case of Lhermitte–Duclos disease as part of Cowden syndrome diagnosed at post-mortem examination of a 42-year-old woman who died of bilateral bronchopneumonia. As a result of this diagnosis, her family was referred for genetic counselling and testing for PTEN gene was recommended. Aim and discussion: Cowden syndrome patients are at increased risk of developing breast, thyroid, and endometrial cancers. Genetic testing for PTEN gene is widely available. Recognition of this syndrome at post-mortem examination offers family members the chance of PTEN testing, regular screening and early detection of cancer. MASSIVE BILATERAL ADRENAL HAEMORRHAGE IN THE SETTING OF SEPTICAEMIA Kyra Thompson1 and Beng Ong2 1Pathology Queensland, Queensland Health, Brisbane, and 2Forensic and Scientific Services, Queensland Health, Brisbane, Qld, Australia Bilateral adrenal haemorrhage is an uncommon finding in the adult autopsy. Causes include stressful illnesses, haemorrhagic diatheses and thromboembolic disease. We present an example of massive bilateral adrenal haemorrhage seen radiographically and at autopsy in a 76-year-old woman with sepsis.
Pathology (2014), 46(S1)
SYSTEMIC SCLEROSIS (SCLERODERMA): AN INSIGHT INTO A RARE CONDITION AND CAUSE OF DEATH Y. L. Tang1 and M. Wang2 1Department of Pathology, Tan Tock Seng Hospital, and 2Forensic Medicine Division, Applied Sciences Group, Health Sciences Authority, Singapore Systemic sclerosis is a debilitating and rare autoimmune disease with only approximately 200 cases in Singapore (from 2008– 2012). Cardiovascular and pulmonary involvement remain the leading causes of mortality in these patients. We present a 46-year-old Chinese male who was found dead in his home. He had presented to the emergency department with generalised weakness, dysphagia and skin thickening prior to his demise, prompting a presumptive diagnosis of scleroderma. He also had anaemia and mild transaminitis. Significant autopsy findings included thickened skin and extensive adhesions in the pleural cavity. Histological sections showed extensive subendocardial fibrosis with vasculopathy affecting the intramural myocardial vessels as well as pulmonary arterial hypertension. Fibrosis involving the wall of the oesophagus and the dermis was also present. An interesting finding was that of extensive hyalinisation of the lymph nodes in the deceased. Our findings concur with those described previously in literature and the likely cause of mortality in our case was due to extensive cardiac and pulmonary involvement. Most of the mortalities occurring from this condition are not subjected to the coroner’s jurisdiction. However, the rare case that presents at the autopsy suite does provide an educational insight to the pathology behind the condition. INCREASING BLOOD ALCOHOL DETECTION AND BLOOD ALCOHOL LEVELS IN SUICIDAL HANGING FROM 2007 TO 2011 R. Tse, R. Lai and A. D. Cala Newcastle Department of Forensic Medicine, John Hunter Hospital, Newcastle, NSW, Australia Aim: It is well known that alcohol intoxication can be a risk factor for suicidal behaviour. This study was undertaken to investigate whether there have been recent changes or trends in relation to blood alcohol levels (BAL) in cases of suicidal hanging, the most frequent suicide method in Australia. Methods: A 5-year retrospective study (2007–2011) was conducted of all non-decomposed suicidal hanging cases at the Newcastle Department of Forensic Medicine, NSW, Australia. Results: A total of 247 suicidal hanging cases (M:F ¼ 8.5:1; mean age 41.3 years) were identified. In 2007, there were 36.73% of cases with alcohol detected (mean BAL of 0.048 g/100 mL) of which 22.45% had a BAL of > 0.1 g/100 mL. This increased to 56.14% of cases with alcohol detected (mean BAL of 0.070 g/100 mL) and 35.09% with a BAL of > 0.1 g/100 mL in 2011. Discussion: There has been a noticeable increase in alcohol intoxication in cases of suicidal hanging during the study period. More people, mainly males, consumed more alcohol prior to suicide in 2011 in comparison with 2007. The reasons for the increase are complex and multiple. Further investigation is warranted to investigate whether this trend is observed nationwide and the reasons for this change.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.